Novel Intraoperative Technique to Visualize the Lower Cervical Spine: A Case Series

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Novel Intraoperative Technique to Visualize the Lower Cervical Spine: A Case Series

Two adequate views of the lower cervical vertebrae are necessary to confirm the 3-dimensional location of any hardware placed during cervical spine fusion. Visualizing the lower cervical vertebrae in 2 planes intraoperatively is often a challenge because the shoulders obstruct the lateral view.1 Techniques have been described to improve lateral visualization, including gentle traction of the arms via wrist restraints or taping the shoulders down inferiorly.2,3 These techniques have their inadequacies, including an association with peripheral nerve injury and brachial plexopathy.4 In patients with stout necks, these methods may still be insufficient to achieve adequate visualization of the lower cervical vertebrae.

Invasive techniques to improve visualization have also been described. In 1 study, exposure had to be extended cephalad to allow for manual counting of cervical vertebrae when the mid- to lower cervical vertebrae had to be identified in a morbidly obese patient.5 More invasive spine procedures are associated with higher rates of complications, increased blood loss, more soft-tissue trauma, and longer hospital stays.6 We present a view 30º oblique from horizontal and 30º cephalad from neutral as a variation of the lateral radiograph that improves visualization of the mid- to lower cervical vertebrae. The authors have obtained the patients’ informed written consent for print and electronic publication of these case reports.

Technique

We used either the Smith-Robinson or Cloward approach to the anterior spine. Both techniques use the avascular plane between the medially located esophagus and trachea and the lateral sternocleidomastoid and carotid sheath to approach the anterior cervical spine. Once adequate exposure was achieved, standard anteroposterior and lateral radiographs were obtained to confirm the correct vertebral level. Gentle caudal traction was applied to the patient’s wrist straps, and when visualization continued to be compromised, a view 30º oblique from horizontal and 30º cephalad from neutral was obtained (Figure 1).

Case Series

Case 1

A 54-year-old man with a body mass index (BMI) of 50 presented with neck and bilateral arm pain, with left greater than right radicular symptoms in the C6 and C7 distribution. Magnetic resonance imaging (MRI) showed disc herniations at C5-C6 and C6-C7 with spinal cord signal changes, and he underwent a C5-C6 and C6-C7 anterior cervical discectomy and fusion. Initial localization was determined using a lateral radiograph and vertebral needle. During hardware placement, anteroposterior and lateral fluoroscopic radiographs confirmed adequate placement of the superior screw, but visualization of the inferior portion of the plate and inferior screw was challenging (Figure 2). Our oblique 30º–30º view provided better visualization of the plate and screws in the lower cervical vertebrae than lateral imaging, and allowed confirmation that the hardware was positioned correctly (Figure 3). It took 1 attempt to achieve adequate visualization with the 30º–30º view.

 

Postoperatively, the patient’s radiculopathy and motor weakness improved. Radiographs confirmed adequate hardware placement, and he was discharged on postoperative day 1 (Figure 4). Imaging at the patient’s 6-week follow-up confirmed adequate fusion from C5-C7, anatomically aligned facet joints, and no hardware failure. The patient’s Neck Disability Index was 31/50 preoperatively and 26/50 at this visit.

Case 2

A 51-year-old man with a BMI of 29 presented with a long-standing history of neck pain and bilateral arm pain left greater than right in the C6 and C7 dermomyotome. MRI showed a broad-based disc herniation with foraminal narrowing at C5-C6 and C6-C7, and the patient underwent a 2-level anterior cervical discectomy and fusion. This patient had pronounced neck musculature, and a deeper than normal incision was required.

Intraoperative lateral fluoroscopy was obtained to confirm the C5-C6 and C6-C7 level prior to discectomy. The musculature of the patient’s neck and shoulder made visualization of the C6-C7 disc space difficult on the lateral radiograph (Figure 5). One attempt was required to obtain the 30º–30º oblique view, which was used to ensure correct placement of the screws and plate (Figure 6).

 

Postoperatively, the patient’s pain had improved, and radiographs confirmed adequate hardware placement. He was discharged 1 day after surgery (Figure 7). Imaging at the patient’s 6-week follow-up confirmed adequate fusion from C5-C7, stable disc spaces, and anatomically aligned facet joints. His Neck Disability Index was 34/50 preoperatively and 32/50 at 2-week follow-up.

Discussion

The aim of this study was to describe an alternative to the lateral radiograph for imaging the cervical spine in patients with challenging anatomy or in procedures involving hardware placement at the lower cervical vertebrae. Techniques have been developed to assist with improved lateral visualization, including gentle traction of the arms via wrist restraints or taping the shoulders down inferiorly.2,3 However, visualization in 2 planes continues to be a challenge in a subset of patients. It is particularly difficult to obtain adequate lateral radiographs of the cervical spine in patients with stout necks.3 In patients with stout necks, there is more obstruction of the radiography path through the cervical spine. This leads to imaging that is unclear or may fail to show the mid- to lower cervical spine. The extent to which one should rely on the 30º–30º oblique technique for adequate visualization of the cervical spine depends on the anatomy of a particular patient. Historically, it is more challenging to obtain satisfactory lateral radiographs in patients with stout necks,3 and these patients have benefited the most from using the 30º–30º degree oblique view.

 

 

Lack of visualization can lead to aborted surgeries or, potentially, surgery at the wrong level.3 A 2008 American Academy of Neurological Surgeons survey indicated that 50% of spine surgeons had performed a wrong-level surgery at least once in their career, and the cervical spine accounted for 21% of all incorrect-level spine surgeries.7 Intraoperative factors reported during cases of wrong-level spinal surgeries included misinterpretation of intraoperative imaging, no intraoperative imaging, and unusual anatomy or physical characteristics.8 Such complications can lead to revision surgery and other significant morbidities for the patient.

In most patients, fluoroscopy allows confirmation of the correct level before disc incision.3 However, operating at a lower cervical level in a patient with a short neck or prominent shoulders poses a significant problem.3 A case report from Singh and colleagues9 described a modified intraoperative fluoroscopic view for spinal level localization at cervicothoracic levels. Their method focuses on identifying the bony lamina and using them as landmarks to count spinal levels, whereas our 30º–30º oblique image is useful for confirmation of adequate hardware placement during anterior cervical spinal fusions. Often, the initial localization of cervical vertebral levels can be achieved with a standard lateral radiograph. We recognized the utility of the 30º–30º oblique view when we were attempting to visualize the inferior aspect of the plate and inferior screw placement.

In patients with stout necks, a lateral radiograph may show only visualization down to C4 or C5.3 Even with applying traction to the arms or taping the shoulders down, it can be impossible to visualize C6, C7, or T1 because the shoulder bones and muscles obstruct the image.3 Using a 30º–30º oblique view, we were able to obtain adequate visualization and assess the accurate placement of hardware.

Conclusion

A 30º oblique view from horizontal and 30º cephalad from neutral radiograph can be used intraoperatively in patients with challenging anatomy to identify placement of hardware at the correct vertebral level in the lower cervical spine. It is a noninvasive technique that can help reduce the risk of wrong-site surgeries without prolonging operation time. This technique describes an alternative to the lateral radiograph and provides a solution to the difficult problem of intraoperative imaging of the mid- to lower cervical spine in 2 adequate planes.

References

1.    Bebawy JF, Koht A, Mirkovic S. Anterior cervical spine surgery. In: Khot A, Sloan TB, Toleikis JR, eds. Monitoring the Nervous System for Anesthesiologists and Other Health Care Professionals. New York, NY: Springer; 2012:539-554.

2.    Abumi K, Shono Y, Ito M, Taneichi H, Kotani Y, Kaneda K. Complications of pedicle screw fixation in reconstructive surgery of the cervical spine. Spine. 2000;25(8):962-969.

3.    Irace C. Intraoperative imaging for verification of the correct level during spinal surgery. In: Fountas KN, ed. Novel Frontiers of Advanced Neuroimaging. Rijeka, Croatia: Intech; 2013:175-188.

4.    Schwartz DM, Sestokas AK, Hilibrand AS, et al. Neurophysiological identification of position-induced neurologic injury during anterior cervical spine surgery. J Clin Monit Comput. 2006;20(6):437-444.

5.    Telfeian AE, Reiter GT, Durham SR, Marcotte P. Spine surgery in morbidly obese patients. J Neurosurg Spine. 2002;97(1):20-24.

6.    Oppenheimer JH, DeCastro I, McDonnell DE. Minimally invasive spine technology and minimally invasive spine surgery: a historical review. Neurosurg Focus. 2009;27(3):E9.

7.    Mody MG, Nourbakhsh A, Stahl DL, Gibbs M, Alfawareh M, Garges KJ. The prevalence of wrong level surgery among spine surgeons. Spine. 2008;33(2):194.

8.    Jhawar BS, Mitsis D, Duggal N. Wrong-sided and wrong-level neurosurgery: A national survey. J Neurosurg Spine. 2007;7(5):467-472.

9.    Singh H, Meyer SA, Hecht AC, Jenkins AL 3rd. Novel fluoroscopic technique for localization at cervicothoracic levels. J Spinal Disord Tech. 2009;22(8):615-618.

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Ryan Rauck, MD, Kari Stammen, AT, Elizabeth Yu, MD, Safdar N. Khan, MD

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Two adequate views of the lower cervical vertebrae are necessary to confirm the 3-dimensional location of any hardware placed during cervical spine fusion. Visualizing the lower cervical vertebrae in 2 planes intraoperatively is often a challenge because the shoulders obstruct the lateral view.1 Techniques have been described to improve lateral visualization, including gentle traction of the arms via wrist restraints or taping the shoulders down inferiorly.2,3 These techniques have their inadequacies, including an association with peripheral nerve injury and brachial plexopathy.4 In patients with stout necks, these methods may still be insufficient to achieve adequate visualization of the lower cervical vertebrae.

Invasive techniques to improve visualization have also been described. In 1 study, exposure had to be extended cephalad to allow for manual counting of cervical vertebrae when the mid- to lower cervical vertebrae had to be identified in a morbidly obese patient.5 More invasive spine procedures are associated with higher rates of complications, increased blood loss, more soft-tissue trauma, and longer hospital stays.6 We present a view 30º oblique from horizontal and 30º cephalad from neutral as a variation of the lateral radiograph that improves visualization of the mid- to lower cervical vertebrae. The authors have obtained the patients’ informed written consent for print and electronic publication of these case reports.

Technique

We used either the Smith-Robinson or Cloward approach to the anterior spine. Both techniques use the avascular plane between the medially located esophagus and trachea and the lateral sternocleidomastoid and carotid sheath to approach the anterior cervical spine. Once adequate exposure was achieved, standard anteroposterior and lateral radiographs were obtained to confirm the correct vertebral level. Gentle caudal traction was applied to the patient’s wrist straps, and when visualization continued to be compromised, a view 30º oblique from horizontal and 30º cephalad from neutral was obtained (Figure 1).

Case Series

Case 1

A 54-year-old man with a body mass index (BMI) of 50 presented with neck and bilateral arm pain, with left greater than right radicular symptoms in the C6 and C7 distribution. Magnetic resonance imaging (MRI) showed disc herniations at C5-C6 and C6-C7 with spinal cord signal changes, and he underwent a C5-C6 and C6-C7 anterior cervical discectomy and fusion. Initial localization was determined using a lateral radiograph and vertebral needle. During hardware placement, anteroposterior and lateral fluoroscopic radiographs confirmed adequate placement of the superior screw, but visualization of the inferior portion of the plate and inferior screw was challenging (Figure 2). Our oblique 30º–30º view provided better visualization of the plate and screws in the lower cervical vertebrae than lateral imaging, and allowed confirmation that the hardware was positioned correctly (Figure 3). It took 1 attempt to achieve adequate visualization with the 30º–30º view.

 

Postoperatively, the patient’s radiculopathy and motor weakness improved. Radiographs confirmed adequate hardware placement, and he was discharged on postoperative day 1 (Figure 4). Imaging at the patient’s 6-week follow-up confirmed adequate fusion from C5-C7, anatomically aligned facet joints, and no hardware failure. The patient’s Neck Disability Index was 31/50 preoperatively and 26/50 at this visit.

Case 2

A 51-year-old man with a BMI of 29 presented with a long-standing history of neck pain and bilateral arm pain left greater than right in the C6 and C7 dermomyotome. MRI showed a broad-based disc herniation with foraminal narrowing at C5-C6 and C6-C7, and the patient underwent a 2-level anterior cervical discectomy and fusion. This patient had pronounced neck musculature, and a deeper than normal incision was required.

Intraoperative lateral fluoroscopy was obtained to confirm the C5-C6 and C6-C7 level prior to discectomy. The musculature of the patient’s neck and shoulder made visualization of the C6-C7 disc space difficult on the lateral radiograph (Figure 5). One attempt was required to obtain the 30º–30º oblique view, which was used to ensure correct placement of the screws and plate (Figure 6).

 

Postoperatively, the patient’s pain had improved, and radiographs confirmed adequate hardware placement. He was discharged 1 day after surgery (Figure 7). Imaging at the patient’s 6-week follow-up confirmed adequate fusion from C5-C7, stable disc spaces, and anatomically aligned facet joints. His Neck Disability Index was 34/50 preoperatively and 32/50 at 2-week follow-up.

Discussion

The aim of this study was to describe an alternative to the lateral radiograph for imaging the cervical spine in patients with challenging anatomy or in procedures involving hardware placement at the lower cervical vertebrae. Techniques have been developed to assist with improved lateral visualization, including gentle traction of the arms via wrist restraints or taping the shoulders down inferiorly.2,3 However, visualization in 2 planes continues to be a challenge in a subset of patients. It is particularly difficult to obtain adequate lateral radiographs of the cervical spine in patients with stout necks.3 In patients with stout necks, there is more obstruction of the radiography path through the cervical spine. This leads to imaging that is unclear or may fail to show the mid- to lower cervical spine. The extent to which one should rely on the 30º–30º oblique technique for adequate visualization of the cervical spine depends on the anatomy of a particular patient. Historically, it is more challenging to obtain satisfactory lateral radiographs in patients with stout necks,3 and these patients have benefited the most from using the 30º–30º degree oblique view.

 

 

Lack of visualization can lead to aborted surgeries or, potentially, surgery at the wrong level.3 A 2008 American Academy of Neurological Surgeons survey indicated that 50% of spine surgeons had performed a wrong-level surgery at least once in their career, and the cervical spine accounted for 21% of all incorrect-level spine surgeries.7 Intraoperative factors reported during cases of wrong-level spinal surgeries included misinterpretation of intraoperative imaging, no intraoperative imaging, and unusual anatomy or physical characteristics.8 Such complications can lead to revision surgery and other significant morbidities for the patient.

In most patients, fluoroscopy allows confirmation of the correct level before disc incision.3 However, operating at a lower cervical level in a patient with a short neck or prominent shoulders poses a significant problem.3 A case report from Singh and colleagues9 described a modified intraoperative fluoroscopic view for spinal level localization at cervicothoracic levels. Their method focuses on identifying the bony lamina and using them as landmarks to count spinal levels, whereas our 30º–30º oblique image is useful for confirmation of adequate hardware placement during anterior cervical spinal fusions. Often, the initial localization of cervical vertebral levels can be achieved with a standard lateral radiograph. We recognized the utility of the 30º–30º oblique view when we were attempting to visualize the inferior aspect of the plate and inferior screw placement.

In patients with stout necks, a lateral radiograph may show only visualization down to C4 or C5.3 Even with applying traction to the arms or taping the shoulders down, it can be impossible to visualize C6, C7, or T1 because the shoulder bones and muscles obstruct the image.3 Using a 30º–30º oblique view, we were able to obtain adequate visualization and assess the accurate placement of hardware.

Conclusion

A 30º oblique view from horizontal and 30º cephalad from neutral radiograph can be used intraoperatively in patients with challenging anatomy to identify placement of hardware at the correct vertebral level in the lower cervical spine. It is a noninvasive technique that can help reduce the risk of wrong-site surgeries without prolonging operation time. This technique describes an alternative to the lateral radiograph and provides a solution to the difficult problem of intraoperative imaging of the mid- to lower cervical spine in 2 adequate planes.

Two adequate views of the lower cervical vertebrae are necessary to confirm the 3-dimensional location of any hardware placed during cervical spine fusion. Visualizing the lower cervical vertebrae in 2 planes intraoperatively is often a challenge because the shoulders obstruct the lateral view.1 Techniques have been described to improve lateral visualization, including gentle traction of the arms via wrist restraints or taping the shoulders down inferiorly.2,3 These techniques have their inadequacies, including an association with peripheral nerve injury and brachial plexopathy.4 In patients with stout necks, these methods may still be insufficient to achieve adequate visualization of the lower cervical vertebrae.

Invasive techniques to improve visualization have also been described. In 1 study, exposure had to be extended cephalad to allow for manual counting of cervical vertebrae when the mid- to lower cervical vertebrae had to be identified in a morbidly obese patient.5 More invasive spine procedures are associated with higher rates of complications, increased blood loss, more soft-tissue trauma, and longer hospital stays.6 We present a view 30º oblique from horizontal and 30º cephalad from neutral as a variation of the lateral radiograph that improves visualization of the mid- to lower cervical vertebrae. The authors have obtained the patients’ informed written consent for print and electronic publication of these case reports.

Technique

We used either the Smith-Robinson or Cloward approach to the anterior spine. Both techniques use the avascular plane between the medially located esophagus and trachea and the lateral sternocleidomastoid and carotid sheath to approach the anterior cervical spine. Once adequate exposure was achieved, standard anteroposterior and lateral radiographs were obtained to confirm the correct vertebral level. Gentle caudal traction was applied to the patient’s wrist straps, and when visualization continued to be compromised, a view 30º oblique from horizontal and 30º cephalad from neutral was obtained (Figure 1).

Case Series

Case 1

A 54-year-old man with a body mass index (BMI) of 50 presented with neck and bilateral arm pain, with left greater than right radicular symptoms in the C6 and C7 distribution. Magnetic resonance imaging (MRI) showed disc herniations at C5-C6 and C6-C7 with spinal cord signal changes, and he underwent a C5-C6 and C6-C7 anterior cervical discectomy and fusion. Initial localization was determined using a lateral radiograph and vertebral needle. During hardware placement, anteroposterior and lateral fluoroscopic radiographs confirmed adequate placement of the superior screw, but visualization of the inferior portion of the plate and inferior screw was challenging (Figure 2). Our oblique 30º–30º view provided better visualization of the plate and screws in the lower cervical vertebrae than lateral imaging, and allowed confirmation that the hardware was positioned correctly (Figure 3). It took 1 attempt to achieve adequate visualization with the 30º–30º view.

 

Postoperatively, the patient’s radiculopathy and motor weakness improved. Radiographs confirmed adequate hardware placement, and he was discharged on postoperative day 1 (Figure 4). Imaging at the patient’s 6-week follow-up confirmed adequate fusion from C5-C7, anatomically aligned facet joints, and no hardware failure. The patient’s Neck Disability Index was 31/50 preoperatively and 26/50 at this visit.

Case 2

A 51-year-old man with a BMI of 29 presented with a long-standing history of neck pain and bilateral arm pain left greater than right in the C6 and C7 dermomyotome. MRI showed a broad-based disc herniation with foraminal narrowing at C5-C6 and C6-C7, and the patient underwent a 2-level anterior cervical discectomy and fusion. This patient had pronounced neck musculature, and a deeper than normal incision was required.

Intraoperative lateral fluoroscopy was obtained to confirm the C5-C6 and C6-C7 level prior to discectomy. The musculature of the patient’s neck and shoulder made visualization of the C6-C7 disc space difficult on the lateral radiograph (Figure 5). One attempt was required to obtain the 30º–30º oblique view, which was used to ensure correct placement of the screws and plate (Figure 6).

 

Postoperatively, the patient’s pain had improved, and radiographs confirmed adequate hardware placement. He was discharged 1 day after surgery (Figure 7). Imaging at the patient’s 6-week follow-up confirmed adequate fusion from C5-C7, stable disc spaces, and anatomically aligned facet joints. His Neck Disability Index was 34/50 preoperatively and 32/50 at 2-week follow-up.

Discussion

The aim of this study was to describe an alternative to the lateral radiograph for imaging the cervical spine in patients with challenging anatomy or in procedures involving hardware placement at the lower cervical vertebrae. Techniques have been developed to assist with improved lateral visualization, including gentle traction of the arms via wrist restraints or taping the shoulders down inferiorly.2,3 However, visualization in 2 planes continues to be a challenge in a subset of patients. It is particularly difficult to obtain adequate lateral radiographs of the cervical spine in patients with stout necks.3 In patients with stout necks, there is more obstruction of the radiography path through the cervical spine. This leads to imaging that is unclear or may fail to show the mid- to lower cervical spine. The extent to which one should rely on the 30º–30º oblique technique for adequate visualization of the cervical spine depends on the anatomy of a particular patient. Historically, it is more challenging to obtain satisfactory lateral radiographs in patients with stout necks,3 and these patients have benefited the most from using the 30º–30º degree oblique view.

 

 

Lack of visualization can lead to aborted surgeries or, potentially, surgery at the wrong level.3 A 2008 American Academy of Neurological Surgeons survey indicated that 50% of spine surgeons had performed a wrong-level surgery at least once in their career, and the cervical spine accounted for 21% of all incorrect-level spine surgeries.7 Intraoperative factors reported during cases of wrong-level spinal surgeries included misinterpretation of intraoperative imaging, no intraoperative imaging, and unusual anatomy or physical characteristics.8 Such complications can lead to revision surgery and other significant morbidities for the patient.

In most patients, fluoroscopy allows confirmation of the correct level before disc incision.3 However, operating at a lower cervical level in a patient with a short neck or prominent shoulders poses a significant problem.3 A case report from Singh and colleagues9 described a modified intraoperative fluoroscopic view for spinal level localization at cervicothoracic levels. Their method focuses on identifying the bony lamina and using them as landmarks to count spinal levels, whereas our 30º–30º oblique image is useful for confirmation of adequate hardware placement during anterior cervical spinal fusions. Often, the initial localization of cervical vertebral levels can be achieved with a standard lateral radiograph. We recognized the utility of the 30º–30º oblique view when we were attempting to visualize the inferior aspect of the plate and inferior screw placement.

In patients with stout necks, a lateral radiograph may show only visualization down to C4 or C5.3 Even with applying traction to the arms or taping the shoulders down, it can be impossible to visualize C6, C7, or T1 because the shoulder bones and muscles obstruct the image.3 Using a 30º–30º oblique view, we were able to obtain adequate visualization and assess the accurate placement of hardware.

Conclusion

A 30º oblique view from horizontal and 30º cephalad from neutral radiograph can be used intraoperatively in patients with challenging anatomy to identify placement of hardware at the correct vertebral level in the lower cervical spine. It is a noninvasive technique that can help reduce the risk of wrong-site surgeries without prolonging operation time. This technique describes an alternative to the lateral radiograph and provides a solution to the difficult problem of intraoperative imaging of the mid- to lower cervical spine in 2 adequate planes.

References

1.    Bebawy JF, Koht A, Mirkovic S. Anterior cervical spine surgery. In: Khot A, Sloan TB, Toleikis JR, eds. Monitoring the Nervous System for Anesthesiologists and Other Health Care Professionals. New York, NY: Springer; 2012:539-554.

2.    Abumi K, Shono Y, Ito M, Taneichi H, Kotani Y, Kaneda K. Complications of pedicle screw fixation in reconstructive surgery of the cervical spine. Spine. 2000;25(8):962-969.

3.    Irace C. Intraoperative imaging for verification of the correct level during spinal surgery. In: Fountas KN, ed. Novel Frontiers of Advanced Neuroimaging. Rijeka, Croatia: Intech; 2013:175-188.

4.    Schwartz DM, Sestokas AK, Hilibrand AS, et al. Neurophysiological identification of position-induced neurologic injury during anterior cervical spine surgery. J Clin Monit Comput. 2006;20(6):437-444.

5.    Telfeian AE, Reiter GT, Durham SR, Marcotte P. Spine surgery in morbidly obese patients. J Neurosurg Spine. 2002;97(1):20-24.

6.    Oppenheimer JH, DeCastro I, McDonnell DE. Minimally invasive spine technology and minimally invasive spine surgery: a historical review. Neurosurg Focus. 2009;27(3):E9.

7.    Mody MG, Nourbakhsh A, Stahl DL, Gibbs M, Alfawareh M, Garges KJ. The prevalence of wrong level surgery among spine surgeons. Spine. 2008;33(2):194.

8.    Jhawar BS, Mitsis D, Duggal N. Wrong-sided and wrong-level neurosurgery: A national survey. J Neurosurg Spine. 2007;7(5):467-472.

9.    Singh H, Meyer SA, Hecht AC, Jenkins AL 3rd. Novel fluoroscopic technique for localization at cervicothoracic levels. J Spinal Disord Tech. 2009;22(8):615-618.

References

1.    Bebawy JF, Koht A, Mirkovic S. Anterior cervical spine surgery. In: Khot A, Sloan TB, Toleikis JR, eds. Monitoring the Nervous System for Anesthesiologists and Other Health Care Professionals. New York, NY: Springer; 2012:539-554.

2.    Abumi K, Shono Y, Ito M, Taneichi H, Kotani Y, Kaneda K. Complications of pedicle screw fixation in reconstructive surgery of the cervical spine. Spine. 2000;25(8):962-969.

3.    Irace C. Intraoperative imaging for verification of the correct level during spinal surgery. In: Fountas KN, ed. Novel Frontiers of Advanced Neuroimaging. Rijeka, Croatia: Intech; 2013:175-188.

4.    Schwartz DM, Sestokas AK, Hilibrand AS, et al. Neurophysiological identification of position-induced neurologic injury during anterior cervical spine surgery. J Clin Monit Comput. 2006;20(6):437-444.

5.    Telfeian AE, Reiter GT, Durham SR, Marcotte P. Spine surgery in morbidly obese patients. J Neurosurg Spine. 2002;97(1):20-24.

6.    Oppenheimer JH, DeCastro I, McDonnell DE. Minimally invasive spine technology and minimally invasive spine surgery: a historical review. Neurosurg Focus. 2009;27(3):E9.

7.    Mody MG, Nourbakhsh A, Stahl DL, Gibbs M, Alfawareh M, Garges KJ. The prevalence of wrong level surgery among spine surgeons. Spine. 2008;33(2):194.

8.    Jhawar BS, Mitsis D, Duggal N. Wrong-sided and wrong-level neurosurgery: A national survey. J Neurosurg Spine. 2007;7(5):467-472.

9.    Singh H, Meyer SA, Hecht AC, Jenkins AL 3rd. Novel fluoroscopic technique for localization at cervicothoracic levels. J Spinal Disord Tech. 2009;22(8):615-618.

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The American Journal of Orthopedics - 45(2)
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Novel Intraoperative Technique to Visualize the Lower Cervical Spine: A Case Series
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technique, online exclusive, spine, cervical spine, radiograph, shoulder, imaging, hardware, rauck, stammen, yu, khan
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Navigating the Alphabet Soup of Labroligamentous Pathology of the Shoulder

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Navigating the Alphabet Soup of Labroligamentous Pathology of the Shoulder

The widespread use of eponyms and acronyms to describe labroligamentous findings in the shoulder has made interpretation of shoulder magnetic resonance imaging (MRI) reports challenging. We review and discuss the appearance of these lesions on shoulder MRI to help the orthopedic surgeon understand these entities as imaging findings.

Glenolabral articular disruption (GLAD) occurs secondary to impaction of the humeral head on the glenoid articular cartilage. There is a resultant defect in the glenoid articular cartilage, which extends to the glenoid labrum. A GLAD lesion is diagnosed only if the glenohumeral ligament and scapular periosteum remain intact1 (Figure 1).

Complete detachment of the anteroinferior labrum with tearing of the anterior glenoid periosteum represents a Bankart lesion. Cartilaginous Bankart lesions are caused by an anterior glenohumeral dislocation with resultant avulsion of the anteroinferior labrum and disruption of the scapular periosteum because of acute traction on the anterior band of the inferior glenohumeral ligament (Figure 2). Anterior instability, caused by disruption of the anterior labroligamentous complex, results. Osseous Bankart lesions occur when the anterior displaced humeral head impacts the anterior inferior glenoid rim, causing a fracture (Figure 3). This loss of the glenoid articular surface area can result in glenohumeral instability. Posterior shoulder dislocations can result in corresponding findings in the posterior inferior glenoid labrum (reverse Bankart lesion) and anterior medial humeral head (reverse Hill-Sachs lesion) (Figure 2).

 

A variant of the Bankart lesion is the anterior labroligamentous periosteal sleeve avulsion (ALPSA). This refers to a medially displaced tear of the anterior labrum with intact periosteal stripping along the medial glenoid2with medial rotation and inferior displacement of the anterior inferior labrum along the scapular neck. An ALPSA lesion can heal via the intact periosteal blood supply. If not repaired, anterior instability will result because of malposition of the labrum, causing a patulous anterior capsule.3 When a corresponding lesion occurs in the posterior labrum because of a posterior dislocation, it is called a posterior labrocapsular periosteal sleeve avulsion (POLPSA) (Figure 4).

Another variant of the Bankart lesion is the Perthes lesion, which is a nondisplaced tear of the anteroinferior labrum with periosteal stripping. This differs from the ALPSA because the detached labrum and periosteum are held in anatomic position, possibly making the lesion difficult to detect on magnetic resonance arthrography (MRA).3 Obtaining images in the abduction external rotation (ABER) position exerts traction on the anterior inferior joint capsule and may make the Perthes lesion more conspicuous.4 When this occurs in the posterior labrum, it is called a reverse Perthes lesion (Figure 5).

In a patient with anterior glenohumeral instability without a Bankart lesion, pathology of the anterior band of the inferior glenohumeral ligament (IGHL) at its humeral attachment must be suspected. Humeral avulsion of the IGHL (HAGL) or its variants can be overlooked on arthroscopy. HAGL is diagnosed on MRA when the normally U-shaped IGHL takes on a J-shape, and joint fluid extravasates across the torn humeral attachment (Figure 6). If there is an avulsed bony fragment from the medial humeral neck, the lesion is termed a bony HAGL (BHAGL). In addition to the findings of a HAGL, a BHAGL shows the osseous fragment and donor site on MRI. Since a BHAGL is a bony avulsion, it can even be suggested on radiography if a bony fragment is seen adjacent to the medial humeral neck.5 These lesions are highly associated with other shoulder injuries, particularly Hill-Sachs deformities and subscapularis tendon tears, and it is imperative, therefore, to search for additional injuries if a HAGL-type injury is seen.6

A more uncommon type of HAGL can occur in the setting of posterior capsulolabral injury. A posterior-band IGHL avulsion from the humerus (PHAGL) has similar imaging findings to a HAGL, except that it involves the posterior band of the IGHL. PHAGLs are usually not associated with an acute injury and are thought to be related to repetitive microtrauma, perhaps since the posterior band of the IGHL is the thinnest portion of the IGHL complex.7

A Kim lesion is an arthroscopic finding described in patients with posterior instability as a superficial defect at the undersurface of the posterior labrum and adjacent glenoid cartilage without detachment or extension to the chondrolabral junction.8 It is, by its nature, a concealed finding on routine MRI but can be more conspicuous in FADIR (flexed, adducted, internally rotated) positioning on MRA, which exerts traction on the posterior joint capsule, allowing intra-articular contrast to fill the tear (Figure 7).

This list describes several of the most commonly encountered acronyms in shoulder MRI. A review of SLAP (superior labrum anterior to posterior) lesions was described in a previous article in the journal’s Imaging Series.9 A thorough understanding of these lesions is helpful in interpreting reports and determining the appropriate treatment for patients with shoulder injuries.

References

1.    Sanders TG, Tirman PF, Linares R, Feller JF, Richardson R. The glenolabral articular disruption lesion: MR arthrography with arthroscopic correlation. AJR Am J Roentgenol. 1999;172(1):171-175.

2.    Beltran J, Jbara M, Maimon R. Shoulder: labrum and bicipital tendon. Top Magn Reson Imaging. 2003;14(1):35-50.

3.    Waldt S, Burkart A, Imhoff AB, Bruegel M, Rummeny EJ, Woertler K. Anterior shoulder instability: accuracy of MR arthrography in the classification of anteroinferior labroligamentous injuries. Radiology. 2005;237(2):578-583.

4.    Schreinemachers SA, van der Hulst VP, Willems J, Bipat S, van der Woude H. Is a single direct MR arthrography series in ABER position as accurate in detecting anteroinferior labroligamentous lesions as conventional MR arthrography? Skeletal Radiol. 2009;38(7):675-683.

5.    Bui-Mansfield LT, Taylor DC, Uhorchak JM, Tenuta JT. Humeral avulsions of the glenohumeral ligament: imaging features and a review of the literature. AJR Am J Roentgenol. 2002;179(3):649-655.

6.    Magee T. Prevalence of HAGL lesions and associated abnormalities on shoulder MR examination. Skeletal Radiol. 2014;43(3):307-313.

7.    Chung CB, Sorenson S, Dwek JR, Resnick D. Humeral avulsion of the posterior band of the inferior glenohumeral ligament: MR arthrography and clinical correlation in 17 patients. AJR Am J Roentgenol. 2004;183(2):355-359.

8.    Kim SH, Ha KI, Yoo JC, Noh KC. Kim’s lesion: an incomplete and concealed avulsion of the posteroinferior labrum in posterior or multidirectional posteroinferior instability of the shoulder. Arthroscopy. 2004;20(7):712-720.

9.    Grubin J, Maderazo A, Fitzpatrick D. Imaging evaluation of superior labral anteroposterior (SLAP) tears. Am J Orthop. 2015;44(10):476-477.

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The widespread use of eponyms and acronyms to describe labroligamentous findings in the shoulder has made interpretation of shoulder magnetic resonance imaging (MRI) reports challenging. We review and discuss the appearance of these lesions on shoulder MRI to help the orthopedic surgeon understand these entities as imaging findings.

Glenolabral articular disruption (GLAD) occurs secondary to impaction of the humeral head on the glenoid articular cartilage. There is a resultant defect in the glenoid articular cartilage, which extends to the glenoid labrum. A GLAD lesion is diagnosed only if the glenohumeral ligament and scapular periosteum remain intact1 (Figure 1).

Complete detachment of the anteroinferior labrum with tearing of the anterior glenoid periosteum represents a Bankart lesion. Cartilaginous Bankart lesions are caused by an anterior glenohumeral dislocation with resultant avulsion of the anteroinferior labrum and disruption of the scapular periosteum because of acute traction on the anterior band of the inferior glenohumeral ligament (Figure 2). Anterior instability, caused by disruption of the anterior labroligamentous complex, results. Osseous Bankart lesions occur when the anterior displaced humeral head impacts the anterior inferior glenoid rim, causing a fracture (Figure 3). This loss of the glenoid articular surface area can result in glenohumeral instability. Posterior shoulder dislocations can result in corresponding findings in the posterior inferior glenoid labrum (reverse Bankart lesion) and anterior medial humeral head (reverse Hill-Sachs lesion) (Figure 2).

 

A variant of the Bankart lesion is the anterior labroligamentous periosteal sleeve avulsion (ALPSA). This refers to a medially displaced tear of the anterior labrum with intact periosteal stripping along the medial glenoid2with medial rotation and inferior displacement of the anterior inferior labrum along the scapular neck. An ALPSA lesion can heal via the intact periosteal blood supply. If not repaired, anterior instability will result because of malposition of the labrum, causing a patulous anterior capsule.3 When a corresponding lesion occurs in the posterior labrum because of a posterior dislocation, it is called a posterior labrocapsular periosteal sleeve avulsion (POLPSA) (Figure 4).

Another variant of the Bankart lesion is the Perthes lesion, which is a nondisplaced tear of the anteroinferior labrum with periosteal stripping. This differs from the ALPSA because the detached labrum and periosteum are held in anatomic position, possibly making the lesion difficult to detect on magnetic resonance arthrography (MRA).3 Obtaining images in the abduction external rotation (ABER) position exerts traction on the anterior inferior joint capsule and may make the Perthes lesion more conspicuous.4 When this occurs in the posterior labrum, it is called a reverse Perthes lesion (Figure 5).

In a patient with anterior glenohumeral instability without a Bankart lesion, pathology of the anterior band of the inferior glenohumeral ligament (IGHL) at its humeral attachment must be suspected. Humeral avulsion of the IGHL (HAGL) or its variants can be overlooked on arthroscopy. HAGL is diagnosed on MRA when the normally U-shaped IGHL takes on a J-shape, and joint fluid extravasates across the torn humeral attachment (Figure 6). If there is an avulsed bony fragment from the medial humeral neck, the lesion is termed a bony HAGL (BHAGL). In addition to the findings of a HAGL, a BHAGL shows the osseous fragment and donor site on MRI. Since a BHAGL is a bony avulsion, it can even be suggested on radiography if a bony fragment is seen adjacent to the medial humeral neck.5 These lesions are highly associated with other shoulder injuries, particularly Hill-Sachs deformities and subscapularis tendon tears, and it is imperative, therefore, to search for additional injuries if a HAGL-type injury is seen.6

A more uncommon type of HAGL can occur in the setting of posterior capsulolabral injury. A posterior-band IGHL avulsion from the humerus (PHAGL) has similar imaging findings to a HAGL, except that it involves the posterior band of the IGHL. PHAGLs are usually not associated with an acute injury and are thought to be related to repetitive microtrauma, perhaps since the posterior band of the IGHL is the thinnest portion of the IGHL complex.7

A Kim lesion is an arthroscopic finding described in patients with posterior instability as a superficial defect at the undersurface of the posterior labrum and adjacent glenoid cartilage without detachment or extension to the chondrolabral junction.8 It is, by its nature, a concealed finding on routine MRI but can be more conspicuous in FADIR (flexed, adducted, internally rotated) positioning on MRA, which exerts traction on the posterior joint capsule, allowing intra-articular contrast to fill the tear (Figure 7).

This list describes several of the most commonly encountered acronyms in shoulder MRI. A review of SLAP (superior labrum anterior to posterior) lesions was described in a previous article in the journal’s Imaging Series.9 A thorough understanding of these lesions is helpful in interpreting reports and determining the appropriate treatment for patients with shoulder injuries.

The widespread use of eponyms and acronyms to describe labroligamentous findings in the shoulder has made interpretation of shoulder magnetic resonance imaging (MRI) reports challenging. We review and discuss the appearance of these lesions on shoulder MRI to help the orthopedic surgeon understand these entities as imaging findings.

Glenolabral articular disruption (GLAD) occurs secondary to impaction of the humeral head on the glenoid articular cartilage. There is a resultant defect in the glenoid articular cartilage, which extends to the glenoid labrum. A GLAD lesion is diagnosed only if the glenohumeral ligament and scapular periosteum remain intact1 (Figure 1).

Complete detachment of the anteroinferior labrum with tearing of the anterior glenoid periosteum represents a Bankart lesion. Cartilaginous Bankart lesions are caused by an anterior glenohumeral dislocation with resultant avulsion of the anteroinferior labrum and disruption of the scapular periosteum because of acute traction on the anterior band of the inferior glenohumeral ligament (Figure 2). Anterior instability, caused by disruption of the anterior labroligamentous complex, results. Osseous Bankart lesions occur when the anterior displaced humeral head impacts the anterior inferior glenoid rim, causing a fracture (Figure 3). This loss of the glenoid articular surface area can result in glenohumeral instability. Posterior shoulder dislocations can result in corresponding findings in the posterior inferior glenoid labrum (reverse Bankart lesion) and anterior medial humeral head (reverse Hill-Sachs lesion) (Figure 2).

 

A variant of the Bankart lesion is the anterior labroligamentous periosteal sleeve avulsion (ALPSA). This refers to a medially displaced tear of the anterior labrum with intact periosteal stripping along the medial glenoid2with medial rotation and inferior displacement of the anterior inferior labrum along the scapular neck. An ALPSA lesion can heal via the intact periosteal blood supply. If not repaired, anterior instability will result because of malposition of the labrum, causing a patulous anterior capsule.3 When a corresponding lesion occurs in the posterior labrum because of a posterior dislocation, it is called a posterior labrocapsular periosteal sleeve avulsion (POLPSA) (Figure 4).

Another variant of the Bankart lesion is the Perthes lesion, which is a nondisplaced tear of the anteroinferior labrum with periosteal stripping. This differs from the ALPSA because the detached labrum and periosteum are held in anatomic position, possibly making the lesion difficult to detect on magnetic resonance arthrography (MRA).3 Obtaining images in the abduction external rotation (ABER) position exerts traction on the anterior inferior joint capsule and may make the Perthes lesion more conspicuous.4 When this occurs in the posterior labrum, it is called a reverse Perthes lesion (Figure 5).

In a patient with anterior glenohumeral instability without a Bankart lesion, pathology of the anterior band of the inferior glenohumeral ligament (IGHL) at its humeral attachment must be suspected. Humeral avulsion of the IGHL (HAGL) or its variants can be overlooked on arthroscopy. HAGL is diagnosed on MRA when the normally U-shaped IGHL takes on a J-shape, and joint fluid extravasates across the torn humeral attachment (Figure 6). If there is an avulsed bony fragment from the medial humeral neck, the lesion is termed a bony HAGL (BHAGL). In addition to the findings of a HAGL, a BHAGL shows the osseous fragment and donor site on MRI. Since a BHAGL is a bony avulsion, it can even be suggested on radiography if a bony fragment is seen adjacent to the medial humeral neck.5 These lesions are highly associated with other shoulder injuries, particularly Hill-Sachs deformities and subscapularis tendon tears, and it is imperative, therefore, to search for additional injuries if a HAGL-type injury is seen.6

A more uncommon type of HAGL can occur in the setting of posterior capsulolabral injury. A posterior-band IGHL avulsion from the humerus (PHAGL) has similar imaging findings to a HAGL, except that it involves the posterior band of the IGHL. PHAGLs are usually not associated with an acute injury and are thought to be related to repetitive microtrauma, perhaps since the posterior band of the IGHL is the thinnest portion of the IGHL complex.7

A Kim lesion is an arthroscopic finding described in patients with posterior instability as a superficial defect at the undersurface of the posterior labrum and adjacent glenoid cartilage without detachment or extension to the chondrolabral junction.8 It is, by its nature, a concealed finding on routine MRI but can be more conspicuous in FADIR (flexed, adducted, internally rotated) positioning on MRA, which exerts traction on the posterior joint capsule, allowing intra-articular contrast to fill the tear (Figure 7).

This list describes several of the most commonly encountered acronyms in shoulder MRI. A review of SLAP (superior labrum anterior to posterior) lesions was described in a previous article in the journal’s Imaging Series.9 A thorough understanding of these lesions is helpful in interpreting reports and determining the appropriate treatment for patients with shoulder injuries.

References

1.    Sanders TG, Tirman PF, Linares R, Feller JF, Richardson R. The glenolabral articular disruption lesion: MR arthrography with arthroscopic correlation. AJR Am J Roentgenol. 1999;172(1):171-175.

2.    Beltran J, Jbara M, Maimon R. Shoulder: labrum and bicipital tendon. Top Magn Reson Imaging. 2003;14(1):35-50.

3.    Waldt S, Burkart A, Imhoff AB, Bruegel M, Rummeny EJ, Woertler K. Anterior shoulder instability: accuracy of MR arthrography in the classification of anteroinferior labroligamentous injuries. Radiology. 2005;237(2):578-583.

4.    Schreinemachers SA, van der Hulst VP, Willems J, Bipat S, van der Woude H. Is a single direct MR arthrography series in ABER position as accurate in detecting anteroinferior labroligamentous lesions as conventional MR arthrography? Skeletal Radiol. 2009;38(7):675-683.

5.    Bui-Mansfield LT, Taylor DC, Uhorchak JM, Tenuta JT. Humeral avulsions of the glenohumeral ligament: imaging features and a review of the literature. AJR Am J Roentgenol. 2002;179(3):649-655.

6.    Magee T. Prevalence of HAGL lesions and associated abnormalities on shoulder MR examination. Skeletal Radiol. 2014;43(3):307-313.

7.    Chung CB, Sorenson S, Dwek JR, Resnick D. Humeral avulsion of the posterior band of the inferior glenohumeral ligament: MR arthrography and clinical correlation in 17 patients. AJR Am J Roentgenol. 2004;183(2):355-359.

8.    Kim SH, Ha KI, Yoo JC, Noh KC. Kim’s lesion: an incomplete and concealed avulsion of the posteroinferior labrum in posterior or multidirectional posteroinferior instability of the shoulder. Arthroscopy. 2004;20(7):712-720.

9.    Grubin J, Maderazo A, Fitzpatrick D. Imaging evaluation of superior labral anteroposterior (SLAP) tears. Am J Orthop. 2015;44(10):476-477.

References

1.    Sanders TG, Tirman PF, Linares R, Feller JF, Richardson R. The glenolabral articular disruption lesion: MR arthrography with arthroscopic correlation. AJR Am J Roentgenol. 1999;172(1):171-175.

2.    Beltran J, Jbara M, Maimon R. Shoulder: labrum and bicipital tendon. Top Magn Reson Imaging. 2003;14(1):35-50.

3.    Waldt S, Burkart A, Imhoff AB, Bruegel M, Rummeny EJ, Woertler K. Anterior shoulder instability: accuracy of MR arthrography in the classification of anteroinferior labroligamentous injuries. Radiology. 2005;237(2):578-583.

4.    Schreinemachers SA, van der Hulst VP, Willems J, Bipat S, van der Woude H. Is a single direct MR arthrography series in ABER position as accurate in detecting anteroinferior labroligamentous lesions as conventional MR arthrography? Skeletal Radiol. 2009;38(7):675-683.

5.    Bui-Mansfield LT, Taylor DC, Uhorchak JM, Tenuta JT. Humeral avulsions of the glenohumeral ligament: imaging features and a review of the literature. AJR Am J Roentgenol. 2002;179(3):649-655.

6.    Magee T. Prevalence of HAGL lesions and associated abnormalities on shoulder MR examination. Skeletal Radiol. 2014;43(3):307-313.

7.    Chung CB, Sorenson S, Dwek JR, Resnick D. Humeral avulsion of the posterior band of the inferior glenohumeral ligament: MR arthrography and clinical correlation in 17 patients. AJR Am J Roentgenol. 2004;183(2):355-359.

8.    Kim SH, Ha KI, Yoo JC, Noh KC. Kim’s lesion: an incomplete and concealed avulsion of the posteroinferior labrum in posterior or multidirectional posteroinferior instability of the shoulder. Arthroscopy. 2004;20(7):712-720.

9.    Grubin J, Maderazo A, Fitzpatrick D. Imaging evaluation of superior labral anteroposterior (SLAP) tears. Am J Orthop. 2015;44(10):476-477.

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3D imaging tracks causes of post-TAVR aortic regurgitation

Approach could translate to multislice CT
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3D imaging tracks causes of post-TAVR aortic regurgitation

Three-dimensional transesophageal echocardiography identified several significant predictors of aortic regurgitation after transcatheter aortic valve replacement, according to a study published online Jan. 5 in JACC Cardiovascular Imaging.

“This is the first study to demonstrate that large prosthetic expansion, elliptical prosthetic shape, and anti-anatomical position are 3D features associated with transvalvular AR,” said Dr. Kentaro Shibayama of Cedars-Sinai Heart Institute in Los Angeles, and his associates. The study also showed that paravalvular AR was inversely related to effective area oversizing, the investigators said (JACC Cardiovasc Imag. 2016 Jan. 6).

Post-TAVR AR continues to affect substantial numbers of patients, despite progress in prosthesis design. Past research has linked paravalvular AR to prosthetic undersizing, long-axis malpositioning, and aortic annular calcification, but the causes of transvalvular AR have not been adequately studied, the researchers said. Using intraprocedural 3D transesophageal echocardiography, they imaged the native annuluses and postoperative prosthetic valves of 201 patients with severe aortic stenosis who received the Edwards SAPIEN device. The investigators also used transthoracic echocardiography to separately grade post-TAVR transvalvular and paravalvular AR as none or trivial, mild, moderate, or severe according to the 2012 Valve Academic Research Consortium criteria (J Am Coll Cardiol. 2012;60:1438-54).Fully 44% of patients developed mild or moderate aortic regurgitation after TAVR, while the rest had no or trivial AR, the investigators said. About three-quarters of AR cases were mild, nearly 25% were moderate, and none were severe. Only 3% of patients had transvalvular AR only, 34% had paravalvular AR only, and 7% had both types of AR. Patients with post-TAVR transvalvular AR had significantly more prosthetic expansion (P less than .05), a more elliptical prosthetic shape at the level of the prosthetic commissure (P less than .01), and malpositioning of the prosthetic commissures in relation to the native commissures (P less than .001), compared with patients without transvalvular AR.

Patients were more likely to have paravalvular AR if they had a lower percentage of effective area oversizing, defined as the prosthetic frame area divided by the area of the native aortic annulus (odds ratio, 0.97; 95% CI: 0.93-0.99, P less than .05). “A mismatch between a larger native aortic valve annulus area and a smaller deployed prosthesis found by intra-procedural 3D TEE may increase the risk of developing mild or greater paravalvular AR,” the researchers explained. Older age also was slightly but significantly linked with mild or moderate paravalvular AR(OR, 1.05; 95% CI, 1.01-1.09, P less than .05).

“Abnormalities related to transvalvular AR after TAVR found in this study may contribute to further deterioration of the prosthesis, warranting careful prospective studies to assess the long-term prognosis of these patients,” the investigators concluded. They cautioned that the number of patients with post-TAVR transvalvular AR was too small to carry out detailed analyses.

The researchers reported no funding sources. Senior author Dr. Takahiro Shiota reported being a speaker for Philips Ultrasound, and three of the other seven coinvestigators reported financial relationships with Edwards, Medtronic, Abbott, Capricor, St. Jude Medical, Philips Ultrasound, and Venus Medtech.

References

Body

This study is important because it reinforces the important role that 3D TEE can play in procedural planning for TAVR and in predicting which patients are more likely to suffer from post-TAVR AR. It is the first study that has highlighted the practical utilization of 3D TEE in this way.

Multislice computed tomography is the preferred imaging modality for TAVR planning in many centers. However, since the imaging resolution of both techniques is similar, and they both have software capable of generating multiplane reconstructions from 3D datasets, I believe that the skill and experience of the imaging expert analyzing the datasets are more important than the modality itself, and the results from this study could probably translate to MSCT.

Although the manufacturers of TAVR valves would have us believe that the issue of postimplant AR has largely been solved by newer valve design, it still remains an important issue and will continue to be so as the technique competes with surgical alternatives. Imaging will continue to play a pivotal role in procedure planning and guidance and, as has been demonstrated by Shibayama et al., 3D TEE can be extremely useful for anticipating and potentially avoiding post-TAVR AR.

Mark Monaghan, Ph.D., is the FESC director of noninvasive cardiology at King’s College Hospital Denmark Hill in London. These comments were taken from his editorial (JACC Cardiovasc Imaging 2016 Jan. 6).

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This study is important because it reinforces the important role that 3D TEE can play in procedural planning for TAVR and in predicting which patients are more likely to suffer from post-TAVR AR. It is the first study that has highlighted the practical utilization of 3D TEE in this way.

Multislice computed tomography is the preferred imaging modality for TAVR planning in many centers. However, since the imaging resolution of both techniques is similar, and they both have software capable of generating multiplane reconstructions from 3D datasets, I believe that the skill and experience of the imaging expert analyzing the datasets are more important than the modality itself, and the results from this study could probably translate to MSCT.

Although the manufacturers of TAVR valves would have us believe that the issue of postimplant AR has largely been solved by newer valve design, it still remains an important issue and will continue to be so as the technique competes with surgical alternatives. Imaging will continue to play a pivotal role in procedure planning and guidance and, as has been demonstrated by Shibayama et al., 3D TEE can be extremely useful for anticipating and potentially avoiding post-TAVR AR.

Mark Monaghan, Ph.D., is the FESC director of noninvasive cardiology at King’s College Hospital Denmark Hill in London. These comments were taken from his editorial (JACC Cardiovasc Imaging 2016 Jan. 6).

Body

This study is important because it reinforces the important role that 3D TEE can play in procedural planning for TAVR and in predicting which patients are more likely to suffer from post-TAVR AR. It is the first study that has highlighted the practical utilization of 3D TEE in this way.

Multislice computed tomography is the preferred imaging modality for TAVR planning in many centers. However, since the imaging resolution of both techniques is similar, and they both have software capable of generating multiplane reconstructions from 3D datasets, I believe that the skill and experience of the imaging expert analyzing the datasets are more important than the modality itself, and the results from this study could probably translate to MSCT.

Although the manufacturers of TAVR valves would have us believe that the issue of postimplant AR has largely been solved by newer valve design, it still remains an important issue and will continue to be so as the technique competes with surgical alternatives. Imaging will continue to play a pivotal role in procedure planning and guidance and, as has been demonstrated by Shibayama et al., 3D TEE can be extremely useful for anticipating and potentially avoiding post-TAVR AR.

Mark Monaghan, Ph.D., is the FESC director of noninvasive cardiology at King’s College Hospital Denmark Hill in London. These comments were taken from his editorial (JACC Cardiovasc Imaging 2016 Jan. 6).

Title
Approach could translate to multislice CT
Approach could translate to multislice CT

Three-dimensional transesophageal echocardiography identified several significant predictors of aortic regurgitation after transcatheter aortic valve replacement, according to a study published online Jan. 5 in JACC Cardiovascular Imaging.

“This is the first study to demonstrate that large prosthetic expansion, elliptical prosthetic shape, and anti-anatomical position are 3D features associated with transvalvular AR,” said Dr. Kentaro Shibayama of Cedars-Sinai Heart Institute in Los Angeles, and his associates. The study also showed that paravalvular AR was inversely related to effective area oversizing, the investigators said (JACC Cardiovasc Imag. 2016 Jan. 6).

Post-TAVR AR continues to affect substantial numbers of patients, despite progress in prosthesis design. Past research has linked paravalvular AR to prosthetic undersizing, long-axis malpositioning, and aortic annular calcification, but the causes of transvalvular AR have not been adequately studied, the researchers said. Using intraprocedural 3D transesophageal echocardiography, they imaged the native annuluses and postoperative prosthetic valves of 201 patients with severe aortic stenosis who received the Edwards SAPIEN device. The investigators also used transthoracic echocardiography to separately grade post-TAVR transvalvular and paravalvular AR as none or trivial, mild, moderate, or severe according to the 2012 Valve Academic Research Consortium criteria (J Am Coll Cardiol. 2012;60:1438-54).Fully 44% of patients developed mild or moderate aortic regurgitation after TAVR, while the rest had no or trivial AR, the investigators said. About three-quarters of AR cases were mild, nearly 25% were moderate, and none were severe. Only 3% of patients had transvalvular AR only, 34% had paravalvular AR only, and 7% had both types of AR. Patients with post-TAVR transvalvular AR had significantly more prosthetic expansion (P less than .05), a more elliptical prosthetic shape at the level of the prosthetic commissure (P less than .01), and malpositioning of the prosthetic commissures in relation to the native commissures (P less than .001), compared with patients without transvalvular AR.

Patients were more likely to have paravalvular AR if they had a lower percentage of effective area oversizing, defined as the prosthetic frame area divided by the area of the native aortic annulus (odds ratio, 0.97; 95% CI: 0.93-0.99, P less than .05). “A mismatch between a larger native aortic valve annulus area and a smaller deployed prosthesis found by intra-procedural 3D TEE may increase the risk of developing mild or greater paravalvular AR,” the researchers explained. Older age also was slightly but significantly linked with mild or moderate paravalvular AR(OR, 1.05; 95% CI, 1.01-1.09, P less than .05).

“Abnormalities related to transvalvular AR after TAVR found in this study may contribute to further deterioration of the prosthesis, warranting careful prospective studies to assess the long-term prognosis of these patients,” the investigators concluded. They cautioned that the number of patients with post-TAVR transvalvular AR was too small to carry out detailed analyses.

The researchers reported no funding sources. Senior author Dr. Takahiro Shiota reported being a speaker for Philips Ultrasound, and three of the other seven coinvestigators reported financial relationships with Edwards, Medtronic, Abbott, Capricor, St. Jude Medical, Philips Ultrasound, and Venus Medtech.

Three-dimensional transesophageal echocardiography identified several significant predictors of aortic regurgitation after transcatheter aortic valve replacement, according to a study published online Jan. 5 in JACC Cardiovascular Imaging.

“This is the first study to demonstrate that large prosthetic expansion, elliptical prosthetic shape, and anti-anatomical position are 3D features associated with transvalvular AR,” said Dr. Kentaro Shibayama of Cedars-Sinai Heart Institute in Los Angeles, and his associates. The study also showed that paravalvular AR was inversely related to effective area oversizing, the investigators said (JACC Cardiovasc Imag. 2016 Jan. 6).

Post-TAVR AR continues to affect substantial numbers of patients, despite progress in prosthesis design. Past research has linked paravalvular AR to prosthetic undersizing, long-axis malpositioning, and aortic annular calcification, but the causes of transvalvular AR have not been adequately studied, the researchers said. Using intraprocedural 3D transesophageal echocardiography, they imaged the native annuluses and postoperative prosthetic valves of 201 patients with severe aortic stenosis who received the Edwards SAPIEN device. The investigators also used transthoracic echocardiography to separately grade post-TAVR transvalvular and paravalvular AR as none or trivial, mild, moderate, or severe according to the 2012 Valve Academic Research Consortium criteria (J Am Coll Cardiol. 2012;60:1438-54).Fully 44% of patients developed mild or moderate aortic regurgitation after TAVR, while the rest had no or trivial AR, the investigators said. About three-quarters of AR cases were mild, nearly 25% were moderate, and none were severe. Only 3% of patients had transvalvular AR only, 34% had paravalvular AR only, and 7% had both types of AR. Patients with post-TAVR transvalvular AR had significantly more prosthetic expansion (P less than .05), a more elliptical prosthetic shape at the level of the prosthetic commissure (P less than .01), and malpositioning of the prosthetic commissures in relation to the native commissures (P less than .001), compared with patients without transvalvular AR.

Patients were more likely to have paravalvular AR if they had a lower percentage of effective area oversizing, defined as the prosthetic frame area divided by the area of the native aortic annulus (odds ratio, 0.97; 95% CI: 0.93-0.99, P less than .05). “A mismatch between a larger native aortic valve annulus area and a smaller deployed prosthesis found by intra-procedural 3D TEE may increase the risk of developing mild or greater paravalvular AR,” the researchers explained. Older age also was slightly but significantly linked with mild or moderate paravalvular AR(OR, 1.05; 95% CI, 1.01-1.09, P less than .05).

“Abnormalities related to transvalvular AR after TAVR found in this study may contribute to further deterioration of the prosthesis, warranting careful prospective studies to assess the long-term prognosis of these patients,” the investigators concluded. They cautioned that the number of patients with post-TAVR transvalvular AR was too small to carry out detailed analyses.

The researchers reported no funding sources. Senior author Dr. Takahiro Shiota reported being a speaker for Philips Ultrasound, and three of the other seven coinvestigators reported financial relationships with Edwards, Medtronic, Abbott, Capricor, St. Jude Medical, Philips Ultrasound, and Venus Medtech.

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Key clinical point: Three-dimensional transesophageal echocardiography identified significant predictors of aortic regurgitation after transcatheter aortic valve replacement.

Major finding: Patients with post-TAVR transvalvular AR had significantly more prosthetic expansion (P less than .05), a more elliptical prosthetic shape (P less than .01), and malpositioning of the prosthetic commissures (P less than .001) compared with patients without transvalvular AR.

Data source: A 3D TEE study of 201 patients with severe aortic stenosis who underwent TAVR with the Edwards SAPIEN device.

Disclosures: The investigators reported no funding sources. Senior author Dr. Takahiro Shiota reported being a speaker for Philips Ultrasound, and three of the other seven coinvestigators reported financial relationships with Edwards, Medtronic, Abbott, Capricor, St. Jude Medical, Philips Ultrasound, and Venus Medtech.

Managing interstitial lung disease detected on CT during lung cancer screening

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Managing interstitial lung disease detected on CT during lung cancer screening

Primary care physicians are playing a bigger role in evaluating the incidental finding of interstitial lung diseases since the recent publication of guidelines recommending computed tomography (CT) to screen for lung cancer.

In August 2011, the National Cancer Institute published its findings from the National Lung Screening Trial, which demonstrated a 20% reduction in mortality from lung cancer in patients at high risk screened with low-dose CT.1 Based on these results, the American Cancer Society, the American College of Chest Physicians, the American Society of Clinical Oncology, and the National Comprehensive Cancer Network recommended annual screening for lung cancer with low-dose CT in adults ages 55 to 74 who have a 30-pack-year smoking history and who currently smoke or have quit within the past 15 years.2 In December 2013, the US Preventive Services Task Force published similar guidelines but increased the age range to include high-risk patients ages 55 to 80.3

Bach et al4 estimated that, in 2010 in the United States, 8.6 million people met the criteria used in the National Lung Screening Trial for low-dose CT screening. These are the same criteria as in the multisociety recommendations cited above.2 With such large numbers of patients eligible for CT screening, internists and other primary care physicians are undoubtedly encountering the incidental discovery of nonmalignant pulmonary diseases such as interstitial lung disease.

This article reviews the radiographic characteristics of the most common interstitial lung diseases the internist may encounter on screening CT in long-term smokers.

Referral to a specialist has been associated with lower rates of morbidity and death,5 and a diagnosis of interstitial lung disease should be confirmed by a pulmonologist and a radiologist specializing in differentiating the subtypes. But the primary care physician now plays a critical role in recognizing the need for further evaluation.

HOW COMMON IS INTERSTITIAL LUNG DISEASE IN SMOKERS?

Several studies have published data on the prevalence of interstitial lung disease in patients undergoing low-dose CT for lung cancer screening.

A trial at Mayo Clinic in current and former smokers identified “diffuse lung disease” in 9 (0.9%) of 1,049 participants.6

A trial in Ireland identified idiopathic pulmonary fibrosis in 6 (1.3%) of 449 current smokers who underwent low-dose CT screening for lung cancer.7

Sverzellati et al8 evaluated 692 participants in the Multicentric Italian Lung Detection CT screening study and reported a respiratory bronchiolitis pattern in 109 (15.7%), a usual interstitial pneumonia pattern in 2 (0.3%), and other patterns of chronic interstitial pneumonia in 26 (3.8%).

The National Lung Screening Trial reported that the frequency of “clinically significant” incidental findings (including pulmonary fibrosis) in all participants was 7.5%.1 A retrospective analysis of 884 participants at a single site in this trial identified interstitial lung abnormalities in 86 participants (9.7%).9 These abnormalities were further categorized as nonfibrotic in 52 (5.9%) of 884, fibrotic in 19 (2.1%) of 884, and mixed fibrotic and nonfibrotic in 15 (1.7%) of 884.

Follow-up CT at 2 years in this trial demonstrated improvement in 50% and progression in 11% of patients who had nonfibrotic abnormalities, while fibrotic abnormalities improved in no cases and progressed in 37%. Interstitial lung abnormalities were more common in those who currently smoked and in those with more pack-years of cigarette smoking.9

In sum, these trials suggest that low-dose CT screening for lung cancer can detect the most common forms of interstitial lung disease in this at-risk population and can characterize them as fibrotic or nonfibrotic, a distinction important for prognosis and subsequent management.

NONFIBROTIC VS FIBROTIC DISEASE

It is important to distinguish between nonfibrotic and fibrotic interstitial lung disease, as fibrotic disease carries a worse prognosis and is treated differently.

Features of nonfibrotic interstitial lung disease:

  • Ground-glass opacities
  • Nodules
  • Mosaic attenuation or consolidation.

Features of fibrotic interstitial lung disease:

  • Combination of ground-glass opacities and reticulation
  • Reticulation by itself
  • Traction bronchiectasis
  • Honeycombing
  • Loss of lung volume.

NONFIBROTIC INTERSTITIAL LUNG DISEASES

Given the strong likelihood that a patient undergoing screening CT is either a current or former smoker, physicians may encounter, in addition to emphysema and lung cancer, the following smoking-related interstitial lung diseases, which are primarily nonfibrotic and which frequently coexist (Table 1):

  • Respiratory bronchiolitis
  • Respiratory bronchiolitis-interstitial lung disease
  • Desquamative interstitial pneumonia
  • Pulmonary Langerhans cell histiocytosis.

Respiratory bronchiolitis

Respiratory bronchiolitis occurs mostly in smokers and does not necessarily lead to respiratory symptoms in all patients.10 It cannot always be identified radiographically but occasionally appears as predominantly upper-lobe, patchy ground-glass opacities or ill-defined centrilobular nodules without evidence of fibrosis (Figure 1).

Respiratory bronchiolitis
Figure 1. In a 36-year-old woman with an 18-pack-year smoking history, high-resolution CT shows respiratory bronchiolitis in the upper lungs, with bilateral centrilobular ground-glass nodules (red arrow), patchy ground-glass opacities (white solid arrow) and bronchial wall thickening (white dashed arrow).

Respiratory bronchiolitis-interstitial lung disease

In rare cases, respiratory bronchiolitis leads to peribronchial fibrosis invading the alveolar walls, which is then classified as respiratory bronchiolitis-interstitial lung disease.11 The CT findings in respiratory bronchiolitis-interstitial lung disease are upper-lobe-predominant centrilobular ground-glass nodules, patchy ground-glass opacities, and bronchial wall thickening (Figure 2).10 Occasionally, mild reticulation is noted without honeycombing. Mild air trapping can be seen in the lower lobes, with centrilobular emphysema in the upper lobes.12

Respiratory bronchiolitis-interstitial lung disease
Figure 2. In a 40-year-old woman with a 15-pack-year smoking history with respiratory bronchiolitis-interstitial lung disease, high-resolution CT obtained through the upper lungs at initial diagnosis (left) shows diffuse faint centrilobular ground-glass nodules (red arrows). High-resolution CT 1 year after smoking cessation (right) shows complete resolution of the centrilobular nodules.

The only successful therapy for respiratory bronchiolitis and respiratory bronchiolitis-interstitial lung disease is smoking cessation. Finding either of these diseases should prompt aggressive counseling by the internist and consideration of referral to a specialist in interstitial lung disease.

Desquamative interstitial pneumonia

Although pathologically different from respiratory bronchiolitis-interstitial lung disease, desquamative interstitial pneumonia has a similar clinical and radiographic presentation. Because their features significantly overlap, they are considered a pathomorphologic continuum, representing degrees of severity of the same disease process caused by prolonged tobacco inhalation.10,13

Widespread ground-glass opacities are the predominant CT finding. These are bilateral and symmetric in distribution in 86%, basal and peripheral in 60%, patchy in 20%, and diffuse in 20% (Figure 3).14 Other frequent findings are mild reticulation with traction bronchiectasis and coexistent emphysema (Figure 4).15 The small peripheral cystic spaces noted in this disease most likely represent dilated bronchioles and alveolar ducts rather than honeycombing.16

Desquamative interstitial pneumonia
Figure 3. In a 49-year-old woman with a 23-pack-year history of smoking, high-resolution CT obtained through the lower lungs shows features of desquamative interstitial pneumonia: diffuse ground-glass opacities predmoninantly in the mid-lower lung. No traction bronchiectasis or architectural distortion is seen.

Desquamative interstitial pneumonia and emphysema
Figure 4. In a 38-year-old man with a 20-pack-year history of smoking, coronal image reformatting shows features of desquamative interstitial pneumonia and emphysema, ie, diffuse distribution of ground-glass opacities with cystic airspaces representing emphysema (red arrow).

No additional treatment beyond elimination of smoking has been proven effective for desquamative interstitial pneumonia, and patients who manage to quit smoking generally have a favorable prognosis.17,18

Pulmonary Langerhans cell histiocytosis

The combination of upper-lobe-predominant cysts and nodules in a young heavy smoker are diagnostic of pulmonary Langerhans cell histiocytosis. The cysts are bizarrely shaped, thin- or thick-walled, and nonuniform in size (Figure 5). The irregular cavitary nodules are centrilobular. The disease characteristically spares the costophrenic angles.

Langerhans cell histiocytosis
Figure 5. In a 46-year-old woman with a 26-pack-year history of smoking, a coronal reformatted image shows various-sized cysts (solid white arrows) and nodules (dashed white arrow) in the upper and midlung zones—a characteristic imaging pattern of smoking-related pulmonary Langerhans cell histiocytosis, which was later confirmed with biopsy in this patient.

Spontaneous pneumothorax is the initial clinical presentation in 15% of patients.16 In the early stages of the disease (nodule-predominant disease without cysts), infection and metastatic disease need to be excluded (Figure 6). In the later stages, the cysts become coalescent, making the distinction between this disease and “burned-out” lymphangioleiomyomatosis or severe emphysema extremely difficult (Figure 7).17 Smoking cessation and corticosteroids are the mainstay of medical therapy for pulmonary Langerhans cell histiocytosis, and about 50% of patients who quit smoking and receive corticosteroids demonstrate partial or complete clearing of the radiographic abnormalities and symptoms (Figure 8).

Pulmonary Langerhans cell histiocytosis
Figure 6. Early-stage pulmonary Langerhans cell histiocytosis (nodule-predominant) mimics metastasis in a 56-year-old woman with a 19-pack-year history of smoking. High-resolution CT obtained through the midlungs shows few irregularly marginated nodules (red arrow), one of which is cavitary (white arrow). There is a background of centrilobular emphysema. No cysts are seen. Histologic study confirmed the diagnosis.
Pulmonary Langerhans cell histiocytosis
Figure 7. In a 49-year-old male smoker with biopsy-confirmed pulmonary Langerhans cell histiocytosis, high-resolution CT obtained through the upper lungs shows numerous well-circumscribed, thin-walled cysts with absence of nodules. This appearance is typical of advanced (“burned out”) pulmonary Langerhans cell histiocytosis and is virtually indistinguishable from emphysema on high-resolution CT.

Pulmonary Langerhans cell histiocytosis
Figure 8. In a 41-year-old woman with a 22-pack-year history of smoking and pulmonary Langerhans cell histiocytosis, high-resolution CT through the midlungs at the time of initial diagnosis (left) shows a few solid (red arrow) and cavitary (white arrow) nodules. The lung bases were spared. No cysts were seen. High-resolution CT 2 years after smoking cessation and oral corticosteroid therapy (right) shows near-complete resolution of the nodules.

FIBROTIC INTERSTITIAL LUNG DISEASES

If CT identifies a diffuse fibrotic pattern, the two most common possibilities (Table 2) are:

  • Nonspecific interstitial pneumonia
  • Usual interstitial pneumonia.

As noted above, these carry a worse prognosis than the nonfibrotic interstitial lung diseases.

Nonspecific interstitial pneumonia

While most frequently idiopathic, the nonspecific interstitial pneumonia pattern can often be seen in connective tissue diseases. It has also been associated with chronic hypersensitivity pneumonitis, drug toxicity, and slowly resolving diffuse alveolar damage.19 Although it is not the only pathologic pattern in interstitial lung disease associated with connective tissue disease, it is the most common pattern in systemic sclerosis, systemic lupus erythematosus, dermatomyositis-polymyositis, and mixed connective tissue disease.20

The parenchymal changes are typically subpleural and symmetric in distribution (Figure 9). In about one-third of cases, there is a peribronchovascular distribution of the abnormalities (Figure 10).

Nonspecific interstitial pneumonia
Figure 9. In a 53-year-old woman with mild dyspnea, high-resolution CT through the lower lungs shows bilateral symmetric peripheral distribution of ground-glass opacities, a feature of nonspecific interstitial pneumonia.

Nonspecific interstitial pneumonia
Figure 10. High-resolution CT through the lower lungs in a 60-year-old woman with nonspecific interstitial pneumonia shows bilateral symmetric peribronchovascular distribution of ground-glass and reticular opacities with traction bronchiectasis.

Ground-glass opacities are the dominant imaging findings, seen in 80% of cases.18 In advanced disease (also referred to as fibrotic nonspecific interstitial pneumonia), patients have accompanying fine or coarse reticular opacities, traction bronchiectasis, and consolidation (Figure 11). Honeycombing is seen in 1% to 5% of patients.21

Nonspecific interstitial pneumonia
Figure 11. High-resolution CT through the lower lungs in a 45-year-old woman with scleroderma and nonspecific interstitial pneumonia shows bilateral symmetric peripheral ground-glass attenuation with reticular opacities, traction bronchiectasis (white solid arrow), traction bronchiolectasis (white dashed arrows), and subpleural sparing. A dilated esophagus (red arrow) correlates with the history of scleroderma.

The most specific sign of nonspecific interstitial pneumonia is sparing of the immediate subpleural lung, apparent in 30% to 50% of patients (Figure 12).22 Subpleural sparing with a peribronchovascular distribution of abnormalities, absence of lobular areas with decreased attenuation, and lack of honeycombing are imaging features that increase the diagnostic confidence of nonspecific interstitial pneumonia (Table 3).23 Clinically, compared with those who have usual interstitial pneumonia (see below), patients are younger and more of them are female. These patients also present with extrapulmonary manifestations such as joint involvement, rash, and Raynaud phenomenon. Therefore, these associated symptoms on presentation can help distinguish nonspecific interstitial pneumonia or usual interstitial pneumonia related to connective tissue disease from the idiopathic forms.

Nonspecific interstitial pneumonia
Figure 12. In a 52-year-old woman with mild dyspnea and fatigue, high-resolution CT through the lower lungs shows bilateral symmetric peripheral ground-glass opacities with sparing of the immediate subpleural lung (white arrows), the most specific sign of nonspecific interstitial pneumonia.

The first step in managing nonspecific interstitial pneumonia is to remove all potential exposure to inhaled substances or to drugs. Although immunosuppressive therapy has never been studied in a randomized controlled trial in this disease, numerous reports suggest that patients may respond to prednisone and to steroid-sparing immunosuppressants.24

In several studies, survival rates in nonspecific interstitial pneumonia were significantly greater than in usual interstitial pneumonia independent of the treatment strategy. In long-term follow-up of patients with idiopathic nonspecific interstitial pneumonia treated with immunosuppressive therapy, two-thirds remained stable or improved.25–27

Although most connective tissue diseases cause a lung pattern of nonspecific interstitial pneumonia, some (eg, rheumatoid arthritis) may present with a pattern of usual interstitial pneumonia. In these cases and in those of advanced fibrotic nonspecific interstitial pneumonia, the prognosis is worse, as the disease is less responsive to immunosuppressive therapy.20

Usual interstitial pneumonia

Usual interstitial pneumonia is the most severe form of lung fibrosis. Most cases are idio­pathic and are termed idiopathic pulmonary fibrosis. Other causes of the usual interstitial pneumonia pattern include domestic and occupational environmental exposures, connective tissue disease, and drug toxicity.28 An epidemiologic association between smoking and usual interstitial pneumonia is well documented.28

Idiopathic pulmonary fibrosis typically affects men ages 50 to 70. Because its risk factors coincide with those of lung cancer, there is a high likelihood of detecting idiopathic pulmonary fibrosis early in this screening population. It has an especially poor prognosis, with a mean survival of 2 to 5 years from the time of diagnosis.18

The distribution of disease in usual interstitial pneumonia is characteristically subpleural and basal. CT features include coarse subpleural reticulation and honeycombing combined with traction bronchiectasis or bronchiolectasis and architectural distortion (Figure 13).18 Honeycombing is the most specific and key diagnostic CT finding for establishing a definitive diagnosis of usual interstitial pneumonia.29 However, ground-glass opacities are present in most patients, typically in the region of interstitial fibrosis, and are always less extensive than the reticulation.30 The findings demonstrate morphologic heterogeneity, with areas of fibrosis adjacent to areas of normal lung (Figure 14).

Figure 13. In a 68-year-old man, high-resolution CT through the lower lungs shows features of usual interstitial pneumonia: bilateral subpleural reticular opacities, traction bronchiectasis (blue arrow), ground-glass opacities (red arrow), and honeycombing, seen as rows of clustered subpleural cystic air spaces (yellow arrow).

Usual interstitial pneumonia
Figure 14. In a 67-year-old man with usual interstitial pneumonia, high-resolution CT through the lower lungs shows basal-predominant subpleural honey-combing (red arrow). Morphologic heterogeneity is seen with areas of fibrosis immediately adjacent to normal lung (white double arrow).

In addition to the aforementioned imaging features, the 2011 American Thoracic Society and European Respiratory Society joint guidelines for the CT diagnosis of usual interstitial pneumonia patterns require the absence of atypical features that suggest an alternative diagnosis, including those seen in nonspecific interstitial pneumonia, such as an upper, midlung, or peribronchovascular distribution and extensive ground-glass attenuation.28 Mild mediastinal lymphadenopathy (usually < 1.5 cm in the short axis) is common in usual interstitial pneumonia.31

Because other chronic interstitial pneumonias that may resemble usual interstitial pneumonia have a more favorable course and may respond to immunosuppressive therapy, establishing an early and accurate diagnosis is of the utmost importance.5 Additionally, the emergence of possible new therapies for idiopathic pulmonary fibrosis makes early referral to a specialist paramount in these cases. Recent studies have demonstrated significant slowing of the progression of disease in idiopathic pulmonary fibrosis with both pirfenidone and nintedanib.32,33

DIAGNOSIS AND MANAGEMENT

Managing interstitial lung disease found on screening CT
Figure 15.

The diagnosis of these nonfibrotic and fibrotic lung diseases is complex. In all cases in which interstitial lung disease is detected on screening CT for lung cancer, the internist should strongly consider further evaluation with dedicated high-resolution CT and early referral to a specialist (Figure 15).

Because smoking cessation is the only recommended treatment for nonfibrotic smoking-related interstitial lung diseases, particular emphasis on smoking cessation counseling is essential.

Referral for bronchoscopy with transbronchial lung biopsy is generally not helpful in the diagnosis of the interstitial lung diseases discussed in this article unless there is a need to rule out infection or neoplasm.34 Referral for surgical lung biopsy may be indicated in some cases of suspected pulmonary Langerhans cell histiocytosis, desquamative interstitial pneumonia, nonspecific interstitial pneumonia, or usual interstitial pneumonia if the diagnosis is uncertain (Tables 1 and 2).35

The American Thoracic Society/European Respiratory Society guidelines suggest a multidisciplinary team approach that includes a pathologist, radiologist, and clinician.35 This approach more readily determines the correct diagnosis and relies less on invasive methods such as surgical biopsy and more on noninvasive methods such as radiology and clinical history. Overall, this will promote earlier access to appropriate therapies, clinical trial enrollment, and in more severe cases, lung transplant.

Currently, 23% of all lung transplants worldwide are performed in patients with idiopathic pulmonary fibrosis. Other forms of pulmonary fibrosis account for 3% to 4% of lung transplants performed.36

Evidence suggests that early referral reduces rates of morbidity and death in these patients. The results of a single-center study37 of patients with idiopathic pulmonary fibrosis demonstrated that a longer delay from the onset of symptoms to evaluation by a specialist at a tertiary care referral center was associated with a higher rate of death from this disease independent of disease severity. Those with the longest delay in referral had a multivariable-adjusted death rate 3.4 times higher than those with the shortest delay.5,37

In summary, with implementation of the new lung cancer screening guidelines, primary care physicians are more often encountering the incidental finding of interstitial lung disease in their patients. Prompt diagnosis of interstitial lung disease helps ensure that patients receive appropriate care and early consideration for clinical trials and lung transplant.

Primary care physicians play a critical role in the initial identification of key characteristics of the interstitial abnormality—namely, whether the pattern is nonfibrotic or fibrotic—and in the correlation of the history and physical findings to expedite the diagnosis. Subsequently, ordering high-resolution CT for more detailed characterization and prompt referral to a specialist in interstitial lung disease allow for a more rapid and accurate diagnosis, specialized therapy, and supportive care.

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Brian D. Southern, MD
Respiratory Institute, Cleveland Clinic; Assistant Professor, Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Cleveland, OH

Rachel G. Scheraga, MD
Respiratory Institute, Cleveland Clinic; Assistant Professor, Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Cleveland, OH

Ruchi Yadav, MD
Associate Staff, Imaging Institute, Cleveland Clinic

Address: Brian D. Southern, MD, Respiratory Institute, A90, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195; e-mail: [email protected]

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interstitial lung disease, computed tomography, CT, lung cancer screening, smoking, fibrotic, nonfibrotic, respiratory bronchiolitis, respiratory bronchiolitis-interstitial lung disease, desquamative interstitial pneumonia, pulmonary Langerhans cell histiocytosis, nonspecific interstitial pneumonia, usual interstitial pneumonia, idiopathic pulmonary fibrosis, Brian Southern, Rachel Scheraga, Ruchi Yadav
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Brian D. Southern, MD
Respiratory Institute, Cleveland Clinic; Assistant Professor, Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Cleveland, OH

Rachel G. Scheraga, MD
Respiratory Institute, Cleveland Clinic; Assistant Professor, Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Cleveland, OH

Ruchi Yadav, MD
Associate Staff, Imaging Institute, Cleveland Clinic

Address: Brian D. Southern, MD, Respiratory Institute, A90, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195; e-mail: [email protected]

Author and Disclosure Information

Brian D. Southern, MD
Respiratory Institute, Cleveland Clinic; Assistant Professor, Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Cleveland, OH

Rachel G. Scheraga, MD
Respiratory Institute, Cleveland Clinic; Assistant Professor, Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Cleveland, OH

Ruchi Yadav, MD
Associate Staff, Imaging Institute, Cleveland Clinic

Address: Brian D. Southern, MD, Respiratory Institute, A90, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195; e-mail: [email protected]

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Primary care physicians are playing a bigger role in evaluating the incidental finding of interstitial lung diseases since the recent publication of guidelines recommending computed tomography (CT) to screen for lung cancer.

In August 2011, the National Cancer Institute published its findings from the National Lung Screening Trial, which demonstrated a 20% reduction in mortality from lung cancer in patients at high risk screened with low-dose CT.1 Based on these results, the American Cancer Society, the American College of Chest Physicians, the American Society of Clinical Oncology, and the National Comprehensive Cancer Network recommended annual screening for lung cancer with low-dose CT in adults ages 55 to 74 who have a 30-pack-year smoking history and who currently smoke or have quit within the past 15 years.2 In December 2013, the US Preventive Services Task Force published similar guidelines but increased the age range to include high-risk patients ages 55 to 80.3

Bach et al4 estimated that, in 2010 in the United States, 8.6 million people met the criteria used in the National Lung Screening Trial for low-dose CT screening. These are the same criteria as in the multisociety recommendations cited above.2 With such large numbers of patients eligible for CT screening, internists and other primary care physicians are undoubtedly encountering the incidental discovery of nonmalignant pulmonary diseases such as interstitial lung disease.

This article reviews the radiographic characteristics of the most common interstitial lung diseases the internist may encounter on screening CT in long-term smokers.

Referral to a specialist has been associated with lower rates of morbidity and death,5 and a diagnosis of interstitial lung disease should be confirmed by a pulmonologist and a radiologist specializing in differentiating the subtypes. But the primary care physician now plays a critical role in recognizing the need for further evaluation.

HOW COMMON IS INTERSTITIAL LUNG DISEASE IN SMOKERS?

Several studies have published data on the prevalence of interstitial lung disease in patients undergoing low-dose CT for lung cancer screening.

A trial at Mayo Clinic in current and former smokers identified “diffuse lung disease” in 9 (0.9%) of 1,049 participants.6

A trial in Ireland identified idiopathic pulmonary fibrosis in 6 (1.3%) of 449 current smokers who underwent low-dose CT screening for lung cancer.7

Sverzellati et al8 evaluated 692 participants in the Multicentric Italian Lung Detection CT screening study and reported a respiratory bronchiolitis pattern in 109 (15.7%), a usual interstitial pneumonia pattern in 2 (0.3%), and other patterns of chronic interstitial pneumonia in 26 (3.8%).

The National Lung Screening Trial reported that the frequency of “clinically significant” incidental findings (including pulmonary fibrosis) in all participants was 7.5%.1 A retrospective analysis of 884 participants at a single site in this trial identified interstitial lung abnormalities in 86 participants (9.7%).9 These abnormalities were further categorized as nonfibrotic in 52 (5.9%) of 884, fibrotic in 19 (2.1%) of 884, and mixed fibrotic and nonfibrotic in 15 (1.7%) of 884.

Follow-up CT at 2 years in this trial demonstrated improvement in 50% and progression in 11% of patients who had nonfibrotic abnormalities, while fibrotic abnormalities improved in no cases and progressed in 37%. Interstitial lung abnormalities were more common in those who currently smoked and in those with more pack-years of cigarette smoking.9

In sum, these trials suggest that low-dose CT screening for lung cancer can detect the most common forms of interstitial lung disease in this at-risk population and can characterize them as fibrotic or nonfibrotic, a distinction important for prognosis and subsequent management.

NONFIBROTIC VS FIBROTIC DISEASE

It is important to distinguish between nonfibrotic and fibrotic interstitial lung disease, as fibrotic disease carries a worse prognosis and is treated differently.

Features of nonfibrotic interstitial lung disease:

  • Ground-glass opacities
  • Nodules
  • Mosaic attenuation or consolidation.

Features of fibrotic interstitial lung disease:

  • Combination of ground-glass opacities and reticulation
  • Reticulation by itself
  • Traction bronchiectasis
  • Honeycombing
  • Loss of lung volume.

NONFIBROTIC INTERSTITIAL LUNG DISEASES

Given the strong likelihood that a patient undergoing screening CT is either a current or former smoker, physicians may encounter, in addition to emphysema and lung cancer, the following smoking-related interstitial lung diseases, which are primarily nonfibrotic and which frequently coexist (Table 1):

  • Respiratory bronchiolitis
  • Respiratory bronchiolitis-interstitial lung disease
  • Desquamative interstitial pneumonia
  • Pulmonary Langerhans cell histiocytosis.

Respiratory bronchiolitis

Respiratory bronchiolitis occurs mostly in smokers and does not necessarily lead to respiratory symptoms in all patients.10 It cannot always be identified radiographically but occasionally appears as predominantly upper-lobe, patchy ground-glass opacities or ill-defined centrilobular nodules without evidence of fibrosis (Figure 1).

Respiratory bronchiolitis
Figure 1. In a 36-year-old woman with an 18-pack-year smoking history, high-resolution CT shows respiratory bronchiolitis in the upper lungs, with bilateral centrilobular ground-glass nodules (red arrow), patchy ground-glass opacities (white solid arrow) and bronchial wall thickening (white dashed arrow).

Respiratory bronchiolitis-interstitial lung disease

In rare cases, respiratory bronchiolitis leads to peribronchial fibrosis invading the alveolar walls, which is then classified as respiratory bronchiolitis-interstitial lung disease.11 The CT findings in respiratory bronchiolitis-interstitial lung disease are upper-lobe-predominant centrilobular ground-glass nodules, patchy ground-glass opacities, and bronchial wall thickening (Figure 2).10 Occasionally, mild reticulation is noted without honeycombing. Mild air trapping can be seen in the lower lobes, with centrilobular emphysema in the upper lobes.12

Respiratory bronchiolitis-interstitial lung disease
Figure 2. In a 40-year-old woman with a 15-pack-year smoking history with respiratory bronchiolitis-interstitial lung disease, high-resolution CT obtained through the upper lungs at initial diagnosis (left) shows diffuse faint centrilobular ground-glass nodules (red arrows). High-resolution CT 1 year after smoking cessation (right) shows complete resolution of the centrilobular nodules.

The only successful therapy for respiratory bronchiolitis and respiratory bronchiolitis-interstitial lung disease is smoking cessation. Finding either of these diseases should prompt aggressive counseling by the internist and consideration of referral to a specialist in interstitial lung disease.

Desquamative interstitial pneumonia

Although pathologically different from respiratory bronchiolitis-interstitial lung disease, desquamative interstitial pneumonia has a similar clinical and radiographic presentation. Because their features significantly overlap, they are considered a pathomorphologic continuum, representing degrees of severity of the same disease process caused by prolonged tobacco inhalation.10,13

Widespread ground-glass opacities are the predominant CT finding. These are bilateral and symmetric in distribution in 86%, basal and peripheral in 60%, patchy in 20%, and diffuse in 20% (Figure 3).14 Other frequent findings are mild reticulation with traction bronchiectasis and coexistent emphysema (Figure 4).15 The small peripheral cystic spaces noted in this disease most likely represent dilated bronchioles and alveolar ducts rather than honeycombing.16

Desquamative interstitial pneumonia
Figure 3. In a 49-year-old woman with a 23-pack-year history of smoking, high-resolution CT obtained through the lower lungs shows features of desquamative interstitial pneumonia: diffuse ground-glass opacities predmoninantly in the mid-lower lung. No traction bronchiectasis or architectural distortion is seen.

Desquamative interstitial pneumonia and emphysema
Figure 4. In a 38-year-old man with a 20-pack-year history of smoking, coronal image reformatting shows features of desquamative interstitial pneumonia and emphysema, ie, diffuse distribution of ground-glass opacities with cystic airspaces representing emphysema (red arrow).

No additional treatment beyond elimination of smoking has been proven effective for desquamative interstitial pneumonia, and patients who manage to quit smoking generally have a favorable prognosis.17,18

Pulmonary Langerhans cell histiocytosis

The combination of upper-lobe-predominant cysts and nodules in a young heavy smoker are diagnostic of pulmonary Langerhans cell histiocytosis. The cysts are bizarrely shaped, thin- or thick-walled, and nonuniform in size (Figure 5). The irregular cavitary nodules are centrilobular. The disease characteristically spares the costophrenic angles.

Langerhans cell histiocytosis
Figure 5. In a 46-year-old woman with a 26-pack-year history of smoking, a coronal reformatted image shows various-sized cysts (solid white arrows) and nodules (dashed white arrow) in the upper and midlung zones—a characteristic imaging pattern of smoking-related pulmonary Langerhans cell histiocytosis, which was later confirmed with biopsy in this patient.

Spontaneous pneumothorax is the initial clinical presentation in 15% of patients.16 In the early stages of the disease (nodule-predominant disease without cysts), infection and metastatic disease need to be excluded (Figure 6). In the later stages, the cysts become coalescent, making the distinction between this disease and “burned-out” lymphangioleiomyomatosis or severe emphysema extremely difficult (Figure 7).17 Smoking cessation and corticosteroids are the mainstay of medical therapy for pulmonary Langerhans cell histiocytosis, and about 50% of patients who quit smoking and receive corticosteroids demonstrate partial or complete clearing of the radiographic abnormalities and symptoms (Figure 8).

Pulmonary Langerhans cell histiocytosis
Figure 6. Early-stage pulmonary Langerhans cell histiocytosis (nodule-predominant) mimics metastasis in a 56-year-old woman with a 19-pack-year history of smoking. High-resolution CT obtained through the midlungs shows few irregularly marginated nodules (red arrow), one of which is cavitary (white arrow). There is a background of centrilobular emphysema. No cysts are seen. Histologic study confirmed the diagnosis.
Pulmonary Langerhans cell histiocytosis
Figure 7. In a 49-year-old male smoker with biopsy-confirmed pulmonary Langerhans cell histiocytosis, high-resolution CT obtained through the upper lungs shows numerous well-circumscribed, thin-walled cysts with absence of nodules. This appearance is typical of advanced (“burned out”) pulmonary Langerhans cell histiocytosis and is virtually indistinguishable from emphysema on high-resolution CT.

Pulmonary Langerhans cell histiocytosis
Figure 8. In a 41-year-old woman with a 22-pack-year history of smoking and pulmonary Langerhans cell histiocytosis, high-resolution CT through the midlungs at the time of initial diagnosis (left) shows a few solid (red arrow) and cavitary (white arrow) nodules. The lung bases were spared. No cysts were seen. High-resolution CT 2 years after smoking cessation and oral corticosteroid therapy (right) shows near-complete resolution of the nodules.

FIBROTIC INTERSTITIAL LUNG DISEASES

If CT identifies a diffuse fibrotic pattern, the two most common possibilities (Table 2) are:

  • Nonspecific interstitial pneumonia
  • Usual interstitial pneumonia.

As noted above, these carry a worse prognosis than the nonfibrotic interstitial lung diseases.

Nonspecific interstitial pneumonia

While most frequently idiopathic, the nonspecific interstitial pneumonia pattern can often be seen in connective tissue diseases. It has also been associated with chronic hypersensitivity pneumonitis, drug toxicity, and slowly resolving diffuse alveolar damage.19 Although it is not the only pathologic pattern in interstitial lung disease associated with connective tissue disease, it is the most common pattern in systemic sclerosis, systemic lupus erythematosus, dermatomyositis-polymyositis, and mixed connective tissue disease.20

The parenchymal changes are typically subpleural and symmetric in distribution (Figure 9). In about one-third of cases, there is a peribronchovascular distribution of the abnormalities (Figure 10).

Nonspecific interstitial pneumonia
Figure 9. In a 53-year-old woman with mild dyspnea, high-resolution CT through the lower lungs shows bilateral symmetric peripheral distribution of ground-glass opacities, a feature of nonspecific interstitial pneumonia.

Nonspecific interstitial pneumonia
Figure 10. High-resolution CT through the lower lungs in a 60-year-old woman with nonspecific interstitial pneumonia shows bilateral symmetric peribronchovascular distribution of ground-glass and reticular opacities with traction bronchiectasis.

Ground-glass opacities are the dominant imaging findings, seen in 80% of cases.18 In advanced disease (also referred to as fibrotic nonspecific interstitial pneumonia), patients have accompanying fine or coarse reticular opacities, traction bronchiectasis, and consolidation (Figure 11). Honeycombing is seen in 1% to 5% of patients.21

Nonspecific interstitial pneumonia
Figure 11. High-resolution CT through the lower lungs in a 45-year-old woman with scleroderma and nonspecific interstitial pneumonia shows bilateral symmetric peripheral ground-glass attenuation with reticular opacities, traction bronchiectasis (white solid arrow), traction bronchiolectasis (white dashed arrows), and subpleural sparing. A dilated esophagus (red arrow) correlates with the history of scleroderma.

The most specific sign of nonspecific interstitial pneumonia is sparing of the immediate subpleural lung, apparent in 30% to 50% of patients (Figure 12).22 Subpleural sparing with a peribronchovascular distribution of abnormalities, absence of lobular areas with decreased attenuation, and lack of honeycombing are imaging features that increase the diagnostic confidence of nonspecific interstitial pneumonia (Table 3).23 Clinically, compared with those who have usual interstitial pneumonia (see below), patients are younger and more of them are female. These patients also present with extrapulmonary manifestations such as joint involvement, rash, and Raynaud phenomenon. Therefore, these associated symptoms on presentation can help distinguish nonspecific interstitial pneumonia or usual interstitial pneumonia related to connective tissue disease from the idiopathic forms.

Nonspecific interstitial pneumonia
Figure 12. In a 52-year-old woman with mild dyspnea and fatigue, high-resolution CT through the lower lungs shows bilateral symmetric peripheral ground-glass opacities with sparing of the immediate subpleural lung (white arrows), the most specific sign of nonspecific interstitial pneumonia.

The first step in managing nonspecific interstitial pneumonia is to remove all potential exposure to inhaled substances or to drugs. Although immunosuppressive therapy has never been studied in a randomized controlled trial in this disease, numerous reports suggest that patients may respond to prednisone and to steroid-sparing immunosuppressants.24

In several studies, survival rates in nonspecific interstitial pneumonia were significantly greater than in usual interstitial pneumonia independent of the treatment strategy. In long-term follow-up of patients with idiopathic nonspecific interstitial pneumonia treated with immunosuppressive therapy, two-thirds remained stable or improved.25–27

Although most connective tissue diseases cause a lung pattern of nonspecific interstitial pneumonia, some (eg, rheumatoid arthritis) may present with a pattern of usual interstitial pneumonia. In these cases and in those of advanced fibrotic nonspecific interstitial pneumonia, the prognosis is worse, as the disease is less responsive to immunosuppressive therapy.20

Usual interstitial pneumonia

Usual interstitial pneumonia is the most severe form of lung fibrosis. Most cases are idio­pathic and are termed idiopathic pulmonary fibrosis. Other causes of the usual interstitial pneumonia pattern include domestic and occupational environmental exposures, connective tissue disease, and drug toxicity.28 An epidemiologic association between smoking and usual interstitial pneumonia is well documented.28

Idiopathic pulmonary fibrosis typically affects men ages 50 to 70. Because its risk factors coincide with those of lung cancer, there is a high likelihood of detecting idiopathic pulmonary fibrosis early in this screening population. It has an especially poor prognosis, with a mean survival of 2 to 5 years from the time of diagnosis.18

The distribution of disease in usual interstitial pneumonia is characteristically subpleural and basal. CT features include coarse subpleural reticulation and honeycombing combined with traction bronchiectasis or bronchiolectasis and architectural distortion (Figure 13).18 Honeycombing is the most specific and key diagnostic CT finding for establishing a definitive diagnosis of usual interstitial pneumonia.29 However, ground-glass opacities are present in most patients, typically in the region of interstitial fibrosis, and are always less extensive than the reticulation.30 The findings demonstrate morphologic heterogeneity, with areas of fibrosis adjacent to areas of normal lung (Figure 14).

Figure 13. In a 68-year-old man, high-resolution CT through the lower lungs shows features of usual interstitial pneumonia: bilateral subpleural reticular opacities, traction bronchiectasis (blue arrow), ground-glass opacities (red arrow), and honeycombing, seen as rows of clustered subpleural cystic air spaces (yellow arrow).

Usual interstitial pneumonia
Figure 14. In a 67-year-old man with usual interstitial pneumonia, high-resolution CT through the lower lungs shows basal-predominant subpleural honey-combing (red arrow). Morphologic heterogeneity is seen with areas of fibrosis immediately adjacent to normal lung (white double arrow).

In addition to the aforementioned imaging features, the 2011 American Thoracic Society and European Respiratory Society joint guidelines for the CT diagnosis of usual interstitial pneumonia patterns require the absence of atypical features that suggest an alternative diagnosis, including those seen in nonspecific interstitial pneumonia, such as an upper, midlung, or peribronchovascular distribution and extensive ground-glass attenuation.28 Mild mediastinal lymphadenopathy (usually < 1.5 cm in the short axis) is common in usual interstitial pneumonia.31

Because other chronic interstitial pneumonias that may resemble usual interstitial pneumonia have a more favorable course and may respond to immunosuppressive therapy, establishing an early and accurate diagnosis is of the utmost importance.5 Additionally, the emergence of possible new therapies for idiopathic pulmonary fibrosis makes early referral to a specialist paramount in these cases. Recent studies have demonstrated significant slowing of the progression of disease in idiopathic pulmonary fibrosis with both pirfenidone and nintedanib.32,33

DIAGNOSIS AND MANAGEMENT

Managing interstitial lung disease found on screening CT
Figure 15.

The diagnosis of these nonfibrotic and fibrotic lung diseases is complex. In all cases in which interstitial lung disease is detected on screening CT for lung cancer, the internist should strongly consider further evaluation with dedicated high-resolution CT and early referral to a specialist (Figure 15).

Because smoking cessation is the only recommended treatment for nonfibrotic smoking-related interstitial lung diseases, particular emphasis on smoking cessation counseling is essential.

Referral for bronchoscopy with transbronchial lung biopsy is generally not helpful in the diagnosis of the interstitial lung diseases discussed in this article unless there is a need to rule out infection or neoplasm.34 Referral for surgical lung biopsy may be indicated in some cases of suspected pulmonary Langerhans cell histiocytosis, desquamative interstitial pneumonia, nonspecific interstitial pneumonia, or usual interstitial pneumonia if the diagnosis is uncertain (Tables 1 and 2).35

The American Thoracic Society/European Respiratory Society guidelines suggest a multidisciplinary team approach that includes a pathologist, radiologist, and clinician.35 This approach more readily determines the correct diagnosis and relies less on invasive methods such as surgical biopsy and more on noninvasive methods such as radiology and clinical history. Overall, this will promote earlier access to appropriate therapies, clinical trial enrollment, and in more severe cases, lung transplant.

Currently, 23% of all lung transplants worldwide are performed in patients with idiopathic pulmonary fibrosis. Other forms of pulmonary fibrosis account for 3% to 4% of lung transplants performed.36

Evidence suggests that early referral reduces rates of morbidity and death in these patients. The results of a single-center study37 of patients with idiopathic pulmonary fibrosis demonstrated that a longer delay from the onset of symptoms to evaluation by a specialist at a tertiary care referral center was associated with a higher rate of death from this disease independent of disease severity. Those with the longest delay in referral had a multivariable-adjusted death rate 3.4 times higher than those with the shortest delay.5,37

In summary, with implementation of the new lung cancer screening guidelines, primary care physicians are more often encountering the incidental finding of interstitial lung disease in their patients. Prompt diagnosis of interstitial lung disease helps ensure that patients receive appropriate care and early consideration for clinical trials and lung transplant.

Primary care physicians play a critical role in the initial identification of key characteristics of the interstitial abnormality—namely, whether the pattern is nonfibrotic or fibrotic—and in the correlation of the history and physical findings to expedite the diagnosis. Subsequently, ordering high-resolution CT for more detailed characterization and prompt referral to a specialist in interstitial lung disease allow for a more rapid and accurate diagnosis, specialized therapy, and supportive care.

Primary care physicians are playing a bigger role in evaluating the incidental finding of interstitial lung diseases since the recent publication of guidelines recommending computed tomography (CT) to screen for lung cancer.

In August 2011, the National Cancer Institute published its findings from the National Lung Screening Trial, which demonstrated a 20% reduction in mortality from lung cancer in patients at high risk screened with low-dose CT.1 Based on these results, the American Cancer Society, the American College of Chest Physicians, the American Society of Clinical Oncology, and the National Comprehensive Cancer Network recommended annual screening for lung cancer with low-dose CT in adults ages 55 to 74 who have a 30-pack-year smoking history and who currently smoke or have quit within the past 15 years.2 In December 2013, the US Preventive Services Task Force published similar guidelines but increased the age range to include high-risk patients ages 55 to 80.3

Bach et al4 estimated that, in 2010 in the United States, 8.6 million people met the criteria used in the National Lung Screening Trial for low-dose CT screening. These are the same criteria as in the multisociety recommendations cited above.2 With such large numbers of patients eligible for CT screening, internists and other primary care physicians are undoubtedly encountering the incidental discovery of nonmalignant pulmonary diseases such as interstitial lung disease.

This article reviews the radiographic characteristics of the most common interstitial lung diseases the internist may encounter on screening CT in long-term smokers.

Referral to a specialist has been associated with lower rates of morbidity and death,5 and a diagnosis of interstitial lung disease should be confirmed by a pulmonologist and a radiologist specializing in differentiating the subtypes. But the primary care physician now plays a critical role in recognizing the need for further evaluation.

HOW COMMON IS INTERSTITIAL LUNG DISEASE IN SMOKERS?

Several studies have published data on the prevalence of interstitial lung disease in patients undergoing low-dose CT for lung cancer screening.

A trial at Mayo Clinic in current and former smokers identified “diffuse lung disease” in 9 (0.9%) of 1,049 participants.6

A trial in Ireland identified idiopathic pulmonary fibrosis in 6 (1.3%) of 449 current smokers who underwent low-dose CT screening for lung cancer.7

Sverzellati et al8 evaluated 692 participants in the Multicentric Italian Lung Detection CT screening study and reported a respiratory bronchiolitis pattern in 109 (15.7%), a usual interstitial pneumonia pattern in 2 (0.3%), and other patterns of chronic interstitial pneumonia in 26 (3.8%).

The National Lung Screening Trial reported that the frequency of “clinically significant” incidental findings (including pulmonary fibrosis) in all participants was 7.5%.1 A retrospective analysis of 884 participants at a single site in this trial identified interstitial lung abnormalities in 86 participants (9.7%).9 These abnormalities were further categorized as nonfibrotic in 52 (5.9%) of 884, fibrotic in 19 (2.1%) of 884, and mixed fibrotic and nonfibrotic in 15 (1.7%) of 884.

Follow-up CT at 2 years in this trial demonstrated improvement in 50% and progression in 11% of patients who had nonfibrotic abnormalities, while fibrotic abnormalities improved in no cases and progressed in 37%. Interstitial lung abnormalities were more common in those who currently smoked and in those with more pack-years of cigarette smoking.9

In sum, these trials suggest that low-dose CT screening for lung cancer can detect the most common forms of interstitial lung disease in this at-risk population and can characterize them as fibrotic or nonfibrotic, a distinction important for prognosis and subsequent management.

NONFIBROTIC VS FIBROTIC DISEASE

It is important to distinguish between nonfibrotic and fibrotic interstitial lung disease, as fibrotic disease carries a worse prognosis and is treated differently.

Features of nonfibrotic interstitial lung disease:

  • Ground-glass opacities
  • Nodules
  • Mosaic attenuation or consolidation.

Features of fibrotic interstitial lung disease:

  • Combination of ground-glass opacities and reticulation
  • Reticulation by itself
  • Traction bronchiectasis
  • Honeycombing
  • Loss of lung volume.

NONFIBROTIC INTERSTITIAL LUNG DISEASES

Given the strong likelihood that a patient undergoing screening CT is either a current or former smoker, physicians may encounter, in addition to emphysema and lung cancer, the following smoking-related interstitial lung diseases, which are primarily nonfibrotic and which frequently coexist (Table 1):

  • Respiratory bronchiolitis
  • Respiratory bronchiolitis-interstitial lung disease
  • Desquamative interstitial pneumonia
  • Pulmonary Langerhans cell histiocytosis.

Respiratory bronchiolitis

Respiratory bronchiolitis occurs mostly in smokers and does not necessarily lead to respiratory symptoms in all patients.10 It cannot always be identified radiographically but occasionally appears as predominantly upper-lobe, patchy ground-glass opacities or ill-defined centrilobular nodules without evidence of fibrosis (Figure 1).

Respiratory bronchiolitis
Figure 1. In a 36-year-old woman with an 18-pack-year smoking history, high-resolution CT shows respiratory bronchiolitis in the upper lungs, with bilateral centrilobular ground-glass nodules (red arrow), patchy ground-glass opacities (white solid arrow) and bronchial wall thickening (white dashed arrow).

Respiratory bronchiolitis-interstitial lung disease

In rare cases, respiratory bronchiolitis leads to peribronchial fibrosis invading the alveolar walls, which is then classified as respiratory bronchiolitis-interstitial lung disease.11 The CT findings in respiratory bronchiolitis-interstitial lung disease are upper-lobe-predominant centrilobular ground-glass nodules, patchy ground-glass opacities, and bronchial wall thickening (Figure 2).10 Occasionally, mild reticulation is noted without honeycombing. Mild air trapping can be seen in the lower lobes, with centrilobular emphysema in the upper lobes.12

Respiratory bronchiolitis-interstitial lung disease
Figure 2. In a 40-year-old woman with a 15-pack-year smoking history with respiratory bronchiolitis-interstitial lung disease, high-resolution CT obtained through the upper lungs at initial diagnosis (left) shows diffuse faint centrilobular ground-glass nodules (red arrows). High-resolution CT 1 year after smoking cessation (right) shows complete resolution of the centrilobular nodules.

The only successful therapy for respiratory bronchiolitis and respiratory bronchiolitis-interstitial lung disease is smoking cessation. Finding either of these diseases should prompt aggressive counseling by the internist and consideration of referral to a specialist in interstitial lung disease.

Desquamative interstitial pneumonia

Although pathologically different from respiratory bronchiolitis-interstitial lung disease, desquamative interstitial pneumonia has a similar clinical and radiographic presentation. Because their features significantly overlap, they are considered a pathomorphologic continuum, representing degrees of severity of the same disease process caused by prolonged tobacco inhalation.10,13

Widespread ground-glass opacities are the predominant CT finding. These are bilateral and symmetric in distribution in 86%, basal and peripheral in 60%, patchy in 20%, and diffuse in 20% (Figure 3).14 Other frequent findings are mild reticulation with traction bronchiectasis and coexistent emphysema (Figure 4).15 The small peripheral cystic spaces noted in this disease most likely represent dilated bronchioles and alveolar ducts rather than honeycombing.16

Desquamative interstitial pneumonia
Figure 3. In a 49-year-old woman with a 23-pack-year history of smoking, high-resolution CT obtained through the lower lungs shows features of desquamative interstitial pneumonia: diffuse ground-glass opacities predmoninantly in the mid-lower lung. No traction bronchiectasis or architectural distortion is seen.

Desquamative interstitial pneumonia and emphysema
Figure 4. In a 38-year-old man with a 20-pack-year history of smoking, coronal image reformatting shows features of desquamative interstitial pneumonia and emphysema, ie, diffuse distribution of ground-glass opacities with cystic airspaces representing emphysema (red arrow).

No additional treatment beyond elimination of smoking has been proven effective for desquamative interstitial pneumonia, and patients who manage to quit smoking generally have a favorable prognosis.17,18

Pulmonary Langerhans cell histiocytosis

The combination of upper-lobe-predominant cysts and nodules in a young heavy smoker are diagnostic of pulmonary Langerhans cell histiocytosis. The cysts are bizarrely shaped, thin- or thick-walled, and nonuniform in size (Figure 5). The irregular cavitary nodules are centrilobular. The disease characteristically spares the costophrenic angles.

Langerhans cell histiocytosis
Figure 5. In a 46-year-old woman with a 26-pack-year history of smoking, a coronal reformatted image shows various-sized cysts (solid white arrows) and nodules (dashed white arrow) in the upper and midlung zones—a characteristic imaging pattern of smoking-related pulmonary Langerhans cell histiocytosis, which was later confirmed with biopsy in this patient.

Spontaneous pneumothorax is the initial clinical presentation in 15% of patients.16 In the early stages of the disease (nodule-predominant disease without cysts), infection and metastatic disease need to be excluded (Figure 6). In the later stages, the cysts become coalescent, making the distinction between this disease and “burned-out” lymphangioleiomyomatosis or severe emphysema extremely difficult (Figure 7).17 Smoking cessation and corticosteroids are the mainstay of medical therapy for pulmonary Langerhans cell histiocytosis, and about 50% of patients who quit smoking and receive corticosteroids demonstrate partial or complete clearing of the radiographic abnormalities and symptoms (Figure 8).

Pulmonary Langerhans cell histiocytosis
Figure 6. Early-stage pulmonary Langerhans cell histiocytosis (nodule-predominant) mimics metastasis in a 56-year-old woman with a 19-pack-year history of smoking. High-resolution CT obtained through the midlungs shows few irregularly marginated nodules (red arrow), one of which is cavitary (white arrow). There is a background of centrilobular emphysema. No cysts are seen. Histologic study confirmed the diagnosis.
Pulmonary Langerhans cell histiocytosis
Figure 7. In a 49-year-old male smoker with biopsy-confirmed pulmonary Langerhans cell histiocytosis, high-resolution CT obtained through the upper lungs shows numerous well-circumscribed, thin-walled cysts with absence of nodules. This appearance is typical of advanced (“burned out”) pulmonary Langerhans cell histiocytosis and is virtually indistinguishable from emphysema on high-resolution CT.

Pulmonary Langerhans cell histiocytosis
Figure 8. In a 41-year-old woman with a 22-pack-year history of smoking and pulmonary Langerhans cell histiocytosis, high-resolution CT through the midlungs at the time of initial diagnosis (left) shows a few solid (red arrow) and cavitary (white arrow) nodules. The lung bases were spared. No cysts were seen. High-resolution CT 2 years after smoking cessation and oral corticosteroid therapy (right) shows near-complete resolution of the nodules.

FIBROTIC INTERSTITIAL LUNG DISEASES

If CT identifies a diffuse fibrotic pattern, the two most common possibilities (Table 2) are:

  • Nonspecific interstitial pneumonia
  • Usual interstitial pneumonia.

As noted above, these carry a worse prognosis than the nonfibrotic interstitial lung diseases.

Nonspecific interstitial pneumonia

While most frequently idiopathic, the nonspecific interstitial pneumonia pattern can often be seen in connective tissue diseases. It has also been associated with chronic hypersensitivity pneumonitis, drug toxicity, and slowly resolving diffuse alveolar damage.19 Although it is not the only pathologic pattern in interstitial lung disease associated with connective tissue disease, it is the most common pattern in systemic sclerosis, systemic lupus erythematosus, dermatomyositis-polymyositis, and mixed connective tissue disease.20

The parenchymal changes are typically subpleural and symmetric in distribution (Figure 9). In about one-third of cases, there is a peribronchovascular distribution of the abnormalities (Figure 10).

Nonspecific interstitial pneumonia
Figure 9. In a 53-year-old woman with mild dyspnea, high-resolution CT through the lower lungs shows bilateral symmetric peripheral distribution of ground-glass opacities, a feature of nonspecific interstitial pneumonia.

Nonspecific interstitial pneumonia
Figure 10. High-resolution CT through the lower lungs in a 60-year-old woman with nonspecific interstitial pneumonia shows bilateral symmetric peribronchovascular distribution of ground-glass and reticular opacities with traction bronchiectasis.

Ground-glass opacities are the dominant imaging findings, seen in 80% of cases.18 In advanced disease (also referred to as fibrotic nonspecific interstitial pneumonia), patients have accompanying fine or coarse reticular opacities, traction bronchiectasis, and consolidation (Figure 11). Honeycombing is seen in 1% to 5% of patients.21

Nonspecific interstitial pneumonia
Figure 11. High-resolution CT through the lower lungs in a 45-year-old woman with scleroderma and nonspecific interstitial pneumonia shows bilateral symmetric peripheral ground-glass attenuation with reticular opacities, traction bronchiectasis (white solid arrow), traction bronchiolectasis (white dashed arrows), and subpleural sparing. A dilated esophagus (red arrow) correlates with the history of scleroderma.

The most specific sign of nonspecific interstitial pneumonia is sparing of the immediate subpleural lung, apparent in 30% to 50% of patients (Figure 12).22 Subpleural sparing with a peribronchovascular distribution of abnormalities, absence of lobular areas with decreased attenuation, and lack of honeycombing are imaging features that increase the diagnostic confidence of nonspecific interstitial pneumonia (Table 3).23 Clinically, compared with those who have usual interstitial pneumonia (see below), patients are younger and more of them are female. These patients also present with extrapulmonary manifestations such as joint involvement, rash, and Raynaud phenomenon. Therefore, these associated symptoms on presentation can help distinguish nonspecific interstitial pneumonia or usual interstitial pneumonia related to connective tissue disease from the idiopathic forms.

Nonspecific interstitial pneumonia
Figure 12. In a 52-year-old woman with mild dyspnea and fatigue, high-resolution CT through the lower lungs shows bilateral symmetric peripheral ground-glass opacities with sparing of the immediate subpleural lung (white arrows), the most specific sign of nonspecific interstitial pneumonia.

The first step in managing nonspecific interstitial pneumonia is to remove all potential exposure to inhaled substances or to drugs. Although immunosuppressive therapy has never been studied in a randomized controlled trial in this disease, numerous reports suggest that patients may respond to prednisone and to steroid-sparing immunosuppressants.24

In several studies, survival rates in nonspecific interstitial pneumonia were significantly greater than in usual interstitial pneumonia independent of the treatment strategy. In long-term follow-up of patients with idiopathic nonspecific interstitial pneumonia treated with immunosuppressive therapy, two-thirds remained stable or improved.25–27

Although most connective tissue diseases cause a lung pattern of nonspecific interstitial pneumonia, some (eg, rheumatoid arthritis) may present with a pattern of usual interstitial pneumonia. In these cases and in those of advanced fibrotic nonspecific interstitial pneumonia, the prognosis is worse, as the disease is less responsive to immunosuppressive therapy.20

Usual interstitial pneumonia

Usual interstitial pneumonia is the most severe form of lung fibrosis. Most cases are idio­pathic and are termed idiopathic pulmonary fibrosis. Other causes of the usual interstitial pneumonia pattern include domestic and occupational environmental exposures, connective tissue disease, and drug toxicity.28 An epidemiologic association between smoking and usual interstitial pneumonia is well documented.28

Idiopathic pulmonary fibrosis typically affects men ages 50 to 70. Because its risk factors coincide with those of lung cancer, there is a high likelihood of detecting idiopathic pulmonary fibrosis early in this screening population. It has an especially poor prognosis, with a mean survival of 2 to 5 years from the time of diagnosis.18

The distribution of disease in usual interstitial pneumonia is characteristically subpleural and basal. CT features include coarse subpleural reticulation and honeycombing combined with traction bronchiectasis or bronchiolectasis and architectural distortion (Figure 13).18 Honeycombing is the most specific and key diagnostic CT finding for establishing a definitive diagnosis of usual interstitial pneumonia.29 However, ground-glass opacities are present in most patients, typically in the region of interstitial fibrosis, and are always less extensive than the reticulation.30 The findings demonstrate morphologic heterogeneity, with areas of fibrosis adjacent to areas of normal lung (Figure 14).

Figure 13. In a 68-year-old man, high-resolution CT through the lower lungs shows features of usual interstitial pneumonia: bilateral subpleural reticular opacities, traction bronchiectasis (blue arrow), ground-glass opacities (red arrow), and honeycombing, seen as rows of clustered subpleural cystic air spaces (yellow arrow).

Usual interstitial pneumonia
Figure 14. In a 67-year-old man with usual interstitial pneumonia, high-resolution CT through the lower lungs shows basal-predominant subpleural honey-combing (red arrow). Morphologic heterogeneity is seen with areas of fibrosis immediately adjacent to normal lung (white double arrow).

In addition to the aforementioned imaging features, the 2011 American Thoracic Society and European Respiratory Society joint guidelines for the CT diagnosis of usual interstitial pneumonia patterns require the absence of atypical features that suggest an alternative diagnosis, including those seen in nonspecific interstitial pneumonia, such as an upper, midlung, or peribronchovascular distribution and extensive ground-glass attenuation.28 Mild mediastinal lymphadenopathy (usually < 1.5 cm in the short axis) is common in usual interstitial pneumonia.31

Because other chronic interstitial pneumonias that may resemble usual interstitial pneumonia have a more favorable course and may respond to immunosuppressive therapy, establishing an early and accurate diagnosis is of the utmost importance.5 Additionally, the emergence of possible new therapies for idiopathic pulmonary fibrosis makes early referral to a specialist paramount in these cases. Recent studies have demonstrated significant slowing of the progression of disease in idiopathic pulmonary fibrosis with both pirfenidone and nintedanib.32,33

DIAGNOSIS AND MANAGEMENT

Managing interstitial lung disease found on screening CT
Figure 15.

The diagnosis of these nonfibrotic and fibrotic lung diseases is complex. In all cases in which interstitial lung disease is detected on screening CT for lung cancer, the internist should strongly consider further evaluation with dedicated high-resolution CT and early referral to a specialist (Figure 15).

Because smoking cessation is the only recommended treatment for nonfibrotic smoking-related interstitial lung diseases, particular emphasis on smoking cessation counseling is essential.

Referral for bronchoscopy with transbronchial lung biopsy is generally not helpful in the diagnosis of the interstitial lung diseases discussed in this article unless there is a need to rule out infection or neoplasm.34 Referral for surgical lung biopsy may be indicated in some cases of suspected pulmonary Langerhans cell histiocytosis, desquamative interstitial pneumonia, nonspecific interstitial pneumonia, or usual interstitial pneumonia if the diagnosis is uncertain (Tables 1 and 2).35

The American Thoracic Society/European Respiratory Society guidelines suggest a multidisciplinary team approach that includes a pathologist, radiologist, and clinician.35 This approach more readily determines the correct diagnosis and relies less on invasive methods such as surgical biopsy and more on noninvasive methods such as radiology and clinical history. Overall, this will promote earlier access to appropriate therapies, clinical trial enrollment, and in more severe cases, lung transplant.

Currently, 23% of all lung transplants worldwide are performed in patients with idiopathic pulmonary fibrosis. Other forms of pulmonary fibrosis account for 3% to 4% of lung transplants performed.36

Evidence suggests that early referral reduces rates of morbidity and death in these patients. The results of a single-center study37 of patients with idiopathic pulmonary fibrosis demonstrated that a longer delay from the onset of symptoms to evaluation by a specialist at a tertiary care referral center was associated with a higher rate of death from this disease independent of disease severity. Those with the longest delay in referral had a multivariable-adjusted death rate 3.4 times higher than those with the shortest delay.5,37

In summary, with implementation of the new lung cancer screening guidelines, primary care physicians are more often encountering the incidental finding of interstitial lung disease in their patients. Prompt diagnosis of interstitial lung disease helps ensure that patients receive appropriate care and early consideration for clinical trials and lung transplant.

Primary care physicians play a critical role in the initial identification of key characteristics of the interstitial abnormality—namely, whether the pattern is nonfibrotic or fibrotic—and in the correlation of the history and physical findings to expedite the diagnosis. Subsequently, ordering high-resolution CT for more detailed characterization and prompt referral to a specialist in interstitial lung disease allow for a more rapid and accurate diagnosis, specialized therapy, and supportive care.

References
  1. National Lung Screening Trial Research Team; Aberle DR, Adams AM, Berg CD, et al. Reduced lung-cancer mortality with low-dose computed tomographic screening. N Engl J Med 2011; 365:395–409.
  2. Detterbeck FC, Lewis SZ, Diekemper R, Addrizzo-Harris D, Alberts WM. Executive summary: diagnosis and management of lung cancer, 3rd ed: American College of Chest Physicians evidence-based clinical practice guidelines. Chest 2013; 143(suppl 5):7S–37S.
  3. Moyer VA; US Preventive Services Task Force. Screening for lung cancer: US Preventive Services Task Force recommendation statement. Ann Intern Med 2014; 160:330–338.
  4. Bach PB, Mirkin JN, Oliver TK, et al. Benefits and harms of CT screening for lung cancer: a systematic review. JAMA 2012; 307:2418–2429.
  5. Lamas DJ, Kawut SM, Bagiella E, Philip N, Arcasoy SM, Lederer DJ. Delayed access and survival in idiopathic pulmonary fibrosis: a cohort study. Am J Respir Crit Care Med 2011; 184:842–847.
  6. Swensen SJ, Jett JR, Hartman TE, et al. Lung cancer screening with CT: Mayo Clinic experience. Radiology 2003; 226:756–761.
  7. MacRedmond R, Logan PM, Lee M, Kenny D, Foley C, Costello RW. Screening for lung cancer using low dose CT scanning. Thorax 2004; 59:237–241.
  8. Sverzellati N, Guerci L, Randi G, et al. Interstitial lung diseases in a lung cancer screening trial. Eur Respir J 2011; 38:392–400.
  9. Jin GY, Lynch D, Chawla A, et al. Interstitial lung abnormalities in a CT lung cancer screening population: prevalence and progression rate. Radiology 2013; 268:563–571.
  10. Heyneman LE, Ward S, Lynch DA, Remy-Jardin M, Johkoh T, Müller NL. Respiratory bronchiolitis, respiratory bronchiolitis-associated interstitial lung disease, and desquamative interstitial pneumonia: different entities or part of the spectrum of the same disease process? AJR Am J Roentgenol 1999; 173:1617–1622.
  11. Moon J, du Bois RM, Colby TV, Hansell DM, Nicholson AG. Clinical significance of respiratory bronchiolitis on open lung biopsy and its relationship to smoking related interstitial lung disease. Thorax 1999; 54:1009–1014.
  12. Holt RM, Schmidt RA, Godwin JD, Raghu G. High resolution CT in respiratory bronchiolitis-associated interstitial lung disease. J Comput Assist Tomogr 1993; 17:46–50.
  13. Ryu JH, Myers JL, Capizzi SA, Douglas WW, Vassallo R, Decker PA. Desquamative interstitial pneumonia and respiratory bronchiolitis-associated interstitial lung disease. Chest 2005; 127:178–184.
  14. Hartman TE, Primack SL, Swensen SJ, Hansell D, McGuinness G, Müller NL. Desquamative interstitial pneumonia: thin-section CT findings in 22 patients. Radiology 1993; 187:787–790.
  15. Akira M, Yamamoto S, Hara H, Sakatani M, Ueda E. Serial computed tomographic evaluation in desquamative interstitial pneumonia. Thorax 1997; 52:333–337.
  16. Lacronique J, Roth C, Battesti JP, Basset F, Chretien J. Chest radiological features of pulmonary histiocytosis X: a report based on 50 adult cases. Thorax 1982; 37:104–109.
  17. Remy-Jardin M, Edme JL, Boulenguez C, Remy J, Mastora I, Sobaszek A. Longitudinal follow-up study of smoker’s lung with thin-section CT in correlation with pulmonary function tests. Radiology 2002; 222:261–270.
  18. Mueller-Mang C, Grosse C, Schmid K, Stiebellehner L, Bankier AA. What every radiologist should know about idiopathic interstitial pneumonias. Radiographics 2007; 27:595–615.
  19. Katzenstein AL, Fiorelli RF. Nonspecific interstitial pneumonia/fibrosis. Histologic features and clinical significance. Am J Surg Pathol 1994; 18:136–147.
  20. Bryson T, Sundaram B, Khanna D, Kazerooni EA. Connective tissue disease-associated interstitial pneumonia and idiopathic interstitial pneumonia: similarity and difference. Semin Ultrasound CT MR 2014; 35:29–38.
  21. Desai SR, Veeraraghavan S, Hansell DM, et al. CT features of lung disease in patients with systemic sclerosis: comparison with idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia. Radiology 2004; 232:560–567.
  22. Tsubamoto M, Müller NL, Johkoh T, et al. Pathologic subgroups of nonspecific interstitial pneumonia: differential diagnosis from other idiopathic interstitial pneumonias on high-resolution computed tomography. J Comput Assist Tomogr 2005; 29:793–800.
  23. Silva CI, Müller NL, Lynch DA, et al. Chronic hypersensitivity pneumonitis: differentiation from idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia by using thin-section CT. Radiology 2008; 246:288–297.
  24. Antin-Ozerkis D, Rubinowitz A. An update on nonspecific interstitial pneumonia. Clin Pulm Med 2010; 17:122–128.
  25. Daniil ZD, Gilchrist FC, Nicholson AG, et al. A histologic pattern of nonspecific interstitial pneumonia is associated with a better prognosis than usual interstitial pneumonia in patients with cryptogenic fibrosing alveolitis. Am J Respir Crit Care Med 1999; 160:899–905.
  26. Travis WD, Matsui K, Moss J, Ferrans VJ. Idiopathic nonspecific interstitial pneumonia: prognostic significance of cellular and fibrosing patterns: survival comparison with usual interstitial pneumonia and desquamative interstitial pneumonia. Am J Surg Pathol 2000; 24:19–33.
  27. Riha RL, Duhig EE, Clarke BE, Steele RH, Slaughter RE, Zimmerman PV. Survival of patients with biopsy-proven usual interstitial pneumonia and nonspecific interstitial pneumonia. Eur Respir J 2002; 19:1114–1118.
  28. Raghu G, Collard HR, Egan JJ, et al; ATS/ERS/JRS/ALAT Committee on Idiopathic Pulmonary Fibrosis. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med 2011; 183:788–824.
  29. du Bois RM. An earlier and more confident diagnosis of idiopathic pulmonary fibrosis. Eur Respir Rev 2012; 21:141–146.
  30. Nishimura K, Kitaichi M, Izumi T, Nagai S, Kanaoka M, Itoh H. Usual interstitial pneumonia: histologic correlation with high-resolution CT. Radiology 1992; 182:337–342.
  31. Souza CA, Müller NL, Lee KS, Johkoh T, Mitsuhiro H, Chong S. Idiopathic interstitial pneumonias: prevalence of mediastinal lymph node enlargement in 206 patients. AJR Am J Roentgenol 2006; 186:995–999.
  32. King TE Jr, Bradford WZ, Castro-Bernardini S, et al; ASCEND Study Group. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med 2014; 370:2083–2092.
  33. Richeldi L, du Bois RM, Raghu G, et al; INPULSIS Trial Investigators. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med 2014; 370:2071–2082.
  34. Bradley B, Branley HM, Egan JJ, et al; British Thoracic Society Interstitial Lung Disease Guideline Group, British Thoracic Society Standards of Care Committee; Thoracic Society of Australia; New Zealand Thoracic Society; Irish Thoracic Society. Interstitial lung disease guideline: the British Thoracic Society in collaboration with the Thoracic Society of Australia and New Zealand and the Irish Thoracic Society. Thorax 2008; 63(suppl 5):v1–v58.
  35. Travis WD, Costabel U, Hansell DM, et al; ATS/ERS Committee on Idiopathic Interstitial Pneumonias. An official American Thoracic Society/European Respiratory Society statement: update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med 2013; 188:733–748.
  36. Stehlik J, Edwards LB, Kucheryavaya AY, et al; International Society of Heart and Lung Transplantation. The Registry of the International Society for Heart and Lung Transplantation: 29th official adult heart transplant report—2012. J Heart Lung Transplant 2012; 31:1052–1064.
  37. Oldham JM, Noth I. Idiopathic pulmonary fibrosis: early detection and referral. Respir Med 2014; 108:819–829.
References
  1. National Lung Screening Trial Research Team; Aberle DR, Adams AM, Berg CD, et al. Reduced lung-cancer mortality with low-dose computed tomographic screening. N Engl J Med 2011; 365:395–409.
  2. Detterbeck FC, Lewis SZ, Diekemper R, Addrizzo-Harris D, Alberts WM. Executive summary: diagnosis and management of lung cancer, 3rd ed: American College of Chest Physicians evidence-based clinical practice guidelines. Chest 2013; 143(suppl 5):7S–37S.
  3. Moyer VA; US Preventive Services Task Force. Screening for lung cancer: US Preventive Services Task Force recommendation statement. Ann Intern Med 2014; 160:330–338.
  4. Bach PB, Mirkin JN, Oliver TK, et al. Benefits and harms of CT screening for lung cancer: a systematic review. JAMA 2012; 307:2418–2429.
  5. Lamas DJ, Kawut SM, Bagiella E, Philip N, Arcasoy SM, Lederer DJ. Delayed access and survival in idiopathic pulmonary fibrosis: a cohort study. Am J Respir Crit Care Med 2011; 184:842–847.
  6. Swensen SJ, Jett JR, Hartman TE, et al. Lung cancer screening with CT: Mayo Clinic experience. Radiology 2003; 226:756–761.
  7. MacRedmond R, Logan PM, Lee M, Kenny D, Foley C, Costello RW. Screening for lung cancer using low dose CT scanning. Thorax 2004; 59:237–241.
  8. Sverzellati N, Guerci L, Randi G, et al. Interstitial lung diseases in a lung cancer screening trial. Eur Respir J 2011; 38:392–400.
  9. Jin GY, Lynch D, Chawla A, et al. Interstitial lung abnormalities in a CT lung cancer screening population: prevalence and progression rate. Radiology 2013; 268:563–571.
  10. Heyneman LE, Ward S, Lynch DA, Remy-Jardin M, Johkoh T, Müller NL. Respiratory bronchiolitis, respiratory bronchiolitis-associated interstitial lung disease, and desquamative interstitial pneumonia: different entities or part of the spectrum of the same disease process? AJR Am J Roentgenol 1999; 173:1617–1622.
  11. Moon J, du Bois RM, Colby TV, Hansell DM, Nicholson AG. Clinical significance of respiratory bronchiolitis on open lung biopsy and its relationship to smoking related interstitial lung disease. Thorax 1999; 54:1009–1014.
  12. Holt RM, Schmidt RA, Godwin JD, Raghu G. High resolution CT in respiratory bronchiolitis-associated interstitial lung disease. J Comput Assist Tomogr 1993; 17:46–50.
  13. Ryu JH, Myers JL, Capizzi SA, Douglas WW, Vassallo R, Decker PA. Desquamative interstitial pneumonia and respiratory bronchiolitis-associated interstitial lung disease. Chest 2005; 127:178–184.
  14. Hartman TE, Primack SL, Swensen SJ, Hansell D, McGuinness G, Müller NL. Desquamative interstitial pneumonia: thin-section CT findings in 22 patients. Radiology 1993; 187:787–790.
  15. Akira M, Yamamoto S, Hara H, Sakatani M, Ueda E. Serial computed tomographic evaluation in desquamative interstitial pneumonia. Thorax 1997; 52:333–337.
  16. Lacronique J, Roth C, Battesti JP, Basset F, Chretien J. Chest radiological features of pulmonary histiocytosis X: a report based on 50 adult cases. Thorax 1982; 37:104–109.
  17. Remy-Jardin M, Edme JL, Boulenguez C, Remy J, Mastora I, Sobaszek A. Longitudinal follow-up study of smoker’s lung with thin-section CT in correlation with pulmonary function tests. Radiology 2002; 222:261–270.
  18. Mueller-Mang C, Grosse C, Schmid K, Stiebellehner L, Bankier AA. What every radiologist should know about idiopathic interstitial pneumonias. Radiographics 2007; 27:595–615.
  19. Katzenstein AL, Fiorelli RF. Nonspecific interstitial pneumonia/fibrosis. Histologic features and clinical significance. Am J Surg Pathol 1994; 18:136–147.
  20. Bryson T, Sundaram B, Khanna D, Kazerooni EA. Connective tissue disease-associated interstitial pneumonia and idiopathic interstitial pneumonia: similarity and difference. Semin Ultrasound CT MR 2014; 35:29–38.
  21. Desai SR, Veeraraghavan S, Hansell DM, et al. CT features of lung disease in patients with systemic sclerosis: comparison with idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia. Radiology 2004; 232:560–567.
  22. Tsubamoto M, Müller NL, Johkoh T, et al. Pathologic subgroups of nonspecific interstitial pneumonia: differential diagnosis from other idiopathic interstitial pneumonias on high-resolution computed tomography. J Comput Assist Tomogr 2005; 29:793–800.
  23. Silva CI, Müller NL, Lynch DA, et al. Chronic hypersensitivity pneumonitis: differentiation from idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia by using thin-section CT. Radiology 2008; 246:288–297.
  24. Antin-Ozerkis D, Rubinowitz A. An update on nonspecific interstitial pneumonia. Clin Pulm Med 2010; 17:122–128.
  25. Daniil ZD, Gilchrist FC, Nicholson AG, et al. A histologic pattern of nonspecific interstitial pneumonia is associated with a better prognosis than usual interstitial pneumonia in patients with cryptogenic fibrosing alveolitis. Am J Respir Crit Care Med 1999; 160:899–905.
  26. Travis WD, Matsui K, Moss J, Ferrans VJ. Idiopathic nonspecific interstitial pneumonia: prognostic significance of cellular and fibrosing patterns: survival comparison with usual interstitial pneumonia and desquamative interstitial pneumonia. Am J Surg Pathol 2000; 24:19–33.
  27. Riha RL, Duhig EE, Clarke BE, Steele RH, Slaughter RE, Zimmerman PV. Survival of patients with biopsy-proven usual interstitial pneumonia and nonspecific interstitial pneumonia. Eur Respir J 2002; 19:1114–1118.
  28. Raghu G, Collard HR, Egan JJ, et al; ATS/ERS/JRS/ALAT Committee on Idiopathic Pulmonary Fibrosis. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med 2011; 183:788–824.
  29. du Bois RM. An earlier and more confident diagnosis of idiopathic pulmonary fibrosis. Eur Respir Rev 2012; 21:141–146.
  30. Nishimura K, Kitaichi M, Izumi T, Nagai S, Kanaoka M, Itoh H. Usual interstitial pneumonia: histologic correlation with high-resolution CT. Radiology 1992; 182:337–342.
  31. Souza CA, Müller NL, Lee KS, Johkoh T, Mitsuhiro H, Chong S. Idiopathic interstitial pneumonias: prevalence of mediastinal lymph node enlargement in 206 patients. AJR Am J Roentgenol 2006; 186:995–999.
  32. King TE Jr, Bradford WZ, Castro-Bernardini S, et al; ASCEND Study Group. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med 2014; 370:2083–2092.
  33. Richeldi L, du Bois RM, Raghu G, et al; INPULSIS Trial Investigators. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med 2014; 370:2071–2082.
  34. Bradley B, Branley HM, Egan JJ, et al; British Thoracic Society Interstitial Lung Disease Guideline Group, British Thoracic Society Standards of Care Committee; Thoracic Society of Australia; New Zealand Thoracic Society; Irish Thoracic Society. Interstitial lung disease guideline: the British Thoracic Society in collaboration with the Thoracic Society of Australia and New Zealand and the Irish Thoracic Society. Thorax 2008; 63(suppl 5):v1–v58.
  35. Travis WD, Costabel U, Hansell DM, et al; ATS/ERS Committee on Idiopathic Interstitial Pneumonias. An official American Thoracic Society/European Respiratory Society statement: update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med 2013; 188:733–748.
  36. Stehlik J, Edwards LB, Kucheryavaya AY, et al; International Society of Heart and Lung Transplantation. The Registry of the International Society for Heart and Lung Transplantation: 29th official adult heart transplant report—2012. J Heart Lung Transplant 2012; 31:1052–1064.
  37. Oldham JM, Noth I. Idiopathic pulmonary fibrosis: early detection and referral. Respir Med 2014; 108:819–829.
Issue
Cleveland Clinic Journal of Medicine - 83(1)
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Cleveland Clinic Journal of Medicine - 83(1)
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Managing interstitial lung disease detected on CT during lung cancer screening
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Managing interstitial lung disease detected on CT during lung cancer screening
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interstitial lung disease, computed tomography, CT, lung cancer screening, smoking, fibrotic, nonfibrotic, respiratory bronchiolitis, respiratory bronchiolitis-interstitial lung disease, desquamative interstitial pneumonia, pulmonary Langerhans cell histiocytosis, nonspecific interstitial pneumonia, usual interstitial pneumonia, idiopathic pulmonary fibrosis, Brian Southern, Rachel Scheraga, Ruchi Yadav
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interstitial lung disease, computed tomography, CT, lung cancer screening, smoking, fibrotic, nonfibrotic, respiratory bronchiolitis, respiratory bronchiolitis-interstitial lung disease, desquamative interstitial pneumonia, pulmonary Langerhans cell histiocytosis, nonspecific interstitial pneumonia, usual interstitial pneumonia, idiopathic pulmonary fibrosis, Brian Southern, Rachel Scheraga, Ruchi Yadav
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KEY POINTS

  • Smoking-related interstitial lung diseases can broadly be categorized as fibrotic or nonfibrotic on the basis of their appearance on CT. Fibrotic disease generally carries a worse prognosis.
  • Nonfibrotic interstitial lung diseases include respiratory bronchiolitis, respiratory bronchiolitis-interstitial lung disease, desquamative interstitial pneumonia, and pulmonary Langerhans cell histiocytosis.
  • Smoking-related fibrotic interstitial lung diseases include nonspecific interstitial pneumonia and usual interstitial pneumonia. A subset of usual interstitial pneumonia, called idiopathic pulmonary fibrosis, carries the worst prognosis of all.
  • If CT detects interstitial lung disease during screening for lung cancer, the clinician should strongly consider further evaluation with dedicated high-resolution CT and early referral to a specialist. Smoking cessation is extremely important.
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Eventration of the diaphragm presenting as spontaneous pneumothorax

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Eventration of the diaphragm presenting as spontaneous pneumothorax

A 25-year-old man with a 2-day history of upper respiratory tract infection presents to the emergency department with the sudden onset of right-sided back and chest pain and shortness of breath after a severe coughing fit.

He is morbidly obese, is a long-time smoker, and has had recurrent exacerbations of asthma with frequent upper respiratory tract infections. He has no history of recent trauma.

A review of systems reveals no significant impairment in exercise tolerance. He has been able to continue doing manual labor at his job as a railroad worker.

Figure 1. Plain radiography shows a large right pneumothorax and an elevated right diaphragm.

Figure 2. Thoracic computed tomography shows right anterior apical pneumothorax with atelectatic and hypoplastic lung in a cross-sectional view (A). A coronal view (B) shows significant elevation of the right hemidiaphragm, with fat, bowel, and kidney within the right hemithorax.

Radiography shows a large right pneumothorax and an elevated right diaphragm (Figure 1). Computed tomography (CT) (Figure 2) reveals a right anterior apical pneumothorax with hypoplastic lung and significant elevation of the right diaphragm with fat, bowel, and kidney within the right thorax. He is hemodynamically stable and shows no signs of bowel obstruction.

The physical examination is normal except for diminished breath sounds on the right side. He is diagnosed with congenital diaphragmatic hernia and spontaneous pneumothorax. A 10-F locking pigtail catheter is inserted under CT guidance, leading to complete resolution of the pneumothorax. He is discharged home the next day with a plan for elective repair of the hernia.

Two months later, he returns for scheduled right thoracotomy to repair the hernia. However, while preparing the chest cavity, the surgeon finds no diaphragmatic hernia and no intra-abdominal content—but rather, a severely elevated and thinned-out diaphragm with uninterrupted continuity. The diagnosis is changed to congenital diaphragmatic eventration, and plication of the diaphragm is performed with a series of interrupted, pledgeted polypropylene sutures.

CONGENITAL EVENTRATION OF THE DIAPHRAGM

Congenital diaphragmatic eventration is a rare developmental defect of the central, muscular portion of the diaphragm. The true prevalence is not known, but early reports identified this condition in less than 0.1% of adult.1

Symptomatic patients usually experience dyspnea secondary to ventilation-perfusion mismatch resulting from chronic atelectasis and lung hypoplasia, as well as impaired ventilation resulting from the limited caudal migration of the diaphragm.2,3 Increased susceptibility to recurrent upper respiratory tract infections and pneumonia is also a common feature.

Although rare, spontaneous pneumothorax can develop in patients such as ours, whose lengthy history of smoking and asthma predisposed him to the development of emphysema-like blebs and bullae and to subsequent rupture of blebs brought on by vigorous coughing that caused an involuntary Valsalva maneuver.4

As in our patient, distinguishing congenital diaphragmatic eventration from hernia preoperatively can be difficult with plain chest radiography. Spiral CT with multiplanar reconstruction or with magnetic resonance imaging can help establish the diagnosis.3 However, a severely attenuated diaphragm can be difficult to visualize on CT, as in our patient, leading to a presumptive diagnosis of diaphragmatic hernia. In such situations, the diagnosis of eventration can only be made intraoperatively.

Surgical repair is indicated only for patients with symptoms. Other potential causes of the symptoms should first be ruled out, however, including primary pulmonary disease, cardiac dysfunction, and morbid obesity.

References
  1. Chin EF, Lynn RB. Surgery of eventration of the diaphragm. J Thorac Surg 1956; 32:6–14.
  2. Ridyard JB, Stewart RM. Regional lung function in unilateral diaphragmatic paralysis. Thorax 1976; 31:438–442.
  3. Shen C, Che G. Congenital eventration of hemidiaphragm in an adult. Ann Thorac Surg 2012; 94:e137–e139.
  4. Porpodis K, Zarogoulidis P, Spyratos D, et al. Pneumothorax and asthma. J Thorac Dis 2014; 6(suppl 1):S152–S161.
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Pauline Go, MD
Department of Surgery, Henry Ford Hospital, Detroit, MI

Zane Hammoud, MD
Division of Thoracic Surgery, Henry Ford Hospital, Detroit, MI

Address: Pauline Go, MD, Department of Surgery, CFP-127, Henry Ford Hospital, 2799 West Grand Boulevard, Detroit, MI 48202; e-mail: [email protected]

Dr. Hammoud has disclosed consulting for Ethicon Inc.

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Pauline Go, MD
Department of Surgery, Henry Ford Hospital, Detroit, MI

Zane Hammoud, MD
Division of Thoracic Surgery, Henry Ford Hospital, Detroit, MI

Address: Pauline Go, MD, Department of Surgery, CFP-127, Henry Ford Hospital, 2799 West Grand Boulevard, Detroit, MI 48202; e-mail: [email protected]

Dr. Hammoud has disclosed consulting for Ethicon Inc.

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Pauline Go, MD
Department of Surgery, Henry Ford Hospital, Detroit, MI

Zane Hammoud, MD
Division of Thoracic Surgery, Henry Ford Hospital, Detroit, MI

Address: Pauline Go, MD, Department of Surgery, CFP-127, Henry Ford Hospital, 2799 West Grand Boulevard, Detroit, MI 48202; e-mail: [email protected]

Dr. Hammoud has disclosed consulting for Ethicon Inc.

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Related Articles

A 25-year-old man with a 2-day history of upper respiratory tract infection presents to the emergency department with the sudden onset of right-sided back and chest pain and shortness of breath after a severe coughing fit.

He is morbidly obese, is a long-time smoker, and has had recurrent exacerbations of asthma with frequent upper respiratory tract infections. He has no history of recent trauma.

A review of systems reveals no significant impairment in exercise tolerance. He has been able to continue doing manual labor at his job as a railroad worker.

Figure 1. Plain radiography shows a large right pneumothorax and an elevated right diaphragm.

Figure 2. Thoracic computed tomography shows right anterior apical pneumothorax with atelectatic and hypoplastic lung in a cross-sectional view (A). A coronal view (B) shows significant elevation of the right hemidiaphragm, with fat, bowel, and kidney within the right hemithorax.

Radiography shows a large right pneumothorax and an elevated right diaphragm (Figure 1). Computed tomography (CT) (Figure 2) reveals a right anterior apical pneumothorax with hypoplastic lung and significant elevation of the right diaphragm with fat, bowel, and kidney within the right thorax. He is hemodynamically stable and shows no signs of bowel obstruction.

The physical examination is normal except for diminished breath sounds on the right side. He is diagnosed with congenital diaphragmatic hernia and spontaneous pneumothorax. A 10-F locking pigtail catheter is inserted under CT guidance, leading to complete resolution of the pneumothorax. He is discharged home the next day with a plan for elective repair of the hernia.

Two months later, he returns for scheduled right thoracotomy to repair the hernia. However, while preparing the chest cavity, the surgeon finds no diaphragmatic hernia and no intra-abdominal content—but rather, a severely elevated and thinned-out diaphragm with uninterrupted continuity. The diagnosis is changed to congenital diaphragmatic eventration, and plication of the diaphragm is performed with a series of interrupted, pledgeted polypropylene sutures.

CONGENITAL EVENTRATION OF THE DIAPHRAGM

Congenital diaphragmatic eventration is a rare developmental defect of the central, muscular portion of the diaphragm. The true prevalence is not known, but early reports identified this condition in less than 0.1% of adult.1

Symptomatic patients usually experience dyspnea secondary to ventilation-perfusion mismatch resulting from chronic atelectasis and lung hypoplasia, as well as impaired ventilation resulting from the limited caudal migration of the diaphragm.2,3 Increased susceptibility to recurrent upper respiratory tract infections and pneumonia is also a common feature.

Although rare, spontaneous pneumothorax can develop in patients such as ours, whose lengthy history of smoking and asthma predisposed him to the development of emphysema-like blebs and bullae and to subsequent rupture of blebs brought on by vigorous coughing that caused an involuntary Valsalva maneuver.4

As in our patient, distinguishing congenital diaphragmatic eventration from hernia preoperatively can be difficult with plain chest radiography. Spiral CT with multiplanar reconstruction or with magnetic resonance imaging can help establish the diagnosis.3 However, a severely attenuated diaphragm can be difficult to visualize on CT, as in our patient, leading to a presumptive diagnosis of diaphragmatic hernia. In such situations, the diagnosis of eventration can only be made intraoperatively.

Surgical repair is indicated only for patients with symptoms. Other potential causes of the symptoms should first be ruled out, however, including primary pulmonary disease, cardiac dysfunction, and morbid obesity.

A 25-year-old man with a 2-day history of upper respiratory tract infection presents to the emergency department with the sudden onset of right-sided back and chest pain and shortness of breath after a severe coughing fit.

He is morbidly obese, is a long-time smoker, and has had recurrent exacerbations of asthma with frequent upper respiratory tract infections. He has no history of recent trauma.

A review of systems reveals no significant impairment in exercise tolerance. He has been able to continue doing manual labor at his job as a railroad worker.

Figure 1. Plain radiography shows a large right pneumothorax and an elevated right diaphragm.

Figure 2. Thoracic computed tomography shows right anterior apical pneumothorax with atelectatic and hypoplastic lung in a cross-sectional view (A). A coronal view (B) shows significant elevation of the right hemidiaphragm, with fat, bowel, and kidney within the right hemithorax.

Radiography shows a large right pneumothorax and an elevated right diaphragm (Figure 1). Computed tomography (CT) (Figure 2) reveals a right anterior apical pneumothorax with hypoplastic lung and significant elevation of the right diaphragm with fat, bowel, and kidney within the right thorax. He is hemodynamically stable and shows no signs of bowel obstruction.

The physical examination is normal except for diminished breath sounds on the right side. He is diagnosed with congenital diaphragmatic hernia and spontaneous pneumothorax. A 10-F locking pigtail catheter is inserted under CT guidance, leading to complete resolution of the pneumothorax. He is discharged home the next day with a plan for elective repair of the hernia.

Two months later, he returns for scheduled right thoracotomy to repair the hernia. However, while preparing the chest cavity, the surgeon finds no diaphragmatic hernia and no intra-abdominal content—but rather, a severely elevated and thinned-out diaphragm with uninterrupted continuity. The diagnosis is changed to congenital diaphragmatic eventration, and plication of the diaphragm is performed with a series of interrupted, pledgeted polypropylene sutures.

CONGENITAL EVENTRATION OF THE DIAPHRAGM

Congenital diaphragmatic eventration is a rare developmental defect of the central, muscular portion of the diaphragm. The true prevalence is not known, but early reports identified this condition in less than 0.1% of adult.1

Symptomatic patients usually experience dyspnea secondary to ventilation-perfusion mismatch resulting from chronic atelectasis and lung hypoplasia, as well as impaired ventilation resulting from the limited caudal migration of the diaphragm.2,3 Increased susceptibility to recurrent upper respiratory tract infections and pneumonia is also a common feature.

Although rare, spontaneous pneumothorax can develop in patients such as ours, whose lengthy history of smoking and asthma predisposed him to the development of emphysema-like blebs and bullae and to subsequent rupture of blebs brought on by vigorous coughing that caused an involuntary Valsalva maneuver.4

As in our patient, distinguishing congenital diaphragmatic eventration from hernia preoperatively can be difficult with plain chest radiography. Spiral CT with multiplanar reconstruction or with magnetic resonance imaging can help establish the diagnosis.3 However, a severely attenuated diaphragm can be difficult to visualize on CT, as in our patient, leading to a presumptive diagnosis of diaphragmatic hernia. In such situations, the diagnosis of eventration can only be made intraoperatively.

Surgical repair is indicated only for patients with symptoms. Other potential causes of the symptoms should first be ruled out, however, including primary pulmonary disease, cardiac dysfunction, and morbid obesity.

References
  1. Chin EF, Lynn RB. Surgery of eventration of the diaphragm. J Thorac Surg 1956; 32:6–14.
  2. Ridyard JB, Stewart RM. Regional lung function in unilateral diaphragmatic paralysis. Thorax 1976; 31:438–442.
  3. Shen C, Che G. Congenital eventration of hemidiaphragm in an adult. Ann Thorac Surg 2012; 94:e137–e139.
  4. Porpodis K, Zarogoulidis P, Spyratos D, et al. Pneumothorax and asthma. J Thorac Dis 2014; 6(suppl 1):S152–S161.
References
  1. Chin EF, Lynn RB. Surgery of eventration of the diaphragm. J Thorac Surg 1956; 32:6–14.
  2. Ridyard JB, Stewart RM. Regional lung function in unilateral diaphragmatic paralysis. Thorax 1976; 31:438–442.
  3. Shen C, Che G. Congenital eventration of hemidiaphragm in an adult. Ann Thorac Surg 2012; 94:e137–e139.
  4. Porpodis K, Zarogoulidis P, Spyratos D, et al. Pneumothorax and asthma. J Thorac Dis 2014; 6(suppl 1):S152–S161.
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Emergency Ultrasound: Deep Vein Thrombosis

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Emergency Ultrasound: Deep Vein Thrombosis
Among the various imaging modalities to assess patients resenting with signs and symptoms of deep vein thrombosis, bedside ultrasound is an excellent starting point.

Point-of-care (POC) ultrasound for the evaluation of deep vein thrombosis (DVT) is a two-region compression examination evaluating for clot in the femoral and popliteal regions. To begin the study, the clinician should place the high-frequency linear probe just distal and parallel to the inguinal ligament in a transverse orientation relative to the underlying vessels. Regarding proper positioning, the patient’s knees should be slightly flexed and the hip externally rotated when performing the examination (Figure 1). If there are any issues in visualization, the clinician should attempt to maximize depth by moving the probe medially. To ensure patient and clinician comfort, the patient should be appropriately draped prior to the examination.

Femoral and Saphenous Vein

After the appropriate positioning has been achieved, the clinician should identify the common femoral vein and artery, and then slide the probe toward the head or feet until the saphenous vein, which empties into the common femoral vein, is identified

(Figure 2). This area can be a nidus for clot formation and must be thoroughly evaluated to ensure a complete study.

The clinician should take care not to apply too much pressure with the probe as this will compress the saphenous vein; therefore, it is important to use a light touch to ensure proper visualization. Once this key region is identified, the clinician should then use the probe to apply pressure to the vessels. The saphenous vein should compress completely upon application of sufficient pressure to the artery. In a normal vein, the anterior and posterior walls should touch and completely obliterate the lumen of the vein. When a clot is present, the walls of the vein will remain separated (Figure 3). A thrombus can be bright on ultrasound, but some DVTs may appear completely black. In some cases, a lack of compressibility may be the only tip-off to the presence of clot.

After fully evaluating the saphenous vein, the clinician should move the probe toward the feet, keeping the vessels centered in the image, while at the same time applying serial compression approximately every centimeter. As the probe is moved distally, the artery will typically bifurcate first and then the vein will bifurcate into the femoral and deep femoral branches. The femoral vein should be followed until it “dives” into the adductor canal. 

Popliteal Region

The second zone of the examination is in the popliteal region. It can be helpful to prop the foot up, or hang the foot off the bed to improve access to this region (Figure 4). Similar to the femoral region, maximizing your depth and then decreasing it once the vessels are identified can help to ensure that you are not mistaking superficial vessels

for the popliteal artery and vein. The field of view is adequate when the underlying bone is visualized on the image. Typically, the popliteal vein will be just superficial to the artery (Figure 5). Once the vessels have been identified, they should be traced back proximally to the first point they are visualized in the popliteal fossa. From this starting point, it is important that the clinician apply serial compression distally until the vessel trifurcates. 

Common Pitfalls

The main pitfall to performing a DVT study is failing to identify the correct vessels. It is important to identify both the artery and vein running together to ensure that the correct location. In larger patients, the relatively shallow depth of the high-frequency

probe may be inadequate limiting the ability to obtain adequate images.

Another common pitfall to keep in mind is that lymph nodes can often be mistaken for clot (Figure 6). A lymph node may be distinguished from a clot by sliding the probe up and down the patient’s leg—lymph nodes will appear as discrete structures, continuous like vasculature. It is essential to evaluate the length of the common femoral, the femoral, and popliteal veins visualized as a recent study by Adhikari et al1 showed that two-point compression studies can miss up to 6% of isolated clots.

Summary

With practice, POC compression ultrasonography of the lower extremities can be used to quickly rule in the diagnosis DVT. Proper patient and probe positioning, as well as the application of appropriate probe pressure at different stages of the examination are essential to accurately visualize and assess the femoral, saphenous, and popliteal veins for the presence of a thrombus.

For a video and more information on performing this scan:

http://www.em.emory.edu/ultrasound/ImageWeek/Soft%20Tissue/Femoral%20DVT.html.

  

  

  

  

  

  

  

  


Dr Meer is an assistant professor and director of emergency ultrasound, department of emergency medicine, Emory University School of Medicine, Atlanta, Georgia. Dr Beck is an assistant professor, department of emergency medicine, Emory University School of Medicine, Atlanta, Georgia. Dr Taylor is an assistant professor and director of postgraduate medical education, department of emergency medicine, Emory University School of Medicine, Atlanta, Georgia.

References

- Emergency Ultrasound: Deep Vein Thrombosis

  1. Adhikari S, Zeger W, Thom C, Fields JM. Isolated deep venous thrombosis: implications for 2-point compression ultrasonography of the lower extremity. Ann Emerg Med. 2015;66(3):262-266.
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Among the various imaging modalities to assess patients resenting with signs and symptoms of deep vein thrombosis, bedside ultrasound is an excellent starting point.
Among the various imaging modalities to assess patients resenting with signs and symptoms of deep vein thrombosis, bedside ultrasound is an excellent starting point.

Point-of-care (POC) ultrasound for the evaluation of deep vein thrombosis (DVT) is a two-region compression examination evaluating for clot in the femoral and popliteal regions. To begin the study, the clinician should place the high-frequency linear probe just distal and parallel to the inguinal ligament in a transverse orientation relative to the underlying vessels. Regarding proper positioning, the patient’s knees should be slightly flexed and the hip externally rotated when performing the examination (Figure 1). If there are any issues in visualization, the clinician should attempt to maximize depth by moving the probe medially. To ensure patient and clinician comfort, the patient should be appropriately draped prior to the examination.

Femoral and Saphenous Vein

After the appropriate positioning has been achieved, the clinician should identify the common femoral vein and artery, and then slide the probe toward the head or feet until the saphenous vein, which empties into the common femoral vein, is identified

(Figure 2). This area can be a nidus for clot formation and must be thoroughly evaluated to ensure a complete study.

The clinician should take care not to apply too much pressure with the probe as this will compress the saphenous vein; therefore, it is important to use a light touch to ensure proper visualization. Once this key region is identified, the clinician should then use the probe to apply pressure to the vessels. The saphenous vein should compress completely upon application of sufficient pressure to the artery. In a normal vein, the anterior and posterior walls should touch and completely obliterate the lumen of the vein. When a clot is present, the walls of the vein will remain separated (Figure 3). A thrombus can be bright on ultrasound, but some DVTs may appear completely black. In some cases, a lack of compressibility may be the only tip-off to the presence of clot.

After fully evaluating the saphenous vein, the clinician should move the probe toward the feet, keeping the vessels centered in the image, while at the same time applying serial compression approximately every centimeter. As the probe is moved distally, the artery will typically bifurcate first and then the vein will bifurcate into the femoral and deep femoral branches. The femoral vein should be followed until it “dives” into the adductor canal. 

Popliteal Region

The second zone of the examination is in the popliteal region. It can be helpful to prop the foot up, or hang the foot off the bed to improve access to this region (Figure 4). Similar to the femoral region, maximizing your depth and then decreasing it once the vessels are identified can help to ensure that you are not mistaking superficial vessels

for the popliteal artery and vein. The field of view is adequate when the underlying bone is visualized on the image. Typically, the popliteal vein will be just superficial to the artery (Figure 5). Once the vessels have been identified, they should be traced back proximally to the first point they are visualized in the popliteal fossa. From this starting point, it is important that the clinician apply serial compression distally until the vessel trifurcates. 

Common Pitfalls

The main pitfall to performing a DVT study is failing to identify the correct vessels. It is important to identify both the artery and vein running together to ensure that the correct location. In larger patients, the relatively shallow depth of the high-frequency

probe may be inadequate limiting the ability to obtain adequate images.

Another common pitfall to keep in mind is that lymph nodes can often be mistaken for clot (Figure 6). A lymph node may be distinguished from a clot by sliding the probe up and down the patient’s leg—lymph nodes will appear as discrete structures, continuous like vasculature. It is essential to evaluate the length of the common femoral, the femoral, and popliteal veins visualized as a recent study by Adhikari et al1 showed that two-point compression studies can miss up to 6% of isolated clots.

Summary

With practice, POC compression ultrasonography of the lower extremities can be used to quickly rule in the diagnosis DVT. Proper patient and probe positioning, as well as the application of appropriate probe pressure at different stages of the examination are essential to accurately visualize and assess the femoral, saphenous, and popliteal veins for the presence of a thrombus.

For a video and more information on performing this scan:

http://www.em.emory.edu/ultrasound/ImageWeek/Soft%20Tissue/Femoral%20DVT.html.

  

  

  

  

  

  

  

  


Dr Meer is an assistant professor and director of emergency ultrasound, department of emergency medicine, Emory University School of Medicine, Atlanta, Georgia. Dr Beck is an assistant professor, department of emergency medicine, Emory University School of Medicine, Atlanta, Georgia. Dr Taylor is an assistant professor and director of postgraduate medical education, department of emergency medicine, Emory University School of Medicine, Atlanta, Georgia.

Point-of-care (POC) ultrasound for the evaluation of deep vein thrombosis (DVT) is a two-region compression examination evaluating for clot in the femoral and popliteal regions. To begin the study, the clinician should place the high-frequency linear probe just distal and parallel to the inguinal ligament in a transverse orientation relative to the underlying vessels. Regarding proper positioning, the patient’s knees should be slightly flexed and the hip externally rotated when performing the examination (Figure 1). If there are any issues in visualization, the clinician should attempt to maximize depth by moving the probe medially. To ensure patient and clinician comfort, the patient should be appropriately draped prior to the examination.

Femoral and Saphenous Vein

After the appropriate positioning has been achieved, the clinician should identify the common femoral vein and artery, and then slide the probe toward the head or feet until the saphenous vein, which empties into the common femoral vein, is identified

(Figure 2). This area can be a nidus for clot formation and must be thoroughly evaluated to ensure a complete study.

The clinician should take care not to apply too much pressure with the probe as this will compress the saphenous vein; therefore, it is important to use a light touch to ensure proper visualization. Once this key region is identified, the clinician should then use the probe to apply pressure to the vessels. The saphenous vein should compress completely upon application of sufficient pressure to the artery. In a normal vein, the anterior and posterior walls should touch and completely obliterate the lumen of the vein. When a clot is present, the walls of the vein will remain separated (Figure 3). A thrombus can be bright on ultrasound, but some DVTs may appear completely black. In some cases, a lack of compressibility may be the only tip-off to the presence of clot.

After fully evaluating the saphenous vein, the clinician should move the probe toward the feet, keeping the vessels centered in the image, while at the same time applying serial compression approximately every centimeter. As the probe is moved distally, the artery will typically bifurcate first and then the vein will bifurcate into the femoral and deep femoral branches. The femoral vein should be followed until it “dives” into the adductor canal. 

Popliteal Region

The second zone of the examination is in the popliteal region. It can be helpful to prop the foot up, or hang the foot off the bed to improve access to this region (Figure 4). Similar to the femoral region, maximizing your depth and then decreasing it once the vessels are identified can help to ensure that you are not mistaking superficial vessels

for the popliteal artery and vein. The field of view is adequate when the underlying bone is visualized on the image. Typically, the popliteal vein will be just superficial to the artery (Figure 5). Once the vessels have been identified, they should be traced back proximally to the first point they are visualized in the popliteal fossa. From this starting point, it is important that the clinician apply serial compression distally until the vessel trifurcates. 

Common Pitfalls

The main pitfall to performing a DVT study is failing to identify the correct vessels. It is important to identify both the artery and vein running together to ensure that the correct location. In larger patients, the relatively shallow depth of the high-frequency

probe may be inadequate limiting the ability to obtain adequate images.

Another common pitfall to keep in mind is that lymph nodes can often be mistaken for clot (Figure 6). A lymph node may be distinguished from a clot by sliding the probe up and down the patient’s leg—lymph nodes will appear as discrete structures, continuous like vasculature. It is essential to evaluate the length of the common femoral, the femoral, and popliteal veins visualized as a recent study by Adhikari et al1 showed that two-point compression studies can miss up to 6% of isolated clots.

Summary

With practice, POC compression ultrasonography of the lower extremities can be used to quickly rule in the diagnosis DVT. Proper patient and probe positioning, as well as the application of appropriate probe pressure at different stages of the examination are essential to accurately visualize and assess the femoral, saphenous, and popliteal veins for the presence of a thrombus.

For a video and more information on performing this scan:

http://www.em.emory.edu/ultrasound/ImageWeek/Soft%20Tissue/Femoral%20DVT.html.

  

  

  

  

  

  

  

  


Dr Meer is an assistant professor and director of emergency ultrasound, department of emergency medicine, Emory University School of Medicine, Atlanta, Georgia. Dr Beck is an assistant professor, department of emergency medicine, Emory University School of Medicine, Atlanta, Georgia. Dr Taylor is an assistant professor and director of postgraduate medical education, department of emergency medicine, Emory University School of Medicine, Atlanta, Georgia.

References

- Emergency Ultrasound: Deep Vein Thrombosis

  1. Adhikari S, Zeger W, Thom C, Fields JM. Isolated deep venous thrombosis: implications for 2-point compression ultrasonography of the lower extremity. Ann Emerg Med. 2015;66(3):262-266.
References

- Emergency Ultrasound: Deep Vein Thrombosis

  1. Adhikari S, Zeger W, Thom C, Fields JM. Isolated deep venous thrombosis: implications for 2-point compression ultrasonography of the lower extremity. Ann Emerg Med. 2015;66(3):262-266.
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Case Report: Perianal Streptococcal Infection

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Case Report: Perianal Streptococcal Infection
A 3-year-old boy is brought to the ED for evaluation of perianal desquamation.

Case

The mother of a 3-year-old boy presented her son to the ED for evaluation after she noticed peeling of the skin in his perianal region. She stated that the peeling had started 1 day prior to presentation. Two days earlier, the mother had brought the same patient to the ED for evaluation of a fever, sore throat, and a slight rash over his face. The boy’s vital signs at the initial presentation were: temperature, 101.8°F; heart rate, 102 beats/minute; and respiratory rate, 28 breaths/minute. Oxygen saturation was 98% on room air.

During this first visit, the mother denied the child having had any fever, chills, headache, sore throat, facial rash, joint pain, or pain on defecation. He had no significant medical history and no known drug allergies. After examination, a throat culture was taken, and the patient was given acetaminophen and discharged home with a diagnosis of viral syndrome.

At the second presentation, physical examination revealed a well-developed child in no distress. The examination was negative except for a 4 x 2 cm area of desquamation present over the perianal region (Figure).

The area of desquamation was dry, mildly erythematous without discharge, and nontender. The patient’s vital signs at this presentation were stable, and he was afebrile. The remaining physical examination findings were normal. The throat culture taken during the first ED presentation was reported as negative. A perianal swab was sent for culture and sensitivity. This was later reported to be positive for group A β-hemolytic streptococci (GABHS), which is sensitive to penicillin. The patient was discharged home in the care of his mother with a prescription of penicillin.  A 10-day follow-up showed complete resolution of the skin rash.

  

Discussion

Perianal streptococcal dermatitis (PSD), which is caused by GABHS, is a frequently overlooked medical entity. Landolt et al1 investigated the prevalence of PSD at the University Children’s Hospital Basel, Switzerland, from October 2000 to May 2001. In this study, 250 randomly selected patients were studied for signs of PSD by history, examination, and culture, the results of which showed that PSD was frequent in the study cohort. The study further underscored that, to appropriately treat affected patients, signs and symptoms of PSD should be searched for systematically.1

The rash in this case was most likely the result of scarlet fever with an unusual presentation of PSD; the signs and symptoms of which include perianal erythema, itching, rectal pain, sometimes blood-streaked stools, rectal bleeding, irritation or pruritus, tissue loss and exudation, secondary constipation, and cellulitis. Perianal streptococcal dermatitis has also been described in the adult literature.2 As with pediatric cases, PSD in adults is usually caused by GABHS.

Evaluation and Diagnosis

A rapid streptococcal test of suspicious areas can confirm the diagnosis. Fever, sore throat, and arthralgia are rare; however, culture from the perianal region grows GABHS. Titers are usually not elevated in laboratory evaluation. A routine skin culture is an alternative diagnostic aid.

Brilliant2 described the bright red color of PSD as a sharply demarcated rash that is caused by GABHS. As previously stated, symptoms include perianal rash, itching, and rectal pain; blood-streaked stools may also be seen in one-third of patients. It primarily occurs in children between 6 months and 10 years of age and is often misdiagnosed and treated inappropriately.3

Prompt diagnosis of GABHS is important. If untreated, it can cause serious systemic infections, especially in elderly and in newborn patients. Treatment with antibiotics resolves the condition in the majority of patients.2 In the acute stage, a white pseudomembrane may be present. As the rash becomes more chronic, the perianal eruption may consist of painful fissures, a dry mucoid discharge, or psoriasiform plaques. Perianal dermatitis can also be caused by Staphylococcus aureus or Candida. Confirmation of the diagnosis is accomplished by culturing a moderate-to-heavy growth of GABHS on 5% sheep-blood agar.

Treatment

A 10-day course of oral penicillin produces resolution of the dermatitis and other symptoms in most patients, but a relapse rate as high as 39% has been reported. Other treatment plans include amoxicillin, 40 mg/kg per day, divided into three doses, and/or topical applications of mupirocin 2% three times per day for 10 days. Penicillin, clindamycin phosphate, and erythromycin have also been used.

Although penicillin is generally recommended for treatment of GABHS infection, amoxicillin is often better tolerated in the pediatric population due to its superior palatability. Early antibiotic treatment causes a dramatic and rapid improvement of symptoms. However, according to Olson et al,4 PSD initially treated with amoxicillin or penicillin is consistently associated with a high risk of clinical recurrence. Whether treatment with a β-lactamase–resistant agent reduces this risk is uncertain.

 

 

  

Conclusion

This case represents an unusual presentation of scarlet fever manifesting as perianal dermatitis caused by GABHS. Although more common in the pediatric population, adult cases have been documented in the literature. As this case illustrates, early recognition and treatment with penicillin (or amoxicillin) produces rapid improvement and resolution of symptoms. 

  


Dr Nibhanipudi is a professor of clinical emergency medicine at New York Medical College - Metropolitan Hospital Center, New York.

References

- Case Report: Perianal Streptococcal Infection

  1. Landolt M, Heininger U. Prevalence of perianal streptococcal dermatitis in children and adolescents [in German]. Praxis (Bern 1994). 2005;94(38):1467-1471.
  2. Kahlke V, Jongen J, Peleikis HG, Herbst RA. Perianal streptococcal dermatitis in adults: its association with pruritic anorectal diseases is mainly caused by group B Streptococci. Colorectal Dis. 2013;15(5):602-607.
  3. Brilliant LC. Perianal streptococcal dermatitis. Am Fam Physician. 2000;61(2):391-393.
  4. Olson D, Edmonson MB. Outcomes in children treated for perineal group A beta-hemolytic streptococcal dermatitis. Pediatr Infect Dis J. 2011;30(11):933-936.
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A 3-year-old boy is brought to the ED for evaluation of perianal desquamation.
A 3-year-old boy is brought to the ED for evaluation of perianal desquamation.

Case

The mother of a 3-year-old boy presented her son to the ED for evaluation after she noticed peeling of the skin in his perianal region. She stated that the peeling had started 1 day prior to presentation. Two days earlier, the mother had brought the same patient to the ED for evaluation of a fever, sore throat, and a slight rash over his face. The boy’s vital signs at the initial presentation were: temperature, 101.8°F; heart rate, 102 beats/minute; and respiratory rate, 28 breaths/minute. Oxygen saturation was 98% on room air.

During this first visit, the mother denied the child having had any fever, chills, headache, sore throat, facial rash, joint pain, or pain on defecation. He had no significant medical history and no known drug allergies. After examination, a throat culture was taken, and the patient was given acetaminophen and discharged home with a diagnosis of viral syndrome.

At the second presentation, physical examination revealed a well-developed child in no distress. The examination was negative except for a 4 x 2 cm area of desquamation present over the perianal region (Figure).

The area of desquamation was dry, mildly erythematous without discharge, and nontender. The patient’s vital signs at this presentation were stable, and he was afebrile. The remaining physical examination findings were normal. The throat culture taken during the first ED presentation was reported as negative. A perianal swab was sent for culture and sensitivity. This was later reported to be positive for group A β-hemolytic streptococci (GABHS), which is sensitive to penicillin. The patient was discharged home in the care of his mother with a prescription of penicillin.  A 10-day follow-up showed complete resolution of the skin rash.

  

Discussion

Perianal streptococcal dermatitis (PSD), which is caused by GABHS, is a frequently overlooked medical entity. Landolt et al1 investigated the prevalence of PSD at the University Children’s Hospital Basel, Switzerland, from October 2000 to May 2001. In this study, 250 randomly selected patients were studied for signs of PSD by history, examination, and culture, the results of which showed that PSD was frequent in the study cohort. The study further underscored that, to appropriately treat affected patients, signs and symptoms of PSD should be searched for systematically.1

The rash in this case was most likely the result of scarlet fever with an unusual presentation of PSD; the signs and symptoms of which include perianal erythema, itching, rectal pain, sometimes blood-streaked stools, rectal bleeding, irritation or pruritus, tissue loss and exudation, secondary constipation, and cellulitis. Perianal streptococcal dermatitis has also been described in the adult literature.2 As with pediatric cases, PSD in adults is usually caused by GABHS.

Evaluation and Diagnosis

A rapid streptococcal test of suspicious areas can confirm the diagnosis. Fever, sore throat, and arthralgia are rare; however, culture from the perianal region grows GABHS. Titers are usually not elevated in laboratory evaluation. A routine skin culture is an alternative diagnostic aid.

Brilliant2 described the bright red color of PSD as a sharply demarcated rash that is caused by GABHS. As previously stated, symptoms include perianal rash, itching, and rectal pain; blood-streaked stools may also be seen in one-third of patients. It primarily occurs in children between 6 months and 10 years of age and is often misdiagnosed and treated inappropriately.3

Prompt diagnosis of GABHS is important. If untreated, it can cause serious systemic infections, especially in elderly and in newborn patients. Treatment with antibiotics resolves the condition in the majority of patients.2 In the acute stage, a white pseudomembrane may be present. As the rash becomes more chronic, the perianal eruption may consist of painful fissures, a dry mucoid discharge, or psoriasiform plaques. Perianal dermatitis can also be caused by Staphylococcus aureus or Candida. Confirmation of the diagnosis is accomplished by culturing a moderate-to-heavy growth of GABHS on 5% sheep-blood agar.

Treatment

A 10-day course of oral penicillin produces resolution of the dermatitis and other symptoms in most patients, but a relapse rate as high as 39% has been reported. Other treatment plans include amoxicillin, 40 mg/kg per day, divided into three doses, and/or topical applications of mupirocin 2% three times per day for 10 days. Penicillin, clindamycin phosphate, and erythromycin have also been used.

Although penicillin is generally recommended for treatment of GABHS infection, amoxicillin is often better tolerated in the pediatric population due to its superior palatability. Early antibiotic treatment causes a dramatic and rapid improvement of symptoms. However, according to Olson et al,4 PSD initially treated with amoxicillin or penicillin is consistently associated with a high risk of clinical recurrence. Whether treatment with a β-lactamase–resistant agent reduces this risk is uncertain.

 

 

  

Conclusion

This case represents an unusual presentation of scarlet fever manifesting as perianal dermatitis caused by GABHS. Although more common in the pediatric population, adult cases have been documented in the literature. As this case illustrates, early recognition and treatment with penicillin (or amoxicillin) produces rapid improvement and resolution of symptoms. 

  


Dr Nibhanipudi is a professor of clinical emergency medicine at New York Medical College - Metropolitan Hospital Center, New York.

Case

The mother of a 3-year-old boy presented her son to the ED for evaluation after she noticed peeling of the skin in his perianal region. She stated that the peeling had started 1 day prior to presentation. Two days earlier, the mother had brought the same patient to the ED for evaluation of a fever, sore throat, and a slight rash over his face. The boy’s vital signs at the initial presentation were: temperature, 101.8°F; heart rate, 102 beats/minute; and respiratory rate, 28 breaths/minute. Oxygen saturation was 98% on room air.

During this first visit, the mother denied the child having had any fever, chills, headache, sore throat, facial rash, joint pain, or pain on defecation. He had no significant medical history and no known drug allergies. After examination, a throat culture was taken, and the patient was given acetaminophen and discharged home with a diagnosis of viral syndrome.

At the second presentation, physical examination revealed a well-developed child in no distress. The examination was negative except for a 4 x 2 cm area of desquamation present over the perianal region (Figure).

The area of desquamation was dry, mildly erythematous without discharge, and nontender. The patient’s vital signs at this presentation were stable, and he was afebrile. The remaining physical examination findings were normal. The throat culture taken during the first ED presentation was reported as negative. A perianal swab was sent for culture and sensitivity. This was later reported to be positive for group A β-hemolytic streptococci (GABHS), which is sensitive to penicillin. The patient was discharged home in the care of his mother with a prescription of penicillin.  A 10-day follow-up showed complete resolution of the skin rash.

  

Discussion

Perianal streptococcal dermatitis (PSD), which is caused by GABHS, is a frequently overlooked medical entity. Landolt et al1 investigated the prevalence of PSD at the University Children’s Hospital Basel, Switzerland, from October 2000 to May 2001. In this study, 250 randomly selected patients were studied for signs of PSD by history, examination, and culture, the results of which showed that PSD was frequent in the study cohort. The study further underscored that, to appropriately treat affected patients, signs and symptoms of PSD should be searched for systematically.1

The rash in this case was most likely the result of scarlet fever with an unusual presentation of PSD; the signs and symptoms of which include perianal erythema, itching, rectal pain, sometimes blood-streaked stools, rectal bleeding, irritation or pruritus, tissue loss and exudation, secondary constipation, and cellulitis. Perianal streptococcal dermatitis has also been described in the adult literature.2 As with pediatric cases, PSD in adults is usually caused by GABHS.

Evaluation and Diagnosis

A rapid streptococcal test of suspicious areas can confirm the diagnosis. Fever, sore throat, and arthralgia are rare; however, culture from the perianal region grows GABHS. Titers are usually not elevated in laboratory evaluation. A routine skin culture is an alternative diagnostic aid.

Brilliant2 described the bright red color of PSD as a sharply demarcated rash that is caused by GABHS. As previously stated, symptoms include perianal rash, itching, and rectal pain; blood-streaked stools may also be seen in one-third of patients. It primarily occurs in children between 6 months and 10 years of age and is often misdiagnosed and treated inappropriately.3

Prompt diagnosis of GABHS is important. If untreated, it can cause serious systemic infections, especially in elderly and in newborn patients. Treatment with antibiotics resolves the condition in the majority of patients.2 In the acute stage, a white pseudomembrane may be present. As the rash becomes more chronic, the perianal eruption may consist of painful fissures, a dry mucoid discharge, or psoriasiform plaques. Perianal dermatitis can also be caused by Staphylococcus aureus or Candida. Confirmation of the diagnosis is accomplished by culturing a moderate-to-heavy growth of GABHS on 5% sheep-blood agar.

Treatment

A 10-day course of oral penicillin produces resolution of the dermatitis and other symptoms in most patients, but a relapse rate as high as 39% has been reported. Other treatment plans include amoxicillin, 40 mg/kg per day, divided into three doses, and/or topical applications of mupirocin 2% three times per day for 10 days. Penicillin, clindamycin phosphate, and erythromycin have also been used.

Although penicillin is generally recommended for treatment of GABHS infection, amoxicillin is often better tolerated in the pediatric population due to its superior palatability. Early antibiotic treatment causes a dramatic and rapid improvement of symptoms. However, according to Olson et al,4 PSD initially treated with amoxicillin or penicillin is consistently associated with a high risk of clinical recurrence. Whether treatment with a β-lactamase–resistant agent reduces this risk is uncertain.

 

 

  

Conclusion

This case represents an unusual presentation of scarlet fever manifesting as perianal dermatitis caused by GABHS. Although more common in the pediatric population, adult cases have been documented in the literature. As this case illustrates, early recognition and treatment with penicillin (or amoxicillin) produces rapid improvement and resolution of symptoms. 

  


Dr Nibhanipudi is a professor of clinical emergency medicine at New York Medical College - Metropolitan Hospital Center, New York.

References

- Case Report: Perianal Streptococcal Infection

  1. Landolt M, Heininger U. Prevalence of perianal streptococcal dermatitis in children and adolescents [in German]. Praxis (Bern 1994). 2005;94(38):1467-1471.
  2. Kahlke V, Jongen J, Peleikis HG, Herbst RA. Perianal streptococcal dermatitis in adults: its association with pruritic anorectal diseases is mainly caused by group B Streptococci. Colorectal Dis. 2013;15(5):602-607.
  3. Brilliant LC. Perianal streptococcal dermatitis. Am Fam Physician. 2000;61(2):391-393.
  4. Olson D, Edmonson MB. Outcomes in children treated for perineal group A beta-hemolytic streptococcal dermatitis. Pediatr Infect Dis J. 2011;30(11):933-936.
References

- Case Report: Perianal Streptococcal Infection

  1. Landolt M, Heininger U. Prevalence of perianal streptococcal dermatitis in children and adolescents [in German]. Praxis (Bern 1994). 2005;94(38):1467-1471.
  2. Kahlke V, Jongen J, Peleikis HG, Herbst RA. Perianal streptococcal dermatitis in adults: its association with pruritic anorectal diseases is mainly caused by group B Streptococci. Colorectal Dis. 2013;15(5):602-607.
  3. Brilliant LC. Perianal streptococcal dermatitis. Am Fam Physician. 2000;61(2):391-393.
  4. Olson D, Edmonson MB. Outcomes in children treated for perineal group A beta-hemolytic streptococcal dermatitis. Pediatr Infect Dis J. 2011;30(11):933-936.
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Patient-Directed Valgus Stress Radiograph of the Knee: A New and Novel Technique

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Medial-compartment partial knee arthroplasty (unicompartmental replacement) is an accepted surgical intervention for anteromedial osteoarthritis of the knee.1 The radiographic investigations required in the workup of these patients should include weight-bearing standing anteroposterior (AP), lateral, and sunrise (Merchant) views, as well as a valgus stress AP radiograph to assess the functionality of the lateral compartment. The method of properly obtaining the valgus stress film has been well described by the Oxford Group.2 Its recommended radiographic technique requires that a surgeon or a radiologic technologist perform the valgus stress maneuver, manually, while another technologist shoots the film. The 2 consequences of this technique are that it requires 2 individuals to obtain the film, and it subjects the individual who is applying the stress to some level of radiation exposure, which is undesirable. Because of this and the time inconvenience, many surgeons omit the valgus stress radiograph, which can lead to the adverse outcome of missing a lateral compartment that is functionally incompetent, resulting in the potential for early lateral compartment progression of osteoarthritis and the need for revision surgery, usually to a total knee arthroplasty.

In an attempt to mitigate these barriers to obtaining the necessary valgus stress radiograph, Dr. Mauerhan’s team developed a technique that could be done with the assistance of the patient and would require only 1 technologist to perform. Additionally, this project was a quality improvement initiative, because it lowered radiation exposure to all personnel involved in obtaining the correct films.

Materials and Methods

We initiated the project using weight-bearing strategies to impart the valgus stress view of the knee. After trying several different wedges and blocks, and varying patient instructions, we realized a different approach to this problem would be required to find an acceptable solution. We redirected our efforts to effectively performing the stress view with the patient in a supine position on the radiograph table. Ultimately, we decided that a much stiffer wedge and a denser object to squeeze would facilitate obtaining a proper film. Considering all available options, a youth size 4 soccer ball (diameter, 11 in) was introduced along with a slightly larger positioning wedge. The soccer ball was wrapped with 4-in Coban wrap (3M) to create a nonslip surface. This change in patient positioning, along with a standardized 7º to 10º cephalic radiographic tube angulation, helped to correct issues with tibial plateau visualization. Once these changes were enacted, we obtained fairly consistent positive results, and we instituted this patient-directed valgus stress view of the knee, along with a manual valgus stress view for comparison.

The protocol for obtaining the patient-directed valgus stress view of the knee is as follows: The patient lays supine with a dense 45º spine-positioning wedge (Burlington Medical Supplies) placed under both knees and the patient’s heels on the examining table. The radiographic tube is angled cephalad 7º to 10º centered on the inferior pole of the patella, using a 40-in source to image-receptor distance, collimated to part; the image receptor is placed under the affected knee, below the positioning wedge. The affected knee is rotated to the “true” AP position (the patella will be centered between the femoral condyles on the AP exposure), and the ball is placed between the patient’s legs just above the ankle joint. The technologist demonstrates to the patient how to squeeze the ball while maintaining contact of heels with the table. The technologist can exit the room and obtain the exposure, which is taken while the patient is squeezing the ball, as shown in Figures 1A and 1B. Examples of the standing AP, manual stress, and patient-directed valgus radiographs are shown in Figures 2A-2C. The entire technique is demonstrated in the Video.

 

 

Vidyard Video

 

 

Results

During the 9 months of this quality improvement project, 78 examinations were performed. Five studies did not show complete correction of the varus deformity. Of these, 3 showed complete correction on a manual valgus stress radiograph, and 2 did not, contraindicating the use of partial knee replacement. Three patients displayed collapse of the lateral compartment, indicating a nonfunctional lateral compartment, and, therefore, were also a contraindication to partial knee arthroplasty. The remaining 70 patients had identical radiographic results with both the manual and patient-directed valgus stress tests. There was no instance of examination failure or need to repeat as a result of difficulty of the examination for the patient. Repeat films because of positioning errors were very rare, usually early in the learning curve, and no more prevalent than when using the manual stress method. The technique was reproducible and easy to teach and adopt.

 

 

Discussion

In total, 73 patients (93.5%) with the patient-directed stress film showed the desired result, either correction of the medial compartment narrowing in conjunction with an intact lateral compartment or narrowing of the lateral compartment. Of the 5 patients (6.5%) whose patient-directed stress films did not show correction of the varus deformity, 3 patients displayed correction with a manually applied stress radiograph and 2 did not. Based on this observation, our recommendation would be for those patients who do not show adequate correction on the patient-directed stress radiograph to have a manual examination to establish the presence or absence of the desired correction.

Performing a valgus stress radiograph is an integral part of the investigation to determine if the patient is an appropriate candidate for partial knee arthroplasty.3 The historical, manually performed valgus stress radiograph requires 2 individuals, 1 to apply the stress with the patient on the table and 1 to shoot the exposure. For the individual or individuals applying this stress, there is an increased radiation exposure that would be undesirable over a long career. The authors developed a new technique using a commercially available spinal positioning wedge and 11-in youth soccer ball wrapped with Coban wrap, as described, which is economical and easy to obtain and use in the clinical setting. We believe this cost-effective method will offer surgeons who perform partial knee arthroplasty a novel method to obtain the important information gleaned from the valgus stress radiograph and to improve surgical outcomes through the preoperative assessment of the lateral compartment. Additionally, as a quality and safety improvement initiative, we believe this technique will reduce radiographic exposure for those performing these studies, and, because the examination can be carried out by a single technologist, it will significantly improve efficiency in the radiology suite.

Conclusion

We have developed a new method of obtaining the important valgus stress radiograph as part of the workup of patients with medial-compartment osteoarthritis of the knee. The technique can be performed with easily obtainable, commercially available products and is reliable 93.5% of the time. It also adds to the efficiency of the radiology suite and reduces radiographic exposure for technologists.

References

 

1.    White SH, Ludkowski PF, Goodfellow JW. Anteromedial osteoarthritis of the knee. J Bone Joint Surg Br. 1991;73(4):582-586.

2.    Goodfellow JW, O’Conner JJ, Dodd CA, Murray DW. Unicompartmental Arthroplasty with the Oxford Knee. Woodeaton, Oxford, England: Goodfellow Publishers Limited; 2006:38-39.

3.    Gibson PH, Goodfellow JW. Stress radiography in degenerative arthritis of the knee. J Bone Joint Surg Br. 1986;68(4):608-609.

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David R. Mauerhan, MD, Kyle D. Cook, RTR, Tonia D. Botts, RTR, and Sherita T. Williams, RTR

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Medial-compartment partial knee arthroplasty (unicompartmental replacement) is an accepted surgical intervention for anteromedial osteoarthritis of the knee.1 The radiographic investigations required in the workup of these patients should include weight-bearing standing anteroposterior (AP), lateral, and sunrise (Merchant) views, as well as a valgus stress AP radiograph to assess the functionality of the lateral compartment. The method of properly obtaining the valgus stress film has been well described by the Oxford Group.2 Its recommended radiographic technique requires that a surgeon or a radiologic technologist perform the valgus stress maneuver, manually, while another technologist shoots the film. The 2 consequences of this technique are that it requires 2 individuals to obtain the film, and it subjects the individual who is applying the stress to some level of radiation exposure, which is undesirable. Because of this and the time inconvenience, many surgeons omit the valgus stress radiograph, which can lead to the adverse outcome of missing a lateral compartment that is functionally incompetent, resulting in the potential for early lateral compartment progression of osteoarthritis and the need for revision surgery, usually to a total knee arthroplasty.

In an attempt to mitigate these barriers to obtaining the necessary valgus stress radiograph, Dr. Mauerhan’s team developed a technique that could be done with the assistance of the patient and would require only 1 technologist to perform. Additionally, this project was a quality improvement initiative, because it lowered radiation exposure to all personnel involved in obtaining the correct films.

Materials and Methods

We initiated the project using weight-bearing strategies to impart the valgus stress view of the knee. After trying several different wedges and blocks, and varying patient instructions, we realized a different approach to this problem would be required to find an acceptable solution. We redirected our efforts to effectively performing the stress view with the patient in a supine position on the radiograph table. Ultimately, we decided that a much stiffer wedge and a denser object to squeeze would facilitate obtaining a proper film. Considering all available options, a youth size 4 soccer ball (diameter, 11 in) was introduced along with a slightly larger positioning wedge. The soccer ball was wrapped with 4-in Coban wrap (3M) to create a nonslip surface. This change in patient positioning, along with a standardized 7º to 10º cephalic radiographic tube angulation, helped to correct issues with tibial plateau visualization. Once these changes were enacted, we obtained fairly consistent positive results, and we instituted this patient-directed valgus stress view of the knee, along with a manual valgus stress view for comparison.

The protocol for obtaining the patient-directed valgus stress view of the knee is as follows: The patient lays supine with a dense 45º spine-positioning wedge (Burlington Medical Supplies) placed under both knees and the patient’s heels on the examining table. The radiographic tube is angled cephalad 7º to 10º centered on the inferior pole of the patella, using a 40-in source to image-receptor distance, collimated to part; the image receptor is placed under the affected knee, below the positioning wedge. The affected knee is rotated to the “true” AP position (the patella will be centered between the femoral condyles on the AP exposure), and the ball is placed between the patient’s legs just above the ankle joint. The technologist demonstrates to the patient how to squeeze the ball while maintaining contact of heels with the table. The technologist can exit the room and obtain the exposure, which is taken while the patient is squeezing the ball, as shown in Figures 1A and 1B. Examples of the standing AP, manual stress, and patient-directed valgus radiographs are shown in Figures 2A-2C. The entire technique is demonstrated in the Video.

 

 

Vidyard Video

 

 

Results

During the 9 months of this quality improvement project, 78 examinations were performed. Five studies did not show complete correction of the varus deformity. Of these, 3 showed complete correction on a manual valgus stress radiograph, and 2 did not, contraindicating the use of partial knee replacement. Three patients displayed collapse of the lateral compartment, indicating a nonfunctional lateral compartment, and, therefore, were also a contraindication to partial knee arthroplasty. The remaining 70 patients had identical radiographic results with both the manual and patient-directed valgus stress tests. There was no instance of examination failure or need to repeat as a result of difficulty of the examination for the patient. Repeat films because of positioning errors were very rare, usually early in the learning curve, and no more prevalent than when using the manual stress method. The technique was reproducible and easy to teach and adopt.

 

 

Discussion

In total, 73 patients (93.5%) with the patient-directed stress film showed the desired result, either correction of the medial compartment narrowing in conjunction with an intact lateral compartment or narrowing of the lateral compartment. Of the 5 patients (6.5%) whose patient-directed stress films did not show correction of the varus deformity, 3 patients displayed correction with a manually applied stress radiograph and 2 did not. Based on this observation, our recommendation would be for those patients who do not show adequate correction on the patient-directed stress radiograph to have a manual examination to establish the presence or absence of the desired correction.

Performing a valgus stress radiograph is an integral part of the investigation to determine if the patient is an appropriate candidate for partial knee arthroplasty.3 The historical, manually performed valgus stress radiograph requires 2 individuals, 1 to apply the stress with the patient on the table and 1 to shoot the exposure. For the individual or individuals applying this stress, there is an increased radiation exposure that would be undesirable over a long career. The authors developed a new technique using a commercially available spinal positioning wedge and 11-in youth soccer ball wrapped with Coban wrap, as described, which is economical and easy to obtain and use in the clinical setting. We believe this cost-effective method will offer surgeons who perform partial knee arthroplasty a novel method to obtain the important information gleaned from the valgus stress radiograph and to improve surgical outcomes through the preoperative assessment of the lateral compartment. Additionally, as a quality and safety improvement initiative, we believe this technique will reduce radiographic exposure for those performing these studies, and, because the examination can be carried out by a single technologist, it will significantly improve efficiency in the radiology suite.

Conclusion

We have developed a new method of obtaining the important valgus stress radiograph as part of the workup of patients with medial-compartment osteoarthritis of the knee. The technique can be performed with easily obtainable, commercially available products and is reliable 93.5% of the time. It also adds to the efficiency of the radiology suite and reduces radiographic exposure for technologists.

Medial-compartment partial knee arthroplasty (unicompartmental replacement) is an accepted surgical intervention for anteromedial osteoarthritis of the knee.1 The radiographic investigations required in the workup of these patients should include weight-bearing standing anteroposterior (AP), lateral, and sunrise (Merchant) views, as well as a valgus stress AP radiograph to assess the functionality of the lateral compartment. The method of properly obtaining the valgus stress film has been well described by the Oxford Group.2 Its recommended radiographic technique requires that a surgeon or a radiologic technologist perform the valgus stress maneuver, manually, while another technologist shoots the film. The 2 consequences of this technique are that it requires 2 individuals to obtain the film, and it subjects the individual who is applying the stress to some level of radiation exposure, which is undesirable. Because of this and the time inconvenience, many surgeons omit the valgus stress radiograph, which can lead to the adverse outcome of missing a lateral compartment that is functionally incompetent, resulting in the potential for early lateral compartment progression of osteoarthritis and the need for revision surgery, usually to a total knee arthroplasty.

In an attempt to mitigate these barriers to obtaining the necessary valgus stress radiograph, Dr. Mauerhan’s team developed a technique that could be done with the assistance of the patient and would require only 1 technologist to perform. Additionally, this project was a quality improvement initiative, because it lowered radiation exposure to all personnel involved in obtaining the correct films.

Materials and Methods

We initiated the project using weight-bearing strategies to impart the valgus stress view of the knee. After trying several different wedges and blocks, and varying patient instructions, we realized a different approach to this problem would be required to find an acceptable solution. We redirected our efforts to effectively performing the stress view with the patient in a supine position on the radiograph table. Ultimately, we decided that a much stiffer wedge and a denser object to squeeze would facilitate obtaining a proper film. Considering all available options, a youth size 4 soccer ball (diameter, 11 in) was introduced along with a slightly larger positioning wedge. The soccer ball was wrapped with 4-in Coban wrap (3M) to create a nonslip surface. This change in patient positioning, along with a standardized 7º to 10º cephalic radiographic tube angulation, helped to correct issues with tibial plateau visualization. Once these changes were enacted, we obtained fairly consistent positive results, and we instituted this patient-directed valgus stress view of the knee, along with a manual valgus stress view for comparison.

The protocol for obtaining the patient-directed valgus stress view of the knee is as follows: The patient lays supine with a dense 45º spine-positioning wedge (Burlington Medical Supplies) placed under both knees and the patient’s heels on the examining table. The radiographic tube is angled cephalad 7º to 10º centered on the inferior pole of the patella, using a 40-in source to image-receptor distance, collimated to part; the image receptor is placed under the affected knee, below the positioning wedge. The affected knee is rotated to the “true” AP position (the patella will be centered between the femoral condyles on the AP exposure), and the ball is placed between the patient’s legs just above the ankle joint. The technologist demonstrates to the patient how to squeeze the ball while maintaining contact of heels with the table. The technologist can exit the room and obtain the exposure, which is taken while the patient is squeezing the ball, as shown in Figures 1A and 1B. Examples of the standing AP, manual stress, and patient-directed valgus radiographs are shown in Figures 2A-2C. The entire technique is demonstrated in the Video.

 

 

Vidyard Video

 

 

Results

During the 9 months of this quality improvement project, 78 examinations were performed. Five studies did not show complete correction of the varus deformity. Of these, 3 showed complete correction on a manual valgus stress radiograph, and 2 did not, contraindicating the use of partial knee replacement. Three patients displayed collapse of the lateral compartment, indicating a nonfunctional lateral compartment, and, therefore, were also a contraindication to partial knee arthroplasty. The remaining 70 patients had identical radiographic results with both the manual and patient-directed valgus stress tests. There was no instance of examination failure or need to repeat as a result of difficulty of the examination for the patient. Repeat films because of positioning errors were very rare, usually early in the learning curve, and no more prevalent than when using the manual stress method. The technique was reproducible and easy to teach and adopt.

 

 

Discussion

In total, 73 patients (93.5%) with the patient-directed stress film showed the desired result, either correction of the medial compartment narrowing in conjunction with an intact lateral compartment or narrowing of the lateral compartment. Of the 5 patients (6.5%) whose patient-directed stress films did not show correction of the varus deformity, 3 patients displayed correction with a manually applied stress radiograph and 2 did not. Based on this observation, our recommendation would be for those patients who do not show adequate correction on the patient-directed stress radiograph to have a manual examination to establish the presence or absence of the desired correction.

Performing a valgus stress radiograph is an integral part of the investigation to determine if the patient is an appropriate candidate for partial knee arthroplasty.3 The historical, manually performed valgus stress radiograph requires 2 individuals, 1 to apply the stress with the patient on the table and 1 to shoot the exposure. For the individual or individuals applying this stress, there is an increased radiation exposure that would be undesirable over a long career. The authors developed a new technique using a commercially available spinal positioning wedge and 11-in youth soccer ball wrapped with Coban wrap, as described, which is economical and easy to obtain and use in the clinical setting. We believe this cost-effective method will offer surgeons who perform partial knee arthroplasty a novel method to obtain the important information gleaned from the valgus stress radiograph and to improve surgical outcomes through the preoperative assessment of the lateral compartment. Additionally, as a quality and safety improvement initiative, we believe this technique will reduce radiographic exposure for those performing these studies, and, because the examination can be carried out by a single technologist, it will significantly improve efficiency in the radiology suite.

Conclusion

We have developed a new method of obtaining the important valgus stress radiograph as part of the workup of patients with medial-compartment osteoarthritis of the knee. The technique can be performed with easily obtainable, commercially available products and is reliable 93.5% of the time. It also adds to the efficiency of the radiology suite and reduces radiographic exposure for technologists.

References

 

1.    White SH, Ludkowski PF, Goodfellow JW. Anteromedial osteoarthritis of the knee. J Bone Joint Surg Br. 1991;73(4):582-586.

2.    Goodfellow JW, O’Conner JJ, Dodd CA, Murray DW. Unicompartmental Arthroplasty with the Oxford Knee. Woodeaton, Oxford, England: Goodfellow Publishers Limited; 2006:38-39.

3.    Gibson PH, Goodfellow JW. Stress radiography in degenerative arthritis of the knee. J Bone Joint Surg Br. 1986;68(4):608-609.

References

 

1.    White SH, Ludkowski PF, Goodfellow JW. Anteromedial osteoarthritis of the knee. J Bone Joint Surg Br. 1991;73(4):582-586.

2.    Goodfellow JW, O’Conner JJ, Dodd CA, Murray DW. Unicompartmental Arthroplasty with the Oxford Knee. Woodeaton, Oxford, England: Goodfellow Publishers Limited; 2006:38-39.

3.    Gibson PH, Goodfellow JW. Stress radiography in degenerative arthritis of the knee. J Bone Joint Surg Br. 1986;68(4):608-609.

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Giant Bone Island of the Tibia in a Child

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Giant Bone Island of the Tibia in a Child

 A bone island is a focus of normal cortical bone located within the medullary cavity. The vast majority of bone islands are small, measuring from 1 mm to 2 cm in size. They are found more frequently in adults than in children. The lesion can be virtually diagnosed on the basis of its characteristic clinical and imaging features. Differential diagnosis may be difficult when the lesion manifests itself uncharacteristically by being symptomatic, very large, and hot on bone scan.1-4

The term giant bone island has been used to describe a large lesion1 that measures more than 2 cm in any dimension.5 Giant bone islands have been described only in adults,1,5-15 and the longest bone island length reported is 10.5 cm.10 They are usually symptomatic and associated with increased radionuclide uptake on bone scintigraphy.14

The history and the clinical and imaging presentation of an even longer, symptomatic, and scintigraphically hot lesion in the tibial diaphysis of a 10-year-old boy is reported. The lesion further exhibited several atypical imaging features necessitating an open biopsy, which confirmed the diagnosis of a giant bone island. The pertinent differential diagnosis and the clinical and radiographic findings after 15-year follow-up are also presented and discussed. The patient provided written informed consent for print and electronic publication of this case report.

Case Report

A 10-year-old boy was admitted for surgical repair of an inguinal hernia. Physical examination revealed a painless but tender anterior bowing of the right tibial diaphysis. The patient was a healthy-appearing white male with normal vital signs, gait, and posture. His parents noticed a slight protuberance of the tibia at age 2.5 years. No medical advice was asked for the bone swelling after that time. After recovery from the inguinal hernia repair 3 weeks later, the bone lesion was thoroughly examined. Radiographs showed an oblong, homogenous region of dense sclerosis in the diaphysis of the right tibia. The lesion had relatively well-defined margins and was located in the medullary cavity. Speculations were not obvious in the periphery of the lesion, which exhibited a sharp circumscription (Figures 1A, 1B). A well-defined lytic area was evident at the distal part of the lesion (Figure 1B). There was no periosteal reaction. Blood and serum chemistries were within normal limits, including serum calcium, phosphorus, and alkaline phosphatase. A conventional 3-phase bone scintigraphy (300 MBq) with technetium-99m HDP (hydroxydiphosphonate) indicated increased uptake in the area of the lesion but no other skeletal abnormality (Figure 2). Computed tomography (CT) showed that the lesion was purely intramedullary and densely blastic. The lesion originated from the medial cortex, which was thickened (Figure 3A). The lesion extended to the anterolateral cortex, which was thinned and included a lytic area. In the distal part of the lesion, the anterolateral cortex was thickened, included lytic areas, and exhibited an anterior portion of cortical destruction (Figure 3B). The fatty marrow adjacent to the region of sclerosis appeared normal. There was no evidence of extraosseous soft-tissue changes. On both T1- and T2-weighted magnetic resonance imaging (MRI), the lesion exhibited low-signal intensity. The lesion measured 10.8×2.2×1 cm. It originated from the medial cortical bone of the tibia, blended into the medullary cavity, and extended anteriorly towards and through the anterior cortex. The area of cortical destruction was clearly evident on the axial MRI. The periosteum was displaced and eroded anteriorly by focal radiating bony streaks. No enhancement was seen after the intravenous administration of gadolinium-diethylenetriamine pentaacetic acid (Gd-DTPA) as a contrast medium. There were no extraosseous soft-tissue changes. In the distal part of the lesion, sagittal and axial MRI showed a 1.2×0.8×0.7-cm well-defined ovoid focus, with characteristics of cystic degeneration that exhibited intermediate-signal intensity on T1-weighted MRI (Figure 4) and high-signal intensity on T2-weighted MRI.

 
 

An open biopsy was performed. Macroscopically, a wedge of compact bone measuring 3×1.7×0.6 cm was taken. Microscopic examination showed a thinned periphery of lamellar (mature) bone with haversian canals and, beneath it, woven (immature) bone with long-surface processes projecting within adjacent cancellous bone (Figure 5A). The woven bone contained loose vascular fibrous tissue. No osteoclasts were noted, and the very few osteoblasts lining the bone trabeculae were small, single-layered, and flat (Figure 5B). There was no evidence of neoplastic cells. There was no abnormality of the periosteum and the surrounding soft tissues.

The histology was pathognomonic of a giant bone island. No additional surgical intervention was recommended.

The postoperative course was uncomplicated, and the patient was discharged 2 weeks later. An above-the-knee plaster was recommended for 3 months and a below-the-knee splint for an additional 2-month period. Full weight-bearing was allowed only after the postsurgical sixth month to prevent an impending fracture. The tibial bowing was tender to pressure or palpation, and the patient reported mild spontaneous pain during follow-up. Radiographs 1 year after surgery indicated that the bone area removed for biopsy was replaced by compact bone. MRI performed 4 years after surgery showed that the volume of the lesion in relation to the host bone was not changed.

 

 

At the last follow-up 15 years after surgery, the anterior tibial bowing was not changed (Figure 6A), but the patient additionally complained of skin irritation after intense training wearing boots during military service. The radiographic appearance of the lesion was also not changed, while the periphery of the lesion exhibited scarce radiating bony streaks with rounded contours (Figures 6B, 6C). The clinical symptoms and signs from wearing military boots completely subsided after a couple of weeks’ rest from daily army activities, but the mild spontaneous pain and the local tenderness over the tibial bowing persisted.

Discussion

Giant bone islands are more likely to be associated with clinical symptoms than the usual small-sized bone island. Some degree of pain was detected in 8 of 10 patients with a giant bone island presented in the literature, but it was induced by trauma in 3 of them.14

Radiographic appearance is among the distinguishing diagnostic features of a giant bone island. It appears as an ovoid, round, or oblong, homogenously dense, single or multiple focus of sclerosis within the medullary cavity; it is oriented along the long axis of the host bone, and it exhibits peripheral pseudopodia or radiating spicules producing the typical “thorny” or “paintbrush” appearance.8,16,17 It does not exhibit cortical penetration and it is not associated with periosteal reaction.10

The CT findings include a sclerotic and hyperdense focus with spiculated margins extending into the adjacent cancellous bone. The lack of bone destruction and soft-tissue mass are also diagnostic.3,7 MRI findings will reflect the low-signal intensity characteristics of cortical bone on all pulse sequences.18

Enostoses usually exhibit no activity on skeletal scintigraphy, while giant lesions generally show increased radiotracer uptake.5,9-11,14,19-27 The latter may result from the increased amount of bone turnover, which is seen more often with larger lesions because of active bone deposition and remodeling.20,21,23,28 Histopathology of a giant bone island appears identical to the well-described pathologic appearance of smaller bone islands. The lesion is composed of compact lamellar bone and haversian systems, which blend with the adjacent spongiosa. The surrounding cancellous bone forms thorn-like trabeculae radiating from the lesion and merging with the cancellous bone.1,4,5,8,28

The presumptive diagnosis of a bone island is based on the clinical findings, imaging features, and follow-up examinations. An asymptomatic, isolated, sclerotic bone lesion showing the typical features of a bone island on plain radiography, CT, and MRI, whatever its size, that is nonactive on bone scan may be easily diagnosed. However, a symptomatic patient with a hot lesion on scintigraphy should be carefully observed. In addition, larger lesions may raise the suspicion of a neoplasm, such as a sclerotic variant of osteosarcoma. In such cases, an open biopsy may be undertaken. No specific treatment is required after the diagnosis has been confirmed. There is no literature to suggest that, after adequate biopsy confirmation, excision or resection is necessary. Follow-up radiographic examination of the lesion should be suggested to monitor for any potential growth.2,10,23

The first giant bone island appearing in a child is presented in this report. The lack of a causative factor leading to the anterior tibial bowing indicated that the bone deformity was caused primarily by the lesion. The present case is unusual for the appearance of several atypical features, some of which have not been previously described. Peripheral radiating spiculated margin was absent on the patient’s initial radiographs and CT imaging. MRI indicated only the presence of radiating bony streaks that displaced and eroded the periosteum on the anterior border of the lesion. The CT findings that the lesion likely originated or was in close proximity with the medial cortex of the tibia were also atypical. It has been previously reported that spinal lesions located immediately below the cortex tend to fuse with the endosteal surface, while similar features may also be seen in the appendicular enostoses.4,29 Other CT findings, such as the thinning of the overlying anterolateral cortical bone, as well as the cortical thickening at the periphery of the lesion associated with areas of soft-tissue attenuation and anterior cortical destruction, have not been described even in the atypical features of a giant bone island. The lytic area resembling a nidus that was evident at the distal part of the lesion was more likely consistent with an area of resorption, which, although rare, has been described on giant lesions.2,9,29 The substantial amount of woven bone transforming to lamellar bone that was evident in the present patient’s microscopic features is also an atypical finding, although it may be expected to some degree in scintigraphically hot, large lesions.28 The clinical and imaging progress of the lesion supported the diagnosis of a giant bone island. The degree of the anterior tibial bowing and the volume of the lesion in relation to the host bone were not changed throughout the follow-up period, indicating that the growth of the lesion followed the growth of the normal bone.

 

 

The differential diagnosis of a giant bone island includes a variety of benign tumors and tumor-like lesions, as well as malignant bone lesions.2,4,23,28,30,31 In the patient presented in this report, the diagnosis of an atypical sclerotic presentation of a nonossifying fibroma or healing stage of this lesion could be consistent with some of the CT findings, including the eccentric origin from the cortex associated with medial cortical thickening, the anterolateral cortical thinning, and the soft-tissue attenuation of cortical areas. In addition, unifocal osteofibrous dysplasia may also present with a long intracortical diaphyseal lucency within an area of marked cortical sclerosis and cause a bowing deformity. Both diagnoses were excluded, since no fibrous stroma was evident on the histologic examination of the lesion. A large or giant long-bone osteoma would be associated with the outer cortical margin of bone but would not involve the intramedullary space. The scintigraphically increased uptake of radioisotope, as well as the CT and MRI findings, were not consistent with the diagnosis of osteoid osteoma, osteoblastoma, or osteomyelitis. Although most imaging findings were consistent with a benign lesion, and contrast-enhanced MRI showed no increased vascularity, anterior cortical disruption necessitated a bone biopsy to rule out any potential malignancy.

The histopathology in association with the clinical and imaging findings indicated the diagnosis of a giant bone island. The increased proportion of maturing woven bone over lamellar bone indicated an active remodeling lesion that could be related to the patient’s age, since the clinical and radiographic features of the lesion were not changed after 15-year follow-up.

Conclusion

This is the first giant bone island diagnosed in a patient before puberty. Its greatest length was 10.8 cm, which is the longest reported in the literature. The imaging appearance included several atypical features that are very rare or have not been reported. Microscopic features indicated less mature lamellar bone and a prominent proportion of maturing woven bone. The clinical and the radiographic appearance of the lesion were not changed after 15-year follow-up.

References

1.    Smith J. Giant bone islands. Radiology. 1973;7(1):35-36.

2.    Mirra JM. Bone Tumors: Clinical, Radiologic and Pathologic Correlations. Philadelphia, PA: Lea & Febiger; 1989.

3.    Greenspan A. Bone island (enostosis): current concept - a review. Skeletal Radiol. 1995;24(2):111-115.

4.    Kransdorf MJ, Peterson JJ, Bancroft LW. MR imaging of the knee: incidental osseous lesions. Radiol Clin North Am. 2007;45(6):943-954.

5.    Gold RH, Mirra JM, Remotti F, Pignatti G. Case report 527: Giant bone island of tibia. Skeletal Radiol. 1989;18(2):129-132.

6.    Onitsuka H. Roentgenologic aspects of bone islands. Radiology. 1977;123(3):607-612.

7.    Ehara S, Kattapuram SV, Rosenberg AE. Giant bone island. Computed tomography findings. Clin Imaging. 1989;13(3):231-233.

8.    Greenspan A, Steiner G, Knutzon R. Bone island (enostosis): clinical significance and radiologic and pathologic correlations. Skeletal Radiol. 1991;20(2):85-90.

9.    Avery GR, Wilsdon JB, Malcolm AJ. Giant bone island with some central resorption. Skeletal Radiol. 1995;24(1):59-60.

10.  Brien EW, Mirra JM, Latanza L, Fedenko A, Luck J Jr. Giant bone island of femur. Case report, literature review, and its distinction from low grade osteosarcoma. Skeletal Radiol. 1995;24(7):546-550.

11.    Greenspan A, Klein MJ. Giant bone island. Skeletal Radiol. 1996;25(1):67-69.

12.  Trombetti A, Noël E. Giant bone islands: a case with 31 years of follow-up. Joint Bone Spine. 2002;69(1):81-84.

13.  Dhaon BK, Gautam VK, Jain P, Jaiswal A, Nigam V. Giant bone island of femur complicating replacement arthroplasty: a report of two cases. J Surg Orthop Adv. 2004;13(4):220-223.

14.  Park HS, Kim JR, Lee SY, Jang KY. Symptomatic giant (10-cm) bone island of the tibia. Skeletal Radiol. 2005;34(6):347-350.

15.  Ikeuchi M, Komatsu M, Tani T. Giant bone island of femur with femoral head necrosis: a case report. Arch Orthop Trauma Surg. 2010;130(4):447-450.

16.  Kim SK, Barry WF Jr. Bone island. Am J Roentgenol Radium Ther Nucl Med. 1964;92:1301-1306. 

17.  Kim SK, Barry WF Jr. Bone islands. Radiology. 1968;90(1):77-78. 

18.  Cerase A, Priolo F. Skeletal benign bone-forming lesions. Eur J Radiol. 1998;27:S91–S97.

19.  Go RT, El-Khoury GY, Wehbe MA. Radionuclide bone image in growing and stable bone island. Skeletal Radiol. 1980;5(1):15-18.

20.  Hall FM, Goldberg RP, Davies JA, Fainsinger MH. Scintigraphic assessment of bone islands. Radiology. 1980;135(3):737-742.

21.  Greenspan A, Stadalnik RC. Bone island: scintigraphic findings and their clinical application. Can Assoc Radiol J. 1995;46(5):368-379.

22.  Sickles EA, Genant HK, Hoffer PB. Increased localization of 99mTc-pyrophosphate in a bone island: case report. J Nucl Med. 1976;17(2):113-115.

23.  Dorfman HD, Czerniak B. Bone Tumors. St Louis: Mosby; 1998.

24.  Ngan H. Growing bone islands. Clin Radiol. 1972;23(2):199-201.

25.  Davies JA, Hall FM, Goldberg RP, Kasdon EJ. Positive bone scan in a bone island. Case report. J Bone Joint Surg Am. 1979;61(6):943-945.

26.  Simon K, Mulligan ME. Growing bone islands revisited. A case report. J Bone Joint Surg Am. 1985;67(5):809-811.

27.  Blank N, Lieber A. The significance of growing bone islands. Radiology. 1965;85(3):508-511.

28.  Greenspan A, Gernot J, Wolfgang R. Differential Diagnosis of Orthopaedic Oncology. Philadelphia, PA: Lippincott Williams & Wilkins; 2007.

29.  Kransdorf MJ, Murphey MD. Osseous tumors. In: Davies AM, Sundaram M, James SLJ, eds. Imaging of Bone Tumors and Tumor-Like Lesions. Berlin, Germany: Springer-Verlag; 2009.

30.  Mödder B, Guhl B, Schaefer HE. Growing bone islands as differential diagnosis of osteoplastic metastases. Rontgenblatter. 1980;33(6):286-288.

31.  Flechner RE, Mills SE. Atlas of Tumor Pathology: Tumors of the Bones and Joints. Washington, DC: Armed Forces Institute of Pathology; 1993.

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 A bone island is a focus of normal cortical bone located within the medullary cavity. The vast majority of bone islands are small, measuring from 1 mm to 2 cm in size. They are found more frequently in adults than in children. The lesion can be virtually diagnosed on the basis of its characteristic clinical and imaging features. Differential diagnosis may be difficult when the lesion manifests itself uncharacteristically by being symptomatic, very large, and hot on bone scan.1-4

The term giant bone island has been used to describe a large lesion1 that measures more than 2 cm in any dimension.5 Giant bone islands have been described only in adults,1,5-15 and the longest bone island length reported is 10.5 cm.10 They are usually symptomatic and associated with increased radionuclide uptake on bone scintigraphy.14

The history and the clinical and imaging presentation of an even longer, symptomatic, and scintigraphically hot lesion in the tibial diaphysis of a 10-year-old boy is reported. The lesion further exhibited several atypical imaging features necessitating an open biopsy, which confirmed the diagnosis of a giant bone island. The pertinent differential diagnosis and the clinical and radiographic findings after 15-year follow-up are also presented and discussed. The patient provided written informed consent for print and electronic publication of this case report.

Case Report

A 10-year-old boy was admitted for surgical repair of an inguinal hernia. Physical examination revealed a painless but tender anterior bowing of the right tibial diaphysis. The patient was a healthy-appearing white male with normal vital signs, gait, and posture. His parents noticed a slight protuberance of the tibia at age 2.5 years. No medical advice was asked for the bone swelling after that time. After recovery from the inguinal hernia repair 3 weeks later, the bone lesion was thoroughly examined. Radiographs showed an oblong, homogenous region of dense sclerosis in the diaphysis of the right tibia. The lesion had relatively well-defined margins and was located in the medullary cavity. Speculations were not obvious in the periphery of the lesion, which exhibited a sharp circumscription (Figures 1A, 1B). A well-defined lytic area was evident at the distal part of the lesion (Figure 1B). There was no periosteal reaction. Blood and serum chemistries were within normal limits, including serum calcium, phosphorus, and alkaline phosphatase. A conventional 3-phase bone scintigraphy (300 MBq) with technetium-99m HDP (hydroxydiphosphonate) indicated increased uptake in the area of the lesion but no other skeletal abnormality (Figure 2). Computed tomography (CT) showed that the lesion was purely intramedullary and densely blastic. The lesion originated from the medial cortex, which was thickened (Figure 3A). The lesion extended to the anterolateral cortex, which was thinned and included a lytic area. In the distal part of the lesion, the anterolateral cortex was thickened, included lytic areas, and exhibited an anterior portion of cortical destruction (Figure 3B). The fatty marrow adjacent to the region of sclerosis appeared normal. There was no evidence of extraosseous soft-tissue changes. On both T1- and T2-weighted magnetic resonance imaging (MRI), the lesion exhibited low-signal intensity. The lesion measured 10.8×2.2×1 cm. It originated from the medial cortical bone of the tibia, blended into the medullary cavity, and extended anteriorly towards and through the anterior cortex. The area of cortical destruction was clearly evident on the axial MRI. The periosteum was displaced and eroded anteriorly by focal radiating bony streaks. No enhancement was seen after the intravenous administration of gadolinium-diethylenetriamine pentaacetic acid (Gd-DTPA) as a contrast medium. There were no extraosseous soft-tissue changes. In the distal part of the lesion, sagittal and axial MRI showed a 1.2×0.8×0.7-cm well-defined ovoid focus, with characteristics of cystic degeneration that exhibited intermediate-signal intensity on T1-weighted MRI (Figure 4) and high-signal intensity on T2-weighted MRI.

 
 

An open biopsy was performed. Macroscopically, a wedge of compact bone measuring 3×1.7×0.6 cm was taken. Microscopic examination showed a thinned periphery of lamellar (mature) bone with haversian canals and, beneath it, woven (immature) bone with long-surface processes projecting within adjacent cancellous bone (Figure 5A). The woven bone contained loose vascular fibrous tissue. No osteoclasts were noted, and the very few osteoblasts lining the bone trabeculae were small, single-layered, and flat (Figure 5B). There was no evidence of neoplastic cells. There was no abnormality of the periosteum and the surrounding soft tissues.

The histology was pathognomonic of a giant bone island. No additional surgical intervention was recommended.

The postoperative course was uncomplicated, and the patient was discharged 2 weeks later. An above-the-knee plaster was recommended for 3 months and a below-the-knee splint for an additional 2-month period. Full weight-bearing was allowed only after the postsurgical sixth month to prevent an impending fracture. The tibial bowing was tender to pressure or palpation, and the patient reported mild spontaneous pain during follow-up. Radiographs 1 year after surgery indicated that the bone area removed for biopsy was replaced by compact bone. MRI performed 4 years after surgery showed that the volume of the lesion in relation to the host bone was not changed.

 

 

At the last follow-up 15 years after surgery, the anterior tibial bowing was not changed (Figure 6A), but the patient additionally complained of skin irritation after intense training wearing boots during military service. The radiographic appearance of the lesion was also not changed, while the periphery of the lesion exhibited scarce radiating bony streaks with rounded contours (Figures 6B, 6C). The clinical symptoms and signs from wearing military boots completely subsided after a couple of weeks’ rest from daily army activities, but the mild spontaneous pain and the local tenderness over the tibial bowing persisted.

Discussion

Giant bone islands are more likely to be associated with clinical symptoms than the usual small-sized bone island. Some degree of pain was detected in 8 of 10 patients with a giant bone island presented in the literature, but it was induced by trauma in 3 of them.14

Radiographic appearance is among the distinguishing diagnostic features of a giant bone island. It appears as an ovoid, round, or oblong, homogenously dense, single or multiple focus of sclerosis within the medullary cavity; it is oriented along the long axis of the host bone, and it exhibits peripheral pseudopodia or radiating spicules producing the typical “thorny” or “paintbrush” appearance.8,16,17 It does not exhibit cortical penetration and it is not associated with periosteal reaction.10

The CT findings include a sclerotic and hyperdense focus with spiculated margins extending into the adjacent cancellous bone. The lack of bone destruction and soft-tissue mass are also diagnostic.3,7 MRI findings will reflect the low-signal intensity characteristics of cortical bone on all pulse sequences.18

Enostoses usually exhibit no activity on skeletal scintigraphy, while giant lesions generally show increased radiotracer uptake.5,9-11,14,19-27 The latter may result from the increased amount of bone turnover, which is seen more often with larger lesions because of active bone deposition and remodeling.20,21,23,28 Histopathology of a giant bone island appears identical to the well-described pathologic appearance of smaller bone islands. The lesion is composed of compact lamellar bone and haversian systems, which blend with the adjacent spongiosa. The surrounding cancellous bone forms thorn-like trabeculae radiating from the lesion and merging with the cancellous bone.1,4,5,8,28

The presumptive diagnosis of a bone island is based on the clinical findings, imaging features, and follow-up examinations. An asymptomatic, isolated, sclerotic bone lesion showing the typical features of a bone island on plain radiography, CT, and MRI, whatever its size, that is nonactive on bone scan may be easily diagnosed. However, a symptomatic patient with a hot lesion on scintigraphy should be carefully observed. In addition, larger lesions may raise the suspicion of a neoplasm, such as a sclerotic variant of osteosarcoma. In such cases, an open biopsy may be undertaken. No specific treatment is required after the diagnosis has been confirmed. There is no literature to suggest that, after adequate biopsy confirmation, excision or resection is necessary. Follow-up radiographic examination of the lesion should be suggested to monitor for any potential growth.2,10,23

The first giant bone island appearing in a child is presented in this report. The lack of a causative factor leading to the anterior tibial bowing indicated that the bone deformity was caused primarily by the lesion. The present case is unusual for the appearance of several atypical features, some of which have not been previously described. Peripheral radiating spiculated margin was absent on the patient’s initial radiographs and CT imaging. MRI indicated only the presence of radiating bony streaks that displaced and eroded the periosteum on the anterior border of the lesion. The CT findings that the lesion likely originated or was in close proximity with the medial cortex of the tibia were also atypical. It has been previously reported that spinal lesions located immediately below the cortex tend to fuse with the endosteal surface, while similar features may also be seen in the appendicular enostoses.4,29 Other CT findings, such as the thinning of the overlying anterolateral cortical bone, as well as the cortical thickening at the periphery of the lesion associated with areas of soft-tissue attenuation and anterior cortical destruction, have not been described even in the atypical features of a giant bone island. The lytic area resembling a nidus that was evident at the distal part of the lesion was more likely consistent with an area of resorption, which, although rare, has been described on giant lesions.2,9,29 The substantial amount of woven bone transforming to lamellar bone that was evident in the present patient’s microscopic features is also an atypical finding, although it may be expected to some degree in scintigraphically hot, large lesions.28 The clinical and imaging progress of the lesion supported the diagnosis of a giant bone island. The degree of the anterior tibial bowing and the volume of the lesion in relation to the host bone were not changed throughout the follow-up period, indicating that the growth of the lesion followed the growth of the normal bone.

 

 

The differential diagnosis of a giant bone island includes a variety of benign tumors and tumor-like lesions, as well as malignant bone lesions.2,4,23,28,30,31 In the patient presented in this report, the diagnosis of an atypical sclerotic presentation of a nonossifying fibroma or healing stage of this lesion could be consistent with some of the CT findings, including the eccentric origin from the cortex associated with medial cortical thickening, the anterolateral cortical thinning, and the soft-tissue attenuation of cortical areas. In addition, unifocal osteofibrous dysplasia may also present with a long intracortical diaphyseal lucency within an area of marked cortical sclerosis and cause a bowing deformity. Both diagnoses were excluded, since no fibrous stroma was evident on the histologic examination of the lesion. A large or giant long-bone osteoma would be associated with the outer cortical margin of bone but would not involve the intramedullary space. The scintigraphically increased uptake of radioisotope, as well as the CT and MRI findings, were not consistent with the diagnosis of osteoid osteoma, osteoblastoma, or osteomyelitis. Although most imaging findings were consistent with a benign lesion, and contrast-enhanced MRI showed no increased vascularity, anterior cortical disruption necessitated a bone biopsy to rule out any potential malignancy.

The histopathology in association with the clinical and imaging findings indicated the diagnosis of a giant bone island. The increased proportion of maturing woven bone over lamellar bone indicated an active remodeling lesion that could be related to the patient’s age, since the clinical and radiographic features of the lesion were not changed after 15-year follow-up.

Conclusion

This is the first giant bone island diagnosed in a patient before puberty. Its greatest length was 10.8 cm, which is the longest reported in the literature. The imaging appearance included several atypical features that are very rare or have not been reported. Microscopic features indicated less mature lamellar bone and a prominent proportion of maturing woven bone. The clinical and the radiographic appearance of the lesion were not changed after 15-year follow-up.

 A bone island is a focus of normal cortical bone located within the medullary cavity. The vast majority of bone islands are small, measuring from 1 mm to 2 cm in size. They are found more frequently in adults than in children. The lesion can be virtually diagnosed on the basis of its characteristic clinical and imaging features. Differential diagnosis may be difficult when the lesion manifests itself uncharacteristically by being symptomatic, very large, and hot on bone scan.1-4

The term giant bone island has been used to describe a large lesion1 that measures more than 2 cm in any dimension.5 Giant bone islands have been described only in adults,1,5-15 and the longest bone island length reported is 10.5 cm.10 They are usually symptomatic and associated with increased radionuclide uptake on bone scintigraphy.14

The history and the clinical and imaging presentation of an even longer, symptomatic, and scintigraphically hot lesion in the tibial diaphysis of a 10-year-old boy is reported. The lesion further exhibited several atypical imaging features necessitating an open biopsy, which confirmed the diagnosis of a giant bone island. The pertinent differential diagnosis and the clinical and radiographic findings after 15-year follow-up are also presented and discussed. The patient provided written informed consent for print and electronic publication of this case report.

Case Report

A 10-year-old boy was admitted for surgical repair of an inguinal hernia. Physical examination revealed a painless but tender anterior bowing of the right tibial diaphysis. The patient was a healthy-appearing white male with normal vital signs, gait, and posture. His parents noticed a slight protuberance of the tibia at age 2.5 years. No medical advice was asked for the bone swelling after that time. After recovery from the inguinal hernia repair 3 weeks later, the bone lesion was thoroughly examined. Radiographs showed an oblong, homogenous region of dense sclerosis in the diaphysis of the right tibia. The lesion had relatively well-defined margins and was located in the medullary cavity. Speculations were not obvious in the periphery of the lesion, which exhibited a sharp circumscription (Figures 1A, 1B). A well-defined lytic area was evident at the distal part of the lesion (Figure 1B). There was no periosteal reaction. Blood and serum chemistries were within normal limits, including serum calcium, phosphorus, and alkaline phosphatase. A conventional 3-phase bone scintigraphy (300 MBq) with technetium-99m HDP (hydroxydiphosphonate) indicated increased uptake in the area of the lesion but no other skeletal abnormality (Figure 2). Computed tomography (CT) showed that the lesion was purely intramedullary and densely blastic. The lesion originated from the medial cortex, which was thickened (Figure 3A). The lesion extended to the anterolateral cortex, which was thinned and included a lytic area. In the distal part of the lesion, the anterolateral cortex was thickened, included lytic areas, and exhibited an anterior portion of cortical destruction (Figure 3B). The fatty marrow adjacent to the region of sclerosis appeared normal. There was no evidence of extraosseous soft-tissue changes. On both T1- and T2-weighted magnetic resonance imaging (MRI), the lesion exhibited low-signal intensity. The lesion measured 10.8×2.2×1 cm. It originated from the medial cortical bone of the tibia, blended into the medullary cavity, and extended anteriorly towards and through the anterior cortex. The area of cortical destruction was clearly evident on the axial MRI. The periosteum was displaced and eroded anteriorly by focal radiating bony streaks. No enhancement was seen after the intravenous administration of gadolinium-diethylenetriamine pentaacetic acid (Gd-DTPA) as a contrast medium. There were no extraosseous soft-tissue changes. In the distal part of the lesion, sagittal and axial MRI showed a 1.2×0.8×0.7-cm well-defined ovoid focus, with characteristics of cystic degeneration that exhibited intermediate-signal intensity on T1-weighted MRI (Figure 4) and high-signal intensity on T2-weighted MRI.

 
 

An open biopsy was performed. Macroscopically, a wedge of compact bone measuring 3×1.7×0.6 cm was taken. Microscopic examination showed a thinned periphery of lamellar (mature) bone with haversian canals and, beneath it, woven (immature) bone with long-surface processes projecting within adjacent cancellous bone (Figure 5A). The woven bone contained loose vascular fibrous tissue. No osteoclasts were noted, and the very few osteoblasts lining the bone trabeculae were small, single-layered, and flat (Figure 5B). There was no evidence of neoplastic cells. There was no abnormality of the periosteum and the surrounding soft tissues.

The histology was pathognomonic of a giant bone island. No additional surgical intervention was recommended.

The postoperative course was uncomplicated, and the patient was discharged 2 weeks later. An above-the-knee plaster was recommended for 3 months and a below-the-knee splint for an additional 2-month period. Full weight-bearing was allowed only after the postsurgical sixth month to prevent an impending fracture. The tibial bowing was tender to pressure or palpation, and the patient reported mild spontaneous pain during follow-up. Radiographs 1 year after surgery indicated that the bone area removed for biopsy was replaced by compact bone. MRI performed 4 years after surgery showed that the volume of the lesion in relation to the host bone was not changed.

 

 

At the last follow-up 15 years after surgery, the anterior tibial bowing was not changed (Figure 6A), but the patient additionally complained of skin irritation after intense training wearing boots during military service. The radiographic appearance of the lesion was also not changed, while the periphery of the lesion exhibited scarce radiating bony streaks with rounded contours (Figures 6B, 6C). The clinical symptoms and signs from wearing military boots completely subsided after a couple of weeks’ rest from daily army activities, but the mild spontaneous pain and the local tenderness over the tibial bowing persisted.

Discussion

Giant bone islands are more likely to be associated with clinical symptoms than the usual small-sized bone island. Some degree of pain was detected in 8 of 10 patients with a giant bone island presented in the literature, but it was induced by trauma in 3 of them.14

Radiographic appearance is among the distinguishing diagnostic features of a giant bone island. It appears as an ovoid, round, or oblong, homogenously dense, single or multiple focus of sclerosis within the medullary cavity; it is oriented along the long axis of the host bone, and it exhibits peripheral pseudopodia or radiating spicules producing the typical “thorny” or “paintbrush” appearance.8,16,17 It does not exhibit cortical penetration and it is not associated with periosteal reaction.10

The CT findings include a sclerotic and hyperdense focus with spiculated margins extending into the adjacent cancellous bone. The lack of bone destruction and soft-tissue mass are also diagnostic.3,7 MRI findings will reflect the low-signal intensity characteristics of cortical bone on all pulse sequences.18

Enostoses usually exhibit no activity on skeletal scintigraphy, while giant lesions generally show increased radiotracer uptake.5,9-11,14,19-27 The latter may result from the increased amount of bone turnover, which is seen more often with larger lesions because of active bone deposition and remodeling.20,21,23,28 Histopathology of a giant bone island appears identical to the well-described pathologic appearance of smaller bone islands. The lesion is composed of compact lamellar bone and haversian systems, which blend with the adjacent spongiosa. The surrounding cancellous bone forms thorn-like trabeculae radiating from the lesion and merging with the cancellous bone.1,4,5,8,28

The presumptive diagnosis of a bone island is based on the clinical findings, imaging features, and follow-up examinations. An asymptomatic, isolated, sclerotic bone lesion showing the typical features of a bone island on plain radiography, CT, and MRI, whatever its size, that is nonactive on bone scan may be easily diagnosed. However, a symptomatic patient with a hot lesion on scintigraphy should be carefully observed. In addition, larger lesions may raise the suspicion of a neoplasm, such as a sclerotic variant of osteosarcoma. In such cases, an open biopsy may be undertaken. No specific treatment is required after the diagnosis has been confirmed. There is no literature to suggest that, after adequate biopsy confirmation, excision or resection is necessary. Follow-up radiographic examination of the lesion should be suggested to monitor for any potential growth.2,10,23

The first giant bone island appearing in a child is presented in this report. The lack of a causative factor leading to the anterior tibial bowing indicated that the bone deformity was caused primarily by the lesion. The present case is unusual for the appearance of several atypical features, some of which have not been previously described. Peripheral radiating spiculated margin was absent on the patient’s initial radiographs and CT imaging. MRI indicated only the presence of radiating bony streaks that displaced and eroded the periosteum on the anterior border of the lesion. The CT findings that the lesion likely originated or was in close proximity with the medial cortex of the tibia were also atypical. It has been previously reported that spinal lesions located immediately below the cortex tend to fuse with the endosteal surface, while similar features may also be seen in the appendicular enostoses.4,29 Other CT findings, such as the thinning of the overlying anterolateral cortical bone, as well as the cortical thickening at the periphery of the lesion associated with areas of soft-tissue attenuation and anterior cortical destruction, have not been described even in the atypical features of a giant bone island. The lytic area resembling a nidus that was evident at the distal part of the lesion was more likely consistent with an area of resorption, which, although rare, has been described on giant lesions.2,9,29 The substantial amount of woven bone transforming to lamellar bone that was evident in the present patient’s microscopic features is also an atypical finding, although it may be expected to some degree in scintigraphically hot, large lesions.28 The clinical and imaging progress of the lesion supported the diagnosis of a giant bone island. The degree of the anterior tibial bowing and the volume of the lesion in relation to the host bone were not changed throughout the follow-up period, indicating that the growth of the lesion followed the growth of the normal bone.

 

 

The differential diagnosis of a giant bone island includes a variety of benign tumors and tumor-like lesions, as well as malignant bone lesions.2,4,23,28,30,31 In the patient presented in this report, the diagnosis of an atypical sclerotic presentation of a nonossifying fibroma or healing stage of this lesion could be consistent with some of the CT findings, including the eccentric origin from the cortex associated with medial cortical thickening, the anterolateral cortical thinning, and the soft-tissue attenuation of cortical areas. In addition, unifocal osteofibrous dysplasia may also present with a long intracortical diaphyseal lucency within an area of marked cortical sclerosis and cause a bowing deformity. Both diagnoses were excluded, since no fibrous stroma was evident on the histologic examination of the lesion. A large or giant long-bone osteoma would be associated with the outer cortical margin of bone but would not involve the intramedullary space. The scintigraphically increased uptake of radioisotope, as well as the CT and MRI findings, were not consistent with the diagnosis of osteoid osteoma, osteoblastoma, or osteomyelitis. Although most imaging findings were consistent with a benign lesion, and contrast-enhanced MRI showed no increased vascularity, anterior cortical disruption necessitated a bone biopsy to rule out any potential malignancy.

The histopathology in association with the clinical and imaging findings indicated the diagnosis of a giant bone island. The increased proportion of maturing woven bone over lamellar bone indicated an active remodeling lesion that could be related to the patient’s age, since the clinical and radiographic features of the lesion were not changed after 15-year follow-up.

Conclusion

This is the first giant bone island diagnosed in a patient before puberty. Its greatest length was 10.8 cm, which is the longest reported in the literature. The imaging appearance included several atypical features that are very rare or have not been reported. Microscopic features indicated less mature lamellar bone and a prominent proportion of maturing woven bone. The clinical and the radiographic appearance of the lesion were not changed after 15-year follow-up.

References

1.    Smith J. Giant bone islands. Radiology. 1973;7(1):35-36.

2.    Mirra JM. Bone Tumors: Clinical, Radiologic and Pathologic Correlations. Philadelphia, PA: Lea & Febiger; 1989.

3.    Greenspan A. Bone island (enostosis): current concept - a review. Skeletal Radiol. 1995;24(2):111-115.

4.    Kransdorf MJ, Peterson JJ, Bancroft LW. MR imaging of the knee: incidental osseous lesions. Radiol Clin North Am. 2007;45(6):943-954.

5.    Gold RH, Mirra JM, Remotti F, Pignatti G. Case report 527: Giant bone island of tibia. Skeletal Radiol. 1989;18(2):129-132.

6.    Onitsuka H. Roentgenologic aspects of bone islands. Radiology. 1977;123(3):607-612.

7.    Ehara S, Kattapuram SV, Rosenberg AE. Giant bone island. Computed tomography findings. Clin Imaging. 1989;13(3):231-233.

8.    Greenspan A, Steiner G, Knutzon R. Bone island (enostosis): clinical significance and radiologic and pathologic correlations. Skeletal Radiol. 1991;20(2):85-90.

9.    Avery GR, Wilsdon JB, Malcolm AJ. Giant bone island with some central resorption. Skeletal Radiol. 1995;24(1):59-60.

10.  Brien EW, Mirra JM, Latanza L, Fedenko A, Luck J Jr. Giant bone island of femur. Case report, literature review, and its distinction from low grade osteosarcoma. Skeletal Radiol. 1995;24(7):546-550.

11.    Greenspan A, Klein MJ. Giant bone island. Skeletal Radiol. 1996;25(1):67-69.

12.  Trombetti A, Noël E. Giant bone islands: a case with 31 years of follow-up. Joint Bone Spine. 2002;69(1):81-84.

13.  Dhaon BK, Gautam VK, Jain P, Jaiswal A, Nigam V. Giant bone island of femur complicating replacement arthroplasty: a report of two cases. J Surg Orthop Adv. 2004;13(4):220-223.

14.  Park HS, Kim JR, Lee SY, Jang KY. Symptomatic giant (10-cm) bone island of the tibia. Skeletal Radiol. 2005;34(6):347-350.

15.  Ikeuchi M, Komatsu M, Tani T. Giant bone island of femur with femoral head necrosis: a case report. Arch Orthop Trauma Surg. 2010;130(4):447-450.

16.  Kim SK, Barry WF Jr. Bone island. Am J Roentgenol Radium Ther Nucl Med. 1964;92:1301-1306. 

17.  Kim SK, Barry WF Jr. Bone islands. Radiology. 1968;90(1):77-78. 

18.  Cerase A, Priolo F. Skeletal benign bone-forming lesions. Eur J Radiol. 1998;27:S91–S97.

19.  Go RT, El-Khoury GY, Wehbe MA. Radionuclide bone image in growing and stable bone island. Skeletal Radiol. 1980;5(1):15-18.

20.  Hall FM, Goldberg RP, Davies JA, Fainsinger MH. Scintigraphic assessment of bone islands. Radiology. 1980;135(3):737-742.

21.  Greenspan A, Stadalnik RC. Bone island: scintigraphic findings and their clinical application. Can Assoc Radiol J. 1995;46(5):368-379.

22.  Sickles EA, Genant HK, Hoffer PB. Increased localization of 99mTc-pyrophosphate in a bone island: case report. J Nucl Med. 1976;17(2):113-115.

23.  Dorfman HD, Czerniak B. Bone Tumors. St Louis: Mosby; 1998.

24.  Ngan H. Growing bone islands. Clin Radiol. 1972;23(2):199-201.

25.  Davies JA, Hall FM, Goldberg RP, Kasdon EJ. Positive bone scan in a bone island. Case report. J Bone Joint Surg Am. 1979;61(6):943-945.

26.  Simon K, Mulligan ME. Growing bone islands revisited. A case report. J Bone Joint Surg Am. 1985;67(5):809-811.

27.  Blank N, Lieber A. The significance of growing bone islands. Radiology. 1965;85(3):508-511.

28.  Greenspan A, Gernot J, Wolfgang R. Differential Diagnosis of Orthopaedic Oncology. Philadelphia, PA: Lippincott Williams & Wilkins; 2007.

29.  Kransdorf MJ, Murphey MD. Osseous tumors. In: Davies AM, Sundaram M, James SLJ, eds. Imaging of Bone Tumors and Tumor-Like Lesions. Berlin, Germany: Springer-Verlag; 2009.

30.  Mödder B, Guhl B, Schaefer HE. Growing bone islands as differential diagnosis of osteoplastic metastases. Rontgenblatter. 1980;33(6):286-288.

31.  Flechner RE, Mills SE. Atlas of Tumor Pathology: Tumors of the Bones and Joints. Washington, DC: Armed Forces Institute of Pathology; 1993.

References

1.    Smith J. Giant bone islands. Radiology. 1973;7(1):35-36.

2.    Mirra JM. Bone Tumors: Clinical, Radiologic and Pathologic Correlations. Philadelphia, PA: Lea & Febiger; 1989.

3.    Greenspan A. Bone island (enostosis): current concept - a review. Skeletal Radiol. 1995;24(2):111-115.

4.    Kransdorf MJ, Peterson JJ, Bancroft LW. MR imaging of the knee: incidental osseous lesions. Radiol Clin North Am. 2007;45(6):943-954.

5.    Gold RH, Mirra JM, Remotti F, Pignatti G. Case report 527: Giant bone island of tibia. Skeletal Radiol. 1989;18(2):129-132.

6.    Onitsuka H. Roentgenologic aspects of bone islands. Radiology. 1977;123(3):607-612.

7.    Ehara S, Kattapuram SV, Rosenberg AE. Giant bone island. Computed tomography findings. Clin Imaging. 1989;13(3):231-233.

8.    Greenspan A, Steiner G, Knutzon R. Bone island (enostosis): clinical significance and radiologic and pathologic correlations. Skeletal Radiol. 1991;20(2):85-90.

9.    Avery GR, Wilsdon JB, Malcolm AJ. Giant bone island with some central resorption. Skeletal Radiol. 1995;24(1):59-60.

10.  Brien EW, Mirra JM, Latanza L, Fedenko A, Luck J Jr. Giant bone island of femur. Case report, literature review, and its distinction from low grade osteosarcoma. Skeletal Radiol. 1995;24(7):546-550.

11.    Greenspan A, Klein MJ. Giant bone island. Skeletal Radiol. 1996;25(1):67-69.

12.  Trombetti A, Noël E. Giant bone islands: a case with 31 years of follow-up. Joint Bone Spine. 2002;69(1):81-84.

13.  Dhaon BK, Gautam VK, Jain P, Jaiswal A, Nigam V. Giant bone island of femur complicating replacement arthroplasty: a report of two cases. J Surg Orthop Adv. 2004;13(4):220-223.

14.  Park HS, Kim JR, Lee SY, Jang KY. Symptomatic giant (10-cm) bone island of the tibia. Skeletal Radiol. 2005;34(6):347-350.

15.  Ikeuchi M, Komatsu M, Tani T. Giant bone island of femur with femoral head necrosis: a case report. Arch Orthop Trauma Surg. 2010;130(4):447-450.

16.  Kim SK, Barry WF Jr. Bone island. Am J Roentgenol Radium Ther Nucl Med. 1964;92:1301-1306. 

17.  Kim SK, Barry WF Jr. Bone islands. Radiology. 1968;90(1):77-78. 

18.  Cerase A, Priolo F. Skeletal benign bone-forming lesions. Eur J Radiol. 1998;27:S91–S97.

19.  Go RT, El-Khoury GY, Wehbe MA. Radionuclide bone image in growing and stable bone island. Skeletal Radiol. 1980;5(1):15-18.

20.  Hall FM, Goldberg RP, Davies JA, Fainsinger MH. Scintigraphic assessment of bone islands. Radiology. 1980;135(3):737-742.

21.  Greenspan A, Stadalnik RC. Bone island: scintigraphic findings and their clinical application. Can Assoc Radiol J. 1995;46(5):368-379.

22.  Sickles EA, Genant HK, Hoffer PB. Increased localization of 99mTc-pyrophosphate in a bone island: case report. J Nucl Med. 1976;17(2):113-115.

23.  Dorfman HD, Czerniak B. Bone Tumors. St Louis: Mosby; 1998.

24.  Ngan H. Growing bone islands. Clin Radiol. 1972;23(2):199-201.

25.  Davies JA, Hall FM, Goldberg RP, Kasdon EJ. Positive bone scan in a bone island. Case report. J Bone Joint Surg Am. 1979;61(6):943-945.

26.  Simon K, Mulligan ME. Growing bone islands revisited. A case report. J Bone Joint Surg Am. 1985;67(5):809-811.

27.  Blank N, Lieber A. The significance of growing bone islands. Radiology. 1965;85(3):508-511.

28.  Greenspan A, Gernot J, Wolfgang R. Differential Diagnosis of Orthopaedic Oncology. Philadelphia, PA: Lippincott Williams & Wilkins; 2007.

29.  Kransdorf MJ, Murphey MD. Osseous tumors. In: Davies AM, Sundaram M, James SLJ, eds. Imaging of Bone Tumors and Tumor-Like Lesions. Berlin, Germany: Springer-Verlag; 2009.

30.  Mödder B, Guhl B, Schaefer HE. Growing bone islands as differential diagnosis of osteoplastic metastases. Rontgenblatter. 1980;33(6):286-288.

31.  Flechner RE, Mills SE. Atlas of Tumor Pathology: Tumors of the Bones and Joints. Washington, DC: Armed Forces Institute of Pathology; 1993.

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Giant Bone Island of the Tibia in a Child
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Nonconsecutive Pars Interarticularis Defects

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Nonconsecutive Pars Interarticularis Defects

Spondylolysis is a bone defect of the pars interarticularis. It is usually seen in adolescents who participate in sporting activities that involve repetitive axial loads to a hyperextended lower back, such as football offensive lineman, throwing athletes, and gymnasts. It occurs frequently in the L5 pars and can be unilateral or bilateral. The majority of reported multiple-level spondylolysis is at consecutive lumbar segments.1-6 Rarely, it affects noncontiguous levels. Most patients respond well to conservative treatment in the form of activity modification and orthosis.7 Surgical intervention is considered if 6 months of conservative management fails, spondylolisthesis develops, or intractable neurologic symptoms arise.

This case report presents an 18-year-old man with noncontiguous spondylolysis at L2 and L5 who was successfully treated with a 1-level pars repair at L2 after failed conservative management. This unique case highlights the importance of using single-photon emission computed tomography (SPECT) scan and diagnostic pars block when planning for surgical treatment in the rare cases of noncontiguous spondylolysis. The patient provided written informed consent for print and electronic publication of this case report.

Case Report

An 18-year-old man presented to the clinic with worsening lower back pain for the previous 4 weeks. He was playing high school baseball and stated the pain was worse when he swung his bat. He had no history of trauma or back pain. Rest was the only alleviating factor, and he was beginning to experience pain when he stood after sitting. He denied any radicular pain. On examination, he had midline tenderness along the upper lumbar spine and pain with lumbar spine extension. His neurologic examination showed normal sensation with 5/5 strength in all key muscle groups. Plain radiograph of the lumbar spine showed an L5 pars defect (Figures 1A, 1B). A SPECT scan showed increased uptake at L2 pars bilaterally; the L5 pars did not show increased uptake (Figures 2A, 2B). A computed tomography (CT) scan confirmed bilateral L2 pars fractures and a left L5 pars fracture (Figures 3A, 3B). Bony changes in the form of marginal sclerosis at the L5, but not the L2, pars suggested that the L2 fracture was acute while the L5 fracture was chronic (Figures 4A, 4B).

 

 

The patient had conservative management for 6 months in the form of lumbosacral orthosis (LSO), cessation of sports activities, and physical therapy. The patient was initially treated with an LSO brace for 3 months, after which he had physical therapy. At 6 month follow-up, he reported continuing, significant back pain. A repeat CT scan of the lumbar spine showed no interval healing of the bilateral L2 or the unilateral L5 pars fractures. As a result of the patient’s noncontiguous pars fractures, a diagnostic CT-guided block of L2 pars was performed to identify which level was his main pain generator (Figure 5). He reported a brief period of complete pain relief after the L2 pars block. With failure of 6 months’ conservative management and positive SPECT scan and diagnostic block, surgical treatment was recommended. Prior to surgical intervention, magnetic resonance imaging was obtained to rule out pathology (eg, disc degeneration, infection, or tumor) other than the pars defect that could require fusion instead of pars repair (Figures 6A, 6B). Because of the patient’s young age, bilateral L2 pars repair rather than fusion was indicated. After 8 months of persistent back pain, he underwent bilateral L2 pars repair with iliac crest autograft, pedicle screws, and sublaminar hook fixation (Figures 7A, 7B). The patient had an uneventful immediate postoperative course. A 6-month postoperative CT scan showed bridging callus at the L2 pars; however, the left L5 pars fracture was still visible (Figures 8A-8C). Over a 6-month postoperative period, the patient had continued improvement in his back pain, advanced his activity as tolerated without problem, and was allowed to resume his sports activities. At 2-year follow-up, he was playing baseball and basketball, and denied any back pain. 

 
 
 

Discussion

Lumbar spondylolysis is commonly seen at the fourth and fifth lumbar vertebrae, and accounts for more than 95% of spondylolysis cases.8 Multiple-level spondylolysis is a relatively rare finding with an incidence varying between 1.2% and 5.6%. The majority of the reported multiple-level cases are adjacent.1-3,6 Adolescents often present with a history of insidious-onset low back pain without radicular symptoms that is exacerbated by activity. Occasionally, an acute injury may elicit the onset of pain. A thorough history with emphasis on pain in relation to activity and sports involvement is beneficial. The patient in the current study was a throwing athlete and presented with 4 weeks of back pain that worsened with activity; he had no history of trauma.

 

 

Radiographic assessment using standing anteroposterior, lateral, and oblique radiographs of the thoracolumbar spine is useful in the initial assessment. A SPECT scan of the lumbosacral spine is highly sensitive for identifying spondylolytic defects when plain radiographs are within normal limits, yet a high index of suspicion remains given the patient’s history and physical examination findings.9,10 Increased radionuclide uptake within the pars indicates a stress reaction and, possibly, a more acute pathology. The plain radiographs of the patient showed only L5 spondylolysis. However, a SPECT scan showed only increased uptake in L2 pars on both sides. These findings suggested chronic L5 and acute L2 pars defects. A thin-cut CT scan gives the best visualization of pars defect and can help in differentiating chronic defect with sclerotic margins versus acute defect with hazy irregular margins. In the current case, the CT scan showed changes consistent with unilateral chronic L5 and bilateral acute L2 pars defects.

The origin of the pain in spondylolysis is from the tissues rich in nociceptive nerve endings in the loose posterior arch. A CT-guided pars block is a very useful diagnostic preoperative tool that confirms the symptomatic level in cases of multilevel pars defect, especially if they are noncontiguous. In this case, the diagnostic preoperative bilateral L2 pars block confirmed that the pain generator was the acute L2 rather than the chronic L5 pars defect. This step assured that surgical treatment involving only the L2 level would be beneficial in alleviating the patient’s back pain after the failure of 6 months of conservative treatment.

Most patients with single-level spondylolysis respond to conservative treatment, especially after early diagnosis and treatment. The traditional nonoperative treatment of children and adolescents with a symptomatic spondylolytic lesion was a period of rest and progressive increased activity with physical therapy. Immobilization with an LSO was reserved for individuals who did not respond to rest and physical therapy.11 However, multiple studies revealed early immobilization achieved results superior to activity restriction alone, and individuals who underwent a period of activity restriction prior to bracing were more likely to experience persistent symptoms.12-14 Our patient underwent conservative treatment for 6 months, in the form of LSO, cessation of sport activities, and physical therapy, which failed to give him relief of his back pain.

Surgical intervention is warranted for adolescents with persistent, debilitating pain intractable to at least a 6-month period of nonoperative management. Additional indications for surgical management are those individuals who present with neurologic deficits and isthmic spondylolisthesis. Surgical treatment involves direct pars repair with iliac crest bone graft and use of a sublaminar hook/pedicle screw construct, cerclage wire, or pars screw.15-18 

In contrast to single-level pars defects that respond well to conservative treatment, there are conflicting reports regarding the management of multiple-level pars fractures; a few reports suggest good outcome with conservative management, but the majority state that surgery is often required and conservative measures are rarely useful.1-4,6 Nayeemuddin and colleagues19 reported a case of a 16-year-old football player who presented with a 4-month history of constant low back pain related to bilateral L3 and L5 pars defects that responded to 1 year of conservative management, when the more acute fractures at L3 showed complete bony union and the patient had symptomatic pain relief and was able to return to full sporting activity.

 Chang and colleagues2 reported 10 patients with adjacent 2-level bilateral spondylolysis treated successfully using a pedicle screw–hook construct with autogenous bone grafting. Ogawa and colleagues5 reported adjacent 2-level spondylolysis in 5 patients and 3-level spondylolysis in 2 patients, who were treated successfully by segmental wire fixation and bone grafting. Ivanic and colleagues15 retrospectively reviewed 113 patients with spondylolysis who were treated with direct repair using a hook-screw construct and showed a pseudoarthrosis rate of 13.3%. Superior fusion rates were observed in patients 14 years and younger compared with older patients, particularly those 20 years and older.15 Roca and colleagues16 prospectively analyzed 19 consecutive cases of spondylolysis that were repaired using a hook-screw construct. Twelve of 13 patients (92%) who were 20 years or younger at the time of the study (average age, 17.2 years) had fusion, whereas, in 6 patients 21 years and older (average age, 27.5 years), no cases of fusion were observed. The patients 20 years or younger had significantly better clinical results than those obtained in the patients 21 years and older. The authors concluded that pedicle screw–hook fixation is a useful alternative in the treatment of spondylolysis in adolescents, but did not recommend this procedure in patients older than 20 years.16

 

 

Conclusion

The current case demonstrates a unique example of rare noncontiguous pars defects successfully treated with primary repair of 1 level when conservative management failed and the symptomatic defect was isolated. It also highlights the importance of investigating the entirety of the lumbar spine when diagnosis of L5 spondylolysis rules out noncontiguous pars defects. The treatment of noncontiguous pars defects is not well defined; this case showed the importance of using a SPECT scan and a diagnostic pars block to help isolate the symptomatic level when surgical management is considered after a failure of conservative treatment. This case shows 2 possible results: the chronic unilateral L5 defect responded to nonsurgical treatment with asymptomatic fibrous nonunion, while the more acute bilateral L2 defect responded to pars repair with pedicle screw–hook fixation and iliac crest bone graft. 

References

1.    Al-Sebai MW, Al-Khawashki H. Spondyloptosis and multiple-level spondylolysis. Eur Spine J. 1999;8(1):75-77.

2.    Chang JH, Lee CH, Wu SS, Lin LC, et al. Management of multiple level spondylolysis of the lumbar spine in young males: a report of six cases. J Formos Med Assoc. 2001;100(7)2:497-502.

3.    Eingorn D, Pizzutillo PD. Pars interarticularis fusion of multiple levels of lumbar spondylolysis. A case report. Spine. 1985;10(3):250-252.

4.    Nozawa S, Shimizu K, Miyamoto K, Tanaka M. Repair of pars interarticularis defect by segmental wire fixation in young athletes with spondylolysis. Am J Sports Med. 2003;31(3):359-364.

5.    Ogawa H, Nishimoto H, Hosoe H, Suzuki N, Kanamori Y, Shimizu K. Clinical outcome after segmental wire fixation and bone grafting for repair of the defects in multiple level lumbar spondylolysis. J Spinal Disord Tech. 2007;20(7):521-525.

6.    Ravichandran G. Multiple lumbar spondylolyses. Spine. 1980;5(6):552-557.

7.    Sys J, Michielsen J, Bracke P, Martens M, Verstreken J. Nonoperative treatment of active spondylolysis in elite athletes with normal X-ray findings: literature review and results of conservative treatment. Eur Spine J. 2001;10(6):498-504.

8.    Saraste H. Spondylolysis and spondylolisthesis. Acta Orthop Scand Suppl. 1993;251:84-86.

9.    Anderson K, Sarwark JF, Conway JJ, Logue ES, Schafer MS. Quantitative assessment with SPECT imaging of stress injuries of the pars interarticularis and response to bracing. J Pediatr Orthop. 2000;20(1):28-33.

10.  Bodner RJ, Heyman S, Drummond DS, Gregg JR. The use of single photon emission computed tomography (SPECT) in the diagnosis of low-back pain in young patients. Spine. 1988;13(10):1155-1160.

11.  Steiner ME, Micheli LJ. Treatment of symptomatic spondylolysis and spondylolisthesis with the modified Boston brace. Spine. 1985;10(10):937-943.

12.  Blanda J, Bethem D, Moats W, Lew M. Defects of pars interarticularis in athletes: a protocol for nonoperative treatment. J Spinal Disord. 1993;6(5):406-411.

13.  Kurd MF, Patel D, Norton R, Picetti G, Friel B, Vaccaro AR. Nonoperative treatment of symptomatic spondylolysis. J Spinal Disord Tech. 2007;20(8):560-564.

14.  Pizzutillo PD, Hummer CD 3rd. Nonoperative treatment for painful adolescent spondylolysis or spondylolisthesis. J Pediatr Orthop. 1989;9(5):538-540.

15.  Ivanic GM, Pink TP, Achatz W, Ward JC, Homann NC, May M. Direct stabilization of lumbar spondylolysis with a hook screw: mean 11-year follow-up period for 113 patients. Spine. 2003;28(3):255-259.

16.  Roca J, Iborra M, Cavanilles-Walker JM, Alberti G. Direct repair of spondylolysis using a new pedicle screw hook fixation: clinical and CT-assessed study: an analysis of 19 patients. J Spinal Disord Tech. 2005;18(suppl):S82-S89.

17.  Schlenzka D, Remes V, Helenius I, et al. Direct repair for treatment of symptomatic spondylolysis and low-grade isthmic spondylolisthesis in young patients: no benefit in comparison to segmental fusion after a mean follow-up of 14.8 years. Eur Spine J. 2006;15(10):1437-1447.

18.  Buck JE. Direct repair of the defect in spondylolisthesis. Preliminary report. J Bone Joint Surg Br. 1970;52(3):432-437.

19.  Nayeemuddin M, Richards PJ, Ahmed EB. The imaging and management of nonconsecutive pars interarticularis defects: a case report and review of literature. Spine J. 2011;11(12):1157-1163.

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american journal of orthopedics, AJO, online exclusive, case report and literature review, case report, pars, interarticularis defects, lumbar spondylolysis, adolescents, pediatrics, spine, fractures, fracture management, pars fracture, back, pain, imaging, elgafy, hart, tanios
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Spondylolysis is a bone defect of the pars interarticularis. It is usually seen in adolescents who participate in sporting activities that involve repetitive axial loads to a hyperextended lower back, such as football offensive lineman, throwing athletes, and gymnasts. It occurs frequently in the L5 pars and can be unilateral or bilateral. The majority of reported multiple-level spondylolysis is at consecutive lumbar segments.1-6 Rarely, it affects noncontiguous levels. Most patients respond well to conservative treatment in the form of activity modification and orthosis.7 Surgical intervention is considered if 6 months of conservative management fails, spondylolisthesis develops, or intractable neurologic symptoms arise.

This case report presents an 18-year-old man with noncontiguous spondylolysis at L2 and L5 who was successfully treated with a 1-level pars repair at L2 after failed conservative management. This unique case highlights the importance of using single-photon emission computed tomography (SPECT) scan and diagnostic pars block when planning for surgical treatment in the rare cases of noncontiguous spondylolysis. The patient provided written informed consent for print and electronic publication of this case report.

Case Report

An 18-year-old man presented to the clinic with worsening lower back pain for the previous 4 weeks. He was playing high school baseball and stated the pain was worse when he swung his bat. He had no history of trauma or back pain. Rest was the only alleviating factor, and he was beginning to experience pain when he stood after sitting. He denied any radicular pain. On examination, he had midline tenderness along the upper lumbar spine and pain with lumbar spine extension. His neurologic examination showed normal sensation with 5/5 strength in all key muscle groups. Plain radiograph of the lumbar spine showed an L5 pars defect (Figures 1A, 1B). A SPECT scan showed increased uptake at L2 pars bilaterally; the L5 pars did not show increased uptake (Figures 2A, 2B). A computed tomography (CT) scan confirmed bilateral L2 pars fractures and a left L5 pars fracture (Figures 3A, 3B). Bony changes in the form of marginal sclerosis at the L5, but not the L2, pars suggested that the L2 fracture was acute while the L5 fracture was chronic (Figures 4A, 4B).

 

 

The patient had conservative management for 6 months in the form of lumbosacral orthosis (LSO), cessation of sports activities, and physical therapy. The patient was initially treated with an LSO brace for 3 months, after which he had physical therapy. At 6 month follow-up, he reported continuing, significant back pain. A repeat CT scan of the lumbar spine showed no interval healing of the bilateral L2 or the unilateral L5 pars fractures. As a result of the patient’s noncontiguous pars fractures, a diagnostic CT-guided block of L2 pars was performed to identify which level was his main pain generator (Figure 5). He reported a brief period of complete pain relief after the L2 pars block. With failure of 6 months’ conservative management and positive SPECT scan and diagnostic block, surgical treatment was recommended. Prior to surgical intervention, magnetic resonance imaging was obtained to rule out pathology (eg, disc degeneration, infection, or tumor) other than the pars defect that could require fusion instead of pars repair (Figures 6A, 6B). Because of the patient’s young age, bilateral L2 pars repair rather than fusion was indicated. After 8 months of persistent back pain, he underwent bilateral L2 pars repair with iliac crest autograft, pedicle screws, and sublaminar hook fixation (Figures 7A, 7B). The patient had an uneventful immediate postoperative course. A 6-month postoperative CT scan showed bridging callus at the L2 pars; however, the left L5 pars fracture was still visible (Figures 8A-8C). Over a 6-month postoperative period, the patient had continued improvement in his back pain, advanced his activity as tolerated without problem, and was allowed to resume his sports activities. At 2-year follow-up, he was playing baseball and basketball, and denied any back pain. 

 
 
 

Discussion

Lumbar spondylolysis is commonly seen at the fourth and fifth lumbar vertebrae, and accounts for more than 95% of spondylolysis cases.8 Multiple-level spondylolysis is a relatively rare finding with an incidence varying between 1.2% and 5.6%. The majority of the reported multiple-level cases are adjacent.1-3,6 Adolescents often present with a history of insidious-onset low back pain without radicular symptoms that is exacerbated by activity. Occasionally, an acute injury may elicit the onset of pain. A thorough history with emphasis on pain in relation to activity and sports involvement is beneficial. The patient in the current study was a throwing athlete and presented with 4 weeks of back pain that worsened with activity; he had no history of trauma.

 

 

Radiographic assessment using standing anteroposterior, lateral, and oblique radiographs of the thoracolumbar spine is useful in the initial assessment. A SPECT scan of the lumbosacral spine is highly sensitive for identifying spondylolytic defects when plain radiographs are within normal limits, yet a high index of suspicion remains given the patient’s history and physical examination findings.9,10 Increased radionuclide uptake within the pars indicates a stress reaction and, possibly, a more acute pathology. The plain radiographs of the patient showed only L5 spondylolysis. However, a SPECT scan showed only increased uptake in L2 pars on both sides. These findings suggested chronic L5 and acute L2 pars defects. A thin-cut CT scan gives the best visualization of pars defect and can help in differentiating chronic defect with sclerotic margins versus acute defect with hazy irregular margins. In the current case, the CT scan showed changes consistent with unilateral chronic L5 and bilateral acute L2 pars defects.

The origin of the pain in spondylolysis is from the tissues rich in nociceptive nerve endings in the loose posterior arch. A CT-guided pars block is a very useful diagnostic preoperative tool that confirms the symptomatic level in cases of multilevel pars defect, especially if they are noncontiguous. In this case, the diagnostic preoperative bilateral L2 pars block confirmed that the pain generator was the acute L2 rather than the chronic L5 pars defect. This step assured that surgical treatment involving only the L2 level would be beneficial in alleviating the patient’s back pain after the failure of 6 months of conservative treatment.

Most patients with single-level spondylolysis respond to conservative treatment, especially after early diagnosis and treatment. The traditional nonoperative treatment of children and adolescents with a symptomatic spondylolytic lesion was a period of rest and progressive increased activity with physical therapy. Immobilization with an LSO was reserved for individuals who did not respond to rest and physical therapy.11 However, multiple studies revealed early immobilization achieved results superior to activity restriction alone, and individuals who underwent a period of activity restriction prior to bracing were more likely to experience persistent symptoms.12-14 Our patient underwent conservative treatment for 6 months, in the form of LSO, cessation of sport activities, and physical therapy, which failed to give him relief of his back pain.

Surgical intervention is warranted for adolescents with persistent, debilitating pain intractable to at least a 6-month period of nonoperative management. Additional indications for surgical management are those individuals who present with neurologic deficits and isthmic spondylolisthesis. Surgical treatment involves direct pars repair with iliac crest bone graft and use of a sublaminar hook/pedicle screw construct, cerclage wire, or pars screw.15-18 

In contrast to single-level pars defects that respond well to conservative treatment, there are conflicting reports regarding the management of multiple-level pars fractures; a few reports suggest good outcome with conservative management, but the majority state that surgery is often required and conservative measures are rarely useful.1-4,6 Nayeemuddin and colleagues19 reported a case of a 16-year-old football player who presented with a 4-month history of constant low back pain related to bilateral L3 and L5 pars defects that responded to 1 year of conservative management, when the more acute fractures at L3 showed complete bony union and the patient had symptomatic pain relief and was able to return to full sporting activity.

 Chang and colleagues2 reported 10 patients with adjacent 2-level bilateral spondylolysis treated successfully using a pedicle screw–hook construct with autogenous bone grafting. Ogawa and colleagues5 reported adjacent 2-level spondylolysis in 5 patients and 3-level spondylolysis in 2 patients, who were treated successfully by segmental wire fixation and bone grafting. Ivanic and colleagues15 retrospectively reviewed 113 patients with spondylolysis who were treated with direct repair using a hook-screw construct and showed a pseudoarthrosis rate of 13.3%. Superior fusion rates were observed in patients 14 years and younger compared with older patients, particularly those 20 years and older.15 Roca and colleagues16 prospectively analyzed 19 consecutive cases of spondylolysis that were repaired using a hook-screw construct. Twelve of 13 patients (92%) who were 20 years or younger at the time of the study (average age, 17.2 years) had fusion, whereas, in 6 patients 21 years and older (average age, 27.5 years), no cases of fusion were observed. The patients 20 years or younger had significantly better clinical results than those obtained in the patients 21 years and older. The authors concluded that pedicle screw–hook fixation is a useful alternative in the treatment of spondylolysis in adolescents, but did not recommend this procedure in patients older than 20 years.16

 

 

Conclusion

The current case demonstrates a unique example of rare noncontiguous pars defects successfully treated with primary repair of 1 level when conservative management failed and the symptomatic defect was isolated. It also highlights the importance of investigating the entirety of the lumbar spine when diagnosis of L5 spondylolysis rules out noncontiguous pars defects. The treatment of noncontiguous pars defects is not well defined; this case showed the importance of using a SPECT scan and a diagnostic pars block to help isolate the symptomatic level when surgical management is considered after a failure of conservative treatment. This case shows 2 possible results: the chronic unilateral L5 defect responded to nonsurgical treatment with asymptomatic fibrous nonunion, while the more acute bilateral L2 defect responded to pars repair with pedicle screw–hook fixation and iliac crest bone graft. 

Spondylolysis is a bone defect of the pars interarticularis. It is usually seen in adolescents who participate in sporting activities that involve repetitive axial loads to a hyperextended lower back, such as football offensive lineman, throwing athletes, and gymnasts. It occurs frequently in the L5 pars and can be unilateral or bilateral. The majority of reported multiple-level spondylolysis is at consecutive lumbar segments.1-6 Rarely, it affects noncontiguous levels. Most patients respond well to conservative treatment in the form of activity modification and orthosis.7 Surgical intervention is considered if 6 months of conservative management fails, spondylolisthesis develops, or intractable neurologic symptoms arise.

This case report presents an 18-year-old man with noncontiguous spondylolysis at L2 and L5 who was successfully treated with a 1-level pars repair at L2 after failed conservative management. This unique case highlights the importance of using single-photon emission computed tomography (SPECT) scan and diagnostic pars block when planning for surgical treatment in the rare cases of noncontiguous spondylolysis. The patient provided written informed consent for print and electronic publication of this case report.

Case Report

An 18-year-old man presented to the clinic with worsening lower back pain for the previous 4 weeks. He was playing high school baseball and stated the pain was worse when he swung his bat. He had no history of trauma or back pain. Rest was the only alleviating factor, and he was beginning to experience pain when he stood after sitting. He denied any radicular pain. On examination, he had midline tenderness along the upper lumbar spine and pain with lumbar spine extension. His neurologic examination showed normal sensation with 5/5 strength in all key muscle groups. Plain radiograph of the lumbar spine showed an L5 pars defect (Figures 1A, 1B). A SPECT scan showed increased uptake at L2 pars bilaterally; the L5 pars did not show increased uptake (Figures 2A, 2B). A computed tomography (CT) scan confirmed bilateral L2 pars fractures and a left L5 pars fracture (Figures 3A, 3B). Bony changes in the form of marginal sclerosis at the L5, but not the L2, pars suggested that the L2 fracture was acute while the L5 fracture was chronic (Figures 4A, 4B).

 

 

The patient had conservative management for 6 months in the form of lumbosacral orthosis (LSO), cessation of sports activities, and physical therapy. The patient was initially treated with an LSO brace for 3 months, after which he had physical therapy. At 6 month follow-up, he reported continuing, significant back pain. A repeat CT scan of the lumbar spine showed no interval healing of the bilateral L2 or the unilateral L5 pars fractures. As a result of the patient’s noncontiguous pars fractures, a diagnostic CT-guided block of L2 pars was performed to identify which level was his main pain generator (Figure 5). He reported a brief period of complete pain relief after the L2 pars block. With failure of 6 months’ conservative management and positive SPECT scan and diagnostic block, surgical treatment was recommended. Prior to surgical intervention, magnetic resonance imaging was obtained to rule out pathology (eg, disc degeneration, infection, or tumor) other than the pars defect that could require fusion instead of pars repair (Figures 6A, 6B). Because of the patient’s young age, bilateral L2 pars repair rather than fusion was indicated. After 8 months of persistent back pain, he underwent bilateral L2 pars repair with iliac crest autograft, pedicle screws, and sublaminar hook fixation (Figures 7A, 7B). The patient had an uneventful immediate postoperative course. A 6-month postoperative CT scan showed bridging callus at the L2 pars; however, the left L5 pars fracture was still visible (Figures 8A-8C). Over a 6-month postoperative period, the patient had continued improvement in his back pain, advanced his activity as tolerated without problem, and was allowed to resume his sports activities. At 2-year follow-up, he was playing baseball and basketball, and denied any back pain. 

 
 
 

Discussion

Lumbar spondylolysis is commonly seen at the fourth and fifth lumbar vertebrae, and accounts for more than 95% of spondylolysis cases.8 Multiple-level spondylolysis is a relatively rare finding with an incidence varying between 1.2% and 5.6%. The majority of the reported multiple-level cases are adjacent.1-3,6 Adolescents often present with a history of insidious-onset low back pain without radicular symptoms that is exacerbated by activity. Occasionally, an acute injury may elicit the onset of pain. A thorough history with emphasis on pain in relation to activity and sports involvement is beneficial. The patient in the current study was a throwing athlete and presented with 4 weeks of back pain that worsened with activity; he had no history of trauma.

 

 

Radiographic assessment using standing anteroposterior, lateral, and oblique radiographs of the thoracolumbar spine is useful in the initial assessment. A SPECT scan of the lumbosacral spine is highly sensitive for identifying spondylolytic defects when plain radiographs are within normal limits, yet a high index of suspicion remains given the patient’s history and physical examination findings.9,10 Increased radionuclide uptake within the pars indicates a stress reaction and, possibly, a more acute pathology. The plain radiographs of the patient showed only L5 spondylolysis. However, a SPECT scan showed only increased uptake in L2 pars on both sides. These findings suggested chronic L5 and acute L2 pars defects. A thin-cut CT scan gives the best visualization of pars defect and can help in differentiating chronic defect with sclerotic margins versus acute defect with hazy irregular margins. In the current case, the CT scan showed changes consistent with unilateral chronic L5 and bilateral acute L2 pars defects.

The origin of the pain in spondylolysis is from the tissues rich in nociceptive nerve endings in the loose posterior arch. A CT-guided pars block is a very useful diagnostic preoperative tool that confirms the symptomatic level in cases of multilevel pars defect, especially if they are noncontiguous. In this case, the diagnostic preoperative bilateral L2 pars block confirmed that the pain generator was the acute L2 rather than the chronic L5 pars defect. This step assured that surgical treatment involving only the L2 level would be beneficial in alleviating the patient’s back pain after the failure of 6 months of conservative treatment.

Most patients with single-level spondylolysis respond to conservative treatment, especially after early diagnosis and treatment. The traditional nonoperative treatment of children and adolescents with a symptomatic spondylolytic lesion was a period of rest and progressive increased activity with physical therapy. Immobilization with an LSO was reserved for individuals who did not respond to rest and physical therapy.11 However, multiple studies revealed early immobilization achieved results superior to activity restriction alone, and individuals who underwent a period of activity restriction prior to bracing were more likely to experience persistent symptoms.12-14 Our patient underwent conservative treatment for 6 months, in the form of LSO, cessation of sport activities, and physical therapy, which failed to give him relief of his back pain.

Surgical intervention is warranted for adolescents with persistent, debilitating pain intractable to at least a 6-month period of nonoperative management. Additional indications for surgical management are those individuals who present with neurologic deficits and isthmic spondylolisthesis. Surgical treatment involves direct pars repair with iliac crest bone graft and use of a sublaminar hook/pedicle screw construct, cerclage wire, or pars screw.15-18 

In contrast to single-level pars defects that respond well to conservative treatment, there are conflicting reports regarding the management of multiple-level pars fractures; a few reports suggest good outcome with conservative management, but the majority state that surgery is often required and conservative measures are rarely useful.1-4,6 Nayeemuddin and colleagues19 reported a case of a 16-year-old football player who presented with a 4-month history of constant low back pain related to bilateral L3 and L5 pars defects that responded to 1 year of conservative management, when the more acute fractures at L3 showed complete bony union and the patient had symptomatic pain relief and was able to return to full sporting activity.

 Chang and colleagues2 reported 10 patients with adjacent 2-level bilateral spondylolysis treated successfully using a pedicle screw–hook construct with autogenous bone grafting. Ogawa and colleagues5 reported adjacent 2-level spondylolysis in 5 patients and 3-level spondylolysis in 2 patients, who were treated successfully by segmental wire fixation and bone grafting. Ivanic and colleagues15 retrospectively reviewed 113 patients with spondylolysis who were treated with direct repair using a hook-screw construct and showed a pseudoarthrosis rate of 13.3%. Superior fusion rates were observed in patients 14 years and younger compared with older patients, particularly those 20 years and older.15 Roca and colleagues16 prospectively analyzed 19 consecutive cases of spondylolysis that were repaired using a hook-screw construct. Twelve of 13 patients (92%) who were 20 years or younger at the time of the study (average age, 17.2 years) had fusion, whereas, in 6 patients 21 years and older (average age, 27.5 years), no cases of fusion were observed. The patients 20 years or younger had significantly better clinical results than those obtained in the patients 21 years and older. The authors concluded that pedicle screw–hook fixation is a useful alternative in the treatment of spondylolysis in adolescents, but did not recommend this procedure in patients older than 20 years.16

 

 

Conclusion

The current case demonstrates a unique example of rare noncontiguous pars defects successfully treated with primary repair of 1 level when conservative management failed and the symptomatic defect was isolated. It also highlights the importance of investigating the entirety of the lumbar spine when diagnosis of L5 spondylolysis rules out noncontiguous pars defects. The treatment of noncontiguous pars defects is not well defined; this case showed the importance of using a SPECT scan and a diagnostic pars block to help isolate the symptomatic level when surgical management is considered after a failure of conservative treatment. This case shows 2 possible results: the chronic unilateral L5 defect responded to nonsurgical treatment with asymptomatic fibrous nonunion, while the more acute bilateral L2 defect responded to pars repair with pedicle screw–hook fixation and iliac crest bone graft. 

References

1.    Al-Sebai MW, Al-Khawashki H. Spondyloptosis and multiple-level spondylolysis. Eur Spine J. 1999;8(1):75-77.

2.    Chang JH, Lee CH, Wu SS, Lin LC, et al. Management of multiple level spondylolysis of the lumbar spine in young males: a report of six cases. J Formos Med Assoc. 2001;100(7)2:497-502.

3.    Eingorn D, Pizzutillo PD. Pars interarticularis fusion of multiple levels of lumbar spondylolysis. A case report. Spine. 1985;10(3):250-252.

4.    Nozawa S, Shimizu K, Miyamoto K, Tanaka M. Repair of pars interarticularis defect by segmental wire fixation in young athletes with spondylolysis. Am J Sports Med. 2003;31(3):359-364.

5.    Ogawa H, Nishimoto H, Hosoe H, Suzuki N, Kanamori Y, Shimizu K. Clinical outcome after segmental wire fixation and bone grafting for repair of the defects in multiple level lumbar spondylolysis. J Spinal Disord Tech. 2007;20(7):521-525.

6.    Ravichandran G. Multiple lumbar spondylolyses. Spine. 1980;5(6):552-557.

7.    Sys J, Michielsen J, Bracke P, Martens M, Verstreken J. Nonoperative treatment of active spondylolysis in elite athletes with normal X-ray findings: literature review and results of conservative treatment. Eur Spine J. 2001;10(6):498-504.

8.    Saraste H. Spondylolysis and spondylolisthesis. Acta Orthop Scand Suppl. 1993;251:84-86.

9.    Anderson K, Sarwark JF, Conway JJ, Logue ES, Schafer MS. Quantitative assessment with SPECT imaging of stress injuries of the pars interarticularis and response to bracing. J Pediatr Orthop. 2000;20(1):28-33.

10.  Bodner RJ, Heyman S, Drummond DS, Gregg JR. The use of single photon emission computed tomography (SPECT) in the diagnosis of low-back pain in young patients. Spine. 1988;13(10):1155-1160.

11.  Steiner ME, Micheli LJ. Treatment of symptomatic spondylolysis and spondylolisthesis with the modified Boston brace. Spine. 1985;10(10):937-943.

12.  Blanda J, Bethem D, Moats W, Lew M. Defects of pars interarticularis in athletes: a protocol for nonoperative treatment. J Spinal Disord. 1993;6(5):406-411.

13.  Kurd MF, Patel D, Norton R, Picetti G, Friel B, Vaccaro AR. Nonoperative treatment of symptomatic spondylolysis. J Spinal Disord Tech. 2007;20(8):560-564.

14.  Pizzutillo PD, Hummer CD 3rd. Nonoperative treatment for painful adolescent spondylolysis or spondylolisthesis. J Pediatr Orthop. 1989;9(5):538-540.

15.  Ivanic GM, Pink TP, Achatz W, Ward JC, Homann NC, May M. Direct stabilization of lumbar spondylolysis with a hook screw: mean 11-year follow-up period for 113 patients. Spine. 2003;28(3):255-259.

16.  Roca J, Iborra M, Cavanilles-Walker JM, Alberti G. Direct repair of spondylolysis using a new pedicle screw hook fixation: clinical and CT-assessed study: an analysis of 19 patients. J Spinal Disord Tech. 2005;18(suppl):S82-S89.

17.  Schlenzka D, Remes V, Helenius I, et al. Direct repair for treatment of symptomatic spondylolysis and low-grade isthmic spondylolisthesis in young patients: no benefit in comparison to segmental fusion after a mean follow-up of 14.8 years. Eur Spine J. 2006;15(10):1437-1447.

18.  Buck JE. Direct repair of the defect in spondylolisthesis. Preliminary report. J Bone Joint Surg Br. 1970;52(3):432-437.

19.  Nayeemuddin M, Richards PJ, Ahmed EB. The imaging and management of nonconsecutive pars interarticularis defects: a case report and review of literature. Spine J. 2011;11(12):1157-1163.

References

1.    Al-Sebai MW, Al-Khawashki H. Spondyloptosis and multiple-level spondylolysis. Eur Spine J. 1999;8(1):75-77.

2.    Chang JH, Lee CH, Wu SS, Lin LC, et al. Management of multiple level spondylolysis of the lumbar spine in young males: a report of six cases. J Formos Med Assoc. 2001;100(7)2:497-502.

3.    Eingorn D, Pizzutillo PD. Pars interarticularis fusion of multiple levels of lumbar spondylolysis. A case report. Spine. 1985;10(3):250-252.

4.    Nozawa S, Shimizu K, Miyamoto K, Tanaka M. Repair of pars interarticularis defect by segmental wire fixation in young athletes with spondylolysis. Am J Sports Med. 2003;31(3):359-364.

5.    Ogawa H, Nishimoto H, Hosoe H, Suzuki N, Kanamori Y, Shimizu K. Clinical outcome after segmental wire fixation and bone grafting for repair of the defects in multiple level lumbar spondylolysis. J Spinal Disord Tech. 2007;20(7):521-525.

6.    Ravichandran G. Multiple lumbar spondylolyses. Spine. 1980;5(6):552-557.

7.    Sys J, Michielsen J, Bracke P, Martens M, Verstreken J. Nonoperative treatment of active spondylolysis in elite athletes with normal X-ray findings: literature review and results of conservative treatment. Eur Spine J. 2001;10(6):498-504.

8.    Saraste H. Spondylolysis and spondylolisthesis. Acta Orthop Scand Suppl. 1993;251:84-86.

9.    Anderson K, Sarwark JF, Conway JJ, Logue ES, Schafer MS. Quantitative assessment with SPECT imaging of stress injuries of the pars interarticularis and response to bracing. J Pediatr Orthop. 2000;20(1):28-33.

10.  Bodner RJ, Heyman S, Drummond DS, Gregg JR. The use of single photon emission computed tomography (SPECT) in the diagnosis of low-back pain in young patients. Spine. 1988;13(10):1155-1160.

11.  Steiner ME, Micheli LJ. Treatment of symptomatic spondylolysis and spondylolisthesis with the modified Boston brace. Spine. 1985;10(10):937-943.

12.  Blanda J, Bethem D, Moats W, Lew M. Defects of pars interarticularis in athletes: a protocol for nonoperative treatment. J Spinal Disord. 1993;6(5):406-411.

13.  Kurd MF, Patel D, Norton R, Picetti G, Friel B, Vaccaro AR. Nonoperative treatment of symptomatic spondylolysis. J Spinal Disord Tech. 2007;20(8):560-564.

14.  Pizzutillo PD, Hummer CD 3rd. Nonoperative treatment for painful adolescent spondylolysis or spondylolisthesis. J Pediatr Orthop. 1989;9(5):538-540.

15.  Ivanic GM, Pink TP, Achatz W, Ward JC, Homann NC, May M. Direct stabilization of lumbar spondylolysis with a hook screw: mean 11-year follow-up period for 113 patients. Spine. 2003;28(3):255-259.

16.  Roca J, Iborra M, Cavanilles-Walker JM, Alberti G. Direct repair of spondylolysis using a new pedicle screw hook fixation: clinical and CT-assessed study: an analysis of 19 patients. J Spinal Disord Tech. 2005;18(suppl):S82-S89.

17.  Schlenzka D, Remes V, Helenius I, et al. Direct repair for treatment of symptomatic spondylolysis and low-grade isthmic spondylolisthesis in young patients: no benefit in comparison to segmental fusion after a mean follow-up of 14.8 years. Eur Spine J. 2006;15(10):1437-1447.

18.  Buck JE. Direct repair of the defect in spondylolisthesis. Preliminary report. J Bone Joint Surg Br. 1970;52(3):432-437.

19.  Nayeemuddin M, Richards PJ, Ahmed EB. The imaging and management of nonconsecutive pars interarticularis defects: a case report and review of literature. Spine J. 2011;11(12):1157-1163.

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The American Journal of Orthopedics - 44(12)
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american journal of orthopedics, AJO, online exclusive, case report and literature review, case report, pars, interarticularis defects, lumbar spondylolysis, adolescents, pediatrics, spine, fractures, fracture management, pars fracture, back, pain, imaging, elgafy, hart, tanios
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american journal of orthopedics, AJO, online exclusive, case report and literature review, case report, pars, interarticularis defects, lumbar spondylolysis, adolescents, pediatrics, spine, fractures, fracture management, pars fracture, back, pain, imaging, elgafy, hart, tanios
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