Using quotes to ensure that the results were only those that include the two words adjacent to one another, rather than separated, I entered the following phrases into my Google search engine:

• “hospitalist burnout” = 1,580 results
• “hospitalist morale” = 208 results
• “hospitalist well-being” = 0 results

I think the number of results suggests the level of interest in each topic and, if that is the case, clearly thinking about how hospitalists are doing in their careers is more commonly done through the paradigm of burnout than the other two terms. (Of course, there may be other terms that I didn’t consider.) In fact, there have been a handful of published studies of hospitalist burnout and job satisfaction.^1,2

Those studies generally have shown both reasonably high levels of job satisfaction and troubling levels of burnout.

But I’ve been thinking about hospitalist morale for a while. I think morale is reasonably distinct from both burnout and job satisfaction.

Causes of a National Decline in Hospitalist Morale

I think hospitalist morale has declined some over the past two or three years across the country. This observation is meaningful because it comes from my experience working with a lot of hospitalist groups coast to coast. But I’m the first to admit it is just anecdotal and is subject to my own biases.

I can think of several things contributing to a decline in morale.

EHR adoption. Near the top of the list is the adoption of EHRs in many hospitals, which typically leads doctors in other specialties to seek hospitalist assistance with EHR-related tasks (e.g. medicine reconciliation and order writing) even in cases where there is little or no clinical reason for hospitalist involvement. Lots of hospitalists complain about this. To be clear, in many hospitals the hospitalists are reasonably content with using the EHR, but they experience ongoing frustration and low morale resulting from nonclinical work other doctors pressure them to take over.

Observation status. Many hospitals began classifying a larger portion of patients as observation status over the last few years; at the same time, patients and families have become more aware of how much of a disadvantage this is. In many cases, it is the hospitalist who takes the brunt of patient and family frustration. This can get awfully stressful and frustrating, and I think it is a contributor to allegations of malpractice.

Budgetary stress. Ever since SHM began collecting survey data in the late 1990s, the financial support hospitals have been providing to hospitalists has increased dramatically. The most recent State of Hospital Medicine report, published in 2104, showed median support provided by hospitals of $156,063 per FTE hospitalist, per year. Some hospitals have begun to resist providing more support, and this translates into stress and lower morale for hospitalists. This is far from a universal issue, but it does lead to lower morale for hospitalists who face it.

Many other factors may be contributing to a national decline in morale, but I think these are some of the most important.

What Can Be Done?

Some hospitalist groups have great morale now and don’t need to do much of anything right now, but some groups should think about a deliberate strategy to improve it.

Sadly, there isn’t a prescription that is sure to work. But there are some things you can try.

Self-care. The field of palliative care has thought a lot about caring for caregivers, and hospitalist groups might want to adopt some of their practices. Search the Internet on “self-care” + “palliative care,” and you’ll find a lot of interesting things. The group I’m part of launched a deliberate program of professionally led and facilitated hospitalist self-care, with high hopes that included mindful meditation, among other things. As soon as we had designed our program, the Mayo Clinic published their favorable experience with a program that was very similar to what we had planned, and I thought we would see similar benefits.³

But, while all who attended the sessions thought they were valuable, attendance was so poor that we ended up cancelling the program. The hospitalists were interested in attending but were either on service and busy seeing patients, or were off and didn’t want to drive in to work solely for the purpose of reducing work stress.

I’m convinced a self-care program is valuable but very tricky to schedule effectively. Maybe others have come up with effective ways of overcoming this problem.

Social connections. Some hospitalist groups seem to have little social and personal connection to other physicians and hospital leaders. I think this results in lower hospitalist morale and tends to be self-reinforcing. If you’re in such a group, you and your hospitalist colleagues should deliberately seek better relationships with other doctors and hospital administrative leaders. Ensure that you visit with others at lunch, talk with them at committee meetings, ask about their vacation and personal activities, and pursue activities with them outside of work.

When these sorts of social connections are strong, work is far more satisfying and you’re much more likely to be treated as a peer by other doctors. I think this is really important and shouldn’t be overlooked if your group is suffering from low morale.

Adaptive work. Lastly, you might want to approach changes to your work and morale as “adaptive work,” rather than “technical work.” Space doesn’t permit a description of these, but it is worth reading about how they differ. Many groups will find value in reframing their approach to aspects of work they don’t like as adaptive work.

References

1. Hinami K, Whelan CT, Wolosin RJ, Miller JA, Wetterneck TB. Worklife and satisfaction of hospitalists: toward flourishing careers. J Gen Intern Med. 2012;27(1):28-36.
2. Hoff TH, Whitcomb WF, Williams K, Nelson JR, Cheesman RA. Characteristics and work experiences of hospitalists in the United States. Arch Intern Med. 2001;161(6):851-858.
3. West CP, Dyrbye LN, Rabatin JT, et al. Intervention to promote physician well-being, job satisfaction, and professionalism: a randomized clinical trial. JAMA Intern Med. 2014;174(4):527-533.

Using quotes to ensure that the results were only those that include the two words adjacent to one another, rather than separated, I entered the following phrases into my Google search engine:

• “hospitalist burnout” = 1,580 results
• “hospitalist morale” = 208 results
• “hospitalist well-being” = 0 results

I think the number of results suggests the level of interest in each topic and, if that is the case, clearly thinking about how hospitalists are doing in their careers is more commonly done through the paradigm of burnout than the other two terms. (Of course, there may be other terms that I didn’t consider.) In fact, there have been a handful of published studies of hospitalist burnout and job satisfaction.^1,2

Those studies generally have shown both reasonably high levels of job satisfaction and troubling levels of burnout.

But I’ve been thinking about hospitalist morale for a while. I think morale is reasonably distinct from both burnout and job satisfaction.

Causes of a National Decline in Hospitalist Morale

I think hospitalist morale has declined some over the past two or three years across the country. This observation is meaningful because it comes from my experience working with a lot of hospitalist groups coast to coast. But I’m the first to admit it is just anecdotal and is subject to my own biases.

I can think of several things contributing to a decline in morale.

EHR adoption. Near the top of the list is the adoption of EHRs in many hospitals, which typically leads doctors in other specialties to seek hospitalist assistance with EHR-related tasks (e.g. medicine reconciliation and order writing) even in cases where there is little or no clinical reason for hospitalist involvement. Lots of hospitalists complain about this. To be clear, in many hospitals the hospitalists are reasonably content with using the EHR, but they experience ongoing frustration and low morale resulting from nonclinical work other doctors pressure them to take over.

Observation status. Many hospitals began classifying a larger portion of patients as observation status over the last few years; at the same time, patients and families have become more aware of how much of a disadvantage this is. In many cases, it is the hospitalist who takes the brunt of patient and family frustration. This can get awfully stressful and frustrating, and I think it is a contributor to allegations of malpractice.

Budgetary stress. Ever since SHM began collecting survey data in the late 1990s, the financial support hospitals have been providing to hospitalists has increased dramatically. The most recent State of Hospital Medicine report, published in 2104, showed median support provided by hospitals of $156,063 per FTE hospitalist, per year. Some hospitals have begun to resist providing more support, and this translates into stress and lower morale for hospitalists. This is far from a universal issue, but it does lead to lower morale for hospitalists who face it.

Many other factors may be contributing to a national decline in morale, but I think these are some of the most important.

What Can Be Done?

Some hospitalist groups have great morale now and don’t need to do much of anything right now, but some groups should think about a deliberate strategy to improve it.

Sadly, there isn’t a prescription that is sure to work. But there are some things you can try.

Self-care. The field of palliative care has thought a lot about caring for caregivers, and hospitalist groups might want to adopt some of their practices. Search the Internet on “self-care” + “palliative care,” and you’ll find a lot of interesting things. The group I’m part of launched a deliberate program of professionally led and facilitated hospitalist self-care, with high hopes that included mindful meditation, among other things. As soon as we had designed our program, the Mayo Clinic published their favorable experience with a program that was very similar to what we had planned, and I thought we would see similar benefits.³

But, while all who attended the sessions thought they were valuable, attendance was so poor that we ended up cancelling the program. The hospitalists were interested in attending but were either on service and busy seeing patients, or were off and didn’t want to drive in to work solely for the purpose of reducing work stress.

I’m convinced a self-care program is valuable but very tricky to schedule effectively. Maybe others have come up with effective ways of overcoming this problem.

Social connections. Some hospitalist groups seem to have little social and personal connection to other physicians and hospital leaders. I think this results in lower hospitalist morale and tends to be self-reinforcing. If you’re in such a group, you and your hospitalist colleagues should deliberately seek better relationships with other doctors and hospital administrative leaders. Ensure that you visit with others at lunch, talk with them at committee meetings, ask about their vacation and personal activities, and pursue activities with them outside of work.

When these sorts of social connections are strong, work is far more satisfying and you’re much more likely to be treated as a peer by other doctors. I think this is really important and shouldn’t be overlooked if your group is suffering from low morale.

Adaptive work. Lastly, you might want to approach changes to your work and morale as “adaptive work,” rather than “technical work.” Space doesn’t permit a description of these, but it is worth reading about how they differ. Many groups will find value in reframing their approach to aspects of work they don’t like as adaptive work.

References

1. Hinami K, Whelan CT, Wolosin RJ, Miller JA, Wetterneck TB. Worklife and satisfaction of hospitalists: toward flourishing careers. J Gen Intern Med. 2012;27(1):28-36.
2. Hoff TH, Whitcomb WF, Williams K, Nelson JR, Cheesman RA. Characteristics and work experiences of hospitalists in the United States. Arch Intern Med. 2001;161(6):851-858.
3. West CP, Dyrbye LN, Rabatin JT, et al. Intervention to promote physician well-being, job satisfaction, and professionalism: a randomized clinical trial. JAMA Intern Med. 2014;174(4):527-533.

Using quotes to ensure that the results were only those that include the two words adjacent to one another, rather than separated, I entered the following phrases into my Google search engine:

• “hospitalist burnout” = 1,580 results
• “hospitalist morale” = 208 results
• “hospitalist well-being” = 0 results

I think the number of results suggests the level of interest in each topic and, if that is the case, clearly thinking about how hospitalists are doing in their careers is more commonly done through the paradigm of burnout than the other two terms. (Of course, there may be other terms that I didn’t consider.) In fact, there have been a handful of published studies of hospitalist burnout and job satisfaction.^1,2

Those studies generally have shown both reasonably high levels of job satisfaction and troubling levels of burnout.

But I’ve been thinking about hospitalist morale for a while. I think morale is reasonably distinct from both burnout and job satisfaction.

Causes of a National Decline in Hospitalist Morale

I think hospitalist morale has declined some over the past two or three years across the country. This observation is meaningful because it comes from my experience working with a lot of hospitalist groups coast to coast. But I’m the first to admit it is just anecdotal and is subject to my own biases.

I can think of several things contributing to a decline in morale.

EHR adoption. Near the top of the list is the adoption of EHRs in many hospitals, which typically leads doctors in other specialties to seek hospitalist assistance with EHR-related tasks (e.g. medicine reconciliation and order writing) even in cases where there is little or no clinical reason for hospitalist involvement. Lots of hospitalists complain about this. To be clear, in many hospitals the hospitalists are reasonably content with using the EHR, but they experience ongoing frustration and low morale resulting from nonclinical work other doctors pressure them to take over.

Observation status. Many hospitals began classifying a larger portion of patients as observation status over the last few years; at the same time, patients and families have become more aware of how much of a disadvantage this is. In many cases, it is the hospitalist who takes the brunt of patient and family frustration. This can get awfully stressful and frustrating, and I think it is a contributor to allegations of malpractice.

Budgetary stress. Ever since SHM began collecting survey data in the late 1990s, the financial support hospitals have been providing to hospitalists has increased dramatically. The most recent State of Hospital Medicine report, published in 2104, showed median support provided by hospitals of $156,063 per FTE hospitalist, per year. Some hospitals have begun to resist providing more support, and this translates into stress and lower morale for hospitalists. This is far from a universal issue, but it does lead to lower morale for hospitalists who face it.

Many other factors may be contributing to a national decline in morale, but I think these are some of the most important.

What Can Be Done?

Some hospitalist groups have great morale now and don’t need to do much of anything right now, but some groups should think about a deliberate strategy to improve it.

Sadly, there isn’t a prescription that is sure to work. But there are some things you can try.

Self-care. The field of palliative care has thought a lot about caring for caregivers, and hospitalist groups might want to adopt some of their practices. Search the Internet on “self-care” + “palliative care,” and you’ll find a lot of interesting things. The group I’m part of launched a deliberate program of professionally led and facilitated hospitalist self-care, with high hopes that included mindful meditation, among other things. As soon as we had designed our program, the Mayo Clinic published their favorable experience with a program that was very similar to what we had planned, and I thought we would see similar benefits.³

But, while all who attended the sessions thought they were valuable, attendance was so poor that we ended up cancelling the program. The hospitalists were interested in attending but were either on service and busy seeing patients, or were off and didn’t want to drive in to work solely for the purpose of reducing work stress.

I’m convinced a self-care program is valuable but very tricky to schedule effectively. Maybe others have come up with effective ways of overcoming this problem.

Social connections. Some hospitalist groups seem to have little social and personal connection to other physicians and hospital leaders. I think this results in lower hospitalist morale and tends to be self-reinforcing. If you’re in such a group, you and your hospitalist colleagues should deliberately seek better relationships with other doctors and hospital administrative leaders. Ensure that you visit with others at lunch, talk with them at committee meetings, ask about their vacation and personal activities, and pursue activities with them outside of work.

When these sorts of social connections are strong, work is far more satisfying and you’re much more likely to be treated as a peer by other doctors. I think this is really important and shouldn’t be overlooked if your group is suffering from low morale.

Adaptive work. Lastly, you might want to approach changes to your work and morale as “adaptive work,” rather than “technical work.” Space doesn’t permit a description of these, but it is worth reading about how they differ. Many groups will find value in reframing their approach to aspects of work they don’t like as adaptive work.

References

1. Hinami K, Whelan CT, Wolosin RJ, Miller JA, Wetterneck TB. Worklife and satisfaction of hospitalists: toward flourishing careers. J Gen Intern Med. 2012;27(1):28-36.
2. Hoff TH, Whitcomb WF, Williams K, Nelson JR, Cheesman RA. Characteristics and work experiences of hospitalists in the United States. Arch Intern Med. 2001;161(6):851-858.
3. West CP, Dyrbye LN, Rabatin JT, et al. Intervention to promote physician well-being, job satisfaction, and professionalism: a randomized clinical trial. JAMA Intern Med. 2014;174(4):527-533.

NEW YORK – Antiandrogen hormones can help stabilize, and even improve, female pattern hair loss.

The pathophysiology of the disorder is unknown, but treatment is based on the assumption that women must be like men, at least when it comes to losing their hair. Intuitively, decreasing androgens should help correct the problem.

R Eko Bintoro/ThinkStockPhotos

The answer, though, is a complicated mix of yes and maybe, Dr. Rochelle Torgerson said at the American Academy of Dermatology summer meeting.

“It used to be assumed that pattern hair loss in women was just the same as it is in men,” said Dr. Torgerson of the Mayo Clinic in Rochester, Minn. “Now there is some evidence that’s not true. In 2010, for example, this was seen in a woman with complete androgen insensitivity syndrome, so in her, androgens were not affecting hair follicles. There must be a place for estrogen.”

Further complicating the picture is the fact that no hormonal medications have FDA approval for hair loss in women, and their use has a history of conflicting data in clinical studies. Still, they remain the cornerstone for treating this physically and emotionally challenging problem.

The initial challenge is simply what to label it at the first visit.

“I have no problem with term ‘androgenetic alopecia,’ since that is what women are seeing when they first look on the Internet for information. But I do try to transition them to ‘female pattern hair loss.’ And I never – ever – use the term ‘male pattern baldness.’ It has a huge impact on women.”

The disease is a progressive miniaturization of the hair follicle over time. The growing cycle slows and the resting phase lengthens. There is progressive thinning over the vertex. Some women may keep most of their frontal hairline, but the vast majority do say it’s thinner than it was.

Spironolactone and oral contraceptives with spironolactone analogues are Dr. Torgerson’s go-to medications for first-line treatment. For spironolactone, she prefers a dose of 100-200 mg/day. Some women experience gastrointestinal upset, dizziness, cramps, breast tenderness, and spotting with these medications.

Her choice for an oral contraceptive is the combination of 20 mcg ethinyl estradiol plus drospirenone, but any oral contraceptive approved for acne may work.

Finasteride and dutasteride are approved for pattern hair loss in men, but not in women. Both inhibit 5 alpha-reductase type II. Dutasteride is more potent that finasteride and also inhibits type 1 alpha-reductase; both of these enzymes convert testosterone into the more potent dihydrotestosterone. The side-effect profile is more moderate than that of spironolactone, but both of the drugs have had mixed results in clinical trials.

One problem with the finasteride trials has been the variation in dosing. The least positive studies used the lowest dose of 1.25 mg. As the dosage increased to 2.5 mg and 5 mg, the benefit increased.

Despite her support for hormonal therapies, Dr. Torgerson doesn’t rely upon them alone – she supports them with the direct action of a 5% minoxidil foam. In addition to prescribing effective therapy, she urges women to actually be patient and to have realistic expectations.

Most women expect dramatic improvement in a short time. “I have no idea where that expectation comes from. This is a slow progressive condition. I agree with them that it’s completely unsexy to have the head of hair they do at that time. But if, in 3 years, they have this same head of hair, that’s going to be an amazing success. And once they have that expectation in their mind, they are usually happy with any other results that they see.”

Dr. Torgerson had no financial conflicts with regard to her presentation.

[email protected]

On Twitter @Alz_Gal

NEW YORK – Antiandrogen hormones can help stabilize, and even improve, female pattern hair loss.

The pathophysiology of the disorder is unknown, but treatment is based on the assumption that women must be like men, at least when it comes to losing their hair. Intuitively, decreasing androgens should help correct the problem.

R Eko Bintoro/ThinkStockPhotos

The answer, though, is a complicated mix of yes and maybe, Dr. Rochelle Torgerson said at the American Academy of Dermatology summer meeting.

“It used to be assumed that pattern hair loss in women was just the same as it is in men,” said Dr. Torgerson of the Mayo Clinic in Rochester, Minn. “Now there is some evidence that’s not true. In 2010, for example, this was seen in a woman with complete androgen insensitivity syndrome, so in her, androgens were not affecting hair follicles. There must be a place for estrogen.”

Further complicating the picture is the fact that no hormonal medications have FDA approval for hair loss in women, and their use has a history of conflicting data in clinical studies. Still, they remain the cornerstone for treating this physically and emotionally challenging problem.

The initial challenge is simply what to label it at the first visit.

“I have no problem with term ‘androgenetic alopecia,’ since that is what women are seeing when they first look on the Internet for information. But I do try to transition them to ‘female pattern hair loss.’ And I never – ever – use the term ‘male pattern baldness.’ It has a huge impact on women.”

The disease is a progressive miniaturization of the hair follicle over time. The growing cycle slows and the resting phase lengthens. There is progressive thinning over the vertex. Some women may keep most of their frontal hairline, but the vast majority do say it’s thinner than it was.

Spironolactone and oral contraceptives with spironolactone analogues are Dr. Torgerson’s go-to medications for first-line treatment. For spironolactone, she prefers a dose of 100-200 mg/day. Some women experience gastrointestinal upset, dizziness, cramps, breast tenderness, and spotting with these medications.

Her choice for an oral contraceptive is the combination of 20 mcg ethinyl estradiol plus drospirenone, but any oral contraceptive approved for acne may work.

Finasteride and dutasteride are approved for pattern hair loss in men, but not in women. Both inhibit 5 alpha-reductase type II. Dutasteride is more potent that finasteride and also inhibits type 1 alpha-reductase; both of these enzymes convert testosterone into the more potent dihydrotestosterone. The side-effect profile is more moderate than that of spironolactone, but both of the drugs have had mixed results in clinical trials.

One problem with the finasteride trials has been the variation in dosing. The least positive studies used the lowest dose of 1.25 mg. As the dosage increased to 2.5 mg and 5 mg, the benefit increased.

Despite her support for hormonal therapies, Dr. Torgerson doesn’t rely upon them alone – she supports them with the direct action of a 5% minoxidil foam. In addition to prescribing effective therapy, she urges women to actually be patient and to have realistic expectations.

Most women expect dramatic improvement in a short time. “I have no idea where that expectation comes from. This is a slow progressive condition. I agree with them that it’s completely unsexy to have the head of hair they do at that time. But if, in 3 years, they have this same head of hair, that’s going to be an amazing success. And once they have that expectation in their mind, they are usually happy with any other results that they see.”

Dr. Torgerson had no financial conflicts with regard to her presentation.

[email protected]

On Twitter @Alz_Gal

NEW YORK – Antiandrogen hormones can help stabilize, and even improve, female pattern hair loss.

The pathophysiology of the disorder is unknown, but treatment is based on the assumption that women must be like men, at least when it comes to losing their hair. Intuitively, decreasing androgens should help correct the problem.

R Eko Bintoro/ThinkStockPhotos

The answer, though, is a complicated mix of yes and maybe, Dr. Rochelle Torgerson said at the American Academy of Dermatology summer meeting.

“It used to be assumed that pattern hair loss in women was just the same as it is in men,” said Dr. Torgerson of the Mayo Clinic in Rochester, Minn. “Now there is some evidence that’s not true. In 2010, for example, this was seen in a woman with complete androgen insensitivity syndrome, so in her, androgens were not affecting hair follicles. There must be a place for estrogen.”

Further complicating the picture is the fact that no hormonal medications have FDA approval for hair loss in women, and their use has a history of conflicting data in clinical studies. Still, they remain the cornerstone for treating this physically and emotionally challenging problem.

The initial challenge is simply what to label it at the first visit.

“I have no problem with term ‘androgenetic alopecia,’ since that is what women are seeing when they first look on the Internet for information. But I do try to transition them to ‘female pattern hair loss.’ And I never – ever – use the term ‘male pattern baldness.’ It has a huge impact on women.”

The disease is a progressive miniaturization of the hair follicle over time. The growing cycle slows and the resting phase lengthens. There is progressive thinning over the vertex. Some women may keep most of their frontal hairline, but the vast majority do say it’s thinner than it was.

Spironolactone and oral contraceptives with spironolactone analogues are Dr. Torgerson’s go-to medications for first-line treatment. For spironolactone, she prefers a dose of 100-200 mg/day. Some women experience gastrointestinal upset, dizziness, cramps, breast tenderness, and spotting with these medications.

Her choice for an oral contraceptive is the combination of 20 mcg ethinyl estradiol plus drospirenone, but any oral contraceptive approved for acne may work.

Finasteride and dutasteride are approved for pattern hair loss in men, but not in women. Both inhibit 5 alpha-reductase type II. Dutasteride is more potent that finasteride and also inhibits type 1 alpha-reductase; both of these enzymes convert testosterone into the more potent dihydrotestosterone. The side-effect profile is more moderate than that of spironolactone, but both of the drugs have had mixed results in clinical trials.

One problem with the finasteride trials has been the variation in dosing. The least positive studies used the lowest dose of 1.25 mg. As the dosage increased to 2.5 mg and 5 mg, the benefit increased.

Despite her support for hormonal therapies, Dr. Torgerson doesn’t rely upon them alone – she supports them with the direct action of a 5% minoxidil foam. In addition to prescribing effective therapy, she urges women to actually be patient and to have realistic expectations.

Most women expect dramatic improvement in a short time. “I have no idea where that expectation comes from. This is a slow progressive condition. I agree with them that it’s completely unsexy to have the head of hair they do at that time. But if, in 3 years, they have this same head of hair, that’s going to be an amazing success. And once they have that expectation in their mind, they are usually happy with any other results that they see.”

Dr. Torgerson had no financial conflicts with regard to her presentation.

[email protected]

On Twitter @Alz_Gal

Prescription medications

Photo courtesy of the CDC

LONDON—Patients with atrial fibrillation (AF) who receive the anticoagulant rivaroxaban as stroke prophylaxis have low rates of major bleeding and stroke, according to real-world data from the XANTUS trial.

Investigators said this finding is similar to clinical trial results with rivaroxaban and suggest the drug is safe and effective for stroke prevention in patients with AF who have a high or low risk of thromboembolic events.

The team reported results of the XANTUS trial in the European Heart Journal and at the ESC Congress 2015 (abstract 5072). The study was sponsored by Bayer, the company developing rivaroxaban in cooperation with Janssen Pharmaceuticals, Inc.

“The findings reaffirm the positive benefit-risk profile of rivaroxaban established in the phase 3 clinical trial ROCKET AF, in which rivaroxaban was shown to provide effective stroke prevention with a similar overall bleeding profile and significantly lower rates of the most feared intracranial and fatal bleeds compared with vitamin K antagonists,” said John A. Camm, MD, of St. George’s University of London in the UK.

“The patients included in ROCKET AF were at moderate to high risk of stroke, with a mean CHADS2 score of 3.5, and the incidence of major bleeding in those taking rivaroxaban was 3.6 per 100 person-years. In XANTUS, patients seen in daily clinical practice had a lower risk of stroke, with a mean CHADS2 score of 2.0, and the incidence rate of major bleeding was lower, at 2.1 per 100 person-years.”

Patients and treatment

For the XANTUS trial, Dr Camm and his colleagues evaluated the outcomes of rivaroxaban use in 6784 patients with non-valvular AF who were treated at 311 centers across Europe and Canada in routine clinical practice.

Patients had newly diagnosed AF (18.5%), paroxysmal AF (40.6%), persistent AF (13.6%), and permanent AF (27%). For 0.2% of patients, data on their exact diagnosis was missing.

Comorbidities included hypertension (74.7%), diabetes mellitus (19.6%), prior stroke/non-central nervous system systemic embolism/transient ischemic attack (19%), congestive heart failure (18.6%), and prior myocardial infarction (10.1%).

The patients’ mean age was 71.5, and 37.2% were older than 75. Their mean CHADS2 score was 2, and their mean CHA2DS2VASc score was 3.4. A little more than half (54.5%) of patients were vitamin K agonist-naïve.

All treatment and dosing decisions for rivaroxaban were at the discretion of the treating physicians. Patients received the drug at 15 mg (n=1410), 20 mg (n=5336), or other doses (n=35).

They were followed for 1 year or until 30 days after premature treatment discontinuation. Bleeding events and major thromboembolic events were centrally adjudicated by an independent committee.

Results

By the end of the observation period, most patients (96.1%) had not experienced treatment-emergent major bleeding, all-cause death, or stroke/systemic embolism. And the majority of patients (80%) continued taking rivaroxaban throughout the 1-year study period.

The rate of stroke was 0.7% per year, and the annual rate of stroke/systemic embolism was 0.8%.

Overall, 2.1% of patients per year experienced treatment-emergent major bleeding. Non-major bleeding events occurred in 15.4% of patients per year.

The yearly rate of fatal bleeding was 0.2%, critical organ bleeding was 0.7%, intracranial hemorrhage was 0.4%, mucosal bleeding was 1.0%, and gastrointestinal bleeding was 0.9%. In addition, 0.9% of patients required transfusions of 2 or more units of packed red blood cells or whole blood per year.

The rate of on-treatment, all-cause mortality was 1.9% per year. There were 118 deaths in all, and some patients had multiple reasons reported as the cause of death. Causes of death were cardiovascular events (n=49), cancer (n=23), bleeding (n=12), infectious disease (n=10), other (n=16), and unexplained (n=9).

“These real-world insights from XANTUS complement and expand on what we already know from clinical trials and provide physicians with reassurance to prescribe rivaroxaban as an effective and well-tolerated treatment option for the broad range of patients with AF seen in their everyday clinical practice,” Dr Camm concluded.

Prescription medications

Photo courtesy of the CDC

LONDON—Patients with atrial fibrillation (AF) who receive the anticoagulant rivaroxaban as stroke prophylaxis have low rates of major bleeding and stroke, according to real-world data from the XANTUS trial.

Investigators said this finding is similar to clinical trial results with rivaroxaban and suggest the drug is safe and effective for stroke prevention in patients with AF who have a high or low risk of thromboembolic events.

The team reported results of the XANTUS trial in the European Heart Journal and at the ESC Congress 2015 (abstract 5072). The study was sponsored by Bayer, the company developing rivaroxaban in cooperation with Janssen Pharmaceuticals, Inc.

“The findings reaffirm the positive benefit-risk profile of rivaroxaban established in the phase 3 clinical trial ROCKET AF, in which rivaroxaban was shown to provide effective stroke prevention with a similar overall bleeding profile and significantly lower rates of the most feared intracranial and fatal bleeds compared with vitamin K antagonists,” said John A. Camm, MD, of St. George’s University of London in the UK.

“The patients included in ROCKET AF were at moderate to high risk of stroke, with a mean CHADS2 score of 3.5, and the incidence of major bleeding in those taking rivaroxaban was 3.6 per 100 person-years. In XANTUS, patients seen in daily clinical practice had a lower risk of stroke, with a mean CHADS2 score of 2.0, and the incidence rate of major bleeding was lower, at 2.1 per 100 person-years.”

Patients and treatment

For the XANTUS trial, Dr Camm and his colleagues evaluated the outcomes of rivaroxaban use in 6784 patients with non-valvular AF who were treated at 311 centers across Europe and Canada in routine clinical practice.

Patients had newly diagnosed AF (18.5%), paroxysmal AF (40.6%), persistent AF (13.6%), and permanent AF (27%). For 0.2% of patients, data on their exact diagnosis was missing.

Comorbidities included hypertension (74.7%), diabetes mellitus (19.6%), prior stroke/non-central nervous system systemic embolism/transient ischemic attack (19%), congestive heart failure (18.6%), and prior myocardial infarction (10.1%).

The patients’ mean age was 71.5, and 37.2% were older than 75. Their mean CHADS2 score was 2, and their mean CHA2DS2VASc score was 3.4. A little more than half (54.5%) of patients were vitamin K agonist-naïve.

All treatment and dosing decisions for rivaroxaban were at the discretion of the treating physicians. Patients received the drug at 15 mg (n=1410), 20 mg (n=5336), or other doses (n=35).

They were followed for 1 year or until 30 days after premature treatment discontinuation. Bleeding events and major thromboembolic events were centrally adjudicated by an independent committee.

Results

By the end of the observation period, most patients (96.1%) had not experienced treatment-emergent major bleeding, all-cause death, or stroke/systemic embolism. And the majority of patients (80%) continued taking rivaroxaban throughout the 1-year study period.

The rate of stroke was 0.7% per year, and the annual rate of stroke/systemic embolism was 0.8%.

Overall, 2.1% of patients per year experienced treatment-emergent major bleeding. Non-major bleeding events occurred in 15.4% of patients per year.

The yearly rate of fatal bleeding was 0.2%, critical organ bleeding was 0.7%, intracranial hemorrhage was 0.4%, mucosal bleeding was 1.0%, and gastrointestinal bleeding was 0.9%. In addition, 0.9% of patients required transfusions of 2 or more units of packed red blood cells or whole blood per year.

The rate of on-treatment, all-cause mortality was 1.9% per year. There were 118 deaths in all, and some patients had multiple reasons reported as the cause of death. Causes of death were cardiovascular events (n=49), cancer (n=23), bleeding (n=12), infectious disease (n=10), other (n=16), and unexplained (n=9).

“These real-world insights from XANTUS complement and expand on what we already know from clinical trials and provide physicians with reassurance to prescribe rivaroxaban as an effective and well-tolerated treatment option for the broad range of patients with AF seen in their everyday clinical practice,” Dr Camm concluded.

Prescription medications

Photo courtesy of the CDC

LONDON—Patients with atrial fibrillation (AF) who receive the anticoagulant rivaroxaban as stroke prophylaxis have low rates of major bleeding and stroke, according to real-world data from the XANTUS trial.

Investigators said this finding is similar to clinical trial results with rivaroxaban and suggest the drug is safe and effective for stroke prevention in patients with AF who have a high or low risk of thromboembolic events.

The team reported results of the XANTUS trial in the European Heart Journal and at the ESC Congress 2015 (abstract 5072). The study was sponsored by Bayer, the company developing rivaroxaban in cooperation with Janssen Pharmaceuticals, Inc.

“The findings reaffirm the positive benefit-risk profile of rivaroxaban established in the phase 3 clinical trial ROCKET AF, in which rivaroxaban was shown to provide effective stroke prevention with a similar overall bleeding profile and significantly lower rates of the most feared intracranial and fatal bleeds compared with vitamin K antagonists,” said John A. Camm, MD, of St. George’s University of London in the UK.

“The patients included in ROCKET AF were at moderate to high risk of stroke, with a mean CHADS2 score of 3.5, and the incidence of major bleeding in those taking rivaroxaban was 3.6 per 100 person-years. In XANTUS, patients seen in daily clinical practice had a lower risk of stroke, with a mean CHADS2 score of 2.0, and the incidence rate of major bleeding was lower, at 2.1 per 100 person-years.”

Patients and treatment

For the XANTUS trial, Dr Camm and his colleagues evaluated the outcomes of rivaroxaban use in 6784 patients with non-valvular AF who were treated at 311 centers across Europe and Canada in routine clinical practice.

Patients had newly diagnosed AF (18.5%), paroxysmal AF (40.6%), persistent AF (13.6%), and permanent AF (27%). For 0.2% of patients, data on their exact diagnosis was missing.

Comorbidities included hypertension (74.7%), diabetes mellitus (19.6%), prior stroke/non-central nervous system systemic embolism/transient ischemic attack (19%), congestive heart failure (18.6%), and prior myocardial infarction (10.1%).

The patients’ mean age was 71.5, and 37.2% were older than 75. Their mean CHADS2 score was 2, and their mean CHA2DS2VASc score was 3.4. A little more than half (54.5%) of patients were vitamin K agonist-naïve.

All treatment and dosing decisions for rivaroxaban were at the discretion of the treating physicians. Patients received the drug at 15 mg (n=1410), 20 mg (n=5336), or other doses (n=35).

They were followed for 1 year or until 30 days after premature treatment discontinuation. Bleeding events and major thromboembolic events were centrally adjudicated by an independent committee.

Results

By the end of the observation period, most patients (96.1%) had not experienced treatment-emergent major bleeding, all-cause death, or stroke/systemic embolism. And the majority of patients (80%) continued taking rivaroxaban throughout the 1-year study period.

The rate of stroke was 0.7% per year, and the annual rate of stroke/systemic embolism was 0.8%.

Overall, 2.1% of patients per year experienced treatment-emergent major bleeding. Non-major bleeding events occurred in 15.4% of patients per year.

The yearly rate of fatal bleeding was 0.2%, critical organ bleeding was 0.7%, intracranial hemorrhage was 0.4%, mucosal bleeding was 1.0%, and gastrointestinal bleeding was 0.9%. In addition, 0.9% of patients required transfusions of 2 or more units of packed red blood cells or whole blood per year.

The rate of on-treatment, all-cause mortality was 1.9% per year. There were 118 deaths in all, and some patients had multiple reasons reported as the cause of death. Causes of death were cardiovascular events (n=49), cancer (n=23), bleeding (n=12), infectious disease (n=10), other (n=16), and unexplained (n=9).

“These real-world insights from XANTUS complement and expand on what we already know from clinical trials and provide physicians with reassurance to prescribe rivaroxaban as an effective and well-tolerated treatment option for the broad range of patients with AF seen in their everyday clinical practice,” Dr Camm concluded.

Drug-eluting stent

LONDON—Extending dual antiplatelet therapy (DAPT) beyond the recommended 12 months after coronary stenting “should be considered” in patients with a drug-eluting stent (DES) who are at low risk for bleeding, according to investigators from the OPTIDUAL trial.

The study showed that receiving DAPT—aspirin and clopidogrel—for an additional 36 months did not decrease the rate of major adverse cardiovascular and cerebrovascular events (MACCE).

However, there was a suggestion that it might reduce ischemic outcomes without excess bleeding.

“Given the lack of harm and the signal for benefit of prolonged DAPT in the OPTIDUAL trial, and the results from prior randomized trials testing long durations of DAPT, prolongation of DAPT beyond 12 months should be considered in patients without high-risk bleeding who have received a drug-eluting coronary stent and are event-free at 12 months,” said study investigator Gérard Helft, MD, PhD, of Hôpital Universitaire Pitié-Salpétrière in Paris, France.

He presented results of the trial at the ESC Congress 2015 (abstract 3159).

The study included 1385 patients from 58 French sites who had undergone percutaneous coronary intervention with placement of at least 1 DES for either stable coronary artery disease or acute coronary syndrome.

All patients had been on DAPT for 1 year and were randomly assigned to continue or to remain on aspirin alone for an additional 36 months.

There was no significant difference between the groups with regard to MACCE, a composite of all-cause death, myocardial infarction, stroke, and major bleeding. These events occurred in 5.8% of patients in the extended-DAPT group and 7.5% in the aspirin-only group (hazard ratio (HR=0.75, P=0.17).

Likewise, there was no significant difference between the treatment groups for any of the components of MACCE—stroke (HR=0.69, P=0.53), myocardial infarction (HR=0.67, P=0.31), major bleeding (HR=0.98, P=0.95), or death (HR=0.65, P=0.18).

There was a non-significant (but borderline) reduction in ischemic outcomes—a post-hoc outcome composite rate of death, myocardial infarction, or stroke—with extended DAPT. These events occurred in 4.2% of patients in the extended-DAPT group and 6.4% in the aspirin-only group (HR=0.64, P=0.06).

“The results are consistent with the recent findings on ischemic outcomes from the DAPT trial regarding the value of prolonging DAPT after DES placement,” Dr Helft said.

“There was no apparent harm, and the post-hoc efficacy signal on MACCE is consistent with the benefit seen in the DAPT trial. Thus, OPTIDUAL adds to the evidence suggesting benefit to extended DAPT after DES in patients who are event-free at 12 months.”

This study was funded by Assistance Publique - Hôpitaux de Paris and the Fédération Française de Cardiologie, with unrestricted grants from Cordis, Boston, Medtronic, Terumo, and Biotronik.

Drug-eluting stent

LONDON—Extending dual antiplatelet therapy (DAPT) beyond the recommended 12 months after coronary stenting “should be considered” in patients with a drug-eluting stent (DES) who are at low risk for bleeding, according to investigators from the OPTIDUAL trial.

The study showed that receiving DAPT—aspirin and clopidogrel—for an additional 36 months did not decrease the rate of major adverse cardiovascular and cerebrovascular events (MACCE).

However, there was a suggestion that it might reduce ischemic outcomes without excess bleeding.

“Given the lack of harm and the signal for benefit of prolonged DAPT in the OPTIDUAL trial, and the results from prior randomized trials testing long durations of DAPT, prolongation of DAPT beyond 12 months should be considered in patients without high-risk bleeding who have received a drug-eluting coronary stent and are event-free at 12 months,” said study investigator Gérard Helft, MD, PhD, of Hôpital Universitaire Pitié-Salpétrière in Paris, France.

He presented results of the trial at the ESC Congress 2015 (abstract 3159).

The study included 1385 patients from 58 French sites who had undergone percutaneous coronary intervention with placement of at least 1 DES for either stable coronary artery disease or acute coronary syndrome.

All patients had been on DAPT for 1 year and were randomly assigned to continue or to remain on aspirin alone for an additional 36 months.

There was no significant difference between the groups with regard to MACCE, a composite of all-cause death, myocardial infarction, stroke, and major bleeding. These events occurred in 5.8% of patients in the extended-DAPT group and 7.5% in the aspirin-only group (hazard ratio (HR=0.75, P=0.17).

Likewise, there was no significant difference between the treatment groups for any of the components of MACCE—stroke (HR=0.69, P=0.53), myocardial infarction (HR=0.67, P=0.31), major bleeding (HR=0.98, P=0.95), or death (HR=0.65, P=0.18).

There was a non-significant (but borderline) reduction in ischemic outcomes—a post-hoc outcome composite rate of death, myocardial infarction, or stroke—with extended DAPT. These events occurred in 4.2% of patients in the extended-DAPT group and 6.4% in the aspirin-only group (HR=0.64, P=0.06).

“The results are consistent with the recent findings on ischemic outcomes from the DAPT trial regarding the value of prolonging DAPT after DES placement,” Dr Helft said.

“There was no apparent harm, and the post-hoc efficacy signal on MACCE is consistent with the benefit seen in the DAPT trial. Thus, OPTIDUAL adds to the evidence suggesting benefit to extended DAPT after DES in patients who are event-free at 12 months.”

This study was funded by Assistance Publique - Hôpitaux de Paris and the Fédération Française de Cardiologie, with unrestricted grants from Cordis, Boston, Medtronic, Terumo, and Biotronik.

Drug-eluting stent

LONDON—Extending dual antiplatelet therapy (DAPT) beyond the recommended 12 months after coronary stenting “should be considered” in patients with a drug-eluting stent (DES) who are at low risk for bleeding, according to investigators from the OPTIDUAL trial.

The study showed that receiving DAPT—aspirin and clopidogrel—for an additional 36 months did not decrease the rate of major adverse cardiovascular and cerebrovascular events (MACCE).

However, there was a suggestion that it might reduce ischemic outcomes without excess bleeding.

“Given the lack of harm and the signal for benefit of prolonged DAPT in the OPTIDUAL trial, and the results from prior randomized trials testing long durations of DAPT, prolongation of DAPT beyond 12 months should be considered in patients without high-risk bleeding who have received a drug-eluting coronary stent and are event-free at 12 months,” said study investigator Gérard Helft, MD, PhD, of Hôpital Universitaire Pitié-Salpétrière in Paris, France.

He presented results of the trial at the ESC Congress 2015 (abstract 3159).

The study included 1385 patients from 58 French sites who had undergone percutaneous coronary intervention with placement of at least 1 DES for either stable coronary artery disease or acute coronary syndrome.

All patients had been on DAPT for 1 year and were randomly assigned to continue or to remain on aspirin alone for an additional 36 months.

There was no significant difference between the groups with regard to MACCE, a composite of all-cause death, myocardial infarction, stroke, and major bleeding. These events occurred in 5.8% of patients in the extended-DAPT group and 7.5% in the aspirin-only group (hazard ratio (HR=0.75, P=0.17).

Likewise, there was no significant difference between the treatment groups for any of the components of MACCE—stroke (HR=0.69, P=0.53), myocardial infarction (HR=0.67, P=0.31), major bleeding (HR=0.98, P=0.95), or death (HR=0.65, P=0.18).

There was a non-significant (but borderline) reduction in ischemic outcomes—a post-hoc outcome composite rate of death, myocardial infarction, or stroke—with extended DAPT. These events occurred in 4.2% of patients in the extended-DAPT group and 6.4% in the aspirin-only group (HR=0.64, P=0.06).

“The results are consistent with the recent findings on ischemic outcomes from the DAPT trial regarding the value of prolonging DAPT after DES placement,” Dr Helft said.

“There was no apparent harm, and the post-hoc efficacy signal on MACCE is consistent with the benefit seen in the DAPT trial. Thus, OPTIDUAL adds to the evidence suggesting benefit to extended DAPT after DES in patients who are event-free at 12 months.”

This study was funded by Assistance Publique - Hôpitaux de Paris and the Fédération Française de Cardiologie, with unrestricted grants from Cordis, Boston, Medtronic, Terumo, and Biotronik.

Doctor consults with cancer

patient and her father

Photo by Rhoda Baer

Exome and transcriptome sequencing results can inform the management of young patients with relapsed, refractory, and rare malignancies, a new study suggests.

In a consecutive case series, sequencing data revealed potentially actionable findings for 46% of patients.

As a result, 15% of patients changed treatment, and 10% underwent genetic counseling.

Investigators described this research in JAMA.

“We found that, for some children with rare, difficult-to-treat, and aggressive cancers, this technology can dramatically change the course of their treatment,” said study author Rajen Mody, MBBS, of the University of Michigan in Ann Arbor.

Dr Mody and his colleagues evaluated 102 patients with relapsed, refractory, or rare cancers. Their median age was 11.5 (range, 0-22).

The patients underwent integrative clinical exome (tumor and germline DNA) and transcriptome (tumor RNA) sequencing. Ninety-one patients (89%) had adequate tumor tissue to complete sequencing, including 28 patients (31%) with hematologic malignancies and 63 (69%) with solid tumors.

All sequencing results were discussed at a precision medicine tumor board, which included pediatric and adult oncologists, pathologists, genetics specialists, and other professionals. This group discussed the results and assessed the feasibility of pursuing treatment options based on the findings.

Actionable findings

Forty-two patients (46%) had potentially actionable findings, 15 (54%) with hematologic malignancies and 27 (43%) with solid tumors.

Actionable findings included a change in diagnosis (n=2), the presence of a genetic anomaly that could be targeted by an approved or experimental drug (n=31), and the need for genetic counseling for inherited cancer risk that could affect the patient or the whole family (n=9).

“We were excited to see an actionable finding in such a substantial percentage of patients, and we think it could potentially be higher over time,” said study author Arul Chinnaiyan, MD, PhD, also of the University of Michigan.

“These are patients who had exhausted all proven therapeutic options or who had an extremely rare diagnosis. If we can find a clinically actionable event and have a chance to act upon it, we show in this study that it can have a big impact on that patient.”

Actions were taken in 23 of the 42 patients. Fourteen patients (15%) had their treatment changed, and 9 of these patients (10%) had durable partial or complete remissions (CRs) as a result.

Nine patients (10%) underwent genetic counseling because of sequencing results. The researchers noted that 4 of these patients had no notable family history to suggest an inherited risk, and they would not otherwise have been referred for genetic counseling.

Hematologic malignancies

Fifteen patients with hematologic malignancies had potentially actionable findings, and 4 underwent treatment changes as a result. (None of the patients required genetic counseling.)

For a patient with pre-B acute lymphoblastic leukemia (ALL), sequencing revealed a homozygous CDKN2A deletion and an ETV6-ABL1 fusion. So the patient was placed on imatinib and had a sustained CR for 21 months.

A patient with early T-cell precursor ALL had a FLT3-ITD mutation, Chr16p gain, Chr16q loss, and FLT3 overexpression. The patient achieved a CR after transplant, was placed on the FLT3 inhibitor sorafenib, and remained in CR for 15 months.

Another patient with pre-B ALL had a FLT3 nonframeshift deletion and BLK and FLT3 overexpression. The patient was in CR for 9 months after a transplant and received sorafenib for 6 months.

A patient with biphenotypic leukemia had mutations in NRAS and PHF6; SPI1, ASXL1, and CBLC frameshift insertions; a JAK3-activating mutation; and JAK3 overexpression. The patient received the JAK3 inhibitor tofacitinib but could not tolerate the full dose and died of progressive disease.

Cost and turn-around time

The cost for sequencing was approximately $6000 per patient and was covered under the research protocol.

It took the researchers about 7 to 8 weeks to report the sequencing results back to treating physicians and families.

“These are early days, and the full promise of precision medicine is yet to be fully realized,” Dr Mody said. “We need better targeted therapies designed for children, and turnaround time for sequencing needs to be less than 2 weeks for it to be a regular part of a patient’s treatment plan.”

Doctor consults with cancer

patient and her father

Photo by Rhoda Baer

Exome and transcriptome sequencing results can inform the management of young patients with relapsed, refractory, and rare malignancies, a new study suggests.

In a consecutive case series, sequencing data revealed potentially actionable findings for 46% of patients.

As a result, 15% of patients changed treatment, and 10% underwent genetic counseling.

Investigators described this research in JAMA.

“We found that, for some children with rare, difficult-to-treat, and aggressive cancers, this technology can dramatically change the course of their treatment,” said study author Rajen Mody, MBBS, of the University of Michigan in Ann Arbor.

Dr Mody and his colleagues evaluated 102 patients with relapsed, refractory, or rare cancers. Their median age was 11.5 (range, 0-22).

The patients underwent integrative clinical exome (tumor and germline DNA) and transcriptome (tumor RNA) sequencing. Ninety-one patients (89%) had adequate tumor tissue to complete sequencing, including 28 patients (31%) with hematologic malignancies and 63 (69%) with solid tumors.

All sequencing results were discussed at a precision medicine tumor board, which included pediatric and adult oncologists, pathologists, genetics specialists, and other professionals. This group discussed the results and assessed the feasibility of pursuing treatment options based on the findings.

Actionable findings

Forty-two patients (46%) had potentially actionable findings, 15 (54%) with hematologic malignancies and 27 (43%) with solid tumors.

Actionable findings included a change in diagnosis (n=2), the presence of a genetic anomaly that could be targeted by an approved or experimental drug (n=31), and the need for genetic counseling for inherited cancer risk that could affect the patient or the whole family (n=9).

“We were excited to see an actionable finding in such a substantial percentage of patients, and we think it could potentially be higher over time,” said study author Arul Chinnaiyan, MD, PhD, also of the University of Michigan.

“These are patients who had exhausted all proven therapeutic options or who had an extremely rare diagnosis. If we can find a clinically actionable event and have a chance to act upon it, we show in this study that it can have a big impact on that patient.”

Actions were taken in 23 of the 42 patients. Fourteen patients (15%) had their treatment changed, and 9 of these patients (10%) had durable partial or complete remissions (CRs) as a result.

Nine patients (10%) underwent genetic counseling because of sequencing results. The researchers noted that 4 of these patients had no notable family history to suggest an inherited risk, and they would not otherwise have been referred for genetic counseling.

Hematologic malignancies

Fifteen patients with hematologic malignancies had potentially actionable findings, and 4 underwent treatment changes as a result. (None of the patients required genetic counseling.)

For a patient with pre-B acute lymphoblastic leukemia (ALL), sequencing revealed a homozygous CDKN2A deletion and an ETV6-ABL1 fusion. So the patient was placed on imatinib and had a sustained CR for 21 months.

A patient with early T-cell precursor ALL had a FLT3-ITD mutation, Chr16p gain, Chr16q loss, and FLT3 overexpression. The patient achieved a CR after transplant, was placed on the FLT3 inhibitor sorafenib, and remained in CR for 15 months.

Another patient with pre-B ALL had a FLT3 nonframeshift deletion and BLK and FLT3 overexpression. The patient was in CR for 9 months after a transplant and received sorafenib for 6 months.

A patient with biphenotypic leukemia had mutations in NRAS and PHF6; SPI1, ASXL1, and CBLC frameshift insertions; a JAK3-activating mutation; and JAK3 overexpression. The patient received the JAK3 inhibitor tofacitinib but could not tolerate the full dose and died of progressive disease.

Cost and turn-around time

The cost for sequencing was approximately $6000 per patient and was covered under the research protocol.

It took the researchers about 7 to 8 weeks to report the sequencing results back to treating physicians and families.

“These are early days, and the full promise of precision medicine is yet to be fully realized,” Dr Mody said. “We need better targeted therapies designed for children, and turnaround time for sequencing needs to be less than 2 weeks for it to be a regular part of a patient’s treatment plan.”

Doctor consults with cancer

patient and her father

Photo by Rhoda Baer

Exome and transcriptome sequencing results can inform the management of young patients with relapsed, refractory, and rare malignancies, a new study suggests.

In a consecutive case series, sequencing data revealed potentially actionable findings for 46% of patients.

As a result, 15% of patients changed treatment, and 10% underwent genetic counseling.

Investigators described this research in JAMA.

“We found that, for some children with rare, difficult-to-treat, and aggressive cancers, this technology can dramatically change the course of their treatment,” said study author Rajen Mody, MBBS, of the University of Michigan in Ann Arbor.

Dr Mody and his colleagues evaluated 102 patients with relapsed, refractory, or rare cancers. Their median age was 11.5 (range, 0-22).

The patients underwent integrative clinical exome (tumor and germline DNA) and transcriptome (tumor RNA) sequencing. Ninety-one patients (89%) had adequate tumor tissue to complete sequencing, including 28 patients (31%) with hematologic malignancies and 63 (69%) with solid tumors.

All sequencing results were discussed at a precision medicine tumor board, which included pediatric and adult oncologists, pathologists, genetics specialists, and other professionals. This group discussed the results and assessed the feasibility of pursuing treatment options based on the findings.

Actionable findings

Forty-two patients (46%) had potentially actionable findings, 15 (54%) with hematologic malignancies and 27 (43%) with solid tumors.

Actionable findings included a change in diagnosis (n=2), the presence of a genetic anomaly that could be targeted by an approved or experimental drug (n=31), and the need for genetic counseling for inherited cancer risk that could affect the patient or the whole family (n=9).

“We were excited to see an actionable finding in such a substantial percentage of patients, and we think it could potentially be higher over time,” said study author Arul Chinnaiyan, MD, PhD, also of the University of Michigan.

“These are patients who had exhausted all proven therapeutic options or who had an extremely rare diagnosis. If we can find a clinically actionable event and have a chance to act upon it, we show in this study that it can have a big impact on that patient.”

Actions were taken in 23 of the 42 patients. Fourteen patients (15%) had their treatment changed, and 9 of these patients (10%) had durable partial or complete remissions (CRs) as a result.

Nine patients (10%) underwent genetic counseling because of sequencing results. The researchers noted that 4 of these patients had no notable family history to suggest an inherited risk, and they would not otherwise have been referred for genetic counseling.

Hematologic malignancies

Fifteen patients with hematologic malignancies had potentially actionable findings, and 4 underwent treatment changes as a result. (None of the patients required genetic counseling.)

For a patient with pre-B acute lymphoblastic leukemia (ALL), sequencing revealed a homozygous CDKN2A deletion and an ETV6-ABL1 fusion. So the patient was placed on imatinib and had a sustained CR for 21 months.

A patient with early T-cell precursor ALL had a FLT3-ITD mutation, Chr16p gain, Chr16q loss, and FLT3 overexpression. The patient achieved a CR after transplant, was placed on the FLT3 inhibitor sorafenib, and remained in CR for 15 months.

Another patient with pre-B ALL had a FLT3 nonframeshift deletion and BLK and FLT3 overexpression. The patient was in CR for 9 months after a transplant and received sorafenib for 6 months.

A patient with biphenotypic leukemia had mutations in NRAS and PHF6; SPI1, ASXL1, and CBLC frameshift insertions; a JAK3-activating mutation; and JAK3 overexpression. The patient received the JAK3 inhibitor tofacitinib but could not tolerate the full dose and died of progressive disease.

Cost and turn-around time

The cost for sequencing was approximately $6000 per patient and was covered under the research protocol.

It took the researchers about 7 to 8 weeks to report the sequencing results back to treating physicians and families.

“These are early days, and the full promise of precision medicine is yet to be fully realized,” Dr Mody said. “We need better targeted therapies designed for children, and turnaround time for sequencing needs to be less than 2 weeks for it to be a regular part of a patient’s treatment plan.”

The Brazilain wasp

Polybia paulista

Photo by Mario Palma/

São Paulo State University

The wasp Polybia paulista protects itself from predators by producing venom known to contain a cancer-fighting toxin.

A study published in Biophysical Journal helps explain how the venom’s toxin, MP1 (Polybia-MP1), selectively kills cancer cells without harming normal cells.

MP1 interacts with lipids that are abnormally distributed on the surface of cancer cells, creating holes that facilitate the escape of molecules crucial for cell function.

“Cancer therapies that attack the lipid composition of the cell membrane would be an entirely new class of anticancer drugs,” said study author Paul Beales, PhD, of the University of Leeds in the UK.

MP1 is known to act against microbial pathogens by disrupting the bacterial cell membrane. The peptide has shown promise for treating cancers, as it can inhibit the growth of prostate and bladder cancer cells, as well as multi-drug resistant leukemic cells.

However, it has not been clear how MP1 selectively destroys cancer cells without harming normal cells. Dr Beales and his colleagues thought an explanation might lie in the unique properties of cancer cell membranes.

In healthy cell membranes, the phospholipids phosphatidylserine (PS) and phosphatidylethanolamine (PE) are located in the inner membrane leaflet facing the inside of the cell. But in cancer cells, PS and PE are embedded in the outer membrane leaflet facing the cell surroundings.

The researchers tested their theory by creating model membranes, some of which contained PE and/or PS, and exposing them to MP1. They used a wide range of imaging and biophysical techniques to characterize MP1’s destructive effects on the membranes.

The team found that PS increased the binding of MP1 to the membrane by a factor of 7 to 8. On the other hand, PE enhanced MP1’s ability to quickly disrupt the membrane, increasing the size of holes by a factor of 20 to 30.

“Formed in only seconds, these large pores are big enough to allow critical molecules such as RNA and proteins to easily escape cells,” said study author João Ruggiero Neto, of São Paulo State University in Brazil.

“The dramatic enhancement of the permeabilization induced by the peptide in the presence of PE and the dimensions of the pores in these membranes was surprising.”

In future studies, the researchers plan to alter MP1’s amino acid sequence to examine how the peptide’s structure relates to its function and further improve the peptide’s selectivity and potency for clinical purposes.

“Understanding the mechanism of action of this peptide will help in translational studies to further assess the potential for this peptide to be used in medicine,” Dr Beales said.

“As it has been shown to be selective to cancer cells and non-toxic to normal cells in the lab, this peptide has the potential to be safe, but further work would be required to prove that.”

The Brazilain wasp

Polybia paulista

Photo by Mario Palma/

São Paulo State University

The wasp Polybia paulista protects itself from predators by producing venom known to contain a cancer-fighting toxin.

A study published in Biophysical Journal helps explain how the venom’s toxin, MP1 (Polybia-MP1), selectively kills cancer cells without harming normal cells.

MP1 interacts with lipids that are abnormally distributed on the surface of cancer cells, creating holes that facilitate the escape of molecules crucial for cell function.

“Cancer therapies that attack the lipid composition of the cell membrane would be an entirely new class of anticancer drugs,” said study author Paul Beales, PhD, of the University of Leeds in the UK.

MP1 is known to act against microbial pathogens by disrupting the bacterial cell membrane. The peptide has shown promise for treating cancers, as it can inhibit the growth of prostate and bladder cancer cells, as well as multi-drug resistant leukemic cells.

However, it has not been clear how MP1 selectively destroys cancer cells without harming normal cells. Dr Beales and his colleagues thought an explanation might lie in the unique properties of cancer cell membranes.

In healthy cell membranes, the phospholipids phosphatidylserine (PS) and phosphatidylethanolamine (PE) are located in the inner membrane leaflet facing the inside of the cell. But in cancer cells, PS and PE are embedded in the outer membrane leaflet facing the cell surroundings.

The researchers tested their theory by creating model membranes, some of which contained PE and/or PS, and exposing them to MP1. They used a wide range of imaging and biophysical techniques to characterize MP1’s destructive effects on the membranes.

The team found that PS increased the binding of MP1 to the membrane by a factor of 7 to 8. On the other hand, PE enhanced MP1’s ability to quickly disrupt the membrane, increasing the size of holes by a factor of 20 to 30.

“Formed in only seconds, these large pores are big enough to allow critical molecules such as RNA and proteins to easily escape cells,” said study author João Ruggiero Neto, of São Paulo State University in Brazil.

“The dramatic enhancement of the permeabilization induced by the peptide in the presence of PE and the dimensions of the pores in these membranes was surprising.”

In future studies, the researchers plan to alter MP1’s amino acid sequence to examine how the peptide’s structure relates to its function and further improve the peptide’s selectivity and potency for clinical purposes.

“Understanding the mechanism of action of this peptide will help in translational studies to further assess the potential for this peptide to be used in medicine,” Dr Beales said.

“As it has been shown to be selective to cancer cells and non-toxic to normal cells in the lab, this peptide has the potential to be safe, but further work would be required to prove that.”

The Brazilain wasp

Polybia paulista

Photo by Mario Palma/

São Paulo State University

The wasp Polybia paulista protects itself from predators by producing venom known to contain a cancer-fighting toxin.

A study published in Biophysical Journal helps explain how the venom’s toxin, MP1 (Polybia-MP1), selectively kills cancer cells without harming normal cells.

MP1 interacts with lipids that are abnormally distributed on the surface of cancer cells, creating holes that facilitate the escape of molecules crucial for cell function.

“Cancer therapies that attack the lipid composition of the cell membrane would be an entirely new class of anticancer drugs,” said study author Paul Beales, PhD, of the University of Leeds in the UK.

MP1 is known to act against microbial pathogens by disrupting the bacterial cell membrane. The peptide has shown promise for treating cancers, as it can inhibit the growth of prostate and bladder cancer cells, as well as multi-drug resistant leukemic cells.

However, it has not been clear how MP1 selectively destroys cancer cells without harming normal cells. Dr Beales and his colleagues thought an explanation might lie in the unique properties of cancer cell membranes.

In healthy cell membranes, the phospholipids phosphatidylserine (PS) and phosphatidylethanolamine (PE) are located in the inner membrane leaflet facing the inside of the cell. But in cancer cells, PS and PE are embedded in the outer membrane leaflet facing the cell surroundings.

The researchers tested their theory by creating model membranes, some of which contained PE and/or PS, and exposing them to MP1. They used a wide range of imaging and biophysical techniques to characterize MP1’s destructive effects on the membranes.

The team found that PS increased the binding of MP1 to the membrane by a factor of 7 to 8. On the other hand, PE enhanced MP1’s ability to quickly disrupt the membrane, increasing the size of holes by a factor of 20 to 30.

“Formed in only seconds, these large pores are big enough to allow critical molecules such as RNA and proteins to easily escape cells,” said study author João Ruggiero Neto, of São Paulo State University in Brazil.

“The dramatic enhancement of the permeabilization induced by the peptide in the presence of PE and the dimensions of the pores in these membranes was surprising.”

In future studies, the researchers plan to alter MP1’s amino acid sequence to examine how the peptide’s structure relates to its function and further improve the peptide’s selectivity and potency for clinical purposes.

“Understanding the mechanism of action of this peptide will help in translational studies to further assess the potential for this peptide to be used in medicine,” Dr Beales said.

“As it has been shown to be selective to cancer cells and non-toxic to normal cells in the lab, this peptide has the potential to be safe, but further work would be required to prove that.”

The Centers for Disease Control and Prevention (CDC) has published its recommendations for the use of influenza vaccines for the 2015-2016 influenza season.¹ (See the CDC’s Web site at http://www.cdc.gov/flu/professionals/vaccination/index.htm.) This Practice Alert describes recent changes in vaccine products, discusses the timing of vaccination, reviews a new algorithm for deciding on the number of doses for children ages 6 months through 8 years, and raises issues to consider when thinking about specific products for individual patients.

Vaccine product modifications for 2015-2016

Influenza vaccines contain either 3 or 4 antigens (trivalent or quadrivalent) and either inactivated or modified live viruses (inactivated influenza vaccine [IIV]) or live attenuated influenza vaccine [LAIV]). These vaccines are produced in eggs, by cell cultures, or with recombinant technology. The vaccine products for this influenza season will contain antigens of one H1N1 virus, one H3N2 virus, and one B virus (trivalent products) or 2 B viruses (quadrivalent products).

The viruses selected for the vaccine are based on the most prevalent types in circulation globally, and vaccine effectiveness in the United States will be directly proportional to how well these strains match those circulating here during the influenza season. In the 2014-2015 influenza season, an antigenic drift in the circulating H3N2 virus rendered influenza vaccines only 23% effective in preventing laboratory-confirmed influenza.²

One product, Afluria, an IIV trivalent product, has been approved for administration using a needle-free jet injector in individuals ages 18 to 64 years.¹ Additionally, intramuscular injection of Afluria is still available for those 18 years and older. All other IIV products are administered via needle and syringe. Vaccination with a jet injector achieves protection equivalent to needle injection, but its use is associated with higher rates of local reactions.

Flublok, another trivalent IIV product, is produced using a recombinant egg-free process. It was originally approved for individuals 18 to 49 years; there is now no upper age limit, providing an egg-free option for an expanded age group. An intradermal option, Fluzone, was a trivalent product last year and will be replaced by Fluzone Intradermal Quadrivalent this season.

A complete list of all influenza products and their respective patient-specific recommendations can be found on the CDC influenza Web site (http://www.cdc.gov/flu/professionals/vaccination/index.htm).

Timing of vaccine administration

Flublok, a trivalent IIV recombinant egg-free product, now has no upper age limit for administration.

Start providing influenza vaccine by the beginning of October and continue offering it throughout the influenza season to those who are unimmunized. In the past, the recommendation was to begin vaccination as soon as vaccine was available. But studies have shown that vaccine effectiveness declines after 6 months, especially among those over age 65 years, which can result in inadequate protection late in the influenza season.^3,4 Administering the vaccine later in the fall may confer greater protection later in the season, but, as a strategy, it could also lead to missed opportunities to vaccinate.

Annual vaccination for the entire population is a public health challenge and the October start date is appropriate middle ground. However, children who need 2 doses should receive the first dose as soon as vaccine is available, and the second dose 4 weeks later.¹

One or two doses in children?

Children ages 6 months through 8 years who are receiving influenza vaccine for the first or second time need 2 doses for maximum immune response. Past algorithms that aided in deciding which children needed 2 doses instead of one considered not only the number and timing of previous doses but also whether the product had contained pandemic H1N1 antigen. The algorithm for the coming year asks just one question: How many doses of influenza vaccine has the child received previously? This is without regard to when or to the specific products. If the answer is 2 or more doses (not necessarily given in the same season or even in consecutive seasons), only one dose is needed this season. If the answer is “one dose” or “none,” 2 doses are recommended this season, separated by at least 4 weeks.

Considerations for individual patients

The Advisory Committee on Immunization Practices (ACIP) recommends annual influenza vaccination for everyone ages 6 months and older who do not have a contraindication. It states no preference for any product for any age. Last year’s preference for LAIV over IIV for children through age 8 years has been changed; either LAIV or IIV is appropriate for this age group.¹ Although quadrivalent vaccines offer some added protection with an additional B virus, do not delay vaccination if only a trivalent product is available.

Use the LAIV only for individuals ages 2 years to 49 years who do not have a contraindication listed in the TABLE.¹ There are other conditions that pose a theoretical increased risk of complications with the use of LAIV (TABLE), but they do not preclude the use of the vaccine. Additionally, anyone providing care for a severely immunosuppressed individual should avoid being vaccinated with LAIV or, if vaccinated with the live virus, avoid contact with the individual for 7 days following vaccination.

Recommendations for use of influenza vaccines in those who say they are allergic to eggs remain unchanged from last year (FIGURE¹). The amount of egg protein in influenza vaccines is very low and serious allergic reactions are rare. The availability of trivalent recombinant vaccine provides an egg-free option for those ages 18 and older.

Vaccines are not all we have to protect the public

The dosing algorithm for children this season asks just one question: How many doses of influenza vaccine has the child received previously?

Remember that while influenza vaccines are recommended and are the most effective intervention to prevent influenza morbidity and mortality, they are imperfect. Their effectiveness varies from year to year, and it wanes with time after administration. The proportion of the population vaccinated is also suboptimal, which makes other prevention interventions important to implement. These include good infection control practices in all health care facilities, social distancing of those who are infectious, infection control practices in homes with an infected person, vaccination of all health care workers, and judicious use of pre- and post-exposure chemoprevention when indicated. These have all been discussed in a previous Practice Alert.⁵

The Centers for Disease Control and Prevention (CDC) has published its recommendations for the use of influenza vaccines for the 2015-2016 influenza season.¹ (See the CDC’s Web site at http://www.cdc.gov/flu/professionals/vaccination/index.htm.) This Practice Alert describes recent changes in vaccine products, discusses the timing of vaccination, reviews a new algorithm for deciding on the number of doses for children ages 6 months through 8 years, and raises issues to consider when thinking about specific products for individual patients.

Vaccine product modifications for 2015-2016

Influenza vaccines contain either 3 or 4 antigens (trivalent or quadrivalent) and either inactivated or modified live viruses (inactivated influenza vaccine [IIV]) or live attenuated influenza vaccine [LAIV]). These vaccines are produced in eggs, by cell cultures, or with recombinant technology. The vaccine products for this influenza season will contain antigens of one H1N1 virus, one H3N2 virus, and one B virus (trivalent products) or 2 B viruses (quadrivalent products).

The viruses selected for the vaccine are based on the most prevalent types in circulation globally, and vaccine effectiveness in the United States will be directly proportional to how well these strains match those circulating here during the influenza season. In the 2014-2015 influenza season, an antigenic drift in the circulating H3N2 virus rendered influenza vaccines only 23% effective in preventing laboratory-confirmed influenza.²

One product, Afluria, an IIV trivalent product, has been approved for administration using a needle-free jet injector in individuals ages 18 to 64 years.¹ Additionally, intramuscular injection of Afluria is still available for those 18 years and older. All other IIV products are administered via needle and syringe. Vaccination with a jet injector achieves protection equivalent to needle injection, but its use is associated with higher rates of local reactions.

Flublok, another trivalent IIV product, is produced using a recombinant egg-free process. It was originally approved for individuals 18 to 49 years; there is now no upper age limit, providing an egg-free option for an expanded age group. An intradermal option, Fluzone, was a trivalent product last year and will be replaced by Fluzone Intradermal Quadrivalent this season.

A complete list of all influenza products and their respective patient-specific recommendations can be found on the CDC influenza Web site (http://www.cdc.gov/flu/professionals/vaccination/index.htm).

Timing of vaccine administration

Flublok, a trivalent IIV recombinant egg-free product, now has no upper age limit for administration.

Start providing influenza vaccine by the beginning of October and continue offering it throughout the influenza season to those who are unimmunized. In the past, the recommendation was to begin vaccination as soon as vaccine was available. But studies have shown that vaccine effectiveness declines after 6 months, especially among those over age 65 years, which can result in inadequate protection late in the influenza season.^3,4 Administering the vaccine later in the fall may confer greater protection later in the season, but, as a strategy, it could also lead to missed opportunities to vaccinate.

Annual vaccination for the entire population is a public health challenge and the October start date is appropriate middle ground. However, children who need 2 doses should receive the first dose as soon as vaccine is available, and the second dose 4 weeks later.¹

One or two doses in children?

Children ages 6 months through 8 years who are receiving influenza vaccine for the first or second time need 2 doses for maximum immune response. Past algorithms that aided in deciding which children needed 2 doses instead of one considered not only the number and timing of previous doses but also whether the product had contained pandemic H1N1 antigen. The algorithm for the coming year asks just one question: How many doses of influenza vaccine has the child received previously? This is without regard to when or to the specific products. If the answer is 2 or more doses (not necessarily given in the same season or even in consecutive seasons), only one dose is needed this season. If the answer is “one dose” or “none,” 2 doses are recommended this season, separated by at least 4 weeks.

Considerations for individual patients

The Advisory Committee on Immunization Practices (ACIP) recommends annual influenza vaccination for everyone ages 6 months and older who do not have a contraindication. It states no preference for any product for any age. Last year’s preference for LAIV over IIV for children through age 8 years has been changed; either LAIV or IIV is appropriate for this age group.¹ Although quadrivalent vaccines offer some added protection with an additional B virus, do not delay vaccination if only a trivalent product is available.

Use the LAIV only for individuals ages 2 years to 49 years who do not have a contraindication listed in the TABLE.¹ There are other conditions that pose a theoretical increased risk of complications with the use of LAIV (TABLE), but they do not preclude the use of the vaccine. Additionally, anyone providing care for a severely immunosuppressed individual should avoid being vaccinated with LAIV or, if vaccinated with the live virus, avoid contact with the individual for 7 days following vaccination.

Recommendations for use of influenza vaccines in those who say they are allergic to eggs remain unchanged from last year (FIGURE¹). The amount of egg protein in influenza vaccines is very low and serious allergic reactions are rare. The availability of trivalent recombinant vaccine provides an egg-free option for those ages 18 and older.

Vaccines are not all we have to protect the public

The dosing algorithm for children this season asks just one question: How many doses of influenza vaccine has the child received previously?

Remember that while influenza vaccines are recommended and are the most effective intervention to prevent influenza morbidity and mortality, they are imperfect. Their effectiveness varies from year to year, and it wanes with time after administration. The proportion of the population vaccinated is also suboptimal, which makes other prevention interventions important to implement. These include good infection control practices in all health care facilities, social distancing of those who are infectious, infection control practices in homes with an infected person, vaccination of all health care workers, and judicious use of pre- and post-exposure chemoprevention when indicated. These have all been discussed in a previous Practice Alert.⁵

The Centers for Disease Control and Prevention (CDC) has published its recommendations for the use of influenza vaccines for the 2015-2016 influenza season.¹ (See the CDC’s Web site at http://www.cdc.gov/flu/professionals/vaccination/index.htm.) This Practice Alert describes recent changes in vaccine products, discusses the timing of vaccination, reviews a new algorithm for deciding on the number of doses for children ages 6 months through 8 years, and raises issues to consider when thinking about specific products for individual patients.

Vaccine product modifications for 2015-2016

Influenza vaccines contain either 3 or 4 antigens (trivalent or quadrivalent) and either inactivated or modified live viruses (inactivated influenza vaccine [IIV]) or live attenuated influenza vaccine [LAIV]). These vaccines are produced in eggs, by cell cultures, or with recombinant technology. The vaccine products for this influenza season will contain antigens of one H1N1 virus, one H3N2 virus, and one B virus (trivalent products) or 2 B viruses (quadrivalent products).

The viruses selected for the vaccine are based on the most prevalent types in circulation globally, and vaccine effectiveness in the United States will be directly proportional to how well these strains match those circulating here during the influenza season. In the 2014-2015 influenza season, an antigenic drift in the circulating H3N2 virus rendered influenza vaccines only 23% effective in preventing laboratory-confirmed influenza.²

One product, Afluria, an IIV trivalent product, has been approved for administration using a needle-free jet injector in individuals ages 18 to 64 years.¹ Additionally, intramuscular injection of Afluria is still available for those 18 years and older. All other IIV products are administered via needle and syringe. Vaccination with a jet injector achieves protection equivalent to needle injection, but its use is associated with higher rates of local reactions.

Flublok, another trivalent IIV product, is produced using a recombinant egg-free process. It was originally approved for individuals 18 to 49 years; there is now no upper age limit, providing an egg-free option for an expanded age group. An intradermal option, Fluzone, was a trivalent product last year and will be replaced by Fluzone Intradermal Quadrivalent this season.

A complete list of all influenza products and their respective patient-specific recommendations can be found on the CDC influenza Web site (http://www.cdc.gov/flu/professionals/vaccination/index.htm).

Timing of vaccine administration

Flublok, a trivalent IIV recombinant egg-free product, now has no upper age limit for administration.

Start providing influenza vaccine by the beginning of October and continue offering it throughout the influenza season to those who are unimmunized. In the past, the recommendation was to begin vaccination as soon as vaccine was available. But studies have shown that vaccine effectiveness declines after 6 months, especially among those over age 65 years, which can result in inadequate protection late in the influenza season.^3,4 Administering the vaccine later in the fall may confer greater protection later in the season, but, as a strategy, it could also lead to missed opportunities to vaccinate.

Annual vaccination for the entire population is a public health challenge and the October start date is appropriate middle ground. However, children who need 2 doses should receive the first dose as soon as vaccine is available, and the second dose 4 weeks later.¹

One or two doses in children?

Children ages 6 months through 8 years who are receiving influenza vaccine for the first or second time need 2 doses for maximum immune response. Past algorithms that aided in deciding which children needed 2 doses instead of one considered not only the number and timing of previous doses but also whether the product had contained pandemic H1N1 antigen. The algorithm for the coming year asks just one question: How many doses of influenza vaccine has the child received previously? This is without regard to when or to the specific products. If the answer is 2 or more doses (not necessarily given in the same season or even in consecutive seasons), only one dose is needed this season. If the answer is “one dose” or “none,” 2 doses are recommended this season, separated by at least 4 weeks.

Considerations for individual patients

The Advisory Committee on Immunization Practices (ACIP) recommends annual influenza vaccination for everyone ages 6 months and older who do not have a contraindication. It states no preference for any product for any age. Last year’s preference for LAIV over IIV for children through age 8 years has been changed; either LAIV or IIV is appropriate for this age group.¹ Although quadrivalent vaccines offer some added protection with an additional B virus, do not delay vaccination if only a trivalent product is available.

Use the LAIV only for individuals ages 2 years to 49 years who do not have a contraindication listed in the TABLE.¹ There are other conditions that pose a theoretical increased risk of complications with the use of LAIV (TABLE), but they do not preclude the use of the vaccine. Additionally, anyone providing care for a severely immunosuppressed individual should avoid being vaccinated with LAIV or, if vaccinated with the live virus, avoid contact with the individual for 7 days following vaccination.

Recommendations for use of influenza vaccines in those who say they are allergic to eggs remain unchanged from last year (FIGURE¹). The amount of egg protein in influenza vaccines is very low and serious allergic reactions are rare. The availability of trivalent recombinant vaccine provides an egg-free option for those ages 18 and older.

Vaccines are not all we have to protect the public

The dosing algorithm for children this season asks just one question: How many doses of influenza vaccine has the child received previously?

Remember that while influenza vaccines are recommended and are the most effective intervention to prevent influenza morbidity and mortality, they are imperfect. Their effectiveness varies from year to year, and it wanes with time after administration. The proportion of the population vaccinated is also suboptimal, which makes other prevention interventions important to implement. These include good infection control practices in all health care facilities, social distancing of those who are infectious, infection control practices in homes with an infected person, vaccination of all health care workers, and judicious use of pre- and post-exposure chemoprevention when indicated. These have all been discussed in a previous Practice Alert.⁵

Applying for a residency program can be a stressful process for medical students. It is a combination of applying for a job in the “real world” and applying to a college or medical school. In certain fields of medicine or surgery, there may be over 600 residency applications for 40 to 80 interviewee slots. Different specialties, as well as programs within a given specialty, take a different number of residents per year. This can vary from 1 to over 20 available spots, depending on the field of medicine or surgery as well as the specific program. Orthopedic surgery residencies, for example, can match between 2 and 12 residents each year. During the 2013–2014 academic year at our institution, there were over 600 applications received for approximately 50 interview slots for a class of 5 orthopedic surgery residents. Nationally, according to publicly available 2013 National Resident Matching Program (NRMP) data, a total of 1038 applicants (833 US medical school seniors) applied for 693 spots in orthopedic surgery, of which 692 were filled, indicating that orthopedic surgery remains one of the most desired fields among medical school seniors.¹ Looking at the statistics provided by the NRMP data, orthopedic applicants remain some of the most competitive, with proportionally higher board scores, publication numbers, and grades, among other factors.¹

Each individual program has its own method for sifting through the applications. At some institutions, the individual “in charge” of the selection committee may look through all applications initially, narrow them down, and then distribute them to the other members of the selection committee to determine the final interviewee list. At other institutions, the initial group of applications may be divided and distributed to the committee members so that each member reviews the applications and ultimately decides upon the interview candidates.

The Electronic Residency Application Service (ERAS) application includes the applicant’s name, birth city, current place of residence, education history, standardized test scores, grades achieved during medical school, letters of recommendation, personal statement, extracurricular activities, volunteer activities, research experience, and languages spoken, along with several other pieces of data, all intended to be able to give the committee a better understanding of the applicant. Interestingly, however, the application also includes a photograph of the applicant.

Countless authors have demonstrated that we make assumptions and reach conclusions without even being aware that this is occurring. This is the theory of “unconscious bias.”^2-5 Unconscious bias applies to how we perceive other people, and occurs when subconscious beliefs or unrecognized stereotypes about specific characteristics, including gender, ethnicity, religion, socioeconomic status, age, and sexual orientation, result in an automatic and unconscious reaction and/or behavior.⁶ Unconscious bias has the ability to affect everything from how health care is delivered to how employees are hired.^7-12 We are all biased, and becoming aware of our biases will help us mitigate them in the workplace.

Title VII of the Civil Rights Act of 1964 requires that employers rely solely on job-related qualifications, and not physical characteristics, in their interviewing and hiring process. The US Equal Employment Opportunity Commission (EEOC), the federal agency that enforces Title VII, includes asking for photographs during the application stage on its list of prohibited practices for employers.¹³ It is our belief that including a photograph in the ERAS application, prior to the selection of interview candidates, may produce unconscious bias in the decision for granting (or not granting) an interview, and this component of the application should be eliminated.

Using a wide spectrum of cultural backgrounds in employers, Dion and colleagues¹⁴ demonstrated that the “what is beautiful is good” bias is present in all cultures when prospective employees are closely matched in qualification. Attractive individuals are thought to have better professional lives and stable marital relationships and personalities, according to previous studies.¹⁴ There has been much research aimed at determining if physical attractiveness is a factor in hiring, and the evidence suggests that the more attractive the applicant is, the greater the chances of being hired.¹⁵ Specifically, Watkins and Johnston¹⁵ have found that attractive people are thought to have better personalities than less attractive people, and that a photograph can influence the hiring decision process.

Bradley Ruffle at Ben-Gurion University and Ze’ev Shtudiner at Ariel University looked at what happens when job hunters include photographs with their curricula vitae (CV), as is the norm in much of Europe and Asia.¹⁶ For over 2500 job postings, they sent 2 identical résumés: one with a photograph and one without a photograph. An equal number of male and female applicants were sent to each posting, as were an equal number of attractive and plain-looking photographs; applications without photographs were also sent as a control group. For men, the results were as expected: CVs of “attractive” men were more likely to elicit a response from the employer (19.7%) compared with those of no-picture men (13.7%) and plain-looking men (9.2%). Interestingly, men who were viewed as “plain-looking” were better off not including a photograph. For the female applicants, however, the results were unexpected: CVs of women without a picture elicited the highest response rate (16.6%), while CVs of “plain-looking” women (13.6%) and of “attractive” women (12.8%) were less likely to receive a response.¹⁶

It is an unfortunate reality that personal preference, bias, and, in some cases, discriminatory hiring practices all factor into the selection process.¹⁷ This is why, as described above, the EEOC includes asking for photographs during the application stage on its list of prohibited practices for employers.¹³ The EEOC website also states: “If needed for identification purposes, a photograph may be obtained after an offer of employment is made and accepted.”¹³ In the residency application scenario, once an applicant has been granted an interview, a photograph can be taken on the day of the interview. With so many interviewees, this may help the interviewers to remember the interviewee. At this point in the process, the applicant has already been granted the interview. The bias associated with merely looking at a photograph is thus eliminated. This is in accordance with Title VII and is clearly different than including a photograph in the initial application, which directly violates Title VII.

Reviewers of applicants may have an unconscious bias due to the applicant’s attractiveness, race, sex, ethnicity, etc. Other, subtler forms of bias may also be present. Without realizing it, people may judge the quality of the photograph, or even what the applicant was wearing in the photograph. In orthopedic surgery, for example, there may be bias in the “size” of the applicant regardless of sex. Reviewers may unconsciously think how is he/she going to hold the leg, cut a rod, reduce a hip, etc. Without even realizing it, this may sway the person reviewing the application to choose one applicant over another. This may occur regardless of the applicant’s actual qualifications as based on the previously described factors, including test scores, grades during medical school, letters of recommendation, personal statement, extracurricular activities, volunteer activities, and research experience.

Unconscious bias is present in everyone. In an ideal world, one would be able to eliminate all sources of unconscious bias in the application process. Bias due to attending an Ivy League school versus a state school, bias due to where the applicant is from, bias due to who wrote the letter of recommendation, along with various other sources of unconscious bias, would be able to be eliminated. Unfortunately, this is not possible. What is possible, however, is to remove the photograph from the application process and to comply with Title VII of the Civil Rights Act of 1964.

Applying for a residency program can be a stressful process for medical students. It is a combination of applying for a job in the “real world” and applying to a college or medical school. In certain fields of medicine or surgery, there may be over 600 residency applications for 40 to 80 interviewee slots. Different specialties, as well as programs within a given specialty, take a different number of residents per year. This can vary from 1 to over 20 available spots, depending on the field of medicine or surgery as well as the specific program. Orthopedic surgery residencies, for example, can match between 2 and 12 residents each year. During the 2013–2014 academic year at our institution, there were over 600 applications received for approximately 50 interview slots for a class of 5 orthopedic surgery residents. Nationally, according to publicly available 2013 National Resident Matching Program (NRMP) data, a total of 1038 applicants (833 US medical school seniors) applied for 693 spots in orthopedic surgery, of which 692 were filled, indicating that orthopedic surgery remains one of the most desired fields among medical school seniors.¹ Looking at the statistics provided by the NRMP data, orthopedic applicants remain some of the most competitive, with proportionally higher board scores, publication numbers, and grades, among other factors.¹

Each individual program has its own method for sifting through the applications. At some institutions, the individual “in charge” of the selection committee may look through all applications initially, narrow them down, and then distribute them to the other members of the selection committee to determine the final interviewee list. At other institutions, the initial group of applications may be divided and distributed to the committee members so that each member reviews the applications and ultimately decides upon the interview candidates.

The Electronic Residency Application Service (ERAS) application includes the applicant’s name, birth city, current place of residence, education history, standardized test scores, grades achieved during medical school, letters of recommendation, personal statement, extracurricular activities, volunteer activities, research experience, and languages spoken, along with several other pieces of data, all intended to be able to give the committee a better understanding of the applicant. Interestingly, however, the application also includes a photograph of the applicant.

Countless authors have demonstrated that we make assumptions and reach conclusions without even being aware that this is occurring. This is the theory of “unconscious bias.”^2-5 Unconscious bias applies to how we perceive other people, and occurs when subconscious beliefs or unrecognized stereotypes about specific characteristics, including gender, ethnicity, religion, socioeconomic status, age, and sexual orientation, result in an automatic and unconscious reaction and/or behavior.⁶ Unconscious bias has the ability to affect everything from how health care is delivered to how employees are hired.^7-12 We are all biased, and becoming aware of our biases will help us mitigate them in the workplace.

Title VII of the Civil Rights Act of 1964 requires that employers rely solely on job-related qualifications, and not physical characteristics, in their interviewing and hiring process. The US Equal Employment Opportunity Commission (EEOC), the federal agency that enforces Title VII, includes asking for photographs during the application stage on its list of prohibited practices for employers.¹³ It is our belief that including a photograph in the ERAS application, prior to the selection of interview candidates, may produce unconscious bias in the decision for granting (or not granting) an interview, and this component of the application should be eliminated.

Using a wide spectrum of cultural backgrounds in employers, Dion and colleagues¹⁴ demonstrated that the “what is beautiful is good” bias is present in all cultures when prospective employees are closely matched in qualification. Attractive individuals are thought to have better professional lives and stable marital relationships and personalities, according to previous studies.¹⁴ There has been much research aimed at determining if physical attractiveness is a factor in hiring, and the evidence suggests that the more attractive the applicant is, the greater the chances of being hired.¹⁵ Specifically, Watkins and Johnston¹⁵ have found that attractive people are thought to have better personalities than less attractive people, and that a photograph can influence the hiring decision process.

Bradley Ruffle at Ben-Gurion University and Ze’ev Shtudiner at Ariel University looked at what happens when job hunters include photographs with their curricula vitae (CV), as is the norm in much of Europe and Asia.¹⁶ For over 2500 job postings, they sent 2 identical résumés: one with a photograph and one without a photograph. An equal number of male and female applicants were sent to each posting, as were an equal number of attractive and plain-looking photographs; applications without photographs were also sent as a control group. For men, the results were as expected: CVs of “attractive” men were more likely to elicit a response from the employer (19.7%) compared with those of no-picture men (13.7%) and plain-looking men (9.2%). Interestingly, men who were viewed as “plain-looking” were better off not including a photograph. For the female applicants, however, the results were unexpected: CVs of women without a picture elicited the highest response rate (16.6%), while CVs of “plain-looking” women (13.6%) and of “attractive” women (12.8%) were less likely to receive a response.¹⁶

It is an unfortunate reality that personal preference, bias, and, in some cases, discriminatory hiring practices all factor into the selection process.¹⁷ This is why, as described above, the EEOC includes asking for photographs during the application stage on its list of prohibited practices for employers.¹³ The EEOC website also states: “If needed for identification purposes, a photograph may be obtained after an offer of employment is made and accepted.”¹³ In the residency application scenario, once an applicant has been granted an interview, a photograph can be taken on the day of the interview. With so many interviewees, this may help the interviewers to remember the interviewee. At this point in the process, the applicant has already been granted the interview. The bias associated with merely looking at a photograph is thus eliminated. This is in accordance with Title VII and is clearly different than including a photograph in the initial application, which directly violates Title VII.

Reviewers of applicants may have an unconscious bias due to the applicant’s attractiveness, race, sex, ethnicity, etc. Other, subtler forms of bias may also be present. Without realizing it, people may judge the quality of the photograph, or even what the applicant was wearing in the photograph. In orthopedic surgery, for example, there may be bias in the “size” of the applicant regardless of sex. Reviewers may unconsciously think how is he/she going to hold the leg, cut a rod, reduce a hip, etc. Without even realizing it, this may sway the person reviewing the application to choose one applicant over another. This may occur regardless of the applicant’s actual qualifications as based on the previously described factors, including test scores, grades during medical school, letters of recommendation, personal statement, extracurricular activities, volunteer activities, and research experience.

Unconscious bias is present in everyone. In an ideal world, one would be able to eliminate all sources of unconscious bias in the application process. Bias due to attending an Ivy League school versus a state school, bias due to where the applicant is from, bias due to who wrote the letter of recommendation, along with various other sources of unconscious bias, would be able to be eliminated. Unfortunately, this is not possible. What is possible, however, is to remove the photograph from the application process and to comply with Title VII of the Civil Rights Act of 1964.

Applying for a residency program can be a stressful process for medical students. It is a combination of applying for a job in the “real world” and applying to a college or medical school. In certain fields of medicine or surgery, there may be over 600 residency applications for 40 to 80 interviewee slots. Different specialties, as well as programs within a given specialty, take a different number of residents per year. This can vary from 1 to over 20 available spots, depending on the field of medicine or surgery as well as the specific program. Orthopedic surgery residencies, for example, can match between 2 and 12 residents each year. During the 2013–2014 academic year at our institution, there were over 600 applications received for approximately 50 interview slots for a class of 5 orthopedic surgery residents. Nationally, according to publicly available 2013 National Resident Matching Program (NRMP) data, a total of 1038 applicants (833 US medical school seniors) applied for 693 spots in orthopedic surgery, of which 692 were filled, indicating that orthopedic surgery remains one of the most desired fields among medical school seniors.¹ Looking at the statistics provided by the NRMP data, orthopedic applicants remain some of the most competitive, with proportionally higher board scores, publication numbers, and grades, among other factors.¹

Each individual program has its own method for sifting through the applications. At some institutions, the individual “in charge” of the selection committee may look through all applications initially, narrow them down, and then distribute them to the other members of the selection committee to determine the final interviewee list. At other institutions, the initial group of applications may be divided and distributed to the committee members so that each member reviews the applications and ultimately decides upon the interview candidates.

The Electronic Residency Application Service (ERAS) application includes the applicant’s name, birth city, current place of residence, education history, standardized test scores, grades achieved during medical school, letters of recommendation, personal statement, extracurricular activities, volunteer activities, research experience, and languages spoken, along with several other pieces of data, all intended to be able to give the committee a better understanding of the applicant. Interestingly, however, the application also includes a photograph of the applicant.

Countless authors have demonstrated that we make assumptions and reach conclusions without even being aware that this is occurring. This is the theory of “unconscious bias.”^2-5 Unconscious bias applies to how we perceive other people, and occurs when subconscious beliefs or unrecognized stereotypes about specific characteristics, including gender, ethnicity, religion, socioeconomic status, age, and sexual orientation, result in an automatic and unconscious reaction and/or behavior.⁶ Unconscious bias has the ability to affect everything from how health care is delivered to how employees are hired.^7-12 We are all biased, and becoming aware of our biases will help us mitigate them in the workplace.

Title VII of the Civil Rights Act of 1964 requires that employers rely solely on job-related qualifications, and not physical characteristics, in their interviewing and hiring process. The US Equal Employment Opportunity Commission (EEOC), the federal agency that enforces Title VII, includes asking for photographs during the application stage on its list of prohibited practices for employers.¹³ It is our belief that including a photograph in the ERAS application, prior to the selection of interview candidates, may produce unconscious bias in the decision for granting (or not granting) an interview, and this component of the application should be eliminated.

Using a wide spectrum of cultural backgrounds in employers, Dion and colleagues¹⁴ demonstrated that the “what is beautiful is good” bias is present in all cultures when prospective employees are closely matched in qualification. Attractive individuals are thought to have better professional lives and stable marital relationships and personalities, according to previous studies.¹⁴ There has been much research aimed at determining if physical attractiveness is a factor in hiring, and the evidence suggests that the more attractive the applicant is, the greater the chances of being hired.¹⁵ Specifically, Watkins and Johnston¹⁵ have found that attractive people are thought to have better personalities than less attractive people, and that a photograph can influence the hiring decision process.

Bradley Ruffle at Ben-Gurion University and Ze’ev Shtudiner at Ariel University looked at what happens when job hunters include photographs with their curricula vitae (CV), as is the norm in much of Europe and Asia.¹⁶ For over 2500 job postings, they sent 2 identical résumés: one with a photograph and one without a photograph. An equal number of male and female applicants were sent to each posting, as were an equal number of attractive and plain-looking photographs; applications without photographs were also sent as a control group. For men, the results were as expected: CVs of “attractive” men were more likely to elicit a response from the employer (19.7%) compared with those of no-picture men (13.7%) and plain-looking men (9.2%). Interestingly, men who were viewed as “plain-looking” were better off not including a photograph. For the female applicants, however, the results were unexpected: CVs of women without a picture elicited the highest response rate (16.6%), while CVs of “plain-looking” women (13.6%) and of “attractive” women (12.8%) were less likely to receive a response.¹⁶

It is an unfortunate reality that personal preference, bias, and, in some cases, discriminatory hiring practices all factor into the selection process.¹⁷ This is why, as described above, the EEOC includes asking for photographs during the application stage on its list of prohibited practices for employers.¹³ The EEOC website also states: “If needed for identification purposes, a photograph may be obtained after an offer of employment is made and accepted.”¹³ In the residency application scenario, once an applicant has been granted an interview, a photograph can be taken on the day of the interview. With so many interviewees, this may help the interviewers to remember the interviewee. At this point in the process, the applicant has already been granted the interview. The bias associated with merely looking at a photograph is thus eliminated. This is in accordance with Title VII and is clearly different than including a photograph in the initial application, which directly violates Title VII.

Reviewers of applicants may have an unconscious bias due to the applicant’s attractiveness, race, sex, ethnicity, etc. Other, subtler forms of bias may also be present. Without realizing it, people may judge the quality of the photograph, or even what the applicant was wearing in the photograph. In orthopedic surgery, for example, there may be bias in the “size” of the applicant regardless of sex. Reviewers may unconsciously think how is he/she going to hold the leg, cut a rod, reduce a hip, etc. Without even realizing it, this may sway the person reviewing the application to choose one applicant over another. This may occur regardless of the applicant’s actual qualifications as based on the previously described factors, including test scores, grades during medical school, letters of recommendation, personal statement, extracurricular activities, volunteer activities, and research experience.

Unconscious bias is present in everyone. In an ideal world, one would be able to eliminate all sources of unconscious bias in the application process. Bias due to attending an Ivy League school versus a state school, bias due to where the applicant is from, bias due to who wrote the letter of recommendation, along with various other sources of unconscious bias, would be able to be eliminated. Unfortunately, this is not possible. What is possible, however, is to remove the photograph from the application process and to comply with Title VII of the Civil Rights Act of 1964.

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.

Posterior spinal fusion for adolescent idiopathic scoliosis is a relatively common procedure. However, intestinal obstruction is a possible complication in the case of an asthenic adolescent with weight loss after surgery. We present the case of a 12-year-old girl who underwent an uncomplicated posterior spinal fusion with instrumentation for scoliosis and who developed nausea, emesis, and abdominal pain. We also discuss the origins, epidemiology, diagnosis, and treatment of superior mesenteric artery syndrome (SMAS), a rare condition. The patient’s parents provided written informed consent for print and electronic publication of this case report.

Case Report

The patient was a 12-year-old girl with juvenile idiopathic scoliosis. She was seen by a pediatric orthopedist at age 8 after her primary care physician noticed a curve in her back during her physical examination. Given her age and primary curve of 25º, magnetic resonance imaging was ordered, which was negative for syrinx, tethered cord, or bony abnormalities. An underarm thoracolumbosacral orthosis (Boston Brace) was prescribed to be worn 23 hours/day. There was inconsistent follow-up over the next 4 years, and her curve progressed to 55º (right thoracic) and 47º in the lumbar spine (Figures 1, 2). Given the magnitude of the curves, surgical intervention was recommended, because bracing would no longer be beneficial. 

The patient was healthy and appeared vibrant with no medical issues. She weighed 49 kg and her height was 162 cm (body mass index [BMI], 18.6; normal). She underwent segmental posterior spinal instrumentation, and a fusion was performed from T4 to L4 using a cobalt chrome rod. Postoperatively, there were no problems. Her diet was slowly advanced from clear liquids to regular food over 3 days. She was discharged on postoperative day 4. She had no abdominal distention, pain, or nausea. The family was instructed about pain medication (oxycodone liquid, 5 mg every 4 hours as needed) and how to prevent and treat constipation. 

Three days after discharge, her mother called to inquire about positioning because the patient was uncomfortable owing to back pain. There were no abdominal complaints, and she was taking her pain medicine every 4 hours. She was instructed to lie in a comfortable position and to ambulate several times daily. The patient took little food or fluids because of a lack of appetite and back pain. On postoperative day 8, she presented to the emergency department with complaints of generalized abdominal pain and 1 day’s emesis. The patient had not had a bowel movement postoperatively. An acute abdominal series (AAS) was obtained (Figure 3), which noted a nonobstructive bowel gas pattern, with some increased colonic fecal retention. The patient was given intravenous (IV) fluids and an IV anti-emetic, and was admitted for observation. The pediatric surgical team evaluated her and concluded her symptoms resulted from constipation. Her symptoms improved over 2 to 3 days, and she had several bowel movements on day 2 after taking polyethylene glycol, sennosides, and bisacodyl suppositories. At discharge, she was noted to be passing gas, and her abdominal examination revealed no tenderness or guarding. She had mild distention, but it had improved from the previous day. She ate breakfast and ambulated several times. She had no complaints of abdominal pain and was released home with her parents. Staff reiterated instructions regarding constipation, diet, and follow-up. Her discharge weight was 48 kg (down 1 kg) and her BMI was 17.2 (down 1.4; underweight). Her height was now 165 cm (up 3 cm). Postoperative radiographs noted stable fixation with corrected curves (Figures 4, 5).

At home, the patient ate little but continued to drink fluids. On postdischarge day 3, she developed nausea, bilious emesis, and generalized abdominal pain. She returned to the emergency department. At this point, the patient weighed 44.5 kg (down 6.6 kg since the initial surgery) and her BMI was 16.1 (down 2.5; underweight). She was admitted, and IV fluids were initiated. She had more than 1300 mL of bilious emesis. A nasogastric (NG) tube was inserted. Initial laboratory findings were unremarkable other than an increase in serum lipase of 261 U/L. Her amylase level was within normal limits. An AAS was again completed and showed a distended stomach and loop of small bowel below the liver with an air fluid level. There were also distended loops of bowel in the pelvis (Figure 6). 

A pediatric surgical consultant examined her the next morning. An upper gastrointestinal series (UGI) was obtained and showed air fluid levels in the stomach with prompt gastric emptying into a normal caliber duodenal bulb. However, with supine positioning, there was significant dilatation of the second portion of the duodenum with abrupt vertical cutoff just to the right of the spine, compatible with SMAS (Figure 7). There was reflux of contrast material into the stomach from the duodenum, with no passage of barium into the distal duodenum. After the UGI, a nasojejunal (NJ) feeding tube was placed. The tip was left at the beginning of the fourth part of the duodenum. Repeated attempts to pass the NJ feeding tube beyond the fourth part of the duodenum were unsuccessful because of massive gastric distention. The patient was taken to the operating room for placement of a Stamm gastrostomy feeding tube with insertion of a transgastric jejunal (G-J) feeding tube under fluoroscopy (Figure 5). The patient had the G-J feeding tube in place for 6 weeks to augment her enteral nutrition. As she gained weight, her duodenal emptying improved. She gradually transitioned to normal oral intake. She has done well since the G-J feeding tube was removed. 

Discussion

Von Rokitansky first described SMAS in the mid-1800s.¹ The exact pathology was further defined 60 years later when vascular involvement was determined to be the definitive mechanism of obstruction.^2-4 Superior mesenteric artery syndrome is caused by the superior mesenteric vessels compressing the third portion of the duodenum, resulting in an extrinsic obstruction. This syndrome is also commonly called Wilkie disease, after Dr. David Wilkie, who first published in 1927 results of a comprehensive series of 75 patients.¹ The syndrome is also known as arteriomesenteric duodenal compression, aortomesenteric syndrome, chronic duodenal ileus, megaduodenum, and cast syndrome.^1,4,5 The term cast syndrome was derived from events in 1878, when Willet applied a body cast to a scoliosis patient who died after what was termed “fatal vomiting.”³

Epidemiology, Incidence, and Prevalence

While not unheard of, SMAS is an uncommon disorder. There have been only 400 documented reports in the English-language literature since 1980.^5-8 Studies have stated that the incidence of the affected population is less than 0.4%.^5,7,9,10 However, SMAS has been reported to have a mortality rate as high as 33% because of the uncommon nature of the disease and prolonged duration between onset of symptoms and diagnosis.^7,9,11,12 The incidence of SMAS is higher after surgical procedures to correct spinal deformities, with rates between 0.5% and 4.7%.^10,12,13 Females are affected more frequently than males (3:2 ratio).^1,9,14 One large study with 80 patients that spanned 10 years reported that female incidence was 66%, and another study with 75 patients also observed that two-thirds of the patients were women.^1,7 This syndrome commonly affects patients who are tall and thin with an asthenic body habitus.^1,6,11,12 Superior mesenteric artery syndrome develops more commonly in younger patients. Previous studies noted that two-thirds of patients were between ages 10 and 39 years.^1,8 However, given the right set of medical conditions, it can occur in patients of any age.^2,9,15,16 In young, thin patients with scoliosis, the risk of developing SMAS after spinal fusion with instrumentation increases, given their already low weight coupled with the surgical intervention at the height of their longitudinal growth spurt.^1,11,12 

Other patients also at increased risk for developing SMAS include those with anorexia nervosa, psychiatric/emotional disorders, or drug addiction. It can also be found in persons on prolonged bedrest, those who have increased their activity and lost weight volitionally, or patients with illness or injuries, such as burns, trauma, or significant postoperative complications that decrease caloric intake and keep them in a supine position.^2,6,17 The syndrome can be acute or chronic in its presentation.

Anatomy and Physiology

The superior mesenteric artery (SMA) comes off the right anterolateral portion of the abdominal aorta, which is just anterior to the L1 vertebra. It passes over the third part of the duodenum, generally at the L2 level (Figure 8A). The duodenum passes across the aorta at the level of the L3 vertebral body and is suspended between the aorta and the SMA by the ligament of Treitz (Figure 8B).³ The angle between the aorta and SMA (aortomesenteric angle) typically ranges from 25º to 60º with an average of 45º (Figure 8A). The distance between the aorta and SMA at the level of the duodenum is called the aortomesenteric distance, and it normally measures from 10 mm to 28 mm. Obstruction is usually observed at 2 mm to 8 mm (Figure 8C).^1,3

Compression and outlet obstruction from narrowing of the SMA aortomesenteric angle can be caused by a multitude of problems.^3,5,9,17 In chronic conditions, narrowing of the aorto-mesenteric angle could be the result of a shortened ligament, or a low origin of the SMA on the aorta, or a high insertion of the duodenum at the ligament of Treitz. Postoperatively, any change in anatomy caused by adhesions could result in compression as well. Most commonly, however, in those with significant weight loss, such as postoperative spinal fusion patients, there is loss of retroperitoneal fat, which normally acts as a cushion around the duodenum. This allows the SMA to move posteriorly obstructing the duodenum. Lying in a recumbent position along with weight loss also puts patients at risk after surgery.^3,5,9,17 SMAS should be distinguished from other conditions that can cause duodenal obstruction, such as duodenal hematomas and congenital webs. 

Symptoms and Patient Presentation

Whether SMAS is acute or chronic, most patients with SMAS present in a similar fashion. Almost all patients with acute SMAS complain of abdominal pain, nausea, and emesis (usually bilious) that usually occur after eating. Early satiety is commonly observed, resulting from delayed gastric emptying. Abdominal pain may improve when patients lie prone and are in the knee-chest, or lateral decubitus, position. These patients frequently have upper abdominal distention because of massive retention of gastric contents.^4,6,16,18,19 Most spinal fusion patients present with these symptoms 7 to 10 days after surgery.^11-13

Diagnosis

Our first diagnostic tool is a comprehensive history and physical examination. Once that is complete, many radiologic tests can be used to confirm the anatomic abnormality. The first test ordered is a simple AAS, which may show a “double bubble sign” (Figure 6), indicative of duodenal obstruction.⁴ There are several other tests, and each facility and surgeon has a preference as to which is considered the “gold standard.” Upper gastrointestinal (GI) barium studies are the simplest and most reliable. The barium test shows foregut anatomy and, to some extent, function. In SMAS patients, one should see duodenal dilatation and failure of the contrast to flow past the third section of the duodenum, along with an abrupt termination of the barium column as the duodenum crosses the vertebrae. This is the traditional method of diagnosis. There is minimal radiation, and the cost is less than that of many other tests, but it can be uncomfortable for the patient.^1-4

At some institutions, an upper GI barium study is combined with angiography, which can be used to measure aortomesenteric angle and distance.^1,3 Other practitioners prefer computed tomography (CT) with 3-dimensional reconstruction, which allows for measurement of the aortomesenteric angle and distance. In 1 study, CT was found to have an extremely high sensitivity and specificity for these measurements.¹⁰ CT angiography also identifies the obstruction with increased sensitivity, but it is rarely necessary and provides more radiation exposure and increased cost.^1,6,14,19 Abdominal ultrasound has been used to measure the angle of the SMA and the aortomesenteric distance. When combined with endoscopy, this offers an alternative way to diagnose SMAS and decreases radiation exposure. However, it may require sedation or anesthesia.^7,15,17 Overall, 3 criteria are used to define whether a patient has SMAS: duodenal dilatation, an aortomesenteric angle that is less than 25º, and an SMA that is shown to be compressing the third part of the duodenum.⁵

Treatment

Conservative treatment of SMAS usually starts by removing any precipitating factors present, such as a splint or cast that was applied for scoliosis, or ending activity associated with significant weight loss. Medical management consists of IV hydration, anti-emetics, oral feeding restriction, posture therapy, and placement of an NG tube for decompression. In most cases, patients will need to have an NJ feeding tube passed distal to the site of obstruction. This provides access for enteral feeding, and patients will gradually gain weight, repleting their retroperitoneal fat stores, which pushes the SMA forward and relieves the pressure on the duodenum. Electrolyte balance should be closely monitored along with weight gain. A nutritionist is often consulted to prevent underfeeding, which can produce a slow return to weight gain, poor wound healing, and loss of lean body muscle mass; or overfeeding, which can result in hyperglycemia and respiratory failure. Once patients are stable on enteral feedings, they can begin a slow return to oral intake.^2-4,7,12 Total parental nutrition may be needed in some cases, but the risks associated with IV feeding usually outweigh the benefits.⁴ Almost all cases of acute SMAS can be successfully treated medically if diagnosed in a timely manner and supportive treatment begins promptly.⁷

Surgical intervention is rarely necessary for acute SMAS, but when conservative measures fail (after a 4- to 6-week trial), or in the presence of peptic ulcer disease or pancreatitis, this may become an appropriate option. In our patient, multiple attempts at passing an NJ feeding tube were unsuccessful, and she needed an operative procedure for insertion of a G-J feeding tube.  

Further surgical intervention is usually reserved for those patients with long-standing SMAS for whom medical management has failed or other issues, such as pancreatitis, colitis, or megaduodenum, have arisen. Many operations are described in the literature. A duodenojejunostomy to bypass the site of the obstruction is one option. Another is duodenal derotation (Strong procedure) to alter the aortomesenteric angle and place the third and fourth duodenal portions to the right of the SMA. Other procedures include a Roux-en-Y duodenojejunostomy and duodenal uncrossing. A lateral duodenojejunostomy between the second portion of the duodenum and the jejunum is considered the simplest surgical technique. It achieves successful outcomes in 90% of cases.^2-5,14 With regards to SMAS and scoliosis, it is extremely rare that this kind of surgical intervention would be necessary.

Conclusion

When planning operative spinal correction in scoliosis patients (especially females) who have a low BMI at the time of surgery and who have increased thoracic stiffness, be alert for signs and symptoms of SMAS. This rare complication can develop, and timely diagnosis and medical management will decrease morbidity and shorten the length of time needed for nutritional rehabilitation.

Posterior spinal fusion for adolescent idiopathic scoliosis is a relatively common procedure. However, intestinal obstruction is a possible complication in the case of an asthenic adolescent with weight loss after surgery. We present the case of a 12-year-old girl who underwent an uncomplicated posterior spinal fusion with instrumentation for scoliosis and who developed nausea, emesis, and abdominal pain. We also discuss the origins, epidemiology, diagnosis, and treatment of superior mesenteric artery syndrome (SMAS), a rare condition. The patient’s parents provided written informed consent for print and electronic publication of this case report.

Case Report

The patient was a 12-year-old girl with juvenile idiopathic scoliosis. She was seen by a pediatric orthopedist at age 8 after her primary care physician noticed a curve in her back during her physical examination. Given her age and primary curve of 25º, magnetic resonance imaging was ordered, which was negative for syrinx, tethered cord, or bony abnormalities. An underarm thoracolumbosacral orthosis (Boston Brace) was prescribed to be worn 23 hours/day. There was inconsistent follow-up over the next 4 years, and her curve progressed to 55º (right thoracic) and 47º in the lumbar spine (Figures 1, 2). Given the magnitude of the curves, surgical intervention was recommended, because bracing would no longer be beneficial. 

The patient was healthy and appeared vibrant with no medical issues. She weighed 49 kg and her height was 162 cm (body mass index [BMI], 18.6; normal). She underwent segmental posterior spinal instrumentation, and a fusion was performed from T4 to L4 using a cobalt chrome rod. Postoperatively, there were no problems. Her diet was slowly advanced from clear liquids to regular food over 3 days. She was discharged on postoperative day 4. She had no abdominal distention, pain, or nausea. The family was instructed about pain medication (oxycodone liquid, 5 mg every 4 hours as needed) and how to prevent and treat constipation. 

Three days after discharge, her mother called to inquire about positioning because the patient was uncomfortable owing to back pain. There were no abdominal complaints, and she was taking her pain medicine every 4 hours. She was instructed to lie in a comfortable position and to ambulate several times daily. The patient took little food or fluids because of a lack of appetite and back pain. On postoperative day 8, she presented to the emergency department with complaints of generalized abdominal pain and 1 day’s emesis. The patient had not had a bowel movement postoperatively. An acute abdominal series (AAS) was obtained (Figure 3), which noted a nonobstructive bowel gas pattern, with some increased colonic fecal retention. The patient was given intravenous (IV) fluids and an IV anti-emetic, and was admitted for observation. The pediatric surgical team evaluated her and concluded her symptoms resulted from constipation. Her symptoms improved over 2 to 3 days, and she had several bowel movements on day 2 after taking polyethylene glycol, sennosides, and bisacodyl suppositories. At discharge, she was noted to be passing gas, and her abdominal examination revealed no tenderness or guarding. She had mild distention, but it had improved from the previous day. She ate breakfast and ambulated several times. She had no complaints of abdominal pain and was released home with her parents. Staff reiterated instructions regarding constipation, diet, and follow-up. Her discharge weight was 48 kg (down 1 kg) and her BMI was 17.2 (down 1.4; underweight). Her height was now 165 cm (up 3 cm). Postoperative radiographs noted stable fixation with corrected curves (Figures 4, 5).

At home, the patient ate little but continued to drink fluids. On postdischarge day 3, she developed nausea, bilious emesis, and generalized abdominal pain. She returned to the emergency department. At this point, the patient weighed 44.5 kg (down 6.6 kg since the initial surgery) and her BMI was 16.1 (down 2.5; underweight). She was admitted, and IV fluids were initiated. She had more than 1300 mL of bilious emesis. A nasogastric (NG) tube was inserted. Initial laboratory findings were unremarkable other than an increase in serum lipase of 261 U/L. Her amylase level was within normal limits. An AAS was again completed and showed a distended stomach and loop of small bowel below the liver with an air fluid level. There were also distended loops of bowel in the pelvis (Figure 6). 

A pediatric surgical consultant examined her the next morning. An upper gastrointestinal series (UGI) was obtained and showed air fluid levels in the stomach with prompt gastric emptying into a normal caliber duodenal bulb. However, with supine positioning, there was significant dilatation of the second portion of the duodenum with abrupt vertical cutoff just to the right of the spine, compatible with SMAS (Figure 7). There was reflux of contrast material into the stomach from the duodenum, with no passage of barium into the distal duodenum. After the UGI, a nasojejunal (NJ) feeding tube was placed. The tip was left at the beginning of the fourth part of the duodenum. Repeated attempts to pass the NJ feeding tube beyond the fourth part of the duodenum were unsuccessful because of massive gastric distention. The patient was taken to the operating room for placement of a Stamm gastrostomy feeding tube with insertion of a transgastric jejunal (G-J) feeding tube under fluoroscopy (Figure 5). The patient had the G-J feeding tube in place for 6 weeks to augment her enteral nutrition. As she gained weight, her duodenal emptying improved. She gradually transitioned to normal oral intake. She has done well since the G-J feeding tube was removed. 

Discussion

Von Rokitansky first described SMAS in the mid-1800s.¹ The exact pathology was further defined 60 years later when vascular involvement was determined to be the definitive mechanism of obstruction.^2-4 Superior mesenteric artery syndrome is caused by the superior mesenteric vessels compressing the third portion of the duodenum, resulting in an extrinsic obstruction. This syndrome is also commonly called Wilkie disease, after Dr. David Wilkie, who first published in 1927 results of a comprehensive series of 75 patients.¹ The syndrome is also known as arteriomesenteric duodenal compression, aortomesenteric syndrome, chronic duodenal ileus, megaduodenum, and cast syndrome.^1,4,5 The term cast syndrome was derived from events in 1878, when Willet applied a body cast to a scoliosis patient who died after what was termed “fatal vomiting.”³

Epidemiology, Incidence, and Prevalence

While not unheard of, SMAS is an uncommon disorder. There have been only 400 documented reports in the English-language literature since 1980.^5-8 Studies have stated that the incidence of the affected population is less than 0.4%.^5,7,9,10 However, SMAS has been reported to have a mortality rate as high as 33% because of the uncommon nature of the disease and prolonged duration between onset of symptoms and diagnosis.^7,9,11,12 The incidence of SMAS is higher after surgical procedures to correct spinal deformities, with rates between 0.5% and 4.7%.^10,12,13 Females are affected more frequently than males (3:2 ratio).^1,9,14 One large study with 80 patients that spanned 10 years reported that female incidence was 66%, and another study with 75 patients also observed that two-thirds of the patients were women.^1,7 This syndrome commonly affects patients who are tall and thin with an asthenic body habitus.^1,6,11,12 Superior mesenteric artery syndrome develops more commonly in younger patients. Previous studies noted that two-thirds of patients were between ages 10 and 39 years.^1,8 However, given the right set of medical conditions, it can occur in patients of any age.^2,9,15,16 In young, thin patients with scoliosis, the risk of developing SMAS after spinal fusion with instrumentation increases, given their already low weight coupled with the surgical intervention at the height of their longitudinal growth spurt.^1,11,12 

Other patients also at increased risk for developing SMAS include those with anorexia nervosa, psychiatric/emotional disorders, or drug addiction. It can also be found in persons on prolonged bedrest, those who have increased their activity and lost weight volitionally, or patients with illness or injuries, such as burns, trauma, or significant postoperative complications that decrease caloric intake and keep them in a supine position.^2,6,17 The syndrome can be acute or chronic in its presentation.

Anatomy and Physiology

The superior mesenteric artery (SMA) comes off the right anterolateral portion of the abdominal aorta, which is just anterior to the L1 vertebra. It passes over the third part of the duodenum, generally at the L2 level (Figure 8A). The duodenum passes across the aorta at the level of the L3 vertebral body and is suspended between the aorta and the SMA by the ligament of Treitz (Figure 8B).³ The angle between the aorta and SMA (aortomesenteric angle) typically ranges from 25º to 60º with an average of 45º (Figure 8A). The distance between the aorta and SMA at the level of the duodenum is called the aortomesenteric distance, and it normally measures from 10 mm to 28 mm. Obstruction is usually observed at 2 mm to 8 mm (Figure 8C).^1,3

Compression and outlet obstruction from narrowing of the SMA aortomesenteric angle can be caused by a multitude of problems.^3,5,9,17 In chronic conditions, narrowing of the aorto-mesenteric angle could be the result of a shortened ligament, or a low origin of the SMA on the aorta, or a high insertion of the duodenum at the ligament of Treitz. Postoperatively, any change in anatomy caused by adhesions could result in compression as well. Most commonly, however, in those with significant weight loss, such as postoperative spinal fusion patients, there is loss of retroperitoneal fat, which normally acts as a cushion around the duodenum. This allows the SMA to move posteriorly obstructing the duodenum. Lying in a recumbent position along with weight loss also puts patients at risk after surgery.^3,5,9,17 SMAS should be distinguished from other conditions that can cause duodenal obstruction, such as duodenal hematomas and congenital webs. 

Symptoms and Patient Presentation

Whether SMAS is acute or chronic, most patients with SMAS present in a similar fashion. Almost all patients with acute SMAS complain of abdominal pain, nausea, and emesis (usually bilious) that usually occur after eating. Early satiety is commonly observed, resulting from delayed gastric emptying. Abdominal pain may improve when patients lie prone and are in the knee-chest, or lateral decubitus, position. These patients frequently have upper abdominal distention because of massive retention of gastric contents.^4,6,16,18,19 Most spinal fusion patients present with these symptoms 7 to 10 days after surgery.^11-13

Diagnosis

Our first diagnostic tool is a comprehensive history and physical examination. Once that is complete, many radiologic tests can be used to confirm the anatomic abnormality. The first test ordered is a simple AAS, which may show a “double bubble sign” (Figure 6), indicative of duodenal obstruction.⁴ There are several other tests, and each facility and surgeon has a preference as to which is considered the “gold standard.” Upper gastrointestinal (GI) barium studies are the simplest and most reliable. The barium test shows foregut anatomy and, to some extent, function. In SMAS patients, one should see duodenal dilatation and failure of the contrast to flow past the third section of the duodenum, along with an abrupt termination of the barium column as the duodenum crosses the vertebrae. This is the traditional method of diagnosis. There is minimal radiation, and the cost is less than that of many other tests, but it can be uncomfortable for the patient.^1-4

At some institutions, an upper GI barium study is combined with angiography, which can be used to measure aortomesenteric angle and distance.^1,3 Other practitioners prefer computed tomography (CT) with 3-dimensional reconstruction, which allows for measurement of the aortomesenteric angle and distance. In 1 study, CT was found to have an extremely high sensitivity and specificity for these measurements.¹⁰ CT angiography also identifies the obstruction with increased sensitivity, but it is rarely necessary and provides more radiation exposure and increased cost.^1,6,14,19 Abdominal ultrasound has been used to measure the angle of the SMA and the aortomesenteric distance. When combined with endoscopy, this offers an alternative way to diagnose SMAS and decreases radiation exposure. However, it may require sedation or anesthesia.^7,15,17 Overall, 3 criteria are used to define whether a patient has SMAS: duodenal dilatation, an aortomesenteric angle that is less than 25º, and an SMA that is shown to be compressing the third part of the duodenum.⁵

Treatment

Conservative treatment of SMAS usually starts by removing any precipitating factors present, such as a splint or cast that was applied for scoliosis, or ending activity associated with significant weight loss. Medical management consists of IV hydration, anti-emetics, oral feeding restriction, posture therapy, and placement of an NG tube for decompression. In most cases, patients will need to have an NJ feeding tube passed distal to the site of obstruction. This provides access for enteral feeding, and patients will gradually gain weight, repleting their retroperitoneal fat stores, which pushes the SMA forward and relieves the pressure on the duodenum. Electrolyte balance should be closely monitored along with weight gain. A nutritionist is often consulted to prevent underfeeding, which can produce a slow return to weight gain, poor wound healing, and loss of lean body muscle mass; or overfeeding, which can result in hyperglycemia and respiratory failure. Once patients are stable on enteral feedings, they can begin a slow return to oral intake.^2-4,7,12 Total parental nutrition may be needed in some cases, but the risks associated with IV feeding usually outweigh the benefits.⁴ Almost all cases of acute SMAS can be successfully treated medically if diagnosed in a timely manner and supportive treatment begins promptly.⁷

Surgical intervention is rarely necessary for acute SMAS, but when conservative measures fail (after a 4- to 6-week trial), or in the presence of peptic ulcer disease or pancreatitis, this may become an appropriate option. In our patient, multiple attempts at passing an NJ feeding tube were unsuccessful, and she needed an operative procedure for insertion of a G-J feeding tube.  

Further surgical intervention is usually reserved for those patients with long-standing SMAS for whom medical management has failed or other issues, such as pancreatitis, colitis, or megaduodenum, have arisen. Many operations are described in the literature. A duodenojejunostomy to bypass the site of the obstruction is one option. Another is duodenal derotation (Strong procedure) to alter the aortomesenteric angle and place the third and fourth duodenal portions to the right of the SMA. Other procedures include a Roux-en-Y duodenojejunostomy and duodenal uncrossing. A lateral duodenojejunostomy between the second portion of the duodenum and the jejunum is considered the simplest surgical technique. It achieves successful outcomes in 90% of cases.^2-5,14 With regards to SMAS and scoliosis, it is extremely rare that this kind of surgical intervention would be necessary.

Conclusion

When planning operative spinal correction in scoliosis patients (especially females) who have a low BMI at the time of surgery and who have increased thoracic stiffness, be alert for signs and symptoms of SMAS. This rare complication can develop, and timely diagnosis and medical management will decrease morbidity and shorten the length of time needed for nutritional rehabilitation.

Posterior spinal fusion for adolescent idiopathic scoliosis is a relatively common procedure. However, intestinal obstruction is a possible complication in the case of an asthenic adolescent with weight loss after surgery. We present the case of a 12-year-old girl who underwent an uncomplicated posterior spinal fusion with instrumentation for scoliosis and who developed nausea, emesis, and abdominal pain. We also discuss the origins, epidemiology, diagnosis, and treatment of superior mesenteric artery syndrome (SMAS), a rare condition. The patient’s parents provided written informed consent for print and electronic publication of this case report.

Case Report

The patient was a 12-year-old girl with juvenile idiopathic scoliosis. She was seen by a pediatric orthopedist at age 8 after her primary care physician noticed a curve in her back during her physical examination. Given her age and primary curve of 25º, magnetic resonance imaging was ordered, which was negative for syrinx, tethered cord, or bony abnormalities. An underarm thoracolumbosacral orthosis (Boston Brace) was prescribed to be worn 23 hours/day. There was inconsistent follow-up over the next 4 years, and her curve progressed to 55º (right thoracic) and 47º in the lumbar spine (Figures 1, 2). Given the magnitude of the curves, surgical intervention was recommended, because bracing would no longer be beneficial. 

The patient was healthy and appeared vibrant with no medical issues. She weighed 49 kg and her height was 162 cm (body mass index [BMI], 18.6; normal). She underwent segmental posterior spinal instrumentation, and a fusion was performed from T4 to L4 using a cobalt chrome rod. Postoperatively, there were no problems. Her diet was slowly advanced from clear liquids to regular food over 3 days. She was discharged on postoperative day 4. She had no abdominal distention, pain, or nausea. The family was instructed about pain medication (oxycodone liquid, 5 mg every 4 hours as needed) and how to prevent and treat constipation. 

Three days after discharge, her mother called to inquire about positioning because the patient was uncomfortable owing to back pain. There were no abdominal complaints, and she was taking her pain medicine every 4 hours. She was instructed to lie in a comfortable position and to ambulate several times daily. The patient took little food or fluids because of a lack of appetite and back pain. On postoperative day 8, she presented to the emergency department with complaints of generalized abdominal pain and 1 day’s emesis. The patient had not had a bowel movement postoperatively. An acute abdominal series (AAS) was obtained (Figure 3), which noted a nonobstructive bowel gas pattern, with some increased colonic fecal retention. The patient was given intravenous (IV) fluids and an IV anti-emetic, and was admitted for observation. The pediatric surgical team evaluated her and concluded her symptoms resulted from constipation. Her symptoms improved over 2 to 3 days, and she had several bowel movements on day 2 after taking polyethylene glycol, sennosides, and bisacodyl suppositories. At discharge, she was noted to be passing gas, and her abdominal examination revealed no tenderness or guarding. She had mild distention, but it had improved from the previous day. She ate breakfast and ambulated several times. She had no complaints of abdominal pain and was released home with her parents. Staff reiterated instructions regarding constipation, diet, and follow-up. Her discharge weight was 48 kg (down 1 kg) and her BMI was 17.2 (down 1.4; underweight). Her height was now 165 cm (up 3 cm). Postoperative radiographs noted stable fixation with corrected curves (Figures 4, 5).

At home, the patient ate little but continued to drink fluids. On postdischarge day 3, she developed nausea, bilious emesis, and generalized abdominal pain. She returned to the emergency department. At this point, the patient weighed 44.5 kg (down 6.6 kg since the initial surgery) and her BMI was 16.1 (down 2.5; underweight). She was admitted, and IV fluids were initiated. She had more than 1300 mL of bilious emesis. A nasogastric (NG) tube was inserted. Initial laboratory findings were unremarkable other than an increase in serum lipase of 261 U/L. Her amylase level was within normal limits. An AAS was again completed and showed a distended stomach and loop of small bowel below the liver with an air fluid level. There were also distended loops of bowel in the pelvis (Figure 6). 

A pediatric surgical consultant examined her the next morning. An upper gastrointestinal series (UGI) was obtained and showed air fluid levels in the stomach with prompt gastric emptying into a normal caliber duodenal bulb. However, with supine positioning, there was significant dilatation of the second portion of the duodenum with abrupt vertical cutoff just to the right of the spine, compatible with SMAS (Figure 7). There was reflux of contrast material into the stomach from the duodenum, with no passage of barium into the distal duodenum. After the UGI, a nasojejunal (NJ) feeding tube was placed. The tip was left at the beginning of the fourth part of the duodenum. Repeated attempts to pass the NJ feeding tube beyond the fourth part of the duodenum were unsuccessful because of massive gastric distention. The patient was taken to the operating room for placement of a Stamm gastrostomy feeding tube with insertion of a transgastric jejunal (G-J) feeding tube under fluoroscopy (Figure 5). The patient had the G-J feeding tube in place for 6 weeks to augment her enteral nutrition. As she gained weight, her duodenal emptying improved. She gradually transitioned to normal oral intake. She has done well since the G-J feeding tube was removed. 

Discussion

Von Rokitansky first described SMAS in the mid-1800s.¹ The exact pathology was further defined 60 years later when vascular involvement was determined to be the definitive mechanism of obstruction.^2-4 Superior mesenteric artery syndrome is caused by the superior mesenteric vessels compressing the third portion of the duodenum, resulting in an extrinsic obstruction. This syndrome is also commonly called Wilkie disease, after Dr. David Wilkie, who first published in 1927 results of a comprehensive series of 75 patients.¹ The syndrome is also known as arteriomesenteric duodenal compression, aortomesenteric syndrome, chronic duodenal ileus, megaduodenum, and cast syndrome.^1,4,5 The term cast syndrome was derived from events in 1878, when Willet applied a body cast to a scoliosis patient who died after what was termed “fatal vomiting.”³

Epidemiology, Incidence, and Prevalence

While not unheard of, SMAS is an uncommon disorder. There have been only 400 documented reports in the English-language literature since 1980.^5-8 Studies have stated that the incidence of the affected population is less than 0.4%.^5,7,9,10 However, SMAS has been reported to have a mortality rate as high as 33% because of the uncommon nature of the disease and prolonged duration between onset of symptoms and diagnosis.^7,9,11,12 The incidence of SMAS is higher after surgical procedures to correct spinal deformities, with rates between 0.5% and 4.7%.^10,12,13 Females are affected more frequently than males (3:2 ratio).^1,9,14 One large study with 80 patients that spanned 10 years reported that female incidence was 66%, and another study with 75 patients also observed that two-thirds of the patients were women.^1,7 This syndrome commonly affects patients who are tall and thin with an asthenic body habitus.^1,6,11,12 Superior mesenteric artery syndrome develops more commonly in younger patients. Previous studies noted that two-thirds of patients were between ages 10 and 39 years.^1,8 However, given the right set of medical conditions, it can occur in patients of any age.^2,9,15,16 In young, thin patients with scoliosis, the risk of developing SMAS after spinal fusion with instrumentation increases, given their already low weight coupled with the surgical intervention at the height of their longitudinal growth spurt.^1,11,12 

Other patients also at increased risk for developing SMAS include those with anorexia nervosa, psychiatric/emotional disorders, or drug addiction. It can also be found in persons on prolonged bedrest, those who have increased their activity and lost weight volitionally, or patients with illness or injuries, such as burns, trauma, or significant postoperative complications that decrease caloric intake and keep them in a supine position.^2,6,17 The syndrome can be acute or chronic in its presentation.

Anatomy and Physiology

The superior mesenteric artery (SMA) comes off the right anterolateral portion of the abdominal aorta, which is just anterior to the L1 vertebra. It passes over the third part of the duodenum, generally at the L2 level (Figure 8A). The duodenum passes across the aorta at the level of the L3 vertebral body and is suspended between the aorta and the SMA by the ligament of Treitz (Figure 8B).³ The angle between the aorta and SMA (aortomesenteric angle) typically ranges from 25º to 60º with an average of 45º (Figure 8A). The distance between the aorta and SMA at the level of the duodenum is called the aortomesenteric distance, and it normally measures from 10 mm to 28 mm. Obstruction is usually observed at 2 mm to 8 mm (Figure 8C).^1,3

Compression and outlet obstruction from narrowing of the SMA aortomesenteric angle can be caused by a multitude of problems.^3,5,9,17 In chronic conditions, narrowing of the aorto-mesenteric angle could be the result of a shortened ligament, or a low origin of the SMA on the aorta, or a high insertion of the duodenum at the ligament of Treitz. Postoperatively, any change in anatomy caused by adhesions could result in compression as well. Most commonly, however, in those with significant weight loss, such as postoperative spinal fusion patients, there is loss of retroperitoneal fat, which normally acts as a cushion around the duodenum. This allows the SMA to move posteriorly obstructing the duodenum. Lying in a recumbent position along with weight loss also puts patients at risk after surgery.^3,5,9,17 SMAS should be distinguished from other conditions that can cause duodenal obstruction, such as duodenal hematomas and congenital webs. 

Symptoms and Patient Presentation

Whether SMAS is acute or chronic, most patients with SMAS present in a similar fashion. Almost all patients with acute SMAS complain of abdominal pain, nausea, and emesis (usually bilious) that usually occur after eating. Early satiety is commonly observed, resulting from delayed gastric emptying. Abdominal pain may improve when patients lie prone and are in the knee-chest, or lateral decubitus, position. These patients frequently have upper abdominal distention because of massive retention of gastric contents.^4,6,16,18,19 Most spinal fusion patients present with these symptoms 7 to 10 days after surgery.^11-13

Diagnosis

Our first diagnostic tool is a comprehensive history and physical examination. Once that is complete, many radiologic tests can be used to confirm the anatomic abnormality. The first test ordered is a simple AAS, which may show a “double bubble sign” (Figure 6), indicative of duodenal obstruction.⁴ There are several other tests, and each facility and surgeon has a preference as to which is considered the “gold standard.” Upper gastrointestinal (GI) barium studies are the simplest and most reliable. The barium test shows foregut anatomy and, to some extent, function. In SMAS patients, one should see duodenal dilatation and failure of the contrast to flow past the third section of the duodenum, along with an abrupt termination of the barium column as the duodenum crosses the vertebrae. This is the traditional method of diagnosis. There is minimal radiation, and the cost is less than that of many other tests, but it can be uncomfortable for the patient.^1-4

At some institutions, an upper GI barium study is combined with angiography, which can be used to measure aortomesenteric angle and distance.^1,3 Other practitioners prefer computed tomography (CT) with 3-dimensional reconstruction, which allows for measurement of the aortomesenteric angle and distance. In 1 study, CT was found to have an extremely high sensitivity and specificity for these measurements.¹⁰ CT angiography also identifies the obstruction with increased sensitivity, but it is rarely necessary and provides more radiation exposure and increased cost.^1,6,14,19 Abdominal ultrasound has been used to measure the angle of the SMA and the aortomesenteric distance. When combined with endoscopy, this offers an alternative way to diagnose SMAS and decreases radiation exposure. However, it may require sedation or anesthesia.^7,15,17 Overall, 3 criteria are used to define whether a patient has SMAS: duodenal dilatation, an aortomesenteric angle that is less than 25º, and an SMA that is shown to be compressing the third part of the duodenum.⁵

Treatment

Conservative treatment of SMAS usually starts by removing any precipitating factors present, such as a splint or cast that was applied for scoliosis, or ending activity associated with significant weight loss. Medical management consists of IV hydration, anti-emetics, oral feeding restriction, posture therapy, and placement of an NG tube for decompression. In most cases, patients will need to have an NJ feeding tube passed distal to the site of obstruction. This provides access for enteral feeding, and patients will gradually gain weight, repleting their retroperitoneal fat stores, which pushes the SMA forward and relieves the pressure on the duodenum. Electrolyte balance should be closely monitored along with weight gain. A nutritionist is often consulted to prevent underfeeding, which can produce a slow return to weight gain, poor wound healing, and loss of lean body muscle mass; or overfeeding, which can result in hyperglycemia and respiratory failure. Once patients are stable on enteral feedings, they can begin a slow return to oral intake.^2-4,7,12 Total parental nutrition may be needed in some cases, but the risks associated with IV feeding usually outweigh the benefits.⁴ Almost all cases of acute SMAS can be successfully treated medically if diagnosed in a timely manner and supportive treatment begins promptly.⁷

Surgical intervention is rarely necessary for acute SMAS, but when conservative measures fail (after a 4- to 6-week trial), or in the presence of peptic ulcer disease or pancreatitis, this may become an appropriate option. In our patient, multiple attempts at passing an NJ feeding tube were unsuccessful, and she needed an operative procedure for insertion of a G-J feeding tube.  

Further surgical intervention is usually reserved for those patients with long-standing SMAS for whom medical management has failed or other issues, such as pancreatitis, colitis, or megaduodenum, have arisen. Many operations are described in the literature. A duodenojejunostomy to bypass the site of the obstruction is one option. Another is duodenal derotation (Strong procedure) to alter the aortomesenteric angle and place the third and fourth duodenal portions to the right of the SMA. Other procedures include a Roux-en-Y duodenojejunostomy and duodenal uncrossing. A lateral duodenojejunostomy between the second portion of the duodenum and the jejunum is considered the simplest surgical technique. It achieves successful outcomes in 90% of cases.^2-5,14 With regards to SMAS and scoliosis, it is extremely rare that this kind of surgical intervention would be necessary.

Conclusion

When planning operative spinal correction in scoliosis patients (especially females) who have a low BMI at the time of surgery and who have increased thoracic stiffness, be alert for signs and symptoms of SMAS. This rare complication can develop, and timely diagnosis and medical management will decrease morbidity and shorten the length of time needed for nutritional rehabilitation.