Imaging

LAS VEGAS – Management of blunt cerebrovascular injuries using 64-channel computed tomographic angiography screening coupled with digital subtraction angiography for a definitive diagnosis is safe and effective for identifying clinically significant injury and for maintaining a low stroke rate, according to a review of 228 cases.

The computed tomographic angiography (CTA) screening was positive in 189 patients (83%), and digital subtraction angiography (DSA) confirmed injury in 104 (55%) of those. The remaining 39 patients were found to have no injury on DSA, Dr. Charles P. Shahan of the University of Tennessee, Memphis reported at the annual meeting of the American Association for the Surgery of Trauma (AAST).

©windcatcher/Thinkstock.com

Stroke related to blunt cerebrovascular injury (BCVI) occurred in five patients (4.8%); three of those patients were symptomatic at the time of presentation, and two became symptomatic while on therapy for a known lesion. None of the patients who had a negative screening CTA, including three with injuries missed on CTA, had a stroke, Dr. Shahan said.

The current study follows a prior study reported at the 2013 AAST annual meeting that suggested that 64-channel multidetector CTA could be the primary screening tool for BCVI. The previously used 32-channel multidetector CTA was found to be inadequate, with a sensitivity of only 52%. Sensitivity increased to 68% with the 64-channel CTA, but the positive predictive value remained remarkably low at 36%, he said.

That study led to a change in the screening algorithm, replacing DSA with CTA for screening, and reserving DSA for definitive BCVI diagnosis following a positive CTA or unexplained neurologic findings, he explained, noting that the rationale was that most injuries missed were low-grade injuries less likely to result in further injury, and that with CTA alone, about two-thirds of patients would be treated unnecessarily because of the false-positive rate.

The purpose of the current study was to evaluate outcomes in the wake of the algorithm change and to assess the potential for missed, clinically significant BCVI.

Study subjects were patients who underwent DSA over an 18-month period after implementation of the algorithm change. Most (64%) were men with a mean age of 43 years and a mean injury severity score of 22 out of 75, indicating moderate or severe injury.

The stroke rate was statistically unchanged in the second study, compared with the first. The findings demonstrate the safety and efficacy of the current management algorithm for BCVI, as well as the value of using DSA to identify false-positive CTA findings. In fact, definitive diagnosis by DSA led to avoidance of potentially harmful anticoagulation in 45% of CTA-positive patients, with no increase in the incidence of strokes resulting from injuries missed by CTA, Dr. Shahan said.

“Considering there were 85 false-positive CTAs, and also considering that our average length of heparin time is approximately 7 days prior to reevaluation, we’ve extrapolated this to nearly 600 heparin infusion days that were avoided by confirmatory DSA testing,” he said, concluding that “CTA with 64-channel multidetector technology with experienced radiology staff in a high-volume center can be safe and effective for BCVI screening.”

He added, however, that false-positive rates with CTA continue to necessitate DSA confirmation to avoid overtreatment.

“We feel that CTA screening with DSA confirmation has allowed us to maintain an acceptably low stroke rate and prevented a tremendous amount of unnecessary anticoagulation in these patients,” he said.

Dr. Clay Cothren Burlew, who was an invited discussant for Dr. Shahan’s paper, applauded Dr. Shahan and his colleagues for “continuing to question the validity of CTA as our primary diagnostic modality for BCVI,” and said the findings made her “stop and think, should we all be doing confirmatory angiography? … Are CTAs actually overcalling 45% of the injuries that we identify?”

Dr. Burlew of the University of Colorado, Denver, questioned whether the high rate of false positives is a result of radiologists who “overcall” questionable findings knowing that a confirmatory angiogram will quickly follow.

“I think this evaluation should be a model for others. Each institution should critically review their individual rates and methods of BCVI diagnosis,” she said, adding that “for centers with a marked increase in the identification of BCVI following institution of CTA as their screening tool, consideration of confirmatory angiography is recommended due to the potential false-positive rate of up to 45%.”

However, confirmatory angiography may not be warranted at centers whose screen yields remain the same with no missed injuries, she said.

“All programs should evaluate their injuries, appropriateness of diagnosis, and impact of subsequent treatment. Only then will we have optimal outcomes,” she concluded.

Dr. Shahan and Dr. Burlew reported having no relevant financial disclosures.

[email protected]

LAS VEGAS – Management of blunt cerebrovascular injuries using 64-channel computed tomographic angiography screening coupled with digital subtraction angiography for a definitive diagnosis is safe and effective for identifying clinically significant injury and for maintaining a low stroke rate, according to a review of 228 cases.

The computed tomographic angiography (CTA) screening was positive in 189 patients (83%), and digital subtraction angiography (DSA) confirmed injury in 104 (55%) of those. The remaining 39 patients were found to have no injury on DSA, Dr. Charles P. Shahan of the University of Tennessee, Memphis reported at the annual meeting of the American Association for the Surgery of Trauma (AAST).

©windcatcher/Thinkstock.com

Stroke related to blunt cerebrovascular injury (BCVI) occurred in five patients (4.8%); three of those patients were symptomatic at the time of presentation, and two became symptomatic while on therapy for a known lesion. None of the patients who had a negative screening CTA, including three with injuries missed on CTA, had a stroke, Dr. Shahan said.

The current study follows a prior study reported at the 2013 AAST annual meeting that suggested that 64-channel multidetector CTA could be the primary screening tool for BCVI. The previously used 32-channel multidetector CTA was found to be inadequate, with a sensitivity of only 52%. Sensitivity increased to 68% with the 64-channel CTA, but the positive predictive value remained remarkably low at 36%, he said.

That study led to a change in the screening algorithm, replacing DSA with CTA for screening, and reserving DSA for definitive BCVI diagnosis following a positive CTA or unexplained neurologic findings, he explained, noting that the rationale was that most injuries missed were low-grade injuries less likely to result in further injury, and that with CTA alone, about two-thirds of patients would be treated unnecessarily because of the false-positive rate.

The purpose of the current study was to evaluate outcomes in the wake of the algorithm change and to assess the potential for missed, clinically significant BCVI.

Study subjects were patients who underwent DSA over an 18-month period after implementation of the algorithm change. Most (64%) were men with a mean age of 43 years and a mean injury severity score of 22 out of 75, indicating moderate or severe injury.

The stroke rate was statistically unchanged in the second study, compared with the first. The findings demonstrate the safety and efficacy of the current management algorithm for BCVI, as well as the value of using DSA to identify false-positive CTA findings. In fact, definitive diagnosis by DSA led to avoidance of potentially harmful anticoagulation in 45% of CTA-positive patients, with no increase in the incidence of strokes resulting from injuries missed by CTA, Dr. Shahan said.

“Considering there were 85 false-positive CTAs, and also considering that our average length of heparin time is approximately 7 days prior to reevaluation, we’ve extrapolated this to nearly 600 heparin infusion days that were avoided by confirmatory DSA testing,” he said, concluding that “CTA with 64-channel multidetector technology with experienced radiology staff in a high-volume center can be safe and effective for BCVI screening.”

He added, however, that false-positive rates with CTA continue to necessitate DSA confirmation to avoid overtreatment.

“We feel that CTA screening with DSA confirmation has allowed us to maintain an acceptably low stroke rate and prevented a tremendous amount of unnecessary anticoagulation in these patients,” he said.

Dr. Clay Cothren Burlew, who was an invited discussant for Dr. Shahan’s paper, applauded Dr. Shahan and his colleagues for “continuing to question the validity of CTA as our primary diagnostic modality for BCVI,” and said the findings made her “stop and think, should we all be doing confirmatory angiography? … Are CTAs actually overcalling 45% of the injuries that we identify?”

Dr. Burlew of the University of Colorado, Denver, questioned whether the high rate of false positives is a result of radiologists who “overcall” questionable findings knowing that a confirmatory angiogram will quickly follow.

“I think this evaluation should be a model for others. Each institution should critically review their individual rates and methods of BCVI diagnosis,” she said, adding that “for centers with a marked increase in the identification of BCVI following institution of CTA as their screening tool, consideration of confirmatory angiography is recommended due to the potential false-positive rate of up to 45%.”

However, confirmatory angiography may not be warranted at centers whose screen yields remain the same with no missed injuries, she said.

“All programs should evaluate their injuries, appropriateness of diagnosis, and impact of subsequent treatment. Only then will we have optimal outcomes,” she concluded.

Dr. Shahan and Dr. Burlew reported having no relevant financial disclosures.

[email protected]

LAS VEGAS – Management of blunt cerebrovascular injuries using 64-channel computed tomographic angiography screening coupled with digital subtraction angiography for a definitive diagnosis is safe and effective for identifying clinically significant injury and for maintaining a low stroke rate, according to a review of 228 cases.

The computed tomographic angiography (CTA) screening was positive in 189 patients (83%), and digital subtraction angiography (DSA) confirmed injury in 104 (55%) of those. The remaining 39 patients were found to have no injury on DSA, Dr. Charles P. Shahan of the University of Tennessee, Memphis reported at the annual meeting of the American Association for the Surgery of Trauma (AAST).

©windcatcher/Thinkstock.com

Stroke related to blunt cerebrovascular injury (BCVI) occurred in five patients (4.8%); three of those patients were symptomatic at the time of presentation, and two became symptomatic while on therapy for a known lesion. None of the patients who had a negative screening CTA, including three with injuries missed on CTA, had a stroke, Dr. Shahan said.

The current study follows a prior study reported at the 2013 AAST annual meeting that suggested that 64-channel multidetector CTA could be the primary screening tool for BCVI. The previously used 32-channel multidetector CTA was found to be inadequate, with a sensitivity of only 52%. Sensitivity increased to 68% with the 64-channel CTA, but the positive predictive value remained remarkably low at 36%, he said.

That study led to a change in the screening algorithm, replacing DSA with CTA for screening, and reserving DSA for definitive BCVI diagnosis following a positive CTA or unexplained neurologic findings, he explained, noting that the rationale was that most injuries missed were low-grade injuries less likely to result in further injury, and that with CTA alone, about two-thirds of patients would be treated unnecessarily because of the false-positive rate.

The purpose of the current study was to evaluate outcomes in the wake of the algorithm change and to assess the potential for missed, clinically significant BCVI.

Study subjects were patients who underwent DSA over an 18-month period after implementation of the algorithm change. Most (64%) were men with a mean age of 43 years and a mean injury severity score of 22 out of 75, indicating moderate or severe injury.

The stroke rate was statistically unchanged in the second study, compared with the first. The findings demonstrate the safety and efficacy of the current management algorithm for BCVI, as well as the value of using DSA to identify false-positive CTA findings. In fact, definitive diagnosis by DSA led to avoidance of potentially harmful anticoagulation in 45% of CTA-positive patients, with no increase in the incidence of strokes resulting from injuries missed by CTA, Dr. Shahan said.

“Considering there were 85 false-positive CTAs, and also considering that our average length of heparin time is approximately 7 days prior to reevaluation, we’ve extrapolated this to nearly 600 heparin infusion days that were avoided by confirmatory DSA testing,” he said, concluding that “CTA with 64-channel multidetector technology with experienced radiology staff in a high-volume center can be safe and effective for BCVI screening.”

He added, however, that false-positive rates with CTA continue to necessitate DSA confirmation to avoid overtreatment.

“We feel that CTA screening with DSA confirmation has allowed us to maintain an acceptably low stroke rate and prevented a tremendous amount of unnecessary anticoagulation in these patients,” he said.

Dr. Clay Cothren Burlew, who was an invited discussant for Dr. Shahan’s paper, applauded Dr. Shahan and his colleagues for “continuing to question the validity of CTA as our primary diagnostic modality for BCVI,” and said the findings made her “stop and think, should we all be doing confirmatory angiography? … Are CTAs actually overcalling 45% of the injuries that we identify?”

Dr. Burlew of the University of Colorado, Denver, questioned whether the high rate of false positives is a result of radiologists who “overcall” questionable findings knowing that a confirmatory angiogram will quickly follow.

“I think this evaluation should be a model for others. Each institution should critically review their individual rates and methods of BCVI diagnosis,” she said, adding that “for centers with a marked increase in the identification of BCVI following institution of CTA as their screening tool, consideration of confirmatory angiography is recommended due to the potential false-positive rate of up to 45%.”

However, confirmatory angiography may not be warranted at centers whose screen yields remain the same with no missed injuries, she said.

“All programs should evaluate their injuries, appropriateness of diagnosis, and impact of subsequent treatment. Only then will we have optimal outcomes,” she concluded.

Dr. Shahan and Dr. Burlew reported having no relevant financial disclosures.

[email protected]

A 63-year-old physician is referred for preoperative evaluation before arthroscopic repair of a torn medial meniscus. Her exercise tolerance was excellent before the knee injury, including running without cardiopulmonary symptoms. She is otherwise healthy except for hypertension that is well controlled on amlodipine. She has no known history of liver or kidney disease, bleeding disorder, recent illness, or complications with anesthesia. She inquires as to whether “routine blood testing” is needed before the procedure.

See related editorial

What laboratory studies, if any, should be ordered?

UNLIKELY TO BE OF BENEFIT

Preoperative laboratory testing is not necessary in this otherwise healthy, asymptomatic patient. In the absence of clinical indications, routine testing before elective, low-risk procedures often increases both the cost of care and the potential anxiety caused by abnormal results that provide no substantial benefit to the patient or the clinician.

Preoperative diagnostic tests should be ordered only to identify and optimize disorders that alter the likelihood of perioperative and postoperative adverse outcomes and to establish a baseline assessment. Yet clinicians often perceive that laboratory testing is required by their organization or by other providers.

A comprehensive history and physical examination are the cornerstones of the effective preoperative evaluation. Preferably, the history and examination should guide further testing rather than ordering a battery of standard tests for all patients. However, selective preoperative laboratory testing may be useful in certain situations, such as in patients undergoing high-risk procedures and those with known underlying conditions or factors that may affect operative management (Table 1).

Unfortunately, high-quality evidence for this selective approach is lacking. According to one observational study,¹ when laboratory testing is appropriate, it is reasonable to use test results already obtained and normal within the preceding 4 months unless the patient has had an interim change in health status.

Definitions of risk stratification (eg, urgency of surgical procedure, graded risk according to type of operation) and tools such as the Revised Cardiac Risk Index can be found in the 2014 American College of Cardiology/American Heart Association guidelines² and may be useful to distinguish healthy patients from those with significant comorbidities, as well as to distinguish low-risk, elective procedures from those that impart higher risk.

Professional societies and guidelines in many countries have criticized the habitual practice of extensive, nonselective laboratory testing.^3–6 Yet despite lack of evidence of benefit, routine preoperative testing is still often done. At an estimated cost of more than $18 billion in the United States annually,⁷ preoperative testing deserves attention, especially in this time of ballooning healthcare costs and increased focus on effective and efficient care.

EVIDENCE AND GUIDELINES

Numerous studies have established that routine laboratory testing rarely changes the preoperative management of the patient or improves surgical outcomes. Narr et al⁸ found that 160 (4%) of 3,782 patients who underwent ambulatory surgery had abnormal test results, and only 10 required treatment. In this study, there was no association between abnormal test results and perioperative management or postoperative adverse events.

In a systematic review, Smetana and Macpherson⁹ noted that the incidence of laboratory test abnormalities that led to a change in management ranged from 0.1% to 2.6%. Notably, clinicians ignore 30% to 60% of abnormal preoperative laboratory results, a practice that may create additional medicolegal risk.⁷

Most guidelines on preoperative testing are based on expert opinion, case series, or consensus

Little evidence exists that helps in the development of guidelines for preoperative laboratory testing. Most guidelines are based on expert opinion, case series, and consensus. As an example of the heterogeneity this creates, the American Society of Anesthesiologists, the Ontario Preoperative Testing Group, and the Canadian Anesthesiologists’ Society provide different recommended indications for preoperative laboratory testing in patients with “advanced age” but do not define a clear minimum age for this cohort.¹⁰

However, one area that does have substantial data is cataract surgery. Patients in their usual state of health who are to undergo this procedure do not require preoperative testing, a claim supported by high-quality evidence including a 2012 Cochrane systematic review.¹¹

Munro et al⁵ performed a systematic review of the evidence behind preoperative laboratory testing, concluding that the power of preoperative tests to predict adverse postoperative outcomes in asymptomatic patients is either weak or nonexistent. The National Institute for Health and Clinical Excellence guidelines of 2003,⁶ the Practice Advisory for Preanesthesia Evaluation of the American Society of Anesthesiologists of 2012,¹² the Institute for Clinical Systems Improvement guideline of 2012,¹³ and a systematic review conducted by Johansson et al¹⁴ found no evidence from high-quality studies to support the claim that routine preoperative testing is beneficial in healthy adults undergoing noncardiac surgery, but that certain patient populations may benefit from selective testing.

A randomized controlled trial evaluated the elimination of preoperative testing in patients undergoing low-risk ambulatory surgery and found no difference in perioperative adverse events in the control and intervention arms.¹⁵ Similar studies achieved the same results.

The Choosing Wisely campaign

The American Board of Internal Medicine Foundation has partnered with medical specialty societies to create lists of common practice patterns that should be questioned and possibly discontinued. These lists are collectively called the Choosing Wisely campaign (www.choosingwisely.org). Avoiding routine preoperative laboratory testing in patients undergoing low-risk surgery without clinical indications can be found in the lists for the American Society of Anesthesiologists, the American Society for Clinical Pathology, and the Society of General Internal Medicine.

THE POSSIBLE HARMS OF TESTING

The prevalence of unrecognized disease that influences the risk of surgery in healthy patients is low, and thus the predictive value of abnormal test values in these patients is low. This leads to substantial false-positivity, which is of uncertain clinical significance and which may in turn cause a cascade of further testing. Not surprisingly, the probability of an abnormal test result increases dramatically with the number of tests ordered, a fact that magnifies the problem of false-positive results.

The costs and harms associated with testing are both direct and indirect. Direct effects include increased healthcare costs of further testing or potentially unnecessary treatment as well as risk associated with additional testing, though these are not common, as there is a low (< 3%) incidence of a change in preoperative management based on an abnormal test result. Likewise, normal results do not appear to substantially reduce the likelihood of postoperative complications.⁹

Indirect effects, which are particularly challenging to measure, may include time lost from employment to pursue further evaluation and anxiety surrounding abnormal results.

THE CLINICAL BOTTOM LINE

Based on over 2 decades of data, our 63-year-old patient should not undergo “routine” preoperative laboratory testing before her upcoming elective, low-risk, noncardiac procedure. Her hypertension is well controlled, and she is taking no medications that may lead to clinically significant metabolic derangements or significant changes in surgical outcome. There are no convincing clinical indications for further laboratory investigation. Further, the results are unlikely to affect the preoperative management and rate of adverse events; the direct and indirect costs may be substantial; and there is a small but tangible risk of harm.

Given the myriad factors that influence unnecessary preoperative testing, a focus on systems-level solutions is paramount. Key steps may include creation and adoption of clear and consistent guidelines, development of clinical care pathways, physician education and modification of practice, interdisciplinary communication and information sharing, economic analysis, and outcomes assessment.

A 63-year-old physician is referred for preoperative evaluation before arthroscopic repair of a torn medial meniscus. Her exercise tolerance was excellent before the knee injury, including running without cardiopulmonary symptoms. She is otherwise healthy except for hypertension that is well controlled on amlodipine. She has no known history of liver or kidney disease, bleeding disorder, recent illness, or complications with anesthesia. She inquires as to whether “routine blood testing” is needed before the procedure.

See related editorial

What laboratory studies, if any, should be ordered?

UNLIKELY TO BE OF BENEFIT

Preoperative laboratory testing is not necessary in this otherwise healthy, asymptomatic patient. In the absence of clinical indications, routine testing before elective, low-risk procedures often increases both the cost of care and the potential anxiety caused by abnormal results that provide no substantial benefit to the patient or the clinician.

Preoperative diagnostic tests should be ordered only to identify and optimize disorders that alter the likelihood of perioperative and postoperative adverse outcomes and to establish a baseline assessment. Yet clinicians often perceive that laboratory testing is required by their organization or by other providers.

A comprehensive history and physical examination are the cornerstones of the effective preoperative evaluation. Preferably, the history and examination should guide further testing rather than ordering a battery of standard tests for all patients. However, selective preoperative laboratory testing may be useful in certain situations, such as in patients undergoing high-risk procedures and those with known underlying conditions or factors that may affect operative management (Table 1).

Unfortunately, high-quality evidence for this selective approach is lacking. According to one observational study,¹ when laboratory testing is appropriate, it is reasonable to use test results already obtained and normal within the preceding 4 months unless the patient has had an interim change in health status.

Definitions of risk stratification (eg, urgency of surgical procedure, graded risk according to type of operation) and tools such as the Revised Cardiac Risk Index can be found in the 2014 American College of Cardiology/American Heart Association guidelines² and may be useful to distinguish healthy patients from those with significant comorbidities, as well as to distinguish low-risk, elective procedures from those that impart higher risk.

Professional societies and guidelines in many countries have criticized the habitual practice of extensive, nonselective laboratory testing.^3–6 Yet despite lack of evidence of benefit, routine preoperative testing is still often done. At an estimated cost of more than $18 billion in the United States annually,⁷ preoperative testing deserves attention, especially in this time of ballooning healthcare costs and increased focus on effective and efficient care.

EVIDENCE AND GUIDELINES

Numerous studies have established that routine laboratory testing rarely changes the preoperative management of the patient or improves surgical outcomes. Narr et al⁸ found that 160 (4%) of 3,782 patients who underwent ambulatory surgery had abnormal test results, and only 10 required treatment. In this study, there was no association between abnormal test results and perioperative management or postoperative adverse events.

In a systematic review, Smetana and Macpherson⁹ noted that the incidence of laboratory test abnormalities that led to a change in management ranged from 0.1% to 2.6%. Notably, clinicians ignore 30% to 60% of abnormal preoperative laboratory results, a practice that may create additional medicolegal risk.⁷

Most guidelines on preoperative testing are based on expert opinion, case series, or consensus

Little evidence exists that helps in the development of guidelines for preoperative laboratory testing. Most guidelines are based on expert opinion, case series, and consensus. As an example of the heterogeneity this creates, the American Society of Anesthesiologists, the Ontario Preoperative Testing Group, and the Canadian Anesthesiologists’ Society provide different recommended indications for preoperative laboratory testing in patients with “advanced age” but do not define a clear minimum age for this cohort.¹⁰

However, one area that does have substantial data is cataract surgery. Patients in their usual state of health who are to undergo this procedure do not require preoperative testing, a claim supported by high-quality evidence including a 2012 Cochrane systematic review.¹¹

Munro et al⁵ performed a systematic review of the evidence behind preoperative laboratory testing, concluding that the power of preoperative tests to predict adverse postoperative outcomes in asymptomatic patients is either weak or nonexistent. The National Institute for Health and Clinical Excellence guidelines of 2003,⁶ the Practice Advisory for Preanesthesia Evaluation of the American Society of Anesthesiologists of 2012,¹² the Institute for Clinical Systems Improvement guideline of 2012,¹³ and a systematic review conducted by Johansson et al¹⁴ found no evidence from high-quality studies to support the claim that routine preoperative testing is beneficial in healthy adults undergoing noncardiac surgery, but that certain patient populations may benefit from selective testing.

A randomized controlled trial evaluated the elimination of preoperative testing in patients undergoing low-risk ambulatory surgery and found no difference in perioperative adverse events in the control and intervention arms.¹⁵ Similar studies achieved the same results.

The Choosing Wisely campaign

The American Board of Internal Medicine Foundation has partnered with medical specialty societies to create lists of common practice patterns that should be questioned and possibly discontinued. These lists are collectively called the Choosing Wisely campaign (www.choosingwisely.org). Avoiding routine preoperative laboratory testing in patients undergoing low-risk surgery without clinical indications can be found in the lists for the American Society of Anesthesiologists, the American Society for Clinical Pathology, and the Society of General Internal Medicine.

THE POSSIBLE HARMS OF TESTING

The prevalence of unrecognized disease that influences the risk of surgery in healthy patients is low, and thus the predictive value of abnormal test values in these patients is low. This leads to substantial false-positivity, which is of uncertain clinical significance and which may in turn cause a cascade of further testing. Not surprisingly, the probability of an abnormal test result increases dramatically with the number of tests ordered, a fact that magnifies the problem of false-positive results.

The costs and harms associated with testing are both direct and indirect. Direct effects include increased healthcare costs of further testing or potentially unnecessary treatment as well as risk associated with additional testing, though these are not common, as there is a low (< 3%) incidence of a change in preoperative management based on an abnormal test result. Likewise, normal results do not appear to substantially reduce the likelihood of postoperative complications.⁹

Indirect effects, which are particularly challenging to measure, may include time lost from employment to pursue further evaluation and anxiety surrounding abnormal results.

THE CLINICAL BOTTOM LINE

Based on over 2 decades of data, our 63-year-old patient should not undergo “routine” preoperative laboratory testing before her upcoming elective, low-risk, noncardiac procedure. Her hypertension is well controlled, and she is taking no medications that may lead to clinically significant metabolic derangements or significant changes in surgical outcome. There are no convincing clinical indications for further laboratory investigation. Further, the results are unlikely to affect the preoperative management and rate of adverse events; the direct and indirect costs may be substantial; and there is a small but tangible risk of harm.

Given the myriad factors that influence unnecessary preoperative testing, a focus on systems-level solutions is paramount. Key steps may include creation and adoption of clear and consistent guidelines, development of clinical care pathways, physician education and modification of practice, interdisciplinary communication and information sharing, economic analysis, and outcomes assessment.

A 63-year-old physician is referred for preoperative evaluation before arthroscopic repair of a torn medial meniscus. Her exercise tolerance was excellent before the knee injury, including running without cardiopulmonary symptoms. She is otherwise healthy except for hypertension that is well controlled on amlodipine. She has no known history of liver or kidney disease, bleeding disorder, recent illness, or complications with anesthesia. She inquires as to whether “routine blood testing” is needed before the procedure.

See related editorial

What laboratory studies, if any, should be ordered?

UNLIKELY TO BE OF BENEFIT

Preoperative laboratory testing is not necessary in this otherwise healthy, asymptomatic patient. In the absence of clinical indications, routine testing before elective, low-risk procedures often increases both the cost of care and the potential anxiety caused by abnormal results that provide no substantial benefit to the patient or the clinician.

Preoperative diagnostic tests should be ordered only to identify and optimize disorders that alter the likelihood of perioperative and postoperative adverse outcomes and to establish a baseline assessment. Yet clinicians often perceive that laboratory testing is required by their organization or by other providers.

A comprehensive history and physical examination are the cornerstones of the effective preoperative evaluation. Preferably, the history and examination should guide further testing rather than ordering a battery of standard tests for all patients. However, selective preoperative laboratory testing may be useful in certain situations, such as in patients undergoing high-risk procedures and those with known underlying conditions or factors that may affect operative management (Table 1).

Unfortunately, high-quality evidence for this selective approach is lacking. According to one observational study,¹ when laboratory testing is appropriate, it is reasonable to use test results already obtained and normal within the preceding 4 months unless the patient has had an interim change in health status.

Definitions of risk stratification (eg, urgency of surgical procedure, graded risk according to type of operation) and tools such as the Revised Cardiac Risk Index can be found in the 2014 American College of Cardiology/American Heart Association guidelines² and may be useful to distinguish healthy patients from those with significant comorbidities, as well as to distinguish low-risk, elective procedures from those that impart higher risk.

Professional societies and guidelines in many countries have criticized the habitual practice of extensive, nonselective laboratory testing.^3–6 Yet despite lack of evidence of benefit, routine preoperative testing is still often done. At an estimated cost of more than $18 billion in the United States annually,⁷ preoperative testing deserves attention, especially in this time of ballooning healthcare costs and increased focus on effective and efficient care.

EVIDENCE AND GUIDELINES

Numerous studies have established that routine laboratory testing rarely changes the preoperative management of the patient or improves surgical outcomes. Narr et al⁸ found that 160 (4%) of 3,782 patients who underwent ambulatory surgery had abnormal test results, and only 10 required treatment. In this study, there was no association between abnormal test results and perioperative management or postoperative adverse events.

In a systematic review, Smetana and Macpherson⁹ noted that the incidence of laboratory test abnormalities that led to a change in management ranged from 0.1% to 2.6%. Notably, clinicians ignore 30% to 60% of abnormal preoperative laboratory results, a practice that may create additional medicolegal risk.⁷

Most guidelines on preoperative testing are based on expert opinion, case series, or consensus

Little evidence exists that helps in the development of guidelines for preoperative laboratory testing. Most guidelines are based on expert opinion, case series, and consensus. As an example of the heterogeneity this creates, the American Society of Anesthesiologists, the Ontario Preoperative Testing Group, and the Canadian Anesthesiologists’ Society provide different recommended indications for preoperative laboratory testing in patients with “advanced age” but do not define a clear minimum age for this cohort.¹⁰

However, one area that does have substantial data is cataract surgery. Patients in their usual state of health who are to undergo this procedure do not require preoperative testing, a claim supported by high-quality evidence including a 2012 Cochrane systematic review.¹¹

Munro et al⁵ performed a systematic review of the evidence behind preoperative laboratory testing, concluding that the power of preoperative tests to predict adverse postoperative outcomes in asymptomatic patients is either weak or nonexistent. The National Institute for Health and Clinical Excellence guidelines of 2003,⁶ the Practice Advisory for Preanesthesia Evaluation of the American Society of Anesthesiologists of 2012,¹² the Institute for Clinical Systems Improvement guideline of 2012,¹³ and a systematic review conducted by Johansson et al¹⁴ found no evidence from high-quality studies to support the claim that routine preoperative testing is beneficial in healthy adults undergoing noncardiac surgery, but that certain patient populations may benefit from selective testing.

A randomized controlled trial evaluated the elimination of preoperative testing in patients undergoing low-risk ambulatory surgery and found no difference in perioperative adverse events in the control and intervention arms.¹⁵ Similar studies achieved the same results.

The Choosing Wisely campaign

The American Board of Internal Medicine Foundation has partnered with medical specialty societies to create lists of common practice patterns that should be questioned and possibly discontinued. These lists are collectively called the Choosing Wisely campaign (www.choosingwisely.org). Avoiding routine preoperative laboratory testing in patients undergoing low-risk surgery without clinical indications can be found in the lists for the American Society of Anesthesiologists, the American Society for Clinical Pathology, and the Society of General Internal Medicine.

THE POSSIBLE HARMS OF TESTING

The prevalence of unrecognized disease that influences the risk of surgery in healthy patients is low, and thus the predictive value of abnormal test values in these patients is low. This leads to substantial false-positivity, which is of uncertain clinical significance and which may in turn cause a cascade of further testing. Not surprisingly, the probability of an abnormal test result increases dramatically with the number of tests ordered, a fact that magnifies the problem of false-positive results.

The costs and harms associated with testing are both direct and indirect. Direct effects include increased healthcare costs of further testing or potentially unnecessary treatment as well as risk associated with additional testing, though these are not common, as there is a low (< 3%) incidence of a change in preoperative management based on an abnormal test result. Likewise, normal results do not appear to substantially reduce the likelihood of postoperative complications.⁹

Indirect effects, which are particularly challenging to measure, may include time lost from employment to pursue further evaluation and anxiety surrounding abnormal results.

THE CLINICAL BOTTOM LINE

Based on over 2 decades of data, our 63-year-old patient should not undergo “routine” preoperative laboratory testing before her upcoming elective, low-risk, noncardiac procedure. Her hypertension is well controlled, and she is taking no medications that may lead to clinically significant metabolic derangements or significant changes in surgical outcome. There are no convincing clinical indications for further laboratory investigation. Further, the results are unlikely to affect the preoperative management and rate of adverse events; the direct and indirect costs may be substantial; and there is a small but tangible risk of harm.

Given the myriad factors that influence unnecessary preoperative testing, a focus on systems-level solutions is paramount. Key steps may include creation and adoption of clear and consistent guidelines, development of clinical care pathways, physician education and modification of practice, interdisciplinary communication and information sharing, economic analysis, and outcomes assessment.

Guidelines and practice advisories issued by several medical societies, including the American Society of Anesthesiologists,¹ American Heart Association (AHA) and American College of Cardiology (ACC),² and Society of General Internal Medicine,³ advise against routine preoperative testing for patients undergoing low-risk surgical procedures. Such testing often includes routine blood chemistry, complete blood cell counts, measures of the clotting system, and cardiac stress testing.

See related article

In this issue of the Cleveland Clinic Journal of Medicine, Dr. Nathan Houchens reviews the evidence against these measures.⁴

Despite a substantial body of evidence going back more than 2 decades that includes prospective randomized controlled trials,^5–10 physicians continue to order unnecessary, ineffective, and costly tests in the perioperative period.¹¹ The process of abandoning current medical practice—a phenomenon known as medical reversal¹²—often takes years,¹³ because it is more difficult to convince physicians to discontinue a current behavior than to implement a new one.¹⁴ The study of what makes physicians accept new therapies and abandon old ones began more than half a century ago.¹⁵

More recently, Cabana et al¹⁶ created a framework to understand why physicians do not follow clinical practice guidelines. Among the reasons are lack of familiarity or agreement with the contents of the guideline, lack of outcome expectancy, inertia of previous practice, and external barriers to implementation.

It is harder to convince physicians to discontinue a current behavior than to implement a new one

The rapid proliferation of guidelines in the past 20 years has led to numerous conflicting recommendations, many of which are based primarily on expert opinion.¹⁷ Guidelines based solely on randomized trials have also come under fire.^18,19

In the case of preoperative testing, the recommendations are generally evidence-based and consistent. Why then do physicians appear to disregard the evidence? We propose several reasons why they might do so.

SOME PHYSICIANS ARE UNFAMILIAR WITH THE EVIDENCE

The complexity of the evidence summarized in guidelines has increased exponentially in the last decade, but physician time to assess the evidence has not increased. For example, the number of references in the executive summary of the ACC/AHA perioperative guidelines increased from 96 in 2002 to 252 in 2014. Most of the recommendations are backed by substantial amounts of high-quality evidence. For example, there are 17 prospective and 13 retrospective studies demonstrating that routine testing with the prothrombin time and the partial thromboplastin time is not helpful in asymptomatic patients.²⁰

Although compliance with medical evidence varies among specialties,²¹ most physicians do not have time to keep up with the ever-increasing amount of information. Specifically in the area of cardiac risk assessment, there has been a rapid proliferation of tests that can be used to assess cardiac risk.^22–28 In a Harris Interactive survey from 2008, physicians reported not applying medical evidence routinely. One-third believed they would do it more if they had the time.²⁹ Without information technology support to provide medical information at the point of care,³⁰ especially in small practices, using evidence may not be practical. Simply making the information available online and not promoting it actively does not improve utilization.³¹

As a consequence, physicians continue to order unnecessary tests, even though they may not feel confident interpreting the results.³²

PHYSICIANS MAY NOT BELIEVE THE EVIDENCE

A lack of transparency in evidence-based guidelines and, sometimes, a lack of flexibility and relevance to clinical practice are important barriers to physicians’ acceptance of and adherence to evidence-based clinical practice guidelines.³⁰

Most physicians do not have time to keep up with the ever-increasing amount of information

Even experts who write guidelines may not be swayed by the evidence. For example, a randomized prospective trial of almost 6,000 patients reported that coronary artery revascularization before elective major vascular surgery does not affect long-term mortality rates.³³ Based on this study, the 2014 ACC/AHA guidelines² advised against revascularization before noncardiac surgery exclusively to reduce perioperative cardiac events. Yet the same guidelines do recommend assessing for myocardial ischemia in patients with elevated risk and poor or unknown functional capacity, using a pharmacologic stress test. Based on the extent of the stress test abnormalities, coronary angiography and revascularization are then suggested for patients willing to undergo coronary artery bypass grafting (CABG) or percutaneous coronary intervention.²

The 2014 European Society of Cardiology and European Society of Anaesthesiology guidelines directly recommend revascularization before high-risk surgery, depending on the extent of a stress-induced perfusion defect.³⁴ This recommendation relies on data from the Coronary Artery Surgery Study registry, which included almost 25,000 patients who underwent coronary angiography from 1975 through 1979. At a mean follow-up of 4.1 years, 1,961 patients underwent high-risk surgery. In this observational cohort, patients who underwent CABG had a lower risk of death and myocardial infarction after surgery.³⁵ The reliance of medical societies³⁴ on data that are more than 30 years old—when operative mortality rates and the treatment of coronary artery disease have changed substantially in the interim and despite the fact that this study did not test whether preoperative revascularization can reduce postoperative mortality—reflects a certain resistance to accept the results of the more recent and relevant randomized trial.³³

Other physicians may also prefer to rely on selective data or to simply defer to guidelines that support their beliefs. Some physicians find that evidence-based guidelines are impractical and rigid and reduce their autonomy.³⁶ For many physicians, trials that use surrogate end points and short-term outcomes are not sufficiently compelling to make them abandon current practice.³⁷ Finally, when members of the guideline committees have financial associations with the pharmaceutical industry, or when corporations interested in the outcomes provide financial support for a trial’s development, the likelihood of a recommendation being trusted and used by physicians is drastically reduced.³⁸

PRACTICING DEFENSIVELY

Even if physicians are familiar with the evidence and believe it, they may choose not to act on it. One reason is fear of litigation.

In court, attorneys can use guidelines as well as articles from medical journals as both exculpatory and inculpatory evidence. But they more frequently rely on the standard of care, or what most physicians would do under similar circumstances. If a patient has a bad outcome, such as a perioperative myocardial infarction or life-threatening bleeding, the defendant may assert that testing was unwarranted because guidelines do not recommend it or because the probability of such an outcome was low. However, because the outcome occurred, the jury may not believe that the probability was low enough not to consider, especially if expert witnesses testify that the standard of care would be to order the test.

In areas of controversy, physicians generally believe that erring on the side of more testing is more defensible in court.³⁹ Indeed, following established practice traditions, learned during residency,^11,40 may absolve physicians in negligence claims if the way medical care was delivered is supported by recognized and respected physicians.⁴¹

Even physicians who write the guidelines may be unswayed by the evidence

As a consequence, physicians prefer to practice the same way their peers do rather than follow the evidence. Unfortunately, the more procedures physicians perform for low-risk patients, the more likely these tests will become accepted as the legal standard of care.⁴² In this vicious circle, the new standard of care can increase the risk of litigation for others.⁴³ Although unnecessary testing that leads to harmful invasive tests or procedures can also result in malpractice litigation, physicians may not consider this possibility.

FINANCIAL INCENTIVES

The threat of malpractice litigation provides a negative financial incentive to keep performing unnecessary tests, but there are a number of positive incentives as well.

First, physicians often feel compelled to order tests when they believe that physicians referring the patients want the tests done, or when they fear that not completing the tests could delay or cancel the scheduled surgery.⁴⁰ Refusing to order the test could result in a loss of future referrals. In contrast, ordering tests allows them to meet expectations, preserve trust, and appear more valuable to referring physicians and their patients.

Insurance companies are complicit in these practices. Paying for unnecessary tests can create direct financial incentives for physicians or institutions that own on-site laboratories or diagnostic imaging equipment. Evidence shows that under those circumstances physicians do order more tests. Self-referral and referral to facilities where physicians have a financial interest is associated with increased healthcare costs.⁴⁴ In addition to direct revenues for the tests performed, physicians may also bill for test interpretation, follow-up visits, and additional procedures generated from test results.

This may be one explanation why the ordering of cardiac tests (stress testing, echocardiography, vascular ultrasonography) by US physicians varies widely from state to state.⁴⁵

RECOMMENDATIONS TO REDUCE INAPPROPRIATE TESTING

To counter these influences, we propose a multifaceted intervention that includes the following:

• Establish preoperative clinics staffed by experts. Despite the large volume of potentially relevant evidence, the number of articles directly supporting or refuting preoperative laboratory testing is small enough that physicians who routinely engage in preoperative assessment should easily master the evidence.
• Identify local leaders who can convince colleagues of the evidence. Distribute evidence summaries or guidelines with references to major articles that support each recommendation.
• Work with clinical practice committees to establish new standards of care within the hospital. Establish hospital care paths to dictate and support local standards of care. Measure individual physician performance and offer feedback with the goal of reducing utilization.
• National societies should recommend that insurance companies remove inappropriate financial incentives. If companies deny payment for inappropriate testing, physicians will stop ordering it. Even requirements for preauthorization of tests should reduce utilization. The Choosing Wisely campaign (www.choosingwisely.org) would be a good place to start.

Guidelines and practice advisories issued by several medical societies, including the American Society of Anesthesiologists,¹ American Heart Association (AHA) and American College of Cardiology (ACC),² and Society of General Internal Medicine,³ advise against routine preoperative testing for patients undergoing low-risk surgical procedures. Such testing often includes routine blood chemistry, complete blood cell counts, measures of the clotting system, and cardiac stress testing.

See related article

In this issue of the Cleveland Clinic Journal of Medicine, Dr. Nathan Houchens reviews the evidence against these measures.⁴

Despite a substantial body of evidence going back more than 2 decades that includes prospective randomized controlled trials,^5–10 physicians continue to order unnecessary, ineffective, and costly tests in the perioperative period.¹¹ The process of abandoning current medical practice—a phenomenon known as medical reversal¹²—often takes years,¹³ because it is more difficult to convince physicians to discontinue a current behavior than to implement a new one.¹⁴ The study of what makes physicians accept new therapies and abandon old ones began more than half a century ago.¹⁵

More recently, Cabana et al¹⁶ created a framework to understand why physicians do not follow clinical practice guidelines. Among the reasons are lack of familiarity or agreement with the contents of the guideline, lack of outcome expectancy, inertia of previous practice, and external barriers to implementation.

It is harder to convince physicians to discontinue a current behavior than to implement a new one

The rapid proliferation of guidelines in the past 20 years has led to numerous conflicting recommendations, many of which are based primarily on expert opinion.¹⁷ Guidelines based solely on randomized trials have also come under fire.^18,19

In the case of preoperative testing, the recommendations are generally evidence-based and consistent. Why then do physicians appear to disregard the evidence? We propose several reasons why they might do so.

SOME PHYSICIANS ARE UNFAMILIAR WITH THE EVIDENCE

The complexity of the evidence summarized in guidelines has increased exponentially in the last decade, but physician time to assess the evidence has not increased. For example, the number of references in the executive summary of the ACC/AHA perioperative guidelines increased from 96 in 2002 to 252 in 2014. Most of the recommendations are backed by substantial amounts of high-quality evidence. For example, there are 17 prospective and 13 retrospective studies demonstrating that routine testing with the prothrombin time and the partial thromboplastin time is not helpful in asymptomatic patients.²⁰

Although compliance with medical evidence varies among specialties,²¹ most physicians do not have time to keep up with the ever-increasing amount of information. Specifically in the area of cardiac risk assessment, there has been a rapid proliferation of tests that can be used to assess cardiac risk.^22–28 In a Harris Interactive survey from 2008, physicians reported not applying medical evidence routinely. One-third believed they would do it more if they had the time.²⁹ Without information technology support to provide medical information at the point of care,³⁰ especially in small practices, using evidence may not be practical. Simply making the information available online and not promoting it actively does not improve utilization.³¹

As a consequence, physicians continue to order unnecessary tests, even though they may not feel confident interpreting the results.³²

PHYSICIANS MAY NOT BELIEVE THE EVIDENCE

A lack of transparency in evidence-based guidelines and, sometimes, a lack of flexibility and relevance to clinical practice are important barriers to physicians’ acceptance of and adherence to evidence-based clinical practice guidelines.³⁰

Most physicians do not have time to keep up with the ever-increasing amount of information

Even experts who write guidelines may not be swayed by the evidence. For example, a randomized prospective trial of almost 6,000 patients reported that coronary artery revascularization before elective major vascular surgery does not affect long-term mortality rates.³³ Based on this study, the 2014 ACC/AHA guidelines² advised against revascularization before noncardiac surgery exclusively to reduce perioperative cardiac events. Yet the same guidelines do recommend assessing for myocardial ischemia in patients with elevated risk and poor or unknown functional capacity, using a pharmacologic stress test. Based on the extent of the stress test abnormalities, coronary angiography and revascularization are then suggested for patients willing to undergo coronary artery bypass grafting (CABG) or percutaneous coronary intervention.²

The 2014 European Society of Cardiology and European Society of Anaesthesiology guidelines directly recommend revascularization before high-risk surgery, depending on the extent of a stress-induced perfusion defect.³⁴ This recommendation relies on data from the Coronary Artery Surgery Study registry, which included almost 25,000 patients who underwent coronary angiography from 1975 through 1979. At a mean follow-up of 4.1 years, 1,961 patients underwent high-risk surgery. In this observational cohort, patients who underwent CABG had a lower risk of death and myocardial infarction after surgery.³⁵ The reliance of medical societies³⁴ on data that are more than 30 years old—when operative mortality rates and the treatment of coronary artery disease have changed substantially in the interim and despite the fact that this study did not test whether preoperative revascularization can reduce postoperative mortality—reflects a certain resistance to accept the results of the more recent and relevant randomized trial.³³

Other physicians may also prefer to rely on selective data or to simply defer to guidelines that support their beliefs. Some physicians find that evidence-based guidelines are impractical and rigid and reduce their autonomy.³⁶ For many physicians, trials that use surrogate end points and short-term outcomes are not sufficiently compelling to make them abandon current practice.³⁷ Finally, when members of the guideline committees have financial associations with the pharmaceutical industry, or when corporations interested in the outcomes provide financial support for a trial’s development, the likelihood of a recommendation being trusted and used by physicians is drastically reduced.³⁸

PRACTICING DEFENSIVELY

Even if physicians are familiar with the evidence and believe it, they may choose not to act on it. One reason is fear of litigation.

In court, attorneys can use guidelines as well as articles from medical journals as both exculpatory and inculpatory evidence. But they more frequently rely on the standard of care, or what most physicians would do under similar circumstances. If a patient has a bad outcome, such as a perioperative myocardial infarction or life-threatening bleeding, the defendant may assert that testing was unwarranted because guidelines do not recommend it or because the probability of such an outcome was low. However, because the outcome occurred, the jury may not believe that the probability was low enough not to consider, especially if expert witnesses testify that the standard of care would be to order the test.

In areas of controversy, physicians generally believe that erring on the side of more testing is more defensible in court.³⁹ Indeed, following established practice traditions, learned during residency,^11,40 may absolve physicians in negligence claims if the way medical care was delivered is supported by recognized and respected physicians.⁴¹

Even physicians who write the guidelines may be unswayed by the evidence

As a consequence, physicians prefer to practice the same way their peers do rather than follow the evidence. Unfortunately, the more procedures physicians perform for low-risk patients, the more likely these tests will become accepted as the legal standard of care.⁴² In this vicious circle, the new standard of care can increase the risk of litigation for others.⁴³ Although unnecessary testing that leads to harmful invasive tests or procedures can also result in malpractice litigation, physicians may not consider this possibility.

FINANCIAL INCENTIVES

The threat of malpractice litigation provides a negative financial incentive to keep performing unnecessary tests, but there are a number of positive incentives as well.

First, physicians often feel compelled to order tests when they believe that physicians referring the patients want the tests done, or when they fear that not completing the tests could delay or cancel the scheduled surgery.⁴⁰ Refusing to order the test could result in a loss of future referrals. In contrast, ordering tests allows them to meet expectations, preserve trust, and appear more valuable to referring physicians and their patients.

Insurance companies are complicit in these practices. Paying for unnecessary tests can create direct financial incentives for physicians or institutions that own on-site laboratories or diagnostic imaging equipment. Evidence shows that under those circumstances physicians do order more tests. Self-referral and referral to facilities where physicians have a financial interest is associated with increased healthcare costs.⁴⁴ In addition to direct revenues for the tests performed, physicians may also bill for test interpretation, follow-up visits, and additional procedures generated from test results.

This may be one explanation why the ordering of cardiac tests (stress testing, echocardiography, vascular ultrasonography) by US physicians varies widely from state to state.⁴⁵

RECOMMENDATIONS TO REDUCE INAPPROPRIATE TESTING

To counter these influences, we propose a multifaceted intervention that includes the following:

• Establish preoperative clinics staffed by experts. Despite the large volume of potentially relevant evidence, the number of articles directly supporting or refuting preoperative laboratory testing is small enough that physicians who routinely engage in preoperative assessment should easily master the evidence.
• Identify local leaders who can convince colleagues of the evidence. Distribute evidence summaries or guidelines with references to major articles that support each recommendation.
• Work with clinical practice committees to establish new standards of care within the hospital. Establish hospital care paths to dictate and support local standards of care. Measure individual physician performance and offer feedback with the goal of reducing utilization.
• National societies should recommend that insurance companies remove inappropriate financial incentives. If companies deny payment for inappropriate testing, physicians will stop ordering it. Even requirements for preauthorization of tests should reduce utilization. The Choosing Wisely campaign (www.choosingwisely.org) would be a good place to start.

Guidelines and practice advisories issued by several medical societies, including the American Society of Anesthesiologists,¹ American Heart Association (AHA) and American College of Cardiology (ACC),² and Society of General Internal Medicine,³ advise against routine preoperative testing for patients undergoing low-risk surgical procedures. Such testing often includes routine blood chemistry, complete blood cell counts, measures of the clotting system, and cardiac stress testing.

See related article

In this issue of the Cleveland Clinic Journal of Medicine, Dr. Nathan Houchens reviews the evidence against these measures.⁴

Despite a substantial body of evidence going back more than 2 decades that includes prospective randomized controlled trials,^5–10 physicians continue to order unnecessary, ineffective, and costly tests in the perioperative period.¹¹ The process of abandoning current medical practice—a phenomenon known as medical reversal¹²—often takes years,¹³ because it is more difficult to convince physicians to discontinue a current behavior than to implement a new one.¹⁴ The study of what makes physicians accept new therapies and abandon old ones began more than half a century ago.¹⁵

More recently, Cabana et al¹⁶ created a framework to understand why physicians do not follow clinical practice guidelines. Among the reasons are lack of familiarity or agreement with the contents of the guideline, lack of outcome expectancy, inertia of previous practice, and external barriers to implementation.

It is harder to convince physicians to discontinue a current behavior than to implement a new one

The rapid proliferation of guidelines in the past 20 years has led to numerous conflicting recommendations, many of which are based primarily on expert opinion.¹⁷ Guidelines based solely on randomized trials have also come under fire.^18,19

In the case of preoperative testing, the recommendations are generally evidence-based and consistent. Why then do physicians appear to disregard the evidence? We propose several reasons why they might do so.

SOME PHYSICIANS ARE UNFAMILIAR WITH THE EVIDENCE

The complexity of the evidence summarized in guidelines has increased exponentially in the last decade, but physician time to assess the evidence has not increased. For example, the number of references in the executive summary of the ACC/AHA perioperative guidelines increased from 96 in 2002 to 252 in 2014. Most of the recommendations are backed by substantial amounts of high-quality evidence. For example, there are 17 prospective and 13 retrospective studies demonstrating that routine testing with the prothrombin time and the partial thromboplastin time is not helpful in asymptomatic patients.²⁰

Although compliance with medical evidence varies among specialties,²¹ most physicians do not have time to keep up with the ever-increasing amount of information. Specifically in the area of cardiac risk assessment, there has been a rapid proliferation of tests that can be used to assess cardiac risk.^22–28 In a Harris Interactive survey from 2008, physicians reported not applying medical evidence routinely. One-third believed they would do it more if they had the time.²⁹ Without information technology support to provide medical information at the point of care,³⁰ especially in small practices, using evidence may not be practical. Simply making the information available online and not promoting it actively does not improve utilization.³¹

As a consequence, physicians continue to order unnecessary tests, even though they may not feel confident interpreting the results.³²

PHYSICIANS MAY NOT BELIEVE THE EVIDENCE

A lack of transparency in evidence-based guidelines and, sometimes, a lack of flexibility and relevance to clinical practice are important barriers to physicians’ acceptance of and adherence to evidence-based clinical practice guidelines.³⁰

Most physicians do not have time to keep up with the ever-increasing amount of information

Even experts who write guidelines may not be swayed by the evidence. For example, a randomized prospective trial of almost 6,000 patients reported that coronary artery revascularization before elective major vascular surgery does not affect long-term mortality rates.³³ Based on this study, the 2014 ACC/AHA guidelines² advised against revascularization before noncardiac surgery exclusively to reduce perioperative cardiac events. Yet the same guidelines do recommend assessing for myocardial ischemia in patients with elevated risk and poor or unknown functional capacity, using a pharmacologic stress test. Based on the extent of the stress test abnormalities, coronary angiography and revascularization are then suggested for patients willing to undergo coronary artery bypass grafting (CABG) or percutaneous coronary intervention.²

The 2014 European Society of Cardiology and European Society of Anaesthesiology guidelines directly recommend revascularization before high-risk surgery, depending on the extent of a stress-induced perfusion defect.³⁴ This recommendation relies on data from the Coronary Artery Surgery Study registry, which included almost 25,000 patients who underwent coronary angiography from 1975 through 1979. At a mean follow-up of 4.1 years, 1,961 patients underwent high-risk surgery. In this observational cohort, patients who underwent CABG had a lower risk of death and myocardial infarction after surgery.³⁵ The reliance of medical societies³⁴ on data that are more than 30 years old—when operative mortality rates and the treatment of coronary artery disease have changed substantially in the interim and despite the fact that this study did not test whether preoperative revascularization can reduce postoperative mortality—reflects a certain resistance to accept the results of the more recent and relevant randomized trial.³³

Other physicians may also prefer to rely on selective data or to simply defer to guidelines that support their beliefs. Some physicians find that evidence-based guidelines are impractical and rigid and reduce their autonomy.³⁶ For many physicians, trials that use surrogate end points and short-term outcomes are not sufficiently compelling to make them abandon current practice.³⁷ Finally, when members of the guideline committees have financial associations with the pharmaceutical industry, or when corporations interested in the outcomes provide financial support for a trial’s development, the likelihood of a recommendation being trusted and used by physicians is drastically reduced.³⁸

PRACTICING DEFENSIVELY

Even if physicians are familiar with the evidence and believe it, they may choose not to act on it. One reason is fear of litigation.

In court, attorneys can use guidelines as well as articles from medical journals as both exculpatory and inculpatory evidence. But they more frequently rely on the standard of care, or what most physicians would do under similar circumstances. If a patient has a bad outcome, such as a perioperative myocardial infarction or life-threatening bleeding, the defendant may assert that testing was unwarranted because guidelines do not recommend it or because the probability of such an outcome was low. However, because the outcome occurred, the jury may not believe that the probability was low enough not to consider, especially if expert witnesses testify that the standard of care would be to order the test.

In areas of controversy, physicians generally believe that erring on the side of more testing is more defensible in court.³⁹ Indeed, following established practice traditions, learned during residency,^11,40 may absolve physicians in negligence claims if the way medical care was delivered is supported by recognized and respected physicians.⁴¹

Even physicians who write the guidelines may be unswayed by the evidence

As a consequence, physicians prefer to practice the same way their peers do rather than follow the evidence. Unfortunately, the more procedures physicians perform for low-risk patients, the more likely these tests will become accepted as the legal standard of care.⁴² In this vicious circle, the new standard of care can increase the risk of litigation for others.⁴³ Although unnecessary testing that leads to harmful invasive tests or procedures can also result in malpractice litigation, physicians may not consider this possibility.

FINANCIAL INCENTIVES

The threat of malpractice litigation provides a negative financial incentive to keep performing unnecessary tests, but there are a number of positive incentives as well.

First, physicians often feel compelled to order tests when they believe that physicians referring the patients want the tests done, or when they fear that not completing the tests could delay or cancel the scheduled surgery.⁴⁰ Refusing to order the test could result in a loss of future referrals. In contrast, ordering tests allows them to meet expectations, preserve trust, and appear more valuable to referring physicians and their patients.

Insurance companies are complicit in these practices. Paying for unnecessary tests can create direct financial incentives for physicians or institutions that own on-site laboratories or diagnostic imaging equipment. Evidence shows that under those circumstances physicians do order more tests. Self-referral and referral to facilities where physicians have a financial interest is associated with increased healthcare costs.⁴⁴ In addition to direct revenues for the tests performed, physicians may also bill for test interpretation, follow-up visits, and additional procedures generated from test results.

This may be one explanation why the ordering of cardiac tests (stress testing, echocardiography, vascular ultrasonography) by US physicians varies widely from state to state.⁴⁵

RECOMMENDATIONS TO REDUCE INAPPROPRIATE TESTING

To counter these influences, we propose a multifaceted intervention that includes the following:

• Establish preoperative clinics staffed by experts. Despite the large volume of potentially relevant evidence, the number of articles directly supporting or refuting preoperative laboratory testing is small enough that physicians who routinely engage in preoperative assessment should easily master the evidence.
• Identify local leaders who can convince colleagues of the evidence. Distribute evidence summaries or guidelines with references to major articles that support each recommendation.
• Work with clinical practice committees to establish new standards of care within the hospital. Establish hospital care paths to dictate and support local standards of care. Measure individual physician performance and offer feedback with the goal of reducing utilization.
• National societies should recommend that insurance companies remove inappropriate financial incentives. If companies deny payment for inappropriate testing, physicians will stop ordering it. Even requirements for preauthorization of tests should reduce utilization. The Choosing Wisely campaign (www.choosingwisely.org) would be a good place to start.

A 43-year-old man with no medical history presented with pain and swelling in his left arm for 2 weeks. He was a regular weight lifter, and his exercise routine included repetitive hyperextension and hyperabduction of his arms while lifting heavy weights.

He had no history of recent trauma or venous cannulation of the left arm. His family history was negative for thrombophilic disorders. Physical examination revealed a swollen and erythematous left arm and visible venous collaterals at the neck, shoulder, and chest. There was no evidence of arterial insufficiency.

Duplex ultrasonography confirmed thrombosis of the left brachial, axillary, and subclavian veins. Further evaluation with computed tomography showed no intrathoracic mass but revealed several subsegmental pulmonary thrombi in the right lung. A screen for thrombophilia was negative. Venography confirmed complete thrombotic occlusion of the subclavian, axillary, and brachial veins (Figure 1).

Catheter-directed thrombolysis with tissue plasminogen activator resulted in complete resolution of the thrombosis, but venography after 3 days of thrombolysis showed 50% residual stenosis of the left subclavian vein where it passes under the first rib (Figure 2). The redness and swelling had markedly improved 2 days after thrombolytic therapy. He was discharged home on rivaroxaban 20 mg daily.

Follow-up venography 2 months later (Figure 3), with the patient performing hyperabduction of the arms, showed a patent subclavian vein with no thrombosis, but dynamic compression and occlusion of the subclavian vein where it passes the first rib. Magnetic resonance imaging (MRI) of the neck showed no cervical (ie, extra) rib and no soft-tissue abnormalities of the scalene triangle.

Following this, the patient underwent resection of the left first rib for decompression of the venous thoracic outlet, which resulted in resolution of his symptoms. He remained asymptomatic at 6-month follow-up.

PAGET-SCHROETTER SYNDROME

Paget-Schroetter syndrome, also referred to as effort-induced or effort thrombosis, is thrombosis of the axillary or subclavian vein associated with strenuous and repetitive activity of the arms. Anatomic abnormalities at the thoracic outlet—cervical rib, congenital bands, hypertrophy of scalene tendons, abnormal insertion of the costoclavicular ligament—and repetitive trauma to the endothelium of the subclavian vein are key factors in its initiation and progression.

The condition is seen primarily in young people who participate in strenuous activities such as rowing, weight lifting, and baseball pitching. It is estimated to be the cause of 40% of cases of primary upper-extremity deep vein thrombosis in the absence of an obvious risk factor or trigger such as a central venous catheter, pacemaker, port, or occult malignancy.¹

A provocative test such as the Adson test or hyperabduction test during MRI or venography helps confirm thoracic outlet obstruction by demonstrating dynamic obstruction.²

TREATMENT CONSIDERATIONS

There are no universal guidelines for the treatment of Paget-Schroetter syndrome. However, the available data^3–5 suggest a multimodal approach that involves early catheter-directed thrombolysis and subsequent surgical decompression of the thoracic outlet. This can restore venous patency and reduce the risk of long-term complications such as rethrombosis and postthrombotic syndrome.^3–5

Surgical treatment includes resection of the first rib and division of the scalene muscles and the costoclavicular ligament. MRI with provocative testing helps guide the surgical approach. Anticoagulation therapy alone—ie, without thrombolysis and surgical decompression—is inadequate as it leads to recurrence of thrombosis and residual symptoms.⁶

Paget-Schroetter syndrome should not be managed the same as lower-extremity deep vein thrombosis because the cause and the exacerbating factors are different.

Unanswered questions

Because we have no data from randomized controlled trials, questions about management remain. What should be the duration of anticoagulation, especially in the absence of coexisting thrombophilia? Is thrombophilia screening useful? What is the optimal timing for starting thrombolytic therapy?

A careful history and heightened suspicion are required to make this diagnosis. If undiagnosed, it carries a risk of significant long-term morbidity and death. Dynamic obstruction during venography, in addition to MRI, can help identify an anatomic obstruction.

A 43-year-old man with no medical history presented with pain and swelling in his left arm for 2 weeks. He was a regular weight lifter, and his exercise routine included repetitive hyperextension and hyperabduction of his arms while lifting heavy weights.

He had no history of recent trauma or venous cannulation of the left arm. His family history was negative for thrombophilic disorders. Physical examination revealed a swollen and erythematous left arm and visible venous collaterals at the neck, shoulder, and chest. There was no evidence of arterial insufficiency.

Duplex ultrasonography confirmed thrombosis of the left brachial, axillary, and subclavian veins. Further evaluation with computed tomography showed no intrathoracic mass but revealed several subsegmental pulmonary thrombi in the right lung. A screen for thrombophilia was negative. Venography confirmed complete thrombotic occlusion of the subclavian, axillary, and brachial veins (Figure 1).

Catheter-directed thrombolysis with tissue plasminogen activator resulted in complete resolution of the thrombosis, but venography after 3 days of thrombolysis showed 50% residual stenosis of the left subclavian vein where it passes under the first rib (Figure 2). The redness and swelling had markedly improved 2 days after thrombolytic therapy. He was discharged home on rivaroxaban 20 mg daily.

Follow-up venography 2 months later (Figure 3), with the patient performing hyperabduction of the arms, showed a patent subclavian vein with no thrombosis, but dynamic compression and occlusion of the subclavian vein where it passes the first rib. Magnetic resonance imaging (MRI) of the neck showed no cervical (ie, extra) rib and no soft-tissue abnormalities of the scalene triangle.

Following this, the patient underwent resection of the left first rib for decompression of the venous thoracic outlet, which resulted in resolution of his symptoms. He remained asymptomatic at 6-month follow-up.

PAGET-SCHROETTER SYNDROME

Paget-Schroetter syndrome, also referred to as effort-induced or effort thrombosis, is thrombosis of the axillary or subclavian vein associated with strenuous and repetitive activity of the arms. Anatomic abnormalities at the thoracic outlet—cervical rib, congenital bands, hypertrophy of scalene tendons, abnormal insertion of the costoclavicular ligament—and repetitive trauma to the endothelium of the subclavian vein are key factors in its initiation and progression.

The condition is seen primarily in young people who participate in strenuous activities such as rowing, weight lifting, and baseball pitching. It is estimated to be the cause of 40% of cases of primary upper-extremity deep vein thrombosis in the absence of an obvious risk factor or trigger such as a central venous catheter, pacemaker, port, or occult malignancy.¹

A provocative test such as the Adson test or hyperabduction test during MRI or venography helps confirm thoracic outlet obstruction by demonstrating dynamic obstruction.²

TREATMENT CONSIDERATIONS

There are no universal guidelines for the treatment of Paget-Schroetter syndrome. However, the available data^3–5 suggest a multimodal approach that involves early catheter-directed thrombolysis and subsequent surgical decompression of the thoracic outlet. This can restore venous patency and reduce the risk of long-term complications such as rethrombosis and postthrombotic syndrome.^3–5

Surgical treatment includes resection of the first rib and division of the scalene muscles and the costoclavicular ligament. MRI with provocative testing helps guide the surgical approach. Anticoagulation therapy alone—ie, without thrombolysis and surgical decompression—is inadequate as it leads to recurrence of thrombosis and residual symptoms.⁶

Paget-Schroetter syndrome should not be managed the same as lower-extremity deep vein thrombosis because the cause and the exacerbating factors are different.

Unanswered questions

Because we have no data from randomized controlled trials, questions about management remain. What should be the duration of anticoagulation, especially in the absence of coexisting thrombophilia? Is thrombophilia screening useful? What is the optimal timing for starting thrombolytic therapy?

A careful history and heightened suspicion are required to make this diagnosis. If undiagnosed, it carries a risk of significant long-term morbidity and death. Dynamic obstruction during venography, in addition to MRI, can help identify an anatomic obstruction.

A 43-year-old man with no medical history presented with pain and swelling in his left arm for 2 weeks. He was a regular weight lifter, and his exercise routine included repetitive hyperextension and hyperabduction of his arms while lifting heavy weights.

He had no history of recent trauma or venous cannulation of the left arm. His family history was negative for thrombophilic disorders. Physical examination revealed a swollen and erythematous left arm and visible venous collaterals at the neck, shoulder, and chest. There was no evidence of arterial insufficiency.

Duplex ultrasonography confirmed thrombosis of the left brachial, axillary, and subclavian veins. Further evaluation with computed tomography showed no intrathoracic mass but revealed several subsegmental pulmonary thrombi in the right lung. A screen for thrombophilia was negative. Venography confirmed complete thrombotic occlusion of the subclavian, axillary, and brachial veins (Figure 1).

Catheter-directed thrombolysis with tissue plasminogen activator resulted in complete resolution of the thrombosis, but venography after 3 days of thrombolysis showed 50% residual stenosis of the left subclavian vein where it passes under the first rib (Figure 2). The redness and swelling had markedly improved 2 days after thrombolytic therapy. He was discharged home on rivaroxaban 20 mg daily.

Follow-up venography 2 months later (Figure 3), with the patient performing hyperabduction of the arms, showed a patent subclavian vein with no thrombosis, but dynamic compression and occlusion of the subclavian vein where it passes the first rib. Magnetic resonance imaging (MRI) of the neck showed no cervical (ie, extra) rib and no soft-tissue abnormalities of the scalene triangle.

Following this, the patient underwent resection of the left first rib for decompression of the venous thoracic outlet, which resulted in resolution of his symptoms. He remained asymptomatic at 6-month follow-up.

PAGET-SCHROETTER SYNDROME

Paget-Schroetter syndrome, also referred to as effort-induced or effort thrombosis, is thrombosis of the axillary or subclavian vein associated with strenuous and repetitive activity of the arms. Anatomic abnormalities at the thoracic outlet—cervical rib, congenital bands, hypertrophy of scalene tendons, abnormal insertion of the costoclavicular ligament—and repetitive trauma to the endothelium of the subclavian vein are key factors in its initiation and progression.

The condition is seen primarily in young people who participate in strenuous activities such as rowing, weight lifting, and baseball pitching. It is estimated to be the cause of 40% of cases of primary upper-extremity deep vein thrombosis in the absence of an obvious risk factor or trigger such as a central venous catheter, pacemaker, port, or occult malignancy.¹

A provocative test such as the Adson test or hyperabduction test during MRI or venography helps confirm thoracic outlet obstruction by demonstrating dynamic obstruction.²

TREATMENT CONSIDERATIONS

There are no universal guidelines for the treatment of Paget-Schroetter syndrome. However, the available data^3–5 suggest a multimodal approach that involves early catheter-directed thrombolysis and subsequent surgical decompression of the thoracic outlet. This can restore venous patency and reduce the risk of long-term complications such as rethrombosis and postthrombotic syndrome.^3–5

Surgical treatment includes resection of the first rib and division of the scalene muscles and the costoclavicular ligament. MRI with provocative testing helps guide the surgical approach. Anticoagulation therapy alone—ie, without thrombolysis and surgical decompression—is inadequate as it leads to recurrence of thrombosis and residual symptoms.⁶

Paget-Schroetter syndrome should not be managed the same as lower-extremity deep vein thrombosis because the cause and the exacerbating factors are different.

Unanswered questions

Because we have no data from randomized controlled trials, questions about management remain. What should be the duration of anticoagulation, especially in the absence of coexisting thrombophilia? Is thrombophilia screening useful? What is the optimal timing for starting thrombolytic therapy?

A careful history and heightened suspicion are required to make this diagnosis. If undiagnosed, it carries a risk of significant long-term morbidity and death. Dynamic obstruction during venography, in addition to MRI, can help identify an anatomic obstruction.

A 75-year-old woman was referred to our pulmonary clinic with a 4-year history of intermittent episodes of persistent cough, occasionally productive of sputum, and mild exertional dyspnea. She had been treated with azithromycin for presumed community-acquired pneumonia, and her symptoms had initially improved. Subsequently, she experienced discrete, recurrent episodes of “bronchitis,” with productive cough and mild exertional dyspnea. Testing for latent tuberculosis had been negative. She reported a 10-pack-year smoking history in the remote past.

Her medical history included asthma, atrial fibrillation, gastroesophageal reflux disorder, hyperlipidemia, osteopenia, hypothyroidism, and allergic rhinitis. Her current medications were metoprolol, propafenone, and warfarin.

ABNORMALITIES ON PREVIOUS IMAGING

Computed tomography (CT) in April 2010 had revealed scattered linear, nodular, and “tree-in-bud” opacities involving the bilateral apices and the upper, middle, and lower lobes of the right lung, suggestive of bronchiolitis. Mild bronchiectasis had also been noted (Figure 1). Chest radiography had demonstrated signs of bronchiectasis and several scattered nodules (Figure 2). These abnormalities were still present on another CT scan in May 2013.

The patient had not undergone bronchoscopy before she was referred to our clinic.

WORKUP AT OUR CLINIC

On examination, the patient was lean, with a body mass index of 20.53 kg/m². She appeared calm, well-groomed, and well-dressed, and had a very polite manner. When she coughed, she tried to suppress it, as if she were self-conscious about it. Her heart rhythm was irregularly irregular with a normal rate.

Expectorated sputum samples were obtained. Stains for acid-fast bacilli were negative, but three cultures were positive for acid-fast bacilli consistent with Mycobacterium avium-intracellulare. Serologic studies were negative for fungal infection and immunoglobulin deficiency.

Based on her symptoms and on the findings of imaging studies and sputum culture, we arrived at the diagnosis of nontuberculous mycobacterial lung infection, specifically, Lady Windermere syndrome.

NONTUBERCULOUS MYOCOBACTERIAL LUNG INFECTION

The diagnosis of nontuberculous mycobacterial lung infection is based on respiratory symptoms, findings on imaging (eg, nodular or cavitary opacities on radiography, or multifocal bronchiectasis and multiple small nodules on CT), and a positive culture for nontuberculous mycobacterial infection in more than two specimens of expectorated sputum or in more than one specimen from bronchoalveolar lavage. Lung biopsy with tissue culture is another way to confirm the diagnosis.

LADY WINDERMERE SYNDROME

Lady Windermere syndrome was described more than 20 years ago.¹ The name derives from the lead character in Oscar Wilde’s play Lady Windermere’s Fan, which satirizes the strict morals and polite manners typical of the Victorian era in Great Britain.²

The patient with Lady Windermere syndrome is typically a thin, lean, well-mannered elderly woman who voluntarily suppresses her cough out of politeness. Suppression of the cough is thought to predispose to lung infection by allowing secretions to collect in the airways, especially in the right middle lobe, which has the longest and narrowest of the lobar bronchi.^3,4

Symptoms of Lady Windermere syndrome include cough, sputum production, and fatigue similar to that of acute or chronic bronchitis. Dyspnea, fever, and hemoptysis are less common.⁵ The differential diagnosis for these symptoms is broad and includes asthma, chronic obstructive pulmonary disease, gastroesophageal reflux disease, pneumonia, bronchiectasis, cystic fibrosis, interstitial lung disease, postnasal drip, lung cancer, and heart failure.

A prospective cohort study by Kim et al⁶ yielded descriptions of typical patients with Lady Windermere syndrome. Patients were tall and lean, tended to have scoliosis, and more commonly had pectus excavatum or mitral valve prolapse; 95% were women, 91% were white, and the average age was 60. The morphologic features are thought to contribute to impaired clearance of airway secretions by altered mechanics during coughing.

HALLMARKS ON IMAGING

Kim et al⁶ reported that the most common findings on lung imaging in nontuberculous mycobacterial infection were bronchiectasis involving the right middle lobe (90%), nodules involving the right lower lobe (73%) and right middle lobe (71%), and, less commonly, a cavitary infiltrate involving the right upper lobe (17%) or right middle lobe (10%).

Key findings on imaging in Lady Windermere syndrome include opacities and “cylindrical bronchiectasis” predominantly involving the right middle lobe or lingula.⁵ Bronchiolar inflammation in response to nontuberculous mycobacterial infection may cause a nodular appearance, often progressing to a tree-in-bud appearance on CT.

Other diagnostic considerations for tree-in-bud appearance on CT include fungal, viral, or other bacterial infection, aspiration pneumonitis, inhalation of a foreign substance, cystic fibrosis, rheumatoid arthritis, SjÖgren syndrome, bronchiolitis obliterans, and neoplastic disease.

CURRENT TREATMENT OPTIONS

Treatment of nontuberculous mycobacterial lung infection, including Lady Windermere syndrome, is not necessary in every case, given the variability in clinical symptoms and in disease progression. Patients with progressive symptoms or radiographic changes should be considered candidates for treatment.

Management is directed at the underlying infection. M avium-intracellulare is ubiquitous in the environment, including in soil and water, and it has been reported as the most common pathogen in nontuberculous mycobacterial lung infection.⁷

Nodular-bronchiectatic nontuberculous mycobacterial lung disease typically progresses more slowly than fibrocavitary disease. For patients with nodular-bronchiectatic disease, follow-up over months or years may be needed before clinical or radiographic changes become apparent.

When treatment is indicated for nodular-bronchiectatic nontuberculous mycobacterial lung infection, it should include a macrolide antibiotic, ethambutol, and rifampin.^7,8 Monotherapy with a macrolide is not recommended because of the risk of macrolide resistance. Addition of an aminoglycoside may be considered when treating fibrocavitary disease or widespread nodular bronchiectatic disease.

Management of bronchiectasis, when present, includes chest physiotherapy, pulmonary hygiene therapy, and awareness of the predisposition for nonmycobacterial lung infection. The decision to prescribe antimicrobials should take into consideration the risks and benefits for each patient.

Because treatment involves multidrug regimens, drug interactions and adverse effects need to be considered and monitored, especially in elderly patients, who may already be taking multiple medications. Treatment should be continued until a patient has negative sputum cultures for acid-fast bacilli while on therapy, for 1 year.

A 75-year-old woman was referred to our pulmonary clinic with a 4-year history of intermittent episodes of persistent cough, occasionally productive of sputum, and mild exertional dyspnea. She had been treated with azithromycin for presumed community-acquired pneumonia, and her symptoms had initially improved. Subsequently, she experienced discrete, recurrent episodes of “bronchitis,” with productive cough and mild exertional dyspnea. Testing for latent tuberculosis had been negative. She reported a 10-pack-year smoking history in the remote past.

Her medical history included asthma, atrial fibrillation, gastroesophageal reflux disorder, hyperlipidemia, osteopenia, hypothyroidism, and allergic rhinitis. Her current medications were metoprolol, propafenone, and warfarin.

ABNORMALITIES ON PREVIOUS IMAGING

Computed tomography (CT) in April 2010 had revealed scattered linear, nodular, and “tree-in-bud” opacities involving the bilateral apices and the upper, middle, and lower lobes of the right lung, suggestive of bronchiolitis. Mild bronchiectasis had also been noted (Figure 1). Chest radiography had demonstrated signs of bronchiectasis and several scattered nodules (Figure 2). These abnormalities were still present on another CT scan in May 2013.

The patient had not undergone bronchoscopy before she was referred to our clinic.

WORKUP AT OUR CLINIC

On examination, the patient was lean, with a body mass index of 20.53 kg/m². She appeared calm, well-groomed, and well-dressed, and had a very polite manner. When she coughed, she tried to suppress it, as if she were self-conscious about it. Her heart rhythm was irregularly irregular with a normal rate.

Expectorated sputum samples were obtained. Stains for acid-fast bacilli were negative, but three cultures were positive for acid-fast bacilli consistent with Mycobacterium avium-intracellulare. Serologic studies were negative for fungal infection and immunoglobulin deficiency.

Based on her symptoms and on the findings of imaging studies and sputum culture, we arrived at the diagnosis of nontuberculous mycobacterial lung infection, specifically, Lady Windermere syndrome.

NONTUBERCULOUS MYOCOBACTERIAL LUNG INFECTION

The diagnosis of nontuberculous mycobacterial lung infection is based on respiratory symptoms, findings on imaging (eg, nodular or cavitary opacities on radiography, or multifocal bronchiectasis and multiple small nodules on CT), and a positive culture for nontuberculous mycobacterial infection in more than two specimens of expectorated sputum or in more than one specimen from bronchoalveolar lavage. Lung biopsy with tissue culture is another way to confirm the diagnosis.

LADY WINDERMERE SYNDROME

Lady Windermere syndrome was described more than 20 years ago.¹ The name derives from the lead character in Oscar Wilde’s play Lady Windermere’s Fan, which satirizes the strict morals and polite manners typical of the Victorian era in Great Britain.²

The patient with Lady Windermere syndrome is typically a thin, lean, well-mannered elderly woman who voluntarily suppresses her cough out of politeness. Suppression of the cough is thought to predispose to lung infection by allowing secretions to collect in the airways, especially in the right middle lobe, which has the longest and narrowest of the lobar bronchi.^3,4

Symptoms of Lady Windermere syndrome include cough, sputum production, and fatigue similar to that of acute or chronic bronchitis. Dyspnea, fever, and hemoptysis are less common.⁵ The differential diagnosis for these symptoms is broad and includes asthma, chronic obstructive pulmonary disease, gastroesophageal reflux disease, pneumonia, bronchiectasis, cystic fibrosis, interstitial lung disease, postnasal drip, lung cancer, and heart failure.

A prospective cohort study by Kim et al⁶ yielded descriptions of typical patients with Lady Windermere syndrome. Patients were tall and lean, tended to have scoliosis, and more commonly had pectus excavatum or mitral valve prolapse; 95% were women, 91% were white, and the average age was 60. The morphologic features are thought to contribute to impaired clearance of airway secretions by altered mechanics during coughing.

HALLMARKS ON IMAGING

Kim et al⁶ reported that the most common findings on lung imaging in nontuberculous mycobacterial infection were bronchiectasis involving the right middle lobe (90%), nodules involving the right lower lobe (73%) and right middle lobe (71%), and, less commonly, a cavitary infiltrate involving the right upper lobe (17%) or right middle lobe (10%).

Key findings on imaging in Lady Windermere syndrome include opacities and “cylindrical bronchiectasis” predominantly involving the right middle lobe or lingula.⁵ Bronchiolar inflammation in response to nontuberculous mycobacterial infection may cause a nodular appearance, often progressing to a tree-in-bud appearance on CT.

Other diagnostic considerations for tree-in-bud appearance on CT include fungal, viral, or other bacterial infection, aspiration pneumonitis, inhalation of a foreign substance, cystic fibrosis, rheumatoid arthritis, SjÖgren syndrome, bronchiolitis obliterans, and neoplastic disease.

CURRENT TREATMENT OPTIONS

Treatment of nontuberculous mycobacterial lung infection, including Lady Windermere syndrome, is not necessary in every case, given the variability in clinical symptoms and in disease progression. Patients with progressive symptoms or radiographic changes should be considered candidates for treatment.

Management is directed at the underlying infection. M avium-intracellulare is ubiquitous in the environment, including in soil and water, and it has been reported as the most common pathogen in nontuberculous mycobacterial lung infection.⁷

Nodular-bronchiectatic nontuberculous mycobacterial lung disease typically progresses more slowly than fibrocavitary disease. For patients with nodular-bronchiectatic disease, follow-up over months or years may be needed before clinical or radiographic changes become apparent.

When treatment is indicated for nodular-bronchiectatic nontuberculous mycobacterial lung infection, it should include a macrolide antibiotic, ethambutol, and rifampin.^7,8 Monotherapy with a macrolide is not recommended because of the risk of macrolide resistance. Addition of an aminoglycoside may be considered when treating fibrocavitary disease or widespread nodular bronchiectatic disease.

Management of bronchiectasis, when present, includes chest physiotherapy, pulmonary hygiene therapy, and awareness of the predisposition for nonmycobacterial lung infection. The decision to prescribe antimicrobials should take into consideration the risks and benefits for each patient.

Because treatment involves multidrug regimens, drug interactions and adverse effects need to be considered and monitored, especially in elderly patients, who may already be taking multiple medications. Treatment should be continued until a patient has negative sputum cultures for acid-fast bacilli while on therapy, for 1 year.

A 75-year-old woman was referred to our pulmonary clinic with a 4-year history of intermittent episodes of persistent cough, occasionally productive of sputum, and mild exertional dyspnea. She had been treated with azithromycin for presumed community-acquired pneumonia, and her symptoms had initially improved. Subsequently, she experienced discrete, recurrent episodes of “bronchitis,” with productive cough and mild exertional dyspnea. Testing for latent tuberculosis had been negative. She reported a 10-pack-year smoking history in the remote past.

Her medical history included asthma, atrial fibrillation, gastroesophageal reflux disorder, hyperlipidemia, osteopenia, hypothyroidism, and allergic rhinitis. Her current medications were metoprolol, propafenone, and warfarin.

ABNORMALITIES ON PREVIOUS IMAGING

Computed tomography (CT) in April 2010 had revealed scattered linear, nodular, and “tree-in-bud” opacities involving the bilateral apices and the upper, middle, and lower lobes of the right lung, suggestive of bronchiolitis. Mild bronchiectasis had also been noted (Figure 1). Chest radiography had demonstrated signs of bronchiectasis and several scattered nodules (Figure 2). These abnormalities were still present on another CT scan in May 2013.

The patient had not undergone bronchoscopy before she was referred to our clinic.

WORKUP AT OUR CLINIC

On examination, the patient was lean, with a body mass index of 20.53 kg/m². She appeared calm, well-groomed, and well-dressed, and had a very polite manner. When she coughed, she tried to suppress it, as if she were self-conscious about it. Her heart rhythm was irregularly irregular with a normal rate.

Expectorated sputum samples were obtained. Stains for acid-fast bacilli were negative, but three cultures were positive for acid-fast bacilli consistent with Mycobacterium avium-intracellulare. Serologic studies were negative for fungal infection and immunoglobulin deficiency.

Based on her symptoms and on the findings of imaging studies and sputum culture, we arrived at the diagnosis of nontuberculous mycobacterial lung infection, specifically, Lady Windermere syndrome.

NONTUBERCULOUS MYOCOBACTERIAL LUNG INFECTION

The diagnosis of nontuberculous mycobacterial lung infection is based on respiratory symptoms, findings on imaging (eg, nodular or cavitary opacities on radiography, or multifocal bronchiectasis and multiple small nodules on CT), and a positive culture for nontuberculous mycobacterial infection in more than two specimens of expectorated sputum or in more than one specimen from bronchoalveolar lavage. Lung biopsy with tissue culture is another way to confirm the diagnosis.

LADY WINDERMERE SYNDROME

Lady Windermere syndrome was described more than 20 years ago.¹ The name derives from the lead character in Oscar Wilde’s play Lady Windermere’s Fan, which satirizes the strict morals and polite manners typical of the Victorian era in Great Britain.²

The patient with Lady Windermere syndrome is typically a thin, lean, well-mannered elderly woman who voluntarily suppresses her cough out of politeness. Suppression of the cough is thought to predispose to lung infection by allowing secretions to collect in the airways, especially in the right middle lobe, which has the longest and narrowest of the lobar bronchi.^3,4

Symptoms of Lady Windermere syndrome include cough, sputum production, and fatigue similar to that of acute or chronic bronchitis. Dyspnea, fever, and hemoptysis are less common.⁵ The differential diagnosis for these symptoms is broad and includes asthma, chronic obstructive pulmonary disease, gastroesophageal reflux disease, pneumonia, bronchiectasis, cystic fibrosis, interstitial lung disease, postnasal drip, lung cancer, and heart failure.

A prospective cohort study by Kim et al⁶ yielded descriptions of typical patients with Lady Windermere syndrome. Patients were tall and lean, tended to have scoliosis, and more commonly had pectus excavatum or mitral valve prolapse; 95% were women, 91% were white, and the average age was 60. The morphologic features are thought to contribute to impaired clearance of airway secretions by altered mechanics during coughing.

HALLMARKS ON IMAGING

Kim et al⁶ reported that the most common findings on lung imaging in nontuberculous mycobacterial infection were bronchiectasis involving the right middle lobe (90%), nodules involving the right lower lobe (73%) and right middle lobe (71%), and, less commonly, a cavitary infiltrate involving the right upper lobe (17%) or right middle lobe (10%).

Key findings on imaging in Lady Windermere syndrome include opacities and “cylindrical bronchiectasis” predominantly involving the right middle lobe or lingula.⁵ Bronchiolar inflammation in response to nontuberculous mycobacterial infection may cause a nodular appearance, often progressing to a tree-in-bud appearance on CT.

Other diagnostic considerations for tree-in-bud appearance on CT include fungal, viral, or other bacterial infection, aspiration pneumonitis, inhalation of a foreign substance, cystic fibrosis, rheumatoid arthritis, SjÖgren syndrome, bronchiolitis obliterans, and neoplastic disease.

CURRENT TREATMENT OPTIONS

Treatment of nontuberculous mycobacterial lung infection, including Lady Windermere syndrome, is not necessary in every case, given the variability in clinical symptoms and in disease progression. Patients with progressive symptoms or radiographic changes should be considered candidates for treatment.

Management is directed at the underlying infection. M avium-intracellulare is ubiquitous in the environment, including in soil and water, and it has been reported as the most common pathogen in nontuberculous mycobacterial lung infection.⁷

Nodular-bronchiectatic nontuberculous mycobacterial lung disease typically progresses more slowly than fibrocavitary disease. For patients with nodular-bronchiectatic disease, follow-up over months or years may be needed before clinical or radiographic changes become apparent.

When treatment is indicated for nodular-bronchiectatic nontuberculous mycobacterial lung infection, it should include a macrolide antibiotic, ethambutol, and rifampin.^7,8 Monotherapy with a macrolide is not recommended because of the risk of macrolide resistance. Addition of an aminoglycoside may be considered when treating fibrocavitary disease or widespread nodular bronchiectatic disease.

Management of bronchiectasis, when present, includes chest physiotherapy, pulmonary hygiene therapy, and awareness of the predisposition for nonmycobacterial lung infection. The decision to prescribe antimicrobials should take into consideration the risks and benefits for each patient.

Because treatment involves multidrug regimens, drug interactions and adverse effects need to be considered and monitored, especially in elderly patients, who may already be taking multiple medications. Treatment should be continued until a patient has negative sputum cultures for acid-fast bacilli while on therapy, for 1 year.