Imaging

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.

Interscalene brachial plexus anesthesia is commonly used for arthroscopic and open procedures of the shoulder. This regional anesthetic targets the trunks of the brachial plexus and anesthetizes the area about the shoulder and proximal arm. Its use may obviate the need for concomitant general anesthesia, potentially reducing the use of postoperative intravenous and oral pain medication. Furthermore, patients often bypass the acute postoperative anesthesia care unit and proceed directly to the ambulatory unit, permitting earlier hospital discharge. Previous reports in the literature have demonstrated higher rates of neurologic, cardiac, and pulmonary complications from this procedure; in particular, the incidence of pneumothorax was reported as high as 3%.¹ Techniques to localize the nerves, such as electrical nerve stimulation and, more recently, ultrasound guidance, have reduced these complication rates.^2,3 Successful administration of the block has been shown to result in satisfactory postoperative pain relief.² However, ultrasound-guided interscalene nerve blocks remain operator-dependent and complications may still occur.

We report a case of tension pneumothorax after arthroscopic rotator cuff repair and subacromial decompression with an ultrasound-guided interscalene block. Immediate recognition and treatment of this complication resulted in a good clinical outcome. The patient provided written informed consent for print and electronic publication of this case report. 

Case Report

A 56-year-old woman presented with 3 months of right shoulder pain after a fall. Examination was pertinent for weakness in forward elevation and positive rotator cuff impingement signs. She remained symptomatic despite a course of nonsurgical management that included cortisone injections and physical therapy. Magnetic resonance imaging of the shoulder showed a full-thickness supraspinatus tear with minimal fatty atrophy. After a discussion of her treatment options, she elected to undergo an arthroscopic rotator cuff repair with subacromial decompression. An evaluation by her internist revealed no pertinent medical history apart from obesity (body mass index, 36). Specifically, there was no reported history of chronic obstructive pulmonary disease or asthma. She denied any prior cigarette smoking.

The patient was evaluated by the regional anesthesia team and was classified as a class 2 airway. An interscalene brachial plexus block was performed using a 2-inch, 22-gauge needle inserted into the interscalene groove. Using an out-of-plane technique under direct ultrasound guidance, 30 mL of 0.52% ropivacaine was injected. The block was considered successful, and no complications, such as resistance, paresthesias, pain, or blood on aspiration, were noted during injection. The patient had no complaints of chest pain or shortness of breath immediately afterward, and all vital signs were stable throughout the procedure.

The patient was brought to the operating room and placed in the beach-chair position. Induction for general anesthesia was started 15 minutes after the regional anesthetic, with 2 intubation attempts necessary because of poor airway visualization. After placement of the endotracheal tube, breath sounds were noted to be equal bilaterally. The arthroscopic procedure consisted of double-row rotator cuff repair, subacromial decompression, and débridement of the glenohumeral joint for synovitis, using standard arthroscopic portals. There were no difficulties with trocar placement, and bleeding was minimal throughout the case. The total surgical time was 150 minutes and a pump pressure of 30 mm Hg was maintained during the arthroscopy.

Within the first 60 minutes of the start of the arthroscopic procedure, the patient was noted to be intermittently hypotensive with mean arterial pressure (MAP) ranging from the 30s to 130s mm Hg and pulse in the 70 to 80 beats/min range. FiO₂ in the 85% to 95% range was maintained throughout the procedure. During that time, 50 μg phenylephrine was administered on 4 separate occasions to maintain her blood pressure. The labile blood pressure was attributed by the anesthesiologist to the beach-chair position. During an attempted extubation upon conclusion of the surgery, the patient became hypotensive with MAP that ranged from the 40s to 60s mm Hg and tachycardic to 90 beats/min. The oxygen saturation was in the low 90s and tidal volume was poor. Absent lung sounds were noted on the right chest. An urgent portable chest radiograph showed a large right-sided tension pneumothorax with mediastinal shift (Figure 1). After an immediate general surgery consultation, a chest tube was placed in the operating room. The patient’s vital signs improved and a repeat chest radiograph revealed successful re-expansion of the lung (Figure 2). She was transferred to the acute postoperative anesthesia care unit and extubated in the intensive care unit later that day.

The patient’s chest tube was removed 2 days later and she was discharged home on hospital day 5 with a completely resolved pneumothorax. She was seen 1 week later in the office for a postoperative visit and reported feeling well without chest pain or shortness of breath.

Discussion

Interscalene brachial plexus anesthesia was first described by Winnie⁴ in 1970. This block targets the trunks of the brachial plexus, which are enclosed in a fascial sheath between the anterior and middle scalene muscles. In this region lie several structures at risk: the phrenic nerve superficially and inferiorly; the carotid sheath located superficially and medially; the subclavian artery parallel to the trunks; and the cupula of the lung that lies deep and inferior to the anterior scalene muscle. Recognized complications of the block include vocal hoarseness, Horner syndrome, and hemidiaphragmatic paresis caused by the temporary blockade of the ipsilateral recurrent laryngeal nerve, stellate ganglion, and phrenic nerve, in that order.⁵ Use of the interscalene block has been associated with minimal risk for pneumothorax, because the needle entry point is superior and directed away from the lung pleura.⁶ This is in contrast to the more inferiorly placed supraclavicular block, located in closer proximity to the lung cupula.⁵

Two different approaches are commonly used during ultrasound-guided nerve blocks. The in-plane approach generates a long-axis view of the needle by advancing the needle parallel with the long axis of the ultrasound probe. While this allows direct visualization of the needle tip, it requires deeper needle insertion from lateral to medial, causing puncture of the middle scalene muscle that may increase patient discomfort and risk nerve injury within the muscle.⁷ The out-of-plane approach used on our patient involves needle insertion parallel to the brachial plexus, but along the short axis of the ultrasound probe. Although this permits the operator to assess the periphery of the nerve, it may lead to poor needle-tip visualization during the procedure. As a result, operators often use a combination of tissue disturbance and “hydrolocation,” in which fluid is injected to indicate the needle-tip location.^8,9

Tension pneumothorax represents the accumulation of air in the pleural space that leads to impaired pulmonary and cardiac function. It is often caused by disruption or puncture of the parietal or visceral pleura, creating a connection between the alveoli and pleural cavity. The gradual buildup of air in the pleural cavity results in increased intrapleural pressure, which compresses and ultimately collapses the ipsilateral lung. Venous compression restricts blood return to the heart and reduces cardiac output. Clinical manifestations include dyspnea, hypoxemia, tachycardia, and hypotension.¹⁰ Multiple techniques were developed to better localize the brachial plexus while reducing injury to nearby structures, including the lung. These include eliciting needle paresthesias, electrical nerve stimulation, and ultrasound guidance. While nerve stimulation was once the gold standard for brachial plexus localization, ultrasound guidance has gained in popularity because of its noninvasive nature and dynamic capability to identify nerves and surrounding structures.¹¹ Perlas and colleagues¹² determined the sensitivity of needle paresthesias and nerve stimulation to be 38% and 75%, respectively, in cases in which plexus localization had been confirmed by ultrasound.

Several studies have reported on the efficacy of interscalene nerve block with either nerve stimulation or ultrasound guidance in the setting of shoulder surgery.^2,3 Bishop and colleagues³ reviewed 547 patients who underwent interscalene regional anesthesia with nerve stimulation for both arthroscopic and open-shoulder procedures. They reported a 97% success rate and 12 (2.3%) minor complications, including sensory neuropathy and complex regional pain syndrome. There were no cases of pneumothorax, cardiac events, or other major complications.³ In a prospective study of 1319 patients, Singh and colleagues² reported a 99.6% success rate using ultrasound-guided interscalene blocks for their shoulder surgeries. A total of 38 adverse events (2.88%) were identified: 14 transient neurologic events, including ear numbness, digital numbness, and brachial plexitis; 1 case of intraoperative bradycardia, and 2 cancellations after the block for chest pain and flank pain, which yielded negative cardiac workups. Other complications included postoperative emergency room visits and hospital admissions for reasons unrelated to the block.² Interscalene regional anesthesia, therefore, provides effective anesthesia for shoulder surgery with low complication rates.

Pneumothorax after ultrasound-guided interscalene block has rarely been reported.^13,14 In a review of 144 ultrasound-guided indwelling interscalene catheter placements, a 98% successful block rate with a single complication of small pneumothorax after total shoulder arthroplasty was reported.¹³ Mandim and colleagues¹⁴ reported a case of pneumothorax in a smoker who underwent an ultrasound-guided brachial plexus block prior to open reduction and internal fixation of an ulnar fracture. While the patient was asymptomatic and vital signs remained stable during the procedure, the patient complained postoperatively of chest pain with hypoxia, tachycardia, and hypotension. A chest radiograph confirmed an ipsilateral pneumothorax, and the patient was treated successfully with chest-tube placement. The authors attributed this complication to a higher pleural dome resulting from a hyperinflated lung caused by chronic smoking. Our patient reported no history of smoking and her preoperative chest radiograph had no evidence of lung disease.

In contrast, several cases of pneumothorax after shoulder surgery have been reported in the absence of nerve block. Oldman and Peng¹ reported a 41-year-old nonsmoker who underwent arthroscopic labral repair and subacromial decompression. The preoperative nerve block was cancelled, and the patient received general endotracheal anesthesia alone. Fifty minutes after the case, the patient developed chest pain and hypoxia. A chest radiograph showed a small pneumothorax that was managed conservatively. The pneumothorax was attributed to spontaneous rupture of a preexisting lung bulla, suggesting that blocks are not always the cause of this complication. Furthermore, Dietzel and Ciullo¹⁵ reported 4 cases of spontaneous pneumothorax within 24 hours of uncomplicated arthroscopic shoulder procedures under general anesthesia in the lateral decubitus position. The patient ages ranged from 22 to 38 years, and medical histories were all significant for preexisting lung disease, remote history of pneumonia, and heavy smoking. Three of the patients experienced symptoms at home the day after surgery. The authors concluded that these cases were likely caused by rupture of blebs or bullae from underlying lung disease; these ruptured blebs or bullae are difficult to detect and usually located in the upper lung. The pressure gradient from the positive pressure of anesthesia and the ipsilateral upper lung is thought to be highest in the lateral decubitus position, increasing their chance of rupture.¹⁵

Finally, Lee and colleagues¹⁶ described 3 patients aged 40 to 45 years who underwent uncomplicated subacromial decompression in the beach-chair position under general anesthesia. Significant shoulder, neck, and axillary swelling were noted after surgery, and a chest radiograph showed tension pneumothorax, subcutaneous emphysema, and pneumomediastinum. The authors speculated that pressure in the subacromial space may become negative relative to atmospheric pressure when the shaver and suction are running, drawing in air through other portals. When the suction is discontinued, fluid infusion may push air into the surrounding tissue, leading to subcutaneous emphysema, which may spread to the mediastinum.¹⁶ 

Conclusion 

Ultrasound-guided interscalene nerve blocks have successfully provided anesthesia for shoulder surgeries with low complication rates. Although the incidence of pneumothorax has decreased significantly with ultrasound guidance, the success of this procedure is highly operator-dependent. We present the case of an otherwise healthy patient without known pulmonary disease who developed a tension pneumothorax after the administration of ultrasound-guided regional and general anesthesia for arthroscopic shoulder surgery. Orthopedic surgeons and anesthesiologists must remain vigilant for pneumothorax during the perioperative period after shoulder surgery performed under interscalene regional aesthesia, particularly in the setting of hypotension, hypoxia, and/or tachycardia. Risk factors, such as history of smoking and preexisting lung disease, may predispose patients to the development of pneumothorax. Timely recognition and placement of a chest tube result in satisfactory clinical outcomes.

Interscalene brachial plexus anesthesia is commonly used for arthroscopic and open procedures of the shoulder. This regional anesthetic targets the trunks of the brachial plexus and anesthetizes the area about the shoulder and proximal arm. Its use may obviate the need for concomitant general anesthesia, potentially reducing the use of postoperative intravenous and oral pain medication. Furthermore, patients often bypass the acute postoperative anesthesia care unit and proceed directly to the ambulatory unit, permitting earlier hospital discharge. Previous reports in the literature have demonstrated higher rates of neurologic, cardiac, and pulmonary complications from this procedure; in particular, the incidence of pneumothorax was reported as high as 3%.¹ Techniques to localize the nerves, such as electrical nerve stimulation and, more recently, ultrasound guidance, have reduced these complication rates.^2,3 Successful administration of the block has been shown to result in satisfactory postoperative pain relief.² However, ultrasound-guided interscalene nerve blocks remain operator-dependent and complications may still occur.

We report a case of tension pneumothorax after arthroscopic rotator cuff repair and subacromial decompression with an ultrasound-guided interscalene block. Immediate recognition and treatment of this complication resulted in a good clinical outcome. The patient provided written informed consent for print and electronic publication of this case report. 

Case Report

A 56-year-old woman presented with 3 months of right shoulder pain after a fall. Examination was pertinent for weakness in forward elevation and positive rotator cuff impingement signs. She remained symptomatic despite a course of nonsurgical management that included cortisone injections and physical therapy. Magnetic resonance imaging of the shoulder showed a full-thickness supraspinatus tear with minimal fatty atrophy. After a discussion of her treatment options, she elected to undergo an arthroscopic rotator cuff repair with subacromial decompression. An evaluation by her internist revealed no pertinent medical history apart from obesity (body mass index, 36). Specifically, there was no reported history of chronic obstructive pulmonary disease or asthma. She denied any prior cigarette smoking.

The patient was evaluated by the regional anesthesia team and was classified as a class 2 airway. An interscalene brachial plexus block was performed using a 2-inch, 22-gauge needle inserted into the interscalene groove. Using an out-of-plane technique under direct ultrasound guidance, 30 mL of 0.52% ropivacaine was injected. The block was considered successful, and no complications, such as resistance, paresthesias, pain, or blood on aspiration, were noted during injection. The patient had no complaints of chest pain or shortness of breath immediately afterward, and all vital signs were stable throughout the procedure.

The patient was brought to the operating room and placed in the beach-chair position. Induction for general anesthesia was started 15 minutes after the regional anesthetic, with 2 intubation attempts necessary because of poor airway visualization. After placement of the endotracheal tube, breath sounds were noted to be equal bilaterally. The arthroscopic procedure consisted of double-row rotator cuff repair, subacromial decompression, and débridement of the glenohumeral joint for synovitis, using standard arthroscopic portals. There were no difficulties with trocar placement, and bleeding was minimal throughout the case. The total surgical time was 150 minutes and a pump pressure of 30 mm Hg was maintained during the arthroscopy.

Within the first 60 minutes of the start of the arthroscopic procedure, the patient was noted to be intermittently hypotensive with mean arterial pressure (MAP) ranging from the 30s to 130s mm Hg and pulse in the 70 to 80 beats/min range. FiO₂ in the 85% to 95% range was maintained throughout the procedure. During that time, 50 μg phenylephrine was administered on 4 separate occasions to maintain her blood pressure. The labile blood pressure was attributed by the anesthesiologist to the beach-chair position. During an attempted extubation upon conclusion of the surgery, the patient became hypotensive with MAP that ranged from the 40s to 60s mm Hg and tachycardic to 90 beats/min. The oxygen saturation was in the low 90s and tidal volume was poor. Absent lung sounds were noted on the right chest. An urgent portable chest radiograph showed a large right-sided tension pneumothorax with mediastinal shift (Figure 1). After an immediate general surgery consultation, a chest tube was placed in the operating room. The patient’s vital signs improved and a repeat chest radiograph revealed successful re-expansion of the lung (Figure 2). She was transferred to the acute postoperative anesthesia care unit and extubated in the intensive care unit later that day.

The patient’s chest tube was removed 2 days later and she was discharged home on hospital day 5 with a completely resolved pneumothorax. She was seen 1 week later in the office for a postoperative visit and reported feeling well without chest pain or shortness of breath.

Discussion

Interscalene brachial plexus anesthesia was first described by Winnie⁴ in 1970. This block targets the trunks of the brachial plexus, which are enclosed in a fascial sheath between the anterior and middle scalene muscles. In this region lie several structures at risk: the phrenic nerve superficially and inferiorly; the carotid sheath located superficially and medially; the subclavian artery parallel to the trunks; and the cupula of the lung that lies deep and inferior to the anterior scalene muscle. Recognized complications of the block include vocal hoarseness, Horner syndrome, and hemidiaphragmatic paresis caused by the temporary blockade of the ipsilateral recurrent laryngeal nerve, stellate ganglion, and phrenic nerve, in that order.⁵ Use of the interscalene block has been associated with minimal risk for pneumothorax, because the needle entry point is superior and directed away from the lung pleura.⁶ This is in contrast to the more inferiorly placed supraclavicular block, located in closer proximity to the lung cupula.⁵

Two different approaches are commonly used during ultrasound-guided nerve blocks. The in-plane approach generates a long-axis view of the needle by advancing the needle parallel with the long axis of the ultrasound probe. While this allows direct visualization of the needle tip, it requires deeper needle insertion from lateral to medial, causing puncture of the middle scalene muscle that may increase patient discomfort and risk nerve injury within the muscle.⁷ The out-of-plane approach used on our patient involves needle insertion parallel to the brachial plexus, but along the short axis of the ultrasound probe. Although this permits the operator to assess the periphery of the nerve, it may lead to poor needle-tip visualization during the procedure. As a result, operators often use a combination of tissue disturbance and “hydrolocation,” in which fluid is injected to indicate the needle-tip location.^8,9

Tension pneumothorax represents the accumulation of air in the pleural space that leads to impaired pulmonary and cardiac function. It is often caused by disruption or puncture of the parietal or visceral pleura, creating a connection between the alveoli and pleural cavity. The gradual buildup of air in the pleural cavity results in increased intrapleural pressure, which compresses and ultimately collapses the ipsilateral lung. Venous compression restricts blood return to the heart and reduces cardiac output. Clinical manifestations include dyspnea, hypoxemia, tachycardia, and hypotension.¹⁰ Multiple techniques were developed to better localize the brachial plexus while reducing injury to nearby structures, including the lung. These include eliciting needle paresthesias, electrical nerve stimulation, and ultrasound guidance. While nerve stimulation was once the gold standard for brachial plexus localization, ultrasound guidance has gained in popularity because of its noninvasive nature and dynamic capability to identify nerves and surrounding structures.¹¹ Perlas and colleagues¹² determined the sensitivity of needle paresthesias and nerve stimulation to be 38% and 75%, respectively, in cases in which plexus localization had been confirmed by ultrasound.

Several studies have reported on the efficacy of interscalene nerve block with either nerve stimulation or ultrasound guidance in the setting of shoulder surgery.^2,3 Bishop and colleagues³ reviewed 547 patients who underwent interscalene regional anesthesia with nerve stimulation for both arthroscopic and open-shoulder procedures. They reported a 97% success rate and 12 (2.3%) minor complications, including sensory neuropathy and complex regional pain syndrome. There were no cases of pneumothorax, cardiac events, or other major complications.³ In a prospective study of 1319 patients, Singh and colleagues² reported a 99.6% success rate using ultrasound-guided interscalene blocks for their shoulder surgeries. A total of 38 adverse events (2.88%) were identified: 14 transient neurologic events, including ear numbness, digital numbness, and brachial plexitis; 1 case of intraoperative bradycardia, and 2 cancellations after the block for chest pain and flank pain, which yielded negative cardiac workups. Other complications included postoperative emergency room visits and hospital admissions for reasons unrelated to the block.² Interscalene regional anesthesia, therefore, provides effective anesthesia for shoulder surgery with low complication rates.

Pneumothorax after ultrasound-guided interscalene block has rarely been reported.^13,14 In a review of 144 ultrasound-guided indwelling interscalene catheter placements, a 98% successful block rate with a single complication of small pneumothorax after total shoulder arthroplasty was reported.¹³ Mandim and colleagues¹⁴ reported a case of pneumothorax in a smoker who underwent an ultrasound-guided brachial plexus block prior to open reduction and internal fixation of an ulnar fracture. While the patient was asymptomatic and vital signs remained stable during the procedure, the patient complained postoperatively of chest pain with hypoxia, tachycardia, and hypotension. A chest radiograph confirmed an ipsilateral pneumothorax, and the patient was treated successfully with chest-tube placement. The authors attributed this complication to a higher pleural dome resulting from a hyperinflated lung caused by chronic smoking. Our patient reported no history of smoking and her preoperative chest radiograph had no evidence of lung disease.

In contrast, several cases of pneumothorax after shoulder surgery have been reported in the absence of nerve block. Oldman and Peng¹ reported a 41-year-old nonsmoker who underwent arthroscopic labral repair and subacromial decompression. The preoperative nerve block was cancelled, and the patient received general endotracheal anesthesia alone. Fifty minutes after the case, the patient developed chest pain and hypoxia. A chest radiograph showed a small pneumothorax that was managed conservatively. The pneumothorax was attributed to spontaneous rupture of a preexisting lung bulla, suggesting that blocks are not always the cause of this complication. Furthermore, Dietzel and Ciullo¹⁵ reported 4 cases of spontaneous pneumothorax within 24 hours of uncomplicated arthroscopic shoulder procedures under general anesthesia in the lateral decubitus position. The patient ages ranged from 22 to 38 years, and medical histories were all significant for preexisting lung disease, remote history of pneumonia, and heavy smoking. Three of the patients experienced symptoms at home the day after surgery. The authors concluded that these cases were likely caused by rupture of blebs or bullae from underlying lung disease; these ruptured blebs or bullae are difficult to detect and usually located in the upper lung. The pressure gradient from the positive pressure of anesthesia and the ipsilateral upper lung is thought to be highest in the lateral decubitus position, increasing their chance of rupture.¹⁵

Finally, Lee and colleagues¹⁶ described 3 patients aged 40 to 45 years who underwent uncomplicated subacromial decompression in the beach-chair position under general anesthesia. Significant shoulder, neck, and axillary swelling were noted after surgery, and a chest radiograph showed tension pneumothorax, subcutaneous emphysema, and pneumomediastinum. The authors speculated that pressure in the subacromial space may become negative relative to atmospheric pressure when the shaver and suction are running, drawing in air through other portals. When the suction is discontinued, fluid infusion may push air into the surrounding tissue, leading to subcutaneous emphysema, which may spread to the mediastinum.¹⁶ 

Conclusion 

Ultrasound-guided interscalene nerve blocks have successfully provided anesthesia for shoulder surgeries with low complication rates. Although the incidence of pneumothorax has decreased significantly with ultrasound guidance, the success of this procedure is highly operator-dependent. We present the case of an otherwise healthy patient without known pulmonary disease who developed a tension pneumothorax after the administration of ultrasound-guided regional and general anesthesia for arthroscopic shoulder surgery. Orthopedic surgeons and anesthesiologists must remain vigilant for pneumothorax during the perioperative period after shoulder surgery performed under interscalene regional aesthesia, particularly in the setting of hypotension, hypoxia, and/or tachycardia. Risk factors, such as history of smoking and preexisting lung disease, may predispose patients to the development of pneumothorax. Timely recognition and placement of a chest tube result in satisfactory clinical outcomes.

Interscalene brachial plexus anesthesia is commonly used for arthroscopic and open procedures of the shoulder. This regional anesthetic targets the trunks of the brachial plexus and anesthetizes the area about the shoulder and proximal arm. Its use may obviate the need for concomitant general anesthesia, potentially reducing the use of postoperative intravenous and oral pain medication. Furthermore, patients often bypass the acute postoperative anesthesia care unit and proceed directly to the ambulatory unit, permitting earlier hospital discharge. Previous reports in the literature have demonstrated higher rates of neurologic, cardiac, and pulmonary complications from this procedure; in particular, the incidence of pneumothorax was reported as high as 3%.¹ Techniques to localize the nerves, such as electrical nerve stimulation and, more recently, ultrasound guidance, have reduced these complication rates.^2,3 Successful administration of the block has been shown to result in satisfactory postoperative pain relief.² However, ultrasound-guided interscalene nerve blocks remain operator-dependent and complications may still occur.

We report a case of tension pneumothorax after arthroscopic rotator cuff repair and subacromial decompression with an ultrasound-guided interscalene block. Immediate recognition and treatment of this complication resulted in a good clinical outcome. The patient provided written informed consent for print and electronic publication of this case report. 

Case Report

A 56-year-old woman presented with 3 months of right shoulder pain after a fall. Examination was pertinent for weakness in forward elevation and positive rotator cuff impingement signs. She remained symptomatic despite a course of nonsurgical management that included cortisone injections and physical therapy. Magnetic resonance imaging of the shoulder showed a full-thickness supraspinatus tear with minimal fatty atrophy. After a discussion of her treatment options, she elected to undergo an arthroscopic rotator cuff repair with subacromial decompression. An evaluation by her internist revealed no pertinent medical history apart from obesity (body mass index, 36). Specifically, there was no reported history of chronic obstructive pulmonary disease or asthma. She denied any prior cigarette smoking.

The patient was evaluated by the regional anesthesia team and was classified as a class 2 airway. An interscalene brachial plexus block was performed using a 2-inch, 22-gauge needle inserted into the interscalene groove. Using an out-of-plane technique under direct ultrasound guidance, 30 mL of 0.52% ropivacaine was injected. The block was considered successful, and no complications, such as resistance, paresthesias, pain, or blood on aspiration, were noted during injection. The patient had no complaints of chest pain or shortness of breath immediately afterward, and all vital signs were stable throughout the procedure.

The patient was brought to the operating room and placed in the beach-chair position. Induction for general anesthesia was started 15 minutes after the regional anesthetic, with 2 intubation attempts necessary because of poor airway visualization. After placement of the endotracheal tube, breath sounds were noted to be equal bilaterally. The arthroscopic procedure consisted of double-row rotator cuff repair, subacromial decompression, and débridement of the glenohumeral joint for synovitis, using standard arthroscopic portals. There were no difficulties with trocar placement, and bleeding was minimal throughout the case. The total surgical time was 150 minutes and a pump pressure of 30 mm Hg was maintained during the arthroscopy.

Within the first 60 minutes of the start of the arthroscopic procedure, the patient was noted to be intermittently hypotensive with mean arterial pressure (MAP) ranging from the 30s to 130s mm Hg and pulse in the 70 to 80 beats/min range. FiO₂ in the 85% to 95% range was maintained throughout the procedure. During that time, 50 μg phenylephrine was administered on 4 separate occasions to maintain her blood pressure. The labile blood pressure was attributed by the anesthesiologist to the beach-chair position. During an attempted extubation upon conclusion of the surgery, the patient became hypotensive with MAP that ranged from the 40s to 60s mm Hg and tachycardic to 90 beats/min. The oxygen saturation was in the low 90s and tidal volume was poor. Absent lung sounds were noted on the right chest. An urgent portable chest radiograph showed a large right-sided tension pneumothorax with mediastinal shift (Figure 1). After an immediate general surgery consultation, a chest tube was placed in the operating room. The patient’s vital signs improved and a repeat chest radiograph revealed successful re-expansion of the lung (Figure 2). She was transferred to the acute postoperative anesthesia care unit and extubated in the intensive care unit later that day.

The patient’s chest tube was removed 2 days later and she was discharged home on hospital day 5 with a completely resolved pneumothorax. She was seen 1 week later in the office for a postoperative visit and reported feeling well without chest pain or shortness of breath.

Discussion

Interscalene brachial plexus anesthesia was first described by Winnie⁴ in 1970. This block targets the trunks of the brachial plexus, which are enclosed in a fascial sheath between the anterior and middle scalene muscles. In this region lie several structures at risk: the phrenic nerve superficially and inferiorly; the carotid sheath located superficially and medially; the subclavian artery parallel to the trunks; and the cupula of the lung that lies deep and inferior to the anterior scalene muscle. Recognized complications of the block include vocal hoarseness, Horner syndrome, and hemidiaphragmatic paresis caused by the temporary blockade of the ipsilateral recurrent laryngeal nerve, stellate ganglion, and phrenic nerve, in that order.⁵ Use of the interscalene block has been associated with minimal risk for pneumothorax, because the needle entry point is superior and directed away from the lung pleura.⁶ This is in contrast to the more inferiorly placed supraclavicular block, located in closer proximity to the lung cupula.⁵

Two different approaches are commonly used during ultrasound-guided nerve blocks. The in-plane approach generates a long-axis view of the needle by advancing the needle parallel with the long axis of the ultrasound probe. While this allows direct visualization of the needle tip, it requires deeper needle insertion from lateral to medial, causing puncture of the middle scalene muscle that may increase patient discomfort and risk nerve injury within the muscle.⁷ The out-of-plane approach used on our patient involves needle insertion parallel to the brachial plexus, but along the short axis of the ultrasound probe. Although this permits the operator to assess the periphery of the nerve, it may lead to poor needle-tip visualization during the procedure. As a result, operators often use a combination of tissue disturbance and “hydrolocation,” in which fluid is injected to indicate the needle-tip location.^8,9

Tension pneumothorax represents the accumulation of air in the pleural space that leads to impaired pulmonary and cardiac function. It is often caused by disruption or puncture of the parietal or visceral pleura, creating a connection between the alveoli and pleural cavity. The gradual buildup of air in the pleural cavity results in increased intrapleural pressure, which compresses and ultimately collapses the ipsilateral lung. Venous compression restricts blood return to the heart and reduces cardiac output. Clinical manifestations include dyspnea, hypoxemia, tachycardia, and hypotension.¹⁰ Multiple techniques were developed to better localize the brachial plexus while reducing injury to nearby structures, including the lung. These include eliciting needle paresthesias, electrical nerve stimulation, and ultrasound guidance. While nerve stimulation was once the gold standard for brachial plexus localization, ultrasound guidance has gained in popularity because of its noninvasive nature and dynamic capability to identify nerves and surrounding structures.¹¹ Perlas and colleagues¹² determined the sensitivity of needle paresthesias and nerve stimulation to be 38% and 75%, respectively, in cases in which plexus localization had been confirmed by ultrasound.

Several studies have reported on the efficacy of interscalene nerve block with either nerve stimulation or ultrasound guidance in the setting of shoulder surgery.^2,3 Bishop and colleagues³ reviewed 547 patients who underwent interscalene regional anesthesia with nerve stimulation for both arthroscopic and open-shoulder procedures. They reported a 97% success rate and 12 (2.3%) minor complications, including sensory neuropathy and complex regional pain syndrome. There were no cases of pneumothorax, cardiac events, or other major complications.³ In a prospective study of 1319 patients, Singh and colleagues² reported a 99.6% success rate using ultrasound-guided interscalene blocks for their shoulder surgeries. A total of 38 adverse events (2.88%) were identified: 14 transient neurologic events, including ear numbness, digital numbness, and brachial plexitis; 1 case of intraoperative bradycardia, and 2 cancellations after the block for chest pain and flank pain, which yielded negative cardiac workups. Other complications included postoperative emergency room visits and hospital admissions for reasons unrelated to the block.² Interscalene regional anesthesia, therefore, provides effective anesthesia for shoulder surgery with low complication rates.

Pneumothorax after ultrasound-guided interscalene block has rarely been reported.^13,14 In a review of 144 ultrasound-guided indwelling interscalene catheter placements, a 98% successful block rate with a single complication of small pneumothorax after total shoulder arthroplasty was reported.¹³ Mandim and colleagues¹⁴ reported a case of pneumothorax in a smoker who underwent an ultrasound-guided brachial plexus block prior to open reduction and internal fixation of an ulnar fracture. While the patient was asymptomatic and vital signs remained stable during the procedure, the patient complained postoperatively of chest pain with hypoxia, tachycardia, and hypotension. A chest radiograph confirmed an ipsilateral pneumothorax, and the patient was treated successfully with chest-tube placement. The authors attributed this complication to a higher pleural dome resulting from a hyperinflated lung caused by chronic smoking. Our patient reported no history of smoking and her preoperative chest radiograph had no evidence of lung disease.

In contrast, several cases of pneumothorax after shoulder surgery have been reported in the absence of nerve block. Oldman and Peng¹ reported a 41-year-old nonsmoker who underwent arthroscopic labral repair and subacromial decompression. The preoperative nerve block was cancelled, and the patient received general endotracheal anesthesia alone. Fifty minutes after the case, the patient developed chest pain and hypoxia. A chest radiograph showed a small pneumothorax that was managed conservatively. The pneumothorax was attributed to spontaneous rupture of a preexisting lung bulla, suggesting that blocks are not always the cause of this complication. Furthermore, Dietzel and Ciullo¹⁵ reported 4 cases of spontaneous pneumothorax within 24 hours of uncomplicated arthroscopic shoulder procedures under general anesthesia in the lateral decubitus position. The patient ages ranged from 22 to 38 years, and medical histories were all significant for preexisting lung disease, remote history of pneumonia, and heavy smoking. Three of the patients experienced symptoms at home the day after surgery. The authors concluded that these cases were likely caused by rupture of blebs or bullae from underlying lung disease; these ruptured blebs or bullae are difficult to detect and usually located in the upper lung. The pressure gradient from the positive pressure of anesthesia and the ipsilateral upper lung is thought to be highest in the lateral decubitus position, increasing their chance of rupture.¹⁵

Finally, Lee and colleagues¹⁶ described 3 patients aged 40 to 45 years who underwent uncomplicated subacromial decompression in the beach-chair position under general anesthesia. Significant shoulder, neck, and axillary swelling were noted after surgery, and a chest radiograph showed tension pneumothorax, subcutaneous emphysema, and pneumomediastinum. The authors speculated that pressure in the subacromial space may become negative relative to atmospheric pressure when the shaver and suction are running, drawing in air through other portals. When the suction is discontinued, fluid infusion may push air into the surrounding tissue, leading to subcutaneous emphysema, which may spread to the mediastinum.¹⁶ 

Conclusion 

Ultrasound-guided interscalene nerve blocks have successfully provided anesthesia for shoulder surgeries with low complication rates. Although the incidence of pneumothorax has decreased significantly with ultrasound guidance, the success of this procedure is highly operator-dependent. We present the case of an otherwise healthy patient without known pulmonary disease who developed a tension pneumothorax after the administration of ultrasound-guided regional and general anesthesia for arthroscopic shoulder surgery. Orthopedic surgeons and anesthesiologists must remain vigilant for pneumothorax during the perioperative period after shoulder surgery performed under interscalene regional aesthesia, particularly in the setting of hypotension, hypoxia, and/or tachycardia. Risk factors, such as history of smoking and preexisting lung disease, may predispose patients to the development of pneumothorax. Timely recognition and placement of a chest tube result in satisfactory clinical outcomes.

Spondylolysis, a defect in the pars interarticularis, is the single most common identifiable source of persistent low back pain in adolescent athletes.^1,2 The diagnosis of spondylolysis is confirmed by radiographic imaging.³ However, there is controversy regarding which imaging modality is preferred—specifically, which to use for first-line advanced imaging after plain radiographs are obtained.³ Single-photon emission computed tomography (SPECT) consistently has been shown to be the most sensitive modality, and it is considered the gold standard.^4-7 Patients with a positive SPECT scan are then routinely imaged with computed tomography (CT) for bone detail and staging of the pars defect.⁸ This imaging or diagnostic sequence yields organ-specific radiation doses (15-30 mSv) as much as 50-fold higher than those of plain radiography.⁹ Recent epidemiologic studies have shown that this organ dose results in an increased risk of cancer, especially in children.¹⁰

Diagnosis is crucial in early-stage lumbar spondylolysis, as osseous healing can occur with conservative treatment.^11,12 High signal change (HSC) in the pedicle or pars interarticularis (Figure 1) on fluid-specific (T2) magnetic resonance imaging (MRI) sequences has been shown to be important in the diagnosis of early spondylolysis and, subsequently, a good predictor of bony healing.^13,14 We conducted a study to determine the clinical and radiographic characteristics associated with the diagnosis of early or active spondylolysis.

Materials and Methods

The study was reviewed and approved by the local institutional review board. Using the International Classification of Diseases, Ninth Revision (ICD-9) diagnosis code for spondylolysis (756.11), we retrospectively identified patients (age, 12-21 years) from 2002–2011 billing data from a single specialty spine practice. Baseline data—including height, weight, sex, age, symptom duration, sporting activities, defect location, pain score, and previous treatments—were collected from a standardized patient intake questionnaire and office medical records. We also determined radiographic data, including level, laterality (right vs left, unilateral vs bilateral), presence of listhesis, and slip grade and percentage. CT scans were reviewed to confirm the spondylolysis diagnosis and to measure parameters described by Fujii and colleagues.¹⁵ These parameters include spondylolysis chronicity (early, progressive, terminal) (Figure 2), distance from defect to posterior margin of vertebral body, and defect angle relative to posterior margin of vertebral body. We also measured sagittal radiographic parameters, including pelvic incidence and lumbar lordosis.

Pars lesions were divided into active and inactive defects¹⁶ based on signal characteristics on either MRI or SPECT (Figure 3). Defects with a positive SPECT or HSC on T2 MRI were classified as active; all other defects were classified as inactive. All MRIs were reviewed by a radiologist, and any mention of HSC in the pedicle or pars of the corresponding level was considered positive. For the sake of accuracy, all MRIs were also reviewed by a spine surgeon. All CT measurements were done by 1 of 2 authors. Demographic, clinical, and radiographic characteristics were compared between patients with active defects and patients with inactive defects. Independent t tests and Fisher exact tests were used to compare continuous and categorical variables, respectively. Threshold P was set at .01 to account for the small sample size and multiple concurrent comparisons.

Results

Fifty-seven patients (29 males, 28 females) with a total of 108 pars defects (6 unilateral, 102 bilateral) were identified. Mean age was 14.64 years. Of the 108 defects, 49 were classified as active and 59 as inactive. SPECT results were available for 52 defects, MRI results for 85, and CT results for 76 (Table 1). There was no difference between the active and inactive groups in age (14.7 vs 14.6 years; P = .083), body mass index (24.2 vs 21.7 kg/m²; P = .034), symptom duration (236.3 vs 397.4 days; P = .016), lumbar lordosis (27.4° vs 32.1°; P = .097), pelvic incidence (59.0° vs 61.2°; P = .488), slip percentage (9.5% vs 14.2%; P = .034), and laterality (right vs left, P = .847; unilateral vs bilateral, P = .281) (Table 2). There was a significant difference between the active and inactive groups in sex (35 vs 19 males; P < .0001) and presence of listhesis (16 vs 35; P = .006) (Table 2).

Of the 49 active defects, 3 were graded as early, 10 as progressive, and 11 as terminal (Table 3). There was a statistically significant (P < .0001) difference between active and inactive lesions for each stage. Mean distance from posterior margin of the vertebral body was 0.57 mm and 0.68 mm for inactive and active lesions, respectively (P = .007). There was no significant difference (P = .294) in the posterior angle of the vertebral body and the defect between inactive (20.54°) and active (24.73°) lesions (Table 3).

Subanalysis by sex showed no difference in age (males, 16.4 years vs females, 18.7 years; P = .073), slip percentage (10.4% vs 13.4%; P = .168), or presence or absence of slip (25 vs 26; P > .99) (Table 4).

Discussion

Increasing MRI resolution combined with increasing concern about unnecessary radiation exposure has added to the attractiveness of MRI in the diagnosis of spondylolysis. Spondylolysis progresses on a continuum, starting with a stress reaction (early or active defect) and ending with either healing or nonunion of the pars defect (terminal defect) (Figure 4). Although risk factors for progression are not clearly defined, Fujii and colleagues¹⁵ showed that the reaction around the defect is the most important factor for osseous union. It would then make sense that the earlier the spondylolytic defect is identified, the higher the likelihood for union, especially with nonoperative treatment such as rest, activity restriction, and bracing.^12,17

There is a lack of consensus regarding MRI use in the diagnosis of spondylolysis. Masci and colleagues¹⁸ prospectively evaluated 50 defects in 39 patients using a 1.5-Tesla MRI scanner, concluded MRI is inferior to SPECT/CT, and recommended that SPECT remain the first-line advanced imaging modality. Conversely, Campbell and colleagues⁴ prospectively evaluated 40 defects in 22 patients using a 1.0-Tesla magnet and concluded that MRI can be used as an effective and reliable first-line advanced imaging modality. These are the only 2 prospective studies conducted within the past decade. Both were underpowered and used outdated technology (newer MRI scanners use 3.0-Tesla magnets). In addition, specific imaging characteristics (eg, edema in pars or pedicle on fluid-specific sequences) that suggest a positive finding—versus overt fracture on T1 MRI—have been recently emphasized. Neither Masci and colleagues¹⁸ nor Campbell and colleagues⁴ detailed what constituted a positive MRI finding. Although an adequately powered prospective study will provide a better analysis of the utility of MRI versus SPECT, such a study is costly and time-consuming. It is important to identify patient and lesion characteristics to help optimize the usefulness of MRI. It is also important to identify the subset of patients most likely to experience osseous healing of active defects,¹⁶ as this is the same subset of patients most likely to respond to nonoperative treatment.

We conducted the present study to identify any clinical or radiographic characteristics associated with the diagnosis of early or active spondylolysis. Almost equal numbers of active and inactive defects (49, 59) were identified. There were no differences in patient characteristics, including age, body mass index, and symptom duration. However, there was a significant sex difference—a relatively high proportion of males with active spondylolysis. This finding, which had been reported before,^16,19,20 is probably the result of several factors, including males’ lower lumbar spine bone mineral density²¹; their relatively less spinal flexibility, which affects the distribution of torsional loads on the spine²²; and their relatively greater participation in sports, especially sports involving high-velocity, torsional loading of the lumbar spine.²³ Studies are needed to delineate the extent to which sex influences the development and persistence of active spondylolytic lesions. Alternatively, a subanalysis revealed an age difference, between our female and male cohorts (18.7 vs 16.4 years), that may have contributed to the high proportion of males with active spondylolysis.

Although the groups’ difference in symptom duration was not significant, it was trending toward significance. As discussed, it could be explained that, along the continuum of disease, earlier defects are more active and either achieve fibrous or osseous union or become chronic and “burn out” to inactive lesions, potentially leading to a listhesis.²⁴ The listhesis association was higher in the inactive group than in the active group (P = .006). The difference in numbers of active and inactive defects at each stage (early, progressive, late) confirms this finding, with no inactive lesions in the early and progressive stages and many fewer active lesions in the terminal stage. Overall, presence of a spondylolisthesis on plain radiographs may obviate the need for SPECT or MRI, as it indicates an inactive chronic lesion—unless new symptoms are suspicious for reactivation or development of previously described adjacent-level pars defects.

No other radiographic parameters were found to be significant—consistent with findings of other studies.^2,5,16 Pelvic incidence has been shown to predict progression of spondylisthesis, but under our study parameters it appears not to be associated with development of a slip.

This study had several weaknesses. First, it was retrospective, and imaging parameters were inconsistent, as we included patients who underwent imaging at other facilities. Second, the timing of imaging was inconsistent. Ideally, the same sequence protocol would be used, and all imaging studies (MRI, SPECT, CT) would be performed within a specific period after the initial concern for a spondylolysis was raised. Last, not all patients underwent all 3 advanced imaging modalities; having all 3 would have allowed for a retrospective comparison of MRI and SPECT sensitivity in detecting spondylolysis. Such a comparison would have been interesting, though it was not the goal of this study.

With its technological improvements and lack of radiation exposure, MRI is becoming more attractive as a first-line advanced imaging modality. Although the superiority of MRI over SPECT is yet to be confirmed, clinical use of MRI in the evaluation of spondylolysis seems to be increasing. It is therefore important to characterize the spondylolytic defects that are readily detected with MRI.

Active or early juvenile spondylolysis appears to be associated with males and absence of an associated listhesis. These clinical and radiographic characteristics may be important in the identification of patients with higher potential for osseous healing after nonoperative treatment.

Spondylolysis, a defect in the pars interarticularis, is the single most common identifiable source of persistent low back pain in adolescent athletes.^1,2 The diagnosis of spondylolysis is confirmed by radiographic imaging.³ However, there is controversy regarding which imaging modality is preferred—specifically, which to use for first-line advanced imaging after plain radiographs are obtained.³ Single-photon emission computed tomography (SPECT) consistently has been shown to be the most sensitive modality, and it is considered the gold standard.^4-7 Patients with a positive SPECT scan are then routinely imaged with computed tomography (CT) for bone detail and staging of the pars defect.⁸ This imaging or diagnostic sequence yields organ-specific radiation doses (15-30 mSv) as much as 50-fold higher than those of plain radiography.⁹ Recent epidemiologic studies have shown that this organ dose results in an increased risk of cancer, especially in children.¹⁰

Diagnosis is crucial in early-stage lumbar spondylolysis, as osseous healing can occur with conservative treatment.^11,12 High signal change (HSC) in the pedicle or pars interarticularis (Figure 1) on fluid-specific (T2) magnetic resonance imaging (MRI) sequences has been shown to be important in the diagnosis of early spondylolysis and, subsequently, a good predictor of bony healing.^13,14 We conducted a study to determine the clinical and radiographic characteristics associated with the diagnosis of early or active spondylolysis.

Materials and Methods

The study was reviewed and approved by the local institutional review board. Using the International Classification of Diseases, Ninth Revision (ICD-9) diagnosis code for spondylolysis (756.11), we retrospectively identified patients (age, 12-21 years) from 2002–2011 billing data from a single specialty spine practice. Baseline data—including height, weight, sex, age, symptom duration, sporting activities, defect location, pain score, and previous treatments—were collected from a standardized patient intake questionnaire and office medical records. We also determined radiographic data, including level, laterality (right vs left, unilateral vs bilateral), presence of listhesis, and slip grade and percentage. CT scans were reviewed to confirm the spondylolysis diagnosis and to measure parameters described by Fujii and colleagues.¹⁵ These parameters include spondylolysis chronicity (early, progressive, terminal) (Figure 2), distance from defect to posterior margin of vertebral body, and defect angle relative to posterior margin of vertebral body. We also measured sagittal radiographic parameters, including pelvic incidence and lumbar lordosis.

Pars lesions were divided into active and inactive defects¹⁶ based on signal characteristics on either MRI or SPECT (Figure 3). Defects with a positive SPECT or HSC on T2 MRI were classified as active; all other defects were classified as inactive. All MRIs were reviewed by a radiologist, and any mention of HSC in the pedicle or pars of the corresponding level was considered positive. For the sake of accuracy, all MRIs were also reviewed by a spine surgeon. All CT measurements were done by 1 of 2 authors. Demographic, clinical, and radiographic characteristics were compared between patients with active defects and patients with inactive defects. Independent t tests and Fisher exact tests were used to compare continuous and categorical variables, respectively. Threshold P was set at .01 to account for the small sample size and multiple concurrent comparisons.

Results

Fifty-seven patients (29 males, 28 females) with a total of 108 pars defects (6 unilateral, 102 bilateral) were identified. Mean age was 14.64 years. Of the 108 defects, 49 were classified as active and 59 as inactive. SPECT results were available for 52 defects, MRI results for 85, and CT results for 76 (Table 1). There was no difference between the active and inactive groups in age (14.7 vs 14.6 years; P = .083), body mass index (24.2 vs 21.7 kg/m²; P = .034), symptom duration (236.3 vs 397.4 days; P = .016), lumbar lordosis (27.4° vs 32.1°; P = .097), pelvic incidence (59.0° vs 61.2°; P = .488), slip percentage (9.5% vs 14.2%; P = .034), and laterality (right vs left, P = .847; unilateral vs bilateral, P = .281) (Table 2). There was a significant difference between the active and inactive groups in sex (35 vs 19 males; P < .0001) and presence of listhesis (16 vs 35; P = .006) (Table 2).

Of the 49 active defects, 3 were graded as early, 10 as progressive, and 11 as terminal (Table 3). There was a statistically significant (P < .0001) difference between active and inactive lesions for each stage. Mean distance from posterior margin of the vertebral body was 0.57 mm and 0.68 mm for inactive and active lesions, respectively (P = .007). There was no significant difference (P = .294) in the posterior angle of the vertebral body and the defect between inactive (20.54°) and active (24.73°) lesions (Table 3).

Subanalysis by sex showed no difference in age (males, 16.4 years vs females, 18.7 years; P = .073), slip percentage (10.4% vs 13.4%; P = .168), or presence or absence of slip (25 vs 26; P > .99) (Table 4).

Discussion

Increasing MRI resolution combined with increasing concern about unnecessary radiation exposure has added to the attractiveness of MRI in the diagnosis of spondylolysis. Spondylolysis progresses on a continuum, starting with a stress reaction (early or active defect) and ending with either healing or nonunion of the pars defect (terminal defect) (Figure 4). Although risk factors for progression are not clearly defined, Fujii and colleagues¹⁵ showed that the reaction around the defect is the most important factor for osseous union. It would then make sense that the earlier the spondylolytic defect is identified, the higher the likelihood for union, especially with nonoperative treatment such as rest, activity restriction, and bracing.^12,17

There is a lack of consensus regarding MRI use in the diagnosis of spondylolysis. Masci and colleagues¹⁸ prospectively evaluated 50 defects in 39 patients using a 1.5-Tesla MRI scanner, concluded MRI is inferior to SPECT/CT, and recommended that SPECT remain the first-line advanced imaging modality. Conversely, Campbell and colleagues⁴ prospectively evaluated 40 defects in 22 patients using a 1.0-Tesla magnet and concluded that MRI can be used as an effective and reliable first-line advanced imaging modality. These are the only 2 prospective studies conducted within the past decade. Both were underpowered and used outdated technology (newer MRI scanners use 3.0-Tesla magnets). In addition, specific imaging characteristics (eg, edema in pars or pedicle on fluid-specific sequences) that suggest a positive finding—versus overt fracture on T1 MRI—have been recently emphasized. Neither Masci and colleagues¹⁸ nor Campbell and colleagues⁴ detailed what constituted a positive MRI finding. Although an adequately powered prospective study will provide a better analysis of the utility of MRI versus SPECT, such a study is costly and time-consuming. It is important to identify patient and lesion characteristics to help optimize the usefulness of MRI. It is also important to identify the subset of patients most likely to experience osseous healing of active defects,¹⁶ as this is the same subset of patients most likely to respond to nonoperative treatment.

We conducted the present study to identify any clinical or radiographic characteristics associated with the diagnosis of early or active spondylolysis. Almost equal numbers of active and inactive defects (49, 59) were identified. There were no differences in patient characteristics, including age, body mass index, and symptom duration. However, there was a significant sex difference—a relatively high proportion of males with active spondylolysis. This finding, which had been reported before,^16,19,20 is probably the result of several factors, including males’ lower lumbar spine bone mineral density²¹; their relatively less spinal flexibility, which affects the distribution of torsional loads on the spine²²; and their relatively greater participation in sports, especially sports involving high-velocity, torsional loading of the lumbar spine.²³ Studies are needed to delineate the extent to which sex influences the development and persistence of active spondylolytic lesions. Alternatively, a subanalysis revealed an age difference, between our female and male cohorts (18.7 vs 16.4 years), that may have contributed to the high proportion of males with active spondylolysis.

Although the groups’ difference in symptom duration was not significant, it was trending toward significance. As discussed, it could be explained that, along the continuum of disease, earlier defects are more active and either achieve fibrous or osseous union or become chronic and “burn out” to inactive lesions, potentially leading to a listhesis.²⁴ The listhesis association was higher in the inactive group than in the active group (P = .006). The difference in numbers of active and inactive defects at each stage (early, progressive, late) confirms this finding, with no inactive lesions in the early and progressive stages and many fewer active lesions in the terminal stage. Overall, presence of a spondylolisthesis on plain radiographs may obviate the need for SPECT or MRI, as it indicates an inactive chronic lesion—unless new symptoms are suspicious for reactivation or development of previously described adjacent-level pars defects.

No other radiographic parameters were found to be significant—consistent with findings of other studies.^2,5,16 Pelvic incidence has been shown to predict progression of spondylisthesis, but under our study parameters it appears not to be associated with development of a slip.

This study had several weaknesses. First, it was retrospective, and imaging parameters were inconsistent, as we included patients who underwent imaging at other facilities. Second, the timing of imaging was inconsistent. Ideally, the same sequence protocol would be used, and all imaging studies (MRI, SPECT, CT) would be performed within a specific period after the initial concern for a spondylolysis was raised. Last, not all patients underwent all 3 advanced imaging modalities; having all 3 would have allowed for a retrospective comparison of MRI and SPECT sensitivity in detecting spondylolysis. Such a comparison would have been interesting, though it was not the goal of this study.

With its technological improvements and lack of radiation exposure, MRI is becoming more attractive as a first-line advanced imaging modality. Although the superiority of MRI over SPECT is yet to be confirmed, clinical use of MRI in the evaluation of spondylolysis seems to be increasing. It is therefore important to characterize the spondylolytic defects that are readily detected with MRI.

Active or early juvenile spondylolysis appears to be associated with males and absence of an associated listhesis. These clinical and radiographic characteristics may be important in the identification of patients with higher potential for osseous healing after nonoperative treatment.

Spondylolysis, a defect in the pars interarticularis, is the single most common identifiable source of persistent low back pain in adolescent athletes.^1,2 The diagnosis of spondylolysis is confirmed by radiographic imaging.³ However, there is controversy regarding which imaging modality is preferred—specifically, which to use for first-line advanced imaging after plain radiographs are obtained.³ Single-photon emission computed tomography (SPECT) consistently has been shown to be the most sensitive modality, and it is considered the gold standard.^4-7 Patients with a positive SPECT scan are then routinely imaged with computed tomography (CT) for bone detail and staging of the pars defect.⁸ This imaging or diagnostic sequence yields organ-specific radiation doses (15-30 mSv) as much as 50-fold higher than those of plain radiography.⁹ Recent epidemiologic studies have shown that this organ dose results in an increased risk of cancer, especially in children.¹⁰

Diagnosis is crucial in early-stage lumbar spondylolysis, as osseous healing can occur with conservative treatment.^11,12 High signal change (HSC) in the pedicle or pars interarticularis (Figure 1) on fluid-specific (T2) magnetic resonance imaging (MRI) sequences has been shown to be important in the diagnosis of early spondylolysis and, subsequently, a good predictor of bony healing.^13,14 We conducted a study to determine the clinical and radiographic characteristics associated with the diagnosis of early or active spondylolysis.

Materials and Methods

The study was reviewed and approved by the local institutional review board. Using the International Classification of Diseases, Ninth Revision (ICD-9) diagnosis code for spondylolysis (756.11), we retrospectively identified patients (age, 12-21 years) from 2002–2011 billing data from a single specialty spine practice. Baseline data—including height, weight, sex, age, symptom duration, sporting activities, defect location, pain score, and previous treatments—were collected from a standardized patient intake questionnaire and office medical records. We also determined radiographic data, including level, laterality (right vs left, unilateral vs bilateral), presence of listhesis, and slip grade and percentage. CT scans were reviewed to confirm the spondylolysis diagnosis and to measure parameters described by Fujii and colleagues.¹⁵ These parameters include spondylolysis chronicity (early, progressive, terminal) (Figure 2), distance from defect to posterior margin of vertebral body, and defect angle relative to posterior margin of vertebral body. We also measured sagittal radiographic parameters, including pelvic incidence and lumbar lordosis.

Pars lesions were divided into active and inactive defects¹⁶ based on signal characteristics on either MRI or SPECT (Figure 3). Defects with a positive SPECT or HSC on T2 MRI were classified as active; all other defects were classified as inactive. All MRIs were reviewed by a radiologist, and any mention of HSC in the pedicle or pars of the corresponding level was considered positive. For the sake of accuracy, all MRIs were also reviewed by a spine surgeon. All CT measurements were done by 1 of 2 authors. Demographic, clinical, and radiographic characteristics were compared between patients with active defects and patients with inactive defects. Independent t tests and Fisher exact tests were used to compare continuous and categorical variables, respectively. Threshold P was set at .01 to account for the small sample size and multiple concurrent comparisons.

Results

Fifty-seven patients (29 males, 28 females) with a total of 108 pars defects (6 unilateral, 102 bilateral) were identified. Mean age was 14.64 years. Of the 108 defects, 49 were classified as active and 59 as inactive. SPECT results were available for 52 defects, MRI results for 85, and CT results for 76 (Table 1). There was no difference between the active and inactive groups in age (14.7 vs 14.6 years; P = .083), body mass index (24.2 vs 21.7 kg/m²; P = .034), symptom duration (236.3 vs 397.4 days; P = .016), lumbar lordosis (27.4° vs 32.1°; P = .097), pelvic incidence (59.0° vs 61.2°; P = .488), slip percentage (9.5% vs 14.2%; P = .034), and laterality (right vs left, P = .847; unilateral vs bilateral, P = .281) (Table 2). There was a significant difference between the active and inactive groups in sex (35 vs 19 males; P < .0001) and presence of listhesis (16 vs 35; P = .006) (Table 2).

Of the 49 active defects, 3 were graded as early, 10 as progressive, and 11 as terminal (Table 3). There was a statistically significant (P < .0001) difference between active and inactive lesions for each stage. Mean distance from posterior margin of the vertebral body was 0.57 mm and 0.68 mm for inactive and active lesions, respectively (P = .007). There was no significant difference (P = .294) in the posterior angle of the vertebral body and the defect between inactive (20.54°) and active (24.73°) lesions (Table 3).

Subanalysis by sex showed no difference in age (males, 16.4 years vs females, 18.7 years; P = .073), slip percentage (10.4% vs 13.4%; P = .168), or presence or absence of slip (25 vs 26; P > .99) (Table 4).

Discussion

Increasing MRI resolution combined with increasing concern about unnecessary radiation exposure has added to the attractiveness of MRI in the diagnosis of spondylolysis. Spondylolysis progresses on a continuum, starting with a stress reaction (early or active defect) and ending with either healing or nonunion of the pars defect (terminal defect) (Figure 4). Although risk factors for progression are not clearly defined, Fujii and colleagues¹⁵ showed that the reaction around the defect is the most important factor for osseous union. It would then make sense that the earlier the spondylolytic defect is identified, the higher the likelihood for union, especially with nonoperative treatment such as rest, activity restriction, and bracing.^12,17

There is a lack of consensus regarding MRI use in the diagnosis of spondylolysis. Masci and colleagues¹⁸ prospectively evaluated 50 defects in 39 patients using a 1.5-Tesla MRI scanner, concluded MRI is inferior to SPECT/CT, and recommended that SPECT remain the first-line advanced imaging modality. Conversely, Campbell and colleagues⁴ prospectively evaluated 40 defects in 22 patients using a 1.0-Tesla magnet and concluded that MRI can be used as an effective and reliable first-line advanced imaging modality. These are the only 2 prospective studies conducted within the past decade. Both were underpowered and used outdated technology (newer MRI scanners use 3.0-Tesla magnets). In addition, specific imaging characteristics (eg, edema in pars or pedicle on fluid-specific sequences) that suggest a positive finding—versus overt fracture on T1 MRI—have been recently emphasized. Neither Masci and colleagues¹⁸ nor Campbell and colleagues⁴ detailed what constituted a positive MRI finding. Although an adequately powered prospective study will provide a better analysis of the utility of MRI versus SPECT, such a study is costly and time-consuming. It is important to identify patient and lesion characteristics to help optimize the usefulness of MRI. It is also important to identify the subset of patients most likely to experience osseous healing of active defects,¹⁶ as this is the same subset of patients most likely to respond to nonoperative treatment.

We conducted the present study to identify any clinical or radiographic characteristics associated with the diagnosis of early or active spondylolysis. Almost equal numbers of active and inactive defects (49, 59) were identified. There were no differences in patient characteristics, including age, body mass index, and symptom duration. However, there was a significant sex difference—a relatively high proportion of males with active spondylolysis. This finding, which had been reported before,^16,19,20 is probably the result of several factors, including males’ lower lumbar spine bone mineral density²¹; their relatively less spinal flexibility, which affects the distribution of torsional loads on the spine²²; and their relatively greater participation in sports, especially sports involving high-velocity, torsional loading of the lumbar spine.²³ Studies are needed to delineate the extent to which sex influences the development and persistence of active spondylolytic lesions. Alternatively, a subanalysis revealed an age difference, between our female and male cohorts (18.7 vs 16.4 years), that may have contributed to the high proportion of males with active spondylolysis.

Although the groups’ difference in symptom duration was not significant, it was trending toward significance. As discussed, it could be explained that, along the continuum of disease, earlier defects are more active and either achieve fibrous or osseous union or become chronic and “burn out” to inactive lesions, potentially leading to a listhesis.²⁴ The listhesis association was higher in the inactive group than in the active group (P = .006). The difference in numbers of active and inactive defects at each stage (early, progressive, late) confirms this finding, with no inactive lesions in the early and progressive stages and many fewer active lesions in the terminal stage. Overall, presence of a spondylolisthesis on plain radiographs may obviate the need for SPECT or MRI, as it indicates an inactive chronic lesion—unless new symptoms are suspicious for reactivation or development of previously described adjacent-level pars defects.

No other radiographic parameters were found to be significant—consistent with findings of other studies.^2,5,16 Pelvic incidence has been shown to predict progression of spondylisthesis, but under our study parameters it appears not to be associated with development of a slip.

This study had several weaknesses. First, it was retrospective, and imaging parameters were inconsistent, as we included patients who underwent imaging at other facilities. Second, the timing of imaging was inconsistent. Ideally, the same sequence protocol would be used, and all imaging studies (MRI, SPECT, CT) would be performed within a specific period after the initial concern for a spondylolysis was raised. Last, not all patients underwent all 3 advanced imaging modalities; having all 3 would have allowed for a retrospective comparison of MRI and SPECT sensitivity in detecting spondylolysis. Such a comparison would have been interesting, though it was not the goal of this study.

With its technological improvements and lack of radiation exposure, MRI is becoming more attractive as a first-line advanced imaging modality. Although the superiority of MRI over SPECT is yet to be confirmed, clinical use of MRI in the evaluation of spondylolysis seems to be increasing. It is therefore important to characterize the spondylolytic defects that are readily detected with MRI.

Active or early juvenile spondylolysis appears to be associated with males and absence of an associated listhesis. These clinical and radiographic characteristics may be important in the identification of patients with higher potential for osseous healing after nonoperative treatment.

Osteochondromas are common benign bone tumors composed of a bony protrusion with an overlying cartilage cap.¹ This lesion constitutes 24% to 40% of all benign bone tumors, and the great majority arise from the metaphyseal region of long bones.² The scapula accounts for only 3% to 5% of all osteochondromas; however, this lesion is the most common benign bone tumor to involve the scapula.³

In contrast, cutaneous bronchogenic cyst of the scapula is an exceedingly rare pathology. The bronchogenic cyst is a congenital cystic mass lined by tracheobronchial structures and respiratory epithelium.⁴ These are most commonly located in the thorax, although numerous remote locations have also been described, including cutaneous cysts.⁵ The overall incidence of bronchogenic cysts is thought to be 1 in 42,000 to 1 in 68,000.⁶ There are only 15 case reports of cutaneous bronchogenic cysts in the scapular region.⁷

We report the case of a novel dual lesion of both an osteochondroma and a contiguous cutaneous bronchogenic cyst in the scapula. The patient’s guardian provided written informed consent for print and electronic publication of this case report.

Case Report

A 12-month-old boy presented to our clinic with the complaint of a mass over the left scapula. The mass was first noted incidentally several weeks earlier during bathing. Examination revealed a firm, subcutaneous, nontender mass measuring 1×2 cm located over the spine of the scapula. There were no overlying skin changes, and there was normal function of the ipsilateral upper extremity. Anteroposterior and lateral chest radiographs revealed no abnormality. Magnetic resonance imaging (MRI) showed an exostosis projecting from the scapular spine measuring 2×6×7 mm with an adjacent cystic mass measuring 5×8×9 mm that was thought to represent bursitis (Figure 1). The decision was made to observe the mass. 

The patient returned to clinic at age 31 months with a new complaint of scant drainage of serous fluid from a pinprick-sized hole located just superolateral to the scapular mass. The child’s mother reported daily manual expression of fluid from the mass via the hole, without which the mass would enlarge. There were no local or systemic signs of infection. A repeat MRI again revealed an exostosis with an adjacent cystic mass with interval enlargement of the cyst (Figure 2). At age 4.5 years, the decision was made to proceed with excision of the osteochondroma and adjacent cystic mass.

The mass was approached via a 2-cm incision designed to excise the tract to the skin. Dissection revealed a sinus tract connecting to a well-defined cystic sac. This sac was attached to the underlying exostosis. The exostosis and attached cyst were excised en bloc. The cyst was opened, revealing foul-smelling, cloudy white fluid that was sent for culture; the specimen was sent for pathology.

The fluid culture grew mixed flora, with no Staphylococcus aureus, group A streptococcus, or Pseudomonas aeruginosa identified. The pathologic examination identified bone with a cartilaginous cap, consistent with osteochondroma (Figure 3), as well as a cyst lined by respiratory epithelium with patchy areas of squamous epithelium and surrounding mucus glands, consistent with bronchogenic cyst (Figure 4). Figure 5 shows the contiguous nature of the 2 lesions.

The postoperative course was uneventful. The patient returned to full use of the left upper extremity and had resolution of all drainage. 

Discussion

Osteochondromas are thought to arise from aberrant growth of the epiphyseal growth plate cartilage. A small portion of the physis herniates past the groove of Ranvier and grows parallel to the normal physis with medullary continuity. This can occur idiopathically or, more rarely, secondary to an identified injury to the growth plate.¹

The formation of bronchogenic cysts is most often attributed to anomalous budding of the ventral foregut during fetal development,⁴ hence the alternative designation of these cysts as foregut cysts. An extrathoracic location of the cyst has been postulated to stem from 2 possible events: a preexisting cyst may migrate out of the thorax prior to closure of the sternal plates, or sternal plate closure may itself pinch off the cyst.^8,9 An alternative explanation is in situ metaplastic development of respiratory epithelium.¹⁰ When located near the skin, these cysts often drain clear fluid.¹¹

Scapular osteochondromas are known to cause various pathologies of the shoulder girdle, including snapping scapula syndrome, chest wall deformity, shoulder impingement, and bursa formation.^12-17 This case, however, is the first known finding of a scapular osteochondroma with a contiguous cutaneous bronchogenic cyst. A putative explanation for their co-occurrence is that local disturbances caused by one lesion stimulated the formation of the second. The direct connection between the bronchogenic cyst and the bone, as has been reported in 3 cases,^7,9,18 seems to favor this explanation. Definitive conclusions regarding any causal relationship are beyond the scope of this single case report.

Definitive management of bronchogenic cysts is complete excision, although the diagnosis is often not made until histopathologic examination has been completed.¹⁹ Osteochondromas are managed with observation unless they are symptomatic.² Malignant degeneration is a rare but documented occurrence in both lesions.^2,20

Conclusion

In approaching the pediatric patient with a cystic mass over the scapula, a cutaneous bronchogenic cyst may be included in the differential diagnosis. This lesion can occur in isolation or can be found with another pathology, such as osteochondroma, as reported here.

Osteochondromas are common benign bone tumors composed of a bony protrusion with an overlying cartilage cap.¹ This lesion constitutes 24% to 40% of all benign bone tumors, and the great majority arise from the metaphyseal region of long bones.² The scapula accounts for only 3% to 5% of all osteochondromas; however, this lesion is the most common benign bone tumor to involve the scapula.³

In contrast, cutaneous bronchogenic cyst of the scapula is an exceedingly rare pathology. The bronchogenic cyst is a congenital cystic mass lined by tracheobronchial structures and respiratory epithelium.⁴ These are most commonly located in the thorax, although numerous remote locations have also been described, including cutaneous cysts.⁵ The overall incidence of bronchogenic cysts is thought to be 1 in 42,000 to 1 in 68,000.⁶ There are only 15 case reports of cutaneous bronchogenic cysts in the scapular region.⁷

We report the case of a novel dual lesion of both an osteochondroma and a contiguous cutaneous bronchogenic cyst in the scapula. The patient’s guardian provided written informed consent for print and electronic publication of this case report.

Case Report

A 12-month-old boy presented to our clinic with the complaint of a mass over the left scapula. The mass was first noted incidentally several weeks earlier during bathing. Examination revealed a firm, subcutaneous, nontender mass measuring 1×2 cm located over the spine of the scapula. There were no overlying skin changes, and there was normal function of the ipsilateral upper extremity. Anteroposterior and lateral chest radiographs revealed no abnormality. Magnetic resonance imaging (MRI) showed an exostosis projecting from the scapular spine measuring 2×6×7 mm with an adjacent cystic mass measuring 5×8×9 mm that was thought to represent bursitis (Figure 1). The decision was made to observe the mass. 

The patient returned to clinic at age 31 months with a new complaint of scant drainage of serous fluid from a pinprick-sized hole located just superolateral to the scapular mass. The child’s mother reported daily manual expression of fluid from the mass via the hole, without which the mass would enlarge. There were no local or systemic signs of infection. A repeat MRI again revealed an exostosis with an adjacent cystic mass with interval enlargement of the cyst (Figure 2). At age 4.5 years, the decision was made to proceed with excision of the osteochondroma and adjacent cystic mass.

The mass was approached via a 2-cm incision designed to excise the tract to the skin. Dissection revealed a sinus tract connecting to a well-defined cystic sac. This sac was attached to the underlying exostosis. The exostosis and attached cyst were excised en bloc. The cyst was opened, revealing foul-smelling, cloudy white fluid that was sent for culture; the specimen was sent for pathology.

The fluid culture grew mixed flora, with no Staphylococcus aureus, group A streptococcus, or Pseudomonas aeruginosa identified. The pathologic examination identified bone with a cartilaginous cap, consistent with osteochondroma (Figure 3), as well as a cyst lined by respiratory epithelium with patchy areas of squamous epithelium and surrounding mucus glands, consistent with bronchogenic cyst (Figure 4). Figure 5 shows the contiguous nature of the 2 lesions.

The postoperative course was uneventful. The patient returned to full use of the left upper extremity and had resolution of all drainage. 

Discussion

Osteochondromas are thought to arise from aberrant growth of the epiphyseal growth plate cartilage. A small portion of the physis herniates past the groove of Ranvier and grows parallel to the normal physis with medullary continuity. This can occur idiopathically or, more rarely, secondary to an identified injury to the growth plate.¹

The formation of bronchogenic cysts is most often attributed to anomalous budding of the ventral foregut during fetal development,⁴ hence the alternative designation of these cysts as foregut cysts. An extrathoracic location of the cyst has been postulated to stem from 2 possible events: a preexisting cyst may migrate out of the thorax prior to closure of the sternal plates, or sternal plate closure may itself pinch off the cyst.^8,9 An alternative explanation is in situ metaplastic development of respiratory epithelium.¹⁰ When located near the skin, these cysts often drain clear fluid.¹¹

Scapular osteochondromas are known to cause various pathologies of the shoulder girdle, including snapping scapula syndrome, chest wall deformity, shoulder impingement, and bursa formation.^12-17 This case, however, is the first known finding of a scapular osteochondroma with a contiguous cutaneous bronchogenic cyst. A putative explanation for their co-occurrence is that local disturbances caused by one lesion stimulated the formation of the second. The direct connection between the bronchogenic cyst and the bone, as has been reported in 3 cases,^7,9,18 seems to favor this explanation. Definitive conclusions regarding any causal relationship are beyond the scope of this single case report.

Definitive management of bronchogenic cysts is complete excision, although the diagnosis is often not made until histopathologic examination has been completed.¹⁹ Osteochondromas are managed with observation unless they are symptomatic.² Malignant degeneration is a rare but documented occurrence in both lesions.^2,20

Conclusion

In approaching the pediatric patient with a cystic mass over the scapula, a cutaneous bronchogenic cyst may be included in the differential diagnosis. This lesion can occur in isolation or can be found with another pathology, such as osteochondroma, as reported here.

Osteochondromas are common benign bone tumors composed of a bony protrusion with an overlying cartilage cap.¹ This lesion constitutes 24% to 40% of all benign bone tumors, and the great majority arise from the metaphyseal region of long bones.² The scapula accounts for only 3% to 5% of all osteochondromas; however, this lesion is the most common benign bone tumor to involve the scapula.³

In contrast, cutaneous bronchogenic cyst of the scapula is an exceedingly rare pathology. The bronchogenic cyst is a congenital cystic mass lined by tracheobronchial structures and respiratory epithelium.⁴ These are most commonly located in the thorax, although numerous remote locations have also been described, including cutaneous cysts.⁵ The overall incidence of bronchogenic cysts is thought to be 1 in 42,000 to 1 in 68,000.⁶ There are only 15 case reports of cutaneous bronchogenic cysts in the scapular region.⁷

We report the case of a novel dual lesion of both an osteochondroma and a contiguous cutaneous bronchogenic cyst in the scapula. The patient’s guardian provided written informed consent for print and electronic publication of this case report.

Case Report

A 12-month-old boy presented to our clinic with the complaint of a mass over the left scapula. The mass was first noted incidentally several weeks earlier during bathing. Examination revealed a firm, subcutaneous, nontender mass measuring 1×2 cm located over the spine of the scapula. There were no overlying skin changes, and there was normal function of the ipsilateral upper extremity. Anteroposterior and lateral chest radiographs revealed no abnormality. Magnetic resonance imaging (MRI) showed an exostosis projecting from the scapular spine measuring 2×6×7 mm with an adjacent cystic mass measuring 5×8×9 mm that was thought to represent bursitis (Figure 1). The decision was made to observe the mass. 

The patient returned to clinic at age 31 months with a new complaint of scant drainage of serous fluid from a pinprick-sized hole located just superolateral to the scapular mass. The child’s mother reported daily manual expression of fluid from the mass via the hole, without which the mass would enlarge. There were no local or systemic signs of infection. A repeat MRI again revealed an exostosis with an adjacent cystic mass with interval enlargement of the cyst (Figure 2). At age 4.5 years, the decision was made to proceed with excision of the osteochondroma and adjacent cystic mass.

The mass was approached via a 2-cm incision designed to excise the tract to the skin. Dissection revealed a sinus tract connecting to a well-defined cystic sac. This sac was attached to the underlying exostosis. The exostosis and attached cyst were excised en bloc. The cyst was opened, revealing foul-smelling, cloudy white fluid that was sent for culture; the specimen was sent for pathology.

The fluid culture grew mixed flora, with no Staphylococcus aureus, group A streptococcus, or Pseudomonas aeruginosa identified. The pathologic examination identified bone with a cartilaginous cap, consistent with osteochondroma (Figure 3), as well as a cyst lined by respiratory epithelium with patchy areas of squamous epithelium and surrounding mucus glands, consistent with bronchogenic cyst (Figure 4). Figure 5 shows the contiguous nature of the 2 lesions.

The postoperative course was uneventful. The patient returned to full use of the left upper extremity and had resolution of all drainage. 

Discussion

Osteochondromas are thought to arise from aberrant growth of the epiphyseal growth plate cartilage. A small portion of the physis herniates past the groove of Ranvier and grows parallel to the normal physis with medullary continuity. This can occur idiopathically or, more rarely, secondary to an identified injury to the growth plate.¹

The formation of bronchogenic cysts is most often attributed to anomalous budding of the ventral foregut during fetal development,⁴ hence the alternative designation of these cysts as foregut cysts. An extrathoracic location of the cyst has been postulated to stem from 2 possible events: a preexisting cyst may migrate out of the thorax prior to closure of the sternal plates, or sternal plate closure may itself pinch off the cyst.^8,9 An alternative explanation is in situ metaplastic development of respiratory epithelium.¹⁰ When located near the skin, these cysts often drain clear fluid.¹¹

Scapular osteochondromas are known to cause various pathologies of the shoulder girdle, including snapping scapula syndrome, chest wall deformity, shoulder impingement, and bursa formation.^12-17 This case, however, is the first known finding of a scapular osteochondroma with a contiguous cutaneous bronchogenic cyst. A putative explanation for their co-occurrence is that local disturbances caused by one lesion stimulated the formation of the second. The direct connection between the bronchogenic cyst and the bone, as has been reported in 3 cases,^7,9,18 seems to favor this explanation. Definitive conclusions regarding any causal relationship are beyond the scope of this single case report.

Definitive management of bronchogenic cysts is complete excision, although the diagnosis is often not made until histopathologic examination has been completed.¹⁹ Osteochondromas are managed with observation unless they are symptomatic.² Malignant degeneration is a rare but documented occurrence in both lesions.^2,20

Conclusion

In approaching the pediatric patient with a cystic mass over the scapula, a cutaneous bronchogenic cyst may be included in the differential diagnosis. This lesion can occur in isolation or can be found with another pathology, such as osteochondroma, as reported here.