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A Second-line Option for Previously Treated Radioiodine-Refractory Differentiated Thyroid Cancer (DTC)
In this special supplement to Federal Practitioner, Dr. Jameel Muzaffar, MD shares insights into a second-line treatment option for previously treated radioiodine-refractory differentiated thyroid cancer (DTC), along with an exploratory analysis of BRAF mutation status. It discusses the challenges associated with metastatic DTC and the significance of understanding factors like BRAF mutation status in treatment decisions. Additionally, it highlights the efficacy and safety profiles of the treatment option.
CA-3326
In this special supplement to Federal Practitioner, Dr. Jameel Muzaffar, MD shares insights into a second-line treatment option for previously treated radioiodine-refractory differentiated thyroid cancer (DTC), along with an exploratory analysis of BRAF mutation status. It discusses the challenges associated with metastatic DTC and the significance of understanding factors like BRAF mutation status in treatment decisions. Additionally, it highlights the efficacy and safety profiles of the treatment option.
CA-3326
In this special supplement to Federal Practitioner, Dr. Jameel Muzaffar, MD shares insights into a second-line treatment option for previously treated radioiodine-refractory differentiated thyroid cancer (DTC), along with an exploratory analysis of BRAF mutation status. It discusses the challenges associated with metastatic DTC and the significance of understanding factors like BRAF mutation status in treatment decisions. Additionally, it highlights the efficacy and safety profiles of the treatment option.
CA-3326
Barriers to Mohs Micrographic Surgery in Japanese Patients With Basal Cell Carcinoma
Margin-controlled surgery for squamous cell carcinoma (SCC) on the lower lip was first performed by Dr. Frederic Mohs on June 30, 1936. Since then, thousands of skin cancer surgeons have refined and adopted the technique. Due to the high cure rate and sparing of normal tissue, Mohs micrographic surgery (MMS) has become the gold standard treatment for facial and special-site nonmelanoma skin cancer worldwide. Mohs micrographic surgery is performed on more than 876,000 tumors annually in the United States.1 Among 3.5 million Americans diagnosed with nonmelanoma skin cancer in 2006, one-quarter were treated with MMS.2 In Japan, basal cell carcinoma (BCC) is the most common skin malignancy, with an incidence of 3.34 cases per 100,000 individuals; SCC is the second most common, with an incidence of 2.5 cases per 100,000 individuals.3
The essential element that makes MMS unique is the careful microscopic examination of the entire margin of the removed specimen. Tissue processing is done with careful en face orientation to ensure that circumferential and deep margins are entirely visible. The surgeon interprets the slides and proceeds to remove the additional tumor as necessary. Because the same physician performs both the surgery and the pathologic assessment throughout the procedure, a precise correlation between the microscopic and surgical findings can be made. The surgeon can begin with smaller margins, removing minimal healthy tissue while removing all the cancer cells, which results in the smallest-possible skin defect and the best prognosis for the malignancy (Figure 1).
At the only facility in Japan offering MMS, the lead author (S.S.) has treated 52 lesions with MMS in 46 patients (2020-2022). Of these patients, 40 were White, 5 were Japanese, and 1 was of African descent. In this case series, we present 5 Japanese patients who had BCC treated with MMS.
Case Series
Patient 1—A 50-year-old Japanese woman presented to dermatology with a brown papule on the nasal tip of 1.25 year’s duration (Figure 2). A biopsy revealed infiltrative BCC (Figure 3), and the patient was referred to the dermatology department at a nearby university hospital. Because the BCC was an aggressive variant, wide local excision (WLE) with subsequent flap reconstruction was recommended as well as radiation therapy. The patient learned about MMS through an internet search and refused both options, seeking MMS treatment at our clinic. Although Japanese health insurance does not cover MMS, the patient had supplemental private insurance that did cover the cost. She provided consent to undergo the procedure. Physical examination revealed a 7.5×6-mm, brown-red macule with ill-defined borders on the tip of the nose. We used a 1.5-mm margin for the first stage of MMS (Figure 4A). The frozen section revealed that the tumor had been entirely excised in the first stage, leaving only a 10.5×9-mm skin defect that was reconstructed with a Dufourmentel flap (Figure 4B). No signs of recurrence were noted at 3.5-year follow-up, and the cosmetic outcome was favorable (Figure 4C). National Comprehensive Cancer Network guidelines recommend a margin greater than 4 mm for infiltrative BCCs4; therefore, our technique reduced the total defect by at least 4 mm in a cosmetically sensitive area. The patient also did not need radiation therapy, which reduced morbidity. She continues to be recurrence free at 3.5-year follow-up.
Patient 2—A 63-year-old Japanese man presented to dermatology with a brown macule on the right lower eyelid of 2 years’ duration. A biopsy of the lesion was positive for nodular BCC. After being advised to undergo WLE and extensive reconstruction with plastic surgery, the patient learned of MMS through an internet search and found our clinic. Physical examination revealed a 7×5-mm brown macule on the right lower eyelid. The patient had supplemental private insurance that covered the cost of MMS, and he provided consent for the procedure. A 1.5-mm margin was taken for the first stage, resulting in a 10×8-mm defect superficial to the orbicularis oculi muscle. The frozen section revealed residual tumor exposure in the dermis at the 9- to 10-o’clock position. A second-stage excision was performed to remove an additional 1.5 mm of skin at the 9- to 12-o’clock position with a thin layer of the orbicularis oculi muscle. The subsequent histologic examination revealed no residual BCC, and the final 13×9-mm skin defect was reconstructed with a rotation flap. There were no signs of recurrence at 2.5-year follow-up with an excellent cosmetic outcome.
Patient 3—A 73-year-old Japanese man presented to a local university dermatology clinic with a new papule on the nose. The dermatologist suggested WLE with 4-mm margins and reconstruction of the skin defect 2 weeks later by a plastic surgeon. The patient was not satisfied with the proposed surgical plan, which led him to learn about MMS on the internet; he subsequently found our clinic. Physical examination revealed a 4×3.5-mm brown papule on the tip of the nose. He understood the nature of MMS and chose to pay out-of-pocket because Japanese health insurance did not cover the procedure. We used a 2-mm margin for the first stage, which created a 7.5×7-mm skin defect. The frozen section pathology revealed no residual BCC at the cut surface. The skin defect was reconstructed with a Limberg rhombic flap. There were no signs of recurrence at 1.5-year follow-up with a favorable cosmetic outcome.
Patient 4—A 45-year-old man presented to a dermatology clinic with a papule on the right side of the nose of 1 year’s duration. A biopsy revealed the lesion was a nodular BCC. The dermatologist recommended WLE at a general hospital, but the patient refused after learning about MMS. He subsequently made an appointment with our clinic. Physical examination revealed a 7×4-mm white papule on the right side of the nose. The patient had private insurance that covered the cost of MMS. The first stage was performed with 1.5-mm margins and was clear of residual tumor. A Limberg rhombic flap from the adjacent cheek was used to repair the final 10×7-mm skin defect. There were no signs of recurrence at 1 year and 9 months’ follow-up with a favorable cosmetic outcome.
Patient 5—A 76-year-old Japanese woman presented to a university hospital near Tokyo with a black papule on the left cutaneous lip of 5 years’ duration. A biopsy revealed nodular BCC, and WLE with flap reconstruction was recommended. The patient’s son learned about MMS through internet research and referred her to our clinic. Physical examination revealed a 7×5-mm black papule on the left upper lip. The patient’s private insurance covered the cost of MMS, and she consented to the procedure. We used a 2-mm initial margin, and the immediate frozen section revealed no signs of BCC at the cut surface. The 11×9-mm skin defect was reconstructed with a Limberg rhombic flap. There were no signs of recurrence at 1.5-year follow-up with a favorable cosmetic outcome.
Comment
We presented 5 cases of MMS in Japanese patients with BCC. More than 7000 new cases of nonmelanoma skin cancer occur every year in Japan.3 Only 0.04% of these cases—the 5 cases presented here—were treated with MMS in Japan in 2020 and 2021, in contrast to 25% in the United States in 2006.2
MMS vs Other BCC Treatments—Mohs micrographic surgery offers 2 distinct advantages over conventional excision: an improved cure rate while achieving a smaller final defect size, generally leading to better cosmetic outcomes. Overall 5-year recurrence rates of BCC are 10% for conventional surgical excision vs 1% for MMS, while the recurrence rates for SCC are 8% and 3%, respectively.5 A study of well-demarcated BCCs smaller than 2 cm that were treated with MMS with 2-mm increments revealed that 95% of the cases were free of malignancy within a 4-mm margin of the normal-appearing skin surrounding the tumor.6 Several articles have reported a 95% cure rate or higher with conventional excision of localized BCC,7 but 4- to 5-mm excision margins are required, resulting in a greater skin defect and a lower cure rate compared to MMS.
Aggressive subtypes of BCC have a higher recurrence rate. Rowe et al8 reported the following 5-year recurrence rates: 5.6% for MMS, 17.4% for conventional surgical excision, 40.0% for curettage and electrodesiccation, and 9.8% for radiation therapy. Primary BCCs with high-risk histologic subtypes has a 10-year recurrence rate of 4.4% with MMS vs 12.2% with conventional excision.9 These findings reveal that MMS yields a better prognosis compared to traditional treatment methods for recurrent BCCs and BCCs of high-risk histologic subtypes.
The primary reason for the excellent cure rate seen in MMS is the ability to perform complete margin assessment. Peripheral and deep en face margin assessment (PDEMA) is crucial in achieving high cure rates with narrow margins. In WLE (Figure 1), vertical sectioning (also known as bread-loafing) does not achieve direct visualization of the entire surgical margin, as this technique only evaluates random sections and does not achieve PDEMA.10 The bread-loafing method is used almost exclusively in Japan and visualizes only 0.1% of the entire margin compared to 100% with MMS.11 Beyond the superior cure rate, the MMS technique often yields smaller final defects compared to WLE. All 5 of our patients achieved complete tumor removal while sparing more normal tissue compared to conventional WLE, which takes at least a 4-mm margin in all directions.
Barriers to Adopting MMS in Japan—There are many barriers to the broader adoption of MMS in Japan. A guideline of the Japanese Dermatological Association says, MMS “is complicated, requires special training for acquisition, and requires time and labor for implementation of a series of processes, and it has not gained wide acceptance in Japan because of these disadvantages.”3 There currently are no MMS training programs in Japan. We refute this statement from the Japanese Dermatological Association because, in our experience, only 1 surgeon plus a single histotechnician familiar with MMS is sufficient for a facility to offer the procedure (the lead author of this study [S.S.] acts as both the surgeon and the histotechnician). Another misconception among some physicians in Japan is that cancer on ethnically Japanese skin is uniquely suited to excision without microscopic verification of tumor clearance because the borders of the tumors are easily identified, which was based on good cure rates for the excision of well-demarcated pigmented BCCs in a Japanese cohort. This study of a Japanese cohort investigated the specimens with the conventional bread-loafing technique but not with the PDEMA.12
Eighty percent (4/5) of our patients presented with nodular BCC, and only 1 required a second stage. In comparison, we also treated 16 White patients with nodular BCC with MMS during the same period, and 31% (5/16) required more than 1 stage, with 1 patient requiring 3 stages. This cohort, however, is too small to demonstrate a statistically significant difference (S.S., unpublished data, 2020-2022).
A study in Singapore reported the postsurgical complication rate and 5-year recurrence rate for 481 tumors (92% BCC and 7.5% SCC). The median follow-up duration after MMS was 36 months, and the recurrence rate was 0.6%. The postsurgical complications included 11 (2.3%) cases with superficial tip necrosis of surgical flaps/grafts, 2 (0.4%) with mild wound dehiscence, 1 (0.2%) with minor surgical site bleeding, and 1 (0.2%) with minor wound infection.13 This study supports the notion that MMS is equally effective for Asian patients.
Awareness of MMS in Japan is lacking, and most Japanese dermatologists do not know about the technique. All 5 patients in our case series asked their dermatologists about alternative treatment options and were not offered MMS. In each case, the patients learned of the technique through internet research.
The lack of insurance reimbursement for MMS in Japan is another barrier. Because the national health insurance does not reimburse for MMS, the procedure is relatively unavailable to most Japanese citizens who cannot pay out-of-pocket for the treatment and do not have supplemental insurance. Mohs micrographic surgery may seem expensive compared to WLE followed by repair; however, in the authors’ experience, in Japan, excision without MMS may require general sedation and multiple surgeries to reconstruct larger skin defects, leading to greater morbidity and risk for the patient.
Conclusion
Mohs micrographic surgery in Japan is in its infancy, and further studies showing recurrence rates and long-term prognosis are needed. Such data should help increase awareness of MMS among Japanese physicians as an excellent treatment option for their patients. Furthermore, as Japan becomes more heterogenous as a society and the US Military increases its presence in the region, the need for MMS is likely to increase.
Acknowledgments—We appreciate the proofreading support by Mark Bivens, MBA, MSc (Tokyo, Japan), as well as the technical support from Ben Tallon, MBChB, and Robyn Mason (both in Tauranga, New Zealand) to start MMS at our clinic.
- Asgari MM, Olson J, Alam M. Needs assessment for Mohs micrographic surgery. Dermatol Clin. 2012;30:167-175. doi:10.1016/j.det.2011.08.010
- Connolly SM, Baker DR, Baker DR, et al. AAD/ACMS/ASDSA/ASMS 2012 appropriate use criteria for Mohs micrographic surgery: a report of the American Academy of Dermatology, American College of Mohs Surgery, American Society for Dermatologic Surgery Association, and the American Society for Mohs Surgery. J Am Acad Dermatol. 2012;67:531-550.
- Ansai SI, Umebayashi Y, Katsumata N, et al. Japanese Dermatological Association Guidelines: outlines of guidelines for cutaneous squamous cell carcinoma 2020. J Dermatol. 2021;48:E288-E311.
- Schmults CD, Blitzblau R, Aasi SZ, et at. Basal cell skin cancer, version 2.2024, NCCN Clinical Practice Guidelines in Oncology. J Natl Compr Canc Netw. 2023;21:1181-1203. doi:10.6004/jncn.2023.0056
- Snow SN, Gunkel J. Mohs surgery. In: Bolognia JL, Schaffer JV, Cerroni L, eds. Dermatology. 4th ed. Elsevier; 2017:2445-2455. doi:10.1016/b978-0-070-94171-3.00041-7
- Wolf DJ, Zitelli JA. Surgical margins for basal cell carcinoma. Arch Dermatol. 1987;123:340-344.
- Quazi SJ, Aslam N, Saleem H, et al. Surgical margin of excision in basal cell carcinoma: a systematic review of literature. Cureus. 2020;12:E9211.
- Rowe DE, Carroll RJ, Day Jus CL. Mohs surgery is the treatment of choice for recurrent (previously treated) basal cell carcinoma. J Dermatol Surg Oncol. 1989;15:424-431.
- Van Loo, Mosterd K, Krekels GA. Surgical excision versus Mohs’ micrographic surgery for basal cell carcinoma of the face. Eur J Cancer. 2014;50:3011-3020.
- Schmults CD, Blitzblau R, Aasi SZ, et al. NCCN Guidelines Insights: Squamous Cell Skin Cancer, Version 1.2022. J Natl Compr Canc Netw. 2021;19:1382-1394.
- Hui AM, Jacobson M, Markowitz O, et al. Mohs micrographic surgery for the treatment of melanoma. Dermatol Clin. 2012;30:503-515.
- Ito T, Inatomi Y, Nagae K, et al. Narrow-margin excision is a safe, reliable treatment for well-defined, primary pigmented basal cell carcinoma: an analysis of 288 lesions in Japan. J Eur Acad Dermatol Venereol. 2015;29:1828-1831.
- Ho WYB, Zhao X, Tan WPM. Mohs micrographic surgery in Singapore: a long-term follow-up review. Ann Acad Med Singap. 2021;50:922-923.
Margin-controlled surgery for squamous cell carcinoma (SCC) on the lower lip was first performed by Dr. Frederic Mohs on June 30, 1936. Since then, thousands of skin cancer surgeons have refined and adopted the technique. Due to the high cure rate and sparing of normal tissue, Mohs micrographic surgery (MMS) has become the gold standard treatment for facial and special-site nonmelanoma skin cancer worldwide. Mohs micrographic surgery is performed on more than 876,000 tumors annually in the United States.1 Among 3.5 million Americans diagnosed with nonmelanoma skin cancer in 2006, one-quarter were treated with MMS.2 In Japan, basal cell carcinoma (BCC) is the most common skin malignancy, with an incidence of 3.34 cases per 100,000 individuals; SCC is the second most common, with an incidence of 2.5 cases per 100,000 individuals.3
The essential element that makes MMS unique is the careful microscopic examination of the entire margin of the removed specimen. Tissue processing is done with careful en face orientation to ensure that circumferential and deep margins are entirely visible. The surgeon interprets the slides and proceeds to remove the additional tumor as necessary. Because the same physician performs both the surgery and the pathologic assessment throughout the procedure, a precise correlation between the microscopic and surgical findings can be made. The surgeon can begin with smaller margins, removing minimal healthy tissue while removing all the cancer cells, which results in the smallest-possible skin defect and the best prognosis for the malignancy (Figure 1).
At the only facility in Japan offering MMS, the lead author (S.S.) has treated 52 lesions with MMS in 46 patients (2020-2022). Of these patients, 40 were White, 5 were Japanese, and 1 was of African descent. In this case series, we present 5 Japanese patients who had BCC treated with MMS.
Case Series
Patient 1—A 50-year-old Japanese woman presented to dermatology with a brown papule on the nasal tip of 1.25 year’s duration (Figure 2). A biopsy revealed infiltrative BCC (Figure 3), and the patient was referred to the dermatology department at a nearby university hospital. Because the BCC was an aggressive variant, wide local excision (WLE) with subsequent flap reconstruction was recommended as well as radiation therapy. The patient learned about MMS through an internet search and refused both options, seeking MMS treatment at our clinic. Although Japanese health insurance does not cover MMS, the patient had supplemental private insurance that did cover the cost. She provided consent to undergo the procedure. Physical examination revealed a 7.5×6-mm, brown-red macule with ill-defined borders on the tip of the nose. We used a 1.5-mm margin for the first stage of MMS (Figure 4A). The frozen section revealed that the tumor had been entirely excised in the first stage, leaving only a 10.5×9-mm skin defect that was reconstructed with a Dufourmentel flap (Figure 4B). No signs of recurrence were noted at 3.5-year follow-up, and the cosmetic outcome was favorable (Figure 4C). National Comprehensive Cancer Network guidelines recommend a margin greater than 4 mm for infiltrative BCCs4; therefore, our technique reduced the total defect by at least 4 mm in a cosmetically sensitive area. The patient also did not need radiation therapy, which reduced morbidity. She continues to be recurrence free at 3.5-year follow-up.
Patient 2—A 63-year-old Japanese man presented to dermatology with a brown macule on the right lower eyelid of 2 years’ duration. A biopsy of the lesion was positive for nodular BCC. After being advised to undergo WLE and extensive reconstruction with plastic surgery, the patient learned of MMS through an internet search and found our clinic. Physical examination revealed a 7×5-mm brown macule on the right lower eyelid. The patient had supplemental private insurance that covered the cost of MMS, and he provided consent for the procedure. A 1.5-mm margin was taken for the first stage, resulting in a 10×8-mm defect superficial to the orbicularis oculi muscle. The frozen section revealed residual tumor exposure in the dermis at the 9- to 10-o’clock position. A second-stage excision was performed to remove an additional 1.5 mm of skin at the 9- to 12-o’clock position with a thin layer of the orbicularis oculi muscle. The subsequent histologic examination revealed no residual BCC, and the final 13×9-mm skin defect was reconstructed with a rotation flap. There were no signs of recurrence at 2.5-year follow-up with an excellent cosmetic outcome.
Patient 3—A 73-year-old Japanese man presented to a local university dermatology clinic with a new papule on the nose. The dermatologist suggested WLE with 4-mm margins and reconstruction of the skin defect 2 weeks later by a plastic surgeon. The patient was not satisfied with the proposed surgical plan, which led him to learn about MMS on the internet; he subsequently found our clinic. Physical examination revealed a 4×3.5-mm brown papule on the tip of the nose. He understood the nature of MMS and chose to pay out-of-pocket because Japanese health insurance did not cover the procedure. We used a 2-mm margin for the first stage, which created a 7.5×7-mm skin defect. The frozen section pathology revealed no residual BCC at the cut surface. The skin defect was reconstructed with a Limberg rhombic flap. There were no signs of recurrence at 1.5-year follow-up with a favorable cosmetic outcome.
Patient 4—A 45-year-old man presented to a dermatology clinic with a papule on the right side of the nose of 1 year’s duration. A biopsy revealed the lesion was a nodular BCC. The dermatologist recommended WLE at a general hospital, but the patient refused after learning about MMS. He subsequently made an appointment with our clinic. Physical examination revealed a 7×4-mm white papule on the right side of the nose. The patient had private insurance that covered the cost of MMS. The first stage was performed with 1.5-mm margins and was clear of residual tumor. A Limberg rhombic flap from the adjacent cheek was used to repair the final 10×7-mm skin defect. There were no signs of recurrence at 1 year and 9 months’ follow-up with a favorable cosmetic outcome.
Patient 5—A 76-year-old Japanese woman presented to a university hospital near Tokyo with a black papule on the left cutaneous lip of 5 years’ duration. A biopsy revealed nodular BCC, and WLE with flap reconstruction was recommended. The patient’s son learned about MMS through internet research and referred her to our clinic. Physical examination revealed a 7×5-mm black papule on the left upper lip. The patient’s private insurance covered the cost of MMS, and she consented to the procedure. We used a 2-mm initial margin, and the immediate frozen section revealed no signs of BCC at the cut surface. The 11×9-mm skin defect was reconstructed with a Limberg rhombic flap. There were no signs of recurrence at 1.5-year follow-up with a favorable cosmetic outcome.
Comment
We presented 5 cases of MMS in Japanese patients with BCC. More than 7000 new cases of nonmelanoma skin cancer occur every year in Japan.3 Only 0.04% of these cases—the 5 cases presented here—were treated with MMS in Japan in 2020 and 2021, in contrast to 25% in the United States in 2006.2
MMS vs Other BCC Treatments—Mohs micrographic surgery offers 2 distinct advantages over conventional excision: an improved cure rate while achieving a smaller final defect size, generally leading to better cosmetic outcomes. Overall 5-year recurrence rates of BCC are 10% for conventional surgical excision vs 1% for MMS, while the recurrence rates for SCC are 8% and 3%, respectively.5 A study of well-demarcated BCCs smaller than 2 cm that were treated with MMS with 2-mm increments revealed that 95% of the cases were free of malignancy within a 4-mm margin of the normal-appearing skin surrounding the tumor.6 Several articles have reported a 95% cure rate or higher with conventional excision of localized BCC,7 but 4- to 5-mm excision margins are required, resulting in a greater skin defect and a lower cure rate compared to MMS.
Aggressive subtypes of BCC have a higher recurrence rate. Rowe et al8 reported the following 5-year recurrence rates: 5.6% for MMS, 17.4% for conventional surgical excision, 40.0% for curettage and electrodesiccation, and 9.8% for radiation therapy. Primary BCCs with high-risk histologic subtypes has a 10-year recurrence rate of 4.4% with MMS vs 12.2% with conventional excision.9 These findings reveal that MMS yields a better prognosis compared to traditional treatment methods for recurrent BCCs and BCCs of high-risk histologic subtypes.
The primary reason for the excellent cure rate seen in MMS is the ability to perform complete margin assessment. Peripheral and deep en face margin assessment (PDEMA) is crucial in achieving high cure rates with narrow margins. In WLE (Figure 1), vertical sectioning (also known as bread-loafing) does not achieve direct visualization of the entire surgical margin, as this technique only evaluates random sections and does not achieve PDEMA.10 The bread-loafing method is used almost exclusively in Japan and visualizes only 0.1% of the entire margin compared to 100% with MMS.11 Beyond the superior cure rate, the MMS technique often yields smaller final defects compared to WLE. All 5 of our patients achieved complete tumor removal while sparing more normal tissue compared to conventional WLE, which takes at least a 4-mm margin in all directions.
Barriers to Adopting MMS in Japan—There are many barriers to the broader adoption of MMS in Japan. A guideline of the Japanese Dermatological Association says, MMS “is complicated, requires special training for acquisition, and requires time and labor for implementation of a series of processes, and it has not gained wide acceptance in Japan because of these disadvantages.”3 There currently are no MMS training programs in Japan. We refute this statement from the Japanese Dermatological Association because, in our experience, only 1 surgeon plus a single histotechnician familiar with MMS is sufficient for a facility to offer the procedure (the lead author of this study [S.S.] acts as both the surgeon and the histotechnician). Another misconception among some physicians in Japan is that cancer on ethnically Japanese skin is uniquely suited to excision without microscopic verification of tumor clearance because the borders of the tumors are easily identified, which was based on good cure rates for the excision of well-demarcated pigmented BCCs in a Japanese cohort. This study of a Japanese cohort investigated the specimens with the conventional bread-loafing technique but not with the PDEMA.12
Eighty percent (4/5) of our patients presented with nodular BCC, and only 1 required a second stage. In comparison, we also treated 16 White patients with nodular BCC with MMS during the same period, and 31% (5/16) required more than 1 stage, with 1 patient requiring 3 stages. This cohort, however, is too small to demonstrate a statistically significant difference (S.S., unpublished data, 2020-2022).
A study in Singapore reported the postsurgical complication rate and 5-year recurrence rate for 481 tumors (92% BCC and 7.5% SCC). The median follow-up duration after MMS was 36 months, and the recurrence rate was 0.6%. The postsurgical complications included 11 (2.3%) cases with superficial tip necrosis of surgical flaps/grafts, 2 (0.4%) with mild wound dehiscence, 1 (0.2%) with minor surgical site bleeding, and 1 (0.2%) with minor wound infection.13 This study supports the notion that MMS is equally effective for Asian patients.
Awareness of MMS in Japan is lacking, and most Japanese dermatologists do not know about the technique. All 5 patients in our case series asked their dermatologists about alternative treatment options and were not offered MMS. In each case, the patients learned of the technique through internet research.
The lack of insurance reimbursement for MMS in Japan is another barrier. Because the national health insurance does not reimburse for MMS, the procedure is relatively unavailable to most Japanese citizens who cannot pay out-of-pocket for the treatment and do not have supplemental insurance. Mohs micrographic surgery may seem expensive compared to WLE followed by repair; however, in the authors’ experience, in Japan, excision without MMS may require general sedation and multiple surgeries to reconstruct larger skin defects, leading to greater morbidity and risk for the patient.
Conclusion
Mohs micrographic surgery in Japan is in its infancy, and further studies showing recurrence rates and long-term prognosis are needed. Such data should help increase awareness of MMS among Japanese physicians as an excellent treatment option for their patients. Furthermore, as Japan becomes more heterogenous as a society and the US Military increases its presence in the region, the need for MMS is likely to increase.
Acknowledgments—We appreciate the proofreading support by Mark Bivens, MBA, MSc (Tokyo, Japan), as well as the technical support from Ben Tallon, MBChB, and Robyn Mason (both in Tauranga, New Zealand) to start MMS at our clinic.
Margin-controlled surgery for squamous cell carcinoma (SCC) on the lower lip was first performed by Dr. Frederic Mohs on June 30, 1936. Since then, thousands of skin cancer surgeons have refined and adopted the technique. Due to the high cure rate and sparing of normal tissue, Mohs micrographic surgery (MMS) has become the gold standard treatment for facial and special-site nonmelanoma skin cancer worldwide. Mohs micrographic surgery is performed on more than 876,000 tumors annually in the United States.1 Among 3.5 million Americans diagnosed with nonmelanoma skin cancer in 2006, one-quarter were treated with MMS.2 In Japan, basal cell carcinoma (BCC) is the most common skin malignancy, with an incidence of 3.34 cases per 100,000 individuals; SCC is the second most common, with an incidence of 2.5 cases per 100,000 individuals.3
The essential element that makes MMS unique is the careful microscopic examination of the entire margin of the removed specimen. Tissue processing is done with careful en face orientation to ensure that circumferential and deep margins are entirely visible. The surgeon interprets the slides and proceeds to remove the additional tumor as necessary. Because the same physician performs both the surgery and the pathologic assessment throughout the procedure, a precise correlation between the microscopic and surgical findings can be made. The surgeon can begin with smaller margins, removing minimal healthy tissue while removing all the cancer cells, which results in the smallest-possible skin defect and the best prognosis for the malignancy (Figure 1).
At the only facility in Japan offering MMS, the lead author (S.S.) has treated 52 lesions with MMS in 46 patients (2020-2022). Of these patients, 40 were White, 5 were Japanese, and 1 was of African descent. In this case series, we present 5 Japanese patients who had BCC treated with MMS.
Case Series
Patient 1—A 50-year-old Japanese woman presented to dermatology with a brown papule on the nasal tip of 1.25 year’s duration (Figure 2). A biopsy revealed infiltrative BCC (Figure 3), and the patient was referred to the dermatology department at a nearby university hospital. Because the BCC was an aggressive variant, wide local excision (WLE) with subsequent flap reconstruction was recommended as well as radiation therapy. The patient learned about MMS through an internet search and refused both options, seeking MMS treatment at our clinic. Although Japanese health insurance does not cover MMS, the patient had supplemental private insurance that did cover the cost. She provided consent to undergo the procedure. Physical examination revealed a 7.5×6-mm, brown-red macule with ill-defined borders on the tip of the nose. We used a 1.5-mm margin for the first stage of MMS (Figure 4A). The frozen section revealed that the tumor had been entirely excised in the first stage, leaving only a 10.5×9-mm skin defect that was reconstructed with a Dufourmentel flap (Figure 4B). No signs of recurrence were noted at 3.5-year follow-up, and the cosmetic outcome was favorable (Figure 4C). National Comprehensive Cancer Network guidelines recommend a margin greater than 4 mm for infiltrative BCCs4; therefore, our technique reduced the total defect by at least 4 mm in a cosmetically sensitive area. The patient also did not need radiation therapy, which reduced morbidity. She continues to be recurrence free at 3.5-year follow-up.
Patient 2—A 63-year-old Japanese man presented to dermatology with a brown macule on the right lower eyelid of 2 years’ duration. A biopsy of the lesion was positive for nodular BCC. After being advised to undergo WLE and extensive reconstruction with plastic surgery, the patient learned of MMS through an internet search and found our clinic. Physical examination revealed a 7×5-mm brown macule on the right lower eyelid. The patient had supplemental private insurance that covered the cost of MMS, and he provided consent for the procedure. A 1.5-mm margin was taken for the first stage, resulting in a 10×8-mm defect superficial to the orbicularis oculi muscle. The frozen section revealed residual tumor exposure in the dermis at the 9- to 10-o’clock position. A second-stage excision was performed to remove an additional 1.5 mm of skin at the 9- to 12-o’clock position with a thin layer of the orbicularis oculi muscle. The subsequent histologic examination revealed no residual BCC, and the final 13×9-mm skin defect was reconstructed with a rotation flap. There were no signs of recurrence at 2.5-year follow-up with an excellent cosmetic outcome.
Patient 3—A 73-year-old Japanese man presented to a local university dermatology clinic with a new papule on the nose. The dermatologist suggested WLE with 4-mm margins and reconstruction of the skin defect 2 weeks later by a plastic surgeon. The patient was not satisfied with the proposed surgical plan, which led him to learn about MMS on the internet; he subsequently found our clinic. Physical examination revealed a 4×3.5-mm brown papule on the tip of the nose. He understood the nature of MMS and chose to pay out-of-pocket because Japanese health insurance did not cover the procedure. We used a 2-mm margin for the first stage, which created a 7.5×7-mm skin defect. The frozen section pathology revealed no residual BCC at the cut surface. The skin defect was reconstructed with a Limberg rhombic flap. There were no signs of recurrence at 1.5-year follow-up with a favorable cosmetic outcome.
Patient 4—A 45-year-old man presented to a dermatology clinic with a papule on the right side of the nose of 1 year’s duration. A biopsy revealed the lesion was a nodular BCC. The dermatologist recommended WLE at a general hospital, but the patient refused after learning about MMS. He subsequently made an appointment with our clinic. Physical examination revealed a 7×4-mm white papule on the right side of the nose. The patient had private insurance that covered the cost of MMS. The first stage was performed with 1.5-mm margins and was clear of residual tumor. A Limberg rhombic flap from the adjacent cheek was used to repair the final 10×7-mm skin defect. There were no signs of recurrence at 1 year and 9 months’ follow-up with a favorable cosmetic outcome.
Patient 5—A 76-year-old Japanese woman presented to a university hospital near Tokyo with a black papule on the left cutaneous lip of 5 years’ duration. A biopsy revealed nodular BCC, and WLE with flap reconstruction was recommended. The patient’s son learned about MMS through internet research and referred her to our clinic. Physical examination revealed a 7×5-mm black papule on the left upper lip. The patient’s private insurance covered the cost of MMS, and she consented to the procedure. We used a 2-mm initial margin, and the immediate frozen section revealed no signs of BCC at the cut surface. The 11×9-mm skin defect was reconstructed with a Limberg rhombic flap. There were no signs of recurrence at 1.5-year follow-up with a favorable cosmetic outcome.
Comment
We presented 5 cases of MMS in Japanese patients with BCC. More than 7000 new cases of nonmelanoma skin cancer occur every year in Japan.3 Only 0.04% of these cases—the 5 cases presented here—were treated with MMS in Japan in 2020 and 2021, in contrast to 25% in the United States in 2006.2
MMS vs Other BCC Treatments—Mohs micrographic surgery offers 2 distinct advantages over conventional excision: an improved cure rate while achieving a smaller final defect size, generally leading to better cosmetic outcomes. Overall 5-year recurrence rates of BCC are 10% for conventional surgical excision vs 1% for MMS, while the recurrence rates for SCC are 8% and 3%, respectively.5 A study of well-demarcated BCCs smaller than 2 cm that were treated with MMS with 2-mm increments revealed that 95% of the cases were free of malignancy within a 4-mm margin of the normal-appearing skin surrounding the tumor.6 Several articles have reported a 95% cure rate or higher with conventional excision of localized BCC,7 but 4- to 5-mm excision margins are required, resulting in a greater skin defect and a lower cure rate compared to MMS.
Aggressive subtypes of BCC have a higher recurrence rate. Rowe et al8 reported the following 5-year recurrence rates: 5.6% for MMS, 17.4% for conventional surgical excision, 40.0% for curettage and electrodesiccation, and 9.8% for radiation therapy. Primary BCCs with high-risk histologic subtypes has a 10-year recurrence rate of 4.4% with MMS vs 12.2% with conventional excision.9 These findings reveal that MMS yields a better prognosis compared to traditional treatment methods for recurrent BCCs and BCCs of high-risk histologic subtypes.
The primary reason for the excellent cure rate seen in MMS is the ability to perform complete margin assessment. Peripheral and deep en face margin assessment (PDEMA) is crucial in achieving high cure rates with narrow margins. In WLE (Figure 1), vertical sectioning (also known as bread-loafing) does not achieve direct visualization of the entire surgical margin, as this technique only evaluates random sections and does not achieve PDEMA.10 The bread-loafing method is used almost exclusively in Japan and visualizes only 0.1% of the entire margin compared to 100% with MMS.11 Beyond the superior cure rate, the MMS technique often yields smaller final defects compared to WLE. All 5 of our patients achieved complete tumor removal while sparing more normal tissue compared to conventional WLE, which takes at least a 4-mm margin in all directions.
Barriers to Adopting MMS in Japan—There are many barriers to the broader adoption of MMS in Japan. A guideline of the Japanese Dermatological Association says, MMS “is complicated, requires special training for acquisition, and requires time and labor for implementation of a series of processes, and it has not gained wide acceptance in Japan because of these disadvantages.”3 There currently are no MMS training programs in Japan. We refute this statement from the Japanese Dermatological Association because, in our experience, only 1 surgeon plus a single histotechnician familiar with MMS is sufficient for a facility to offer the procedure (the lead author of this study [S.S.] acts as both the surgeon and the histotechnician). Another misconception among some physicians in Japan is that cancer on ethnically Japanese skin is uniquely suited to excision without microscopic verification of tumor clearance because the borders of the tumors are easily identified, which was based on good cure rates for the excision of well-demarcated pigmented BCCs in a Japanese cohort. This study of a Japanese cohort investigated the specimens with the conventional bread-loafing technique but not with the PDEMA.12
Eighty percent (4/5) of our patients presented with nodular BCC, and only 1 required a second stage. In comparison, we also treated 16 White patients with nodular BCC with MMS during the same period, and 31% (5/16) required more than 1 stage, with 1 patient requiring 3 stages. This cohort, however, is too small to demonstrate a statistically significant difference (S.S., unpublished data, 2020-2022).
A study in Singapore reported the postsurgical complication rate and 5-year recurrence rate for 481 tumors (92% BCC and 7.5% SCC). The median follow-up duration after MMS was 36 months, and the recurrence rate was 0.6%. The postsurgical complications included 11 (2.3%) cases with superficial tip necrosis of surgical flaps/grafts, 2 (0.4%) with mild wound dehiscence, 1 (0.2%) with minor surgical site bleeding, and 1 (0.2%) with minor wound infection.13 This study supports the notion that MMS is equally effective for Asian patients.
Awareness of MMS in Japan is lacking, and most Japanese dermatologists do not know about the technique. All 5 patients in our case series asked their dermatologists about alternative treatment options and were not offered MMS. In each case, the patients learned of the technique through internet research.
The lack of insurance reimbursement for MMS in Japan is another barrier. Because the national health insurance does not reimburse for MMS, the procedure is relatively unavailable to most Japanese citizens who cannot pay out-of-pocket for the treatment and do not have supplemental insurance. Mohs micrographic surgery may seem expensive compared to WLE followed by repair; however, in the authors’ experience, in Japan, excision without MMS may require general sedation and multiple surgeries to reconstruct larger skin defects, leading to greater morbidity and risk for the patient.
Conclusion
Mohs micrographic surgery in Japan is in its infancy, and further studies showing recurrence rates and long-term prognosis are needed. Such data should help increase awareness of MMS among Japanese physicians as an excellent treatment option for their patients. Furthermore, as Japan becomes more heterogenous as a society and the US Military increases its presence in the region, the need for MMS is likely to increase.
Acknowledgments—We appreciate the proofreading support by Mark Bivens, MBA, MSc (Tokyo, Japan), as well as the technical support from Ben Tallon, MBChB, and Robyn Mason (both in Tauranga, New Zealand) to start MMS at our clinic.
- Asgari MM, Olson J, Alam M. Needs assessment for Mohs micrographic surgery. Dermatol Clin. 2012;30:167-175. doi:10.1016/j.det.2011.08.010
- Connolly SM, Baker DR, Baker DR, et al. AAD/ACMS/ASDSA/ASMS 2012 appropriate use criteria for Mohs micrographic surgery: a report of the American Academy of Dermatology, American College of Mohs Surgery, American Society for Dermatologic Surgery Association, and the American Society for Mohs Surgery. J Am Acad Dermatol. 2012;67:531-550.
- Ansai SI, Umebayashi Y, Katsumata N, et al. Japanese Dermatological Association Guidelines: outlines of guidelines for cutaneous squamous cell carcinoma 2020. J Dermatol. 2021;48:E288-E311.
- Schmults CD, Blitzblau R, Aasi SZ, et at. Basal cell skin cancer, version 2.2024, NCCN Clinical Practice Guidelines in Oncology. J Natl Compr Canc Netw. 2023;21:1181-1203. doi:10.6004/jncn.2023.0056
- Snow SN, Gunkel J. Mohs surgery. In: Bolognia JL, Schaffer JV, Cerroni L, eds. Dermatology. 4th ed. Elsevier; 2017:2445-2455. doi:10.1016/b978-0-070-94171-3.00041-7
- Wolf DJ, Zitelli JA. Surgical margins for basal cell carcinoma. Arch Dermatol. 1987;123:340-344.
- Quazi SJ, Aslam N, Saleem H, et al. Surgical margin of excision in basal cell carcinoma: a systematic review of literature. Cureus. 2020;12:E9211.
- Rowe DE, Carroll RJ, Day Jus CL. Mohs surgery is the treatment of choice for recurrent (previously treated) basal cell carcinoma. J Dermatol Surg Oncol. 1989;15:424-431.
- Van Loo, Mosterd K, Krekels GA. Surgical excision versus Mohs’ micrographic surgery for basal cell carcinoma of the face. Eur J Cancer. 2014;50:3011-3020.
- Schmults CD, Blitzblau R, Aasi SZ, et al. NCCN Guidelines Insights: Squamous Cell Skin Cancer, Version 1.2022. J Natl Compr Canc Netw. 2021;19:1382-1394.
- Hui AM, Jacobson M, Markowitz O, et al. Mohs micrographic surgery for the treatment of melanoma. Dermatol Clin. 2012;30:503-515.
- Ito T, Inatomi Y, Nagae K, et al. Narrow-margin excision is a safe, reliable treatment for well-defined, primary pigmented basal cell carcinoma: an analysis of 288 lesions in Japan. J Eur Acad Dermatol Venereol. 2015;29:1828-1831.
- Ho WYB, Zhao X, Tan WPM. Mohs micrographic surgery in Singapore: a long-term follow-up review. Ann Acad Med Singap. 2021;50:922-923.
- Asgari MM, Olson J, Alam M. Needs assessment for Mohs micrographic surgery. Dermatol Clin. 2012;30:167-175. doi:10.1016/j.det.2011.08.010
- Connolly SM, Baker DR, Baker DR, et al. AAD/ACMS/ASDSA/ASMS 2012 appropriate use criteria for Mohs micrographic surgery: a report of the American Academy of Dermatology, American College of Mohs Surgery, American Society for Dermatologic Surgery Association, and the American Society for Mohs Surgery. J Am Acad Dermatol. 2012;67:531-550.
- Ansai SI, Umebayashi Y, Katsumata N, et al. Japanese Dermatological Association Guidelines: outlines of guidelines for cutaneous squamous cell carcinoma 2020. J Dermatol. 2021;48:E288-E311.
- Schmults CD, Blitzblau R, Aasi SZ, et at. Basal cell skin cancer, version 2.2024, NCCN Clinical Practice Guidelines in Oncology. J Natl Compr Canc Netw. 2023;21:1181-1203. doi:10.6004/jncn.2023.0056
- Snow SN, Gunkel J. Mohs surgery. In: Bolognia JL, Schaffer JV, Cerroni L, eds. Dermatology. 4th ed. Elsevier; 2017:2445-2455. doi:10.1016/b978-0-070-94171-3.00041-7
- Wolf DJ, Zitelli JA. Surgical margins for basal cell carcinoma. Arch Dermatol. 1987;123:340-344.
- Quazi SJ, Aslam N, Saleem H, et al. Surgical margin of excision in basal cell carcinoma: a systematic review of literature. Cureus. 2020;12:E9211.
- Rowe DE, Carroll RJ, Day Jus CL. Mohs surgery is the treatment of choice for recurrent (previously treated) basal cell carcinoma. J Dermatol Surg Oncol. 1989;15:424-431.
- Van Loo, Mosterd K, Krekels GA. Surgical excision versus Mohs’ micrographic surgery for basal cell carcinoma of the face. Eur J Cancer. 2014;50:3011-3020.
- Schmults CD, Blitzblau R, Aasi SZ, et al. NCCN Guidelines Insights: Squamous Cell Skin Cancer, Version 1.2022. J Natl Compr Canc Netw. 2021;19:1382-1394.
- Hui AM, Jacobson M, Markowitz O, et al. Mohs micrographic surgery for the treatment of melanoma. Dermatol Clin. 2012;30:503-515.
- Ito T, Inatomi Y, Nagae K, et al. Narrow-margin excision is a safe, reliable treatment for well-defined, primary pigmented basal cell carcinoma: an analysis of 288 lesions in Japan. J Eur Acad Dermatol Venereol. 2015;29:1828-1831.
- Ho WYB, Zhao X, Tan WPM. Mohs micrographic surgery in Singapore: a long-term follow-up review. Ann Acad Med Singap. 2021;50:922-923.
Practice Points
- Mohs micrographic surgery (MMS) is a safe and effective treatment method for nonmelanoma skin cancer. In some cases, this procedure is superior to standard wide local excision and repair.
- For the broader adaptation of this vital technique in Japan—where MMS is not well established—increased awareness of treatment outcomes among Japanese physicians is needed.
Tackling Inflammatory and Infectious Nail Disorders in Children
Nail disorders are common among pediatric patients but often are underdiagnosed or misdiagnosed because of their unique disease manifestations. These conditions may severely impact quality of life. There are few nail disease clinical trials that include children. Consequently, most treatment recommendations are based on case series and expert consensus recommendations. We review inflammatory and infectious nail disorders in pediatric patients. By describing characteristics, clinical manifestations, and management approaches for these conditions, we aim to provide guidance to dermatologists in their diagnosis and treatment.
INFLAMMATORY NAIL DISORDERS
Nail Psoriasis
Nail involvement in children with psoriasis is common, with prevalence estimates ranging from 17% to 39.2%.1 Nail matrix psoriasis may manifest with pitting (large irregular pits) and leukonychia as well as chromonychia and nail plate crumbling. Onycholysis, oil drop spots (salmon patches), and subungual hyperkeratosis can be seen in nail bed psoriasis. Nail pitting is the most frequently observed clinical finding (Figure 1).2,3 In a cross-sectional multicenter study of 313 children with cutaneous psoriasis in France, nail findings were present in 101 patients (32.3%). There were associations between nail findings and presence of psoriatic arthritis (P=.03), palmoplantar psoriasis (P<.001), and severity of psoriatic disease, defined as use of systemic treatment with phototherapy (psoralen plus UVA, UVB), traditional systemic treatment (acitretin, methotrexate, cyclosporine), or a biologic (P=.003).4
Topical steroids and vitamin D analogues may be used with or without occlusion and may be efficacious.5 Several case reports describe systemic treatments for psoriasis in children, including methotrexate, acitretin, and apremilast (approved for children 6 years and older for plaque psoriasis by the US Food and Drug Administration [FDA]).2 There are 5 biologic drugs currently approved for the treatment of pediatric psoriasis—adalimumab, etanercept, ustekinumab, secukinumab, ixekizumab—and 6 drugs currently undergoing phase 3 studies—brodalumab, guselkumab, risankizumab, tildrakizumab, certolizumab pegol, and deucravacitinib (Table 1).6-15 Adalimumab is specifically approved for moderate to severe nail psoriasis in adults 18 years and older.
Intralesional steroid injections are sometimes useful in the management of nail matrix psoriasis; however, appropriate patient selection is critical due to the pain associated with the procedure. In a prospective study of 16 children (age range, 9–17 years) with nail psoriasis treated with intralesional triamcinolone (ILTAC) 2.5 to 5 mg/mL every 4 to 8 weeks for a minimum of 3 to 6 months, 9 patients achieved resolution and 6 had improvement of clinical findings.16 Local adverse events were mild, including injection-site pain (66%), subungual hematoma (n=1), Beau lines (n=1), proximal nail fold hypopigmentation (n=2), and proximal nail fold atrophy (n=2). Because the proximal nail fold in children is thinner than in adults, there may be an increased risk for nail fold hypopigmentation and atrophy in children. Therefore, a maximum ILTAC concentration of 2.5 mg/mL with 0.2 mL maximum volume per nail per session is recommended for children younger than 15 years.16
Nail Lichen Planus
Nail lichen planus (NLP) is uncommon in children, with few biopsy-proven cases documented in the literature.17 Common clinical findings are onychorrhexis, nail plate thinning, fissuring, splitting, and atrophy with koilonychia.5 Although pterygium development (irreversible nail matrix scarring) is uncommon in pediatric patients, NLP can be progressive and may cause irreversible destruction of the nail matrix and subsequent nail loss, warranting therapeutic intervention.18
Treatment of NLP may be difficult, as there are no options that work in all patients. Current literature supports the use of systemic corticosteroids or ILTAC for the treatment of NLP; however, recurrence rates can be high. According to an expert consensus paper on NLP treatment, ILTAC may be injected in a concentration of 2.5, 5, or 10 mg/mL according to disease severity.19 In severe or resistant cases, intramuscular (IM) triamcinolone may be considered, especially if more than 3 nails are affected. A dosage of 0.5 to 1 mg/kg/mo for at least 3 to 6 months is recommended for both children and adults, with 1 mg/kg/mo recommended in the active treatment phase (first 2–3 months).19 In a retrospective review of 5 pediatric patients with NLP treated with IM triamcinolone 0.5 mg/kg/mo, 3 patients had resolution and 2 improved with treatment.20 In a prospective study of 10 children with NLP, IM triamcinolone at a dosage of 0.5 to 1 mg/kg every 30 days for 3 to 6 months resulted in resolution of nail findings in 9 patients.17 In a prospective study of 14 pediatric patients with NLP treated with 2.5 to 5 mg/mL of ILTAC, 10 achieved resolution and 3 improved.16
Intralesional triamcinolone injections may be better suited for teenagers compared to younger children who may be more apprehensive of needles. To minimize pain, it is recommended to inject ILTAC slowly at room temperature, with use of “talkesthesia” and vibration devices, 1% lidocaine, or ethyl chloride spray.18
Trachyonychia
Trachyonychia is characterized by the presence of sandpaperlike nails. It manifests with brittle thin nails with longitudinal ridging, onychoschizia, and thickened hyperkeratotic cuticles. Trachyonychia typically involves multiple nails, with a peak age of onset between 3 and 12 years.21,22 There are 2 variants: the opaque type with rough longitudinal ridging, and the shiny variant with opalescent nails and pits that reflect light. The opaque variant is more common and is associated with psoriasis, whereas the shiny variant is less common and is associated with alopecia areata.23 Although most cases are idiopathic, some are associated with psoriasis and alopecia areata, as previously noted, as well as atopic dermatitis (AD) and lichen planus.22,24
Fortunately, trachyonychia does not lead to permanent nail damage or pterygium, making treatment primarily focused on addressing functional and cosmetic concerns.24 Spontaneous resolution occurs in approximately 50% of patients. In a prospective study of 11 patients with idiopathic trachyonychia, there was partial improvement in 5 of 9 patients treated with topical steroids, 1 with only petrolatum, and 1 with vitamin supplements. Complete resolution was reported in 1 patient treated with topical steroids.25 Because trachyonychia often is self-resolving, no treatment is required and a conservative approach is strongly recommended.26 Treatment options include topical corticosteroids, tazarotene, and 5-fluorouracil. Intralesional triamcinolone, systemic cyclosporine, retinoids, systemic corticosteroids, and tofacitinib have been described in case reports, though none of these have been shown to be 100% efficacious.24
Nail Lichen Striatus
Lichen striatus involving the nail is uncommon and is characterized by onycholysis, longitudinal ridging, splitting, and fraying, as well as what appears to be a subungual tumor. It can encompass the entire nail or may be isolated to a portion of the nail (Figure 2). Usually, a Blaschko-linear array of flesh-colored papules on the more proximal digit directly adjacent to the nail dystrophy will be seen, though nail findings can occur in isolation.27-29 The underlying pathophysiology is not clear; however, one hypothesis is that a triggering event, such as trauma, induces the expression of antigens that elicit a self-limiting immune-mediated response by CD8 T lymphocytes.30
Generally, nail lichen striatus spontaneously resolves in 1 to 2 years without treatment. In a prospective study of 5 patients with nail lichen striatus, the median time to resolution was 22.6 months (range, 10–30 months).31 Topical steroids may be used for pruritus. In one case report, a 3-year-old boy with nail lichen striatus of 4 months’ duration was treated with tacrolimus ointment 0.03% daily for 3 months.28
Nail AD
Nail changes with AD may be more common in adults than children or are underreported. In a study of 777 adults with AD, nail dystrophy was present in 124 patients (16%), whereas in a study of 250 pediatric patients with AD (aged 0-2 years), nail dystrophy was present in only 4 patients.32,33
Periungual inflammation from AD causes the nail changes.34 In a cross-sectional study of 24 pediatric patients with nail dystrophy due to AD, transverse grooves (Beau lines) were present in 25% (6/24), nail pitting in 16.7% (4/24), koilonychia in 16.7% (4/24), trachyonychia in 12.5% (3/24), leukonychia in 12.5% (3/24), brachyonychia in 8.3% (2/24), melanonychia in 8.3% (2/24), onychomadesis in 8.3% (2/24), onychoschizia in 8.3% (2/24), and onycholysis in 8.3% (2/24). There was an association between disease severity and presence of toenail dystrophy (P=.03).35
Topical steroids with or without occlusion can be used to treat nail changes. Although there is limited literature describing the treatment of nail AD in children, a 61-year-old man with nail changes associated with AD achieved resolution with 3 months of treatment with dupilumab.36 Anecdotally, most patients will improve with usual cutaneous AD management.
INFECTIOUS NAIL DISORDERS
Viral Infections
Hand, Foot, and Mouth Disease—Hand, foot, and mouth disease (HFMD) is a common childhood viral infection caused by various enteroviruses, most commonly coxsackievirus A16, with the A6 variant causing more severe disease. Fever and painful vesicles involving the oral mucosa as well as palms and soles give the disease its name. Nail changes are common. In a prospective study involving 130 patients with laboratory-confirmed coxsackievirus CA6 serotype infection, 37% developed onychomadesis vs only 5% of 145 cases with non-CA6 enterovirus infection who developed nail findings. There was an association between CA6 infection and presence of nail changes (P<.001).37
Findings ranging from transverse grooves (Beau lines) to complete nail shedding (onychomadesis)(Figure 3) may be seen.38,39 Nail findings in HFMD are due to transient inhibition of nail growth and present approximately 3 to 6 weeks after infection.40 Onychomadesis is seen in 30% to 68% of patients with HFMD.37,41,42 Nail findings in HFMD spontaneously resolve with nail growth (2–3 mm per month for fingernails and 1 mm per month for toenails) and do not require specific treatment. Although the appearance of nail changes associated with HFMD can be disturbing, dermatologists can reassure children and their parents that the nails will resolve with the next cycle of growth.
Kawasaki Disease—Kawasaki disease (KD) is a vasculitis primarily affecting children and infants. Although the specific pathogen and pathophysiology is not entirely clear, clinical observations have suggested an infectious cause, most likely a virus.43 In Japan, more than 15,000 cases of KD are documented annually, while approximately 4200 cases are seen in the United States.44 In a prospective study from 1984 to 1990, 4 of 26 (15.4%) patients with KD presented with nail manifestations during the late acute phase or early convalescent phase of disease. There were no significant associations between nail dystrophy and severity of KD, such as coronary artery aneurysm.45
Nail changes reported in children with KD include onychomadesis, onycholysis, orange-brown chromonychia, splinter hemorrhages, Beau lines, and pincer nails. In a review of nail changes associated with KD from 1980 to 2021, orange-brown transverse chromonychia, which may evolve into transverse leukonychia, was the most common nail finding reported, occurring in 17 of 31 (54.8%) patients.44 It has been hypothesized that nail changes may result from blood flow disturbance due to the underlying vasculitis.46 Nail changes appear several weeks after the onset of fever and are self-limited. Resolution occurs with nail growth, with no treatment required.
FUNGAL INFECTIONS
Onychomycosis
Onychomycosis is a fungal infection of the nails that occurs in 0.2% to 5.5% of pediatric patients, and its prevalence may be increasing, which may be due to environmental factors or increased rates of diabetes mellitus and obesity in the pediatric population.47 Onychomycosis represents 15.5% of nail dystrophies in pediatric patients.48 Some dermatologists treat presumptive onychomycosis without confirmation; however, we do not recommend that approach. Because the differential is broad and the duration of treatment is long, mycologic examination (potassium hydroxide preparation, fungal culture, polymerase chain reaction, and/or histopathology) should be obtained to confirm onychomycosis prior to initiation of antifungal management. Family members of affected individuals should be evaluated and treated, if indicated, for onychomycosis and tinea pedis, as household transmission is common.
Currently, there are 2 topical FDA-approved treatments for pediatric onychomycosis in children 6 years and older (Table 2).49,50 There is a discussion of the need for confirmatory testing for onychomycosis in children, particularly when systemic treatment is prescribed. In a retrospective review of 269 pediatric patients with onychomycosis prescribed terbinafine, 53.5% (n=144) underwent laboratory monitoring of liver function and complete blood cell counts, and 12.5% had grade 1 laboratory abnormalities either prior to (12/144 [8.3%]) or during (6/144 [4.2%]) therapy.51 Baseline transaminase monitoring is recommended, though subsequent routine laboratory monitoring in healthy children may have limited utility with associated increased costs, incidental findings, and patient discomfort and likely is not needed.51
Pediatric onychomycosis responds better to topical therapy than adult disease, and pediatric patients do not always require systemic treatment.52 Ciclopirox is not FDA approved for the treatment of pediatric onychomycosis, but in a 32-week clinical trial of ciclopirox lacquer 8% use in 40 patients, 77% (27/35) of treated patients achieved mycologic cure. Overall, 71% of treated patients (25/35) vs 22% (2/9) of controls achieved efficacy (defined as investigator global assessment score of 2 or lower).52 In an open-label, single-arm clinical trial assessing tavaborole solution 5% applied once daily for 48 weeks for the treatment of toenail onychomycosis in pediatric patients (aged 6–17 years), 36.2% (20/55) of patients achieved mycologic cure, and 8.5% (5/55) achieved complete cure at week 52 with mild or minimal adverse effects.53 In an open-label, phase 4 study of the safety and efficacy of efinaconazole solution 10% applied once daily for 48 weeks in pediatric patients (aged 6 to 16 years) (n=60), 65% (35/60) achieved mycologic cure, 42% (25/60) achieved clinical cure, and 40% (24/60) achieved complete cure at 52 weeks. The most common adverse effects of efinaconazole were local and included ingrown toenail (1/60), application-site dermatitis (1/60), application-site vesicles (1/60), and application-site pain (1/60).54
In a systematic review of systemic antifungals for onychomycosis in 151 pediatric patients, itraconazole, fluconazole, griseofulvin, and terbinafine resulted in complete cure rates similar to those of the adult population, with excellent safety profiles.55 Depending on the situation, initiation of treatment with topical medications followed by addition of systemic antifungal agents only if needed may be an appropriate course of action.
BACTERIAL INFECTIONS
Acute Paronychia
Acute paronychia is a nail-fold infection that develops after the protective nail barrier has been compromised.56 In children, thumb-sucking, nail-biting, frequent oral manipulation of the digits, and poor skin hygiene are risk factors. Acute paronychia also may develop in association with congenital malalignment of the great toenails.57
Clinical manifestations include localized pain, erythema, and nail fold edema (Figure 4). Purulent material and abscess formation may ensue. Staphylococcus aureus as well as methicillin-resistant S aureus and Streptococcus pyogenes are classically the most common causes of acute paronychia. Treatment of paronychia is based on severity. In mild cases, warm soaks with topical antibiotics are indicated. Oral antibiotics should be prescribed for more severe presentations. If there is no improvement after 48 hours, surgical drainage is required to facilitate healing.56
FINAL THOUGHTS
Inflammatory and infectious nail disorders in children are relatively common and may impact the physical and emotional well-being of young patients. By understanding the distinctive features of these nail disorders in pediatric patients, dermatologists can provide anticipatory guidance and informed treatment options to children and their parents. Further research is needed to expand our understanding of pediatric nail disorders and create targeted therapeutic interventions, particularly for NLP and psoriasis.
- Uber M, Carvalho VO, Abagge KT, et al. Clinical features and nail clippings in 52 children with psoriasis. Pediatr Dermatol. 2018;35:202-207. doi:10.1111/pde.13402
- Plachouri KM, Mulita F, Georgiou S. Management of pediatric nail psoriasis. Cutis. 2021;108:292-294. doi:10.12788/cutis.0386
- Smith RJ, Rubin AI. Pediatric nail disorders: a review. Curr Opin Pediatr. 2020;32:506-515. doi:10.1097/mop.0000000000000921
- Pourchot D, Bodemer C, Phan A, et al. Nail psoriasis: a systematic evaluation in 313 children with psoriasis. Pediatr Dermatol. 2017;34:58-63. doi:10.1111/pde.13028
- Richert B, André J. Nail disorders in children: diagnosis and management. Am J Clin Dermatol. 2011;12:101-112. doi:10.2165/11537110-000000000-00000
- Lee JYY. Severe 20-nail psoriasis successfully treated by low dose methotrexate. Dermatol Online J. 2009;15:8.
- Nogueira M, Paller AS, Torres T. Targeted therapy for pediatric psoriasis. Paediatr Drugs. May 2021;23:203-212. doi:10.1007/s40272-021-00443-5
- Hanoodi M, Mittal M. Methotrexate. StatPearls [Internet]. Updated August 16, 2023. Accessed July 1, 2024. https://www.ncbi.nlm.nih.gov/books/NBK556114/
- Teran CG, Teran-Escalera CN, Balderrama C. A severe case of erythrodermic psoriasis associated with advanced nail and joint manifestations: a case report. J Med Case Rep. 2010;4:179. doi:10.1186/1752-1947-4-179
- Paller AS, Seyger MMB, Magariños GA, et al. Long-term efficacy and safety of up to 108 weeks of ixekizumab in pediatric patients with moderate to severe plaque psoriasis: the IXORA-PEDS randomized clinical trial. JAMA Dermatol. 2022;158:533-541. doi:10.1001/jamadermatol.2022.0655
- Diotallevi F, Simonetti O, Rizzetto G, et al. Biological treatments for pediatric psoriasis: state of the art and future perspectives. Int J Mol Sci. 2022;23:11128. doi:10.3390/ijms231911128
- Nash P, Mease PJ, Kirkham B, et al. Secukinumab provides sustained improvement in nail psoriasis, signs and symptoms of psoriatic arthritis and low rate of radiographic progression in patients with concomitant nail involvement: 2-year results from the Phase III FUTURE 5 study. Clin Exp Rheumatol. 2022;40:952-959. doi:10.55563/clinexprheumatol/3nuz51
- Wells LE, Evans T, Hilton R, et al. Use of secukinumab in a pediatric patient leads to significant improvement in nail psoriasis and psoriatic arthritis. Pediatr Dermatol. 2019;36:384-385. doi:10.1111/pde.13767
- Watabe D, Endoh K, Maeda F, et al. Childhood-onset psoriaticonycho-pachydermo-periostitis treated successfully with infliximab. Eur J Dermatol. 2015;25:506-508. doi:10.1684/ejd.2015.2616
- Pereira TM, Vieira AP, Fernandes JC, et al. Anti-TNF-alpha therapy in childhood pustular psoriasis. Dermatology. 2006;213:350-352. doi:10.1159/000096202
- Iorizzo M, Gioia Di Chiacchio N, Di Chiacchio N, et al. Intralesional steroid injections for inflammatory nail dystrophies in the pediatric population. Pediatr Dermatol. 2023;40:759-761. doi:10.1111/pde.15295
- Tosti A, Piraccini BM, Cambiaghi S, et al. Nail lichen planus in children: clinical features, response to treatment, and long-term follow-up. Arch Dermatol. 2001;137:1027-1032.
- Lipner SR. Nail lichen planus: a true nail emergency. J Am Acad Dermatol. 2019;80:e177-e178. doi:10.1016/j.jaad.2018.11.065
- Iorizzo M, Tosti A, Starace M, et al. Isolated nail lichen planus: an expert consensus on treatment of the classical form. J Am Acad Dermatol. 2020;83:1717-1723. doi:10.1016/j.jaad.2020.02.056
- Piraccini BM, Saccani E, Starace M, et al. Nail lichen planus: response to treatment and long term follow-up. Eur J Dermatol. 2010;20:489-496. doi:10.1684/ejd.2010.0952
- Mahajan R, Kaushik A, De D, et al. Pediatric trachyonychia- a retrospective study of 17 cases. Indian J Dermatol. 2021;66:689-690. doi:10.4103/ijd.ijd_42_21
- Leung AKC, Leong KF, Barankin B. Trachyonychia. J Pediatr. 2020;216:239-239.e1. doi:10.1016/j.jpeds.2019.08.034
- Haber JS, Chairatchaneeboon M, Rubin AI. Trachyonychia: review and update on clinical aspects, histology, and therapy. Skin Appendage Disord. 2017;2:109-115. doi:10.1159/000449063
- Jacobsen AA, Tosti A. Trachyonychia and twenty-nail dystrophy: a comprehensive review and discussion of diagnostic accuracy. Skin Appendage Disord. 2016;2:7-13. doi:10.1159/000445544
- Kumar MG, Ciliberto H, Bayliss SJ. Long-term follow-up of pediatric trachyonychia. Pediatr Dermatol. 2015;32:198-200. doi:10.1111/pde.12427
- Tosti A, Piraccini BM, Iorizzo M. Trachyonychia and related disorders: evaluation and treatment plans. Dermatolog Ther. 2002;15:121-125. doi:10.1046/j.1529-8019.2002.01511.x
- Leung AKC, Leong KF, Barankin B. Lichen striatus with nail involvement in a 6-year-old boy. Case Rep Pediatr. 2020;2020:1494760. doi:10.1155/2020/1494760
- Kim GW, Kim SH, Seo SH, et al. Lichen striatus with nail abnormality successfully treated with tacrolimus ointment. J Dermatol. 2009;36:616-617. doi:10.1111/j.1346-8138.2009.00720.x
- Iorizzo M, Rubin AI, Starace M. Nail lichen striatus: is dermoscopy useful for the diagnosis? Pediatr Dermatol. 2019;36:859-863. doi:10.1111/pde.13916
- Karp DL, Cohen BA. Onychodystrophy in lichen striatus. Pediatr Dermatol. 1993;10:359-361. doi:10.1111/j.1525-1470.1993.tb00399.x
- Tosti A, Peluso AM, Misciali C, et al. Nail lichen striatus: clinical features and long-term follow-up of five patients. J Am Acad Dermatol. 1997;36(6, pt 1):908-913. doi:10.1016/s0190-9622(97)80270-8
- Simpson EL, Thompson MM, Hanifin JM. Prevalence and morphology of hand eczema in patients with atopic dermatitis. Dermatitis. 2006;17:123-127. doi:10.2310/6620.2006.06005
- Sarifakioglu E, Yilmaz AE, Gorpelioglu C. Nail alterations in 250 infant patients: a clinical study. J Eur Acad Dermatol Venereol. 2008;22:741-744. doi:10.1111/j.1468-3083.2008.02592.x
- Milanesi N, D’Erme AM, Gola M. Nail improvement during alitretinoin treatment: three case reports and review of the literature. Clin Exp Dermatol. 2015;40:533-536. doi:10.1111/ced.12584
- Chung BY, Choi YW, Kim HO, et al. Nail dystrophy in patients with atopic dermatitis and its association with disease severity. Ann Dermatol. 2019;31:121-126. doi:10.5021/ad.2019.31.2.121
- Navarro-Triviño FJ, Vega-Castillo JJ, Ruiz-Villaverde R. Nail changes successfully treated with dupilumab in a patient with severe atopic dermatitis. Australas J Dermatol. 2021;62:e468-e469. doi:10.1111/ajd.13633
- Wei SH, Huang YP, Liu MC, et al. An outbreak of coxsackievirus A6 hand, foot, and mouth disease associated with onychomadesis in Taiwan, 2010. BMC Infect Dis. 2011;11:346. doi:10.1186/1471-2334-11-346
- Shin JY, Cho BK, Park HJ. A clinical study of nail changes occurring secondary to hand-foot-mouth disease: onychomadesis and Beau’s lines. Ann Dermatol. 2014;26:280-283. doi:10.5021/ad.2014.26.2.280
- Verma S, Singal A. Nail changes in hand-foot-and-mouth disease (HFMD). Indian Dermatol Online J. 2021;12:656-657. doi:10.4103 /idoj.IDOJ_271_20
- Giordano LMC, de la Fuente LA, Lorca JMB, et al. Onychomadesis secondary to hand-foot-mouth disease: a frequent manifestation and cause of concern for parents. Article in Spanish. Rev Chil Pediatr. 2018;89:380-383. doi:10.4067/s0370-41062018005000203
- Justino MCA, da SMD, Souza MF, et al. Atypical hand-foot-mouth disease in Belém, Amazon region, northern Brazil, with detection of coxsackievirus A6. J Clin Virol. 2020;126:104307. doi:10.1016/j.jcv.2020.104307
- Cheng FF, Zhang BB, Cao ML, et al. Clinical characteristics of 68 children with atypical hand, foot, and mouth disease caused by coxsackievirus A6: a single-center retrospective analysis. Transl Pediatr. 2022;11:1502-1509. doi:10.21037/tp-22-352
- Nagata S. Causes of Kawasaki disease-from past to present. Front Pediatr. 2019;7:18. doi:10.3389/fped.2019.00018
- Mitsuishi T, Miyata K, Ando A, et al. Characteristic nail lesions in Kawasaki disease: case series and literature review. J Dermatol. 2022;49:232-238. doi:10.1111/1346-8138.16276
- Lindsley CB. Nail-bed lines in Kawasaki disease. Am J Dis Child. 1992;146:659-660. doi:10.1001/archpedi.1992.02160180017005
- Matsumura O, Nakagishi Y. Pincer nails upon convalescence from Kawasaki disease. J Pediatr. 2022;246:279. doi:10.1016/j.jpeds.2022.03.002
- Solís-Arias MP, García-Romero MT. Onychomycosis in children. a review. Int J Dermatol. 2017;56:123-130. doi:10.1111/ijd.13392
- Gupta AK, Mays RR, Versteeg SG, et al. Onychomycosis in children: safety and efficacy of antifungal agents. Pediatr Dermatol. 2018;35:552-559. doi:10.1111/pde.13561
- 49. Gupta AK, Venkataraman M, Shear NH, et al. Labeled use of efinaconazole topical solution 10% in treating onychomycosis in children and a review of the management of pediatric onychomycosis. Dermatol Ther. 2020;33:e13613. doi:10.1111/dth.13613
- Falotico JM, Lipner SR. Updated perspectives on the diagnosis and management of onychomycosis. Clin Cosmet Investig Dermatol. 2022;15:1933-1957. doi:10.2147/ccid.S362635
- Patel D, Castelo-Soccio LA, Rubin AI, et al. Laboratory monitoring during systemic terbinafine therapy for pediatric onychomycosis. JAMA Dermatol. 2017;153:1326-1327. doi:10.1001/jamadermatol.2017.4483
- Friedlander SF, Chan YC, Chan YH, et al. Onychomycosis does not always require systemic treatment for cure: a trial using topical therapy. Pediatr Dermatol. 2013;30:316-322. doi:10.1111/pde.12064
- Rich P, Spellman M, Purohit V, et al. Tavaborole 5% topical solution for the treatment of toenail onychomycosis in pediatric patients: results from a phase 4 open-label study. J Drugs Dermatol. 2019;18:190-195.
- Gupta AK, Venkataraman M, Abramovits W, et al. JUBLIA (efinaconazole 10% solution) in the treatment of pediatric onychomycosis. Skinmed. 2021;19:206-210.
- Gupta AK, Paquet M. Systemic antifungals to treat onychomycosis in children: a systematic review. Pediatr Dermatol. 2013;30:294-302. doi:10.1111/pde.12048
- Leggit JC. Acute and chronic paronychia. Am Fam Physician. 2017;96:44-51.
- Lipner SR, Scher RK. Congenital malalignment of the great toenails with acute paronychia. Pediatr Dermatol. 2016;33:e288-e289.doi:10.1111/pde.12924
Nail disorders are common among pediatric patients but often are underdiagnosed or misdiagnosed because of their unique disease manifestations. These conditions may severely impact quality of life. There are few nail disease clinical trials that include children. Consequently, most treatment recommendations are based on case series and expert consensus recommendations. We review inflammatory and infectious nail disorders in pediatric patients. By describing characteristics, clinical manifestations, and management approaches for these conditions, we aim to provide guidance to dermatologists in their diagnosis and treatment.
INFLAMMATORY NAIL DISORDERS
Nail Psoriasis
Nail involvement in children with psoriasis is common, with prevalence estimates ranging from 17% to 39.2%.1 Nail matrix psoriasis may manifest with pitting (large irregular pits) and leukonychia as well as chromonychia and nail plate crumbling. Onycholysis, oil drop spots (salmon patches), and subungual hyperkeratosis can be seen in nail bed psoriasis. Nail pitting is the most frequently observed clinical finding (Figure 1).2,3 In a cross-sectional multicenter study of 313 children with cutaneous psoriasis in France, nail findings were present in 101 patients (32.3%). There were associations between nail findings and presence of psoriatic arthritis (P=.03), palmoplantar psoriasis (P<.001), and severity of psoriatic disease, defined as use of systemic treatment with phototherapy (psoralen plus UVA, UVB), traditional systemic treatment (acitretin, methotrexate, cyclosporine), or a biologic (P=.003).4
Topical steroids and vitamin D analogues may be used with or without occlusion and may be efficacious.5 Several case reports describe systemic treatments for psoriasis in children, including methotrexate, acitretin, and apremilast (approved for children 6 years and older for plaque psoriasis by the US Food and Drug Administration [FDA]).2 There are 5 biologic drugs currently approved for the treatment of pediatric psoriasis—adalimumab, etanercept, ustekinumab, secukinumab, ixekizumab—and 6 drugs currently undergoing phase 3 studies—brodalumab, guselkumab, risankizumab, tildrakizumab, certolizumab pegol, and deucravacitinib (Table 1).6-15 Adalimumab is specifically approved for moderate to severe nail psoriasis in adults 18 years and older.
Intralesional steroid injections are sometimes useful in the management of nail matrix psoriasis; however, appropriate patient selection is critical due to the pain associated with the procedure. In a prospective study of 16 children (age range, 9–17 years) with nail psoriasis treated with intralesional triamcinolone (ILTAC) 2.5 to 5 mg/mL every 4 to 8 weeks for a minimum of 3 to 6 months, 9 patients achieved resolution and 6 had improvement of clinical findings.16 Local adverse events were mild, including injection-site pain (66%), subungual hematoma (n=1), Beau lines (n=1), proximal nail fold hypopigmentation (n=2), and proximal nail fold atrophy (n=2). Because the proximal nail fold in children is thinner than in adults, there may be an increased risk for nail fold hypopigmentation and atrophy in children. Therefore, a maximum ILTAC concentration of 2.5 mg/mL with 0.2 mL maximum volume per nail per session is recommended for children younger than 15 years.16
Nail Lichen Planus
Nail lichen planus (NLP) is uncommon in children, with few biopsy-proven cases documented in the literature.17 Common clinical findings are onychorrhexis, nail plate thinning, fissuring, splitting, and atrophy with koilonychia.5 Although pterygium development (irreversible nail matrix scarring) is uncommon in pediatric patients, NLP can be progressive and may cause irreversible destruction of the nail matrix and subsequent nail loss, warranting therapeutic intervention.18
Treatment of NLP may be difficult, as there are no options that work in all patients. Current literature supports the use of systemic corticosteroids or ILTAC for the treatment of NLP; however, recurrence rates can be high. According to an expert consensus paper on NLP treatment, ILTAC may be injected in a concentration of 2.5, 5, or 10 mg/mL according to disease severity.19 In severe or resistant cases, intramuscular (IM) triamcinolone may be considered, especially if more than 3 nails are affected. A dosage of 0.5 to 1 mg/kg/mo for at least 3 to 6 months is recommended for both children and adults, with 1 mg/kg/mo recommended in the active treatment phase (first 2–3 months).19 In a retrospective review of 5 pediatric patients with NLP treated with IM triamcinolone 0.5 mg/kg/mo, 3 patients had resolution and 2 improved with treatment.20 In a prospective study of 10 children with NLP, IM triamcinolone at a dosage of 0.5 to 1 mg/kg every 30 days for 3 to 6 months resulted in resolution of nail findings in 9 patients.17 In a prospective study of 14 pediatric patients with NLP treated with 2.5 to 5 mg/mL of ILTAC, 10 achieved resolution and 3 improved.16
Intralesional triamcinolone injections may be better suited for teenagers compared to younger children who may be more apprehensive of needles. To minimize pain, it is recommended to inject ILTAC slowly at room temperature, with use of “talkesthesia” and vibration devices, 1% lidocaine, or ethyl chloride spray.18
Trachyonychia
Trachyonychia is characterized by the presence of sandpaperlike nails. It manifests with brittle thin nails with longitudinal ridging, onychoschizia, and thickened hyperkeratotic cuticles. Trachyonychia typically involves multiple nails, with a peak age of onset between 3 and 12 years.21,22 There are 2 variants: the opaque type with rough longitudinal ridging, and the shiny variant with opalescent nails and pits that reflect light. The opaque variant is more common and is associated with psoriasis, whereas the shiny variant is less common and is associated with alopecia areata.23 Although most cases are idiopathic, some are associated with psoriasis and alopecia areata, as previously noted, as well as atopic dermatitis (AD) and lichen planus.22,24
Fortunately, trachyonychia does not lead to permanent nail damage or pterygium, making treatment primarily focused on addressing functional and cosmetic concerns.24 Spontaneous resolution occurs in approximately 50% of patients. In a prospective study of 11 patients with idiopathic trachyonychia, there was partial improvement in 5 of 9 patients treated with topical steroids, 1 with only petrolatum, and 1 with vitamin supplements. Complete resolution was reported in 1 patient treated with topical steroids.25 Because trachyonychia often is self-resolving, no treatment is required and a conservative approach is strongly recommended.26 Treatment options include topical corticosteroids, tazarotene, and 5-fluorouracil. Intralesional triamcinolone, systemic cyclosporine, retinoids, systemic corticosteroids, and tofacitinib have been described in case reports, though none of these have been shown to be 100% efficacious.24
Nail Lichen Striatus
Lichen striatus involving the nail is uncommon and is characterized by onycholysis, longitudinal ridging, splitting, and fraying, as well as what appears to be a subungual tumor. It can encompass the entire nail or may be isolated to a portion of the nail (Figure 2). Usually, a Blaschko-linear array of flesh-colored papules on the more proximal digit directly adjacent to the nail dystrophy will be seen, though nail findings can occur in isolation.27-29 The underlying pathophysiology is not clear; however, one hypothesis is that a triggering event, such as trauma, induces the expression of antigens that elicit a self-limiting immune-mediated response by CD8 T lymphocytes.30
Generally, nail lichen striatus spontaneously resolves in 1 to 2 years without treatment. In a prospective study of 5 patients with nail lichen striatus, the median time to resolution was 22.6 months (range, 10–30 months).31 Topical steroids may be used for pruritus. In one case report, a 3-year-old boy with nail lichen striatus of 4 months’ duration was treated with tacrolimus ointment 0.03% daily for 3 months.28
Nail AD
Nail changes with AD may be more common in adults than children or are underreported. In a study of 777 adults with AD, nail dystrophy was present in 124 patients (16%), whereas in a study of 250 pediatric patients with AD (aged 0-2 years), nail dystrophy was present in only 4 patients.32,33
Periungual inflammation from AD causes the nail changes.34 In a cross-sectional study of 24 pediatric patients with nail dystrophy due to AD, transverse grooves (Beau lines) were present in 25% (6/24), nail pitting in 16.7% (4/24), koilonychia in 16.7% (4/24), trachyonychia in 12.5% (3/24), leukonychia in 12.5% (3/24), brachyonychia in 8.3% (2/24), melanonychia in 8.3% (2/24), onychomadesis in 8.3% (2/24), onychoschizia in 8.3% (2/24), and onycholysis in 8.3% (2/24). There was an association between disease severity and presence of toenail dystrophy (P=.03).35
Topical steroids with or without occlusion can be used to treat nail changes. Although there is limited literature describing the treatment of nail AD in children, a 61-year-old man with nail changes associated with AD achieved resolution with 3 months of treatment with dupilumab.36 Anecdotally, most patients will improve with usual cutaneous AD management.
INFECTIOUS NAIL DISORDERS
Viral Infections
Hand, Foot, and Mouth Disease—Hand, foot, and mouth disease (HFMD) is a common childhood viral infection caused by various enteroviruses, most commonly coxsackievirus A16, with the A6 variant causing more severe disease. Fever and painful vesicles involving the oral mucosa as well as palms and soles give the disease its name. Nail changes are common. In a prospective study involving 130 patients with laboratory-confirmed coxsackievirus CA6 serotype infection, 37% developed onychomadesis vs only 5% of 145 cases with non-CA6 enterovirus infection who developed nail findings. There was an association between CA6 infection and presence of nail changes (P<.001).37
Findings ranging from transverse grooves (Beau lines) to complete nail shedding (onychomadesis)(Figure 3) may be seen.38,39 Nail findings in HFMD are due to transient inhibition of nail growth and present approximately 3 to 6 weeks after infection.40 Onychomadesis is seen in 30% to 68% of patients with HFMD.37,41,42 Nail findings in HFMD spontaneously resolve with nail growth (2–3 mm per month for fingernails and 1 mm per month for toenails) and do not require specific treatment. Although the appearance of nail changes associated with HFMD can be disturbing, dermatologists can reassure children and their parents that the nails will resolve with the next cycle of growth.
Kawasaki Disease—Kawasaki disease (KD) is a vasculitis primarily affecting children and infants. Although the specific pathogen and pathophysiology is not entirely clear, clinical observations have suggested an infectious cause, most likely a virus.43 In Japan, more than 15,000 cases of KD are documented annually, while approximately 4200 cases are seen in the United States.44 In a prospective study from 1984 to 1990, 4 of 26 (15.4%) patients with KD presented with nail manifestations during the late acute phase or early convalescent phase of disease. There were no significant associations between nail dystrophy and severity of KD, such as coronary artery aneurysm.45
Nail changes reported in children with KD include onychomadesis, onycholysis, orange-brown chromonychia, splinter hemorrhages, Beau lines, and pincer nails. In a review of nail changes associated with KD from 1980 to 2021, orange-brown transverse chromonychia, which may evolve into transverse leukonychia, was the most common nail finding reported, occurring in 17 of 31 (54.8%) patients.44 It has been hypothesized that nail changes may result from blood flow disturbance due to the underlying vasculitis.46 Nail changes appear several weeks after the onset of fever and are self-limited. Resolution occurs with nail growth, with no treatment required.
FUNGAL INFECTIONS
Onychomycosis
Onychomycosis is a fungal infection of the nails that occurs in 0.2% to 5.5% of pediatric patients, and its prevalence may be increasing, which may be due to environmental factors or increased rates of diabetes mellitus and obesity in the pediatric population.47 Onychomycosis represents 15.5% of nail dystrophies in pediatric patients.48 Some dermatologists treat presumptive onychomycosis without confirmation; however, we do not recommend that approach. Because the differential is broad and the duration of treatment is long, mycologic examination (potassium hydroxide preparation, fungal culture, polymerase chain reaction, and/or histopathology) should be obtained to confirm onychomycosis prior to initiation of antifungal management. Family members of affected individuals should be evaluated and treated, if indicated, for onychomycosis and tinea pedis, as household transmission is common.
Currently, there are 2 topical FDA-approved treatments for pediatric onychomycosis in children 6 years and older (Table 2).49,50 There is a discussion of the need for confirmatory testing for onychomycosis in children, particularly when systemic treatment is prescribed. In a retrospective review of 269 pediatric patients with onychomycosis prescribed terbinafine, 53.5% (n=144) underwent laboratory monitoring of liver function and complete blood cell counts, and 12.5% had grade 1 laboratory abnormalities either prior to (12/144 [8.3%]) or during (6/144 [4.2%]) therapy.51 Baseline transaminase monitoring is recommended, though subsequent routine laboratory monitoring in healthy children may have limited utility with associated increased costs, incidental findings, and patient discomfort and likely is not needed.51
Pediatric onychomycosis responds better to topical therapy than adult disease, and pediatric patients do not always require systemic treatment.52 Ciclopirox is not FDA approved for the treatment of pediatric onychomycosis, but in a 32-week clinical trial of ciclopirox lacquer 8% use in 40 patients, 77% (27/35) of treated patients achieved mycologic cure. Overall, 71% of treated patients (25/35) vs 22% (2/9) of controls achieved efficacy (defined as investigator global assessment score of 2 or lower).52 In an open-label, single-arm clinical trial assessing tavaborole solution 5% applied once daily for 48 weeks for the treatment of toenail onychomycosis in pediatric patients (aged 6–17 years), 36.2% (20/55) of patients achieved mycologic cure, and 8.5% (5/55) achieved complete cure at week 52 with mild or minimal adverse effects.53 In an open-label, phase 4 study of the safety and efficacy of efinaconazole solution 10% applied once daily for 48 weeks in pediatric patients (aged 6 to 16 years) (n=60), 65% (35/60) achieved mycologic cure, 42% (25/60) achieved clinical cure, and 40% (24/60) achieved complete cure at 52 weeks. The most common adverse effects of efinaconazole were local and included ingrown toenail (1/60), application-site dermatitis (1/60), application-site vesicles (1/60), and application-site pain (1/60).54
In a systematic review of systemic antifungals for onychomycosis in 151 pediatric patients, itraconazole, fluconazole, griseofulvin, and terbinafine resulted in complete cure rates similar to those of the adult population, with excellent safety profiles.55 Depending on the situation, initiation of treatment with topical medications followed by addition of systemic antifungal agents only if needed may be an appropriate course of action.
BACTERIAL INFECTIONS
Acute Paronychia
Acute paronychia is a nail-fold infection that develops after the protective nail barrier has been compromised.56 In children, thumb-sucking, nail-biting, frequent oral manipulation of the digits, and poor skin hygiene are risk factors. Acute paronychia also may develop in association with congenital malalignment of the great toenails.57
Clinical manifestations include localized pain, erythema, and nail fold edema (Figure 4). Purulent material and abscess formation may ensue. Staphylococcus aureus as well as methicillin-resistant S aureus and Streptococcus pyogenes are classically the most common causes of acute paronychia. Treatment of paronychia is based on severity. In mild cases, warm soaks with topical antibiotics are indicated. Oral antibiotics should be prescribed for more severe presentations. If there is no improvement after 48 hours, surgical drainage is required to facilitate healing.56
FINAL THOUGHTS
Inflammatory and infectious nail disorders in children are relatively common and may impact the physical and emotional well-being of young patients. By understanding the distinctive features of these nail disorders in pediatric patients, dermatologists can provide anticipatory guidance and informed treatment options to children and their parents. Further research is needed to expand our understanding of pediatric nail disorders and create targeted therapeutic interventions, particularly for NLP and psoriasis.
Nail disorders are common among pediatric patients but often are underdiagnosed or misdiagnosed because of their unique disease manifestations. These conditions may severely impact quality of life. There are few nail disease clinical trials that include children. Consequently, most treatment recommendations are based on case series and expert consensus recommendations. We review inflammatory and infectious nail disorders in pediatric patients. By describing characteristics, clinical manifestations, and management approaches for these conditions, we aim to provide guidance to dermatologists in their diagnosis and treatment.
INFLAMMATORY NAIL DISORDERS
Nail Psoriasis
Nail involvement in children with psoriasis is common, with prevalence estimates ranging from 17% to 39.2%.1 Nail matrix psoriasis may manifest with pitting (large irregular pits) and leukonychia as well as chromonychia and nail plate crumbling. Onycholysis, oil drop spots (salmon patches), and subungual hyperkeratosis can be seen in nail bed psoriasis. Nail pitting is the most frequently observed clinical finding (Figure 1).2,3 In a cross-sectional multicenter study of 313 children with cutaneous psoriasis in France, nail findings were present in 101 patients (32.3%). There were associations between nail findings and presence of psoriatic arthritis (P=.03), palmoplantar psoriasis (P<.001), and severity of psoriatic disease, defined as use of systemic treatment with phototherapy (psoralen plus UVA, UVB), traditional systemic treatment (acitretin, methotrexate, cyclosporine), or a biologic (P=.003).4
Topical steroids and vitamin D analogues may be used with or without occlusion and may be efficacious.5 Several case reports describe systemic treatments for psoriasis in children, including methotrexate, acitretin, and apremilast (approved for children 6 years and older for plaque psoriasis by the US Food and Drug Administration [FDA]).2 There are 5 biologic drugs currently approved for the treatment of pediatric psoriasis—adalimumab, etanercept, ustekinumab, secukinumab, ixekizumab—and 6 drugs currently undergoing phase 3 studies—brodalumab, guselkumab, risankizumab, tildrakizumab, certolizumab pegol, and deucravacitinib (Table 1).6-15 Adalimumab is specifically approved for moderate to severe nail psoriasis in adults 18 years and older.
Intralesional steroid injections are sometimes useful in the management of nail matrix psoriasis; however, appropriate patient selection is critical due to the pain associated with the procedure. In a prospective study of 16 children (age range, 9–17 years) with nail psoriasis treated with intralesional triamcinolone (ILTAC) 2.5 to 5 mg/mL every 4 to 8 weeks for a minimum of 3 to 6 months, 9 patients achieved resolution and 6 had improvement of clinical findings.16 Local adverse events were mild, including injection-site pain (66%), subungual hematoma (n=1), Beau lines (n=1), proximal nail fold hypopigmentation (n=2), and proximal nail fold atrophy (n=2). Because the proximal nail fold in children is thinner than in adults, there may be an increased risk for nail fold hypopigmentation and atrophy in children. Therefore, a maximum ILTAC concentration of 2.5 mg/mL with 0.2 mL maximum volume per nail per session is recommended for children younger than 15 years.16
Nail Lichen Planus
Nail lichen planus (NLP) is uncommon in children, with few biopsy-proven cases documented in the literature.17 Common clinical findings are onychorrhexis, nail plate thinning, fissuring, splitting, and atrophy with koilonychia.5 Although pterygium development (irreversible nail matrix scarring) is uncommon in pediatric patients, NLP can be progressive and may cause irreversible destruction of the nail matrix and subsequent nail loss, warranting therapeutic intervention.18
Treatment of NLP may be difficult, as there are no options that work in all patients. Current literature supports the use of systemic corticosteroids or ILTAC for the treatment of NLP; however, recurrence rates can be high. According to an expert consensus paper on NLP treatment, ILTAC may be injected in a concentration of 2.5, 5, or 10 mg/mL according to disease severity.19 In severe or resistant cases, intramuscular (IM) triamcinolone may be considered, especially if more than 3 nails are affected. A dosage of 0.5 to 1 mg/kg/mo for at least 3 to 6 months is recommended for both children and adults, with 1 mg/kg/mo recommended in the active treatment phase (first 2–3 months).19 In a retrospective review of 5 pediatric patients with NLP treated with IM triamcinolone 0.5 mg/kg/mo, 3 patients had resolution and 2 improved with treatment.20 In a prospective study of 10 children with NLP, IM triamcinolone at a dosage of 0.5 to 1 mg/kg every 30 days for 3 to 6 months resulted in resolution of nail findings in 9 patients.17 In a prospective study of 14 pediatric patients with NLP treated with 2.5 to 5 mg/mL of ILTAC, 10 achieved resolution and 3 improved.16
Intralesional triamcinolone injections may be better suited for teenagers compared to younger children who may be more apprehensive of needles. To minimize pain, it is recommended to inject ILTAC slowly at room temperature, with use of “talkesthesia” and vibration devices, 1% lidocaine, or ethyl chloride spray.18
Trachyonychia
Trachyonychia is characterized by the presence of sandpaperlike nails. It manifests with brittle thin nails with longitudinal ridging, onychoschizia, and thickened hyperkeratotic cuticles. Trachyonychia typically involves multiple nails, with a peak age of onset between 3 and 12 years.21,22 There are 2 variants: the opaque type with rough longitudinal ridging, and the shiny variant with opalescent nails and pits that reflect light. The opaque variant is more common and is associated with psoriasis, whereas the shiny variant is less common and is associated with alopecia areata.23 Although most cases are idiopathic, some are associated with psoriasis and alopecia areata, as previously noted, as well as atopic dermatitis (AD) and lichen planus.22,24
Fortunately, trachyonychia does not lead to permanent nail damage or pterygium, making treatment primarily focused on addressing functional and cosmetic concerns.24 Spontaneous resolution occurs in approximately 50% of patients. In a prospective study of 11 patients with idiopathic trachyonychia, there was partial improvement in 5 of 9 patients treated with topical steroids, 1 with only petrolatum, and 1 with vitamin supplements. Complete resolution was reported in 1 patient treated with topical steroids.25 Because trachyonychia often is self-resolving, no treatment is required and a conservative approach is strongly recommended.26 Treatment options include topical corticosteroids, tazarotene, and 5-fluorouracil. Intralesional triamcinolone, systemic cyclosporine, retinoids, systemic corticosteroids, and tofacitinib have been described in case reports, though none of these have been shown to be 100% efficacious.24
Nail Lichen Striatus
Lichen striatus involving the nail is uncommon and is characterized by onycholysis, longitudinal ridging, splitting, and fraying, as well as what appears to be a subungual tumor. It can encompass the entire nail or may be isolated to a portion of the nail (Figure 2). Usually, a Blaschko-linear array of flesh-colored papules on the more proximal digit directly adjacent to the nail dystrophy will be seen, though nail findings can occur in isolation.27-29 The underlying pathophysiology is not clear; however, one hypothesis is that a triggering event, such as trauma, induces the expression of antigens that elicit a self-limiting immune-mediated response by CD8 T lymphocytes.30
Generally, nail lichen striatus spontaneously resolves in 1 to 2 years without treatment. In a prospective study of 5 patients with nail lichen striatus, the median time to resolution was 22.6 months (range, 10–30 months).31 Topical steroids may be used for pruritus. In one case report, a 3-year-old boy with nail lichen striatus of 4 months’ duration was treated with tacrolimus ointment 0.03% daily for 3 months.28
Nail AD
Nail changes with AD may be more common in adults than children or are underreported. In a study of 777 adults with AD, nail dystrophy was present in 124 patients (16%), whereas in a study of 250 pediatric patients with AD (aged 0-2 years), nail dystrophy was present in only 4 patients.32,33
Periungual inflammation from AD causes the nail changes.34 In a cross-sectional study of 24 pediatric patients with nail dystrophy due to AD, transverse grooves (Beau lines) were present in 25% (6/24), nail pitting in 16.7% (4/24), koilonychia in 16.7% (4/24), trachyonychia in 12.5% (3/24), leukonychia in 12.5% (3/24), brachyonychia in 8.3% (2/24), melanonychia in 8.3% (2/24), onychomadesis in 8.3% (2/24), onychoschizia in 8.3% (2/24), and onycholysis in 8.3% (2/24). There was an association between disease severity and presence of toenail dystrophy (P=.03).35
Topical steroids with or without occlusion can be used to treat nail changes. Although there is limited literature describing the treatment of nail AD in children, a 61-year-old man with nail changes associated with AD achieved resolution with 3 months of treatment with dupilumab.36 Anecdotally, most patients will improve with usual cutaneous AD management.
INFECTIOUS NAIL DISORDERS
Viral Infections
Hand, Foot, and Mouth Disease—Hand, foot, and mouth disease (HFMD) is a common childhood viral infection caused by various enteroviruses, most commonly coxsackievirus A16, with the A6 variant causing more severe disease. Fever and painful vesicles involving the oral mucosa as well as palms and soles give the disease its name. Nail changes are common. In a prospective study involving 130 patients with laboratory-confirmed coxsackievirus CA6 serotype infection, 37% developed onychomadesis vs only 5% of 145 cases with non-CA6 enterovirus infection who developed nail findings. There was an association between CA6 infection and presence of nail changes (P<.001).37
Findings ranging from transverse grooves (Beau lines) to complete nail shedding (onychomadesis)(Figure 3) may be seen.38,39 Nail findings in HFMD are due to transient inhibition of nail growth and present approximately 3 to 6 weeks after infection.40 Onychomadesis is seen in 30% to 68% of patients with HFMD.37,41,42 Nail findings in HFMD spontaneously resolve with nail growth (2–3 mm per month for fingernails and 1 mm per month for toenails) and do not require specific treatment. Although the appearance of nail changes associated with HFMD can be disturbing, dermatologists can reassure children and their parents that the nails will resolve with the next cycle of growth.
Kawasaki Disease—Kawasaki disease (KD) is a vasculitis primarily affecting children and infants. Although the specific pathogen and pathophysiology is not entirely clear, clinical observations have suggested an infectious cause, most likely a virus.43 In Japan, more than 15,000 cases of KD are documented annually, while approximately 4200 cases are seen in the United States.44 In a prospective study from 1984 to 1990, 4 of 26 (15.4%) patients with KD presented with nail manifestations during the late acute phase or early convalescent phase of disease. There were no significant associations between nail dystrophy and severity of KD, such as coronary artery aneurysm.45
Nail changes reported in children with KD include onychomadesis, onycholysis, orange-brown chromonychia, splinter hemorrhages, Beau lines, and pincer nails. In a review of nail changes associated with KD from 1980 to 2021, orange-brown transverse chromonychia, which may evolve into transverse leukonychia, was the most common nail finding reported, occurring in 17 of 31 (54.8%) patients.44 It has been hypothesized that nail changes may result from blood flow disturbance due to the underlying vasculitis.46 Nail changes appear several weeks after the onset of fever and are self-limited. Resolution occurs with nail growth, with no treatment required.
FUNGAL INFECTIONS
Onychomycosis
Onychomycosis is a fungal infection of the nails that occurs in 0.2% to 5.5% of pediatric patients, and its prevalence may be increasing, which may be due to environmental factors or increased rates of diabetes mellitus and obesity in the pediatric population.47 Onychomycosis represents 15.5% of nail dystrophies in pediatric patients.48 Some dermatologists treat presumptive onychomycosis without confirmation; however, we do not recommend that approach. Because the differential is broad and the duration of treatment is long, mycologic examination (potassium hydroxide preparation, fungal culture, polymerase chain reaction, and/or histopathology) should be obtained to confirm onychomycosis prior to initiation of antifungal management. Family members of affected individuals should be evaluated and treated, if indicated, for onychomycosis and tinea pedis, as household transmission is common.
Currently, there are 2 topical FDA-approved treatments for pediatric onychomycosis in children 6 years and older (Table 2).49,50 There is a discussion of the need for confirmatory testing for onychomycosis in children, particularly when systemic treatment is prescribed. In a retrospective review of 269 pediatric patients with onychomycosis prescribed terbinafine, 53.5% (n=144) underwent laboratory monitoring of liver function and complete blood cell counts, and 12.5% had grade 1 laboratory abnormalities either prior to (12/144 [8.3%]) or during (6/144 [4.2%]) therapy.51 Baseline transaminase monitoring is recommended, though subsequent routine laboratory monitoring in healthy children may have limited utility with associated increased costs, incidental findings, and patient discomfort and likely is not needed.51
Pediatric onychomycosis responds better to topical therapy than adult disease, and pediatric patients do not always require systemic treatment.52 Ciclopirox is not FDA approved for the treatment of pediatric onychomycosis, but in a 32-week clinical trial of ciclopirox lacquer 8% use in 40 patients, 77% (27/35) of treated patients achieved mycologic cure. Overall, 71% of treated patients (25/35) vs 22% (2/9) of controls achieved efficacy (defined as investigator global assessment score of 2 or lower).52 In an open-label, single-arm clinical trial assessing tavaborole solution 5% applied once daily for 48 weeks for the treatment of toenail onychomycosis in pediatric patients (aged 6–17 years), 36.2% (20/55) of patients achieved mycologic cure, and 8.5% (5/55) achieved complete cure at week 52 with mild or minimal adverse effects.53 In an open-label, phase 4 study of the safety and efficacy of efinaconazole solution 10% applied once daily for 48 weeks in pediatric patients (aged 6 to 16 years) (n=60), 65% (35/60) achieved mycologic cure, 42% (25/60) achieved clinical cure, and 40% (24/60) achieved complete cure at 52 weeks. The most common adverse effects of efinaconazole were local and included ingrown toenail (1/60), application-site dermatitis (1/60), application-site vesicles (1/60), and application-site pain (1/60).54
In a systematic review of systemic antifungals for onychomycosis in 151 pediatric patients, itraconazole, fluconazole, griseofulvin, and terbinafine resulted in complete cure rates similar to those of the adult population, with excellent safety profiles.55 Depending on the situation, initiation of treatment with topical medications followed by addition of systemic antifungal agents only if needed may be an appropriate course of action.
BACTERIAL INFECTIONS
Acute Paronychia
Acute paronychia is a nail-fold infection that develops after the protective nail barrier has been compromised.56 In children, thumb-sucking, nail-biting, frequent oral manipulation of the digits, and poor skin hygiene are risk factors. Acute paronychia also may develop in association with congenital malalignment of the great toenails.57
Clinical manifestations include localized pain, erythema, and nail fold edema (Figure 4). Purulent material and abscess formation may ensue. Staphylococcus aureus as well as methicillin-resistant S aureus and Streptococcus pyogenes are classically the most common causes of acute paronychia. Treatment of paronychia is based on severity. In mild cases, warm soaks with topical antibiotics are indicated. Oral antibiotics should be prescribed for more severe presentations. If there is no improvement after 48 hours, surgical drainage is required to facilitate healing.56
FINAL THOUGHTS
Inflammatory and infectious nail disorders in children are relatively common and may impact the physical and emotional well-being of young patients. By understanding the distinctive features of these nail disorders in pediatric patients, dermatologists can provide anticipatory guidance and informed treatment options to children and their parents. Further research is needed to expand our understanding of pediatric nail disorders and create targeted therapeutic interventions, particularly for NLP and psoriasis.
- Uber M, Carvalho VO, Abagge KT, et al. Clinical features and nail clippings in 52 children with psoriasis. Pediatr Dermatol. 2018;35:202-207. doi:10.1111/pde.13402
- Plachouri KM, Mulita F, Georgiou S. Management of pediatric nail psoriasis. Cutis. 2021;108:292-294. doi:10.12788/cutis.0386
- Smith RJ, Rubin AI. Pediatric nail disorders: a review. Curr Opin Pediatr. 2020;32:506-515. doi:10.1097/mop.0000000000000921
- Pourchot D, Bodemer C, Phan A, et al. Nail psoriasis: a systematic evaluation in 313 children with psoriasis. Pediatr Dermatol. 2017;34:58-63. doi:10.1111/pde.13028
- Richert B, André J. Nail disorders in children: diagnosis and management. Am J Clin Dermatol. 2011;12:101-112. doi:10.2165/11537110-000000000-00000
- Lee JYY. Severe 20-nail psoriasis successfully treated by low dose methotrexate. Dermatol Online J. 2009;15:8.
- Nogueira M, Paller AS, Torres T. Targeted therapy for pediatric psoriasis. Paediatr Drugs. May 2021;23:203-212. doi:10.1007/s40272-021-00443-5
- Hanoodi M, Mittal M. Methotrexate. StatPearls [Internet]. Updated August 16, 2023. Accessed July 1, 2024. https://www.ncbi.nlm.nih.gov/books/NBK556114/
- Teran CG, Teran-Escalera CN, Balderrama C. A severe case of erythrodermic psoriasis associated with advanced nail and joint manifestations: a case report. J Med Case Rep. 2010;4:179. doi:10.1186/1752-1947-4-179
- Paller AS, Seyger MMB, Magariños GA, et al. Long-term efficacy and safety of up to 108 weeks of ixekizumab in pediatric patients with moderate to severe plaque psoriasis: the IXORA-PEDS randomized clinical trial. JAMA Dermatol. 2022;158:533-541. doi:10.1001/jamadermatol.2022.0655
- Diotallevi F, Simonetti O, Rizzetto G, et al. Biological treatments for pediatric psoriasis: state of the art and future perspectives. Int J Mol Sci. 2022;23:11128. doi:10.3390/ijms231911128
- Nash P, Mease PJ, Kirkham B, et al. Secukinumab provides sustained improvement in nail psoriasis, signs and symptoms of psoriatic arthritis and low rate of radiographic progression in patients with concomitant nail involvement: 2-year results from the Phase III FUTURE 5 study. Clin Exp Rheumatol. 2022;40:952-959. doi:10.55563/clinexprheumatol/3nuz51
- Wells LE, Evans T, Hilton R, et al. Use of secukinumab in a pediatric patient leads to significant improvement in nail psoriasis and psoriatic arthritis. Pediatr Dermatol. 2019;36:384-385. doi:10.1111/pde.13767
- Watabe D, Endoh K, Maeda F, et al. Childhood-onset psoriaticonycho-pachydermo-periostitis treated successfully with infliximab. Eur J Dermatol. 2015;25:506-508. doi:10.1684/ejd.2015.2616
- Pereira TM, Vieira AP, Fernandes JC, et al. Anti-TNF-alpha therapy in childhood pustular psoriasis. Dermatology. 2006;213:350-352. doi:10.1159/000096202
- Iorizzo M, Gioia Di Chiacchio N, Di Chiacchio N, et al. Intralesional steroid injections for inflammatory nail dystrophies in the pediatric population. Pediatr Dermatol. 2023;40:759-761. doi:10.1111/pde.15295
- Tosti A, Piraccini BM, Cambiaghi S, et al. Nail lichen planus in children: clinical features, response to treatment, and long-term follow-up. Arch Dermatol. 2001;137:1027-1032.
- Lipner SR. Nail lichen planus: a true nail emergency. J Am Acad Dermatol. 2019;80:e177-e178. doi:10.1016/j.jaad.2018.11.065
- Iorizzo M, Tosti A, Starace M, et al. Isolated nail lichen planus: an expert consensus on treatment of the classical form. J Am Acad Dermatol. 2020;83:1717-1723. doi:10.1016/j.jaad.2020.02.056
- Piraccini BM, Saccani E, Starace M, et al. Nail lichen planus: response to treatment and long term follow-up. Eur J Dermatol. 2010;20:489-496. doi:10.1684/ejd.2010.0952
- Mahajan R, Kaushik A, De D, et al. Pediatric trachyonychia- a retrospective study of 17 cases. Indian J Dermatol. 2021;66:689-690. doi:10.4103/ijd.ijd_42_21
- Leung AKC, Leong KF, Barankin B. Trachyonychia. J Pediatr. 2020;216:239-239.e1. doi:10.1016/j.jpeds.2019.08.034
- Haber JS, Chairatchaneeboon M, Rubin AI. Trachyonychia: review and update on clinical aspects, histology, and therapy. Skin Appendage Disord. 2017;2:109-115. doi:10.1159/000449063
- Jacobsen AA, Tosti A. Trachyonychia and twenty-nail dystrophy: a comprehensive review and discussion of diagnostic accuracy. Skin Appendage Disord. 2016;2:7-13. doi:10.1159/000445544
- Kumar MG, Ciliberto H, Bayliss SJ. Long-term follow-up of pediatric trachyonychia. Pediatr Dermatol. 2015;32:198-200. doi:10.1111/pde.12427
- Tosti A, Piraccini BM, Iorizzo M. Trachyonychia and related disorders: evaluation and treatment plans. Dermatolog Ther. 2002;15:121-125. doi:10.1046/j.1529-8019.2002.01511.x
- Leung AKC, Leong KF, Barankin B. Lichen striatus with nail involvement in a 6-year-old boy. Case Rep Pediatr. 2020;2020:1494760. doi:10.1155/2020/1494760
- Kim GW, Kim SH, Seo SH, et al. Lichen striatus with nail abnormality successfully treated with tacrolimus ointment. J Dermatol. 2009;36:616-617. doi:10.1111/j.1346-8138.2009.00720.x
- Iorizzo M, Rubin AI, Starace M. Nail lichen striatus: is dermoscopy useful for the diagnosis? Pediatr Dermatol. 2019;36:859-863. doi:10.1111/pde.13916
- Karp DL, Cohen BA. Onychodystrophy in lichen striatus. Pediatr Dermatol. 1993;10:359-361. doi:10.1111/j.1525-1470.1993.tb00399.x
- Tosti A, Peluso AM, Misciali C, et al. Nail lichen striatus: clinical features and long-term follow-up of five patients. J Am Acad Dermatol. 1997;36(6, pt 1):908-913. doi:10.1016/s0190-9622(97)80270-8
- Simpson EL, Thompson MM, Hanifin JM. Prevalence and morphology of hand eczema in patients with atopic dermatitis. Dermatitis. 2006;17:123-127. doi:10.2310/6620.2006.06005
- Sarifakioglu E, Yilmaz AE, Gorpelioglu C. Nail alterations in 250 infant patients: a clinical study. J Eur Acad Dermatol Venereol. 2008;22:741-744. doi:10.1111/j.1468-3083.2008.02592.x
- Milanesi N, D’Erme AM, Gola M. Nail improvement during alitretinoin treatment: three case reports and review of the literature. Clin Exp Dermatol. 2015;40:533-536. doi:10.1111/ced.12584
- Chung BY, Choi YW, Kim HO, et al. Nail dystrophy in patients with atopic dermatitis and its association with disease severity. Ann Dermatol. 2019;31:121-126. doi:10.5021/ad.2019.31.2.121
- Navarro-Triviño FJ, Vega-Castillo JJ, Ruiz-Villaverde R. Nail changes successfully treated with dupilumab in a patient with severe atopic dermatitis. Australas J Dermatol. 2021;62:e468-e469. doi:10.1111/ajd.13633
- Wei SH, Huang YP, Liu MC, et al. An outbreak of coxsackievirus A6 hand, foot, and mouth disease associated with onychomadesis in Taiwan, 2010. BMC Infect Dis. 2011;11:346. doi:10.1186/1471-2334-11-346
- Shin JY, Cho BK, Park HJ. A clinical study of nail changes occurring secondary to hand-foot-mouth disease: onychomadesis and Beau’s lines. Ann Dermatol. 2014;26:280-283. doi:10.5021/ad.2014.26.2.280
- Verma S, Singal A. Nail changes in hand-foot-and-mouth disease (HFMD). Indian Dermatol Online J. 2021;12:656-657. doi:10.4103 /idoj.IDOJ_271_20
- Giordano LMC, de la Fuente LA, Lorca JMB, et al. Onychomadesis secondary to hand-foot-mouth disease: a frequent manifestation and cause of concern for parents. Article in Spanish. Rev Chil Pediatr. 2018;89:380-383. doi:10.4067/s0370-41062018005000203
- Justino MCA, da SMD, Souza MF, et al. Atypical hand-foot-mouth disease in Belém, Amazon region, northern Brazil, with detection of coxsackievirus A6. J Clin Virol. 2020;126:104307. doi:10.1016/j.jcv.2020.104307
- Cheng FF, Zhang BB, Cao ML, et al. Clinical characteristics of 68 children with atypical hand, foot, and mouth disease caused by coxsackievirus A6: a single-center retrospective analysis. Transl Pediatr. 2022;11:1502-1509. doi:10.21037/tp-22-352
- Nagata S. Causes of Kawasaki disease-from past to present. Front Pediatr. 2019;7:18. doi:10.3389/fped.2019.00018
- Mitsuishi T, Miyata K, Ando A, et al. Characteristic nail lesions in Kawasaki disease: case series and literature review. J Dermatol. 2022;49:232-238. doi:10.1111/1346-8138.16276
- Lindsley CB. Nail-bed lines in Kawasaki disease. Am J Dis Child. 1992;146:659-660. doi:10.1001/archpedi.1992.02160180017005
- Matsumura O, Nakagishi Y. Pincer nails upon convalescence from Kawasaki disease. J Pediatr. 2022;246:279. doi:10.1016/j.jpeds.2022.03.002
- Solís-Arias MP, García-Romero MT. Onychomycosis in children. a review. Int J Dermatol. 2017;56:123-130. doi:10.1111/ijd.13392
- Gupta AK, Mays RR, Versteeg SG, et al. Onychomycosis in children: safety and efficacy of antifungal agents. Pediatr Dermatol. 2018;35:552-559. doi:10.1111/pde.13561
- 49. Gupta AK, Venkataraman M, Shear NH, et al. Labeled use of efinaconazole topical solution 10% in treating onychomycosis in children and a review of the management of pediatric onychomycosis. Dermatol Ther. 2020;33:e13613. doi:10.1111/dth.13613
- Falotico JM, Lipner SR. Updated perspectives on the diagnosis and management of onychomycosis. Clin Cosmet Investig Dermatol. 2022;15:1933-1957. doi:10.2147/ccid.S362635
- Patel D, Castelo-Soccio LA, Rubin AI, et al. Laboratory monitoring during systemic terbinafine therapy for pediatric onychomycosis. JAMA Dermatol. 2017;153:1326-1327. doi:10.1001/jamadermatol.2017.4483
- Friedlander SF, Chan YC, Chan YH, et al. Onychomycosis does not always require systemic treatment for cure: a trial using topical therapy. Pediatr Dermatol. 2013;30:316-322. doi:10.1111/pde.12064
- Rich P, Spellman M, Purohit V, et al. Tavaborole 5% topical solution for the treatment of toenail onychomycosis in pediatric patients: results from a phase 4 open-label study. J Drugs Dermatol. 2019;18:190-195.
- Gupta AK, Venkataraman M, Abramovits W, et al. JUBLIA (efinaconazole 10% solution) in the treatment of pediatric onychomycosis. Skinmed. 2021;19:206-210.
- Gupta AK, Paquet M. Systemic antifungals to treat onychomycosis in children: a systematic review. Pediatr Dermatol. 2013;30:294-302. doi:10.1111/pde.12048
- Leggit JC. Acute and chronic paronychia. Am Fam Physician. 2017;96:44-51.
- Lipner SR, Scher RK. Congenital malalignment of the great toenails with acute paronychia. Pediatr Dermatol. 2016;33:e288-e289.doi:10.1111/pde.12924
- Uber M, Carvalho VO, Abagge KT, et al. Clinical features and nail clippings in 52 children with psoriasis. Pediatr Dermatol. 2018;35:202-207. doi:10.1111/pde.13402
- Plachouri KM, Mulita F, Georgiou S. Management of pediatric nail psoriasis. Cutis. 2021;108:292-294. doi:10.12788/cutis.0386
- Smith RJ, Rubin AI. Pediatric nail disorders: a review. Curr Opin Pediatr. 2020;32:506-515. doi:10.1097/mop.0000000000000921
- Pourchot D, Bodemer C, Phan A, et al. Nail psoriasis: a systematic evaluation in 313 children with psoriasis. Pediatr Dermatol. 2017;34:58-63. doi:10.1111/pde.13028
- Richert B, André J. Nail disorders in children: diagnosis and management. Am J Clin Dermatol. 2011;12:101-112. doi:10.2165/11537110-000000000-00000
- Lee JYY. Severe 20-nail psoriasis successfully treated by low dose methotrexate. Dermatol Online J. 2009;15:8.
- Nogueira M, Paller AS, Torres T. Targeted therapy for pediatric psoriasis. Paediatr Drugs. May 2021;23:203-212. doi:10.1007/s40272-021-00443-5
- Hanoodi M, Mittal M. Methotrexate. StatPearls [Internet]. Updated August 16, 2023. Accessed July 1, 2024. https://www.ncbi.nlm.nih.gov/books/NBK556114/
- Teran CG, Teran-Escalera CN, Balderrama C. A severe case of erythrodermic psoriasis associated with advanced nail and joint manifestations: a case report. J Med Case Rep. 2010;4:179. doi:10.1186/1752-1947-4-179
- Paller AS, Seyger MMB, Magariños GA, et al. Long-term efficacy and safety of up to 108 weeks of ixekizumab in pediatric patients with moderate to severe plaque psoriasis: the IXORA-PEDS randomized clinical trial. JAMA Dermatol. 2022;158:533-541. doi:10.1001/jamadermatol.2022.0655
- Diotallevi F, Simonetti O, Rizzetto G, et al. Biological treatments for pediatric psoriasis: state of the art and future perspectives. Int J Mol Sci. 2022;23:11128. doi:10.3390/ijms231911128
- Nash P, Mease PJ, Kirkham B, et al. Secukinumab provides sustained improvement in nail psoriasis, signs and symptoms of psoriatic arthritis and low rate of radiographic progression in patients with concomitant nail involvement: 2-year results from the Phase III FUTURE 5 study. Clin Exp Rheumatol. 2022;40:952-959. doi:10.55563/clinexprheumatol/3nuz51
- Wells LE, Evans T, Hilton R, et al. Use of secukinumab in a pediatric patient leads to significant improvement in nail psoriasis and psoriatic arthritis. Pediatr Dermatol. 2019;36:384-385. doi:10.1111/pde.13767
- Watabe D, Endoh K, Maeda F, et al. Childhood-onset psoriaticonycho-pachydermo-periostitis treated successfully with infliximab. Eur J Dermatol. 2015;25:506-508. doi:10.1684/ejd.2015.2616
- Pereira TM, Vieira AP, Fernandes JC, et al. Anti-TNF-alpha therapy in childhood pustular psoriasis. Dermatology. 2006;213:350-352. doi:10.1159/000096202
- Iorizzo M, Gioia Di Chiacchio N, Di Chiacchio N, et al. Intralesional steroid injections for inflammatory nail dystrophies in the pediatric population. Pediatr Dermatol. 2023;40:759-761. doi:10.1111/pde.15295
- Tosti A, Piraccini BM, Cambiaghi S, et al. Nail lichen planus in children: clinical features, response to treatment, and long-term follow-up. Arch Dermatol. 2001;137:1027-1032.
- Lipner SR. Nail lichen planus: a true nail emergency. J Am Acad Dermatol. 2019;80:e177-e178. doi:10.1016/j.jaad.2018.11.065
- Iorizzo M, Tosti A, Starace M, et al. Isolated nail lichen planus: an expert consensus on treatment of the classical form. J Am Acad Dermatol. 2020;83:1717-1723. doi:10.1016/j.jaad.2020.02.056
- Piraccini BM, Saccani E, Starace M, et al. Nail lichen planus: response to treatment and long term follow-up. Eur J Dermatol. 2010;20:489-496. doi:10.1684/ejd.2010.0952
- Mahajan R, Kaushik A, De D, et al. Pediatric trachyonychia- a retrospective study of 17 cases. Indian J Dermatol. 2021;66:689-690. doi:10.4103/ijd.ijd_42_21
- Leung AKC, Leong KF, Barankin B. Trachyonychia. J Pediatr. 2020;216:239-239.e1. doi:10.1016/j.jpeds.2019.08.034
- Haber JS, Chairatchaneeboon M, Rubin AI. Trachyonychia: review and update on clinical aspects, histology, and therapy. Skin Appendage Disord. 2017;2:109-115. doi:10.1159/000449063
- Jacobsen AA, Tosti A. Trachyonychia and twenty-nail dystrophy: a comprehensive review and discussion of diagnostic accuracy. Skin Appendage Disord. 2016;2:7-13. doi:10.1159/000445544
- Kumar MG, Ciliberto H, Bayliss SJ. Long-term follow-up of pediatric trachyonychia. Pediatr Dermatol. 2015;32:198-200. doi:10.1111/pde.12427
- Tosti A, Piraccini BM, Iorizzo M. Trachyonychia and related disorders: evaluation and treatment plans. Dermatolog Ther. 2002;15:121-125. doi:10.1046/j.1529-8019.2002.01511.x
- Leung AKC, Leong KF, Barankin B. Lichen striatus with nail involvement in a 6-year-old boy. Case Rep Pediatr. 2020;2020:1494760. doi:10.1155/2020/1494760
- Kim GW, Kim SH, Seo SH, et al. Lichen striatus with nail abnormality successfully treated with tacrolimus ointment. J Dermatol. 2009;36:616-617. doi:10.1111/j.1346-8138.2009.00720.x
- Iorizzo M, Rubin AI, Starace M. Nail lichen striatus: is dermoscopy useful for the diagnosis? Pediatr Dermatol. 2019;36:859-863. doi:10.1111/pde.13916
- Karp DL, Cohen BA. Onychodystrophy in lichen striatus. Pediatr Dermatol. 1993;10:359-361. doi:10.1111/j.1525-1470.1993.tb00399.x
- Tosti A, Peluso AM, Misciali C, et al. Nail lichen striatus: clinical features and long-term follow-up of five patients. J Am Acad Dermatol. 1997;36(6, pt 1):908-913. doi:10.1016/s0190-9622(97)80270-8
- Simpson EL, Thompson MM, Hanifin JM. Prevalence and morphology of hand eczema in patients with atopic dermatitis. Dermatitis. 2006;17:123-127. doi:10.2310/6620.2006.06005
- Sarifakioglu E, Yilmaz AE, Gorpelioglu C. Nail alterations in 250 infant patients: a clinical study. J Eur Acad Dermatol Venereol. 2008;22:741-744. doi:10.1111/j.1468-3083.2008.02592.x
- Milanesi N, D’Erme AM, Gola M. Nail improvement during alitretinoin treatment: three case reports and review of the literature. Clin Exp Dermatol. 2015;40:533-536. doi:10.1111/ced.12584
- Chung BY, Choi YW, Kim HO, et al. Nail dystrophy in patients with atopic dermatitis and its association with disease severity. Ann Dermatol. 2019;31:121-126. doi:10.5021/ad.2019.31.2.121
- Navarro-Triviño FJ, Vega-Castillo JJ, Ruiz-Villaverde R. Nail changes successfully treated with dupilumab in a patient with severe atopic dermatitis. Australas J Dermatol. 2021;62:e468-e469. doi:10.1111/ajd.13633
- Wei SH, Huang YP, Liu MC, et al. An outbreak of coxsackievirus A6 hand, foot, and mouth disease associated with onychomadesis in Taiwan, 2010. BMC Infect Dis. 2011;11:346. doi:10.1186/1471-2334-11-346
- Shin JY, Cho BK, Park HJ. A clinical study of nail changes occurring secondary to hand-foot-mouth disease: onychomadesis and Beau’s lines. Ann Dermatol. 2014;26:280-283. doi:10.5021/ad.2014.26.2.280
- Verma S, Singal A. Nail changes in hand-foot-and-mouth disease (HFMD). Indian Dermatol Online J. 2021;12:656-657. doi:10.4103 /idoj.IDOJ_271_20
- Giordano LMC, de la Fuente LA, Lorca JMB, et al. Onychomadesis secondary to hand-foot-mouth disease: a frequent manifestation and cause of concern for parents. Article in Spanish. Rev Chil Pediatr. 2018;89:380-383. doi:10.4067/s0370-41062018005000203
- Justino MCA, da SMD, Souza MF, et al. Atypical hand-foot-mouth disease in Belém, Amazon region, northern Brazil, with detection of coxsackievirus A6. J Clin Virol. 2020;126:104307. doi:10.1016/j.jcv.2020.104307
- Cheng FF, Zhang BB, Cao ML, et al. Clinical characteristics of 68 children with atypical hand, foot, and mouth disease caused by coxsackievirus A6: a single-center retrospective analysis. Transl Pediatr. 2022;11:1502-1509. doi:10.21037/tp-22-352
- Nagata S. Causes of Kawasaki disease-from past to present. Front Pediatr. 2019;7:18. doi:10.3389/fped.2019.00018
- Mitsuishi T, Miyata K, Ando A, et al. Characteristic nail lesions in Kawasaki disease: case series and literature review. J Dermatol. 2022;49:232-238. doi:10.1111/1346-8138.16276
- Lindsley CB. Nail-bed lines in Kawasaki disease. Am J Dis Child. 1992;146:659-660. doi:10.1001/archpedi.1992.02160180017005
- Matsumura O, Nakagishi Y. Pincer nails upon convalescence from Kawasaki disease. J Pediatr. 2022;246:279. doi:10.1016/j.jpeds.2022.03.002
- Solís-Arias MP, García-Romero MT. Onychomycosis in children. a review. Int J Dermatol. 2017;56:123-130. doi:10.1111/ijd.13392
- Gupta AK, Mays RR, Versteeg SG, et al. Onychomycosis in children: safety and efficacy of antifungal agents. Pediatr Dermatol. 2018;35:552-559. doi:10.1111/pde.13561
- 49. Gupta AK, Venkataraman M, Shear NH, et al. Labeled use of efinaconazole topical solution 10% in treating onychomycosis in children and a review of the management of pediatric onychomycosis. Dermatol Ther. 2020;33:e13613. doi:10.1111/dth.13613
- Falotico JM, Lipner SR. Updated perspectives on the diagnosis and management of onychomycosis. Clin Cosmet Investig Dermatol. 2022;15:1933-1957. doi:10.2147/ccid.S362635
- Patel D, Castelo-Soccio LA, Rubin AI, et al. Laboratory monitoring during systemic terbinafine therapy for pediatric onychomycosis. JAMA Dermatol. 2017;153:1326-1327. doi:10.1001/jamadermatol.2017.4483
- Friedlander SF, Chan YC, Chan YH, et al. Onychomycosis does not always require systemic treatment for cure: a trial using topical therapy. Pediatr Dermatol. 2013;30:316-322. doi:10.1111/pde.12064
- Rich P, Spellman M, Purohit V, et al. Tavaborole 5% topical solution for the treatment of toenail onychomycosis in pediatric patients: results from a phase 4 open-label study. J Drugs Dermatol. 2019;18:190-195.
- Gupta AK, Venkataraman M, Abramovits W, et al. JUBLIA (efinaconazole 10% solution) in the treatment of pediatric onychomycosis. Skinmed. 2021;19:206-210.
- Gupta AK, Paquet M. Systemic antifungals to treat onychomycosis in children: a systematic review. Pediatr Dermatol. 2013;30:294-302. doi:10.1111/pde.12048
- Leggit JC. Acute and chronic paronychia. Am Fam Physician. 2017;96:44-51.
- Lipner SR, Scher RK. Congenital malalignment of the great toenails with acute paronychia. Pediatr Dermatol. 2016;33:e288-e289.doi:10.1111/pde.12924
Practice Points
- Nail plate pitting is the most common clinical sign of nail psoriasis in children.
- Nail changes are common in hand, foot, and mouth disease, with the most frequent being onychomadesis.
- Because onychomycosis may resemble other nail disorders, mycologic confirmation is recommended to avoid misdiagnosis.
- Many nail conditions in children self-resolve but recognizing these manifestations is important in providing anticipatory guidance to patients and caregivers.
Weakness on one side of the body
FHM is a rare phenotype of migraine with aura with a characteristic presentation of motor aura. Motor aura presents as unilateral muscle weakness that tends to be felt first in the hands or arm and may spread to the face. To date, three distinct types have been identified by mutations in one of three genes. Type 1 is the most common and is associated with mutations in the gene CACNA1A. Mutations in ATP1A2 underlie type 2 FHM, and mutations in SCN1A underlie type 3 FHM.
FHM is distinguished from other hemiplegic migraine by family history of one or more affected first- or second-degree relatives. Genetic studies have shown FHM to have autosomal dominant inheritance. From half to three quarters of patients with FHM will have one of the more than 30 identified mutations on CACNA1A that diagnose type 1 FHM. These mutations affect transmission of glutamate in the neurons and neuronal reactions, increasing the susceptibility to cortical spreading depression associated with migraine. Mutations in ATP1A2 are found in about 20% of patients with FHM (type 2). More than 80 individual mutations have been identified, which alter sodium-potassium metabolism in neurons. About 5% of patients have type 3 FHM, associated with mutations in SCN1A that create gain of function or loss of function in neuronal voltage-gated sodium channels. Studies of other possible genes and mutations in relation to FHM, including PRRT2, are ongoing, but to date the associations are not clearly established.
Patients with FHM may also report sensory symptoms, visual disturbances, or aphasia. FHM generally affects people in their teens and twenties (women more than men) and has an estimated prevalence of 0.003% of the population. On average, patients report having two to three attacks per year, and some patients go for extended periods without a recurrent attack. Motor aura may occur on the same or opposite side of the body as headache and may alternate affected sides with each attack. Differential diagnoses that should be ruled out include transient ischemic attacks, infections (eg, meningitis, encephalitis), tumors, seizures, other inherited disorders, and metabolic issues.
Like other forms of migraine with aura, FHM is treated with abortive and/or preventive medications. Given the rarity of FHM, there are few studies specifically in families with this phenotype. Patients should be counseled on trigger avoidance to limit exposure. Acute treatment includes nonsteroidal anti-inflammatory drugs, acetaminophen, and other nonopioid pain relievers. The class of calcitonin gene-related peptide (CGRP) antagonists (rimegepant, ubrogepant, zavegepant) may be considered. However, with FHM, medications associated with ischemia must be avoided. As such, triptans and ergotamines are generally contraindicated, as are beta-blockers. Patients with FHM and more frequent or severe attacks may be considered for preventive treatment to improve function and quality of life and avoid reliance on acute therapies. Options include CGRP monoclonal antibodies (mAbs), administered subcutaneously or by intravenous infusion, and onabotulinumtoxinA injection. Current CGRP mAbs include eptinezumab, erenumab, fremanezumab, and galcanezumab. Combined CGRP mAb therapy with onabotulinumtoxinA may be an effective alternative for patients with resistant FHM.
Heidi Moawad, MD, Clinical Assistant Professor, Department of Medical Education, Case Western Reserve University School of Medicine, Cleveland, Ohio.
Heidi Moawad, MD, has disclosed no relevant financial relationships.
Image Quizzes are fictional or fictionalized clinical scenarios intended to provide evidence-based educational takeaways.
FHM is a rare phenotype of migraine with aura with a characteristic presentation of motor aura. Motor aura presents as unilateral muscle weakness that tends to be felt first in the hands or arm and may spread to the face. To date, three distinct types have been identified by mutations in one of three genes. Type 1 is the most common and is associated with mutations in the gene CACNA1A. Mutations in ATP1A2 underlie type 2 FHM, and mutations in SCN1A underlie type 3 FHM.
FHM is distinguished from other hemiplegic migraine by family history of one or more affected first- or second-degree relatives. Genetic studies have shown FHM to have autosomal dominant inheritance. From half to three quarters of patients with FHM will have one of the more than 30 identified mutations on CACNA1A that diagnose type 1 FHM. These mutations affect transmission of glutamate in the neurons and neuronal reactions, increasing the susceptibility to cortical spreading depression associated with migraine. Mutations in ATP1A2 are found in about 20% of patients with FHM (type 2). More than 80 individual mutations have been identified, which alter sodium-potassium metabolism in neurons. About 5% of patients have type 3 FHM, associated with mutations in SCN1A that create gain of function or loss of function in neuronal voltage-gated sodium channels. Studies of other possible genes and mutations in relation to FHM, including PRRT2, are ongoing, but to date the associations are not clearly established.
Patients with FHM may also report sensory symptoms, visual disturbances, or aphasia. FHM generally affects people in their teens and twenties (women more than men) and has an estimated prevalence of 0.003% of the population. On average, patients report having two to three attacks per year, and some patients go for extended periods without a recurrent attack. Motor aura may occur on the same or opposite side of the body as headache and may alternate affected sides with each attack. Differential diagnoses that should be ruled out include transient ischemic attacks, infections (eg, meningitis, encephalitis), tumors, seizures, other inherited disorders, and metabolic issues.
Like other forms of migraine with aura, FHM is treated with abortive and/or preventive medications. Given the rarity of FHM, there are few studies specifically in families with this phenotype. Patients should be counseled on trigger avoidance to limit exposure. Acute treatment includes nonsteroidal anti-inflammatory drugs, acetaminophen, and other nonopioid pain relievers. The class of calcitonin gene-related peptide (CGRP) antagonists (rimegepant, ubrogepant, zavegepant) may be considered. However, with FHM, medications associated with ischemia must be avoided. As such, triptans and ergotamines are generally contraindicated, as are beta-blockers. Patients with FHM and more frequent or severe attacks may be considered for preventive treatment to improve function and quality of life and avoid reliance on acute therapies. Options include CGRP monoclonal antibodies (mAbs), administered subcutaneously or by intravenous infusion, and onabotulinumtoxinA injection. Current CGRP mAbs include eptinezumab, erenumab, fremanezumab, and galcanezumab. Combined CGRP mAb therapy with onabotulinumtoxinA may be an effective alternative for patients with resistant FHM.
Heidi Moawad, MD, Clinical Assistant Professor, Department of Medical Education, Case Western Reserve University School of Medicine, Cleveland, Ohio.
Heidi Moawad, MD, has disclosed no relevant financial relationships.
Image Quizzes are fictional or fictionalized clinical scenarios intended to provide evidence-based educational takeaways.
FHM is a rare phenotype of migraine with aura with a characteristic presentation of motor aura. Motor aura presents as unilateral muscle weakness that tends to be felt first in the hands or arm and may spread to the face. To date, three distinct types have been identified by mutations in one of three genes. Type 1 is the most common and is associated with mutations in the gene CACNA1A. Mutations in ATP1A2 underlie type 2 FHM, and mutations in SCN1A underlie type 3 FHM.
FHM is distinguished from other hemiplegic migraine by family history of one or more affected first- or second-degree relatives. Genetic studies have shown FHM to have autosomal dominant inheritance. From half to three quarters of patients with FHM will have one of the more than 30 identified mutations on CACNA1A that diagnose type 1 FHM. These mutations affect transmission of glutamate in the neurons and neuronal reactions, increasing the susceptibility to cortical spreading depression associated with migraine. Mutations in ATP1A2 are found in about 20% of patients with FHM (type 2). More than 80 individual mutations have been identified, which alter sodium-potassium metabolism in neurons. About 5% of patients have type 3 FHM, associated with mutations in SCN1A that create gain of function or loss of function in neuronal voltage-gated sodium channels. Studies of other possible genes and mutations in relation to FHM, including PRRT2, are ongoing, but to date the associations are not clearly established.
Patients with FHM may also report sensory symptoms, visual disturbances, or aphasia. FHM generally affects people in their teens and twenties (women more than men) and has an estimated prevalence of 0.003% of the population. On average, patients report having two to three attacks per year, and some patients go for extended periods without a recurrent attack. Motor aura may occur on the same or opposite side of the body as headache and may alternate affected sides with each attack. Differential diagnoses that should be ruled out include transient ischemic attacks, infections (eg, meningitis, encephalitis), tumors, seizures, other inherited disorders, and metabolic issues.
Like other forms of migraine with aura, FHM is treated with abortive and/or preventive medications. Given the rarity of FHM, there are few studies specifically in families with this phenotype. Patients should be counseled on trigger avoidance to limit exposure. Acute treatment includes nonsteroidal anti-inflammatory drugs, acetaminophen, and other nonopioid pain relievers. The class of calcitonin gene-related peptide (CGRP) antagonists (rimegepant, ubrogepant, zavegepant) may be considered. However, with FHM, medications associated with ischemia must be avoided. As such, triptans and ergotamines are generally contraindicated, as are beta-blockers. Patients with FHM and more frequent or severe attacks may be considered for preventive treatment to improve function and quality of life and avoid reliance on acute therapies. Options include CGRP monoclonal antibodies (mAbs), administered subcutaneously or by intravenous infusion, and onabotulinumtoxinA injection. Current CGRP mAbs include eptinezumab, erenumab, fremanezumab, and galcanezumab. Combined CGRP mAb therapy with onabotulinumtoxinA may be an effective alternative for patients with resistant FHM.
Heidi Moawad, MD, Clinical Assistant Professor, Department of Medical Education, Case Western Reserve University School of Medicine, Cleveland, Ohio.
Heidi Moawad, MD, has disclosed no relevant financial relationships.
Image Quizzes are fictional or fictionalized clinical scenarios intended to provide evidence-based educational takeaways.
The patient is 35-year-old woman presenting for recurrent, unilateral headaches associated with weakness in the hand, arm, or face on one side of the body. The patient says this weakness sometimes occurs on the right side and other times on the left, often with a tingling sensation in the affected side, and is followed by an intense headache lasting for several hours.
She notes that the headaches started after recovery from a mild case of COVID. Over the past 2 years, five attacks have occurred, all following a similar pattern. With each attack, the motor weakness fully resolved with resolution of the headache. Two of the headaches were preceded by visual disturbances that resolved with headache onset.
Physical exam reveals an apparently healthy woman without fever or respiratory symptoms. Weight, blood pressure, and heart rate are within healthy ranges. All lab work is within normal ranges. Her facial appearance is normal at presentation, but she shows a photo taken during her last attack, in which she shows left side facial paralysis. Family history includes her mother with hemiplegic migraine and father with type 2 diabetes. You suspect familial hemiplegic migraine (FHM) and order genetic testing.
Optimal JAK Inhibition for Severe Alopecia Areata
Alopecia areata (AA) is an autoimmune condition that affects children, adolescents, and adults. Severe AA often causes significant burdens, physical discomfort, and psychological stress. Yet, response to therapy is often unpredictable, running the gamut from being refractory to treatment to spontaneous remission.
Dermatologists Raj Chovatiya from Chicago Medical School and Jason Hawkes from the Pacific Skin Institute discuss how to assess AA severity and appropriate therapies, particularly the evolving landscape of JAK inhibitors for patients with severe AA.
The panelists begin by defining severe AA on the basis of the Severity of Alopecia Tool (SALT) score, which assesses AA severity by percentage of hair loss. A patient who has lost over 50% of scalp hair is considered to have severe AA.
Until recently, traditional therapy for severe AA has relied on injectable and systemic corticosteroids, both of which have drawbacks for patients with severe disease. The emergence of Janus kinase (JAK) inhibitors has offered another option for these patients. The panelists discuss recent clinical trials that have shown promising results from JAK inhibitors for treatment of severe AA.
--
Raj Chovatiya, MD, PhD, MSCI, Clinical Associate Professor, Chicago Medical School, Rosalind Franklin University of Medicine and Science, North Chicago, Illinois; Founder and Director, Center for Medical Dermatology and Immunology Research, Chicago, Illinois
Raj Chovatiya, MD, PhD, MSCI, has disclosed the following relevant financial relationships:
Serve(d) as a director, officer, partner, employee, advisor, consultant, or trustee for: AbbVie; Amgen; Apogee Therapeutics; Arcutis; Argenx; ASLAN Pharmaceuticals; Beiersdorf; Boehringer Ingelheim; Bristol Myers Squibb; Cara Therapeutics; Dermavant; Eli Lilly
Serve(d) as a speaker or a member of a speakers bureau for: AbbVie; Arcutis; Beiersdorf; Boehringer Ingelheim; Bristol Myers Squibb; Dermavant; Eli Lilly and Company; Incyte; LEO Pharma
Alopecia areata (AA) is an autoimmune condition that affects children, adolescents, and adults. Severe AA often causes significant burdens, physical discomfort, and psychological stress. Yet, response to therapy is often unpredictable, running the gamut from being refractory to treatment to spontaneous remission.
Dermatologists Raj Chovatiya from Chicago Medical School and Jason Hawkes from the Pacific Skin Institute discuss how to assess AA severity and appropriate therapies, particularly the evolving landscape of JAK inhibitors for patients with severe AA.
The panelists begin by defining severe AA on the basis of the Severity of Alopecia Tool (SALT) score, which assesses AA severity by percentage of hair loss. A patient who has lost over 50% of scalp hair is considered to have severe AA.
Until recently, traditional therapy for severe AA has relied on injectable and systemic corticosteroids, both of which have drawbacks for patients with severe disease. The emergence of Janus kinase (JAK) inhibitors has offered another option for these patients. The panelists discuss recent clinical trials that have shown promising results from JAK inhibitors for treatment of severe AA.
--
Raj Chovatiya, MD, PhD, MSCI, Clinical Associate Professor, Chicago Medical School, Rosalind Franklin University of Medicine and Science, North Chicago, Illinois; Founder and Director, Center for Medical Dermatology and Immunology Research, Chicago, Illinois
Raj Chovatiya, MD, PhD, MSCI, has disclosed the following relevant financial relationships:
Serve(d) as a director, officer, partner, employee, advisor, consultant, or trustee for: AbbVie; Amgen; Apogee Therapeutics; Arcutis; Argenx; ASLAN Pharmaceuticals; Beiersdorf; Boehringer Ingelheim; Bristol Myers Squibb; Cara Therapeutics; Dermavant; Eli Lilly
Serve(d) as a speaker or a member of a speakers bureau for: AbbVie; Arcutis; Beiersdorf; Boehringer Ingelheim; Bristol Myers Squibb; Dermavant; Eli Lilly and Company; Incyte; LEO Pharma
Alopecia areata (AA) is an autoimmune condition that affects children, adolescents, and adults. Severe AA often causes significant burdens, physical discomfort, and psychological stress. Yet, response to therapy is often unpredictable, running the gamut from being refractory to treatment to spontaneous remission.
Dermatologists Raj Chovatiya from Chicago Medical School and Jason Hawkes from the Pacific Skin Institute discuss how to assess AA severity and appropriate therapies, particularly the evolving landscape of JAK inhibitors for patients with severe AA.
The panelists begin by defining severe AA on the basis of the Severity of Alopecia Tool (SALT) score, which assesses AA severity by percentage of hair loss. A patient who has lost over 50% of scalp hair is considered to have severe AA.
Until recently, traditional therapy for severe AA has relied on injectable and systemic corticosteroids, both of which have drawbacks for patients with severe disease. The emergence of Janus kinase (JAK) inhibitors has offered another option for these patients. The panelists discuss recent clinical trials that have shown promising results from JAK inhibitors for treatment of severe AA.
--
Raj Chovatiya, MD, PhD, MSCI, Clinical Associate Professor, Chicago Medical School, Rosalind Franklin University of Medicine and Science, North Chicago, Illinois; Founder and Director, Center for Medical Dermatology and Immunology Research, Chicago, Illinois
Raj Chovatiya, MD, PhD, MSCI, has disclosed the following relevant financial relationships:
Serve(d) as a director, officer, partner, employee, advisor, consultant, or trustee for: AbbVie; Amgen; Apogee Therapeutics; Arcutis; Argenx; ASLAN Pharmaceuticals; Beiersdorf; Boehringer Ingelheim; Bristol Myers Squibb; Cara Therapeutics; Dermavant; Eli Lilly
Serve(d) as a speaker or a member of a speakers bureau for: AbbVie; Arcutis; Beiersdorf; Boehringer Ingelheim; Bristol Myers Squibb; Dermavant; Eli Lilly and Company; Incyte; LEO Pharma
Depression Diagnosis
Editor's Note: This article was created using several editorial tools, including AI, as part of the process. Human editors reviewed this content before publication.
Editor's Note: This article was created using several editorial tools, including AI, as part of the process. Human editors reviewed this content before publication.
Editor's Note: This article was created using several editorial tools, including AI, as part of the process. Human editors reviewed this content before publication.
Measuring Restrictive Lung Disease Severity Using FEV1 vs TLC
Respiratory diseases have varied clinical presentations and are classified as restrictive, obstructive, mixed, or normal. Restrictive lung diseases have reduced lung volumes, either due to an alteration in lung parenchyma or a disease of the pleura, chest wall, or neuromuscular apparatus. If caused by parenchymal lung disease, restrictive lung disorders are accompanied by reduced gas transfer, which may be portrayed clinically by desaturation after exercise. Based on anatomical structures, the causes of lung volume reduction may be intrinsic or extrinsic. Intrinsic causes correspond to diseases of the lung parenchyma, such as idiopathic fibrotic diseases, connective-tissue diseases, drug-induced lung diseases, and other primary diseases of the lungs. Extrinsic causes refer to disorders outside the lungs or extra-pulmonary diseases such as neuromuscular and nonmuscular diseases of the chest wall.1 For example, obesity and myasthenia gravis can cause restrictive lung diseases, one through mechanical interference of lung expansion and the other through neuromuscular impedance of thoracic cage expansion. All these diseases eventually result in lung restriction, impaired lung function, and respiratory failure. This heterogenicity of disease makes establishing a single severity criterion difficult.
Laboratory testing, imaging studies, and examinations are important for determining the pulmonary disease and its course and progression. The pulmonary function test (PFT), which consists of multiple procedures that are performed depending on the information needed, has been an essential tool in practice for the pulmonologist. The PFT includes spirometry, lung volume measurement, respiratory muscle strength, diffusion capacity, and a broncho-provocation test. Each test has a particular role in assisting the diagnosis and/or follow-up of the patient. Spirometry is frequently used due to its range of dynamic physiological parameters, ease of use, and accessibility. It is used for the diagnosis of pulmonary symptoms, in the assessment of disability, and preoperatory evaluation, including lung resection surgery, assisting in the diagnosis, monitoring, and therapy response of pulmonary diseases.
A systematic approach to PFT interpretation is recommended by several societies, such as the American Thoracic Society (ATS) and the European Respiratory Society (ERS).2 The pulmonary function test results must be reproducible and meet established standards to ensure reliable and consistent clinical outcomes. A restrictive respiratory disease is defined by a decrease in total lung capacity (TLC) (< 5% of predicted value) and a normal forced expiratory volume in 1 second (FEV1)/forced vital capacity (FVC) ratio.2 Although other findings—such as a decrease in vital capacity—should prompt an investigation into whether the patient has a possible restrictive respiratory disease, the sole presence of this parameter is not definitive or diagnostic of a restrictive impairment.2-4 The assessment of severity is typically determined by TLC. Unfortunately, the severity of a restrictive respiratory disease and the degree of patient discomfort do not always correlate when utilizing just TLC. Pulmonary sarcoidosis, for example, is a granulomatous lung disease with a restrictive PFT pattern and a disease burden that may vary over time. Having a more consistent method of grading the severity of the restrictive lung disease may help guide treatment. The modified Medical Research Council (mMRC) scale, a 5-point dyspnea scale, is widely used in assessing the severity of dyspnea in various respiratory conditions, including chronic obstructive pulmonary disease (COPD), where its scores have been associated with patient mortality.1,5 The goal of this study was to document the associations between objective parameters obtained through PFT and other variables, with an established measurement of dyspnea to assess the severity grade of restrictive lung diseases.
Methods
This retrospective record review at the Veterans Affairs Caribbean Healthcare System (VACHS) in San Juan, Puerto Rico, wasconducted using the Veterans Health Information Systems and Technology Architecture to identify patients with a PFT, including spirometry, that indicated a restrictive ventilator pattern based on the current ATS/ERS Task Force on Lung Function Testing.2 Patients were included if they were aged ≥ 21 years, PFT with TLC ≤ 80% predicted, mMRC score documented on PFT, and documented diffusing capacity of the lung for carbon monoxide (DLCO). Patients were excluded if their FEV1/vital capacity (VC) was < 70% predicted using the largest VC, or no mMRC score was available. All patients meeting the inclusion criteria were considered regardless of comorbidities.
The PFT results of all adult patients, including those performed between June 1, 2013, and January 6, 2016, were submitted to spirometry, and lung volume measurements were analyzed. Sociodemographic information was collected, including sex, ethnicity, age, height, weight, and basal metabolic index. Other data found in PFTs, such as smoking status, smoking in packs/year, mMRC score, predicted TLC value, imaging present (chest X-ray, computed tomography), and hospitalizations and exacerbations within 1 year were collected. In addition, we examined the predicted values for FEV1, DLCO, and DLCO/VA (calculated using the Ayer equation), FVC (calculated using the Knudson equation), expiratory reserve volume, inspiratory VC, and slow VC. PaO2, PaCO2, and Alveolar-arterial gradients also were collected.6-9 Information about heart failure status was gathered through medical evaluation of notes and cardiac studies. All categorical variables were correlated with Spearman analysis and quantitative variables with average percentages. P values were calculated with analysis of variance.
Results
Of 6461 VACHS patient records reviewed, 415 met the inclusion criteria. Patients were divided according to their mMRC score: 65 had mMRC score of 0, 87 had an mMRC score of 1, 2 had an mMRC score of 2, 146 had an mMRC of 3, and 115 had an mMRC score of 4. The population was primarily male (98.6%) and of Hispanic ethnicity (96.4%), with a mean age of 72 years (Table 1). Most patients (n = 269, 64.0%) were prior smokers, while 135 patients (32.5%) had never smoked, and 11 (2.7%) were current smokers. At baseline, 169 patients (41.4%) had interstitial lung disease, 39 (9.6%) had chest wall disorders, 29 (7.1%) had occupational exposure, 25 (6.1%) had pneumonitis, and 14 (3.4%) had neuromuscular disorders.
There was a statistically significant relationship between mMRC score and hospitalization and FEV1 but not TLC (Table 2). As mMRC increased, so did hospitalizations: a total of 168 patients (40.5%) were hospitalized; 24 patients (36.9%) had an mMRC score of 0, 30 patients (34.0%) had an mMRC score of 1, 2 patients (100%) had an mMRC score of 2, 54 patients (37.0%) had an mMRC score of 3, and 58 patients (50.0%) had an mMRC score of 4 (P = .04). Mean (SD) TLC values increased as mMRC scores increased. Mean (SD) TLC was 70.5% (33.0) for the entire population; 68.8% (7.2) for patients with an mMRC score of 0, 70.8% (5.8) for patients with an mMRC score of 1, 75.0% (1.4) for patients with an mMRC score of 2, 70.1% (7.2) for patients with an mMRC score of 3, and 71.5% (62.1) for patients with an mMRC score of 4 (P = .10) (Figure 1). There was an associated decrease in mean (SD) FEV1 with mMRC. Mean (SD) FEV1 was 76.2% (18.9) for the entire population; 81.7% (19.3) for patients with an mMRC score of 0, 80.9% (18) for patients with an mMRC score of 1, 93.5% (34.6) for patients with an mMRC score of 2, 76.2% (17.1) for patients with an mMRC score of 3, and 69.2% (19.4) for patients with an mMRC score of 4; (P < .001) (Figure 2).
The correlation between mMRC and FEV1 (r = 0.25, P < .001) was stronger than the correlation between mMRC and TLC (r = 0.15, P < .001). The correlations for DLCO (P < .001), DLCO/VA (P < .001), hemoglobin (P < .02), and PaO2 (P < .001) were all statistically significant (P < .005), but with no strong identifiable trend.
Discussion
The patient population of this study was primarily older males of Hispanic ethnicity with a history of smoking. There was no association between body mass index or smoking status with worsening dyspnea as measured with mMRC scores. We observed no significant correlation between mMRC scores and various factors such as comorbidities including heart conditions, and epidemiological factors like the etiology of lung disease, including both intrinsic and extrinsic causes. This lack of association was anticipated, as restrictive lung diseases in our study predominantly arose from intrinsic pulmonary etiologies, such as interstitial lung disease. A difference between more hospitalizations and worsening dyspnea was identified. There was a slightly higher correlation between FEV1 and mMRC scores when compared with TLC and mMRC scores concerning worsening dyspnea, which could indicate that the use of FEV1 should be preferred over previous recommendations to use TLC.10 Other guidelines have utilized exercise capacity via the 6-minute walk test as a marker of severity with spirometry values and found that DLCO was correlated with severity.11
The latest ERS/ATS guidelines recommend z scores for grading the severity of obstructive lung diseases but do not recommend them for the diagnosis of restrictive lung diseases.12 A z score encompasses diverse variables (eg, age, sex, and ethnicity) to provide more uniform and consistent results. Other studies have been done to relate z scores to other spirometry variables with restrictive lung disease. One such study indicates the potential benefit of using FVC alone to grade restrictive lung diseases.13 There continues to be great diversity in the interpretation of pulmonary function tests, and we believe the information gathered can provide valuable insight for managing patients with restrictive lung diseases.
Limitations
Only 2 patients reported an mMRC score of 2 in our study. This may have affected statistical outcomes. It also may reveal possible deficits in the efficacy of patient education on the mMRC scale. This study was also limited by its small sample size, single center location, and the distribution of patients that reported an mMRC favored either low or high values. The patients in this study, who were all veterans, may not be representative of other patient populations.
Conclusions
There continue to be few factors associated with the physiological severity of the defective oxygen delivery and reported dyspnea of a patient with restrictive lung disease that allows for an accurate, repeatable grading of severity. Using FEV1 instead of TLC to determine the severity of a restrictive lung disease should be reconsidered. We could not find any other strong correlation among other factors studied. Further research should be conducted to continue looking for variables that more accurately depict patient dyspnea in restrictive lung disease.
Acknowledgments
This study is based upon work supported by the Veterans Affairs Caribbean Healthcare System in San Juan, Puerto Rico, and is the result of work supported by Pulmonary & Critical Care Medicine service, with resources and the use of its facilities.
1. Hegewald MJ, Crapo RO. Pulmonary function testing. In: Broaddus VC, Ernst JD, King Jr TE, eds. Murray and Nadel’s Textbook of Respiratory Medicine. 5th ed. Saunders; 2010:522-553.
2. Pellegrino R, Viegi G, Brusasco V, et al. Interpretative strategies for lung function tests. Eur Respir J. 2005;26(5):948-968. doi:10.1183/09031936.05.00035205
3. Rabe KF, Beghé B, Luppi F, Fabbri LM. Update in chronic obstructive pulmonary disease 2006. Am J Respir Crit Care Med. 2007;175(12):1222-1232. doi:10.1164/rccm.200704-586UP
4. Global Initiative for Chronic Obstructive Lung Disease (GOLD). Spirometry for health care providers Accessed April 30, 2024. https://goldcopd.org/wp-content/uploads/2016/04/GOLD_Spirometry_2010.pdf
5. Mannino DM, Holguin F, Pavlin BI, Ferdinands JM. Risk factors for prevalence of and mortality related to restriction on spirometry: findings from the First National Health and Nutrition Examination Survey and follow-up. Int J Tuberc Lung Dis. 2005;9(6):613-621.
6. Knudson RJ, Lebowitz MD, Holberg CJ, Burrows B. Changes in the normal maximal expiratory flow-volume curve with growth and aging. Am Rev Respir Dis. 1983;127(6):725-734. doi:10.1164/arrd.1983.127.6.725
7. Knudson RJ, Burrows B, Lebowitz MD. The maximal expiratory flow-volume curve: its use in the detection of ventilatory abnormalities in a population study. Am Rev Respir Dis. 1976;114(5):871-879. doi:10.1164/arrd.1976.114.5.871
8. Knudson RJ, Lebowitz MD, Burton AP, Knudson DE. The closing volume test: evaluation of nitrogen and bolus methods in a random population. Am Rev Respir Dis. 1977;115(3):423-434. doi:10.1164/arrd.1977.115.3.423
9. Ayers LN, Ginsberg ML, Fein J, Wasserman K. Diffusing capacity, specific diffusing capacity and interpretation of diffusion defects. West J Med. 1975;123(4):255-264.
10. Lung function testing: selection of reference values and interpretative strategies. American Thoracic Society. Am Rev Respir Dis. 1991;144(5):1202-1218. doi:10.1164/ajrccm/144.5.1202
11. Larson J, Wrzos K, Corazalla E, Wang Q, Kim HJ, Cho RJ. Should FEV1 be used to grade restrictive impairment? A single-center comparison of lung function parameters to 6-minute walk test in patients with restrictive lung disease. HSOA J Pulm Med Respir Res. 2023;9:082. doi:10.24966/PMRR-0177/100082
12. Stanojevic S, Kaminsky DA, Miller MR, et al. ERS/ATS technical standard on interpretive strategies for routine lung function tests. Eur Respir J. 2022;60(1):2101499. Published 2022 Jul 13. doi:10.1183/13993003.01499-2021
13. Myrberg T, Lindberg A, Eriksson B, et al. Restrictive spirometry versus restrictive lung function using the GLI reference values. Clin Physiol Funct Imaging. 2022;42(3):181-189. doi:10.1111/cpf.12745
Rebeca Vazquez-Nieves, MDa; Vanessa Fonseca-Ferrer, MDa; Juan Irizarry-Nieves, MDa; Edgardo Adorno-Fontanez, MDa; William Rodriguez-Cintron, MDa,b,c
Correspondence: Juan Irizarry-Nieves ([email protected])
aVeterans Affairs Caribbean Healthcare System, San Juan, Puerto Rico
bUniversity of Puerto Rico School of Medicine, San Juan
cUniversidad Central del Caribe School of Medicine, San Juan, Puerto Rico
Author disclosures
The authors report no actual or potential conflicts of interest or outside sources of funding with regard to this article.
Disclaimer
The opinions expressed herein are those of the authors and do not necessarily reflect those of Federal Practitioner, Frontline Medical Communications Inc., the US Government, or any of its agencies.
Ethics and consent
All documentation was approved by the Veterans Affairs Caribbean Healthcare System institutional review board.Appropriate waivers were obtained and there are no findings of incompliance present.
Rebeca Vazquez-Nieves, MDa; Vanessa Fonseca-Ferrer, MDa; Juan Irizarry-Nieves, MDa; Edgardo Adorno-Fontanez, MDa; William Rodriguez-Cintron, MDa,b,c
Correspondence: Juan Irizarry-Nieves ([email protected])
aVeterans Affairs Caribbean Healthcare System, San Juan, Puerto Rico
bUniversity of Puerto Rico School of Medicine, San Juan
cUniversidad Central del Caribe School of Medicine, San Juan, Puerto Rico
Author disclosures
The authors report no actual or potential conflicts of interest or outside sources of funding with regard to this article.
Disclaimer
The opinions expressed herein are those of the authors and do not necessarily reflect those of Federal Practitioner, Frontline Medical Communications Inc., the US Government, or any of its agencies.
Ethics and consent
All documentation was approved by the Veterans Affairs Caribbean Healthcare System institutional review board.Appropriate waivers were obtained and there are no findings of incompliance present.
Rebeca Vazquez-Nieves, MDa; Vanessa Fonseca-Ferrer, MDa; Juan Irizarry-Nieves, MDa; Edgardo Adorno-Fontanez, MDa; William Rodriguez-Cintron, MDa,b,c
Correspondence: Juan Irizarry-Nieves ([email protected])
aVeterans Affairs Caribbean Healthcare System, San Juan, Puerto Rico
bUniversity of Puerto Rico School of Medicine, San Juan
cUniversidad Central del Caribe School of Medicine, San Juan, Puerto Rico
Author disclosures
The authors report no actual or potential conflicts of interest or outside sources of funding with regard to this article.
Disclaimer
The opinions expressed herein are those of the authors and do not necessarily reflect those of Federal Practitioner, Frontline Medical Communications Inc., the US Government, or any of its agencies.
Ethics and consent
All documentation was approved by the Veterans Affairs Caribbean Healthcare System institutional review board.Appropriate waivers were obtained and there are no findings of incompliance present.
Respiratory diseases have varied clinical presentations and are classified as restrictive, obstructive, mixed, or normal. Restrictive lung diseases have reduced lung volumes, either due to an alteration in lung parenchyma or a disease of the pleura, chest wall, or neuromuscular apparatus. If caused by parenchymal lung disease, restrictive lung disorders are accompanied by reduced gas transfer, which may be portrayed clinically by desaturation after exercise. Based on anatomical structures, the causes of lung volume reduction may be intrinsic or extrinsic. Intrinsic causes correspond to diseases of the lung parenchyma, such as idiopathic fibrotic diseases, connective-tissue diseases, drug-induced lung diseases, and other primary diseases of the lungs. Extrinsic causes refer to disorders outside the lungs or extra-pulmonary diseases such as neuromuscular and nonmuscular diseases of the chest wall.1 For example, obesity and myasthenia gravis can cause restrictive lung diseases, one through mechanical interference of lung expansion and the other through neuromuscular impedance of thoracic cage expansion. All these diseases eventually result in lung restriction, impaired lung function, and respiratory failure. This heterogenicity of disease makes establishing a single severity criterion difficult.
Laboratory testing, imaging studies, and examinations are important for determining the pulmonary disease and its course and progression. The pulmonary function test (PFT), which consists of multiple procedures that are performed depending on the information needed, has been an essential tool in practice for the pulmonologist. The PFT includes spirometry, lung volume measurement, respiratory muscle strength, diffusion capacity, and a broncho-provocation test. Each test has a particular role in assisting the diagnosis and/or follow-up of the patient. Spirometry is frequently used due to its range of dynamic physiological parameters, ease of use, and accessibility. It is used for the diagnosis of pulmonary symptoms, in the assessment of disability, and preoperatory evaluation, including lung resection surgery, assisting in the diagnosis, monitoring, and therapy response of pulmonary diseases.
A systematic approach to PFT interpretation is recommended by several societies, such as the American Thoracic Society (ATS) and the European Respiratory Society (ERS).2 The pulmonary function test results must be reproducible and meet established standards to ensure reliable and consistent clinical outcomes. A restrictive respiratory disease is defined by a decrease in total lung capacity (TLC) (< 5% of predicted value) and a normal forced expiratory volume in 1 second (FEV1)/forced vital capacity (FVC) ratio.2 Although other findings—such as a decrease in vital capacity—should prompt an investigation into whether the patient has a possible restrictive respiratory disease, the sole presence of this parameter is not definitive or diagnostic of a restrictive impairment.2-4 The assessment of severity is typically determined by TLC. Unfortunately, the severity of a restrictive respiratory disease and the degree of patient discomfort do not always correlate when utilizing just TLC. Pulmonary sarcoidosis, for example, is a granulomatous lung disease with a restrictive PFT pattern and a disease burden that may vary over time. Having a more consistent method of grading the severity of the restrictive lung disease may help guide treatment. The modified Medical Research Council (mMRC) scale, a 5-point dyspnea scale, is widely used in assessing the severity of dyspnea in various respiratory conditions, including chronic obstructive pulmonary disease (COPD), where its scores have been associated with patient mortality.1,5 The goal of this study was to document the associations between objective parameters obtained through PFT and other variables, with an established measurement of dyspnea to assess the severity grade of restrictive lung diseases.
Methods
This retrospective record review at the Veterans Affairs Caribbean Healthcare System (VACHS) in San Juan, Puerto Rico, wasconducted using the Veterans Health Information Systems and Technology Architecture to identify patients with a PFT, including spirometry, that indicated a restrictive ventilator pattern based on the current ATS/ERS Task Force on Lung Function Testing.2 Patients were included if they were aged ≥ 21 years, PFT with TLC ≤ 80% predicted, mMRC score documented on PFT, and documented diffusing capacity of the lung for carbon monoxide (DLCO). Patients were excluded if their FEV1/vital capacity (VC) was < 70% predicted using the largest VC, or no mMRC score was available. All patients meeting the inclusion criteria were considered regardless of comorbidities.
The PFT results of all adult patients, including those performed between June 1, 2013, and January 6, 2016, were submitted to spirometry, and lung volume measurements were analyzed. Sociodemographic information was collected, including sex, ethnicity, age, height, weight, and basal metabolic index. Other data found in PFTs, such as smoking status, smoking in packs/year, mMRC score, predicted TLC value, imaging present (chest X-ray, computed tomography), and hospitalizations and exacerbations within 1 year were collected. In addition, we examined the predicted values for FEV1, DLCO, and DLCO/VA (calculated using the Ayer equation), FVC (calculated using the Knudson equation), expiratory reserve volume, inspiratory VC, and slow VC. PaO2, PaCO2, and Alveolar-arterial gradients also were collected.6-9 Information about heart failure status was gathered through medical evaluation of notes and cardiac studies. All categorical variables were correlated with Spearman analysis and quantitative variables with average percentages. P values were calculated with analysis of variance.
Results
Of 6461 VACHS patient records reviewed, 415 met the inclusion criteria. Patients were divided according to their mMRC score: 65 had mMRC score of 0, 87 had an mMRC score of 1, 2 had an mMRC score of 2, 146 had an mMRC of 3, and 115 had an mMRC score of 4. The population was primarily male (98.6%) and of Hispanic ethnicity (96.4%), with a mean age of 72 years (Table 1). Most patients (n = 269, 64.0%) were prior smokers, while 135 patients (32.5%) had never smoked, and 11 (2.7%) were current smokers. At baseline, 169 patients (41.4%) had interstitial lung disease, 39 (9.6%) had chest wall disorders, 29 (7.1%) had occupational exposure, 25 (6.1%) had pneumonitis, and 14 (3.4%) had neuromuscular disorders.
There was a statistically significant relationship between mMRC score and hospitalization and FEV1 but not TLC (Table 2). As mMRC increased, so did hospitalizations: a total of 168 patients (40.5%) were hospitalized; 24 patients (36.9%) had an mMRC score of 0, 30 patients (34.0%) had an mMRC score of 1, 2 patients (100%) had an mMRC score of 2, 54 patients (37.0%) had an mMRC score of 3, and 58 patients (50.0%) had an mMRC score of 4 (P = .04). Mean (SD) TLC values increased as mMRC scores increased. Mean (SD) TLC was 70.5% (33.0) for the entire population; 68.8% (7.2) for patients with an mMRC score of 0, 70.8% (5.8) for patients with an mMRC score of 1, 75.0% (1.4) for patients with an mMRC score of 2, 70.1% (7.2) for patients with an mMRC score of 3, and 71.5% (62.1) for patients with an mMRC score of 4 (P = .10) (Figure 1). There was an associated decrease in mean (SD) FEV1 with mMRC. Mean (SD) FEV1 was 76.2% (18.9) for the entire population; 81.7% (19.3) for patients with an mMRC score of 0, 80.9% (18) for patients with an mMRC score of 1, 93.5% (34.6) for patients with an mMRC score of 2, 76.2% (17.1) for patients with an mMRC score of 3, and 69.2% (19.4) for patients with an mMRC score of 4; (P < .001) (Figure 2).
The correlation between mMRC and FEV1 (r = 0.25, P < .001) was stronger than the correlation between mMRC and TLC (r = 0.15, P < .001). The correlations for DLCO (P < .001), DLCO/VA (P < .001), hemoglobin (P < .02), and PaO2 (P < .001) were all statistically significant (P < .005), but with no strong identifiable trend.
Discussion
The patient population of this study was primarily older males of Hispanic ethnicity with a history of smoking. There was no association between body mass index or smoking status with worsening dyspnea as measured with mMRC scores. We observed no significant correlation between mMRC scores and various factors such as comorbidities including heart conditions, and epidemiological factors like the etiology of lung disease, including both intrinsic and extrinsic causes. This lack of association was anticipated, as restrictive lung diseases in our study predominantly arose from intrinsic pulmonary etiologies, such as interstitial lung disease. A difference between more hospitalizations and worsening dyspnea was identified. There was a slightly higher correlation between FEV1 and mMRC scores when compared with TLC and mMRC scores concerning worsening dyspnea, which could indicate that the use of FEV1 should be preferred over previous recommendations to use TLC.10 Other guidelines have utilized exercise capacity via the 6-minute walk test as a marker of severity with spirometry values and found that DLCO was correlated with severity.11
The latest ERS/ATS guidelines recommend z scores for grading the severity of obstructive lung diseases but do not recommend them for the diagnosis of restrictive lung diseases.12 A z score encompasses diverse variables (eg, age, sex, and ethnicity) to provide more uniform and consistent results. Other studies have been done to relate z scores to other spirometry variables with restrictive lung disease. One such study indicates the potential benefit of using FVC alone to grade restrictive lung diseases.13 There continues to be great diversity in the interpretation of pulmonary function tests, and we believe the information gathered can provide valuable insight for managing patients with restrictive lung diseases.
Limitations
Only 2 patients reported an mMRC score of 2 in our study. This may have affected statistical outcomes. It also may reveal possible deficits in the efficacy of patient education on the mMRC scale. This study was also limited by its small sample size, single center location, and the distribution of patients that reported an mMRC favored either low or high values. The patients in this study, who were all veterans, may not be representative of other patient populations.
Conclusions
There continue to be few factors associated with the physiological severity of the defective oxygen delivery and reported dyspnea of a patient with restrictive lung disease that allows for an accurate, repeatable grading of severity. Using FEV1 instead of TLC to determine the severity of a restrictive lung disease should be reconsidered. We could not find any other strong correlation among other factors studied. Further research should be conducted to continue looking for variables that more accurately depict patient dyspnea in restrictive lung disease.
Acknowledgments
This study is based upon work supported by the Veterans Affairs Caribbean Healthcare System in San Juan, Puerto Rico, and is the result of work supported by Pulmonary & Critical Care Medicine service, with resources and the use of its facilities.
Respiratory diseases have varied clinical presentations and are classified as restrictive, obstructive, mixed, or normal. Restrictive lung diseases have reduced lung volumes, either due to an alteration in lung parenchyma or a disease of the pleura, chest wall, or neuromuscular apparatus. If caused by parenchymal lung disease, restrictive lung disorders are accompanied by reduced gas transfer, which may be portrayed clinically by desaturation after exercise. Based on anatomical structures, the causes of lung volume reduction may be intrinsic or extrinsic. Intrinsic causes correspond to diseases of the lung parenchyma, such as idiopathic fibrotic diseases, connective-tissue diseases, drug-induced lung diseases, and other primary diseases of the lungs. Extrinsic causes refer to disorders outside the lungs or extra-pulmonary diseases such as neuromuscular and nonmuscular diseases of the chest wall.1 For example, obesity and myasthenia gravis can cause restrictive lung diseases, one through mechanical interference of lung expansion and the other through neuromuscular impedance of thoracic cage expansion. All these diseases eventually result in lung restriction, impaired lung function, and respiratory failure. This heterogenicity of disease makes establishing a single severity criterion difficult.
Laboratory testing, imaging studies, and examinations are important for determining the pulmonary disease and its course and progression. The pulmonary function test (PFT), which consists of multiple procedures that are performed depending on the information needed, has been an essential tool in practice for the pulmonologist. The PFT includes spirometry, lung volume measurement, respiratory muscle strength, diffusion capacity, and a broncho-provocation test. Each test has a particular role in assisting the diagnosis and/or follow-up of the patient. Spirometry is frequently used due to its range of dynamic physiological parameters, ease of use, and accessibility. It is used for the diagnosis of pulmonary symptoms, in the assessment of disability, and preoperatory evaluation, including lung resection surgery, assisting in the diagnosis, monitoring, and therapy response of pulmonary diseases.
A systematic approach to PFT interpretation is recommended by several societies, such as the American Thoracic Society (ATS) and the European Respiratory Society (ERS).2 The pulmonary function test results must be reproducible and meet established standards to ensure reliable and consistent clinical outcomes. A restrictive respiratory disease is defined by a decrease in total lung capacity (TLC) (< 5% of predicted value) and a normal forced expiratory volume in 1 second (FEV1)/forced vital capacity (FVC) ratio.2 Although other findings—such as a decrease in vital capacity—should prompt an investigation into whether the patient has a possible restrictive respiratory disease, the sole presence of this parameter is not definitive or diagnostic of a restrictive impairment.2-4 The assessment of severity is typically determined by TLC. Unfortunately, the severity of a restrictive respiratory disease and the degree of patient discomfort do not always correlate when utilizing just TLC. Pulmonary sarcoidosis, for example, is a granulomatous lung disease with a restrictive PFT pattern and a disease burden that may vary over time. Having a more consistent method of grading the severity of the restrictive lung disease may help guide treatment. The modified Medical Research Council (mMRC) scale, a 5-point dyspnea scale, is widely used in assessing the severity of dyspnea in various respiratory conditions, including chronic obstructive pulmonary disease (COPD), where its scores have been associated with patient mortality.1,5 The goal of this study was to document the associations between objective parameters obtained through PFT and other variables, with an established measurement of dyspnea to assess the severity grade of restrictive lung diseases.
Methods
This retrospective record review at the Veterans Affairs Caribbean Healthcare System (VACHS) in San Juan, Puerto Rico, wasconducted using the Veterans Health Information Systems and Technology Architecture to identify patients with a PFT, including spirometry, that indicated a restrictive ventilator pattern based on the current ATS/ERS Task Force on Lung Function Testing.2 Patients were included if they were aged ≥ 21 years, PFT with TLC ≤ 80% predicted, mMRC score documented on PFT, and documented diffusing capacity of the lung for carbon monoxide (DLCO). Patients were excluded if their FEV1/vital capacity (VC) was < 70% predicted using the largest VC, or no mMRC score was available. All patients meeting the inclusion criteria were considered regardless of comorbidities.
The PFT results of all adult patients, including those performed between June 1, 2013, and January 6, 2016, were submitted to spirometry, and lung volume measurements were analyzed. Sociodemographic information was collected, including sex, ethnicity, age, height, weight, and basal metabolic index. Other data found in PFTs, such as smoking status, smoking in packs/year, mMRC score, predicted TLC value, imaging present (chest X-ray, computed tomography), and hospitalizations and exacerbations within 1 year were collected. In addition, we examined the predicted values for FEV1, DLCO, and DLCO/VA (calculated using the Ayer equation), FVC (calculated using the Knudson equation), expiratory reserve volume, inspiratory VC, and slow VC. PaO2, PaCO2, and Alveolar-arterial gradients also were collected.6-9 Information about heart failure status was gathered through medical evaluation of notes and cardiac studies. All categorical variables were correlated with Spearman analysis and quantitative variables with average percentages. P values were calculated with analysis of variance.
Results
Of 6461 VACHS patient records reviewed, 415 met the inclusion criteria. Patients were divided according to their mMRC score: 65 had mMRC score of 0, 87 had an mMRC score of 1, 2 had an mMRC score of 2, 146 had an mMRC of 3, and 115 had an mMRC score of 4. The population was primarily male (98.6%) and of Hispanic ethnicity (96.4%), with a mean age of 72 years (Table 1). Most patients (n = 269, 64.0%) were prior smokers, while 135 patients (32.5%) had never smoked, and 11 (2.7%) were current smokers. At baseline, 169 patients (41.4%) had interstitial lung disease, 39 (9.6%) had chest wall disorders, 29 (7.1%) had occupational exposure, 25 (6.1%) had pneumonitis, and 14 (3.4%) had neuromuscular disorders.
There was a statistically significant relationship between mMRC score and hospitalization and FEV1 but not TLC (Table 2). As mMRC increased, so did hospitalizations: a total of 168 patients (40.5%) were hospitalized; 24 patients (36.9%) had an mMRC score of 0, 30 patients (34.0%) had an mMRC score of 1, 2 patients (100%) had an mMRC score of 2, 54 patients (37.0%) had an mMRC score of 3, and 58 patients (50.0%) had an mMRC score of 4 (P = .04). Mean (SD) TLC values increased as mMRC scores increased. Mean (SD) TLC was 70.5% (33.0) for the entire population; 68.8% (7.2) for patients with an mMRC score of 0, 70.8% (5.8) for patients with an mMRC score of 1, 75.0% (1.4) for patients with an mMRC score of 2, 70.1% (7.2) for patients with an mMRC score of 3, and 71.5% (62.1) for patients with an mMRC score of 4 (P = .10) (Figure 1). There was an associated decrease in mean (SD) FEV1 with mMRC. Mean (SD) FEV1 was 76.2% (18.9) for the entire population; 81.7% (19.3) for patients with an mMRC score of 0, 80.9% (18) for patients with an mMRC score of 1, 93.5% (34.6) for patients with an mMRC score of 2, 76.2% (17.1) for patients with an mMRC score of 3, and 69.2% (19.4) for patients with an mMRC score of 4; (P < .001) (Figure 2).
The correlation between mMRC and FEV1 (r = 0.25, P < .001) was stronger than the correlation between mMRC and TLC (r = 0.15, P < .001). The correlations for DLCO (P < .001), DLCO/VA (P < .001), hemoglobin (P < .02), and PaO2 (P < .001) were all statistically significant (P < .005), but with no strong identifiable trend.
Discussion
The patient population of this study was primarily older males of Hispanic ethnicity with a history of smoking. There was no association between body mass index or smoking status with worsening dyspnea as measured with mMRC scores. We observed no significant correlation between mMRC scores and various factors such as comorbidities including heart conditions, and epidemiological factors like the etiology of lung disease, including both intrinsic and extrinsic causes. This lack of association was anticipated, as restrictive lung diseases in our study predominantly arose from intrinsic pulmonary etiologies, such as interstitial lung disease. A difference between more hospitalizations and worsening dyspnea was identified. There was a slightly higher correlation between FEV1 and mMRC scores when compared with TLC and mMRC scores concerning worsening dyspnea, which could indicate that the use of FEV1 should be preferred over previous recommendations to use TLC.10 Other guidelines have utilized exercise capacity via the 6-minute walk test as a marker of severity with spirometry values and found that DLCO was correlated with severity.11
The latest ERS/ATS guidelines recommend z scores for grading the severity of obstructive lung diseases but do not recommend them for the diagnosis of restrictive lung diseases.12 A z score encompasses diverse variables (eg, age, sex, and ethnicity) to provide more uniform and consistent results. Other studies have been done to relate z scores to other spirometry variables with restrictive lung disease. One such study indicates the potential benefit of using FVC alone to grade restrictive lung diseases.13 There continues to be great diversity in the interpretation of pulmonary function tests, and we believe the information gathered can provide valuable insight for managing patients with restrictive lung diseases.
Limitations
Only 2 patients reported an mMRC score of 2 in our study. This may have affected statistical outcomes. It also may reveal possible deficits in the efficacy of patient education on the mMRC scale. This study was also limited by its small sample size, single center location, and the distribution of patients that reported an mMRC favored either low or high values. The patients in this study, who were all veterans, may not be representative of other patient populations.
Conclusions
There continue to be few factors associated with the physiological severity of the defective oxygen delivery and reported dyspnea of a patient with restrictive lung disease that allows for an accurate, repeatable grading of severity. Using FEV1 instead of TLC to determine the severity of a restrictive lung disease should be reconsidered. We could not find any other strong correlation among other factors studied. Further research should be conducted to continue looking for variables that more accurately depict patient dyspnea in restrictive lung disease.
Acknowledgments
This study is based upon work supported by the Veterans Affairs Caribbean Healthcare System in San Juan, Puerto Rico, and is the result of work supported by Pulmonary & Critical Care Medicine service, with resources and the use of its facilities.
1. Hegewald MJ, Crapo RO. Pulmonary function testing. In: Broaddus VC, Ernst JD, King Jr TE, eds. Murray and Nadel’s Textbook of Respiratory Medicine. 5th ed. Saunders; 2010:522-553.
2. Pellegrino R, Viegi G, Brusasco V, et al. Interpretative strategies for lung function tests. Eur Respir J. 2005;26(5):948-968. doi:10.1183/09031936.05.00035205
3. Rabe KF, Beghé B, Luppi F, Fabbri LM. Update in chronic obstructive pulmonary disease 2006. Am J Respir Crit Care Med. 2007;175(12):1222-1232. doi:10.1164/rccm.200704-586UP
4. Global Initiative for Chronic Obstructive Lung Disease (GOLD). Spirometry for health care providers Accessed April 30, 2024. https://goldcopd.org/wp-content/uploads/2016/04/GOLD_Spirometry_2010.pdf
5. Mannino DM, Holguin F, Pavlin BI, Ferdinands JM. Risk factors for prevalence of and mortality related to restriction on spirometry: findings from the First National Health and Nutrition Examination Survey and follow-up. Int J Tuberc Lung Dis. 2005;9(6):613-621.
6. Knudson RJ, Lebowitz MD, Holberg CJ, Burrows B. Changes in the normal maximal expiratory flow-volume curve with growth and aging. Am Rev Respir Dis. 1983;127(6):725-734. doi:10.1164/arrd.1983.127.6.725
7. Knudson RJ, Burrows B, Lebowitz MD. The maximal expiratory flow-volume curve: its use in the detection of ventilatory abnormalities in a population study. Am Rev Respir Dis. 1976;114(5):871-879. doi:10.1164/arrd.1976.114.5.871
8. Knudson RJ, Lebowitz MD, Burton AP, Knudson DE. The closing volume test: evaluation of nitrogen and bolus methods in a random population. Am Rev Respir Dis. 1977;115(3):423-434. doi:10.1164/arrd.1977.115.3.423
9. Ayers LN, Ginsberg ML, Fein J, Wasserman K. Diffusing capacity, specific diffusing capacity and interpretation of diffusion defects. West J Med. 1975;123(4):255-264.
10. Lung function testing: selection of reference values and interpretative strategies. American Thoracic Society. Am Rev Respir Dis. 1991;144(5):1202-1218. doi:10.1164/ajrccm/144.5.1202
11. Larson J, Wrzos K, Corazalla E, Wang Q, Kim HJ, Cho RJ. Should FEV1 be used to grade restrictive impairment? A single-center comparison of lung function parameters to 6-minute walk test in patients with restrictive lung disease. HSOA J Pulm Med Respir Res. 2023;9:082. doi:10.24966/PMRR-0177/100082
12. Stanojevic S, Kaminsky DA, Miller MR, et al. ERS/ATS technical standard on interpretive strategies for routine lung function tests. Eur Respir J. 2022;60(1):2101499. Published 2022 Jul 13. doi:10.1183/13993003.01499-2021
13. Myrberg T, Lindberg A, Eriksson B, et al. Restrictive spirometry versus restrictive lung function using the GLI reference values. Clin Physiol Funct Imaging. 2022;42(3):181-189. doi:10.1111/cpf.12745
1. Hegewald MJ, Crapo RO. Pulmonary function testing. In: Broaddus VC, Ernst JD, King Jr TE, eds. Murray and Nadel’s Textbook of Respiratory Medicine. 5th ed. Saunders; 2010:522-553.
2. Pellegrino R, Viegi G, Brusasco V, et al. Interpretative strategies for lung function tests. Eur Respir J. 2005;26(5):948-968. doi:10.1183/09031936.05.00035205
3. Rabe KF, Beghé B, Luppi F, Fabbri LM. Update in chronic obstructive pulmonary disease 2006. Am J Respir Crit Care Med. 2007;175(12):1222-1232. doi:10.1164/rccm.200704-586UP
4. Global Initiative for Chronic Obstructive Lung Disease (GOLD). Spirometry for health care providers Accessed April 30, 2024. https://goldcopd.org/wp-content/uploads/2016/04/GOLD_Spirometry_2010.pdf
5. Mannino DM, Holguin F, Pavlin BI, Ferdinands JM. Risk factors for prevalence of and mortality related to restriction on spirometry: findings from the First National Health and Nutrition Examination Survey and follow-up. Int J Tuberc Lung Dis. 2005;9(6):613-621.
6. Knudson RJ, Lebowitz MD, Holberg CJ, Burrows B. Changes in the normal maximal expiratory flow-volume curve with growth and aging. Am Rev Respir Dis. 1983;127(6):725-734. doi:10.1164/arrd.1983.127.6.725
7. Knudson RJ, Burrows B, Lebowitz MD. The maximal expiratory flow-volume curve: its use in the detection of ventilatory abnormalities in a population study. Am Rev Respir Dis. 1976;114(5):871-879. doi:10.1164/arrd.1976.114.5.871
8. Knudson RJ, Lebowitz MD, Burton AP, Knudson DE. The closing volume test: evaluation of nitrogen and bolus methods in a random population. Am Rev Respir Dis. 1977;115(3):423-434. doi:10.1164/arrd.1977.115.3.423
9. Ayers LN, Ginsberg ML, Fein J, Wasserman K. Diffusing capacity, specific diffusing capacity and interpretation of diffusion defects. West J Med. 1975;123(4):255-264.
10. Lung function testing: selection of reference values and interpretative strategies. American Thoracic Society. Am Rev Respir Dis. 1991;144(5):1202-1218. doi:10.1164/ajrccm/144.5.1202
11. Larson J, Wrzos K, Corazalla E, Wang Q, Kim HJ, Cho RJ. Should FEV1 be used to grade restrictive impairment? A single-center comparison of lung function parameters to 6-minute walk test in patients with restrictive lung disease. HSOA J Pulm Med Respir Res. 2023;9:082. doi:10.24966/PMRR-0177/100082
12. Stanojevic S, Kaminsky DA, Miller MR, et al. ERS/ATS technical standard on interpretive strategies for routine lung function tests. Eur Respir J. 2022;60(1):2101499. Published 2022 Jul 13. doi:10.1183/13993003.01499-2021
13. Myrberg T, Lindberg A, Eriksson B, et al. Restrictive spirometry versus restrictive lung function using the GLI reference values. Clin Physiol Funct Imaging. 2022;42(3):181-189. doi:10.1111/cpf.12745
The Role of High Reliability Organization Foundational Practices in Building a Culture of Safety
Increasing complexities within health care systems are significant impediments to the consistent delivery of safe and effective patient care. These impediments include an increase in specialization of care, staff shortages, burnout, poor coordination of services and access to care, as well as rising costs.1 High reliability organizations (HROs) provide safe, high-quality, and effective care in highly complex and risk-prone environments without causing harm or experiencing catastrophic events.2
Within the US Department of Veterans Affairs (VA), the Veterans Health Administration (VHA) operates the nation’s largest integrated health care system, providing care to > 9 million veterans. The VHA formally launched plans for an enterprise-wide HRO in February 2019. During the first year, 18 medical facilities comprised cohort1 of the journey to high reliability. Cohort 2 began in October 2020 and consisted of 54 facilities. Cohort 3 started in October 2021 with 67 facilities.3
Health care organizations seeking high reliability exercise a philosophy aimed at learning from errors and addressing system failures. High reliability is accomplished by implementing 5 principles: (1) sensitivity to operations (a heightened understanding of the current state of systems); (2) preoccupation with failure (striving to anticipate risks that might suggest a much larger system problem); (3) reluctance to simplify (avoiding making any assumptions regarding the causes of failures); (4) commitment to resilience (preparing for potential failures and bouncing back when they occur); and (5) deference to expertise (deferring to individuals with the skills and proficiency to make the best decisions).2 The VHA also recognized that a successful journey to high reliability—in addition to achieving a culture of safety—relies on the implementation of foundational HRO practices: leader rounding, visual management systems, safety forums, and safety huddles. This article describes an initiative for how these foundational practices were implemented in a large integrated health care system.
BACKGROUND
The VHA has focused on 4 foundational components as part of its enterprise activities and support structure to implement HRO principles and practices. These components were selected based on pilot activities that preceded the enterprise-wide effort, reviews of the literature, and expert consultation with both government and private sector health systems. To support the implementation of these practices, the VHA provided training, toolkits, HRO executive leader coaching, and peer-to-peer mentoring. As the VHA enters its fifth year seeking high reliability, we undertook an initiative to reflect on our own experiences and refine our practices based on an updated literature review.
As part of this enterprise-wide initiative, we conducted a literature review from 2018 to March 2023 seeking recent evidence describing the value of implementing the 4 foundational HRO practices to advance high reliability and improve patient safety. A 5-year period was used to ensure recency and value of evidence.
Eligible literature was identified in PubMed, PsycINFO, the Cumulative Index to Nursing and Allied Health Literature, ScienceDirect, Scopus, the Cochrane Library, and ProQuest Dissertations & Theses Global. Inclusion and exclusion criteria were peer-reviewed interdisciplinary documents(eg, publications, dissertations, conference proceedings, and grey literature) written in English. Search terms included high reliability organizations, foundational practices, and patient safety. Boolean operators (AND, OR) were also used in the search. The search resulted in a dearth of evidence that addressed implementation of all 4 foundational practices across a health care system. Retrieved evidence focused on the implementation of only 1 particular foundational practice in a specific health care setting. In addition to describing the formal processes for the implementation of each foundational HRO practice, a brief description of representative examples of strong practices within the VHA is provided.
To support the implementation of HROs, the VHA paired HRO executive leader coaches with select medical center directors and their leadership teams. Executive leader coaches also support an organization’s HRO Lead and HRO Champion. The HRO Lead coordinates and facilitates the implementation of HRO principles and practices in pursuit of no harm across an organization. The HRO Champion supports the same as the HRO Lead, but typically has a different specialty background. For example, if the HRO Lead has an administrative background, the HRO Champion would have a clinical background.
Coaching focuses heavily on supporting site-specific implementation and sustainment of the 4 HRO foundational practices. The aim is to accelerate change, build enduring capacity, foster a safety culture, and accelerate HRO maturity. To measure change, HRO executive leader coaches track the progress of their aligned VA medical centers (VAMCs) using the Organizational Learning Tool (OLT). This tool was developed to provide information such as a facility summary and relationships between a medical center director, HRO Lead, HRO Champion, and the executive leader coach (Figure 1). The OLT also serves as a structured process to measure leader coaching performance against mutually agreed upon objectives that ultimately contribute to enterprise outcomes. It also collects data on the progress in implementing foundational practices, strong practices, needs and gaps, and more (Figure 2). Data collected from facilities supported by HRO executive leader coaches on whether foundational practices are in place are briefly described.
Leader Rounding
Leader rounding for high reliability ensures effective, bidirectional communication and collaboration among all disciplines to improve patient safety. It is an essential feature of a robust patient safety culture and an important method for demonstrating leadership engagement with high reliability.4,5 These rounds are conducted by organizational leadership (eg, executive teams, department/service chiefs, or unit managers) and frontline staff from different areas. They are specifically focused on high reliability, patient and staff safety, and improvement efforts. The aim is to learn about daily challenges that may contribute to patient harm.4
Leader rounding has been found to be highly effective at improving leadership visibility across the organization. It enhances interaction and open communication with frontline staff, fostering leader-staff collaboration and shared decision-making,as well as promoting leadership understanding of operational, clinical, nonclinical (eg, administrative, nutrition services, or facilities management), and patient/family experience issues.4 Collaboration among team members fosters the delivery of more effective and efficient care, increases staff satisfaction, and improves employee retention.6 Leader rounding for high reliability significantly contributes to the breakdown of power barriers by giving team members voice and agency, ultimately leading to deeper engagement.7
It is important that leader rounding for high reliability occurs as planned and when possible, scheduled in advance. This helps to avoid rounding at peak times when care activities are being performed.4,6 When scheduling conflicts arise, another leader should be sent to participate in rounds.4 Developing a list of questions in advance allows leadership to prepare messaging to share with staff as it relates to high reliability and patient safety (Table).4,6,8
Closing the loop improves bidirectional communication and is critical to leader rounding for high reliability. Closed-loop communication and following up on and/or closing out issues raised during rounding empowers the sharing of information, which is critical for advancing a culture of safety.4,8 Enhanced feedback is also associated with greater workforce engagement, staff feeling more connected to quality improvement activities, and lower rates of employee burnout.7 It is important to recognize that senior leaders are not responsible for resolving all issues. If a team or manager can resolve concerns that are raised, this should be encouraged and supported. Maintaining accountability at the lowest level of the organization promotes principles and practices of high reliability (Figure 3).4,8
The VA Bedford Healthcare System created and implemented a strong practice for leader rounding for high reliability. This phased implementation involved creating an evidence-based process, deciding on an appropriate cadence, developing a tracking tool, and measuring impact to determine the overall effectiveness of leader rounding for high reliability.4
Visual Management Systems
A visual management system (VMS) displays clinical and operational performance aligned with HRO goals and practices. It is used to view and guide discussions between interdisciplinary teams during tiered safety huddles, leader rounds for high reliability, and frontline staff on the current status and safety trends in a particular area.8,9 A VMS is highly effective in creating an environment where all staff members, especially frontline workers, feel empowered to voice their concerns related to safety or to identify improvement opportunities.8,10 Increased leader engagement in patient safety and heightened transparency of information associated with the use of a VMS improves staff morale and professional satisfaction.10
A VMS may be a dry-erase or whiteboard display, paper-based display, or electronic status board.8 VMSs are usually located in or near work settings (eg, nurses’ station, staff break room, or conference room).8 Although they can take different forms and display several types of information, a VMS should be easy to update and meet the specific needs of a work area. In the VHA, a VMS displays: (1) essential information for staff members to effectively perform their work; (2) improvement project ideas; (3) current work in progress; (4) tracking of implemented improvement activities; (5) strong practices that have been effective; and (6) staff recognition for those who have enhanced patient safety, including the reporting of close calls and near misses.
The VHA uses the MESS (methods, equipment, staffing, and supplies) VMS format. This format empowers staff to identify whether proper procedures and practices are in place, essential equipment and supplies are readily available in the quantity needed, and appropriate staffing is on hand to provide safe, high-quality patient care.8 Colored magnets are used as visual cues in a stoplight classification system to identify low or no safety risks (green), at risk (yellow), or high risk (red). Green coded issues are addressed locally by a manager or supervisor. Yellow coded concerns require increased staff and leadership vigilance. Red coded issues indicate that patient care would be impacted that day and therefore need to be immediately escalated and addressed with senior leaders to mitigate the threat.4,11 Dayton VAMC successfully implemented a VMS, using both physical and electronic visual management boards. The Dayton VAMC VMS boards are closely tied to tiered safety huddles and leader rounding for high reliability.
Safety Forums
Safety forums are another foundational practice of VHA health care organizations seeking high reliability. Recurring monthly, safety forums focus on reinforcing HRO principles and practices, safety programs, the importance and appreciation of reporting, and just culture. The emphasis on just culture reminds staff that adverse events in the organization are viewed as valuable learning opportunities to understand the factors leading to the situation as opposed to immediately assigning blame.12
Psychological safety is another important focus. When individuals feel psychologically safe, they are more likely to voice concerns and act without fear of reprisal, which supports a culture of safety.13 Safety forums are open to all members of the health care organization, including both clinical and nonclinical staff. Forums can be conducted by an HRO Lead, HRO Champion, Patient Safety Manager, or even executive leadership. Rotating the responsibility of leading these forums demonstrates that high reliability and safety are everyone’s responsibility.
Safety forums publicly review and discuss errors, adverse events, close calls, and near misses. Time is also spent discussing root cause analysis trends and highlighting continuous process improvement principles and current projects. During safety forums, leaders should recognize individuals for safety behaviors and reward reporting through a safety awards program.14 All forums should conclude with a question-and-answer session. Forums typically occur in virtual 30-minute sessionsbut can last up to 60 minutes when guest speakers attend and continuing education credit is offered.
The Jesse Brown VAMC in Chicago developed an interactive monthly safety forum appealing to a broad audience. Each forum is attended by about 200 staff members and includes leader engagement and panel discussions led by the chief medical officer, with topics on both patient and team safety connecting with HRO principles. A planning committee prepares guest speakers and offers continuing education credits.
Tiered Safety Huddles
Based on the processes of high reliability industries like aviation and nuclear power, tiered safety huddles have been increasingly adopted in health care. Huddles (health care, utilizing, deliberate, discussion,linking, and events) are department-level interdisciplinary meetings that last no more than 15 minutes.15 Their purpose is to improve communication by sharing day-to-day information across multiple disciplines, identify issues that may impact the delivery of care (eg, patient and staff safety concerns, staffing issues, or inadequate supplies) and resolve problems.
Tiered safety huddles are gaining popularity, especially in organizations seeking high reliability. They are more complex than traditional huddles because of the mechanics of elevating safety issues (eg, bedside to executive leadership teams), feedback loops, and sequencing, among other factors.15,16
Tiered safety huddles are focused, transparent forums with multidisciplinary staff, including frontline workers, along with senior leadership.15,16 When initially implemented, tiered safety huddles may take longer than the suggested 15 minutes; however, as teams become more experienced, huddles become more efficient.15 The goal of tiered safety huddles is to proactively identify, share, address, and resolve problems that have the potential to impact the delivery of safe and quality patient care. This may include addressing staffing shortfalls, inadequate allocation of supplies and equipment, operational issues, etc.8,15 Critical to theeffective utilization of tiered safety huddles is the appropriate escalation of issues between tiers. The most critical issues are elevated to higher tiers so they are addressed by the most qualified person in the organization.
Deciding on the number of tiers typically depends on the size and scope of services provided by the health care organization or integrated system.For example, tiered huddles in the VHA originate at the point of service (eg, critical care unit). Tier 1 includes staff members at the unit/team level along with immediate supervisors/managers. Tier 2 involves departments and service lines (eg, pharmacy, podiatry, or internal medicine) including their respective leadership. Tier 3 is the executive leadership team. This process allows for bidirectional communication instead of the traditional hierarchical communication pathway (Figure 4). Issues identified that cannot be addressed at a particular tier are elevated to the next tier. Elevated issues typically involve systems or processes requiring attention and resolution by senior leadership.15 Tier 4 huddles at the Veterans Integrated Services Network level and Tier 5 huddles at the VHA Central Office level are being initiated. These additional levels will more effectively identify system-level risks and issues that may impact multiple VHA facilities and may be addressed through centralized functions and resources.
Tiered safety huddles have been found to be instrumental to ensuring the flow of information across organizations, improving multidisciplinary and leadership engagement and collaboration, as well as increasing accountability for safety.Tiered safety huddles increase situational awareness, which improves an organization’s ability to appropriately respond to safety concerns.Furthermore, tiered safety huddles enhance teamwork and interprofessional collaboration, and have been found to significantly increase the reporting of patient safety events.15-19
The VA Connecticut Healthcare System tiered huddles followed a pilot testing implementation process. After receiving executive-level commitment, an evidence-based process was enacted, including staff education, selecting a VMS, determining tier interaction, and deciding on metrics to track.15
Implementing Foundational Practices
To examine the progress of the implementation of the 4 foundational HRO practices, quarterly metrics derived from the OLT are reviewed to determine whether each is being implemented and sustained. The OLT also tracks progress over time. For example, at the 27 cohort 2 and lead sites that initiated leader coaching in 2021 and continued through 2022, coaches observed a 27% increase in leader rounding for high reliability and a 46% increase in the use of VMSs. For the 66 cohort 3 sites that began leader coaching in 2022, coaches documented similar changes, ranging from a 40% increase in leader rounding for high reliability to a 66% increase in the use of safety forums. Additional data continue to be collected and analyzed to publish more comprehensive findings.
DISCUSSION
Incorporating leader rounding for high reliability, VMSs, safety forums, and tiered safety huddles into daily operations is critical to building and sustaining a robust culture of safety.8 The 4 foundational HRO practices are instrumental in providing psychologically safe forums for staff to share concerns and actively participate. These practices also promote continual, efficient bidirectional communication throughout organizational lines and across services. The increased visibility and transparency of leaders demonstrate the importance of fostering trust, enhancing closed-loop communication with issues that arise, and building momentum to achieve high reliability. The interconnectedness of the foundational HRO practices identified and implemented by the VHA helps foster teamwork and collaboration built on trust, respect, enthusiasm for improvement, and the delivery of exceptional patient care.
CONCLUSIONS
Incorporating the 4 foundational practices into daily operations is beneficial to the delivery of safe, high-quality health care. This effective and sustained application can strengthen a health care organization on its journey to high reliability and establishing a culture of safety. To be effective, these foundational practices should be personalized to support the unique circumstances of every health care environment. While the exact methodology by which organizations implement these practices may differ, they will help organizations approach patient safety in a more transparent and thoughtful manner.
Acknowledgments
The authors thank Aaron M. Sawyer, PhD, PMP, and Jessica Fankhauser, MA, for their unwavering administrative support, and Jeff Wright for exceptional graphic design support.
1. Figueroa CA, Harrison R, Chauhan A, Meyer L. Priorities and challenges for health leadership and workforce management globally: a rapid review. BMC Health Serv Res. 2019;19(1):239. Published 2019 Apr 24. doi:10.1186/s12913-019-4080-7
2. What is a high reliability organization (HRO) in healthcare? Vizient. Accessed May 22, 2024. https://www.vizientinc.com/our-solutions/care-delivery-excellence/reliable-care-delivery
3. US Department of Veterans Affairs, VHA National Center for Patient Safety. VHA’s HRO journey officially begins. March 29, 2019. Accessed May 22, 2024. https://www.patientsafety.va.gov/features/VHA_s_HRO_journey_officially_begins.asp
4. Murray JS, Clifford J, Scott D, Kelly S, Hanover C. Leader rounding for high reliability and improved patient safety. Fed Pract. 2024;41(1):16-21. doi:10.12788/fp.0444
5. Ryan L, Jackson D, Woods C, Usher K. Intentional rounding – an integrative literature review. J Adv Nurs. 2019;75(6):1151-1161. doi:10.1111/jan.13897
6. Hedenstrom M, Harrilson A, Heath M, Dyess S. “What’s old is new again”: innovative health care leader rounding—a strategy to foster connection. Nurse
7. Blake PG, Bacon CT. Structured rounding to improve staff nurse satisfaction with leadership. Nurse Lead. 2020;18(5):461-466. doi:10.1016/j.mnl.2020.04.009
8. US Department of Veterans Affairs, Veterans Health Administration. Leader’s guide to foundational high reliability organization (HRO) practices. https://dvagov.sharepoint.com/sites/OHT-PMO/high-reliability/Pages/default.aspx
9. Goyal A, Glanzman H, Quinn M, et al. Do bedside whiteboards enhance communication in hospitals? An exploratory multimethod study of patient and nurse perspectives. BMJ Qual Saf. 2020;29(10):1-2. doi:10.1136/bmjqs-2019-01020810. Williamsson A, Dellve L, Karltun A. Nurses’ use of visual management in hospitals-a longitudinal, quantitative study on its implications on systems performance and working conditions. J Adv Nurs. 2019;75(4):760-771. doi:10.1111/jan.13855
11. Prineas S, Culwick M, Endlich Y. A proposed system for standardization of colour-coding stages of escalating criticality in clinical incidents. Curr Opin Anaesthesiol. 2021;34(6):752-760. doi:10.1097/ACO.0000000000001071
12. Murray JS, Clifford J, Larson S, Lee JK, Sculli GL. Implementing just culture to improve patient safety. Mil Med. 2023;188(7-8):1596-1599. doi:10.1093/milmed/usac115
13. Murray JS, Kelly S, Hanover C. Promoting psychological safety in healthcare organizations. Mil Med. 2022;187(7-8):808-810. doi:10.1093/milmed/usac041
14. Merchant NB, O’Neal J, Murray JS. Development of a safety awards program at a veterans affairs health care system: a quality improvement initiative. J Clin Outcomes Manag. 2023;30(1):9-16. doi:10.12788/jcom.0120
15. Merchant NB, O’Neal J, Montoya A, Cox GR, Murray JS. Creating a process for the implementation of tiered huddles in a veterans affairs medical center. Mil Med. 2023;188(5-6):901-906. doi:10.1093/milmed/usac073
16. Mihaljevic T. Tiered daily huddles: the power of teamwork in managing large healthcare organisations. BMJ Qual Saf. 2020;29(12):1050-1052. doi:10.1136/bmjqs-2019-010575
17. Franklin BJ, Gandhi TK, Bates DW, et al. Impact of multidisciplinary team huddles on patient safety: a systematic review and proposed taxonomy. BMJ Qual Saf. 2020;29(10):1-2. doi:10.1136/bmjqs-2019-009911
18. Pimentel CB, Snow AL, Carnes SL, et al. Huddles and their effectiveness at the frontlines of clinical care: a scoping review. J Gen Intern Med. 2021;36(9):2772-2783. doi:10.1007/s11606-021-06632-9
19. Adapa K, Ivester T, Shea C, et al. The effect of a system-level tiered huddle system on reporting patient safety events: an interrupted time series analysis. Jt Comm J Qual Patient Saf. 2022;48(12):642-652. doi:10.1016/j.jcjq.2022.08.005
Increasing complexities within health care systems are significant impediments to the consistent delivery of safe and effective patient care. These impediments include an increase in specialization of care, staff shortages, burnout, poor coordination of services and access to care, as well as rising costs.1 High reliability organizations (HROs) provide safe, high-quality, and effective care in highly complex and risk-prone environments without causing harm or experiencing catastrophic events.2
Within the US Department of Veterans Affairs (VA), the Veterans Health Administration (VHA) operates the nation’s largest integrated health care system, providing care to > 9 million veterans. The VHA formally launched plans for an enterprise-wide HRO in February 2019. During the first year, 18 medical facilities comprised cohort1 of the journey to high reliability. Cohort 2 began in October 2020 and consisted of 54 facilities. Cohort 3 started in October 2021 with 67 facilities.3
Health care organizations seeking high reliability exercise a philosophy aimed at learning from errors and addressing system failures. High reliability is accomplished by implementing 5 principles: (1) sensitivity to operations (a heightened understanding of the current state of systems); (2) preoccupation with failure (striving to anticipate risks that might suggest a much larger system problem); (3) reluctance to simplify (avoiding making any assumptions regarding the causes of failures); (4) commitment to resilience (preparing for potential failures and bouncing back when they occur); and (5) deference to expertise (deferring to individuals with the skills and proficiency to make the best decisions).2 The VHA also recognized that a successful journey to high reliability—in addition to achieving a culture of safety—relies on the implementation of foundational HRO practices: leader rounding, visual management systems, safety forums, and safety huddles. This article describes an initiative for how these foundational practices were implemented in a large integrated health care system.
BACKGROUND
The VHA has focused on 4 foundational components as part of its enterprise activities and support structure to implement HRO principles and practices. These components were selected based on pilot activities that preceded the enterprise-wide effort, reviews of the literature, and expert consultation with both government and private sector health systems. To support the implementation of these practices, the VHA provided training, toolkits, HRO executive leader coaching, and peer-to-peer mentoring. As the VHA enters its fifth year seeking high reliability, we undertook an initiative to reflect on our own experiences and refine our practices based on an updated literature review.
As part of this enterprise-wide initiative, we conducted a literature review from 2018 to March 2023 seeking recent evidence describing the value of implementing the 4 foundational HRO practices to advance high reliability and improve patient safety. A 5-year period was used to ensure recency and value of evidence.
Eligible literature was identified in PubMed, PsycINFO, the Cumulative Index to Nursing and Allied Health Literature, ScienceDirect, Scopus, the Cochrane Library, and ProQuest Dissertations & Theses Global. Inclusion and exclusion criteria were peer-reviewed interdisciplinary documents(eg, publications, dissertations, conference proceedings, and grey literature) written in English. Search terms included high reliability organizations, foundational practices, and patient safety. Boolean operators (AND, OR) were also used in the search. The search resulted in a dearth of evidence that addressed implementation of all 4 foundational practices across a health care system. Retrieved evidence focused on the implementation of only 1 particular foundational practice in a specific health care setting. In addition to describing the formal processes for the implementation of each foundational HRO practice, a brief description of representative examples of strong practices within the VHA is provided.
To support the implementation of HROs, the VHA paired HRO executive leader coaches with select medical center directors and their leadership teams. Executive leader coaches also support an organization’s HRO Lead and HRO Champion. The HRO Lead coordinates and facilitates the implementation of HRO principles and practices in pursuit of no harm across an organization. The HRO Champion supports the same as the HRO Lead, but typically has a different specialty background. For example, if the HRO Lead has an administrative background, the HRO Champion would have a clinical background.
Coaching focuses heavily on supporting site-specific implementation and sustainment of the 4 HRO foundational practices. The aim is to accelerate change, build enduring capacity, foster a safety culture, and accelerate HRO maturity. To measure change, HRO executive leader coaches track the progress of their aligned VA medical centers (VAMCs) using the Organizational Learning Tool (OLT). This tool was developed to provide information such as a facility summary and relationships between a medical center director, HRO Lead, HRO Champion, and the executive leader coach (Figure 1). The OLT also serves as a structured process to measure leader coaching performance against mutually agreed upon objectives that ultimately contribute to enterprise outcomes. It also collects data on the progress in implementing foundational practices, strong practices, needs and gaps, and more (Figure 2). Data collected from facilities supported by HRO executive leader coaches on whether foundational practices are in place are briefly described.
Leader Rounding
Leader rounding for high reliability ensures effective, bidirectional communication and collaboration among all disciplines to improve patient safety. It is an essential feature of a robust patient safety culture and an important method for demonstrating leadership engagement with high reliability.4,5 These rounds are conducted by organizational leadership (eg, executive teams, department/service chiefs, or unit managers) and frontline staff from different areas. They are specifically focused on high reliability, patient and staff safety, and improvement efforts. The aim is to learn about daily challenges that may contribute to patient harm.4
Leader rounding has been found to be highly effective at improving leadership visibility across the organization. It enhances interaction and open communication with frontline staff, fostering leader-staff collaboration and shared decision-making,as well as promoting leadership understanding of operational, clinical, nonclinical (eg, administrative, nutrition services, or facilities management), and patient/family experience issues.4 Collaboration among team members fosters the delivery of more effective and efficient care, increases staff satisfaction, and improves employee retention.6 Leader rounding for high reliability significantly contributes to the breakdown of power barriers by giving team members voice and agency, ultimately leading to deeper engagement.7
It is important that leader rounding for high reliability occurs as planned and when possible, scheduled in advance. This helps to avoid rounding at peak times when care activities are being performed.4,6 When scheduling conflicts arise, another leader should be sent to participate in rounds.4 Developing a list of questions in advance allows leadership to prepare messaging to share with staff as it relates to high reliability and patient safety (Table).4,6,8
Closing the loop improves bidirectional communication and is critical to leader rounding for high reliability. Closed-loop communication and following up on and/or closing out issues raised during rounding empowers the sharing of information, which is critical for advancing a culture of safety.4,8 Enhanced feedback is also associated with greater workforce engagement, staff feeling more connected to quality improvement activities, and lower rates of employee burnout.7 It is important to recognize that senior leaders are not responsible for resolving all issues. If a team or manager can resolve concerns that are raised, this should be encouraged and supported. Maintaining accountability at the lowest level of the organization promotes principles and practices of high reliability (Figure 3).4,8
The VA Bedford Healthcare System created and implemented a strong practice for leader rounding for high reliability. This phased implementation involved creating an evidence-based process, deciding on an appropriate cadence, developing a tracking tool, and measuring impact to determine the overall effectiveness of leader rounding for high reliability.4
Visual Management Systems
A visual management system (VMS) displays clinical and operational performance aligned with HRO goals and practices. It is used to view and guide discussions between interdisciplinary teams during tiered safety huddles, leader rounds for high reliability, and frontline staff on the current status and safety trends in a particular area.8,9 A VMS is highly effective in creating an environment where all staff members, especially frontline workers, feel empowered to voice their concerns related to safety or to identify improvement opportunities.8,10 Increased leader engagement in patient safety and heightened transparency of information associated with the use of a VMS improves staff morale and professional satisfaction.10
A VMS may be a dry-erase or whiteboard display, paper-based display, or electronic status board.8 VMSs are usually located in or near work settings (eg, nurses’ station, staff break room, or conference room).8 Although they can take different forms and display several types of information, a VMS should be easy to update and meet the specific needs of a work area. In the VHA, a VMS displays: (1) essential information for staff members to effectively perform their work; (2) improvement project ideas; (3) current work in progress; (4) tracking of implemented improvement activities; (5) strong practices that have been effective; and (6) staff recognition for those who have enhanced patient safety, including the reporting of close calls and near misses.
The VHA uses the MESS (methods, equipment, staffing, and supplies) VMS format. This format empowers staff to identify whether proper procedures and practices are in place, essential equipment and supplies are readily available in the quantity needed, and appropriate staffing is on hand to provide safe, high-quality patient care.8 Colored magnets are used as visual cues in a stoplight classification system to identify low or no safety risks (green), at risk (yellow), or high risk (red). Green coded issues are addressed locally by a manager or supervisor. Yellow coded concerns require increased staff and leadership vigilance. Red coded issues indicate that patient care would be impacted that day and therefore need to be immediately escalated and addressed with senior leaders to mitigate the threat.4,11 Dayton VAMC successfully implemented a VMS, using both physical and electronic visual management boards. The Dayton VAMC VMS boards are closely tied to tiered safety huddles and leader rounding for high reliability.
Safety Forums
Safety forums are another foundational practice of VHA health care organizations seeking high reliability. Recurring monthly, safety forums focus on reinforcing HRO principles and practices, safety programs, the importance and appreciation of reporting, and just culture. The emphasis on just culture reminds staff that adverse events in the organization are viewed as valuable learning opportunities to understand the factors leading to the situation as opposed to immediately assigning blame.12
Psychological safety is another important focus. When individuals feel psychologically safe, they are more likely to voice concerns and act without fear of reprisal, which supports a culture of safety.13 Safety forums are open to all members of the health care organization, including both clinical and nonclinical staff. Forums can be conducted by an HRO Lead, HRO Champion, Patient Safety Manager, or even executive leadership. Rotating the responsibility of leading these forums demonstrates that high reliability and safety are everyone’s responsibility.
Safety forums publicly review and discuss errors, adverse events, close calls, and near misses. Time is also spent discussing root cause analysis trends and highlighting continuous process improvement principles and current projects. During safety forums, leaders should recognize individuals for safety behaviors and reward reporting through a safety awards program.14 All forums should conclude with a question-and-answer session. Forums typically occur in virtual 30-minute sessionsbut can last up to 60 minutes when guest speakers attend and continuing education credit is offered.
The Jesse Brown VAMC in Chicago developed an interactive monthly safety forum appealing to a broad audience. Each forum is attended by about 200 staff members and includes leader engagement and panel discussions led by the chief medical officer, with topics on both patient and team safety connecting with HRO principles. A planning committee prepares guest speakers and offers continuing education credits.
Tiered Safety Huddles
Based on the processes of high reliability industries like aviation and nuclear power, tiered safety huddles have been increasingly adopted in health care. Huddles (health care, utilizing, deliberate, discussion,linking, and events) are department-level interdisciplinary meetings that last no more than 15 minutes.15 Their purpose is to improve communication by sharing day-to-day information across multiple disciplines, identify issues that may impact the delivery of care (eg, patient and staff safety concerns, staffing issues, or inadequate supplies) and resolve problems.
Tiered safety huddles are gaining popularity, especially in organizations seeking high reliability. They are more complex than traditional huddles because of the mechanics of elevating safety issues (eg, bedside to executive leadership teams), feedback loops, and sequencing, among other factors.15,16
Tiered safety huddles are focused, transparent forums with multidisciplinary staff, including frontline workers, along with senior leadership.15,16 When initially implemented, tiered safety huddles may take longer than the suggested 15 minutes; however, as teams become more experienced, huddles become more efficient.15 The goal of tiered safety huddles is to proactively identify, share, address, and resolve problems that have the potential to impact the delivery of safe and quality patient care. This may include addressing staffing shortfalls, inadequate allocation of supplies and equipment, operational issues, etc.8,15 Critical to theeffective utilization of tiered safety huddles is the appropriate escalation of issues between tiers. The most critical issues are elevated to higher tiers so they are addressed by the most qualified person in the organization.
Deciding on the number of tiers typically depends on the size and scope of services provided by the health care organization or integrated system.For example, tiered huddles in the VHA originate at the point of service (eg, critical care unit). Tier 1 includes staff members at the unit/team level along with immediate supervisors/managers. Tier 2 involves departments and service lines (eg, pharmacy, podiatry, or internal medicine) including their respective leadership. Tier 3 is the executive leadership team. This process allows for bidirectional communication instead of the traditional hierarchical communication pathway (Figure 4). Issues identified that cannot be addressed at a particular tier are elevated to the next tier. Elevated issues typically involve systems or processes requiring attention and resolution by senior leadership.15 Tier 4 huddles at the Veterans Integrated Services Network level and Tier 5 huddles at the VHA Central Office level are being initiated. These additional levels will more effectively identify system-level risks and issues that may impact multiple VHA facilities and may be addressed through centralized functions and resources.
Tiered safety huddles have been found to be instrumental to ensuring the flow of information across organizations, improving multidisciplinary and leadership engagement and collaboration, as well as increasing accountability for safety.Tiered safety huddles increase situational awareness, which improves an organization’s ability to appropriately respond to safety concerns.Furthermore, tiered safety huddles enhance teamwork and interprofessional collaboration, and have been found to significantly increase the reporting of patient safety events.15-19
The VA Connecticut Healthcare System tiered huddles followed a pilot testing implementation process. After receiving executive-level commitment, an evidence-based process was enacted, including staff education, selecting a VMS, determining tier interaction, and deciding on metrics to track.15
Implementing Foundational Practices
To examine the progress of the implementation of the 4 foundational HRO practices, quarterly metrics derived from the OLT are reviewed to determine whether each is being implemented and sustained. The OLT also tracks progress over time. For example, at the 27 cohort 2 and lead sites that initiated leader coaching in 2021 and continued through 2022, coaches observed a 27% increase in leader rounding for high reliability and a 46% increase in the use of VMSs. For the 66 cohort 3 sites that began leader coaching in 2022, coaches documented similar changes, ranging from a 40% increase in leader rounding for high reliability to a 66% increase in the use of safety forums. Additional data continue to be collected and analyzed to publish more comprehensive findings.
DISCUSSION
Incorporating leader rounding for high reliability, VMSs, safety forums, and tiered safety huddles into daily operations is critical to building and sustaining a robust culture of safety.8 The 4 foundational HRO practices are instrumental in providing psychologically safe forums for staff to share concerns and actively participate. These practices also promote continual, efficient bidirectional communication throughout organizational lines and across services. The increased visibility and transparency of leaders demonstrate the importance of fostering trust, enhancing closed-loop communication with issues that arise, and building momentum to achieve high reliability. The interconnectedness of the foundational HRO practices identified and implemented by the VHA helps foster teamwork and collaboration built on trust, respect, enthusiasm for improvement, and the delivery of exceptional patient care.
CONCLUSIONS
Incorporating the 4 foundational practices into daily operations is beneficial to the delivery of safe, high-quality health care. This effective and sustained application can strengthen a health care organization on its journey to high reliability and establishing a culture of safety. To be effective, these foundational practices should be personalized to support the unique circumstances of every health care environment. While the exact methodology by which organizations implement these practices may differ, they will help organizations approach patient safety in a more transparent and thoughtful manner.
Acknowledgments
The authors thank Aaron M. Sawyer, PhD, PMP, and Jessica Fankhauser, MA, for their unwavering administrative support, and Jeff Wright for exceptional graphic design support.
Increasing complexities within health care systems are significant impediments to the consistent delivery of safe and effective patient care. These impediments include an increase in specialization of care, staff shortages, burnout, poor coordination of services and access to care, as well as rising costs.1 High reliability organizations (HROs) provide safe, high-quality, and effective care in highly complex and risk-prone environments without causing harm or experiencing catastrophic events.2
Within the US Department of Veterans Affairs (VA), the Veterans Health Administration (VHA) operates the nation’s largest integrated health care system, providing care to > 9 million veterans. The VHA formally launched plans for an enterprise-wide HRO in February 2019. During the first year, 18 medical facilities comprised cohort1 of the journey to high reliability. Cohort 2 began in October 2020 and consisted of 54 facilities. Cohort 3 started in October 2021 with 67 facilities.3
Health care organizations seeking high reliability exercise a philosophy aimed at learning from errors and addressing system failures. High reliability is accomplished by implementing 5 principles: (1) sensitivity to operations (a heightened understanding of the current state of systems); (2) preoccupation with failure (striving to anticipate risks that might suggest a much larger system problem); (3) reluctance to simplify (avoiding making any assumptions regarding the causes of failures); (4) commitment to resilience (preparing for potential failures and bouncing back when they occur); and (5) deference to expertise (deferring to individuals with the skills and proficiency to make the best decisions).2 The VHA also recognized that a successful journey to high reliability—in addition to achieving a culture of safety—relies on the implementation of foundational HRO practices: leader rounding, visual management systems, safety forums, and safety huddles. This article describes an initiative for how these foundational practices were implemented in a large integrated health care system.
BACKGROUND
The VHA has focused on 4 foundational components as part of its enterprise activities and support structure to implement HRO principles and practices. These components were selected based on pilot activities that preceded the enterprise-wide effort, reviews of the literature, and expert consultation with both government and private sector health systems. To support the implementation of these practices, the VHA provided training, toolkits, HRO executive leader coaching, and peer-to-peer mentoring. As the VHA enters its fifth year seeking high reliability, we undertook an initiative to reflect on our own experiences and refine our practices based on an updated literature review.
As part of this enterprise-wide initiative, we conducted a literature review from 2018 to March 2023 seeking recent evidence describing the value of implementing the 4 foundational HRO practices to advance high reliability and improve patient safety. A 5-year period was used to ensure recency and value of evidence.
Eligible literature was identified in PubMed, PsycINFO, the Cumulative Index to Nursing and Allied Health Literature, ScienceDirect, Scopus, the Cochrane Library, and ProQuest Dissertations & Theses Global. Inclusion and exclusion criteria were peer-reviewed interdisciplinary documents(eg, publications, dissertations, conference proceedings, and grey literature) written in English. Search terms included high reliability organizations, foundational practices, and patient safety. Boolean operators (AND, OR) were also used in the search. The search resulted in a dearth of evidence that addressed implementation of all 4 foundational practices across a health care system. Retrieved evidence focused on the implementation of only 1 particular foundational practice in a specific health care setting. In addition to describing the formal processes for the implementation of each foundational HRO practice, a brief description of representative examples of strong practices within the VHA is provided.
To support the implementation of HROs, the VHA paired HRO executive leader coaches with select medical center directors and their leadership teams. Executive leader coaches also support an organization’s HRO Lead and HRO Champion. The HRO Lead coordinates and facilitates the implementation of HRO principles and practices in pursuit of no harm across an organization. The HRO Champion supports the same as the HRO Lead, but typically has a different specialty background. For example, if the HRO Lead has an administrative background, the HRO Champion would have a clinical background.
Coaching focuses heavily on supporting site-specific implementation and sustainment of the 4 HRO foundational practices. The aim is to accelerate change, build enduring capacity, foster a safety culture, and accelerate HRO maturity. To measure change, HRO executive leader coaches track the progress of their aligned VA medical centers (VAMCs) using the Organizational Learning Tool (OLT). This tool was developed to provide information such as a facility summary and relationships between a medical center director, HRO Lead, HRO Champion, and the executive leader coach (Figure 1). The OLT also serves as a structured process to measure leader coaching performance against mutually agreed upon objectives that ultimately contribute to enterprise outcomes. It also collects data on the progress in implementing foundational practices, strong practices, needs and gaps, and more (Figure 2). Data collected from facilities supported by HRO executive leader coaches on whether foundational practices are in place are briefly described.
Leader Rounding
Leader rounding for high reliability ensures effective, bidirectional communication and collaboration among all disciplines to improve patient safety. It is an essential feature of a robust patient safety culture and an important method for demonstrating leadership engagement with high reliability.4,5 These rounds are conducted by organizational leadership (eg, executive teams, department/service chiefs, or unit managers) and frontline staff from different areas. They are specifically focused on high reliability, patient and staff safety, and improvement efforts. The aim is to learn about daily challenges that may contribute to patient harm.4
Leader rounding has been found to be highly effective at improving leadership visibility across the organization. It enhances interaction and open communication with frontline staff, fostering leader-staff collaboration and shared decision-making,as well as promoting leadership understanding of operational, clinical, nonclinical (eg, administrative, nutrition services, or facilities management), and patient/family experience issues.4 Collaboration among team members fosters the delivery of more effective and efficient care, increases staff satisfaction, and improves employee retention.6 Leader rounding for high reliability significantly contributes to the breakdown of power barriers by giving team members voice and agency, ultimately leading to deeper engagement.7
It is important that leader rounding for high reliability occurs as planned and when possible, scheduled in advance. This helps to avoid rounding at peak times when care activities are being performed.4,6 When scheduling conflicts arise, another leader should be sent to participate in rounds.4 Developing a list of questions in advance allows leadership to prepare messaging to share with staff as it relates to high reliability and patient safety (Table).4,6,8
Closing the loop improves bidirectional communication and is critical to leader rounding for high reliability. Closed-loop communication and following up on and/or closing out issues raised during rounding empowers the sharing of information, which is critical for advancing a culture of safety.4,8 Enhanced feedback is also associated with greater workforce engagement, staff feeling more connected to quality improvement activities, and lower rates of employee burnout.7 It is important to recognize that senior leaders are not responsible for resolving all issues. If a team or manager can resolve concerns that are raised, this should be encouraged and supported. Maintaining accountability at the lowest level of the organization promotes principles and practices of high reliability (Figure 3).4,8
The VA Bedford Healthcare System created and implemented a strong practice for leader rounding for high reliability. This phased implementation involved creating an evidence-based process, deciding on an appropriate cadence, developing a tracking tool, and measuring impact to determine the overall effectiveness of leader rounding for high reliability.4
Visual Management Systems
A visual management system (VMS) displays clinical and operational performance aligned with HRO goals and practices. It is used to view and guide discussions between interdisciplinary teams during tiered safety huddles, leader rounds for high reliability, and frontline staff on the current status and safety trends in a particular area.8,9 A VMS is highly effective in creating an environment where all staff members, especially frontline workers, feel empowered to voice their concerns related to safety or to identify improvement opportunities.8,10 Increased leader engagement in patient safety and heightened transparency of information associated with the use of a VMS improves staff morale and professional satisfaction.10
A VMS may be a dry-erase or whiteboard display, paper-based display, or electronic status board.8 VMSs are usually located in or near work settings (eg, nurses’ station, staff break room, or conference room).8 Although they can take different forms and display several types of information, a VMS should be easy to update and meet the specific needs of a work area. In the VHA, a VMS displays: (1) essential information for staff members to effectively perform their work; (2) improvement project ideas; (3) current work in progress; (4) tracking of implemented improvement activities; (5) strong practices that have been effective; and (6) staff recognition for those who have enhanced patient safety, including the reporting of close calls and near misses.
The VHA uses the MESS (methods, equipment, staffing, and supplies) VMS format. This format empowers staff to identify whether proper procedures and practices are in place, essential equipment and supplies are readily available in the quantity needed, and appropriate staffing is on hand to provide safe, high-quality patient care.8 Colored magnets are used as visual cues in a stoplight classification system to identify low or no safety risks (green), at risk (yellow), or high risk (red). Green coded issues are addressed locally by a manager or supervisor. Yellow coded concerns require increased staff and leadership vigilance. Red coded issues indicate that patient care would be impacted that day and therefore need to be immediately escalated and addressed with senior leaders to mitigate the threat.4,11 Dayton VAMC successfully implemented a VMS, using both physical and electronic visual management boards. The Dayton VAMC VMS boards are closely tied to tiered safety huddles and leader rounding for high reliability.
Safety Forums
Safety forums are another foundational practice of VHA health care organizations seeking high reliability. Recurring monthly, safety forums focus on reinforcing HRO principles and practices, safety programs, the importance and appreciation of reporting, and just culture. The emphasis on just culture reminds staff that adverse events in the organization are viewed as valuable learning opportunities to understand the factors leading to the situation as opposed to immediately assigning blame.12
Psychological safety is another important focus. When individuals feel psychologically safe, they are more likely to voice concerns and act without fear of reprisal, which supports a culture of safety.13 Safety forums are open to all members of the health care organization, including both clinical and nonclinical staff. Forums can be conducted by an HRO Lead, HRO Champion, Patient Safety Manager, or even executive leadership. Rotating the responsibility of leading these forums demonstrates that high reliability and safety are everyone’s responsibility.
Safety forums publicly review and discuss errors, adverse events, close calls, and near misses. Time is also spent discussing root cause analysis trends and highlighting continuous process improvement principles and current projects. During safety forums, leaders should recognize individuals for safety behaviors and reward reporting through a safety awards program.14 All forums should conclude with a question-and-answer session. Forums typically occur in virtual 30-minute sessionsbut can last up to 60 minutes when guest speakers attend and continuing education credit is offered.
The Jesse Brown VAMC in Chicago developed an interactive monthly safety forum appealing to a broad audience. Each forum is attended by about 200 staff members and includes leader engagement and panel discussions led by the chief medical officer, with topics on both patient and team safety connecting with HRO principles. A planning committee prepares guest speakers and offers continuing education credits.
Tiered Safety Huddles
Based on the processes of high reliability industries like aviation and nuclear power, tiered safety huddles have been increasingly adopted in health care. Huddles (health care, utilizing, deliberate, discussion,linking, and events) are department-level interdisciplinary meetings that last no more than 15 minutes.15 Their purpose is to improve communication by sharing day-to-day information across multiple disciplines, identify issues that may impact the delivery of care (eg, patient and staff safety concerns, staffing issues, or inadequate supplies) and resolve problems.
Tiered safety huddles are gaining popularity, especially in organizations seeking high reliability. They are more complex than traditional huddles because of the mechanics of elevating safety issues (eg, bedside to executive leadership teams), feedback loops, and sequencing, among other factors.15,16
Tiered safety huddles are focused, transparent forums with multidisciplinary staff, including frontline workers, along with senior leadership.15,16 When initially implemented, tiered safety huddles may take longer than the suggested 15 minutes; however, as teams become more experienced, huddles become more efficient.15 The goal of tiered safety huddles is to proactively identify, share, address, and resolve problems that have the potential to impact the delivery of safe and quality patient care. This may include addressing staffing shortfalls, inadequate allocation of supplies and equipment, operational issues, etc.8,15 Critical to theeffective utilization of tiered safety huddles is the appropriate escalation of issues between tiers. The most critical issues are elevated to higher tiers so they are addressed by the most qualified person in the organization.
Deciding on the number of tiers typically depends on the size and scope of services provided by the health care organization or integrated system.For example, tiered huddles in the VHA originate at the point of service (eg, critical care unit). Tier 1 includes staff members at the unit/team level along with immediate supervisors/managers. Tier 2 involves departments and service lines (eg, pharmacy, podiatry, or internal medicine) including their respective leadership. Tier 3 is the executive leadership team. This process allows for bidirectional communication instead of the traditional hierarchical communication pathway (Figure 4). Issues identified that cannot be addressed at a particular tier are elevated to the next tier. Elevated issues typically involve systems or processes requiring attention and resolution by senior leadership.15 Tier 4 huddles at the Veterans Integrated Services Network level and Tier 5 huddles at the VHA Central Office level are being initiated. These additional levels will more effectively identify system-level risks and issues that may impact multiple VHA facilities and may be addressed through centralized functions and resources.
Tiered safety huddles have been found to be instrumental to ensuring the flow of information across organizations, improving multidisciplinary and leadership engagement and collaboration, as well as increasing accountability for safety.Tiered safety huddles increase situational awareness, which improves an organization’s ability to appropriately respond to safety concerns.Furthermore, tiered safety huddles enhance teamwork and interprofessional collaboration, and have been found to significantly increase the reporting of patient safety events.15-19
The VA Connecticut Healthcare System tiered huddles followed a pilot testing implementation process. After receiving executive-level commitment, an evidence-based process was enacted, including staff education, selecting a VMS, determining tier interaction, and deciding on metrics to track.15
Implementing Foundational Practices
To examine the progress of the implementation of the 4 foundational HRO practices, quarterly metrics derived from the OLT are reviewed to determine whether each is being implemented and sustained. The OLT also tracks progress over time. For example, at the 27 cohort 2 and lead sites that initiated leader coaching in 2021 and continued through 2022, coaches observed a 27% increase in leader rounding for high reliability and a 46% increase in the use of VMSs. For the 66 cohort 3 sites that began leader coaching in 2022, coaches documented similar changes, ranging from a 40% increase in leader rounding for high reliability to a 66% increase in the use of safety forums. Additional data continue to be collected and analyzed to publish more comprehensive findings.
DISCUSSION
Incorporating leader rounding for high reliability, VMSs, safety forums, and tiered safety huddles into daily operations is critical to building and sustaining a robust culture of safety.8 The 4 foundational HRO practices are instrumental in providing psychologically safe forums for staff to share concerns and actively participate. These practices also promote continual, efficient bidirectional communication throughout organizational lines and across services. The increased visibility and transparency of leaders demonstrate the importance of fostering trust, enhancing closed-loop communication with issues that arise, and building momentum to achieve high reliability. The interconnectedness of the foundational HRO practices identified and implemented by the VHA helps foster teamwork and collaboration built on trust, respect, enthusiasm for improvement, and the delivery of exceptional patient care.
CONCLUSIONS
Incorporating the 4 foundational practices into daily operations is beneficial to the delivery of safe, high-quality health care. This effective and sustained application can strengthen a health care organization on its journey to high reliability and establishing a culture of safety. To be effective, these foundational practices should be personalized to support the unique circumstances of every health care environment. While the exact methodology by which organizations implement these practices may differ, they will help organizations approach patient safety in a more transparent and thoughtful manner.
Acknowledgments
The authors thank Aaron M. Sawyer, PhD, PMP, and Jessica Fankhauser, MA, for their unwavering administrative support, and Jeff Wright for exceptional graphic design support.
1. Figueroa CA, Harrison R, Chauhan A, Meyer L. Priorities and challenges for health leadership and workforce management globally: a rapid review. BMC Health Serv Res. 2019;19(1):239. Published 2019 Apr 24. doi:10.1186/s12913-019-4080-7
2. What is a high reliability organization (HRO) in healthcare? Vizient. Accessed May 22, 2024. https://www.vizientinc.com/our-solutions/care-delivery-excellence/reliable-care-delivery
3. US Department of Veterans Affairs, VHA National Center for Patient Safety. VHA’s HRO journey officially begins. March 29, 2019. Accessed May 22, 2024. https://www.patientsafety.va.gov/features/VHA_s_HRO_journey_officially_begins.asp
4. Murray JS, Clifford J, Scott D, Kelly S, Hanover C. Leader rounding for high reliability and improved patient safety. Fed Pract. 2024;41(1):16-21. doi:10.12788/fp.0444
5. Ryan L, Jackson D, Woods C, Usher K. Intentional rounding – an integrative literature review. J Adv Nurs. 2019;75(6):1151-1161. doi:10.1111/jan.13897
6. Hedenstrom M, Harrilson A, Heath M, Dyess S. “What’s old is new again”: innovative health care leader rounding—a strategy to foster connection. Nurse
7. Blake PG, Bacon CT. Structured rounding to improve staff nurse satisfaction with leadership. Nurse Lead. 2020;18(5):461-466. doi:10.1016/j.mnl.2020.04.009
8. US Department of Veterans Affairs, Veterans Health Administration. Leader’s guide to foundational high reliability organization (HRO) practices. https://dvagov.sharepoint.com/sites/OHT-PMO/high-reliability/Pages/default.aspx
9. Goyal A, Glanzman H, Quinn M, et al. Do bedside whiteboards enhance communication in hospitals? An exploratory multimethod study of patient and nurse perspectives. BMJ Qual Saf. 2020;29(10):1-2. doi:10.1136/bmjqs-2019-01020810. Williamsson A, Dellve L, Karltun A. Nurses’ use of visual management in hospitals-a longitudinal, quantitative study on its implications on systems performance and working conditions. J Adv Nurs. 2019;75(4):760-771. doi:10.1111/jan.13855
11. Prineas S, Culwick M, Endlich Y. A proposed system for standardization of colour-coding stages of escalating criticality in clinical incidents. Curr Opin Anaesthesiol. 2021;34(6):752-760. doi:10.1097/ACO.0000000000001071
12. Murray JS, Clifford J, Larson S, Lee JK, Sculli GL. Implementing just culture to improve patient safety. Mil Med. 2023;188(7-8):1596-1599. doi:10.1093/milmed/usac115
13. Murray JS, Kelly S, Hanover C. Promoting psychological safety in healthcare organizations. Mil Med. 2022;187(7-8):808-810. doi:10.1093/milmed/usac041
14. Merchant NB, O’Neal J, Murray JS. Development of a safety awards program at a veterans affairs health care system: a quality improvement initiative. J Clin Outcomes Manag. 2023;30(1):9-16. doi:10.12788/jcom.0120
15. Merchant NB, O’Neal J, Montoya A, Cox GR, Murray JS. Creating a process for the implementation of tiered huddles in a veterans affairs medical center. Mil Med. 2023;188(5-6):901-906. doi:10.1093/milmed/usac073
16. Mihaljevic T. Tiered daily huddles: the power of teamwork in managing large healthcare organisations. BMJ Qual Saf. 2020;29(12):1050-1052. doi:10.1136/bmjqs-2019-010575
17. Franklin BJ, Gandhi TK, Bates DW, et al. Impact of multidisciplinary team huddles on patient safety: a systematic review and proposed taxonomy. BMJ Qual Saf. 2020;29(10):1-2. doi:10.1136/bmjqs-2019-009911
18. Pimentel CB, Snow AL, Carnes SL, et al. Huddles and their effectiveness at the frontlines of clinical care: a scoping review. J Gen Intern Med. 2021;36(9):2772-2783. doi:10.1007/s11606-021-06632-9
19. Adapa K, Ivester T, Shea C, et al. The effect of a system-level tiered huddle system on reporting patient safety events: an interrupted time series analysis. Jt Comm J Qual Patient Saf. 2022;48(12):642-652. doi:10.1016/j.jcjq.2022.08.005
1. Figueroa CA, Harrison R, Chauhan A, Meyer L. Priorities and challenges for health leadership and workforce management globally: a rapid review. BMC Health Serv Res. 2019;19(1):239. Published 2019 Apr 24. doi:10.1186/s12913-019-4080-7
2. What is a high reliability organization (HRO) in healthcare? Vizient. Accessed May 22, 2024. https://www.vizientinc.com/our-solutions/care-delivery-excellence/reliable-care-delivery
3. US Department of Veterans Affairs, VHA National Center for Patient Safety. VHA’s HRO journey officially begins. March 29, 2019. Accessed May 22, 2024. https://www.patientsafety.va.gov/features/VHA_s_HRO_journey_officially_begins.asp
4. Murray JS, Clifford J, Scott D, Kelly S, Hanover C. Leader rounding for high reliability and improved patient safety. Fed Pract. 2024;41(1):16-21. doi:10.12788/fp.0444
5. Ryan L, Jackson D, Woods C, Usher K. Intentional rounding – an integrative literature review. J Adv Nurs. 2019;75(6):1151-1161. doi:10.1111/jan.13897
6. Hedenstrom M, Harrilson A, Heath M, Dyess S. “What’s old is new again”: innovative health care leader rounding—a strategy to foster connection. Nurse
7. Blake PG, Bacon CT. Structured rounding to improve staff nurse satisfaction with leadership. Nurse Lead. 2020;18(5):461-466. doi:10.1016/j.mnl.2020.04.009
8. US Department of Veterans Affairs, Veterans Health Administration. Leader’s guide to foundational high reliability organization (HRO) practices. https://dvagov.sharepoint.com/sites/OHT-PMO/high-reliability/Pages/default.aspx
9. Goyal A, Glanzman H, Quinn M, et al. Do bedside whiteboards enhance communication in hospitals? An exploratory multimethod study of patient and nurse perspectives. BMJ Qual Saf. 2020;29(10):1-2. doi:10.1136/bmjqs-2019-01020810. Williamsson A, Dellve L, Karltun A. Nurses’ use of visual management in hospitals-a longitudinal, quantitative study on its implications on systems performance and working conditions. J Adv Nurs. 2019;75(4):760-771. doi:10.1111/jan.13855
11. Prineas S, Culwick M, Endlich Y. A proposed system for standardization of colour-coding stages of escalating criticality in clinical incidents. Curr Opin Anaesthesiol. 2021;34(6):752-760. doi:10.1097/ACO.0000000000001071
12. Murray JS, Clifford J, Larson S, Lee JK, Sculli GL. Implementing just culture to improve patient safety. Mil Med. 2023;188(7-8):1596-1599. doi:10.1093/milmed/usac115
13. Murray JS, Kelly S, Hanover C. Promoting psychological safety in healthcare organizations. Mil Med. 2022;187(7-8):808-810. doi:10.1093/milmed/usac041
14. Merchant NB, O’Neal J, Murray JS. Development of a safety awards program at a veterans affairs health care system: a quality improvement initiative. J Clin Outcomes Manag. 2023;30(1):9-16. doi:10.12788/jcom.0120
15. Merchant NB, O’Neal J, Montoya A, Cox GR, Murray JS. Creating a process for the implementation of tiered huddles in a veterans affairs medical center. Mil Med. 2023;188(5-6):901-906. doi:10.1093/milmed/usac073
16. Mihaljevic T. Tiered daily huddles: the power of teamwork in managing large healthcare organisations. BMJ Qual Saf. 2020;29(12):1050-1052. doi:10.1136/bmjqs-2019-010575
17. Franklin BJ, Gandhi TK, Bates DW, et al. Impact of multidisciplinary team huddles on patient safety: a systematic review and proposed taxonomy. BMJ Qual Saf. 2020;29(10):1-2. doi:10.1136/bmjqs-2019-009911
18. Pimentel CB, Snow AL, Carnes SL, et al. Huddles and their effectiveness at the frontlines of clinical care: a scoping review. J Gen Intern Med. 2021;36(9):2772-2783. doi:10.1007/s11606-021-06632-9
19. Adapa K, Ivester T, Shea C, et al. The effect of a system-level tiered huddle system on reporting patient safety events: an interrupted time series analysis. Jt Comm J Qual Patient Saf. 2022;48(12):642-652. doi:10.1016/j.jcjq.2022.08.005
Suspected Orbital Compartment Syndrome Leading to Visual Loss After Pterional Craniotomy
Perioperative visual loss (POVL) is a well-documented yet uncommon complication of nonocular surgery. Patients undergoing cardiac and spinal surgery are at the greatest risk, though POVL may occur during other neurosurgical and vascular procedures as well. The most common causes of POVL are central retinal artery occlusion (CRAO) and ischemic optic neuropathy (ION),1-3 though cases of orbital compartment syndrome (OCS) have also been reported.4-7
We describe a case of POVL during a temporal meningioma excision using the pterional approach. Though the etiology is not fully understood, the patient’s clinical course was complicated by a third cranial nerve (CN III) palsy and CRAO, which, together with the patient’s presentation, were consistent with previously documented cases of OCS. The goals of this case report are to increase awareness of this surgical outcome, identify practices that may have contributed to its development, and delineate methods to minimize its occurrence.
Informed consent regarding this research was obtained from the patient and an institutional Health Insurance Portability and Accountability Act authorization form was completed. This manuscript adheres to the applicable Enhancing the Quality and Transparency of Health Research guideline.8
Case Presentation
A 47-year-old woman underwent a left temporal craniotomy for resection of a sphenoid wing meningioma discovered during a workup for persistent headaches. She had no medical history of diabetes, hypertension, coronary artery disease, or ophthalmic disease. Two months before her scheduled surgery, the patient reported bilateral blurry vision and underwent ophthalmologic evaluation. Her intraocular pressure (IOP) was normal, and she had no pupillary or retinal disease. She showed evidence of decreased vision in her left eye, suggesting a possible mass effect from her meningioma. Subsequent imaging of the optic nerve and retina had unremarkable physiology (Figure 1). Preoperative magnetic resonance imaging (MRI) demonstrated a stable enhancing mass involving the left great sphenoid wing and left cavernous sinus(Figure 2). There was a superior mass effect on the left middle cerebral artery, but all vessels were patent without evidence of thrombosis.
The patient underwent general anesthesia with invasive hemodynamic monitoring used throughout the procedure. She was induced with fentanyl, propofol, and rocuronium; anesthesia was maintained with isoflurane and a remifentanil infusion. Hypotension was treated with phenylephrine and intravenous fluids. Intraoperative neuromonitoring with electroencephalogram (EEG) and somatosensory evoked potentials was performed. During the surgery, the patient was positioned supine in a Mayfield 3-point head fixation system. All pressure points were padded appropriately and continually checked. A standard left pterional craniotomy was performed, and the scalp was reflected anteriorly and secured using fish hooks with rubber bands. The operation did not violate the cavernous sinus or orbital compartment. There was no evidence of active bleeding upon inspection nor with the Valsalva maneuver. No changes were noted in EEG or somatosensory evoked potentials; blood pressure remained within 20 mm Hg of the patient’s baseline. She was extubated at the end of the 10-hour case and was hemodynamically stable upon transport to the surgical intensive care unit. Postoperative imaging confirmed the successful removal of the left sphenoid wing meningioma.
The patient’s postoperative examination demonstrated a 5 mm dilated, nonresponsive left pupil, though the patient did not report visual loss at that time. Defects were noted in the inferior oblique, superior, inferior, and medial rectus muscles, consistent with CN III palsy. The surgery included manipulation of CN III, which made this a possible outcome, but an alternate causative pathology like OCS was not immediately suspected. Postoperative computed tomography (CT) showed an expected pneumocephalus and left scalp swelling without evidence of mass effect or midline shift.
On the morning of postoperative Day 1, the patient reported vision loss in her left eye, while her clinical examination revealed erythema and conjunctival chemosis with left eyelid swelling. The ophthalmologic evaluation was notable for a continued leftCN III palsy with intact lateral rectus and superior oblique function, a nonreactive and dilated left eye with 3+ afferent pupillary defect by reverse (light perception), pallor throughout, a flat cherry red macula with blurred disc margins, left upper eyelid edema, and 18 mm Hg intraocular pressure bilaterally (reference range, 8 to 21 mm Hg). Fundoscopic examination showed a clear vitreous without plaques or occlusions, no perivascular sheathing, and no retinal hemorrhages. CT angiography revealed small outpouchings at the superolateral aspect of the left and right cavernous carotid, consistent with atherosclerotic calcifications. An echocardiogram revealed a Valsalva-dependent patent foramen ovale, but a venous Doppler ultrasound yielded negative results.
Repeat MRI showed denervation of the left medial rectus and minimal left-sided proptosis. A 3-month ophthalmologic follow-up revealed a persistent CN III palsy, including an afferent pupillary defect, absence of light perception in her left eye, and continued ophthalmoplegia. Repeat examination showed a left-sided 4+ afferent pupillary defect unreactive to light, 4+ pallor surrounding the optic nerve, macular atrophy, sclerotic vessels, and 17 mm Hg intraocular pressure bilaterally. The eye had diffuse atrophy of the inner retina and significant patchy atrophy of the outer retinal components without neovascularization of the iris. Postoperative retinal imaging can be seen in Figure 3. Her vision loss persisted at this encounter and has continued through subsequent follow-up examinations.
Discussion
Perioperative visual loss is a rare surgical complication, with an estimated incidence of once in every 60,000 to 125,000 cases.9 The mechanism of injury is variable and dependent upon the type of surgical intervention, with cardiac and spine surgeries carrying the greatest risk.10,11 The injury often results in either CRAO or ION, which may result in visual loss.1-3 POVL can also occur in the aftermath of rapid changes in intracranial pressure during decompressive craniotomies, though the pathophysiology in such cases is not well understood.5
Among the myriad ways in which POVL can occur, neurosurgical cases carry the unique risk of direct cranial nerve injury. Such an insult can lead to vision loss via optic nerve damage or ophthalmoplegia if damage occurs to CN III, IV, or VI. This can occur during manipulation or resection, especially if the surgical approach involves the orbital cavity or the cavernous sinus. Though neither space was entered in this patient, direct injury cannot be ruled out as the etiology for either her vision loss or persistent ophthalmoplegia. An alternate causative scenario for both symptoms involve an impaired blood supply, with the vision loss potentially occurring secondary to CRAO and the ophthalmoplegia to an alternate cause of decreased blood flow. It is unclear which of these 2 conditions occurred first or if they occurred due to the same insult, but OCS could lead to both. Though it is a less common etiology for POVL, this patient’s presentation was similar to those in previously reported cases, and OCS was identified as the likely diagnosis.
OCS is precipitated by an elevated orbital pressure, which leads to ischemia of the retina and damage to orbital contents. Though associated with retrobulbar hemorrhage and orbital trauma, another proposed mechanism for OCS is extrinsic orbital compression, resulting in increased IOP and subsequent CRAO.10 A cherry red spot is visible on fundoscopy, as only the macula with its thin retinal layer will permit the choroidal vessels to be visualized. In a separate process, the relative increase in orbital pressure may lead to impaired perfusion or damage of CN III. However, a causative relationship between the 2 may be difficult to establish. Such an injury to the oculomotor nerve is demonstrated by impaired function of the inferior oblique, superior rectus, inferior rectus, and medial rectus muscles, which may persist even after the compressive symptoms of OCS have resolved.12 Other reported symptoms of OCS include erythema, ophthalmoplegia, conjunctival chemosis, ptosis, corneal abrasion, and eyelid edema.12-15
Alternate Diagnoses
OCS is a diagnosis of exclusion, and several alternate mechanisms were considered before identifying it as the likely etiology. The patient’s preoperative imaging demonstrated a stable enhancing mass involving the left great sphenoid wing and left cavernous sinus, with displacement of the left middle cerebral artery, left cavernous internal carotid artery, and left optic canal. Dissection and removal of this tumor could have compromised the arterial or venous blood supply to the orbit, thus causing ischemia to the retina and other ocular structures. CN III was manipulated during surgery, and it may have been inadvertently damaged during exposure or resection of the tumor.
The patient’s Valsalva-dependent patent foramen ovale put her at risk of a paroxysmal embolus as an alternate explanation, particularly as a Valsalva maneuver was utilized to confirm hemostasis. The patient did not, however, demonstrate any evidence of venous thromboembolism (VTE) on ultrasound, nor did she have the common risk factors of hypertension, diabetes, or smoking history that would increase VTE risk.16Her cancer diagnosis and surgical status may have put her at risk of VTE, but she did not have any clinical or laboratory values suggestive of hypercoagulability. Had an embolism occurred, it may have compromised the orbital blood supply and led to the CRAO. A similar scenario may have occurred from an atherosclerotic plaque in either of her carotid arteries, as she did have evidence of atherosclerosis on postoperative CT angiography. However, atherosclerosis as a risk factor for POVL appears to be related more to its impact upon impaired blood supply rather than as an embolic source. The patient did not have any significant intraoperative hypotensive episodes, making ION in the setting of atherosclerosis and hypotension a less likely etiology.17
This patient differed from other reported OCS cases. She was never placed in a prone or jackknife position, nor was she agitated or straining for a sustained period. These factors, along with the fact that the orbital compartment was not entered, decreased the likelihood of intraorbital hemorrhage and other intrinsic causes of elevated IOP.12 Additionally, the presentation of our patient’s vision loss was delayed compared with other cases, despite clinicians observing a dilated left pupil and CN III palsy on examination immediately after surgery.14 It is significant to note that OCS may not demonstrate a significant increase in IOP once the source of compression is removed, which may explain the absence of proptosis on her postoperative examination.
The diagnosis of OCS was primarily implicated by the positioning of the myocutaneous flap during the pterional approach to craniotomy. It was retracted anteriorly and superiorly, ultimately resting over her left orbit for most of the 10-hour surgery. Kim and colleagues found that myocutaneous flaps may increase IOP as much as 17.5 mm Hg if improperly positioned, providing an unrecognized source of compression and increasing the risk of damage to orbital contents. According to their review, elevated IOP > 40 mm Hg, particularly over several hours, can compromise blood flow to the optic nerve and increase the risk for POVL.18 The flap was secured using fish hooks and rubber bands. However, it is suspected that the orbital rim did not fully support its pressure, thereby resting to some degree directly on the globe for an extended period and compromising the orbital blood supply. There are no current methods for measuring intraoperative IOP, though surrogate markers are under investigation and may yield clinical utility.18 The myocutaneous flap was created and positioned by the surgeons, but it may be that increased vigilance and communication from the anesthesia and nursing teams could have prevented it from remaining in an improper position.
Conclusions
Despite having few reported cases, OCS must be considered in neurosurgical patients with ophthalmoplegia and CRAO on postoperative examinations. Myocutaneous flaps that are retracted across the orbit can lead to significant elevations in IOP, leading to vision loss, which likely occurred with the patient in this case. Though protecting neurovascular structures is within the purview of the surgeon, all members of the intraoperative team should assist with ensuring proper flap positioning. These measures can help ensure adequate blood flow to the ophthalmic artery, decrease the likelihood of elevated IOP due to extrinsic compression, and help prevent the development of POVL and OCS in these patients.
1. Biousse V, Nahab F, Newman NJ. Management of acute retinal ischemia: follow the guidelines! Ophthalmology. 2018;125(10):1597-1607. doi:10.1016/j.ophtha.2018.03.054
2. Biousse V, Newman NJ. Ischemic optic neuropathies. N Engl J Med. 2015;372(25):2428-2436. doi:10.1056/NEJMra1413352
3. Shah SH, Chen YF, Moss HE, Rubin DS, Joslin CE, Roth S. Predicting risk of perioperative ischemic optic neuropathy in spine fusion surgery: a cohort study using the national inpatient sample. Anesth Analg. 2020;130(4):967-974. doi:10.1213/ANE.0000000000004383
4. Habets JGV, Haeren RHL, Lie SAN, Bauer NJC, Dings JTA. Acute monocular blindness due to orbital compartment syndrome following pterional craniotomy. World Neurosurg. 2018;114:72-75. doi:10.1016/j.wneu.2018.03.013
5. Vahedi P, Meshkini A, Mohajernezhadfard Z, Tubbs RS. Post-craniotomy blindness in the supine position: Unlikely or ignored? Asian J Neurosurg. 2013;8(1):36-41. doi:10.4103/1793-5482.110278
6. Kang S, Yang Y, Kim T, Kim J. Sudden unilateral blindness after intracranial aneurysm surgery. Acta Neurochir (Wien). 1997;139(3):221-226. doi:10.1007/BF01844755
7. Zimmerman CF, Van Patten PD, Golnik KC, Kopitnik TA Jr, Anand R. Orbital infarction syndrome after surgery for intracranial aneurysms. Ophthalmology. 1995;102(4):594-598. doi:10.1016/s0161-6420(95)30979-7
8. Gagnier JJ, Kienle G, Altman DG, et al. The CARE guidelines: consensus-based clinical case reporting guideline development. BMJ Case Rep. 23;2013:bcr2013201554. doi:10.1136/bcr-2013-201554
9. Raphael J, Moss HE, Roth S. Perioperative visual loss in cardiac surgery. J Cardiothorac Vasc Anesth. 2019;33(5):1420-429. doi:10.1053/j.jvca.2018.11.035
10. Kansakar P, Sundar G. Vision loss associated with orbital surgery - a major review. Orbit. 2020;39(3):197-208. doi:10.1080/01676830.2019.1658790
11. Dohlman JC, Yoon MK. Principles of protection of the eye and vision in orbital surgery. J Neurol Surg B Skull Base. 2020;81(4):381-384. doi:10.1055/s-0040-1714077
12. Pahl FH, de Oliveira MF, Dal Col Lúcio JE, Souza E Castro EF. Orbital compartment syndrome after frontotemporal craniotomy: case report and review of literature. World Neurosurg. 2018;109:218-221. doi:10.1016/j.wneu.2017.09.167
13. Grossman W, Ward WT. Central retinal artery occlusion after scoliosis surgery with a horseshoe headrest. Case report and literature review. Spine (Phila Pa 1976). 1993;18(9):1226-1228. doi:10.1097/00007632-199307000-00017
14. Newman NJ. Perioperative visual loss after nonocular surgeries. Am J Ophthalmol. 2008;145(4):604-610. doi:10.1016/j.ajo.2007.09.016
15. Roth S, Tung A, Ksiazek S. Visual loss in a prone-positioned spine surgery patient with the head on a foam headrest and goggles covering the eyes: an old complication with a new mechanism. Anesth Analg. 2007;104(5):1185-1187. doi:10.1213/01.ane.0000264319.57758.55
16. Katz DA, Karlin LI. Visual field defect after posterior spine fusion. Spine (Phila Pa 1976). 2005;30(3):E83-E85. doi:10.1097/01.brs.0000152169.48117.c7
17. Nickels TJ, Manlapaz MR, Farag E. Perioperative visual loss after spine surgery. World J Orthop. 2014;5(2):100-106. Published 2014 April 18. doi:10.5312/wjo.v5.i2.100
18. Kim TS, Hur JW, Park DH, et al. Extraocular ressure measurements to avoid orbital compartment syndrome in aneurysm surgery. World Neurosurg. 2018;118:e601-e609. doi:10.1016/j.wneu.2018.06.248
Perioperative visual loss (POVL) is a well-documented yet uncommon complication of nonocular surgery. Patients undergoing cardiac and spinal surgery are at the greatest risk, though POVL may occur during other neurosurgical and vascular procedures as well. The most common causes of POVL are central retinal artery occlusion (CRAO) and ischemic optic neuropathy (ION),1-3 though cases of orbital compartment syndrome (OCS) have also been reported.4-7
We describe a case of POVL during a temporal meningioma excision using the pterional approach. Though the etiology is not fully understood, the patient’s clinical course was complicated by a third cranial nerve (CN III) palsy and CRAO, which, together with the patient’s presentation, were consistent with previously documented cases of OCS. The goals of this case report are to increase awareness of this surgical outcome, identify practices that may have contributed to its development, and delineate methods to minimize its occurrence.
Informed consent regarding this research was obtained from the patient and an institutional Health Insurance Portability and Accountability Act authorization form was completed. This manuscript adheres to the applicable Enhancing the Quality and Transparency of Health Research guideline.8
Case Presentation
A 47-year-old woman underwent a left temporal craniotomy for resection of a sphenoid wing meningioma discovered during a workup for persistent headaches. She had no medical history of diabetes, hypertension, coronary artery disease, or ophthalmic disease. Two months before her scheduled surgery, the patient reported bilateral blurry vision and underwent ophthalmologic evaluation. Her intraocular pressure (IOP) was normal, and she had no pupillary or retinal disease. She showed evidence of decreased vision in her left eye, suggesting a possible mass effect from her meningioma. Subsequent imaging of the optic nerve and retina had unremarkable physiology (Figure 1). Preoperative magnetic resonance imaging (MRI) demonstrated a stable enhancing mass involving the left great sphenoid wing and left cavernous sinus(Figure 2). There was a superior mass effect on the left middle cerebral artery, but all vessels were patent without evidence of thrombosis.
The patient underwent general anesthesia with invasive hemodynamic monitoring used throughout the procedure. She was induced with fentanyl, propofol, and rocuronium; anesthesia was maintained with isoflurane and a remifentanil infusion. Hypotension was treated with phenylephrine and intravenous fluids. Intraoperative neuromonitoring with electroencephalogram (EEG) and somatosensory evoked potentials was performed. During the surgery, the patient was positioned supine in a Mayfield 3-point head fixation system. All pressure points were padded appropriately and continually checked. A standard left pterional craniotomy was performed, and the scalp was reflected anteriorly and secured using fish hooks with rubber bands. The operation did not violate the cavernous sinus or orbital compartment. There was no evidence of active bleeding upon inspection nor with the Valsalva maneuver. No changes were noted in EEG or somatosensory evoked potentials; blood pressure remained within 20 mm Hg of the patient’s baseline. She was extubated at the end of the 10-hour case and was hemodynamically stable upon transport to the surgical intensive care unit. Postoperative imaging confirmed the successful removal of the left sphenoid wing meningioma.
The patient’s postoperative examination demonstrated a 5 mm dilated, nonresponsive left pupil, though the patient did not report visual loss at that time. Defects were noted in the inferior oblique, superior, inferior, and medial rectus muscles, consistent with CN III palsy. The surgery included manipulation of CN III, which made this a possible outcome, but an alternate causative pathology like OCS was not immediately suspected. Postoperative computed tomography (CT) showed an expected pneumocephalus and left scalp swelling without evidence of mass effect or midline shift.
On the morning of postoperative Day 1, the patient reported vision loss in her left eye, while her clinical examination revealed erythema and conjunctival chemosis with left eyelid swelling. The ophthalmologic evaluation was notable for a continued leftCN III palsy with intact lateral rectus and superior oblique function, a nonreactive and dilated left eye with 3+ afferent pupillary defect by reverse (light perception), pallor throughout, a flat cherry red macula with blurred disc margins, left upper eyelid edema, and 18 mm Hg intraocular pressure bilaterally (reference range, 8 to 21 mm Hg). Fundoscopic examination showed a clear vitreous without plaques or occlusions, no perivascular sheathing, and no retinal hemorrhages. CT angiography revealed small outpouchings at the superolateral aspect of the left and right cavernous carotid, consistent with atherosclerotic calcifications. An echocardiogram revealed a Valsalva-dependent patent foramen ovale, but a venous Doppler ultrasound yielded negative results.
Repeat MRI showed denervation of the left medial rectus and minimal left-sided proptosis. A 3-month ophthalmologic follow-up revealed a persistent CN III palsy, including an afferent pupillary defect, absence of light perception in her left eye, and continued ophthalmoplegia. Repeat examination showed a left-sided 4+ afferent pupillary defect unreactive to light, 4+ pallor surrounding the optic nerve, macular atrophy, sclerotic vessels, and 17 mm Hg intraocular pressure bilaterally. The eye had diffuse atrophy of the inner retina and significant patchy atrophy of the outer retinal components without neovascularization of the iris. Postoperative retinal imaging can be seen in Figure 3. Her vision loss persisted at this encounter and has continued through subsequent follow-up examinations.
Discussion
Perioperative visual loss is a rare surgical complication, with an estimated incidence of once in every 60,000 to 125,000 cases.9 The mechanism of injury is variable and dependent upon the type of surgical intervention, with cardiac and spine surgeries carrying the greatest risk.10,11 The injury often results in either CRAO or ION, which may result in visual loss.1-3 POVL can also occur in the aftermath of rapid changes in intracranial pressure during decompressive craniotomies, though the pathophysiology in such cases is not well understood.5
Among the myriad ways in which POVL can occur, neurosurgical cases carry the unique risk of direct cranial nerve injury. Such an insult can lead to vision loss via optic nerve damage or ophthalmoplegia if damage occurs to CN III, IV, or VI. This can occur during manipulation or resection, especially if the surgical approach involves the orbital cavity or the cavernous sinus. Though neither space was entered in this patient, direct injury cannot be ruled out as the etiology for either her vision loss or persistent ophthalmoplegia. An alternate causative scenario for both symptoms involve an impaired blood supply, with the vision loss potentially occurring secondary to CRAO and the ophthalmoplegia to an alternate cause of decreased blood flow. It is unclear which of these 2 conditions occurred first or if they occurred due to the same insult, but OCS could lead to both. Though it is a less common etiology for POVL, this patient’s presentation was similar to those in previously reported cases, and OCS was identified as the likely diagnosis.
OCS is precipitated by an elevated orbital pressure, which leads to ischemia of the retina and damage to orbital contents. Though associated with retrobulbar hemorrhage and orbital trauma, another proposed mechanism for OCS is extrinsic orbital compression, resulting in increased IOP and subsequent CRAO.10 A cherry red spot is visible on fundoscopy, as only the macula with its thin retinal layer will permit the choroidal vessels to be visualized. In a separate process, the relative increase in orbital pressure may lead to impaired perfusion or damage of CN III. However, a causative relationship between the 2 may be difficult to establish. Such an injury to the oculomotor nerve is demonstrated by impaired function of the inferior oblique, superior rectus, inferior rectus, and medial rectus muscles, which may persist even after the compressive symptoms of OCS have resolved.12 Other reported symptoms of OCS include erythema, ophthalmoplegia, conjunctival chemosis, ptosis, corneal abrasion, and eyelid edema.12-15
Alternate Diagnoses
OCS is a diagnosis of exclusion, and several alternate mechanisms were considered before identifying it as the likely etiology. The patient’s preoperative imaging demonstrated a stable enhancing mass involving the left great sphenoid wing and left cavernous sinus, with displacement of the left middle cerebral artery, left cavernous internal carotid artery, and left optic canal. Dissection and removal of this tumor could have compromised the arterial or venous blood supply to the orbit, thus causing ischemia to the retina and other ocular structures. CN III was manipulated during surgery, and it may have been inadvertently damaged during exposure or resection of the tumor.
The patient’s Valsalva-dependent patent foramen ovale put her at risk of a paroxysmal embolus as an alternate explanation, particularly as a Valsalva maneuver was utilized to confirm hemostasis. The patient did not, however, demonstrate any evidence of venous thromboembolism (VTE) on ultrasound, nor did she have the common risk factors of hypertension, diabetes, or smoking history that would increase VTE risk.16Her cancer diagnosis and surgical status may have put her at risk of VTE, but she did not have any clinical or laboratory values suggestive of hypercoagulability. Had an embolism occurred, it may have compromised the orbital blood supply and led to the CRAO. A similar scenario may have occurred from an atherosclerotic plaque in either of her carotid arteries, as she did have evidence of atherosclerosis on postoperative CT angiography. However, atherosclerosis as a risk factor for POVL appears to be related more to its impact upon impaired blood supply rather than as an embolic source. The patient did not have any significant intraoperative hypotensive episodes, making ION in the setting of atherosclerosis and hypotension a less likely etiology.17
This patient differed from other reported OCS cases. She was never placed in a prone or jackknife position, nor was she agitated or straining for a sustained period. These factors, along with the fact that the orbital compartment was not entered, decreased the likelihood of intraorbital hemorrhage and other intrinsic causes of elevated IOP.12 Additionally, the presentation of our patient’s vision loss was delayed compared with other cases, despite clinicians observing a dilated left pupil and CN III palsy on examination immediately after surgery.14 It is significant to note that OCS may not demonstrate a significant increase in IOP once the source of compression is removed, which may explain the absence of proptosis on her postoperative examination.
The diagnosis of OCS was primarily implicated by the positioning of the myocutaneous flap during the pterional approach to craniotomy. It was retracted anteriorly and superiorly, ultimately resting over her left orbit for most of the 10-hour surgery. Kim and colleagues found that myocutaneous flaps may increase IOP as much as 17.5 mm Hg if improperly positioned, providing an unrecognized source of compression and increasing the risk of damage to orbital contents. According to their review, elevated IOP > 40 mm Hg, particularly over several hours, can compromise blood flow to the optic nerve and increase the risk for POVL.18 The flap was secured using fish hooks and rubber bands. However, it is suspected that the orbital rim did not fully support its pressure, thereby resting to some degree directly on the globe for an extended period and compromising the orbital blood supply. There are no current methods for measuring intraoperative IOP, though surrogate markers are under investigation and may yield clinical utility.18 The myocutaneous flap was created and positioned by the surgeons, but it may be that increased vigilance and communication from the anesthesia and nursing teams could have prevented it from remaining in an improper position.
Conclusions
Despite having few reported cases, OCS must be considered in neurosurgical patients with ophthalmoplegia and CRAO on postoperative examinations. Myocutaneous flaps that are retracted across the orbit can lead to significant elevations in IOP, leading to vision loss, which likely occurred with the patient in this case. Though protecting neurovascular structures is within the purview of the surgeon, all members of the intraoperative team should assist with ensuring proper flap positioning. These measures can help ensure adequate blood flow to the ophthalmic artery, decrease the likelihood of elevated IOP due to extrinsic compression, and help prevent the development of POVL and OCS in these patients.
Perioperative visual loss (POVL) is a well-documented yet uncommon complication of nonocular surgery. Patients undergoing cardiac and spinal surgery are at the greatest risk, though POVL may occur during other neurosurgical and vascular procedures as well. The most common causes of POVL are central retinal artery occlusion (CRAO) and ischemic optic neuropathy (ION),1-3 though cases of orbital compartment syndrome (OCS) have also been reported.4-7
We describe a case of POVL during a temporal meningioma excision using the pterional approach. Though the etiology is not fully understood, the patient’s clinical course was complicated by a third cranial nerve (CN III) palsy and CRAO, which, together with the patient’s presentation, were consistent with previously documented cases of OCS. The goals of this case report are to increase awareness of this surgical outcome, identify practices that may have contributed to its development, and delineate methods to minimize its occurrence.
Informed consent regarding this research was obtained from the patient and an institutional Health Insurance Portability and Accountability Act authorization form was completed. This manuscript adheres to the applicable Enhancing the Quality and Transparency of Health Research guideline.8
Case Presentation
A 47-year-old woman underwent a left temporal craniotomy for resection of a sphenoid wing meningioma discovered during a workup for persistent headaches. She had no medical history of diabetes, hypertension, coronary artery disease, or ophthalmic disease. Two months before her scheduled surgery, the patient reported bilateral blurry vision and underwent ophthalmologic evaluation. Her intraocular pressure (IOP) was normal, and she had no pupillary or retinal disease. She showed evidence of decreased vision in her left eye, suggesting a possible mass effect from her meningioma. Subsequent imaging of the optic nerve and retina had unremarkable physiology (Figure 1). Preoperative magnetic resonance imaging (MRI) demonstrated a stable enhancing mass involving the left great sphenoid wing and left cavernous sinus(Figure 2). There was a superior mass effect on the left middle cerebral artery, but all vessels were patent without evidence of thrombosis.
The patient underwent general anesthesia with invasive hemodynamic monitoring used throughout the procedure. She was induced with fentanyl, propofol, and rocuronium; anesthesia was maintained with isoflurane and a remifentanil infusion. Hypotension was treated with phenylephrine and intravenous fluids. Intraoperative neuromonitoring with electroencephalogram (EEG) and somatosensory evoked potentials was performed. During the surgery, the patient was positioned supine in a Mayfield 3-point head fixation system. All pressure points were padded appropriately and continually checked. A standard left pterional craniotomy was performed, and the scalp was reflected anteriorly and secured using fish hooks with rubber bands. The operation did not violate the cavernous sinus or orbital compartment. There was no evidence of active bleeding upon inspection nor with the Valsalva maneuver. No changes were noted in EEG or somatosensory evoked potentials; blood pressure remained within 20 mm Hg of the patient’s baseline. She was extubated at the end of the 10-hour case and was hemodynamically stable upon transport to the surgical intensive care unit. Postoperative imaging confirmed the successful removal of the left sphenoid wing meningioma.
The patient’s postoperative examination demonstrated a 5 mm dilated, nonresponsive left pupil, though the patient did not report visual loss at that time. Defects were noted in the inferior oblique, superior, inferior, and medial rectus muscles, consistent with CN III palsy. The surgery included manipulation of CN III, which made this a possible outcome, but an alternate causative pathology like OCS was not immediately suspected. Postoperative computed tomography (CT) showed an expected pneumocephalus and left scalp swelling without evidence of mass effect or midline shift.
On the morning of postoperative Day 1, the patient reported vision loss in her left eye, while her clinical examination revealed erythema and conjunctival chemosis with left eyelid swelling. The ophthalmologic evaluation was notable for a continued leftCN III palsy with intact lateral rectus and superior oblique function, a nonreactive and dilated left eye with 3+ afferent pupillary defect by reverse (light perception), pallor throughout, a flat cherry red macula with blurred disc margins, left upper eyelid edema, and 18 mm Hg intraocular pressure bilaterally (reference range, 8 to 21 mm Hg). Fundoscopic examination showed a clear vitreous without plaques or occlusions, no perivascular sheathing, and no retinal hemorrhages. CT angiography revealed small outpouchings at the superolateral aspect of the left and right cavernous carotid, consistent with atherosclerotic calcifications. An echocardiogram revealed a Valsalva-dependent patent foramen ovale, but a venous Doppler ultrasound yielded negative results.
Repeat MRI showed denervation of the left medial rectus and minimal left-sided proptosis. A 3-month ophthalmologic follow-up revealed a persistent CN III palsy, including an afferent pupillary defect, absence of light perception in her left eye, and continued ophthalmoplegia. Repeat examination showed a left-sided 4+ afferent pupillary defect unreactive to light, 4+ pallor surrounding the optic nerve, macular atrophy, sclerotic vessels, and 17 mm Hg intraocular pressure bilaterally. The eye had diffuse atrophy of the inner retina and significant patchy atrophy of the outer retinal components without neovascularization of the iris. Postoperative retinal imaging can be seen in Figure 3. Her vision loss persisted at this encounter and has continued through subsequent follow-up examinations.
Discussion
Perioperative visual loss is a rare surgical complication, with an estimated incidence of once in every 60,000 to 125,000 cases.9 The mechanism of injury is variable and dependent upon the type of surgical intervention, with cardiac and spine surgeries carrying the greatest risk.10,11 The injury often results in either CRAO or ION, which may result in visual loss.1-3 POVL can also occur in the aftermath of rapid changes in intracranial pressure during decompressive craniotomies, though the pathophysiology in such cases is not well understood.5
Among the myriad ways in which POVL can occur, neurosurgical cases carry the unique risk of direct cranial nerve injury. Such an insult can lead to vision loss via optic nerve damage or ophthalmoplegia if damage occurs to CN III, IV, or VI. This can occur during manipulation or resection, especially if the surgical approach involves the orbital cavity or the cavernous sinus. Though neither space was entered in this patient, direct injury cannot be ruled out as the etiology for either her vision loss or persistent ophthalmoplegia. An alternate causative scenario for both symptoms involve an impaired blood supply, with the vision loss potentially occurring secondary to CRAO and the ophthalmoplegia to an alternate cause of decreased blood flow. It is unclear which of these 2 conditions occurred first or if they occurred due to the same insult, but OCS could lead to both. Though it is a less common etiology for POVL, this patient’s presentation was similar to those in previously reported cases, and OCS was identified as the likely diagnosis.
OCS is precipitated by an elevated orbital pressure, which leads to ischemia of the retina and damage to orbital contents. Though associated with retrobulbar hemorrhage and orbital trauma, another proposed mechanism for OCS is extrinsic orbital compression, resulting in increased IOP and subsequent CRAO.10 A cherry red spot is visible on fundoscopy, as only the macula with its thin retinal layer will permit the choroidal vessels to be visualized. In a separate process, the relative increase in orbital pressure may lead to impaired perfusion or damage of CN III. However, a causative relationship between the 2 may be difficult to establish. Such an injury to the oculomotor nerve is demonstrated by impaired function of the inferior oblique, superior rectus, inferior rectus, and medial rectus muscles, which may persist even after the compressive symptoms of OCS have resolved.12 Other reported symptoms of OCS include erythema, ophthalmoplegia, conjunctival chemosis, ptosis, corneal abrasion, and eyelid edema.12-15
Alternate Diagnoses
OCS is a diagnosis of exclusion, and several alternate mechanisms were considered before identifying it as the likely etiology. The patient’s preoperative imaging demonstrated a stable enhancing mass involving the left great sphenoid wing and left cavernous sinus, with displacement of the left middle cerebral artery, left cavernous internal carotid artery, and left optic canal. Dissection and removal of this tumor could have compromised the arterial or venous blood supply to the orbit, thus causing ischemia to the retina and other ocular structures. CN III was manipulated during surgery, and it may have been inadvertently damaged during exposure or resection of the tumor.
The patient’s Valsalva-dependent patent foramen ovale put her at risk of a paroxysmal embolus as an alternate explanation, particularly as a Valsalva maneuver was utilized to confirm hemostasis. The patient did not, however, demonstrate any evidence of venous thromboembolism (VTE) on ultrasound, nor did she have the common risk factors of hypertension, diabetes, or smoking history that would increase VTE risk.16Her cancer diagnosis and surgical status may have put her at risk of VTE, but she did not have any clinical or laboratory values suggestive of hypercoagulability. Had an embolism occurred, it may have compromised the orbital blood supply and led to the CRAO. A similar scenario may have occurred from an atherosclerotic plaque in either of her carotid arteries, as she did have evidence of atherosclerosis on postoperative CT angiography. However, atherosclerosis as a risk factor for POVL appears to be related more to its impact upon impaired blood supply rather than as an embolic source. The patient did not have any significant intraoperative hypotensive episodes, making ION in the setting of atherosclerosis and hypotension a less likely etiology.17
This patient differed from other reported OCS cases. She was never placed in a prone or jackknife position, nor was she agitated or straining for a sustained period. These factors, along with the fact that the orbital compartment was not entered, decreased the likelihood of intraorbital hemorrhage and other intrinsic causes of elevated IOP.12 Additionally, the presentation of our patient’s vision loss was delayed compared with other cases, despite clinicians observing a dilated left pupil and CN III palsy on examination immediately after surgery.14 It is significant to note that OCS may not demonstrate a significant increase in IOP once the source of compression is removed, which may explain the absence of proptosis on her postoperative examination.
The diagnosis of OCS was primarily implicated by the positioning of the myocutaneous flap during the pterional approach to craniotomy. It was retracted anteriorly and superiorly, ultimately resting over her left orbit for most of the 10-hour surgery. Kim and colleagues found that myocutaneous flaps may increase IOP as much as 17.5 mm Hg if improperly positioned, providing an unrecognized source of compression and increasing the risk of damage to orbital contents. According to their review, elevated IOP > 40 mm Hg, particularly over several hours, can compromise blood flow to the optic nerve and increase the risk for POVL.18 The flap was secured using fish hooks and rubber bands. However, it is suspected that the orbital rim did not fully support its pressure, thereby resting to some degree directly on the globe for an extended period and compromising the orbital blood supply. There are no current methods for measuring intraoperative IOP, though surrogate markers are under investigation and may yield clinical utility.18 The myocutaneous flap was created and positioned by the surgeons, but it may be that increased vigilance and communication from the anesthesia and nursing teams could have prevented it from remaining in an improper position.
Conclusions
Despite having few reported cases, OCS must be considered in neurosurgical patients with ophthalmoplegia and CRAO on postoperative examinations. Myocutaneous flaps that are retracted across the orbit can lead to significant elevations in IOP, leading to vision loss, which likely occurred with the patient in this case. Though protecting neurovascular structures is within the purview of the surgeon, all members of the intraoperative team should assist with ensuring proper flap positioning. These measures can help ensure adequate blood flow to the ophthalmic artery, decrease the likelihood of elevated IOP due to extrinsic compression, and help prevent the development of POVL and OCS in these patients.
1. Biousse V, Nahab F, Newman NJ. Management of acute retinal ischemia: follow the guidelines! Ophthalmology. 2018;125(10):1597-1607. doi:10.1016/j.ophtha.2018.03.054
2. Biousse V, Newman NJ. Ischemic optic neuropathies. N Engl J Med. 2015;372(25):2428-2436. doi:10.1056/NEJMra1413352
3. Shah SH, Chen YF, Moss HE, Rubin DS, Joslin CE, Roth S. Predicting risk of perioperative ischemic optic neuropathy in spine fusion surgery: a cohort study using the national inpatient sample. Anesth Analg. 2020;130(4):967-974. doi:10.1213/ANE.0000000000004383
4. Habets JGV, Haeren RHL, Lie SAN, Bauer NJC, Dings JTA. Acute monocular blindness due to orbital compartment syndrome following pterional craniotomy. World Neurosurg. 2018;114:72-75. doi:10.1016/j.wneu.2018.03.013
5. Vahedi P, Meshkini A, Mohajernezhadfard Z, Tubbs RS. Post-craniotomy blindness in the supine position: Unlikely or ignored? Asian J Neurosurg. 2013;8(1):36-41. doi:10.4103/1793-5482.110278
6. Kang S, Yang Y, Kim T, Kim J. Sudden unilateral blindness after intracranial aneurysm surgery. Acta Neurochir (Wien). 1997;139(3):221-226. doi:10.1007/BF01844755
7. Zimmerman CF, Van Patten PD, Golnik KC, Kopitnik TA Jr, Anand R. Orbital infarction syndrome after surgery for intracranial aneurysms. Ophthalmology. 1995;102(4):594-598. doi:10.1016/s0161-6420(95)30979-7
8. Gagnier JJ, Kienle G, Altman DG, et al. The CARE guidelines: consensus-based clinical case reporting guideline development. BMJ Case Rep. 23;2013:bcr2013201554. doi:10.1136/bcr-2013-201554
9. Raphael J, Moss HE, Roth S. Perioperative visual loss in cardiac surgery. J Cardiothorac Vasc Anesth. 2019;33(5):1420-429. doi:10.1053/j.jvca.2018.11.035
10. Kansakar P, Sundar G. Vision loss associated with orbital surgery - a major review. Orbit. 2020;39(3):197-208. doi:10.1080/01676830.2019.1658790
11. Dohlman JC, Yoon MK. Principles of protection of the eye and vision in orbital surgery. J Neurol Surg B Skull Base. 2020;81(4):381-384. doi:10.1055/s-0040-1714077
12. Pahl FH, de Oliveira MF, Dal Col Lúcio JE, Souza E Castro EF. Orbital compartment syndrome after frontotemporal craniotomy: case report and review of literature. World Neurosurg. 2018;109:218-221. doi:10.1016/j.wneu.2017.09.167
13. Grossman W, Ward WT. Central retinal artery occlusion after scoliosis surgery with a horseshoe headrest. Case report and literature review. Spine (Phila Pa 1976). 1993;18(9):1226-1228. doi:10.1097/00007632-199307000-00017
14. Newman NJ. Perioperative visual loss after nonocular surgeries. Am J Ophthalmol. 2008;145(4):604-610. doi:10.1016/j.ajo.2007.09.016
15. Roth S, Tung A, Ksiazek S. Visual loss in a prone-positioned spine surgery patient with the head on a foam headrest and goggles covering the eyes: an old complication with a new mechanism. Anesth Analg. 2007;104(5):1185-1187. doi:10.1213/01.ane.0000264319.57758.55
16. Katz DA, Karlin LI. Visual field defect after posterior spine fusion. Spine (Phila Pa 1976). 2005;30(3):E83-E85. doi:10.1097/01.brs.0000152169.48117.c7
17. Nickels TJ, Manlapaz MR, Farag E. Perioperative visual loss after spine surgery. World J Orthop. 2014;5(2):100-106. Published 2014 April 18. doi:10.5312/wjo.v5.i2.100
18. Kim TS, Hur JW, Park DH, et al. Extraocular ressure measurements to avoid orbital compartment syndrome in aneurysm surgery. World Neurosurg. 2018;118:e601-e609. doi:10.1016/j.wneu.2018.06.248
1. Biousse V, Nahab F, Newman NJ. Management of acute retinal ischemia: follow the guidelines! Ophthalmology. 2018;125(10):1597-1607. doi:10.1016/j.ophtha.2018.03.054
2. Biousse V, Newman NJ. Ischemic optic neuropathies. N Engl J Med. 2015;372(25):2428-2436. doi:10.1056/NEJMra1413352
3. Shah SH, Chen YF, Moss HE, Rubin DS, Joslin CE, Roth S. Predicting risk of perioperative ischemic optic neuropathy in spine fusion surgery: a cohort study using the national inpatient sample. Anesth Analg. 2020;130(4):967-974. doi:10.1213/ANE.0000000000004383
4. Habets JGV, Haeren RHL, Lie SAN, Bauer NJC, Dings JTA. Acute monocular blindness due to orbital compartment syndrome following pterional craniotomy. World Neurosurg. 2018;114:72-75. doi:10.1016/j.wneu.2018.03.013
5. Vahedi P, Meshkini A, Mohajernezhadfard Z, Tubbs RS. Post-craniotomy blindness in the supine position: Unlikely or ignored? Asian J Neurosurg. 2013;8(1):36-41. doi:10.4103/1793-5482.110278
6. Kang S, Yang Y, Kim T, Kim J. Sudden unilateral blindness after intracranial aneurysm surgery. Acta Neurochir (Wien). 1997;139(3):221-226. doi:10.1007/BF01844755
7. Zimmerman CF, Van Patten PD, Golnik KC, Kopitnik TA Jr, Anand R. Orbital infarction syndrome after surgery for intracranial aneurysms. Ophthalmology. 1995;102(4):594-598. doi:10.1016/s0161-6420(95)30979-7
8. Gagnier JJ, Kienle G, Altman DG, et al. The CARE guidelines: consensus-based clinical case reporting guideline development. BMJ Case Rep. 23;2013:bcr2013201554. doi:10.1136/bcr-2013-201554
9. Raphael J, Moss HE, Roth S. Perioperative visual loss in cardiac surgery. J Cardiothorac Vasc Anesth. 2019;33(5):1420-429. doi:10.1053/j.jvca.2018.11.035
10. Kansakar P, Sundar G. Vision loss associated with orbital surgery - a major review. Orbit. 2020;39(3):197-208. doi:10.1080/01676830.2019.1658790
11. Dohlman JC, Yoon MK. Principles of protection of the eye and vision in orbital surgery. J Neurol Surg B Skull Base. 2020;81(4):381-384. doi:10.1055/s-0040-1714077
12. Pahl FH, de Oliveira MF, Dal Col Lúcio JE, Souza E Castro EF. Orbital compartment syndrome after frontotemporal craniotomy: case report and review of literature. World Neurosurg. 2018;109:218-221. doi:10.1016/j.wneu.2017.09.167
13. Grossman W, Ward WT. Central retinal artery occlusion after scoliosis surgery with a horseshoe headrest. Case report and literature review. Spine (Phila Pa 1976). 1993;18(9):1226-1228. doi:10.1097/00007632-199307000-00017
14. Newman NJ. Perioperative visual loss after nonocular surgeries. Am J Ophthalmol. 2008;145(4):604-610. doi:10.1016/j.ajo.2007.09.016
15. Roth S, Tung A, Ksiazek S. Visual loss in a prone-positioned spine surgery patient with the head on a foam headrest and goggles covering the eyes: an old complication with a new mechanism. Anesth Analg. 2007;104(5):1185-1187. doi:10.1213/01.ane.0000264319.57758.55
16. Katz DA, Karlin LI. Visual field defect after posterior spine fusion. Spine (Phila Pa 1976). 2005;30(3):E83-E85. doi:10.1097/01.brs.0000152169.48117.c7
17. Nickels TJ, Manlapaz MR, Farag E. Perioperative visual loss after spine surgery. World J Orthop. 2014;5(2):100-106. Published 2014 April 18. doi:10.5312/wjo.v5.i2.100
18. Kim TS, Hur JW, Park DH, et al. Extraocular ressure measurements to avoid orbital compartment syndrome in aneurysm surgery. World Neurosurg. 2018;118:e601-e609. doi:10.1016/j.wneu.2018.06.248
Act Fast With Traction Alopecia to Avoid Permanent Hair Loss
Traction alopecia (TA) is a common type of alopecia that ultimately can result in permanent hair loss. It often is caused or worsened by repetitive and prolonged hairstyling practices such as tight ponytails, braids, or locs, or use of wigs or weaves.1 Use of headwear, as in certain religious or ethnic groups, also can be contributory.2 Individuals participating in or training for occupations involving military service or ballet are at risk for TA due to hairstyling-specific policies. Early stages of TA are reversible with proper treatment and avoidance of exacerbating factors, emphasizing the importance of prompt recognition.3
Epidemiology
Data on the true prevalence of TA are lacking. It can occur in individuals of any race or any hair type. However, it is most common in women of African descent, affecting approximately one-third of this population.4 Other commonly affected groups include ballerinas and active-duty service members due to tight ponytails and buns, as well as the Sikh population due to the use of turbans as a part of their religious practice.2,5,6
Traction alopecia also impacts children, particularly those of African descent. A 2007 study of schoolchildren in South Africa determined that more than 17% of young African girls had evidence of TA—even some as young as 6 years of age.7
Traction alopecia can be caused or exacerbated by the use of hair clips and bobby pins that aid holding styles in place.8 Hair shaft morphology may contribute to the risk for TA, with more tightly coiled hair types being more susceptible.8 Variables such as use of chemical relaxers also increase the risk for disease, especially when combined with high-tension styling methods such as braids.9
Key clinical features
Patients with TA clinically present with hair loss and breakage in areas with tension, most commonly the marginal areas of the scalp as well as the frontal hairline and temporal scalp. Hair loss can result in a “fringe sign,” in which a patient may have preservation of a thin line of hairs at the frontal aspect of the hairline with a band of hair loss behind.10 This presentation may be used to differentiate TA from other forms of alopecia, including frontal fibrosing alopecia and female pattern hair loss. When the hair loss is not marginal, it may mimic other forms of patchy hair loss including alopecia areata and trichotillomania. Other clinical findings in TA may include broken hairs, pustules, and follicular papules.10 Patients also may describe symptoms such as scalp tenderness with specific hairstyles or headaches,11 or they may be completely asymptomatic.
Trichoscopy can be helpful in guiding diagnosis and treatment. Patients with TA often have perifollicular erythema and hair casts (cylindrical structures that encircle the proximal hair shafts) in the earlier stages of the disease, with eventual loss of follicular ostia in the later stages.10,12 Hair casts also may indicate ongoing traction.12 The flambeau sign—white tracks seen on trichoscopy in the direction the hair is pulled—resembles a lit torch.13
Worth noting
Early-stage TA can be reversed by avoiding hair tension. However, patients may not be amenable to this due to personal hairstyling preferences, job duties, or religious practices. Treatment with topical or intralesional steroids or even oral antibiotics such as doxycycline for its anti-inflammatory ability may result in regrowth of lost hair if the follicles are not permanently lost and exacerbating factors are avoided.3,14 Both topical and oral minoxidil have been used with success, with minoxidil thought to increase hair density by extending the anagen (growth) phase of hair follicles.3,15 Culturally sensitive patient counseling on the condition and potential exacerbating factors is critical.16
At later stages of the disease—after loss of follicular ostia has occurred—surgical interventions should be considered,17 such as hair transplantation, which can be successful but remains a technical challenge due to variability in hair shaft curvature.18 Additionally, the cost of the procedure can limit use, and some patients may not be optimal candidates due to the extent of their hair loss. Traction alopecia may not be the only hair loss condition present. Examining the scalp is important even if the chief area of concern is the marginal scalp.
Health disparity highlight
Prevention, early identification, and treatment initiated in a timely fashion are crucial to prevent permanent hair loss. There are added societal and cultural pressures that impact hairstyle and hair care practices, especially for those with tightly coiled hair.19 Historically, tightly coiled hair has been unfairly viewed as “unprofessional,” “unkempt,” and a challenge to “manage” by some. Thus, heat, chemical relaxers, and tight hairstyles holding hair in one position have been used to straighten the hair permanently or temporarily or to keep it maintained in a style that did not necessitate excessive manipulation—often contributing to further tension on the hair.
Military service branches have evaluated and changed some hair-related policies to reflect the diverse hair types of military personnel.20 The CROWN Act (www.thecrownact.com/about)—“Creating a Respectful and Open World for Natural Hair”—is a model law passed by 26 states that prohibits race-based hair discrimination, which is the denial of employment and educational opportunities because of hair texture. Although the law has not been passed in every state, it may help individuals with tightly coiled hair to embrace natural hairstyles. However, even hairstyles with one’s own natural curl pattern can contribute to tension and thus potential development of TA.
1. Larrondo J, McMichael AJ. Traction alopecia. JAMA Dermatol. 2023;159:676. doi:10.1001/jamadermatol.2022.6298
2. James J, Saladi RN, Fox JL. Traction alopecia in Sikh male patients. J Am Board Fam Med. 2007;20:497-498. doi:10.3122/jabfm.2007.05.070076
3. Callender VD, McMichael AJ, Cohen GF. Medical and surgical therapies for alopecias in black women. Dermatol Ther. 2004;17:164-176.
4. Loussouarn G, El Rawadi C, Genain G. Diversity of hair growth profiles. Int J Dermatol. 2005;44(suppl 1):6-9.
5. Samrao AChen CZedek Det al. Traction alopecia in a ballerina: clinicopathologic features. Arch Dermatol. 2010;146:918-935. doi:10.1001/archdermatol.2010.183
6. Korona-Bailey J, Banaag A, Nguyen DR, et al. Free the bun: prevalence of alopecia among active duty service women, fiscal years 2010-2019. Mil Med. 2023;188:e492-e496. doi:10.1093/milmed/usab274
7. Khumalo NP, Jessop S, Gumedze F, et al. Hairdressing is associated with scalp disease in African schoolchildren. Br J Dermatol. 2007;157:106-110. doi:10.1111/j.1365-2133.2007.07987.x
8. Billero V, Miteva M. Traction alopecia: the root of the problem. Clin Cosmet Investig Dermatol. 2018;11:149-159. doi:10.2147/CCID.S137296
9. Haskin A, Aguh C. All hairstyles are not created equal: what the dermatologist needs to know about black hairstyling practices and the risk of traction alopecia (TA). J Am Acad Dermatol. 2016;75:606-611. doi:10.1016/j.jaad.2016.02.1162
10. Samrao A, Price VH, Zedek D, et al. The “fringe sign”—a useful clinical finding in traction alopecia of the marginal hair line. Dermatol Online J. 2011;17:1.
11. Kararizou E, Bougea AM, Giotopoulou D, et al. An update on the less-known group of other primary headaches—a review. Eur Neurol Rev. 2014;9:71-77. doi:10.17925/ENR.2014.09.01.71
12. Tosti A, Miteva M, Torres F, et al. Hair casts are a dermoscopic clue for the diagnosis of traction alopecia. Br J Dermatol. 2010;163:1353-1355.
13. Agrawal S, Daruwalla SB, Dhurat RS. The flambeau sign—a new dermoscopy finding in a case of marginal traction alopecia. Australas J Dermatol. 2020;61:49-50. doi:10.1111/ajd.13187
14. Lawson CN, Hollinger J, Sethi S, et al. Updates in the understanding and treatments of skin & hair disorders in women of color. Int J Womens Dermatol. 2017;3:S21-S37.
15. Awad A, Chim I, Sharma P, et al. Low-dose oral minoxidil improves hair density in traction alopecia. J Am Acad Dermatol. 2023;89:157-159. doi:10.1016/j.jaad.2023.02.024
16. Grayson C, Heath CR. Counseling about traction alopecia: a “compliment, discuss, and suggest” method. Cutis. 2021;108:20-22.
17. Ozçelik D. Extensive traction alopecia attributable to ponytail hairstyle and its treatment with hair transplantation. Aesthetic Plast Surg. 2005;29:325-327. doi:10.1007/s00266-005-0004-5
18. Singh MK, Avram MR. Technical considerations for follicular unit extraction in African-American hair. Dermatol Surg. 2013;39:1282-1284. doi:10.1111/dsu.12229
19. Jones NL, Heath CR. Hair at the intersection of dermatology and anthropology: a conversation on race and relationships. Pediatr Dermatol. 2021;38(suppl 2):158-160.
20. Franklin JMM, Wohltmann WE, Wong EB. From buns to braids and ponytails: entering a new era of female military hair-grooming standards. Cutis. 2021;108:31-35. doi:10.12788/cutis.0296
Traction alopecia (TA) is a common type of alopecia that ultimately can result in permanent hair loss. It often is caused or worsened by repetitive and prolonged hairstyling practices such as tight ponytails, braids, or locs, or use of wigs or weaves.1 Use of headwear, as in certain religious or ethnic groups, also can be contributory.2 Individuals participating in or training for occupations involving military service or ballet are at risk for TA due to hairstyling-specific policies. Early stages of TA are reversible with proper treatment and avoidance of exacerbating factors, emphasizing the importance of prompt recognition.3
Epidemiology
Data on the true prevalence of TA are lacking. It can occur in individuals of any race or any hair type. However, it is most common in women of African descent, affecting approximately one-third of this population.4 Other commonly affected groups include ballerinas and active-duty service members due to tight ponytails and buns, as well as the Sikh population due to the use of turbans as a part of their religious practice.2,5,6
Traction alopecia also impacts children, particularly those of African descent. A 2007 study of schoolchildren in South Africa determined that more than 17% of young African girls had evidence of TA—even some as young as 6 years of age.7
Traction alopecia can be caused or exacerbated by the use of hair clips and bobby pins that aid holding styles in place.8 Hair shaft morphology may contribute to the risk for TA, with more tightly coiled hair types being more susceptible.8 Variables such as use of chemical relaxers also increase the risk for disease, especially when combined with high-tension styling methods such as braids.9
Key clinical features
Patients with TA clinically present with hair loss and breakage in areas with tension, most commonly the marginal areas of the scalp as well as the frontal hairline and temporal scalp. Hair loss can result in a “fringe sign,” in which a patient may have preservation of a thin line of hairs at the frontal aspect of the hairline with a band of hair loss behind.10 This presentation may be used to differentiate TA from other forms of alopecia, including frontal fibrosing alopecia and female pattern hair loss. When the hair loss is not marginal, it may mimic other forms of patchy hair loss including alopecia areata and trichotillomania. Other clinical findings in TA may include broken hairs, pustules, and follicular papules.10 Patients also may describe symptoms such as scalp tenderness with specific hairstyles or headaches,11 or they may be completely asymptomatic.
Trichoscopy can be helpful in guiding diagnosis and treatment. Patients with TA often have perifollicular erythema and hair casts (cylindrical structures that encircle the proximal hair shafts) in the earlier stages of the disease, with eventual loss of follicular ostia in the later stages.10,12 Hair casts also may indicate ongoing traction.12 The flambeau sign—white tracks seen on trichoscopy in the direction the hair is pulled—resembles a lit torch.13
Worth noting
Early-stage TA can be reversed by avoiding hair tension. However, patients may not be amenable to this due to personal hairstyling preferences, job duties, or religious practices. Treatment with topical or intralesional steroids or even oral antibiotics such as doxycycline for its anti-inflammatory ability may result in regrowth of lost hair if the follicles are not permanently lost and exacerbating factors are avoided.3,14 Both topical and oral minoxidil have been used with success, with minoxidil thought to increase hair density by extending the anagen (growth) phase of hair follicles.3,15 Culturally sensitive patient counseling on the condition and potential exacerbating factors is critical.16
At later stages of the disease—after loss of follicular ostia has occurred—surgical interventions should be considered,17 such as hair transplantation, which can be successful but remains a technical challenge due to variability in hair shaft curvature.18 Additionally, the cost of the procedure can limit use, and some patients may not be optimal candidates due to the extent of their hair loss. Traction alopecia may not be the only hair loss condition present. Examining the scalp is important even if the chief area of concern is the marginal scalp.
Health disparity highlight
Prevention, early identification, and treatment initiated in a timely fashion are crucial to prevent permanent hair loss. There are added societal and cultural pressures that impact hairstyle and hair care practices, especially for those with tightly coiled hair.19 Historically, tightly coiled hair has been unfairly viewed as “unprofessional,” “unkempt,” and a challenge to “manage” by some. Thus, heat, chemical relaxers, and tight hairstyles holding hair in one position have been used to straighten the hair permanently or temporarily or to keep it maintained in a style that did not necessitate excessive manipulation—often contributing to further tension on the hair.
Military service branches have evaluated and changed some hair-related policies to reflect the diverse hair types of military personnel.20 The CROWN Act (www.thecrownact.com/about)—“Creating a Respectful and Open World for Natural Hair”—is a model law passed by 26 states that prohibits race-based hair discrimination, which is the denial of employment and educational opportunities because of hair texture. Although the law has not been passed in every state, it may help individuals with tightly coiled hair to embrace natural hairstyles. However, even hairstyles with one’s own natural curl pattern can contribute to tension and thus potential development of TA.
Traction alopecia (TA) is a common type of alopecia that ultimately can result in permanent hair loss. It often is caused or worsened by repetitive and prolonged hairstyling practices such as tight ponytails, braids, or locs, or use of wigs or weaves.1 Use of headwear, as in certain religious or ethnic groups, also can be contributory.2 Individuals participating in or training for occupations involving military service or ballet are at risk for TA due to hairstyling-specific policies. Early stages of TA are reversible with proper treatment and avoidance of exacerbating factors, emphasizing the importance of prompt recognition.3
Epidemiology
Data on the true prevalence of TA are lacking. It can occur in individuals of any race or any hair type. However, it is most common in women of African descent, affecting approximately one-third of this population.4 Other commonly affected groups include ballerinas and active-duty service members due to tight ponytails and buns, as well as the Sikh population due to the use of turbans as a part of their religious practice.2,5,6
Traction alopecia also impacts children, particularly those of African descent. A 2007 study of schoolchildren in South Africa determined that more than 17% of young African girls had evidence of TA—even some as young as 6 years of age.7
Traction alopecia can be caused or exacerbated by the use of hair clips and bobby pins that aid holding styles in place.8 Hair shaft morphology may contribute to the risk for TA, with more tightly coiled hair types being more susceptible.8 Variables such as use of chemical relaxers also increase the risk for disease, especially when combined with high-tension styling methods such as braids.9
Key clinical features
Patients with TA clinically present with hair loss and breakage in areas with tension, most commonly the marginal areas of the scalp as well as the frontal hairline and temporal scalp. Hair loss can result in a “fringe sign,” in which a patient may have preservation of a thin line of hairs at the frontal aspect of the hairline with a band of hair loss behind.10 This presentation may be used to differentiate TA from other forms of alopecia, including frontal fibrosing alopecia and female pattern hair loss. When the hair loss is not marginal, it may mimic other forms of patchy hair loss including alopecia areata and trichotillomania. Other clinical findings in TA may include broken hairs, pustules, and follicular papules.10 Patients also may describe symptoms such as scalp tenderness with specific hairstyles or headaches,11 or they may be completely asymptomatic.
Trichoscopy can be helpful in guiding diagnosis and treatment. Patients with TA often have perifollicular erythema and hair casts (cylindrical structures that encircle the proximal hair shafts) in the earlier stages of the disease, with eventual loss of follicular ostia in the later stages.10,12 Hair casts also may indicate ongoing traction.12 The flambeau sign—white tracks seen on trichoscopy in the direction the hair is pulled—resembles a lit torch.13
Worth noting
Early-stage TA can be reversed by avoiding hair tension. However, patients may not be amenable to this due to personal hairstyling preferences, job duties, or religious practices. Treatment with topical or intralesional steroids or even oral antibiotics such as doxycycline for its anti-inflammatory ability may result in regrowth of lost hair if the follicles are not permanently lost and exacerbating factors are avoided.3,14 Both topical and oral minoxidil have been used with success, with minoxidil thought to increase hair density by extending the anagen (growth) phase of hair follicles.3,15 Culturally sensitive patient counseling on the condition and potential exacerbating factors is critical.16
At later stages of the disease—after loss of follicular ostia has occurred—surgical interventions should be considered,17 such as hair transplantation, which can be successful but remains a technical challenge due to variability in hair shaft curvature.18 Additionally, the cost of the procedure can limit use, and some patients may not be optimal candidates due to the extent of their hair loss. Traction alopecia may not be the only hair loss condition present. Examining the scalp is important even if the chief area of concern is the marginal scalp.
Health disparity highlight
Prevention, early identification, and treatment initiated in a timely fashion are crucial to prevent permanent hair loss. There are added societal and cultural pressures that impact hairstyle and hair care practices, especially for those with tightly coiled hair.19 Historically, tightly coiled hair has been unfairly viewed as “unprofessional,” “unkempt,” and a challenge to “manage” by some. Thus, heat, chemical relaxers, and tight hairstyles holding hair in one position have been used to straighten the hair permanently or temporarily or to keep it maintained in a style that did not necessitate excessive manipulation—often contributing to further tension on the hair.
Military service branches have evaluated and changed some hair-related policies to reflect the diverse hair types of military personnel.20 The CROWN Act (www.thecrownact.com/about)—“Creating a Respectful and Open World for Natural Hair”—is a model law passed by 26 states that prohibits race-based hair discrimination, which is the denial of employment and educational opportunities because of hair texture. Although the law has not been passed in every state, it may help individuals with tightly coiled hair to embrace natural hairstyles. However, even hairstyles with one’s own natural curl pattern can contribute to tension and thus potential development of TA.
1. Larrondo J, McMichael AJ. Traction alopecia. JAMA Dermatol. 2023;159:676. doi:10.1001/jamadermatol.2022.6298
2. James J, Saladi RN, Fox JL. Traction alopecia in Sikh male patients. J Am Board Fam Med. 2007;20:497-498. doi:10.3122/jabfm.2007.05.070076
3. Callender VD, McMichael AJ, Cohen GF. Medical and surgical therapies for alopecias in black women. Dermatol Ther. 2004;17:164-176.
4. Loussouarn G, El Rawadi C, Genain G. Diversity of hair growth profiles. Int J Dermatol. 2005;44(suppl 1):6-9.
5. Samrao AChen CZedek Det al. Traction alopecia in a ballerina: clinicopathologic features. Arch Dermatol. 2010;146:918-935. doi:10.1001/archdermatol.2010.183
6. Korona-Bailey J, Banaag A, Nguyen DR, et al. Free the bun: prevalence of alopecia among active duty service women, fiscal years 2010-2019. Mil Med. 2023;188:e492-e496. doi:10.1093/milmed/usab274
7. Khumalo NP, Jessop S, Gumedze F, et al. Hairdressing is associated with scalp disease in African schoolchildren. Br J Dermatol. 2007;157:106-110. doi:10.1111/j.1365-2133.2007.07987.x
8. Billero V, Miteva M. Traction alopecia: the root of the problem. Clin Cosmet Investig Dermatol. 2018;11:149-159. doi:10.2147/CCID.S137296
9. Haskin A, Aguh C. All hairstyles are not created equal: what the dermatologist needs to know about black hairstyling practices and the risk of traction alopecia (TA). J Am Acad Dermatol. 2016;75:606-611. doi:10.1016/j.jaad.2016.02.1162
10. Samrao A, Price VH, Zedek D, et al. The “fringe sign”—a useful clinical finding in traction alopecia of the marginal hair line. Dermatol Online J. 2011;17:1.
11. Kararizou E, Bougea AM, Giotopoulou D, et al. An update on the less-known group of other primary headaches—a review. Eur Neurol Rev. 2014;9:71-77. doi:10.17925/ENR.2014.09.01.71
12. Tosti A, Miteva M, Torres F, et al. Hair casts are a dermoscopic clue for the diagnosis of traction alopecia. Br J Dermatol. 2010;163:1353-1355.
13. Agrawal S, Daruwalla SB, Dhurat RS. The flambeau sign—a new dermoscopy finding in a case of marginal traction alopecia. Australas J Dermatol. 2020;61:49-50. doi:10.1111/ajd.13187
14. Lawson CN, Hollinger J, Sethi S, et al. Updates in the understanding and treatments of skin & hair disorders in women of color. Int J Womens Dermatol. 2017;3:S21-S37.
15. Awad A, Chim I, Sharma P, et al. Low-dose oral minoxidil improves hair density in traction alopecia. J Am Acad Dermatol. 2023;89:157-159. doi:10.1016/j.jaad.2023.02.024
16. Grayson C, Heath CR. Counseling about traction alopecia: a “compliment, discuss, and suggest” method. Cutis. 2021;108:20-22.
17. Ozçelik D. Extensive traction alopecia attributable to ponytail hairstyle and its treatment with hair transplantation. Aesthetic Plast Surg. 2005;29:325-327. doi:10.1007/s00266-005-0004-5
18. Singh MK, Avram MR. Technical considerations for follicular unit extraction in African-American hair. Dermatol Surg. 2013;39:1282-1284. doi:10.1111/dsu.12229
19. Jones NL, Heath CR. Hair at the intersection of dermatology and anthropology: a conversation on race and relationships. Pediatr Dermatol. 2021;38(suppl 2):158-160.
20. Franklin JMM, Wohltmann WE, Wong EB. From buns to braids and ponytails: entering a new era of female military hair-grooming standards. Cutis. 2021;108:31-35. doi:10.12788/cutis.0296
1. Larrondo J, McMichael AJ. Traction alopecia. JAMA Dermatol. 2023;159:676. doi:10.1001/jamadermatol.2022.6298
2. James J, Saladi RN, Fox JL. Traction alopecia in Sikh male patients. J Am Board Fam Med. 2007;20:497-498. doi:10.3122/jabfm.2007.05.070076
3. Callender VD, McMichael AJ, Cohen GF. Medical and surgical therapies for alopecias in black women. Dermatol Ther. 2004;17:164-176.
4. Loussouarn G, El Rawadi C, Genain G. Diversity of hair growth profiles. Int J Dermatol. 2005;44(suppl 1):6-9.
5. Samrao AChen CZedek Det al. Traction alopecia in a ballerina: clinicopathologic features. Arch Dermatol. 2010;146:918-935. doi:10.1001/archdermatol.2010.183
6. Korona-Bailey J, Banaag A, Nguyen DR, et al. Free the bun: prevalence of alopecia among active duty service women, fiscal years 2010-2019. Mil Med. 2023;188:e492-e496. doi:10.1093/milmed/usab274
7. Khumalo NP, Jessop S, Gumedze F, et al. Hairdressing is associated with scalp disease in African schoolchildren. Br J Dermatol. 2007;157:106-110. doi:10.1111/j.1365-2133.2007.07987.x
8. Billero V, Miteva M. Traction alopecia: the root of the problem. Clin Cosmet Investig Dermatol. 2018;11:149-159. doi:10.2147/CCID.S137296
9. Haskin A, Aguh C. All hairstyles are not created equal: what the dermatologist needs to know about black hairstyling practices and the risk of traction alopecia (TA). J Am Acad Dermatol. 2016;75:606-611. doi:10.1016/j.jaad.2016.02.1162
10. Samrao A, Price VH, Zedek D, et al. The “fringe sign”—a useful clinical finding in traction alopecia of the marginal hair line. Dermatol Online J. 2011;17:1.
11. Kararizou E, Bougea AM, Giotopoulou D, et al. An update on the less-known group of other primary headaches—a review. Eur Neurol Rev. 2014;9:71-77. doi:10.17925/ENR.2014.09.01.71
12. Tosti A, Miteva M, Torres F, et al. Hair casts are a dermoscopic clue for the diagnosis of traction alopecia. Br J Dermatol. 2010;163:1353-1355.
13. Agrawal S, Daruwalla SB, Dhurat RS. The flambeau sign—a new dermoscopy finding in a case of marginal traction alopecia. Australas J Dermatol. 2020;61:49-50. doi:10.1111/ajd.13187
14. Lawson CN, Hollinger J, Sethi S, et al. Updates in the understanding and treatments of skin & hair disorders in women of color. Int J Womens Dermatol. 2017;3:S21-S37.
15. Awad A, Chim I, Sharma P, et al. Low-dose oral minoxidil improves hair density in traction alopecia. J Am Acad Dermatol. 2023;89:157-159. doi:10.1016/j.jaad.2023.02.024
16. Grayson C, Heath CR. Counseling about traction alopecia: a “compliment, discuss, and suggest” method. Cutis. 2021;108:20-22.
17. Ozçelik D. Extensive traction alopecia attributable to ponytail hairstyle and its treatment with hair transplantation. Aesthetic Plast Surg. 2005;29:325-327. doi:10.1007/s00266-005-0004-5
18. Singh MK, Avram MR. Technical considerations for follicular unit extraction in African-American hair. Dermatol Surg. 2013;39:1282-1284. doi:10.1111/dsu.12229
19. Jones NL, Heath CR. Hair at the intersection of dermatology and anthropology: a conversation on race and relationships. Pediatr Dermatol. 2021;38(suppl 2):158-160.
20. Franklin JMM, Wohltmann WE, Wong EB. From buns to braids and ponytails: entering a new era of female military hair-grooming standards. Cutis. 2021;108:31-35. doi:10.12788/cutis.0296