SNOWMASS, COLO. – Progress in the understanding and treatment of immunoglobulin G4–related disease is occurring “at lightning speed,” John H. Stone, MD, said at the Winter Rheumatology Symposium sponsored by the American College of Rheumatology.

Eight or nine years ago no one had heard of immunoglobulin G4–related disease (IgG4-RD). Today, because of the broad swath the disease cuts, it’s a hot research topic in every subspecialty of medicine as well as surgery, pathology, and radiology.

Bruce Jancin/Frontline Medical News

This new understanding of IgG4-RD, he added, is opening the door to novel treatments.

“This is not a new disease. It was there when we were all in medical school, and for hundreds of years before that. But it’s really only in the last decade that we have come to understand that the disease can affect literally every organ system in the body with syndromes that we once thought were isolated organ-specific syndromes but we now recognize are part of a multiorgan disease currently called IgG4-related disease,” the rheumatologist said.

IgG4-RD is an immune-mediated fibroinflammatory condition characterized histopathologically by three hallmark features in involved tissue: obliterative phlebitis, storiform fibrosis, and a dense lymphoplasmacytic infiltrate.

Clinically, IgG4-RD often presents as a mass lesion that can affect any organ.

“I have many patients who’ve undergone modified Whipple procedures because they were thought to have adenocarcinoma of the pancreas,” according to Dr. Stone.

Other common presentations include Riedel’s thyroiditis, autoimmune pancreatitis, sclerosing cholangitis, sialadenitis, dacryoadenitis, periaortitis, an eosinophilic rash, and pseudotumor of the lung, lymph nodes, or orbits.

“Retroperitoneal fibrosis is a common and underappreciated manifestation. It may be the most common subsyndrome associated with IgG4-related disease,” he observed.

Another common presentation involves atopic disease – asthma, allergic rhinitis, eczema, eosinophilia, nasal polyps – developing out of the blue in middle age or later life. This observation led some other investigators to posit that IgG4-RD is a T-helper type 2–driven disease, an assertion debunked by Dr. Stone and coworkers (Allergy. 2014 Feb;69[2]:269-72).

Dr. Stone and his coinvestigators have published the largest series of patients with biopsy-proven IgG4-RD reported to date (Arthritis Rheumatol. 2015 Sep; 67[9]:2466-75). The average age at disease onset was 50 years. Of note, multiorgan involvement was the norm: 24% of patients had two organs involved, and 38% had three or more.

Analysis of this large patient series has led Dr. Stone to a surprising conclusion about the nature of IgG4-RD: “We have greatly overemphasized the importance of IgG4 in this condition,” he asserted.

Indeed, a mere 51% of the patients with clinically active untreated IgG4-RD in his series had an elevated serum IgG level. Dr. Stone characterized IgG4 as “kind of a wimpy antibody” incapable of driving the disease process because it is a noninflammatory immunoglobulin. This has led to speculation that IgG4 functions as what he termed an “antigen sink,” attempting to bind antigen at sites of inflammation.

But while an elevated serum IgG4 is of limited utility for diagnostic purposes, Dr. Stone and coworkers have demonstrated that it is of value as a predictor of relapse. Among patients with a treatment-induced remission, those in the top quartile in terms of baseline pretreatment serum IgG4 were 6.2-fold more likely to relapse (Rheumatology [Oxford]. 2016 Jun;55[6]:1000-8).

“This is a very useful marker for patients who are going to need chronic ongoing therapy. The notion of putting such patients on steroids for months and years is not appealing,” he said.

Levels of circulating plasmablasts as measured by peripheral blood flow cytometry, especially IgG4-positive plasmablasts, have proven much more helpful than serum IgG4 levels as a diagnostic tool, a reliable biomarker of disease activity, and a therapeutic target. Levels of these short-lived CD19+CD38+CD27+ plasmablasts are enormously elevated independent of serum IgG4 in patients with active IgG4-RD.

“One of the questions I’m most often asked is whether IgG4-related disease is a premalignant condition. My answer is no. The plasmablast expansion is oligoclonal, not polyclonal,” Dr. Stone continued.

He described IgG4-RD as “a continuous dance between T cells and B cells.” The latest thinking regarding pathogenesis is that type 2 T follicular helper cells activate B cells, which become memory B cells or plasmablasts. These activated B cells and plasmablasts present antigen to CD4+ cytotoxic T cells at sites of disease. Dr. Stone and his coinvestigators recently identified these CD4+ cytotoxic T cells as a novel population of clonally expanded T cells with SLAMF7 as a surface marker. The cells secrete interferon-gamma, interleukin-1, and transforming growth factor-beta, all of which are capable of driving the intense fibrosis characteristic of IgG4-RD. In addition, these CD4+ cytotoxic T cells secrete granzyme B and perforin, previously thought to be released mainly by natural killer T cells.

Joint American College of Rheumatology/European League Against Rheumatism classification criteria for the disease are expected to be finalized this winter at the Third International Symposium on IgG4-Related Diseases.

Treatment with rituxumab

Glucocorticoids remain the first-line therapy in IgG4-related disease, but it’s essential to bear in mind that their long-term efficacy in this immune-mediated fibroinflammatory disease is the exception rather than the rule, Dr. Stone said at the symposium.

Dr. Stone was a coauthor of an international expert consensus statement on the treatment of IgG4-related disease (IgG4-RD), which emphasized that point (Arthritis Rheumatol. 2015 Jul;67[7]:1688-99).

“I typically start with prednisone at 40 mg/day, and there’s a dramatic response in these patients. Then I taper them off after 2-3 months. If 2-3 months doesn’t put them into a long-term sustained remission, it’s time to go to something else,” said Dr. Stone.

So what’s the next move, then, after steroids fail? Dr. Stone was a pioneer in the strikingly successful use of B cell depletion via rituximab (Rituxan) in patients with IgG4-RD. First he and his coinvestigators demonstrated that this off-label use of rituximab led to rapid clinical and histologic improvement (Ann Rheum Dis. 2015 Jun; 74[6]:1171-7), then they showed it also causes levels of circulating plasmablasts, serum IgG4, and biomarkers of fibrosis to plunge, suggesting B cell depletion may halt the destructive process of collagen deposition that characterizes this immune-related disease (Ann Rheum Dis. 2015 Dec;74[12]:2236-43). They have also reported that patients with very elevated baseline serum IgG4 levels are at more than sixfold increased risk of relapse at a median of 244 days from their first rituximab infusion (Rheumatology [Oxford]. 2016 Jun;55[6]:1000-8).

The success with rituximab is just one example of how improved understanding of the pathophysiology of IgG4-RD has opened the door to novel treatments. Dr. Stone is the lead investigator in an ongoing phase II, open-label study in which 15 patients with active IgG4-RD will receive intravenous XmAb5871 every 2 weeks for 6 months to evaluate the effect on the IgG4-RD Responder Index. XmAb5871 is a monoclonal antibody that binds to CD19 and FCgammaRIIb in order to downregulate activated B cells and plasmablasts. It is also being developed for treatment of systemic lupus erythematosus.

Dr. Stone and his coinvestigators are working on a therapeutic approach to IgG4-RD that targets antigen presentation by activated B cells to CD4+ cytotoxic T cells at sites of disease. These CD4+ cytotoxic T cells contain signaling lymphocyte activation molecule F7 (SLAMF7) as a surface marker. Elotuzumab (Empliciti), an immunostimulatory humanized monoclonal antibody targeting SLAMF7, is already on the market for treatment of multiple myeloma, he noted.

Dr. Stone reported receiving IgG4-RD-related research funding from and serving as a consultant to Genentech and Xencor, which is developing XmAb5871.

[email protected]

SNOWMASS, COLO. – Progress in the understanding and treatment of immunoglobulin G4–related disease is occurring “at lightning speed,” John H. Stone, MD, said at the Winter Rheumatology Symposium sponsored by the American College of Rheumatology.

Eight or nine years ago no one had heard of immunoglobulin G4–related disease (IgG4-RD). Today, because of the broad swath the disease cuts, it’s a hot research topic in every subspecialty of medicine as well as surgery, pathology, and radiology.

Bruce Jancin/Frontline Medical News

This new understanding of IgG4-RD, he added, is opening the door to novel treatments.

“This is not a new disease. It was there when we were all in medical school, and for hundreds of years before that. But it’s really only in the last decade that we have come to understand that the disease can affect literally every organ system in the body with syndromes that we once thought were isolated organ-specific syndromes but we now recognize are part of a multiorgan disease currently called IgG4-related disease,” the rheumatologist said.

IgG4-RD is an immune-mediated fibroinflammatory condition characterized histopathologically by three hallmark features in involved tissue: obliterative phlebitis, storiform fibrosis, and a dense lymphoplasmacytic infiltrate.

Clinically, IgG4-RD often presents as a mass lesion that can affect any organ.

“I have many patients who’ve undergone modified Whipple procedures because they were thought to have adenocarcinoma of the pancreas,” according to Dr. Stone.

Other common presentations include Riedel’s thyroiditis, autoimmune pancreatitis, sclerosing cholangitis, sialadenitis, dacryoadenitis, periaortitis, an eosinophilic rash, and pseudotumor of the lung, lymph nodes, or orbits.

“Retroperitoneal fibrosis is a common and underappreciated manifestation. It may be the most common subsyndrome associated with IgG4-related disease,” he observed.

Another common presentation involves atopic disease – asthma, allergic rhinitis, eczema, eosinophilia, nasal polyps – developing out of the blue in middle age or later life. This observation led some other investigators to posit that IgG4-RD is a T-helper type 2–driven disease, an assertion debunked by Dr. Stone and coworkers (Allergy. 2014 Feb;69[2]:269-72).

Dr. Stone and his coinvestigators have published the largest series of patients with biopsy-proven IgG4-RD reported to date (Arthritis Rheumatol. 2015 Sep; 67[9]:2466-75). The average age at disease onset was 50 years. Of note, multiorgan involvement was the norm: 24% of patients had two organs involved, and 38% had three or more.

Analysis of this large patient series has led Dr. Stone to a surprising conclusion about the nature of IgG4-RD: “We have greatly overemphasized the importance of IgG4 in this condition,” he asserted.

Indeed, a mere 51% of the patients with clinically active untreated IgG4-RD in his series had an elevated serum IgG level. Dr. Stone characterized IgG4 as “kind of a wimpy antibody” incapable of driving the disease process because it is a noninflammatory immunoglobulin. This has led to speculation that IgG4 functions as what he termed an “antigen sink,” attempting to bind antigen at sites of inflammation.

But while an elevated serum IgG4 is of limited utility for diagnostic purposes, Dr. Stone and coworkers have demonstrated that it is of value as a predictor of relapse. Among patients with a treatment-induced remission, those in the top quartile in terms of baseline pretreatment serum IgG4 were 6.2-fold more likely to relapse (Rheumatology [Oxford]. 2016 Jun;55[6]:1000-8).

“This is a very useful marker for patients who are going to need chronic ongoing therapy. The notion of putting such patients on steroids for months and years is not appealing,” he said.

Levels of circulating plasmablasts as measured by peripheral blood flow cytometry, especially IgG4-positive plasmablasts, have proven much more helpful than serum IgG4 levels as a diagnostic tool, a reliable biomarker of disease activity, and a therapeutic target. Levels of these short-lived CD19+CD38+CD27+ plasmablasts are enormously elevated independent of serum IgG4 in patients with active IgG4-RD.

“One of the questions I’m most often asked is whether IgG4-related disease is a premalignant condition. My answer is no. The plasmablast expansion is oligoclonal, not polyclonal,” Dr. Stone continued.

He described IgG4-RD as “a continuous dance between T cells and B cells.” The latest thinking regarding pathogenesis is that type 2 T follicular helper cells activate B cells, which become memory B cells or plasmablasts. These activated B cells and plasmablasts present antigen to CD4+ cytotoxic T cells at sites of disease. Dr. Stone and his coinvestigators recently identified these CD4+ cytotoxic T cells as a novel population of clonally expanded T cells with SLAMF7 as a surface marker. The cells secrete interferon-gamma, interleukin-1, and transforming growth factor-beta, all of which are capable of driving the intense fibrosis characteristic of IgG4-RD. In addition, these CD4+ cytotoxic T cells secrete granzyme B and perforin, previously thought to be released mainly by natural killer T cells.

Joint American College of Rheumatology/European League Against Rheumatism classification criteria for the disease are expected to be finalized this winter at the Third International Symposium on IgG4-Related Diseases.

Treatment with rituxumab

Glucocorticoids remain the first-line therapy in IgG4-related disease, but it’s essential to bear in mind that their long-term efficacy in this immune-mediated fibroinflammatory disease is the exception rather than the rule, Dr. Stone said at the symposium.

Dr. Stone was a coauthor of an international expert consensus statement on the treatment of IgG4-related disease (IgG4-RD), which emphasized that point (Arthritis Rheumatol. 2015 Jul;67[7]:1688-99).

“I typically start with prednisone at 40 mg/day, and there’s a dramatic response in these patients. Then I taper them off after 2-3 months. If 2-3 months doesn’t put them into a long-term sustained remission, it’s time to go to something else,” said Dr. Stone.

So what’s the next move, then, after steroids fail? Dr. Stone was a pioneer in the strikingly successful use of B cell depletion via rituximab (Rituxan) in patients with IgG4-RD. First he and his coinvestigators demonstrated that this off-label use of rituximab led to rapid clinical and histologic improvement (Ann Rheum Dis. 2015 Jun; 74[6]:1171-7), then they showed it also causes levels of circulating plasmablasts, serum IgG4, and biomarkers of fibrosis to plunge, suggesting B cell depletion may halt the destructive process of collagen deposition that characterizes this immune-related disease (Ann Rheum Dis. 2015 Dec;74[12]:2236-43). They have also reported that patients with very elevated baseline serum IgG4 levels are at more than sixfold increased risk of relapse at a median of 244 days from their first rituximab infusion (Rheumatology [Oxford]. 2016 Jun;55[6]:1000-8).

The success with rituximab is just one example of how improved understanding of the pathophysiology of IgG4-RD has opened the door to novel treatments. Dr. Stone is the lead investigator in an ongoing phase II, open-label study in which 15 patients with active IgG4-RD will receive intravenous XmAb5871 every 2 weeks for 6 months to evaluate the effect on the IgG4-RD Responder Index. XmAb5871 is a monoclonal antibody that binds to CD19 and FCgammaRIIb in order to downregulate activated B cells and plasmablasts. It is also being developed for treatment of systemic lupus erythematosus.

Dr. Stone and his coinvestigators are working on a therapeutic approach to IgG4-RD that targets antigen presentation by activated B cells to CD4+ cytotoxic T cells at sites of disease. These CD4+ cytotoxic T cells contain signaling lymphocyte activation molecule F7 (SLAMF7) as a surface marker. Elotuzumab (Empliciti), an immunostimulatory humanized monoclonal antibody targeting SLAMF7, is already on the market for treatment of multiple myeloma, he noted.

Dr. Stone reported receiving IgG4-RD-related research funding from and serving as a consultant to Genentech and Xencor, which is developing XmAb5871.

[email protected]

SNOWMASS, COLO. – Progress in the understanding and treatment of immunoglobulin G4–related disease is occurring “at lightning speed,” John H. Stone, MD, said at the Winter Rheumatology Symposium sponsored by the American College of Rheumatology.

Eight or nine years ago no one had heard of immunoglobulin G4–related disease (IgG4-RD). Today, because of the broad swath the disease cuts, it’s a hot research topic in every subspecialty of medicine as well as surgery, pathology, and radiology.

Bruce Jancin/Frontline Medical News

This new understanding of IgG4-RD, he added, is opening the door to novel treatments.

“This is not a new disease. It was there when we were all in medical school, and for hundreds of years before that. But it’s really only in the last decade that we have come to understand that the disease can affect literally every organ system in the body with syndromes that we once thought were isolated organ-specific syndromes but we now recognize are part of a multiorgan disease currently called IgG4-related disease,” the rheumatologist said.

IgG4-RD is an immune-mediated fibroinflammatory condition characterized histopathologically by three hallmark features in involved tissue: obliterative phlebitis, storiform fibrosis, and a dense lymphoplasmacytic infiltrate.

Clinically, IgG4-RD often presents as a mass lesion that can affect any organ.

“I have many patients who’ve undergone modified Whipple procedures because they were thought to have adenocarcinoma of the pancreas,” according to Dr. Stone.

Other common presentations include Riedel’s thyroiditis, autoimmune pancreatitis, sclerosing cholangitis, sialadenitis, dacryoadenitis, periaortitis, an eosinophilic rash, and pseudotumor of the lung, lymph nodes, or orbits.

“Retroperitoneal fibrosis is a common and underappreciated manifestation. It may be the most common subsyndrome associated with IgG4-related disease,” he observed.

Another common presentation involves atopic disease – asthma, allergic rhinitis, eczema, eosinophilia, nasal polyps – developing out of the blue in middle age or later life. This observation led some other investigators to posit that IgG4-RD is a T-helper type 2–driven disease, an assertion debunked by Dr. Stone and coworkers (Allergy. 2014 Feb;69[2]:269-72).

Dr. Stone and his coinvestigators have published the largest series of patients with biopsy-proven IgG4-RD reported to date (Arthritis Rheumatol. 2015 Sep; 67[9]:2466-75). The average age at disease onset was 50 years. Of note, multiorgan involvement was the norm: 24% of patients had two organs involved, and 38% had three or more.

Analysis of this large patient series has led Dr. Stone to a surprising conclusion about the nature of IgG4-RD: “We have greatly overemphasized the importance of IgG4 in this condition,” he asserted.

Indeed, a mere 51% of the patients with clinically active untreated IgG4-RD in his series had an elevated serum IgG level. Dr. Stone characterized IgG4 as “kind of a wimpy antibody” incapable of driving the disease process because it is a noninflammatory immunoglobulin. This has led to speculation that IgG4 functions as what he termed an “antigen sink,” attempting to bind antigen at sites of inflammation.

But while an elevated serum IgG4 is of limited utility for diagnostic purposes, Dr. Stone and coworkers have demonstrated that it is of value as a predictor of relapse. Among patients with a treatment-induced remission, those in the top quartile in terms of baseline pretreatment serum IgG4 were 6.2-fold more likely to relapse (Rheumatology [Oxford]. 2016 Jun;55[6]:1000-8).

“This is a very useful marker for patients who are going to need chronic ongoing therapy. The notion of putting such patients on steroids for months and years is not appealing,” he said.

Levels of circulating plasmablasts as measured by peripheral blood flow cytometry, especially IgG4-positive plasmablasts, have proven much more helpful than serum IgG4 levels as a diagnostic tool, a reliable biomarker of disease activity, and a therapeutic target. Levels of these short-lived CD19+CD38+CD27+ plasmablasts are enormously elevated independent of serum IgG4 in patients with active IgG4-RD.

“One of the questions I’m most often asked is whether IgG4-related disease is a premalignant condition. My answer is no. The plasmablast expansion is oligoclonal, not polyclonal,” Dr. Stone continued.

He described IgG4-RD as “a continuous dance between T cells and B cells.” The latest thinking regarding pathogenesis is that type 2 T follicular helper cells activate B cells, which become memory B cells or plasmablasts. These activated B cells and plasmablasts present antigen to CD4+ cytotoxic T cells at sites of disease. Dr. Stone and his coinvestigators recently identified these CD4+ cytotoxic T cells as a novel population of clonally expanded T cells with SLAMF7 as a surface marker. The cells secrete interferon-gamma, interleukin-1, and transforming growth factor-beta, all of which are capable of driving the intense fibrosis characteristic of IgG4-RD. In addition, these CD4+ cytotoxic T cells secrete granzyme B and perforin, previously thought to be released mainly by natural killer T cells.

Joint American College of Rheumatology/European League Against Rheumatism classification criteria for the disease are expected to be finalized this winter at the Third International Symposium on IgG4-Related Diseases.

Treatment with rituxumab

Glucocorticoids remain the first-line therapy in IgG4-related disease, but it’s essential to bear in mind that their long-term efficacy in this immune-mediated fibroinflammatory disease is the exception rather than the rule, Dr. Stone said at the symposium.

Dr. Stone was a coauthor of an international expert consensus statement on the treatment of IgG4-related disease (IgG4-RD), which emphasized that point (Arthritis Rheumatol. 2015 Jul;67[7]:1688-99).

“I typically start with prednisone at 40 mg/day, and there’s a dramatic response in these patients. Then I taper them off after 2-3 months. If 2-3 months doesn’t put them into a long-term sustained remission, it’s time to go to something else,” said Dr. Stone.

So what’s the next move, then, after steroids fail? Dr. Stone was a pioneer in the strikingly successful use of B cell depletion via rituximab (Rituxan) in patients with IgG4-RD. First he and his coinvestigators demonstrated that this off-label use of rituximab led to rapid clinical and histologic improvement (Ann Rheum Dis. 2015 Jun; 74[6]:1171-7), then they showed it also causes levels of circulating plasmablasts, serum IgG4, and biomarkers of fibrosis to plunge, suggesting B cell depletion may halt the destructive process of collagen deposition that characterizes this immune-related disease (Ann Rheum Dis. 2015 Dec;74[12]:2236-43). They have also reported that patients with very elevated baseline serum IgG4 levels are at more than sixfold increased risk of relapse at a median of 244 days from their first rituximab infusion (Rheumatology [Oxford]. 2016 Jun;55[6]:1000-8).

The success with rituximab is just one example of how improved understanding of the pathophysiology of IgG4-RD has opened the door to novel treatments. Dr. Stone is the lead investigator in an ongoing phase II, open-label study in which 15 patients with active IgG4-RD will receive intravenous XmAb5871 every 2 weeks for 6 months to evaluate the effect on the IgG4-RD Responder Index. XmAb5871 is a monoclonal antibody that binds to CD19 and FCgammaRIIb in order to downregulate activated B cells and plasmablasts. It is also being developed for treatment of systemic lupus erythematosus.

Dr. Stone and his coinvestigators are working on a therapeutic approach to IgG4-RD that targets antigen presentation by activated B cells to CD4+ cytotoxic T cells at sites of disease. These CD4+ cytotoxic T cells contain signaling lymphocyte activation molecule F7 (SLAMF7) as a surface marker. Elotuzumab (Empliciti), an immunostimulatory humanized monoclonal antibody targeting SLAMF7, is already on the market for treatment of multiple myeloma, he noted.

Dr. Stone reported receiving IgG4-RD-related research funding from and serving as a consultant to Genentech and Xencor, which is developing XmAb5871.

[email protected]

Flu activity in the United States continued to increase as half of the states reached the highest level of influenza-like illness (ILI) activity in the week ending Feb. 11, according to the Centers for Disease Control and Prevention.

For the week, the 25 states at level 10 on the CDC’s 1-10 scale of ILI activity were joined in the high range by Illinois and Kentucky at level 9 and Iowa at level 8, the CDC reported. The previous week, there were 23 states in the high range.

[email protected]

Flu activity in the United States continued to increase as half of the states reached the highest level of influenza-like illness (ILI) activity in the week ending Feb. 11, according to the Centers for Disease Control and Prevention.

For the week, the 25 states at level 10 on the CDC’s 1-10 scale of ILI activity were joined in the high range by Illinois and Kentucky at level 9 and Iowa at level 8, the CDC reported. The previous week, there were 23 states in the high range.

[email protected]

Flu activity in the United States continued to increase as half of the states reached the highest level of influenza-like illness (ILI) activity in the week ending Feb. 11, according to the Centers for Disease Control and Prevention.

For the week, the 25 states at level 10 on the CDC’s 1-10 scale of ILI activity were joined in the high range by Illinois and Kentucky at level 9 and Iowa at level 8, the CDC reported. The previous week, there were 23 states in the high range.

[email protected]

LAS VEGAS – Individuals with intellectual disability experience behavioral and psychiatric illness at higher rates than the general population, according to Bryan H. King, MD.

“Increasingly, these individuals are showing up in all of our clinical practices,” Dr. King said at an annual psychopharmacology update held by the Nevada Psychiatric Association. “In this area, it’s not so much that the population doesn’t experience psychiatric illness, but the diagnosis can be challenging because the presentation of symptoms may be different. For someone who can’t articulate whether they’re feeling anxious, fearful, or nervous, it’s more challenging to make a diagnosis.”

1. “Antipsychotics, particularly risperidone, appear to be effective in reducing problem behaviors associated with intellectual disability.

2. “For attention-deficit/hyperactivity disorder symptoms, methylphenidate has been shown to be effective, and atomoxetine and alpha-agonists might be beneficial.

3. “Lithium might be effective in reducing aggression. Evidence for the use of antiepileptic drugs, anxiolytics, and naltrexone for management of problem behaviors is insufficient to draw conclusions.

4. “Antidepressants are often poorly tolerated and do not appear to be effective in decreasing repetitive or stereotypic behaviors associated with intellectual disability.

5. “Melatonin appears to improve sleep in people with intellectual disability.”

Dr. King noted that the data for using lithium in people with intellectual disability “are very old. There’s been nothing recent to help us fine-tune the indications.” He said naltrexone is among the best studied in this population, “especially for self-injurious behavior. The two large placebo-controlled trials were negative. In my own clinical experience, I have not seen it helpful.”

Dr. King disclosed that he has received research funding from the National Institutes of Health, Janssen, and Roche. He also is a consultant for Care Management Technologies and Neurotrope.

[email protected]

LAS VEGAS – Individuals with intellectual disability experience behavioral and psychiatric illness at higher rates than the general population, according to Bryan H. King, MD.

“Increasingly, these individuals are showing up in all of our clinical practices,” Dr. King said at an annual psychopharmacology update held by the Nevada Psychiatric Association. “In this area, it’s not so much that the population doesn’t experience psychiatric illness, but the diagnosis can be challenging because the presentation of symptoms may be different. For someone who can’t articulate whether they’re feeling anxious, fearful, or nervous, it’s more challenging to make a diagnosis.”

1. “Antipsychotics, particularly risperidone, appear to be effective in reducing problem behaviors associated with intellectual disability.

2. “For attention-deficit/hyperactivity disorder symptoms, methylphenidate has been shown to be effective, and atomoxetine and alpha-agonists might be beneficial.

3. “Lithium might be effective in reducing aggression. Evidence for the use of antiepileptic drugs, anxiolytics, and naltrexone for management of problem behaviors is insufficient to draw conclusions.

4. “Antidepressants are often poorly tolerated and do not appear to be effective in decreasing repetitive or stereotypic behaviors associated with intellectual disability.

5. “Melatonin appears to improve sleep in people with intellectual disability.”

Dr. King noted that the data for using lithium in people with intellectual disability “are very old. There’s been nothing recent to help us fine-tune the indications.” He said naltrexone is among the best studied in this population, “especially for self-injurious behavior. The two large placebo-controlled trials were negative. In my own clinical experience, I have not seen it helpful.”

Dr. King disclosed that he has received research funding from the National Institutes of Health, Janssen, and Roche. He also is a consultant for Care Management Technologies and Neurotrope.

[email protected]

LAS VEGAS – Individuals with intellectual disability experience behavioral and psychiatric illness at higher rates than the general population, according to Bryan H. King, MD.

“Increasingly, these individuals are showing up in all of our clinical practices,” Dr. King said at an annual psychopharmacology update held by the Nevada Psychiatric Association. “In this area, it’s not so much that the population doesn’t experience psychiatric illness, but the diagnosis can be challenging because the presentation of symptoms may be different. For someone who can’t articulate whether they’re feeling anxious, fearful, or nervous, it’s more challenging to make a diagnosis.”

1. “Antipsychotics, particularly risperidone, appear to be effective in reducing problem behaviors associated with intellectual disability.

2. “For attention-deficit/hyperactivity disorder symptoms, methylphenidate has been shown to be effective, and atomoxetine and alpha-agonists might be beneficial.

3. “Lithium might be effective in reducing aggression. Evidence for the use of antiepileptic drugs, anxiolytics, and naltrexone for management of problem behaviors is insufficient to draw conclusions.

4. “Antidepressants are often poorly tolerated and do not appear to be effective in decreasing repetitive or stereotypic behaviors associated with intellectual disability.

5. “Melatonin appears to improve sleep in people with intellectual disability.”

Dr. King noted that the data for using lithium in people with intellectual disability “are very old. There’s been nothing recent to help us fine-tune the indications.” He said naltrexone is among the best studied in this population, “especially for self-injurious behavior. The two large placebo-controlled trials were negative. In my own clinical experience, I have not seen it helpful.”

Dr. King disclosed that he has received research funding from the National Institutes of Health, Janssen, and Roche. He also is a consultant for Care Management Technologies and Neurotrope.

[email protected]

Lupus erythematosus tumidus (LET) is a relatively rare condition but may simply be underdiagnosed in the literature. It presents as urticarialike papules and plaques in sun-exposed areas, characterized by induration and erythema. Lesions occur on the face, neck, upper extremities, and trunk and heal without scarring.^1,2 Rarely, lesions can show fine scaling and associated pruritus, but most often the lesions are asymptomatic.³

Case Report

A 45-year-old woman presented with 2 asymptomatic self-described bald spots on the top of the head of 2 months’ duration. The patient denied prior treatment of the lesions and noted one patch was resolving. She reported no involvement of the eyebrows, eyelashes, and axillary and pubic hair. A review of systems was negative. The patient denied personal or family history of lupus, thyroid disease, or vitiligo.

Clinical examination revealed a 1.1-cm round patch of nonscarring alopecia on the right vertex scalp and a 0.9-cm round patch of nonscarring alopecia with moderate hair regrowth on the left vertex scalp. There was no erythema, scaling, or induration. The rest of the scalp was normal in appearance and the eyebrows and eyelashes were uninvolved. The patient was diagnosed with alopecia areata and was treated with 10 mg/mL of intralesional triamcinolone once monthly for 4 months.

The patient initially showed improvement with moderate hair regrowth. After 4 months of treatment, she developed 3 new 1- to 1.5-cm erythematous alopecic patches on the vertex scalp and had worsening in the initial patches (Figure 1). Given the resistance to standard therapy and the onset of multiple new areas with evidence of inflammatory involvement, a punch biopsy was performed. Histopathologic examination revealed a fairly unremarkable epidermis and a dense dermal inflammatory infiltrate that was present both in the superficial and deep dermis (Figure 2). The inflammatory cells, which appeared to be predominantly comprised of lymphocytes, had a predilection for the vasculature but also were observed within the interstitial dermis. Additionally, mucin appeared to be slightly increased in the deep dermis. The lymphocytic phenotype was confirmed by immunohistochemical studies for CD20 and CD3. The most likely possibilities for this reaction pattern were LET, Jessner lymphocytic infiltrate of the skin (JLIS), gyrate erythema, and lymphoma; however, the immunohistochemical studies effectively ruled out lymphoma. Additionally, there was pronounced dermal mucin noted in the specimen. The patient was diagnosed with LET of the scalp based on the constellation of findings.

Comment

The classification of LET as a single unique entity or disease process sui generis has been in flux in the last decade. Its similarities to JLIS and other forms of chronic cutaneous lupus erythematosus (CCLE) have brought debate.^4-6 In 1930, Gougerot and Burnier⁷ documented the first case of LET in the literature, describing smooth, infiltrated, erythematous lesions with no desquamation or other superficial changes seen in 5 patients.

In 2000, interest in LET and other forms of CCLE was increasing, and reports in the literature paralleled. That year, Kuhn et al⁴ reported 40 cases of LET, characterizing the clinical and histological features of each case to demonstrate that LET should be separate from other forms of CCLE. Until then, it is likely that many lesions that should have been classified as LET were instead classified as various forms of CCLE. The investigators maintained that LET also should be distinct from JLIS because it is associated with UV exposure.⁴ Kuhn et al⁸ reviewed phototesting in 60 patients with LET in 2001 and confirmed this subset was the most photosensitive type of lupus erythematosus.

In general, the histopathologic and immunohistochemical studies in LET and JLIS can be quite similar. Relatively distinguishing histopathologic findings in JLIS include no evidence of epidermal atrophy, basal vacuolar change, or follicular plugging, as well as negative immunofluorescence studies. Both entities show a predominantly T-cell population with a smaller component of B cells and thus a distinction cannot be made based on relative proportions of T and B cells in lesions.²

In 2003, Alexiades-Armenakas et al⁶ determined immunohistochemical criteria for LET, finding a predominance of T cells and more CD4 lymphocytes than CD8 lymphocytes with a mean ratio of roughly 3 to 1. Their study results maintained LET should be classified as a form of CCLE due to the chronicity of the lesions, the serologic profile with negative anti–double-stranded DNA, anticentromere, anti-Smith, anti-Ro/Sjögren syndrome antigen A, anti-La/Sjögren syndrome antigen B, and anti-nuclear ribonucleoprotein antibodies and the rare association with systemic disease.⁶ This conclusion was further solidified by a review published that same year citing unique histopathological features when compared to subacute cutaneous LE and discoid lupus erythematosus.⁵

This case illustrates the importance of histologic evaluation in determining the correct diagnosis in a patient with alopecia areata recalcitrant to treatment. Including LET in the differential of alopecic patches on the scalp could prove beneficial for patients, as LET responds well to antimalarial drugs and photoprotection.⁹ This patient had a normal antinuclear antibody panel and no signs or symptoms of systemic lupus. It was recommended that she avoid sun exposure and begin treatment with hydroxychloroquine but she declined. At a follow-up visit 6 months later she reported the lesions had improved, but a permanent wig had been sewn over the area, so it could not be examined.

Lupus erythematosus tumidus (LET) is a relatively rare condition but may simply be underdiagnosed in the literature. It presents as urticarialike papules and plaques in sun-exposed areas, characterized by induration and erythema. Lesions occur on the face, neck, upper extremities, and trunk and heal without scarring.^1,2 Rarely, lesions can show fine scaling and associated pruritus, but most often the lesions are asymptomatic.³

Case Report

A 45-year-old woman presented with 2 asymptomatic self-described bald spots on the top of the head of 2 months’ duration. The patient denied prior treatment of the lesions and noted one patch was resolving. She reported no involvement of the eyebrows, eyelashes, and axillary and pubic hair. A review of systems was negative. The patient denied personal or family history of lupus, thyroid disease, or vitiligo.

Clinical examination revealed a 1.1-cm round patch of nonscarring alopecia on the right vertex scalp and a 0.9-cm round patch of nonscarring alopecia with moderate hair regrowth on the left vertex scalp. There was no erythema, scaling, or induration. The rest of the scalp was normal in appearance and the eyebrows and eyelashes were uninvolved. The patient was diagnosed with alopecia areata and was treated with 10 mg/mL of intralesional triamcinolone once monthly for 4 months.

The patient initially showed improvement with moderate hair regrowth. After 4 months of treatment, she developed 3 new 1- to 1.5-cm erythematous alopecic patches on the vertex scalp and had worsening in the initial patches (Figure 1). Given the resistance to standard therapy and the onset of multiple new areas with evidence of inflammatory involvement, a punch biopsy was performed. Histopathologic examination revealed a fairly unremarkable epidermis and a dense dermal inflammatory infiltrate that was present both in the superficial and deep dermis (Figure 2). The inflammatory cells, which appeared to be predominantly comprised of lymphocytes, had a predilection for the vasculature but also were observed within the interstitial dermis. Additionally, mucin appeared to be slightly increased in the deep dermis. The lymphocytic phenotype was confirmed by immunohistochemical studies for CD20 and CD3. The most likely possibilities for this reaction pattern were LET, Jessner lymphocytic infiltrate of the skin (JLIS), gyrate erythema, and lymphoma; however, the immunohistochemical studies effectively ruled out lymphoma. Additionally, there was pronounced dermal mucin noted in the specimen. The patient was diagnosed with LET of the scalp based on the constellation of findings.

Comment

The classification of LET as a single unique entity or disease process sui generis has been in flux in the last decade. Its similarities to JLIS and other forms of chronic cutaneous lupus erythematosus (CCLE) have brought debate.^4-6 In 1930, Gougerot and Burnier⁷ documented the first case of LET in the literature, describing smooth, infiltrated, erythematous lesions with no desquamation or other superficial changes seen in 5 patients.

In 2000, interest in LET and other forms of CCLE was increasing, and reports in the literature paralleled. That year, Kuhn et al⁴ reported 40 cases of LET, characterizing the clinical and histological features of each case to demonstrate that LET should be separate from other forms of CCLE. Until then, it is likely that many lesions that should have been classified as LET were instead classified as various forms of CCLE. The investigators maintained that LET also should be distinct from JLIS because it is associated with UV exposure.⁴ Kuhn et al⁸ reviewed phototesting in 60 patients with LET in 2001 and confirmed this subset was the most photosensitive type of lupus erythematosus.

In general, the histopathologic and immunohistochemical studies in LET and JLIS can be quite similar. Relatively distinguishing histopathologic findings in JLIS include no evidence of epidermal atrophy, basal vacuolar change, or follicular plugging, as well as negative immunofluorescence studies. Both entities show a predominantly T-cell population with a smaller component of B cells and thus a distinction cannot be made based on relative proportions of T and B cells in lesions.²

In 2003, Alexiades-Armenakas et al⁶ determined immunohistochemical criteria for LET, finding a predominance of T cells and more CD4 lymphocytes than CD8 lymphocytes with a mean ratio of roughly 3 to 1. Their study results maintained LET should be classified as a form of CCLE due to the chronicity of the lesions, the serologic profile with negative anti–double-stranded DNA, anticentromere, anti-Smith, anti-Ro/Sjögren syndrome antigen A, anti-La/Sjögren syndrome antigen B, and anti-nuclear ribonucleoprotein antibodies and the rare association with systemic disease.⁶ This conclusion was further solidified by a review published that same year citing unique histopathological features when compared to subacute cutaneous LE and discoid lupus erythematosus.⁵

This case illustrates the importance of histologic evaluation in determining the correct diagnosis in a patient with alopecia areata recalcitrant to treatment. Including LET in the differential of alopecic patches on the scalp could prove beneficial for patients, as LET responds well to antimalarial drugs and photoprotection.⁹ This patient had a normal antinuclear antibody panel and no signs or symptoms of systemic lupus. It was recommended that she avoid sun exposure and begin treatment with hydroxychloroquine but she declined. At a follow-up visit 6 months later she reported the lesions had improved, but a permanent wig had been sewn over the area, so it could not be examined.

Lupus erythematosus tumidus (LET) is a relatively rare condition but may simply be underdiagnosed in the literature. It presents as urticarialike papules and plaques in sun-exposed areas, characterized by induration and erythema. Lesions occur on the face, neck, upper extremities, and trunk and heal without scarring.^1,2 Rarely, lesions can show fine scaling and associated pruritus, but most often the lesions are asymptomatic.³

Case Report

A 45-year-old woman presented with 2 asymptomatic self-described bald spots on the top of the head of 2 months’ duration. The patient denied prior treatment of the lesions and noted one patch was resolving. She reported no involvement of the eyebrows, eyelashes, and axillary and pubic hair. A review of systems was negative. The patient denied personal or family history of lupus, thyroid disease, or vitiligo.

Clinical examination revealed a 1.1-cm round patch of nonscarring alopecia on the right vertex scalp and a 0.9-cm round patch of nonscarring alopecia with moderate hair regrowth on the left vertex scalp. There was no erythema, scaling, or induration. The rest of the scalp was normal in appearance and the eyebrows and eyelashes were uninvolved. The patient was diagnosed with alopecia areata and was treated with 10 mg/mL of intralesional triamcinolone once monthly for 4 months.

The patient initially showed improvement with moderate hair regrowth. After 4 months of treatment, she developed 3 new 1- to 1.5-cm erythematous alopecic patches on the vertex scalp and had worsening in the initial patches (Figure 1). Given the resistance to standard therapy and the onset of multiple new areas with evidence of inflammatory involvement, a punch biopsy was performed. Histopathologic examination revealed a fairly unremarkable epidermis and a dense dermal inflammatory infiltrate that was present both in the superficial and deep dermis (Figure 2). The inflammatory cells, which appeared to be predominantly comprised of lymphocytes, had a predilection for the vasculature but also were observed within the interstitial dermis. Additionally, mucin appeared to be slightly increased in the deep dermis. The lymphocytic phenotype was confirmed by immunohistochemical studies for CD20 and CD3. The most likely possibilities for this reaction pattern were LET, Jessner lymphocytic infiltrate of the skin (JLIS), gyrate erythema, and lymphoma; however, the immunohistochemical studies effectively ruled out lymphoma. Additionally, there was pronounced dermal mucin noted in the specimen. The patient was diagnosed with LET of the scalp based on the constellation of findings.

Comment

The classification of LET as a single unique entity or disease process sui generis has been in flux in the last decade. Its similarities to JLIS and other forms of chronic cutaneous lupus erythematosus (CCLE) have brought debate.^4-6 In 1930, Gougerot and Burnier⁷ documented the first case of LET in the literature, describing smooth, infiltrated, erythematous lesions with no desquamation or other superficial changes seen in 5 patients.

In 2000, interest in LET and other forms of CCLE was increasing, and reports in the literature paralleled. That year, Kuhn et al⁴ reported 40 cases of LET, characterizing the clinical and histological features of each case to demonstrate that LET should be separate from other forms of CCLE. Until then, it is likely that many lesions that should have been classified as LET were instead classified as various forms of CCLE. The investigators maintained that LET also should be distinct from JLIS because it is associated with UV exposure.⁴ Kuhn et al⁸ reviewed phototesting in 60 patients with LET in 2001 and confirmed this subset was the most photosensitive type of lupus erythematosus.

In general, the histopathologic and immunohistochemical studies in LET and JLIS can be quite similar. Relatively distinguishing histopathologic findings in JLIS include no evidence of epidermal atrophy, basal vacuolar change, or follicular plugging, as well as negative immunofluorescence studies. Both entities show a predominantly T-cell population with a smaller component of B cells and thus a distinction cannot be made based on relative proportions of T and B cells in lesions.²

In 2003, Alexiades-Armenakas et al⁶ determined immunohistochemical criteria for LET, finding a predominance of T cells and more CD4 lymphocytes than CD8 lymphocytes with a mean ratio of roughly 3 to 1. Their study results maintained LET should be classified as a form of CCLE due to the chronicity of the lesions, the serologic profile with negative anti–double-stranded DNA, anticentromere, anti-Smith, anti-Ro/Sjögren syndrome antigen A, anti-La/Sjögren syndrome antigen B, and anti-nuclear ribonucleoprotein antibodies and the rare association with systemic disease.⁶ This conclusion was further solidified by a review published that same year citing unique histopathological features when compared to subacute cutaneous LE and discoid lupus erythematosus.⁵

This case illustrates the importance of histologic evaluation in determining the correct diagnosis in a patient with alopecia areata recalcitrant to treatment. Including LET in the differential of alopecic patches on the scalp could prove beneficial for patients, as LET responds well to antimalarial drugs and photoprotection.⁹ This patient had a normal antinuclear antibody panel and no signs or symptoms of systemic lupus. It was recommended that she avoid sun exposure and begin treatment with hydroxychloroquine but she declined. At a follow-up visit 6 months later she reported the lesions had improved, but a permanent wig had been sewn over the area, so it could not be examined.

A collaborative care model significantly mitigated mild depression in adults aged 65 and older, compared with usual care in the short term, based on data from 705 patients. The findings were published online Feb. 21.

“There is limited research about older people with mild depressive disorders who have insufficient levels of depressive symptoms to meet diagnostic criteria (called subclinical, subthreshold, or subsyndromal depression) but also reduced quality of life and function,” wrote Simon Gilbody, PhD, of the University of York (England) and colleagues. However, subthreshold depression increases the risk of a severe depressive illness, the researchers added.

Dundanim/shutterstock.com

A collaborative care model significantly mitigated mild depression in adults aged 65 and older, compared with usual care in the short term, based on data from 705 patients. The findings were published online Feb. 21.

“There is limited research about older people with mild depressive disorders who have insufficient levels of depressive symptoms to meet diagnostic criteria (called subclinical, subthreshold, or subsyndromal depression) but also reduced quality of life and function,” wrote Simon Gilbody, PhD, of the University of York (England) and colleagues. However, subthreshold depression increases the risk of a severe depressive illness, the researchers added.

Dundanim/shutterstock.com

A collaborative care model significantly mitigated mild depression in adults aged 65 and older, compared with usual care in the short term, based on data from 705 patients. The findings were published online Feb. 21.

“There is limited research about older people with mild depressive disorders who have insufficient levels of depressive symptoms to meet diagnostic criteria (called subclinical, subthreshold, or subsyndromal depression) but also reduced quality of life and function,” wrote Simon Gilbody, PhD, of the University of York (England) and colleagues. However, subthreshold depression increases the risk of a severe depressive illness, the researchers added.

Dundanim/shutterstock.com

LAS VEGAS – There remains a great unmet need for more effective and rapidly acting treatments for major depressive disorder, and research is revealing that both new and existing drugs may help, according to one expert.

One argument for additional treatment options is the current rate of suicide in the United States, which ranks as the 10th leading cause of death among persons aged 10 years and older, Gerard Sanacora, MD, PhD, said at an annual psychopharmacology update held by the Nevada Psychiatric Association. Another argument for new antidepressants stems from the results of the STAR*D trial, which found that 37% of patients with major depressive disorder who were treated with citalopram monotherapy had remission with the first treatment.

[email protected]

LAS VEGAS – There remains a great unmet need for more effective and rapidly acting treatments for major depressive disorder, and research is revealing that both new and existing drugs may help, according to one expert.

One argument for additional treatment options is the current rate of suicide in the United States, which ranks as the 10th leading cause of death among persons aged 10 years and older, Gerard Sanacora, MD, PhD, said at an annual psychopharmacology update held by the Nevada Psychiatric Association. Another argument for new antidepressants stems from the results of the STAR*D trial, which found that 37% of patients with major depressive disorder who were treated with citalopram monotherapy had remission with the first treatment.

[email protected]

LAS VEGAS – There remains a great unmet need for more effective and rapidly acting treatments for major depressive disorder, and research is revealing that both new and existing drugs may help, according to one expert.

One argument for additional treatment options is the current rate of suicide in the United States, which ranks as the 10th leading cause of death among persons aged 10 years and older, Gerard Sanacora, MD, PhD, said at an annual psychopharmacology update held by the Nevada Psychiatric Association. Another argument for new antidepressants stems from the results of the STAR*D trial, which found that 37% of patients with major depressive disorder who were treated with citalopram monotherapy had remission with the first treatment.

[email protected]

Light therapy significantly reduced excessive daytime sleepiness, improved sleep quality, decreased overnight awakenings, shortened sleep latency, enhanced daytime alertness and activity level, and improved motor symptoms in patients with Parkinson’s disease, according to a report published online Feb. 20 in JAMA Neurology.

The noninvasive, nonpharmacologic treatment was well tolerated, and patient adherence was excellent in a small, multicenter, randomized controlled trial. Light therapy is widely available as a treatment for several sleep and psychiatric disorders and is “relatively easy to prescribe and incorporate into a clinical practice,” said Aleksandar Videnovic, MD, of the department of neurology at Massachusetts General Hospital and the division of sleep medicine at Harvard Medical School, both in Boston, and his associates.

Silvia Jansen/iStockphoto

Light therapy significantly reduced excessive daytime sleepiness, improved sleep quality, decreased overnight awakenings, shortened sleep latency, enhanced daytime alertness and activity level, and improved motor symptoms in patients with Parkinson’s disease, according to a report published online Feb. 20 in JAMA Neurology.

The noninvasive, nonpharmacologic treatment was well tolerated, and patient adherence was excellent in a small, multicenter, randomized controlled trial. Light therapy is widely available as a treatment for several sleep and psychiatric disorders and is “relatively easy to prescribe and incorporate into a clinical practice,” said Aleksandar Videnovic, MD, of the department of neurology at Massachusetts General Hospital and the division of sleep medicine at Harvard Medical School, both in Boston, and his associates.

Silvia Jansen/iStockphoto

Light therapy significantly reduced excessive daytime sleepiness, improved sleep quality, decreased overnight awakenings, shortened sleep latency, enhanced daytime alertness and activity level, and improved motor symptoms in patients with Parkinson’s disease, according to a report published online Feb. 20 in JAMA Neurology.

The noninvasive, nonpharmacologic treatment was well tolerated, and patient adherence was excellent in a small, multicenter, randomized controlled trial. Light therapy is widely available as a treatment for several sleep and psychiatric disorders and is “relatively easy to prescribe and incorporate into a clinical practice,” said Aleksandar Videnovic, MD, of the department of neurology at Massachusetts General Hospital and the division of sleep medicine at Harvard Medical School, both in Boston, and his associates.

Silvia Jansen/iStockphoto

Take-Home Points

• Anchors placed posterior to the biceps during SLAP repair are at risk for glenoid vault penetration and/or suprascapular nerve (SSN) injury.
• Vault penetration and SSN injury are avoided by using a Port of Wilmington (PW) portal instead of an anterior portal.
• A percutaneous PW portal is safe and passes through the rotator cuff muscle only.

Since being classified by Snyder and colleagues,¹ various arthroscopic techniques have been used to repair superior labrum anterior and posterior (SLAP) tears, particularly type II tears. Despite being commonly performed, repairs of SLAP lesions remain challenging. There is high variability in the rate of good/excellent functional outcomes and athletes’ return to previous level of play after SLAP repairs.^2,3 Furthermore, the rate of complications after SLAP repair is as high as 5%.⁴

One of the most common complications of repair of a type II SLAP tear is nerve injury.⁴ In particular, suprascapular nerve (SSN) injury has occurred after arthroscopic repair of SLAP tears.^5,6 Three cadaveric studies have demonstrated that glenoid vault penetration is common during placement of knotted anchors for SLAP repair and that the SSN is at risk during placement of these anchors.^7-9 However, 2 of the 3 studies used only an anterior portal in their evaluation of anchor placement. Safety of anchor placement posterior to the biceps tendon may be improved with a percutaneous approach using a Port of Wilmington (PW) portal.^10,11 No studies have evaluated the risk of glenoid vault penetration and SSN injury with shorter knotless anchors.

We conducted a study to compare a standard anterosuperolateral (ASL) portal with a percutaneous PW portal for knotless anchors placed posterior to the biceps tendon during repair of SLAP tears. We hypothesized that anchors placed through the PW portal would be less likely to penetrate the glenoid vault and would be farther from the SSN in the event of bone penetration.

Materials and Methods

Six matched pairs of fresh human cadaveric shoulders were used in this study. Each specimen included the scapula, the clavicle, and the humerus. All 6 specimens were male, and their mean age was 41.2 years (range, 23-59 years). Shoulder arthroscopy was performed for placement of SLAP anchors, and open dissection followed.

Anchor Placement

The scapula was clamped and the shoulder placed in the lateral decubitus position with 30° of abduction, 20° of forward flexion, and neutral rotation.¹⁰ A standard posterior glenohumeral viewing portal was established and a 30° arthroscope inserted. Both shoulders of each matched pair were randomly assigned to anchor placement through either an ASL portal or a PW portal. Two anchors were placed in the superior glenoid to simulate repair of a posterior SLAP tear.¹¹ Each was a 2.9-mm short (12.5-mm) knotless anchor (BioComposite PushLock; Arthrex) that included a polyetheretherketone (PEEK) eyelet for threading sutures before anchor placement. A drill guide was inserted according to manufacturer guidelines, and a 2.9-mm drill was used to make a bone socket 18 mm deep. The anchor eyelet was loaded with suture tape (Labral Tape; Arthrex), and the anchor and suture were inserted into the socket. The sutures were left uncut to aid in anchor visualization during open dissection. On a right shoulder, the first anchor was placed just posterior to the biceps tendon, at 11 o’clock, and the second anchor about 1 cm posterior to the first, at 10 o’clock. All anchors were placed by an arthroscopy fellowship–trained shoulder surgeon. Before placement, anchor location was confirmed by another arthroscopy fellowship–trained shoulder surgeon.

The ASL portal was created, with an 18-gauge spinal needle and an outside-in technique, about 1 cm lateral to the anterolateral corner of the acromion.

Cadaveric Dissection

After anchor placement, another shoulder surgeon performed the dissection. Skin, subcutaneous tissue, deltoid, and clavicle were removed. In the percutaneous specimens, PW portal location relative to rotator cuff was recorded before cuff removal. After overlying soft tissues were removed from a specimen, the anchors were examined for glenoid vault penetration. In the setting of vault penetration, digital calipers were used to measure the shortest distance from anchor to SSN.

Results

In the ASL portal group, 8 (66.7%) of 12 anchors (4/6 at 11 o’clock, 4/6 at 10 o’clock) penetrated the medial glenoid vault.

In the PW portal group, 2 (16.7%) of 12 anchors (1/6 at 11 o’clock, 1/6 at 10 o’clock, both from a single specimen) penetrated the medial glenoid vault. Actually, in each case the eyelet and not the anchor penetrated the vault. In the penetration cases, distance to SSN was 20 mm for the 11 o’clock anchor and 8 mm for the 10 o’clock anchor (Table). Of the 6 portals, 3 passed through the supraspinatus muscle, 2 through the infraspinatus musculotendinous junction, and 1 through the infraspinatus muscle.

Discussion

Our study findings support the hypothesis that SLAP repair anchors placed posterior to the biceps tendon are more likely to remain in bone with use of a percutaneous approach relative to an ASL approach. Our findings also support the growing body of evidence that such anchors placed with an anterior approach increase the risk for SSN injury.

Three other cadaveric studies have evaluated anchor placement for SLAP repair. Chan and colleagues⁷ evaluated drill penetration during bone socket preparation for SLAP repair in 21 matched pairs of formalin-embalmed cadavers. A 20-mm drill was used for correspondence to a 14.5-mm anchor, though no anchors were inserted, and sockets were created in an open manner. Through a mimicked ASL portal, 1 socket was made anterior to the biceps tendon, at 1 o’clock; then, through a mimicked PW portal, 2 sockets were made posterior to the tendon, at 11 o’clock and 9 to 10 o’clock. Glenoid vault penetration occurred in 29% of the 42 anterior sockets, but only 1 anchor (2.4%) touched the SSN. Penetration did not occur with the 11 o’clock anchors. The 9 to 10 o’clock anchor was at highest risk for SSN injury (9.5%, 4 cases). The study was limited by lack of anchor placement and open creation of bone sockets in embalmed cadavers.

Koh and colleagues⁸ evaluated arthroscopic placement of anterior SLAP anchors in 6 matched pairs of fresh-frozen cadavers. Through an ASL portal, each 14.5-mm knotted anchor was placed anterior to the biceps tendon, at 1 o’clock. As in the study by Chan and colleagues,⁷ drill depth was 20 mm. Notably, anchors were seated 2 mm beyond manufacturer recommendations, and the cadavers were of Asian origin, likely indicating smaller glenoids compared to specimens from North America or Europe. All 12 anchors penetrated the glenoid vault; mean distance to SSN was 3.1 mm.

Morgan and colleagues⁹ compared anterior and ASL portals created for SLAP repairs in 10 matched-pair cadavers. Anchors were placed at 1 o’clock, 11 o’clock, and 10 o’clock. As in the studies by Chan and colleagues⁷ and Koh and colleagues,⁸ 14.5-mm knotted anchors were used. One anterior anchor (10%) placed through an ASL portal penetrated the cortex by 1 mm, and 2 anterior anchors (20%) placed through anterior portals penetrated the cortex (1 was completely out of the bone). Overall, 65% of 11 o’clock anchors and 100% of 10 o’clock anchors violated the glenoid vault. With the 11 o’clock anchors, mean distance to SSN was 6 mm for ASL portals and 4.2 mm for anterior portals; with the 10 o’clock anchors, mean distance to SSN was 8 mm for ASL portals and 2.1 mm for anterior portals.

Overall, the results of these 3 studies suggest that, with use of ASL portals, placement of SLAP anchors anterior to the biceps tendon is safe. Using the same portals, however, anchors placed posterior to the tendon are at higher risk for glenoid vault penetration. Supporting these findings are our study’s penetration rates: 66.7% for anchors placed through ASL portals and 16.7% for anchors placed through percutaneous PW portals. The different rates are not surprising given that the coracoid process projects anterior to the glenoid and provides additional bone stock for placement of anchors anteriorly vs posteriorly. Therefore, with percutaneous PW portals, the approach angle directs the anchor toward the bone of the coracoid base. Furthermore, the SSN passes nearest the posterior aspect of the glenoid. In a study by Shishido and Kikuchi,¹² the distance from the posterior rim of the glenoid to the SSN was 18 mm, and from the superior rim was 29 mm. Therefore, anchors placed with an anterior approach naturally are directed toward the SSN.

Conclusion

This study was the first to examine glenoid vault penetration and SSN proximity with short anchors for SLAP repair. The risk for glenoid vault penetration during repair of SLAP tears posterior to the biceps tendon was reduced by anchor placement with a percutaneous posterior approach. The percutaneous posterior approach also directs the anchor away from the SSN.

Am J Orthop. 2017;46(1):E60-E64. Copyright Frontline Medical Communications Inc. 2017. All rights reserved.

Take-Home Points

• Anchors placed posterior to the biceps during SLAP repair are at risk for glenoid vault penetration and/or suprascapular nerve (SSN) injury.
• Vault penetration and SSN injury are avoided by using a Port of Wilmington (PW) portal instead of an anterior portal.
• A percutaneous PW portal is safe and passes through the rotator cuff muscle only.

Since being classified by Snyder and colleagues,¹ various arthroscopic techniques have been used to repair superior labrum anterior and posterior (SLAP) tears, particularly type II tears. Despite being commonly performed, repairs of SLAP lesions remain challenging. There is high variability in the rate of good/excellent functional outcomes and athletes’ return to previous level of play after SLAP repairs.^2,3 Furthermore, the rate of complications after SLAP repair is as high as 5%.⁴

One of the most common complications of repair of a type II SLAP tear is nerve injury.⁴ In particular, suprascapular nerve (SSN) injury has occurred after arthroscopic repair of SLAP tears.^5,6 Three cadaveric studies have demonstrated that glenoid vault penetration is common during placement of knotted anchors for SLAP repair and that the SSN is at risk during placement of these anchors.^7-9 However, 2 of the 3 studies used only an anterior portal in their evaluation of anchor placement. Safety of anchor placement posterior to the biceps tendon may be improved with a percutaneous approach using a Port of Wilmington (PW) portal.^10,11 No studies have evaluated the risk of glenoid vault penetration and SSN injury with shorter knotless anchors.

We conducted a study to compare a standard anterosuperolateral (ASL) portal with a percutaneous PW portal for knotless anchors placed posterior to the biceps tendon during repair of SLAP tears. We hypothesized that anchors placed through the PW portal would be less likely to penetrate the glenoid vault and would be farther from the SSN in the event of bone penetration.

Materials and Methods

Six matched pairs of fresh human cadaveric shoulders were used in this study. Each specimen included the scapula, the clavicle, and the humerus. All 6 specimens were male, and their mean age was 41.2 years (range, 23-59 years). Shoulder arthroscopy was performed for placement of SLAP anchors, and open dissection followed.

Anchor Placement

The scapula was clamped and the shoulder placed in the lateral decubitus position with 30° of abduction, 20° of forward flexion, and neutral rotation.¹⁰ A standard posterior glenohumeral viewing portal was established and a 30° arthroscope inserted. Both shoulders of each matched pair were randomly assigned to anchor placement through either an ASL portal or a PW portal. Two anchors were placed in the superior glenoid to simulate repair of a posterior SLAP tear.¹¹ Each was a 2.9-mm short (12.5-mm) knotless anchor (BioComposite PushLock; Arthrex) that included a polyetheretherketone (PEEK) eyelet for threading sutures before anchor placement. A drill guide was inserted according to manufacturer guidelines, and a 2.9-mm drill was used to make a bone socket 18 mm deep. The anchor eyelet was loaded with suture tape (Labral Tape; Arthrex), and the anchor and suture were inserted into the socket. The sutures were left uncut to aid in anchor visualization during open dissection. On a right shoulder, the first anchor was placed just posterior to the biceps tendon, at 11 o’clock, and the second anchor about 1 cm posterior to the first, at 10 o’clock. All anchors were placed by an arthroscopy fellowship–trained shoulder surgeon. Before placement, anchor location was confirmed by another arthroscopy fellowship–trained shoulder surgeon.

The ASL portal was created, with an 18-gauge spinal needle and an outside-in technique, about 1 cm lateral to the anterolateral corner of the acromion.

Cadaveric Dissection

After anchor placement, another shoulder surgeon performed the dissection. Skin, subcutaneous tissue, deltoid, and clavicle were removed. In the percutaneous specimens, PW portal location relative to rotator cuff was recorded before cuff removal. After overlying soft tissues were removed from a specimen, the anchors were examined for glenoid vault penetration. In the setting of vault penetration, digital calipers were used to measure the shortest distance from anchor to SSN.

Results

In the ASL portal group, 8 (66.7%) of 12 anchors (4/6 at 11 o’clock, 4/6 at 10 o’clock) penetrated the medial glenoid vault.

In the PW portal group, 2 (16.7%) of 12 anchors (1/6 at 11 o’clock, 1/6 at 10 o’clock, both from a single specimen) penetrated the medial glenoid vault. Actually, in each case the eyelet and not the anchor penetrated the vault. In the penetration cases, distance to SSN was 20 mm for the 11 o’clock anchor and 8 mm for the 10 o’clock anchor (Table). Of the 6 portals, 3 passed through the supraspinatus muscle, 2 through the infraspinatus musculotendinous junction, and 1 through the infraspinatus muscle.

Discussion

Our study findings support the hypothesis that SLAP repair anchors placed posterior to the biceps tendon are more likely to remain in bone with use of a percutaneous approach relative to an ASL approach. Our findings also support the growing body of evidence that such anchors placed with an anterior approach increase the risk for SSN injury.

Three other cadaveric studies have evaluated anchor placement for SLAP repair. Chan and colleagues⁷ evaluated drill penetration during bone socket preparation for SLAP repair in 21 matched pairs of formalin-embalmed cadavers. A 20-mm drill was used for correspondence to a 14.5-mm anchor, though no anchors were inserted, and sockets were created in an open manner. Through a mimicked ASL portal, 1 socket was made anterior to the biceps tendon, at 1 o’clock; then, through a mimicked PW portal, 2 sockets were made posterior to the tendon, at 11 o’clock and 9 to 10 o’clock. Glenoid vault penetration occurred in 29% of the 42 anterior sockets, but only 1 anchor (2.4%) touched the SSN. Penetration did not occur with the 11 o’clock anchors. The 9 to 10 o’clock anchor was at highest risk for SSN injury (9.5%, 4 cases). The study was limited by lack of anchor placement and open creation of bone sockets in embalmed cadavers.

Koh and colleagues⁸ evaluated arthroscopic placement of anterior SLAP anchors in 6 matched pairs of fresh-frozen cadavers. Through an ASL portal, each 14.5-mm knotted anchor was placed anterior to the biceps tendon, at 1 o’clock. As in the study by Chan and colleagues,⁷ drill depth was 20 mm. Notably, anchors were seated 2 mm beyond manufacturer recommendations, and the cadavers were of Asian origin, likely indicating smaller glenoids compared to specimens from North America or Europe. All 12 anchors penetrated the glenoid vault; mean distance to SSN was 3.1 mm.

Morgan and colleagues⁹ compared anterior and ASL portals created for SLAP repairs in 10 matched-pair cadavers. Anchors were placed at 1 o’clock, 11 o’clock, and 10 o’clock. As in the studies by Chan and colleagues⁷ and Koh and colleagues,⁸ 14.5-mm knotted anchors were used. One anterior anchor (10%) placed through an ASL portal penetrated the cortex by 1 mm, and 2 anterior anchors (20%) placed through anterior portals penetrated the cortex (1 was completely out of the bone). Overall, 65% of 11 o’clock anchors and 100% of 10 o’clock anchors violated the glenoid vault. With the 11 o’clock anchors, mean distance to SSN was 6 mm for ASL portals and 4.2 mm for anterior portals; with the 10 o’clock anchors, mean distance to SSN was 8 mm for ASL portals and 2.1 mm for anterior portals.

Overall, the results of these 3 studies suggest that, with use of ASL portals, placement of SLAP anchors anterior to the biceps tendon is safe. Using the same portals, however, anchors placed posterior to the tendon are at higher risk for glenoid vault penetration. Supporting these findings are our study’s penetration rates: 66.7% for anchors placed through ASL portals and 16.7% for anchors placed through percutaneous PW portals. The different rates are not surprising given that the coracoid process projects anterior to the glenoid and provides additional bone stock for placement of anchors anteriorly vs posteriorly. Therefore, with percutaneous PW portals, the approach angle directs the anchor toward the bone of the coracoid base. Furthermore, the SSN passes nearest the posterior aspect of the glenoid. In a study by Shishido and Kikuchi,¹² the distance from the posterior rim of the glenoid to the SSN was 18 mm, and from the superior rim was 29 mm. Therefore, anchors placed with an anterior approach naturally are directed toward the SSN.

Conclusion

This study was the first to examine glenoid vault penetration and SSN proximity with short anchors for SLAP repair. The risk for glenoid vault penetration during repair of SLAP tears posterior to the biceps tendon was reduced by anchor placement with a percutaneous posterior approach. The percutaneous posterior approach also directs the anchor away from the SSN.

Am J Orthop. 2017;46(1):E60-E64. Copyright Frontline Medical Communications Inc. 2017. All rights reserved.

Take-Home Points

• Anchors placed posterior to the biceps during SLAP repair are at risk for glenoid vault penetration and/or suprascapular nerve (SSN) injury.
• Vault penetration and SSN injury are avoided by using a Port of Wilmington (PW) portal instead of an anterior portal.
• A percutaneous PW portal is safe and passes through the rotator cuff muscle only.

Since being classified by Snyder and colleagues,¹ various arthroscopic techniques have been used to repair superior labrum anterior and posterior (SLAP) tears, particularly type II tears. Despite being commonly performed, repairs of SLAP lesions remain challenging. There is high variability in the rate of good/excellent functional outcomes and athletes’ return to previous level of play after SLAP repairs.^2,3 Furthermore, the rate of complications after SLAP repair is as high as 5%.⁴

One of the most common complications of repair of a type II SLAP tear is nerve injury.⁴ In particular, suprascapular nerve (SSN) injury has occurred after arthroscopic repair of SLAP tears.^5,6 Three cadaveric studies have demonstrated that glenoid vault penetration is common during placement of knotted anchors for SLAP repair and that the SSN is at risk during placement of these anchors.^7-9 However, 2 of the 3 studies used only an anterior portal in their evaluation of anchor placement. Safety of anchor placement posterior to the biceps tendon may be improved with a percutaneous approach using a Port of Wilmington (PW) portal.^10,11 No studies have evaluated the risk of glenoid vault penetration and SSN injury with shorter knotless anchors.

We conducted a study to compare a standard anterosuperolateral (ASL) portal with a percutaneous PW portal for knotless anchors placed posterior to the biceps tendon during repair of SLAP tears. We hypothesized that anchors placed through the PW portal would be less likely to penetrate the glenoid vault and would be farther from the SSN in the event of bone penetration.

Materials and Methods

Six matched pairs of fresh human cadaveric shoulders were used in this study. Each specimen included the scapula, the clavicle, and the humerus. All 6 specimens were male, and their mean age was 41.2 years (range, 23-59 years). Shoulder arthroscopy was performed for placement of SLAP anchors, and open dissection followed.

Anchor Placement

The scapula was clamped and the shoulder placed in the lateral decubitus position with 30° of abduction, 20° of forward flexion, and neutral rotation.¹⁰ A standard posterior glenohumeral viewing portal was established and a 30° arthroscope inserted. Both shoulders of each matched pair were randomly assigned to anchor placement through either an ASL portal or a PW portal. Two anchors were placed in the superior glenoid to simulate repair of a posterior SLAP tear.¹¹ Each was a 2.9-mm short (12.5-mm) knotless anchor (BioComposite PushLock; Arthrex) that included a polyetheretherketone (PEEK) eyelet for threading sutures before anchor placement. A drill guide was inserted according to manufacturer guidelines, and a 2.9-mm drill was used to make a bone socket 18 mm deep. The anchor eyelet was loaded with suture tape (Labral Tape; Arthrex), and the anchor and suture were inserted into the socket. The sutures were left uncut to aid in anchor visualization during open dissection. On a right shoulder, the first anchor was placed just posterior to the biceps tendon, at 11 o’clock, and the second anchor about 1 cm posterior to the first, at 10 o’clock. All anchors were placed by an arthroscopy fellowship–trained shoulder surgeon. Before placement, anchor location was confirmed by another arthroscopy fellowship–trained shoulder surgeon.

The ASL portal was created, with an 18-gauge spinal needle and an outside-in technique, about 1 cm lateral to the anterolateral corner of the acromion.

Cadaveric Dissection

After anchor placement, another shoulder surgeon performed the dissection. Skin, subcutaneous tissue, deltoid, and clavicle were removed. In the percutaneous specimens, PW portal location relative to rotator cuff was recorded before cuff removal. After overlying soft tissues were removed from a specimen, the anchors were examined for glenoid vault penetration. In the setting of vault penetration, digital calipers were used to measure the shortest distance from anchor to SSN.

Results

In the ASL portal group, 8 (66.7%) of 12 anchors (4/6 at 11 o’clock, 4/6 at 10 o’clock) penetrated the medial glenoid vault.

In the PW portal group, 2 (16.7%) of 12 anchors (1/6 at 11 o’clock, 1/6 at 10 o’clock, both from a single specimen) penetrated the medial glenoid vault. Actually, in each case the eyelet and not the anchor penetrated the vault. In the penetration cases, distance to SSN was 20 mm for the 11 o’clock anchor and 8 mm for the 10 o’clock anchor (Table). Of the 6 portals, 3 passed through the supraspinatus muscle, 2 through the infraspinatus musculotendinous junction, and 1 through the infraspinatus muscle.

Discussion

Our study findings support the hypothesis that SLAP repair anchors placed posterior to the biceps tendon are more likely to remain in bone with use of a percutaneous approach relative to an ASL approach. Our findings also support the growing body of evidence that such anchors placed with an anterior approach increase the risk for SSN injury.

Three other cadaveric studies have evaluated anchor placement for SLAP repair. Chan and colleagues⁷ evaluated drill penetration during bone socket preparation for SLAP repair in 21 matched pairs of formalin-embalmed cadavers. A 20-mm drill was used for correspondence to a 14.5-mm anchor, though no anchors were inserted, and sockets were created in an open manner. Through a mimicked ASL portal, 1 socket was made anterior to the biceps tendon, at 1 o’clock; then, through a mimicked PW portal, 2 sockets were made posterior to the tendon, at 11 o’clock and 9 to 10 o’clock. Glenoid vault penetration occurred in 29% of the 42 anterior sockets, but only 1 anchor (2.4%) touched the SSN. Penetration did not occur with the 11 o’clock anchors. The 9 to 10 o’clock anchor was at highest risk for SSN injury (9.5%, 4 cases). The study was limited by lack of anchor placement and open creation of bone sockets in embalmed cadavers.

Koh and colleagues⁸ evaluated arthroscopic placement of anterior SLAP anchors in 6 matched pairs of fresh-frozen cadavers. Through an ASL portal, each 14.5-mm knotted anchor was placed anterior to the biceps tendon, at 1 o’clock. As in the study by Chan and colleagues,⁷ drill depth was 20 mm. Notably, anchors were seated 2 mm beyond manufacturer recommendations, and the cadavers were of Asian origin, likely indicating smaller glenoids compared to specimens from North America or Europe. All 12 anchors penetrated the glenoid vault; mean distance to SSN was 3.1 mm.

Morgan and colleagues⁹ compared anterior and ASL portals created for SLAP repairs in 10 matched-pair cadavers. Anchors were placed at 1 o’clock, 11 o’clock, and 10 o’clock. As in the studies by Chan and colleagues⁷ and Koh and colleagues,⁸ 14.5-mm knotted anchors were used. One anterior anchor (10%) placed through an ASL portal penetrated the cortex by 1 mm, and 2 anterior anchors (20%) placed through anterior portals penetrated the cortex (1 was completely out of the bone). Overall, 65% of 11 o’clock anchors and 100% of 10 o’clock anchors violated the glenoid vault. With the 11 o’clock anchors, mean distance to SSN was 6 mm for ASL portals and 4.2 mm for anterior portals; with the 10 o’clock anchors, mean distance to SSN was 8 mm for ASL portals and 2.1 mm for anterior portals.

Overall, the results of these 3 studies suggest that, with use of ASL portals, placement of SLAP anchors anterior to the biceps tendon is safe. Using the same portals, however, anchors placed posterior to the tendon are at higher risk for glenoid vault penetration. Supporting these findings are our study’s penetration rates: 66.7% for anchors placed through ASL portals and 16.7% for anchors placed through percutaneous PW portals. The different rates are not surprising given that the coracoid process projects anterior to the glenoid and provides additional bone stock for placement of anchors anteriorly vs posteriorly. Therefore, with percutaneous PW portals, the approach angle directs the anchor toward the bone of the coracoid base. Furthermore, the SSN passes nearest the posterior aspect of the glenoid. In a study by Shishido and Kikuchi,¹² the distance from the posterior rim of the glenoid to the SSN was 18 mm, and from the superior rim was 29 mm. Therefore, anchors placed with an anterior approach naturally are directed toward the SSN.

Conclusion

This study was the first to examine glenoid vault penetration and SSN proximity with short anchors for SLAP repair. The risk for glenoid vault penetration during repair of SLAP tears posterior to the biceps tendon was reduced by anchor placement with a percutaneous posterior approach. The percutaneous posterior approach also directs the anchor away from the SSN.

Am J Orthop. 2017;46(1):E60-E64. Copyright Frontline Medical Communications Inc. 2017. All rights reserved.

Some areas of psychiatry would benefit from more controversy. One of them is the prescription of antidepressants to young people dealing with romantic disappointments.

I have seen many young men and women given an antidepressant for the very painful, but ordinary, romantic break-ups characteristic of this phase of life, who then become habituated to the drug. They take the medication indefinitely, their brains accommodate neurophysiologically to the presence of the chemical, and they become unable to discontinue it without intolerable withdrawal symptoms that look like an underlying illness. A parallel phenomenon occurs not infrequently with the use of amphetamines (and other stimulants) for attention-deficit hyperactivity disorder that is at times mistakenly diagnosed in this age group.

Antidepressants for early romantic disappointments

Mr. A, now in his 30s, became sullen and withdrawn at age 16 after a girl refused his romantic approaches. His well-intentioned parents took him to a psychiatrist, who, after a brief evaluation, prescribed fluoxetine. Mr. A is now well adjusted and happily married but unable to get off fluoxetine. Even when it is carefully tapered, 2 or 3 months after it is discontinued, he becomes anxious and depressed. This is an iatrogenic problem. It is not related to goings-on in his mind or his life; rather it is the result of his brain’s accommodation to a medication, producing a serious withdrawal syndrome.

His original psychiatrist made only a descriptive diagnosis. He did not inquire about what was going on in Mr. A’s mind and thus could not make a dynamic diagnosis (that is, a diagnosis of a patient’s central emotional conflicts, ability to function in relation to other people, strengths, and weaknesses). Mr. A, like many adolescents, had a lot of anxiety and guilt about sexual and romantic involvement, and potential success. He defended against his anxiety and guilt by assuring himself life would never work out for him. When the girl he admired rebuffed him, he immediately concluded this would perpetually be his fate, so the girl’s refusal was particularly painful. Mr. A feels that had this dynamic been discussed with him at the time, he may well not have needed medication at all.

Ms. B, like Mr. A, was prescribed antidepressants for depressive reactions to early romantic disappointments. Likewise, she self-punitively convinced herself, despite easily attracting men’s attentions, that these disappointments meant a lifetime alone. Ms. B has a family history of depression (although neither of her brothers struggles with it), and she felt that she needed the medications to help negotiate difficult periods. But should she have been on them for extended periods of time? Therapeutic attention to her emotional conflicts helped her to form lasting relationships, marry, and have children. Unable to get off the medications, she had to deal with the risks of their use during pregnancy, which she then subjected to the same sort of guilty self-accusations as she previously had used to limit her romantic prospects.

Ms. C came to me on three medications – one for each of her significant romantic break-ups. She, too, was depressively self-diminishing, beginning therapy by letting me know all the things she could think of that might make me think less of her. Understanding some of the reasons for her self-deprecation helped her toward better romantic relationships but did not give her the courage to get off her medications. Pregnancy, however, led her to promptly and successfully discontinue an antidepressant and a mood stabilizer (she has never had any symptoms suggestive of manic depression). She remained on a low dose of a selective serotonin reuptake inhibitor, had an uneventful pregnancy, and then fell in love with a charming baby.

Principles for consideration

• Psychiatrists (and other mental health professionals and primary care physicians treating mental illness) should always make a dynamic, and not merely a descriptive, diagnosis. Even with a more clearly biologically driven problem, such as bipolar disorder, the patient’s personality and conflicts matter.

• Psychiatrists should be very judicious about prescribing medications in adolescence and young adulthood, especially for difficulties adapting to the typical events of those phases of life. Expert psychotherapy should be the first choice in these instances.

• Medication, when necessary, should be prescribed for as limited a time as possible. It is important for young people to advance their own development, not feel needlessly beholden to medications, not get iatrogenically dependent on them, and not feel that they have “diseases” they don’t have.

Amphetamines for misdiagnosed ADHD

When Ms. D’s family moved to a new house, she, her brother, and her sister, each attended a new school. Unlike her siblings, Ms. D, who was in high school, had a difficult adjustment. Her grades fell. She was taken to a psychiatrist who diagnosed ADHD and prescribed amphetamines. The psychiatrist paid little attention to her prior lack of difficulty in school or her struggles making new friends. Nor did the psychiatrist learn that Ms. D had to ward off the seductive advances of an older teacher (although Ms. D would likely not have been immediately forthcoming about this at the time).

When Ms. D came to me as a college student, for troubles with anger, anxiety, and some depression, she was religiously taking 70 mg of amphetamines daily. After I learned a bit about her and raised the question of whether she actually had ADHD, and whether it might make sense to consider tapering the amphetamines, she was appalled and looked like a toddler who was afraid I was about to steal her candy. Helping her to get off the unneeded medication was a multiyear process.

First, she had to recognize that it was prescribed to treat a problem she probably didn’t have, and second, that it was failing to help her with the problems she did have. As we attended to some of her actual emotional conflicts, she became willing to experiment with lower doses. She was able to see that her work was little changed as the dose was lowered, and that her difficulties with school had more to do with feelings toward classmates and teachers than with the presence or absence of amphetamines. After a protracted struggle, finally off the medication, she felt in charge of her life and no longer believed there was something inherently wrong with her mind or her brain.

Mr. E was the only son in a high-powered academic family. His older sisters were all intellectual standouts. Early in high school, he received his first B as a grade in a course. He was taken to a pediatrician, diagnosed with ADHD, and put on stimulants. Like Ms. D, he came to believe that he needed them. In college, he began to develop some magical aspects to his thinking, a potential side effect of the stimulants. It was very difficult to help him see either that he had a problem with his thinking or that it might be attributable to the medication.

Principles to consider

• If the ADHD wasn’t there in elementary school or before, it is unlikely that an adolescent or young adult has new-onset ADHD. A new or newly amplified conflict is occurring in the person’s mind and life A dynamic diagnosis, as always, is essential.

• When medication is prescribed for actual ADHD, as with anything else, the question of how long it will be taken must be asked. For life? Until other means of adaptation are accomplished? Until adequate outcome studies of long-term use of the medication are performed?

Helping patients to get off unneeded, or no longer needed, medications can be a difficult task. Their emotional attachments to the medications can be intense and varied. For some, the prescription is a sign of being loved and cared for. For others, it represents a certification of a deficit, appeases guilt about success, and/or attests to the need for special consideration. Insofar as the medication has been helpful, it may have come to be regarded as a dearly loved friend, or even a part of the self.

When medication has been helpful, there is also, of course, concern about the potential return of the difficulties for which it was prescribed. Few patients are told at the time of first prescription that there is potential risk of habituation and return of, or potential exaggeration of, symptoms with discontinuation. This type of discussion is more difficult to have in situations in which a prescription is urgently needed and the patient is reluctant, but is still not often done in those instances in which a prescription is more optional than essential. The picture is seldom simple.

These few comments only scratch the surface of the difficulties doctors and patients face in helping patients to discontinue their medications. Residency programs pay a lot of attention to helping trainees learn to prescribe medications; rarely do they sufficiently educate residents how to help patients discontinue them. The fact that so many residencies currently pay limited attention to interventions apart from medication contributes further to the difficulty.

Medications have saved the life of many a psychiatric patient. Some patients need medication for life. But some end up on medication for life, even in some instances when the medication may not have been needed in the first place. Although it is often a difficult task, we need to do a better job of distinguishing which patients are which.
 

Dr. Blum is a psychiatrist and psychoanalyst in private practice in Philadelphia. He teaches in the departments of anthropology and psychiatry at the University of Pennsylvania and at the Psychoanalytic Center of Philadelphia.

Some areas of psychiatry would benefit from more controversy. One of them is the prescription of antidepressants to young people dealing with romantic disappointments.

I have seen many young men and women given an antidepressant for the very painful, but ordinary, romantic break-ups characteristic of this phase of life, who then become habituated to the drug. They take the medication indefinitely, their brains accommodate neurophysiologically to the presence of the chemical, and they become unable to discontinue it without intolerable withdrawal symptoms that look like an underlying illness. A parallel phenomenon occurs not infrequently with the use of amphetamines (and other stimulants) for attention-deficit hyperactivity disorder that is at times mistakenly diagnosed in this age group.

Antidepressants for early romantic disappointments

Mr. A, now in his 30s, became sullen and withdrawn at age 16 after a girl refused his romantic approaches. His well-intentioned parents took him to a psychiatrist, who, after a brief evaluation, prescribed fluoxetine. Mr. A is now well adjusted and happily married but unable to get off fluoxetine. Even when it is carefully tapered, 2 or 3 months after it is discontinued, he becomes anxious and depressed. This is an iatrogenic problem. It is not related to goings-on in his mind or his life; rather it is the result of his brain’s accommodation to a medication, producing a serious withdrawal syndrome.

His original psychiatrist made only a descriptive diagnosis. He did not inquire about what was going on in Mr. A’s mind and thus could not make a dynamic diagnosis (that is, a diagnosis of a patient’s central emotional conflicts, ability to function in relation to other people, strengths, and weaknesses). Mr. A, like many adolescents, had a lot of anxiety and guilt about sexual and romantic involvement, and potential success. He defended against his anxiety and guilt by assuring himself life would never work out for him. When the girl he admired rebuffed him, he immediately concluded this would perpetually be his fate, so the girl’s refusal was particularly painful. Mr. A feels that had this dynamic been discussed with him at the time, he may well not have needed medication at all.

Ms. B, like Mr. A, was prescribed antidepressants for depressive reactions to early romantic disappointments. Likewise, she self-punitively convinced herself, despite easily attracting men’s attentions, that these disappointments meant a lifetime alone. Ms. B has a family history of depression (although neither of her brothers struggles with it), and she felt that she needed the medications to help negotiate difficult periods. But should she have been on them for extended periods of time? Therapeutic attention to her emotional conflicts helped her to form lasting relationships, marry, and have children. Unable to get off the medications, she had to deal with the risks of their use during pregnancy, which she then subjected to the same sort of guilty self-accusations as she previously had used to limit her romantic prospects.

Ms. C came to me on three medications – one for each of her significant romantic break-ups. She, too, was depressively self-diminishing, beginning therapy by letting me know all the things she could think of that might make me think less of her. Understanding some of the reasons for her self-deprecation helped her toward better romantic relationships but did not give her the courage to get off her medications. Pregnancy, however, led her to promptly and successfully discontinue an antidepressant and a mood stabilizer (she has never had any symptoms suggestive of manic depression). She remained on a low dose of a selective serotonin reuptake inhibitor, had an uneventful pregnancy, and then fell in love with a charming baby.

Principles for consideration

• Psychiatrists (and other mental health professionals and primary care physicians treating mental illness) should always make a dynamic, and not merely a descriptive, diagnosis. Even with a more clearly biologically driven problem, such as bipolar disorder, the patient’s personality and conflicts matter.

• Psychiatrists should be very judicious about prescribing medications in adolescence and young adulthood, especially for difficulties adapting to the typical events of those phases of life. Expert psychotherapy should be the first choice in these instances.

• Medication, when necessary, should be prescribed for as limited a time as possible. It is important for young people to advance their own development, not feel needlessly beholden to medications, not get iatrogenically dependent on them, and not feel that they have “diseases” they don’t have.

Amphetamines for misdiagnosed ADHD

When Ms. D’s family moved to a new house, she, her brother, and her sister, each attended a new school. Unlike her siblings, Ms. D, who was in high school, had a difficult adjustment. Her grades fell. She was taken to a psychiatrist who diagnosed ADHD and prescribed amphetamines. The psychiatrist paid little attention to her prior lack of difficulty in school or her struggles making new friends. Nor did the psychiatrist learn that Ms. D had to ward off the seductive advances of an older teacher (although Ms. D would likely not have been immediately forthcoming about this at the time).

When Ms. D came to me as a college student, for troubles with anger, anxiety, and some depression, she was religiously taking 70 mg of amphetamines daily. After I learned a bit about her and raised the question of whether she actually had ADHD, and whether it might make sense to consider tapering the amphetamines, she was appalled and looked like a toddler who was afraid I was about to steal her candy. Helping her to get off the unneeded medication was a multiyear process.

First, she had to recognize that it was prescribed to treat a problem she probably didn’t have, and second, that it was failing to help her with the problems she did have. As we attended to some of her actual emotional conflicts, she became willing to experiment with lower doses. She was able to see that her work was little changed as the dose was lowered, and that her difficulties with school had more to do with feelings toward classmates and teachers than with the presence or absence of amphetamines. After a protracted struggle, finally off the medication, she felt in charge of her life and no longer believed there was something inherently wrong with her mind or her brain.

Mr. E was the only son in a high-powered academic family. His older sisters were all intellectual standouts. Early in high school, he received his first B as a grade in a course. He was taken to a pediatrician, diagnosed with ADHD, and put on stimulants. Like Ms. D, he came to believe that he needed them. In college, he began to develop some magical aspects to his thinking, a potential side effect of the stimulants. It was very difficult to help him see either that he had a problem with his thinking or that it might be attributable to the medication.

Principles to consider

• If the ADHD wasn’t there in elementary school or before, it is unlikely that an adolescent or young adult has new-onset ADHD. A new or newly amplified conflict is occurring in the person’s mind and life A dynamic diagnosis, as always, is essential.

• When medication is prescribed for actual ADHD, as with anything else, the question of how long it will be taken must be asked. For life? Until other means of adaptation are accomplished? Until adequate outcome studies of long-term use of the medication are performed?

Helping patients to get off unneeded, or no longer needed, medications can be a difficult task. Their emotional attachments to the medications can be intense and varied. For some, the prescription is a sign of being loved and cared for. For others, it represents a certification of a deficit, appeases guilt about success, and/or attests to the need for special consideration. Insofar as the medication has been helpful, it may have come to be regarded as a dearly loved friend, or even a part of the self.

When medication has been helpful, there is also, of course, concern about the potential return of the difficulties for which it was prescribed. Few patients are told at the time of first prescription that there is potential risk of habituation and return of, or potential exaggeration of, symptoms with discontinuation. This type of discussion is more difficult to have in situations in which a prescription is urgently needed and the patient is reluctant, but is still not often done in those instances in which a prescription is more optional than essential. The picture is seldom simple.

These few comments only scratch the surface of the difficulties doctors and patients face in helping patients to discontinue their medications. Residency programs pay a lot of attention to helping trainees learn to prescribe medications; rarely do they sufficiently educate residents how to help patients discontinue them. The fact that so many residencies currently pay limited attention to interventions apart from medication contributes further to the difficulty.

Medications have saved the life of many a psychiatric patient. Some patients need medication for life. But some end up on medication for life, even in some instances when the medication may not have been needed in the first place. Although it is often a difficult task, we need to do a better job of distinguishing which patients are which.
 

Dr. Blum is a psychiatrist and psychoanalyst in private practice in Philadelphia. He teaches in the departments of anthropology and psychiatry at the University of Pennsylvania and at the Psychoanalytic Center of Philadelphia.

Some areas of psychiatry would benefit from more controversy. One of them is the prescription of antidepressants to young people dealing with romantic disappointments.

I have seen many young men and women given an antidepressant for the very painful, but ordinary, romantic break-ups characteristic of this phase of life, who then become habituated to the drug. They take the medication indefinitely, their brains accommodate neurophysiologically to the presence of the chemical, and they become unable to discontinue it without intolerable withdrawal symptoms that look like an underlying illness. A parallel phenomenon occurs not infrequently with the use of amphetamines (and other stimulants) for attention-deficit hyperactivity disorder that is at times mistakenly diagnosed in this age group.

Antidepressants for early romantic disappointments

Mr. A, now in his 30s, became sullen and withdrawn at age 16 after a girl refused his romantic approaches. His well-intentioned parents took him to a psychiatrist, who, after a brief evaluation, prescribed fluoxetine. Mr. A is now well adjusted and happily married but unable to get off fluoxetine. Even when it is carefully tapered, 2 or 3 months after it is discontinued, he becomes anxious and depressed. This is an iatrogenic problem. It is not related to goings-on in his mind or his life; rather it is the result of his brain’s accommodation to a medication, producing a serious withdrawal syndrome.

His original psychiatrist made only a descriptive diagnosis. He did not inquire about what was going on in Mr. A’s mind and thus could not make a dynamic diagnosis (that is, a diagnosis of a patient’s central emotional conflicts, ability to function in relation to other people, strengths, and weaknesses). Mr. A, like many adolescents, had a lot of anxiety and guilt about sexual and romantic involvement, and potential success. He defended against his anxiety and guilt by assuring himself life would never work out for him. When the girl he admired rebuffed him, he immediately concluded this would perpetually be his fate, so the girl’s refusal was particularly painful. Mr. A feels that had this dynamic been discussed with him at the time, he may well not have needed medication at all.

Ms. B, like Mr. A, was prescribed antidepressants for depressive reactions to early romantic disappointments. Likewise, she self-punitively convinced herself, despite easily attracting men’s attentions, that these disappointments meant a lifetime alone. Ms. B has a family history of depression (although neither of her brothers struggles with it), and she felt that she needed the medications to help negotiate difficult periods. But should she have been on them for extended periods of time? Therapeutic attention to her emotional conflicts helped her to form lasting relationships, marry, and have children. Unable to get off the medications, she had to deal with the risks of their use during pregnancy, which she then subjected to the same sort of guilty self-accusations as she previously had used to limit her romantic prospects.

Ms. C came to me on three medications – one for each of her significant romantic break-ups. She, too, was depressively self-diminishing, beginning therapy by letting me know all the things she could think of that might make me think less of her. Understanding some of the reasons for her self-deprecation helped her toward better romantic relationships but did not give her the courage to get off her medications. Pregnancy, however, led her to promptly and successfully discontinue an antidepressant and a mood stabilizer (she has never had any symptoms suggestive of manic depression). She remained on a low dose of a selective serotonin reuptake inhibitor, had an uneventful pregnancy, and then fell in love with a charming baby.

Principles for consideration

• Psychiatrists (and other mental health professionals and primary care physicians treating mental illness) should always make a dynamic, and not merely a descriptive, diagnosis. Even with a more clearly biologically driven problem, such as bipolar disorder, the patient’s personality and conflicts matter.

• Psychiatrists should be very judicious about prescribing medications in adolescence and young adulthood, especially for difficulties adapting to the typical events of those phases of life. Expert psychotherapy should be the first choice in these instances.

• Medication, when necessary, should be prescribed for as limited a time as possible. It is important for young people to advance their own development, not feel needlessly beholden to medications, not get iatrogenically dependent on them, and not feel that they have “diseases” they don’t have.

Amphetamines for misdiagnosed ADHD

When Ms. D’s family moved to a new house, she, her brother, and her sister, each attended a new school. Unlike her siblings, Ms. D, who was in high school, had a difficult adjustment. Her grades fell. She was taken to a psychiatrist who diagnosed ADHD and prescribed amphetamines. The psychiatrist paid little attention to her prior lack of difficulty in school or her struggles making new friends. Nor did the psychiatrist learn that Ms. D had to ward off the seductive advances of an older teacher (although Ms. D would likely not have been immediately forthcoming about this at the time).

When Ms. D came to me as a college student, for troubles with anger, anxiety, and some depression, she was religiously taking 70 mg of amphetamines daily. After I learned a bit about her and raised the question of whether she actually had ADHD, and whether it might make sense to consider tapering the amphetamines, she was appalled and looked like a toddler who was afraid I was about to steal her candy. Helping her to get off the unneeded medication was a multiyear process.

First, she had to recognize that it was prescribed to treat a problem she probably didn’t have, and second, that it was failing to help her with the problems she did have. As we attended to some of her actual emotional conflicts, she became willing to experiment with lower doses. She was able to see that her work was little changed as the dose was lowered, and that her difficulties with school had more to do with feelings toward classmates and teachers than with the presence or absence of amphetamines. After a protracted struggle, finally off the medication, she felt in charge of her life and no longer believed there was something inherently wrong with her mind or her brain.

Mr. E was the only son in a high-powered academic family. His older sisters were all intellectual standouts. Early in high school, he received his first B as a grade in a course. He was taken to a pediatrician, diagnosed with ADHD, and put on stimulants. Like Ms. D, he came to believe that he needed them. In college, he began to develop some magical aspects to his thinking, a potential side effect of the stimulants. It was very difficult to help him see either that he had a problem with his thinking or that it might be attributable to the medication.

Principles to consider

• If the ADHD wasn’t there in elementary school or before, it is unlikely that an adolescent or young adult has new-onset ADHD. A new or newly amplified conflict is occurring in the person’s mind and life A dynamic diagnosis, as always, is essential.

• When medication is prescribed for actual ADHD, as with anything else, the question of how long it will be taken must be asked. For life? Until other means of adaptation are accomplished? Until adequate outcome studies of long-term use of the medication are performed?

Helping patients to get off unneeded, or no longer needed, medications can be a difficult task. Their emotional attachments to the medications can be intense and varied. For some, the prescription is a sign of being loved and cared for. For others, it represents a certification of a deficit, appeases guilt about success, and/or attests to the need for special consideration. Insofar as the medication has been helpful, it may have come to be regarded as a dearly loved friend, or even a part of the self.

When medication has been helpful, there is also, of course, concern about the potential return of the difficulties for which it was prescribed. Few patients are told at the time of first prescription that there is potential risk of habituation and return of, or potential exaggeration of, symptoms with discontinuation. This type of discussion is more difficult to have in situations in which a prescription is urgently needed and the patient is reluctant, but is still not often done in those instances in which a prescription is more optional than essential. The picture is seldom simple.

These few comments only scratch the surface of the difficulties doctors and patients face in helping patients to discontinue their medications. Residency programs pay a lot of attention to helping trainees learn to prescribe medications; rarely do they sufficiently educate residents how to help patients discontinue them. The fact that so many residencies currently pay limited attention to interventions apart from medication contributes further to the difficulty.

Medications have saved the life of many a psychiatric patient. Some patients need medication for life. But some end up on medication for life, even in some instances when the medication may not have been needed in the first place. Although it is often a difficult task, we need to do a better job of distinguishing which patients are which.
 

Dr. Blum is a psychiatrist and psychoanalyst in private practice in Philadelphia. He teaches in the departments of anthropology and psychiatry at the University of Pennsylvania and at the Psychoanalytic Center of Philadelphia.

The knowledge about Loeys-Dietz syndrome has evolved quickly since Hal Dietz, MD, and Bart Loeys, MD, at Johns Hopkins University, Baltimore, first reported on it in 2005. Now, another team of Johns Hopkins investigators have reported that an aggressive approach with aortic root replacement coupled with valve-sparing whenever possible produces favorable results, but that clinicians must follow these patients closely with cardiovascular imaging.

“Growing experience with Loeys-Dietz syndrome has confirmed early impressions of its aggressive nature and proclivity toward aortic catastrophe,” Nishant D. Patel, MD, and his coauthors said in the February issue of the Journal of Thoracic and Cardiovascular Surgery (2017;153:406-12). They reported on results of all 79 patients with Loeys-Dietz syndrome (LDS) who had cardiovascular surgery at Johns Hopkins. There were two (3%) deaths during surgery and eight (10%) late deaths.

Patients with LDS are at risk for dissection early when the aortic root reaches 4 cm. Despite what they termed “favorable” outcomes of surgery, Dr. Patel and his coauthors acknowledged that reintervention rates for this population are high – 19 patients (24%) had subsequent operations. That suggests cardiac surgeons must closely monitor these patients. “Meticulous follow-up with cardiovascular surveillance imaging remains important for management, particularly as clinical LDS subtypes are characterized and more tailored treatment is developed,” Dr. Patel and his coauthors reported.

They advise echocardiography every 3 to 6 months for the first year after surgery and then every 6 to 12 months afterward. Full-body imaging should occur at least every 2 years.

“In particular, patients with type B dissections should be monitored aggressively for aneurysm growth,” Dr. Patel and his coauthors said. They recommend imaging at seven to 14 days after dissection, then repeat imaging at 1, 3, 6, and 12 months, and then yearly thereafter.

They noted that four LDS subtypes have been identified. Although those with LDS1 and 2 subtypes are prone to aortic rupture at an earlier age and at smaller aortic diameters than other connective tissue disorders, the medical and surgical management for all subtypes are similar, Dr. Patel and his coauthors indicated.

“Certain congenital heart defects are more common among patients with LDS, compared with the normal population, including patent ductus arteriosus and mitral valve prolapse/insufficiency,” they said. Genotype is one factor that determines the need for surgery in LDS patients, Dr. Patel and his coauthors said. Others are growth rate, aortic valve function, family history, and severity of noncardiac phenotype.

The 79 patients in the study were divided almost evenly between gender, and the average age at first operation was 24.9 years; 38 were children younger than 18 years and 20 had a previous sternotomy.

Aortic root replacement represented the predominant operation in the group, accounting for 65 operations (82.3%), of which 52 (80%) were valve-sparing procedures and the remainder were composite valve-graft procedures. The other procedures the researchers performed were nine aortic arch replacements (11.4%), three open thoracoabdominal repairs (3.8%) and two ascending aorta replacements (2.5%).

“Valve-sparing root replacement has become a safe and reliable option for appropriately selected younger patients with LDS,” Dr. Patel and his coauthors wrote. Five patients needed a second operation on the aortic valve or root; three of them had a Florida sleeve procedure. “Based on these initial outcomes with the Florida sleeve at our institution, we have abandoned this procedure in favor of conventional valve-sparing root replacement,” Dr. Patel and his coauthors stated.

Dr. Patel and his coauthors had no financial relationships to disclose.
 

The knowledge about Loeys-Dietz syndrome has evolved quickly since Hal Dietz, MD, and Bart Loeys, MD, at Johns Hopkins University, Baltimore, first reported on it in 2005. Now, another team of Johns Hopkins investigators have reported that an aggressive approach with aortic root replacement coupled with valve-sparing whenever possible produces favorable results, but that clinicians must follow these patients closely with cardiovascular imaging.

“Growing experience with Loeys-Dietz syndrome has confirmed early impressions of its aggressive nature and proclivity toward aortic catastrophe,” Nishant D. Patel, MD, and his coauthors said in the February issue of the Journal of Thoracic and Cardiovascular Surgery (2017;153:406-12). They reported on results of all 79 patients with Loeys-Dietz syndrome (LDS) who had cardiovascular surgery at Johns Hopkins. There were two (3%) deaths during surgery and eight (10%) late deaths.

Patients with LDS are at risk for dissection early when the aortic root reaches 4 cm. Despite what they termed “favorable” outcomes of surgery, Dr. Patel and his coauthors acknowledged that reintervention rates for this population are high – 19 patients (24%) had subsequent operations. That suggests cardiac surgeons must closely monitor these patients. “Meticulous follow-up with cardiovascular surveillance imaging remains important for management, particularly as clinical LDS subtypes are characterized and more tailored treatment is developed,” Dr. Patel and his coauthors reported.

They advise echocardiography every 3 to 6 months for the first year after surgery and then every 6 to 12 months afterward. Full-body imaging should occur at least every 2 years.

“In particular, patients with type B dissections should be monitored aggressively for aneurysm growth,” Dr. Patel and his coauthors said. They recommend imaging at seven to 14 days after dissection, then repeat imaging at 1, 3, 6, and 12 months, and then yearly thereafter.

They noted that four LDS subtypes have been identified. Although those with LDS1 and 2 subtypes are prone to aortic rupture at an earlier age and at smaller aortic diameters than other connective tissue disorders, the medical and surgical management for all subtypes are similar, Dr. Patel and his coauthors indicated.

“Certain congenital heart defects are more common among patients with LDS, compared with the normal population, including patent ductus arteriosus and mitral valve prolapse/insufficiency,” they said. Genotype is one factor that determines the need for surgery in LDS patients, Dr. Patel and his coauthors said. Others are growth rate, aortic valve function, family history, and severity of noncardiac phenotype.

The 79 patients in the study were divided almost evenly between gender, and the average age at first operation was 24.9 years; 38 were children younger than 18 years and 20 had a previous sternotomy.

Aortic root replacement represented the predominant operation in the group, accounting for 65 operations (82.3%), of which 52 (80%) were valve-sparing procedures and the remainder were composite valve-graft procedures. The other procedures the researchers performed were nine aortic arch replacements (11.4%), three open thoracoabdominal repairs (3.8%) and two ascending aorta replacements (2.5%).

“Valve-sparing root replacement has become a safe and reliable option for appropriately selected younger patients with LDS,” Dr. Patel and his coauthors wrote. Five patients needed a second operation on the aortic valve or root; three of them had a Florida sleeve procedure. “Based on these initial outcomes with the Florida sleeve at our institution, we have abandoned this procedure in favor of conventional valve-sparing root replacement,” Dr. Patel and his coauthors stated.

Dr. Patel and his coauthors had no financial relationships to disclose.
 

The knowledge about Loeys-Dietz syndrome has evolved quickly since Hal Dietz, MD, and Bart Loeys, MD, at Johns Hopkins University, Baltimore, first reported on it in 2005. Now, another team of Johns Hopkins investigators have reported that an aggressive approach with aortic root replacement coupled with valve-sparing whenever possible produces favorable results, but that clinicians must follow these patients closely with cardiovascular imaging.

“Growing experience with Loeys-Dietz syndrome has confirmed early impressions of its aggressive nature and proclivity toward aortic catastrophe,” Nishant D. Patel, MD, and his coauthors said in the February issue of the Journal of Thoracic and Cardiovascular Surgery (2017;153:406-12). They reported on results of all 79 patients with Loeys-Dietz syndrome (LDS) who had cardiovascular surgery at Johns Hopkins. There were two (3%) deaths during surgery and eight (10%) late deaths.

Patients with LDS are at risk for dissection early when the aortic root reaches 4 cm. Despite what they termed “favorable” outcomes of surgery, Dr. Patel and his coauthors acknowledged that reintervention rates for this population are high – 19 patients (24%) had subsequent operations. That suggests cardiac surgeons must closely monitor these patients. “Meticulous follow-up with cardiovascular surveillance imaging remains important for management, particularly as clinical LDS subtypes are characterized and more tailored treatment is developed,” Dr. Patel and his coauthors reported.

They advise echocardiography every 3 to 6 months for the first year after surgery and then every 6 to 12 months afterward. Full-body imaging should occur at least every 2 years.

“In particular, patients with type B dissections should be monitored aggressively for aneurysm growth,” Dr. Patel and his coauthors said. They recommend imaging at seven to 14 days after dissection, then repeat imaging at 1, 3, 6, and 12 months, and then yearly thereafter.

They noted that four LDS subtypes have been identified. Although those with LDS1 and 2 subtypes are prone to aortic rupture at an earlier age and at smaller aortic diameters than other connective tissue disorders, the medical and surgical management for all subtypes are similar, Dr. Patel and his coauthors indicated.

“Certain congenital heart defects are more common among patients with LDS, compared with the normal population, including patent ductus arteriosus and mitral valve prolapse/insufficiency,” they said. Genotype is one factor that determines the need for surgery in LDS patients, Dr. Patel and his coauthors said. Others are growth rate, aortic valve function, family history, and severity of noncardiac phenotype.

The 79 patients in the study were divided almost evenly between gender, and the average age at first operation was 24.9 years; 38 were children younger than 18 years and 20 had a previous sternotomy.

Aortic root replacement represented the predominant operation in the group, accounting for 65 operations (82.3%), of which 52 (80%) were valve-sparing procedures and the remainder were composite valve-graft procedures. The other procedures the researchers performed were nine aortic arch replacements (11.4%), three open thoracoabdominal repairs (3.8%) and two ascending aorta replacements (2.5%).

“Valve-sparing root replacement has become a safe and reliable option for appropriately selected younger patients with LDS,” Dr. Patel and his coauthors wrote. Five patients needed a second operation on the aortic valve or root; three of them had a Florida sleeve procedure. “Based on these initial outcomes with the Florida sleeve at our institution, we have abandoned this procedure in favor of conventional valve-sparing root replacement,” Dr. Patel and his coauthors stated.

Dr. Patel and his coauthors had no financial relationships to disclose.