MIAMI—When evaluating a patient with a movement disorder that seems to be rare, neurologists should employ their clinical expertise and consider a broad range of possible causes, said Anthony E. Lang, MD, Professor and Director of Neurology at the University of Toronto.

“Not everything is going to be diagnosed with whole exome sequencing,” Dr. Lang said at the First Pan American Parkinson’s Disease and Movement Disorders Congress. “Think much more broadly than that, or you are going to miss some important diagnoses and, in some cases, miss quite treatable diagnoses.”

A patient’s history, the physical and neurologic exam, and laboratory and genetic testing are important tools in developing a differential diagnosis. Once a diagnosis has been made, certain disorders may entail a risk of long-term complications that require preventive measures and surveillance.

Dr. Lang cochairs an annual session at the International Congress of Parkinson’s Disease and Movement Disorders that features video presentations of unusual cases. “The more you are aware of and the more you have seen, the more you may have an impact on helping the patient,” he said.

Think Broadly

Approximately 80% of rare diseases are genetic, and modern molecular genetic tests increasingly allow neurologists to make accurate diagnoses. Other causes of rare movement disorders include autoimmune disease, infection, neoplasms, environmental exposures, degenerative disorders, and deficiency states.

Camilo Toro, MD, a neurologist with the NIH Undiagnosed Diseases Program, advises that the classical phenotypes of rare diseases often represent the worst-case scenario, Dr. Lang said. Neurologists increasingly are recognizing broader phenotypic variability and milder forms of rare genetic diseases. In addition, the boundaries between pediatric and adult genetic disorders are blurred, and a patient with more than one genetic disorder may have a blended phenotype. It is also possible that a patient has a rare presentation of a common disorder.

Patient and Family History

Neurologists should consider how a movement disorder began (eg, triggers or precipitants) and whether a patient has other neurologic or general medical illnesses. A patient’s old images, videos, and laboratory tests may offer insights. Genetic counselors can be a valuable resource for neurologists who are interested in learning how to take a careful family history.

Possible sources of confusion about the inheritance of genetic disorders include de novo mutations, missed family history of a disorder (eg, if a relative has a mild manifestation), incomplete penetrance of dominant disorders, incorrect attribution of paternity, and the possibility of pseudodominance in inbred families with recessive disorders. Other types of inheritance include maternal inheritance, maternal imprinting (eg, in myoclonus dystonia), and uniparental disomy.

General and Neurologic Examinations

A patient’s habitus, stature, and facial dysmorphism may be informative. After seeing a patient with Woodhouse-Sakati syndrome in a case that had been presented at a conference, Dr. Lang recognized that he had a patient with the same facial characteristics. “Sure enough, the patient has Woodhouse-Sakati syndrome,” he said.

Patients with 22q11.2 deletion syndrome may have variable craniofacial features, and Dr. Lang’s clinic is following a number of patients with parkinsonism or other movement disorders as a consequence of this disorder.

Skin and related features, including telangiectasia, pigmentation, and nails, may suggest a diagnosis (eg, blue lunula in Wilson’s disease), as can the heart and other organs.

In addition to movements, other information can be gleaned from the eye during an examination, such as the presence of a sunflower cataract (which may suggest Wilson’s disease), a cherry-red spot on the retina (sialidosis), or choreoretinitis with maculopathy and optic atrophy (eg, subacute sclerosing panencephalitis).

During the neurologic examination, neurologists can assess behavior, language, cranial nerves, upper and lower motor neuron signs, eye movement disorders (eg, opsoclonus, oculogyric crisis, oculomotor apraxia, and supranuclear gaze palsies), and the phenomenology, distribution, and timing of movement disorders. In addition, a sensory exam may be useful. Parkinsonism with pure dorsal column sensory abnormalities, for example, is characteristic of POLG1 mutations.

Laboratory Tests

Many laboratory tests are available, and neurologists should use them selectively. As a visiting professor, Dr. Lang sees many patients who have undergone unnecessary tests. “Do not use a shotgun approach,” Dr. Lang said. “Be focused. Use them intelligently.”

Various metabolic pathways can be altered in patients with inborn errors of metabolism, thus creating risk of decompensation. Tests for patients suspected of having an inborn error of metabolism may include blood gases, anion gap, ammonia, glucose, lactate, uric acid, creatine kinase, amino acids, insulin, urine organic acids, ketones, and reducing substances.

Blood can be tested for heavy metals, vitamin deficiencies, and antibodies. CSF testing may be invaluable, including real-time quaking-induced conversion in patients with a suspected prion disorder or, when warranted, evaluation for extremely rare disorders such as testing for folate in patients with suspected cerebral folate deficiency.

Tissue biopsies, EEG, and electroretinogram may reveal useful information, and movement disorders laboratory testing, including assessments for functional movement disorder studies (eg, back-averaging) can help diagnose selected conditions, including psychogenic movement disorders.

Imaging

Neurologists should be comfortable looking at neuroimaging to identify patterns of atrophy, the presence of heavy metals, or the occurrence of calcium, for example. Some rare movement disorders require vascular imaging. “Unexpected normal findings should be a clue as well,” he said. Dr. Lang described a patient who was thought to have cerebral palsy, but the patient’s MRI was normal. The patient ultimately was found to have ADCY5-related dyskinesia.

Basal ganglia lesions and recurrent episodes of encephalopathy may indicate that a patient has biotin-responsive basal ganglia disease. “This is a critical disorder for us to recognize,” Dr. Lang said, because a patient who is treated early enough with biotin and thiamine can make a striking recovery.

Punctate changes throughout the posterior fossa, the cerebellum, and the pons, as well as other regions, are characteristic of a vascular abnormality called chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS), which can be treated with prednisone.

Genetic Testing

Genetic testing can help establish a diagnosis, but even in careful studies, whole exome sequencing may fail to provide a diagnosis in more than 40% of cases.

Problems with next-generation sequencing include incidental findings, variants of unknown significance, and the possibility of patients having two or more concomitant diseases or incompletely penetrant pathogenic variants. In addition, clinical exome sequencing does not detect repeat expansion disorders, copy number variations, structural variants, or abnormalities in the noncoding part of the genome.

A recently published case report further highlights the need for clinical expertise amid advances in genetic testing, Dr. Lang said. Zittel et al reported a case of a patient who came to a clinic with genetic test results showing that she had GCH1 and TH mutations. Clinically, however, neurologists determined that she had a functional movement disorder. When they went back to the original genetic testing laboratory, they discovered that the patient had doctored the form with the genetic test results. The authors deemed it a case of Munchausen syndrome by genetics.

Treatment

Neurologists should especially consider the possibility of movement disorders caused by autoimmune disease, infection, deficiency states, toxins, drugs, and treatable inborn errors of metabolism because treatment may alter the natural history of the disease and patient outcomes in these cases, Dr. Lang said.

Treatment of metabolic disorders typically requires a multidisciplinary team and may consist of substrate reduction, removal or enhanced clearance of toxic metabolites, replenishment of depleted metabolites, enzyme therapy, cell or organ replacement, and gene therapy.

Certain patients require long-term screening and follow-up to monitor for and prevent complications.

Avoiding infection, trauma, surgery, and vaccination may be important for patients with defects of small molecules. Patients with McLeod syndrome are at risk of transfusion reactions, and patients with ataxia-telangiectasia mutated gene variants should avoid ionizing radiation because of increased risk of malignancy. “If you are following patients with any of these rare disorders, you need to be aware of the long-term consequences,” Dr. Lang said.

In addition, online resources can provide useful information for neurologists who are evaluating patients with potentially rare movement disorders, including OMIM, GeneReviews, Orphanet, Face2Gene, SimulConsult, and MDSGene, Dr. Lang said.

—Jake Remaly

Suggested Reading

Gupta A, Lang AE. Psychogenic movement disorders. Curr Opin Neurol. 2009;22(4):430-436.

Sethi KD, Lang AE. Will new genetic techniques like exome sequencing obviate the need for clinical expertise? No. Mov Disord Clin Pract. 2017;4(1):39-41.

Ure RJ, Dhanju S, Lang AE, Fasano A. Unusual tremor syndromes: know in order to recognise. J Neurol Neurosurg Psychiatry. 2016;87(11):1191-1203.

Zittel S, Lohmann K, Bauer P, et al. Munchausen syndrome by genetics: Next-generation challenges for clinicians. Neurology. 2017;88(10):1000-1001.

MIAMI—When evaluating a patient with a movement disorder that seems to be rare, neurologists should employ their clinical expertise and consider a broad range of possible causes, said Anthony E. Lang, MD, Professor and Director of Neurology at the University of Toronto.

“Not everything is going to be diagnosed with whole exome sequencing,” Dr. Lang said at the First Pan American Parkinson’s Disease and Movement Disorders Congress. “Think much more broadly than that, or you are going to miss some important diagnoses and, in some cases, miss quite treatable diagnoses.”

A patient’s history, the physical and neurologic exam, and laboratory and genetic testing are important tools in developing a differential diagnosis. Once a diagnosis has been made, certain disorders may entail a risk of long-term complications that require preventive measures and surveillance.

Dr. Lang cochairs an annual session at the International Congress of Parkinson’s Disease and Movement Disorders that features video presentations of unusual cases. “The more you are aware of and the more you have seen, the more you may have an impact on helping the patient,” he said.

Think Broadly

Approximately 80% of rare diseases are genetic, and modern molecular genetic tests increasingly allow neurologists to make accurate diagnoses. Other causes of rare movement disorders include autoimmune disease, infection, neoplasms, environmental exposures, degenerative disorders, and deficiency states.

Camilo Toro, MD, a neurologist with the NIH Undiagnosed Diseases Program, advises that the classical phenotypes of rare diseases often represent the worst-case scenario, Dr. Lang said. Neurologists increasingly are recognizing broader phenotypic variability and milder forms of rare genetic diseases. In addition, the boundaries between pediatric and adult genetic disorders are blurred, and a patient with more than one genetic disorder may have a blended phenotype. It is also possible that a patient has a rare presentation of a common disorder.

Patient and Family History

Neurologists should consider how a movement disorder began (eg, triggers or precipitants) and whether a patient has other neurologic or general medical illnesses. A patient’s old images, videos, and laboratory tests may offer insights. Genetic counselors can be a valuable resource for neurologists who are interested in learning how to take a careful family history.

Possible sources of confusion about the inheritance of genetic disorders include de novo mutations, missed family history of a disorder (eg, if a relative has a mild manifestation), incomplete penetrance of dominant disorders, incorrect attribution of paternity, and the possibility of pseudodominance in inbred families with recessive disorders. Other types of inheritance include maternal inheritance, maternal imprinting (eg, in myoclonus dystonia), and uniparental disomy.

General and Neurologic Examinations

A patient’s habitus, stature, and facial dysmorphism may be informative. After seeing a patient with Woodhouse-Sakati syndrome in a case that had been presented at a conference, Dr. Lang recognized that he had a patient with the same facial characteristics. “Sure enough, the patient has Woodhouse-Sakati syndrome,” he said.

Patients with 22q11.2 deletion syndrome may have variable craniofacial features, and Dr. Lang’s clinic is following a number of patients with parkinsonism or other movement disorders as a consequence of this disorder.

Skin and related features, including telangiectasia, pigmentation, and nails, may suggest a diagnosis (eg, blue lunula in Wilson’s disease), as can the heart and other organs.

In addition to movements, other information can be gleaned from the eye during an examination, such as the presence of a sunflower cataract (which may suggest Wilson’s disease), a cherry-red spot on the retina (sialidosis), or choreoretinitis with maculopathy and optic atrophy (eg, subacute sclerosing panencephalitis).

During the neurologic examination, neurologists can assess behavior, language, cranial nerves, upper and lower motor neuron signs, eye movement disorders (eg, opsoclonus, oculogyric crisis, oculomotor apraxia, and supranuclear gaze palsies), and the phenomenology, distribution, and timing of movement disorders. In addition, a sensory exam may be useful. Parkinsonism with pure dorsal column sensory abnormalities, for example, is characteristic of POLG1 mutations.

Laboratory Tests

Many laboratory tests are available, and neurologists should use them selectively. As a visiting professor, Dr. Lang sees many patients who have undergone unnecessary tests. “Do not use a shotgun approach,” Dr. Lang said. “Be focused. Use them intelligently.”

Various metabolic pathways can be altered in patients with inborn errors of metabolism, thus creating risk of decompensation. Tests for patients suspected of having an inborn error of metabolism may include blood gases, anion gap, ammonia, glucose, lactate, uric acid, creatine kinase, amino acids, insulin, urine organic acids, ketones, and reducing substances.

Blood can be tested for heavy metals, vitamin deficiencies, and antibodies. CSF testing may be invaluable, including real-time quaking-induced conversion in patients with a suspected prion disorder or, when warranted, evaluation for extremely rare disorders such as testing for folate in patients with suspected cerebral folate deficiency.

Tissue biopsies, EEG, and electroretinogram may reveal useful information, and movement disorders laboratory testing, including assessments for functional movement disorder studies (eg, back-averaging) can help diagnose selected conditions, including psychogenic movement disorders.

Imaging

Neurologists should be comfortable looking at neuroimaging to identify patterns of atrophy, the presence of heavy metals, or the occurrence of calcium, for example. Some rare movement disorders require vascular imaging. “Unexpected normal findings should be a clue as well,” he said. Dr. Lang described a patient who was thought to have cerebral palsy, but the patient’s MRI was normal. The patient ultimately was found to have ADCY5-related dyskinesia.

Basal ganglia lesions and recurrent episodes of encephalopathy may indicate that a patient has biotin-responsive basal ganglia disease. “This is a critical disorder for us to recognize,” Dr. Lang said, because a patient who is treated early enough with biotin and thiamine can make a striking recovery.

Punctate changes throughout the posterior fossa, the cerebellum, and the pons, as well as other regions, are characteristic of a vascular abnormality called chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS), which can be treated with prednisone.

Genetic Testing

Genetic testing can help establish a diagnosis, but even in careful studies, whole exome sequencing may fail to provide a diagnosis in more than 40% of cases.

Problems with next-generation sequencing include incidental findings, variants of unknown significance, and the possibility of patients having two or more concomitant diseases or incompletely penetrant pathogenic variants. In addition, clinical exome sequencing does not detect repeat expansion disorders, copy number variations, structural variants, or abnormalities in the noncoding part of the genome.

A recently published case report further highlights the need for clinical expertise amid advances in genetic testing, Dr. Lang said. Zittel et al reported a case of a patient who came to a clinic with genetic test results showing that she had GCH1 and TH mutations. Clinically, however, neurologists determined that she had a functional movement disorder. When they went back to the original genetic testing laboratory, they discovered that the patient had doctored the form with the genetic test results. The authors deemed it a case of Munchausen syndrome by genetics.

Treatment

Neurologists should especially consider the possibility of movement disorders caused by autoimmune disease, infection, deficiency states, toxins, drugs, and treatable inborn errors of metabolism because treatment may alter the natural history of the disease and patient outcomes in these cases, Dr. Lang said.

Treatment of metabolic disorders typically requires a multidisciplinary team and may consist of substrate reduction, removal or enhanced clearance of toxic metabolites, replenishment of depleted metabolites, enzyme therapy, cell or organ replacement, and gene therapy.

Certain patients require long-term screening and follow-up to monitor for and prevent complications.

Avoiding infection, trauma, surgery, and vaccination may be important for patients with defects of small molecules. Patients with McLeod syndrome are at risk of transfusion reactions, and patients with ataxia-telangiectasia mutated gene variants should avoid ionizing radiation because of increased risk of malignancy. “If you are following patients with any of these rare disorders, you need to be aware of the long-term consequences,” Dr. Lang said.

In addition, online resources can provide useful information for neurologists who are evaluating patients with potentially rare movement disorders, including OMIM, GeneReviews, Orphanet, Face2Gene, SimulConsult, and MDSGene, Dr. Lang said.

—Jake Remaly

Suggested Reading

Gupta A, Lang AE. Psychogenic movement disorders. Curr Opin Neurol. 2009;22(4):430-436.

Sethi KD, Lang AE. Will new genetic techniques like exome sequencing obviate the need for clinical expertise? No. Mov Disord Clin Pract. 2017;4(1):39-41.

Ure RJ, Dhanju S, Lang AE, Fasano A. Unusual tremor syndromes: know in order to recognise. J Neurol Neurosurg Psychiatry. 2016;87(11):1191-1203.

Zittel S, Lohmann K, Bauer P, et al. Munchausen syndrome by genetics: Next-generation challenges for clinicians. Neurology. 2017;88(10):1000-1001.

MIAMI—When evaluating a patient with a movement disorder that seems to be rare, neurologists should employ their clinical expertise and consider a broad range of possible causes, said Anthony E. Lang, MD, Professor and Director of Neurology at the University of Toronto.

“Not everything is going to be diagnosed with whole exome sequencing,” Dr. Lang said at the First Pan American Parkinson’s Disease and Movement Disorders Congress. “Think much more broadly than that, or you are going to miss some important diagnoses and, in some cases, miss quite treatable diagnoses.”

A patient’s history, the physical and neurologic exam, and laboratory and genetic testing are important tools in developing a differential diagnosis. Once a diagnosis has been made, certain disorders may entail a risk of long-term complications that require preventive measures and surveillance.

Dr. Lang cochairs an annual session at the International Congress of Parkinson’s Disease and Movement Disorders that features video presentations of unusual cases. “The more you are aware of and the more you have seen, the more you may have an impact on helping the patient,” he said.

Think Broadly

Approximately 80% of rare diseases are genetic, and modern molecular genetic tests increasingly allow neurologists to make accurate diagnoses. Other causes of rare movement disorders include autoimmune disease, infection, neoplasms, environmental exposures, degenerative disorders, and deficiency states.

Camilo Toro, MD, a neurologist with the NIH Undiagnosed Diseases Program, advises that the classical phenotypes of rare diseases often represent the worst-case scenario, Dr. Lang said. Neurologists increasingly are recognizing broader phenotypic variability and milder forms of rare genetic diseases. In addition, the boundaries between pediatric and adult genetic disorders are blurred, and a patient with more than one genetic disorder may have a blended phenotype. It is also possible that a patient has a rare presentation of a common disorder.

Patient and Family History

Neurologists should consider how a movement disorder began (eg, triggers or precipitants) and whether a patient has other neurologic or general medical illnesses. A patient’s old images, videos, and laboratory tests may offer insights. Genetic counselors can be a valuable resource for neurologists who are interested in learning how to take a careful family history.

Possible sources of confusion about the inheritance of genetic disorders include de novo mutations, missed family history of a disorder (eg, if a relative has a mild manifestation), incomplete penetrance of dominant disorders, incorrect attribution of paternity, and the possibility of pseudodominance in inbred families with recessive disorders. Other types of inheritance include maternal inheritance, maternal imprinting (eg, in myoclonus dystonia), and uniparental disomy.

General and Neurologic Examinations

A patient’s habitus, stature, and facial dysmorphism may be informative. After seeing a patient with Woodhouse-Sakati syndrome in a case that had been presented at a conference, Dr. Lang recognized that he had a patient with the same facial characteristics. “Sure enough, the patient has Woodhouse-Sakati syndrome,” he said.

Patients with 22q11.2 deletion syndrome may have variable craniofacial features, and Dr. Lang’s clinic is following a number of patients with parkinsonism or other movement disorders as a consequence of this disorder.

Skin and related features, including telangiectasia, pigmentation, and nails, may suggest a diagnosis (eg, blue lunula in Wilson’s disease), as can the heart and other organs.

In addition to movements, other information can be gleaned from the eye during an examination, such as the presence of a sunflower cataract (which may suggest Wilson’s disease), a cherry-red spot on the retina (sialidosis), or choreoretinitis with maculopathy and optic atrophy (eg, subacute sclerosing panencephalitis).

During the neurologic examination, neurologists can assess behavior, language, cranial nerves, upper and lower motor neuron signs, eye movement disorders (eg, opsoclonus, oculogyric crisis, oculomotor apraxia, and supranuclear gaze palsies), and the phenomenology, distribution, and timing of movement disorders. In addition, a sensory exam may be useful. Parkinsonism with pure dorsal column sensory abnormalities, for example, is characteristic of POLG1 mutations.

Laboratory Tests

Many laboratory tests are available, and neurologists should use them selectively. As a visiting professor, Dr. Lang sees many patients who have undergone unnecessary tests. “Do not use a shotgun approach,” Dr. Lang said. “Be focused. Use them intelligently.”

Various metabolic pathways can be altered in patients with inborn errors of metabolism, thus creating risk of decompensation. Tests for patients suspected of having an inborn error of metabolism may include blood gases, anion gap, ammonia, glucose, lactate, uric acid, creatine kinase, amino acids, insulin, urine organic acids, ketones, and reducing substances.

Blood can be tested for heavy metals, vitamin deficiencies, and antibodies. CSF testing may be invaluable, including real-time quaking-induced conversion in patients with a suspected prion disorder or, when warranted, evaluation for extremely rare disorders such as testing for folate in patients with suspected cerebral folate deficiency.

Tissue biopsies, EEG, and electroretinogram may reveal useful information, and movement disorders laboratory testing, including assessments for functional movement disorder studies (eg, back-averaging) can help diagnose selected conditions, including psychogenic movement disorders.

Imaging

Neurologists should be comfortable looking at neuroimaging to identify patterns of atrophy, the presence of heavy metals, or the occurrence of calcium, for example. Some rare movement disorders require vascular imaging. “Unexpected normal findings should be a clue as well,” he said. Dr. Lang described a patient who was thought to have cerebral palsy, but the patient’s MRI was normal. The patient ultimately was found to have ADCY5-related dyskinesia.

Basal ganglia lesions and recurrent episodes of encephalopathy may indicate that a patient has biotin-responsive basal ganglia disease. “This is a critical disorder for us to recognize,” Dr. Lang said, because a patient who is treated early enough with biotin and thiamine can make a striking recovery.

Punctate changes throughout the posterior fossa, the cerebellum, and the pons, as well as other regions, are characteristic of a vascular abnormality called chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS), which can be treated with prednisone.

Genetic Testing

Genetic testing can help establish a diagnosis, but even in careful studies, whole exome sequencing may fail to provide a diagnosis in more than 40% of cases.

Problems with next-generation sequencing include incidental findings, variants of unknown significance, and the possibility of patients having two or more concomitant diseases or incompletely penetrant pathogenic variants. In addition, clinical exome sequencing does not detect repeat expansion disorders, copy number variations, structural variants, or abnormalities in the noncoding part of the genome.

A recently published case report further highlights the need for clinical expertise amid advances in genetic testing, Dr. Lang said. Zittel et al reported a case of a patient who came to a clinic with genetic test results showing that she had GCH1 and TH mutations. Clinically, however, neurologists determined that she had a functional movement disorder. When they went back to the original genetic testing laboratory, they discovered that the patient had doctored the form with the genetic test results. The authors deemed it a case of Munchausen syndrome by genetics.

Treatment

Neurologists should especially consider the possibility of movement disorders caused by autoimmune disease, infection, deficiency states, toxins, drugs, and treatable inborn errors of metabolism because treatment may alter the natural history of the disease and patient outcomes in these cases, Dr. Lang said.

Treatment of metabolic disorders typically requires a multidisciplinary team and may consist of substrate reduction, removal or enhanced clearance of toxic metabolites, replenishment of depleted metabolites, enzyme therapy, cell or organ replacement, and gene therapy.

Certain patients require long-term screening and follow-up to monitor for and prevent complications.

Avoiding infection, trauma, surgery, and vaccination may be important for patients with defects of small molecules. Patients with McLeod syndrome are at risk of transfusion reactions, and patients with ataxia-telangiectasia mutated gene variants should avoid ionizing radiation because of increased risk of malignancy. “If you are following patients with any of these rare disorders, you need to be aware of the long-term consequences,” Dr. Lang said.

In addition, online resources can provide useful information for neurologists who are evaluating patients with potentially rare movement disorders, including OMIM, GeneReviews, Orphanet, Face2Gene, SimulConsult, and MDSGene, Dr. Lang said.

—Jake Remaly

Suggested Reading

Gupta A, Lang AE. Psychogenic movement disorders. Curr Opin Neurol. 2009;22(4):430-436.

Sethi KD, Lang AE. Will new genetic techniques like exome sequencing obviate the need for clinical expertise? No. Mov Disord Clin Pract. 2017;4(1):39-41.

Ure RJ, Dhanju S, Lang AE, Fasano A. Unusual tremor syndromes: know in order to recognise. J Neurol Neurosurg Psychiatry. 2016;87(11):1191-1203.

Zittel S, Lohmann K, Bauer P, et al. Munchausen syndrome by genetics: Next-generation challenges for clinicians. Neurology. 2017;88(10):1000-1001.

“HOW AND WHEN UMBILICAL CORD GAS ANALYSIS CAN JUSTIFY YOUR OBSTETRIC MANAGEMENT”

MICHAEL G. ROSS, MD, MPH (MARCH 2017)

Cord gas analysis can be beneficial but has drawbacks

In his article, Dr. Ross makes a few statements I would like to challenge. He gives a list of indications for cord gas analysis, even with a vigorous newborn. I would suggest that doing so is not only unnecessary, but could get the delivering provider in trouble. Normal gases with a vigorous infant are not actionable, and neither are abnormal gases with a vigorous infant. The latter situation could, however, lower the bar for a lawsuit if any neurologic pathology is diagnosed in the child.

At our hospital, blood gas assessments generate charges of $90 for each arterial and venous sample. The author states that gases are helpful for staff education. If that is the purposeof measuring the gases when Apgar scores are normal, then the bill for the gases should be sent to the staff, not the patient or insurance company.

The precise reason for doing cord gases is to prove you are a good doctor. If the Apgar scores are low, a healthy set of gases shows that your interventions were timely and appropriate. Normal gases prevent lawsuits in this situation.

Joe Walsh, MD
Philadelphia, Pennsylvania

Dr. Ross responds

I appreciate the comments of Dr. Walsh, who suggests that we should not obtain cord gases in vigorous infants due, in part, to the hospital charges. There are several reasons for the indications detailed in the article. Although normal Apgar scores would appear to negate the potential for severe metabolic acidosis, Apgar scoring accuracy has been challenged in medical legal cases. Furthermore, there may be newborn complications (eg, pre-existing hypoxic injury, intraventricular bleed) that may not be recognized immediately, yet hypoxemia and acidosis may be alleged to have contributed to the outcome. The actual cost of running a blood gas sample is far less than the $90 hospital charges. Nevertheless, if hospital charge is a concern, I recommend that the physician obtain a cord gas sample immediately following the delivery and determine whether to run the sample after the 5-minute Apgar score is obtained.

Share your thoughts! Send your Letter to the Editor to [email protected]. Please include your name and the city and state in which you practice.

“HOW AND WHEN UMBILICAL CORD GAS ANALYSIS CAN JUSTIFY YOUR OBSTETRIC MANAGEMENT”

MICHAEL G. ROSS, MD, MPH (MARCH 2017)

Cord gas analysis can be beneficial but has drawbacks

In his article, Dr. Ross makes a few statements I would like to challenge. He gives a list of indications for cord gas analysis, even with a vigorous newborn. I would suggest that doing so is not only unnecessary, but could get the delivering provider in trouble. Normal gases with a vigorous infant are not actionable, and neither are abnormal gases with a vigorous infant. The latter situation could, however, lower the bar for a lawsuit if any neurologic pathology is diagnosed in the child.

At our hospital, blood gas assessments generate charges of $90 for each arterial and venous sample. The author states that gases are helpful for staff education. If that is the purposeof measuring the gases when Apgar scores are normal, then the bill for the gases should be sent to the staff, not the patient or insurance company.

The precise reason for doing cord gases is to prove you are a good doctor. If the Apgar scores are low, a healthy set of gases shows that your interventions were timely and appropriate. Normal gases prevent lawsuits in this situation.

Joe Walsh, MD
Philadelphia, Pennsylvania

Dr. Ross responds

I appreciate the comments of Dr. Walsh, who suggests that we should not obtain cord gases in vigorous infants due, in part, to the hospital charges. There are several reasons for the indications detailed in the article. Although normal Apgar scores would appear to negate the potential for severe metabolic acidosis, Apgar scoring accuracy has been challenged in medical legal cases. Furthermore, there may be newborn complications (eg, pre-existing hypoxic injury, intraventricular bleed) that may not be recognized immediately, yet hypoxemia and acidosis may be alleged to have contributed to the outcome. The actual cost of running a blood gas sample is far less than the $90 hospital charges. Nevertheless, if hospital charge is a concern, I recommend that the physician obtain a cord gas sample immediately following the delivery and determine whether to run the sample after the 5-minute Apgar score is obtained.

Share your thoughts! Send your Letter to the Editor to [email protected]. Please include your name and the city and state in which you practice.

“HOW AND WHEN UMBILICAL CORD GAS ANALYSIS CAN JUSTIFY YOUR OBSTETRIC MANAGEMENT”

MICHAEL G. ROSS, MD, MPH (MARCH 2017)

Cord gas analysis can be beneficial but has drawbacks

In his article, Dr. Ross makes a few statements I would like to challenge. He gives a list of indications for cord gas analysis, even with a vigorous newborn. I would suggest that doing so is not only unnecessary, but could get the delivering provider in trouble. Normal gases with a vigorous infant are not actionable, and neither are abnormal gases with a vigorous infant. The latter situation could, however, lower the bar for a lawsuit if any neurologic pathology is diagnosed in the child.

At our hospital, blood gas assessments generate charges of $90 for each arterial and venous sample. The author states that gases are helpful for staff education. If that is the purposeof measuring the gases when Apgar scores are normal, then the bill for the gases should be sent to the staff, not the patient or insurance company.

The precise reason for doing cord gases is to prove you are a good doctor. If the Apgar scores are low, a healthy set of gases shows that your interventions were timely and appropriate. Normal gases prevent lawsuits in this situation.

Joe Walsh, MD
Philadelphia, Pennsylvania

Dr. Ross responds

I appreciate the comments of Dr. Walsh, who suggests that we should not obtain cord gases in vigorous infants due, in part, to the hospital charges. There are several reasons for the indications detailed in the article. Although normal Apgar scores would appear to negate the potential for severe metabolic acidosis, Apgar scoring accuracy has been challenged in medical legal cases. Furthermore, there may be newborn complications (eg, pre-existing hypoxic injury, intraventricular bleed) that may not be recognized immediately, yet hypoxemia and acidosis may be alleged to have contributed to the outcome. The actual cost of running a blood gas sample is far less than the $90 hospital charges. Nevertheless, if hospital charge is a concern, I recommend that the physician obtain a cord gas sample immediately following the delivery and determine whether to run the sample after the 5-minute Apgar score is obtained.

Share your thoughts! Send your Letter to the Editor to [email protected]. Please include your name and the city and state in which you practice.

Stigma in Media and Society

Although neurologists and the general public acknowledge the symptoms and disability associated with a disease, they generally discount or fail to recognize its associated stigma, said Dr. Shapiro. Internalized stigma is a person’s sense that he or she is kept at a social distance, and enacted stigma refers to instances of discrimination on the basis of a person’s condition. Consequences of stigma include psychologic distress, low self-esteem, poor social outcomes, and poor health outcomes.

In a 2006 analysis, Caspermeyer et al examined 1,203 newspaper articles about neurologic conditions. They found that, after articles about epilepsy, articles about migraine contained the highest frequency of stigmatizing language (29%). Although migraine was among the most prevalent diseases in the analysis, it was among the least covered topics in newspaper articles.

To determine whether migraine is associated with stigma, Young and colleagues administered the Stigma Scale for Chronic Illness, a questionnaire, to patients with episodic migraine, chronic migraine, or epilepsy. The investigators observed that patients with chronic migraine and patients with epilepsy faced a similar amount of stigma. They further observed that stigma correlated most strongly with inability to work and was greater for chronic migraine than for epilepsy or episodic migraine.

Level of Stigma by Disease

Following these studies, Dr. Shapiro and colleagues investigated externalized stigma, or the rejection of others because of their condition. They polled adult members of the Amazon Mechanical Turk community, a group of approximately 500,000 people who voluntarily respond to surveys for modest monetary compensation. Compared with the US population, the Amazon Mechanical Turk community includes more women, Caucasians, young people, liberals, educated people, secular people, and people with low incomes. The group better represents the US population, however, than general Internet or university convenience samples do.

The investigators presented each respondent with one of four vignettes about people with a disease that does not respond well to treatment and that sometimes results in missed work or family activities. The vignettes were identical except for the condition named. The four conditions were migraine, epilepsy, panic disorder, and asthma. After reviewing the vignette, each respondent completed a validated stigma-assessment instrument using a Likert scale for each item.

In all, 765 people completed the instrument. Respondents’ mean age was 28, and 60% of the sample was female. There was no difference in the level of stigma attributed to migraine, panic disorder, or epilepsy, but respondents attributed less stigma to asthma than to the other three conditions. Noting the similar level of stigma between migraine and epilepsy, Dr. Shapiro said, “Epilepsy historically has been associated with demonic possession. If ever there were a disease that you would assume would have social distance or stigma attached, it certainly would be epilepsy.”

Disease and Productivity Loss

In a second study, Dr. Shapiro and colleagues presented members of the Amazon Mechanical Turk community with one of five vignettes. The vignettes described the following cases:

• A woman with migraine on four days per month who missed no workdays per year
• A woman with migraine on four days per month who missed two workdays per year
• A woman with migraine on 20 days per month who missed 10 workdays per year
• A man with migraine on four days per month who missed two workdays per year
• A woman with seizures on four days per month who missed two workdays per year.

Respondents then completed a social distance scale that included questions about the respondent’s willingness to socialize with, trust, hire, or allow the person in the vignette to marry into his or her family.

The researchers found that the level of externalized stigma was the same for the woman with migraine who missed two workdays per year as it was for the woman with epilepsy who missed two workdays per year. Respondents believed that the person with epilepsy would care more about whether he or she was a burden on others, compared with the migraineur. Respondents also said that the person with epilepsy would try harder and would be less likely to malinger, compared with the migraineur.

The gender of the person in the vignette was not associated with the level of stigma, said Dr. Shapiro. On the other hand, men were much more likely to stigmatize a man or a woman with migraine than women were.

In addition, the researchers found that the woman with chronic migraine who missed 10 workdays per year was much more likely to be stigmatized than migraineurs who missed fewer workdays. People who missed more workdays were less likely to be seen as trying hard, less likely to be interviewed, more likely to be considered malingerers, and less likely to be considered trustworthy.

Fear of Pain and Social Distance

As part of the same study, respondents completed instruments that measured fear of pain and empathy, and also provided demographic information, including migraine status. Overall, fear of pain was similar between migraineurs and nonmigraineurs. Migraineurs feared migraine as much as they feared falling down a flight of stairs or having a car door slammed on the hand. Nonmigraineurs, however, considered migraine to be less severe than migraineurs did. Among nonmigraineurs, greater fear of pain was associated with greater social distance from migraineurs. But in the same group, greater fear of migraine was associated with less social distance from migraineurs.

Furthermore, Dr. Shapiro’s group noted that the more migraine is part of a person’s experience, the less social distance that person maintains from migraineurs. Similarly, they found that as empathy increased, the social distance to migraine decreased.

Other findings included that younger people were more likely to stigmatize migraine than older people, and that non-Caucasians were more likely to stigmatize migraine than Caucasians. The gender of the stigmatizer was a dominant influence on the amount of stigma.

Reasons for Migraine Stigma

Various hypotheses offer potential reasons for stigmatizing migraine. Approximately 75% of migraineurs are women, and migraine changes with hormonal fluctuations. Hence, sexism against women may be one cause of stigma.

Also, migraine has been considered a behavior problem or conversion disorder for decades, said Dr. Shapiro. One illustration of this point is that a monograph published in 1926 identified migraine as a neurosis. In 1894, Freud described migraine as the result of a failure to find release after sexual stimulation.

Migraine is associated with headache, and headache has various connotations. The type of headache with which most people are familiar is tension-type headache, thus the general public may be likely to minimize the severity or importance of migraine. The word “headache” also connotes “concern” or “annoyance,” which may contribute to a minimization of migraine’s severity.

Whatever its origin, the stigma associated with migraine often is overlooked, said Dr. Shapiro. Neurologists should consider the potential effects of stigma on health outcomes as they treat patients with headache, he concluded.

—Erik Greb

Suggested Reading

Caspermeyer JJ, Sylvester EJ, Drazkowski JF, et al. Evaluation of stigmatizing language and medical errors in neurology coverage by US newspapers. Mayo Clin Proc. 2006;81(3):300-306.

Evans RW, Evans RE. A survey of neurologists on the likeability of headaches and other neurological disorders. Headache. 2010;50(7):1126-1129.

Evans RW, Evans RE, Kell HJ. A survey of family doctors on the likeability of migraine and other common diseases and their prevalence of migraine. Cephalalgia. 2010;30(5):620-623.

Young WB, Park JE, Tian IX, Kempner J. The stigma of migraine. PLoS One. 2013;8(1):e54074.

Stigma in Media and Society

Although neurologists and the general public acknowledge the symptoms and disability associated with a disease, they generally discount or fail to recognize its associated stigma, said Dr. Shapiro. Internalized stigma is a person’s sense that he or she is kept at a social distance, and enacted stigma refers to instances of discrimination on the basis of a person’s condition. Consequences of stigma include psychologic distress, low self-esteem, poor social outcomes, and poor health outcomes.

In a 2006 analysis, Caspermeyer et al examined 1,203 newspaper articles about neurologic conditions. They found that, after articles about epilepsy, articles about migraine contained the highest frequency of stigmatizing language (29%). Although migraine was among the most prevalent diseases in the analysis, it was among the least covered topics in newspaper articles.

To determine whether migraine is associated with stigma, Young and colleagues administered the Stigma Scale for Chronic Illness, a questionnaire, to patients with episodic migraine, chronic migraine, or epilepsy. The investigators observed that patients with chronic migraine and patients with epilepsy faced a similar amount of stigma. They further observed that stigma correlated most strongly with inability to work and was greater for chronic migraine than for epilepsy or episodic migraine.

Level of Stigma by Disease

Following these studies, Dr. Shapiro and colleagues investigated externalized stigma, or the rejection of others because of their condition. They polled adult members of the Amazon Mechanical Turk community, a group of approximately 500,000 people who voluntarily respond to surveys for modest monetary compensation. Compared with the US population, the Amazon Mechanical Turk community includes more women, Caucasians, young people, liberals, educated people, secular people, and people with low incomes. The group better represents the US population, however, than general Internet or university convenience samples do.

The investigators presented each respondent with one of four vignettes about people with a disease that does not respond well to treatment and that sometimes results in missed work or family activities. The vignettes were identical except for the condition named. The four conditions were migraine, epilepsy, panic disorder, and asthma. After reviewing the vignette, each respondent completed a validated stigma-assessment instrument using a Likert scale for each item.

In all, 765 people completed the instrument. Respondents’ mean age was 28, and 60% of the sample was female. There was no difference in the level of stigma attributed to migraine, panic disorder, or epilepsy, but respondents attributed less stigma to asthma than to the other three conditions. Noting the similar level of stigma between migraine and epilepsy, Dr. Shapiro said, “Epilepsy historically has been associated with demonic possession. If ever there were a disease that you would assume would have social distance or stigma attached, it certainly would be epilepsy.”

Disease and Productivity Loss

In a second study, Dr. Shapiro and colleagues presented members of the Amazon Mechanical Turk community with one of five vignettes. The vignettes described the following cases:

• A woman with migraine on four days per month who missed no workdays per year
• A woman with migraine on four days per month who missed two workdays per year
• A woman with migraine on 20 days per month who missed 10 workdays per year
• A man with migraine on four days per month who missed two workdays per year
• A woman with seizures on four days per month who missed two workdays per year.

Respondents then completed a social distance scale that included questions about the respondent’s willingness to socialize with, trust, hire, or allow the person in the vignette to marry into his or her family.

The researchers found that the level of externalized stigma was the same for the woman with migraine who missed two workdays per year as it was for the woman with epilepsy who missed two workdays per year. Respondents believed that the person with epilepsy would care more about whether he or she was a burden on others, compared with the migraineur. Respondents also said that the person with epilepsy would try harder and would be less likely to malinger, compared with the migraineur.

The gender of the person in the vignette was not associated with the level of stigma, said Dr. Shapiro. On the other hand, men were much more likely to stigmatize a man or a woman with migraine than women were.

In addition, the researchers found that the woman with chronic migraine who missed 10 workdays per year was much more likely to be stigmatized than migraineurs who missed fewer workdays. People who missed more workdays were less likely to be seen as trying hard, less likely to be interviewed, more likely to be considered malingerers, and less likely to be considered trustworthy.

Fear of Pain and Social Distance

As part of the same study, respondents completed instruments that measured fear of pain and empathy, and also provided demographic information, including migraine status. Overall, fear of pain was similar between migraineurs and nonmigraineurs. Migraineurs feared migraine as much as they feared falling down a flight of stairs or having a car door slammed on the hand. Nonmigraineurs, however, considered migraine to be less severe than migraineurs did. Among nonmigraineurs, greater fear of pain was associated with greater social distance from migraineurs. But in the same group, greater fear of migraine was associated with less social distance from migraineurs.

Furthermore, Dr. Shapiro’s group noted that the more migraine is part of a person’s experience, the less social distance that person maintains from migraineurs. Similarly, they found that as empathy increased, the social distance to migraine decreased.

Other findings included that younger people were more likely to stigmatize migraine than older people, and that non-Caucasians were more likely to stigmatize migraine than Caucasians. The gender of the stigmatizer was a dominant influence on the amount of stigma.

Reasons for Migraine Stigma

Various hypotheses offer potential reasons for stigmatizing migraine. Approximately 75% of migraineurs are women, and migraine changes with hormonal fluctuations. Hence, sexism against women may be one cause of stigma.

Also, migraine has been considered a behavior problem or conversion disorder for decades, said Dr. Shapiro. One illustration of this point is that a monograph published in 1926 identified migraine as a neurosis. In 1894, Freud described migraine as the result of a failure to find release after sexual stimulation.

Migraine is associated with headache, and headache has various connotations. The type of headache with which most people are familiar is tension-type headache, thus the general public may be likely to minimize the severity or importance of migraine. The word “headache” also connotes “concern” or “annoyance,” which may contribute to a minimization of migraine’s severity.

Whatever its origin, the stigma associated with migraine often is overlooked, said Dr. Shapiro. Neurologists should consider the potential effects of stigma on health outcomes as they treat patients with headache, he concluded.

—Erik Greb

Suggested Reading

Caspermeyer JJ, Sylvester EJ, Drazkowski JF, et al. Evaluation of stigmatizing language and medical errors in neurology coverage by US newspapers. Mayo Clin Proc. 2006;81(3):300-306.

Evans RW, Evans RE. A survey of neurologists on the likeability of headaches and other neurological disorders. Headache. 2010;50(7):1126-1129.

Evans RW, Evans RE, Kell HJ. A survey of family doctors on the likeability of migraine and other common diseases and their prevalence of migraine. Cephalalgia. 2010;30(5):620-623.

Young WB, Park JE, Tian IX, Kempner J. The stigma of migraine. PLoS One. 2013;8(1):e54074.

Stigma in Media and Society

Although neurologists and the general public acknowledge the symptoms and disability associated with a disease, they generally discount or fail to recognize its associated stigma, said Dr. Shapiro. Internalized stigma is a person’s sense that he or she is kept at a social distance, and enacted stigma refers to instances of discrimination on the basis of a person’s condition. Consequences of stigma include psychologic distress, low self-esteem, poor social outcomes, and poor health outcomes.

In a 2006 analysis, Caspermeyer et al examined 1,203 newspaper articles about neurologic conditions. They found that, after articles about epilepsy, articles about migraine contained the highest frequency of stigmatizing language (29%). Although migraine was among the most prevalent diseases in the analysis, it was among the least covered topics in newspaper articles.

To determine whether migraine is associated with stigma, Young and colleagues administered the Stigma Scale for Chronic Illness, a questionnaire, to patients with episodic migraine, chronic migraine, or epilepsy. The investigators observed that patients with chronic migraine and patients with epilepsy faced a similar amount of stigma. They further observed that stigma correlated most strongly with inability to work and was greater for chronic migraine than for epilepsy or episodic migraine.

Level of Stigma by Disease

Following these studies, Dr. Shapiro and colleagues investigated externalized stigma, or the rejection of others because of their condition. They polled adult members of the Amazon Mechanical Turk community, a group of approximately 500,000 people who voluntarily respond to surveys for modest monetary compensation. Compared with the US population, the Amazon Mechanical Turk community includes more women, Caucasians, young people, liberals, educated people, secular people, and people with low incomes. The group better represents the US population, however, than general Internet or university convenience samples do.

The investigators presented each respondent with one of four vignettes about people with a disease that does not respond well to treatment and that sometimes results in missed work or family activities. The vignettes were identical except for the condition named. The four conditions were migraine, epilepsy, panic disorder, and asthma. After reviewing the vignette, each respondent completed a validated stigma-assessment instrument using a Likert scale for each item.

In all, 765 people completed the instrument. Respondents’ mean age was 28, and 60% of the sample was female. There was no difference in the level of stigma attributed to migraine, panic disorder, or epilepsy, but respondents attributed less stigma to asthma than to the other three conditions. Noting the similar level of stigma between migraine and epilepsy, Dr. Shapiro said, “Epilepsy historically has been associated with demonic possession. If ever there were a disease that you would assume would have social distance or stigma attached, it certainly would be epilepsy.”

Disease and Productivity Loss

In a second study, Dr. Shapiro and colleagues presented members of the Amazon Mechanical Turk community with one of five vignettes. The vignettes described the following cases:

• A woman with migraine on four days per month who missed no workdays per year
• A woman with migraine on four days per month who missed two workdays per year
• A woman with migraine on 20 days per month who missed 10 workdays per year
• A man with migraine on four days per month who missed two workdays per year
• A woman with seizures on four days per month who missed two workdays per year.

Respondents then completed a social distance scale that included questions about the respondent’s willingness to socialize with, trust, hire, or allow the person in the vignette to marry into his or her family.

The researchers found that the level of externalized stigma was the same for the woman with migraine who missed two workdays per year as it was for the woman with epilepsy who missed two workdays per year. Respondents believed that the person with epilepsy would care more about whether he or she was a burden on others, compared with the migraineur. Respondents also said that the person with epilepsy would try harder and would be less likely to malinger, compared with the migraineur.

The gender of the person in the vignette was not associated with the level of stigma, said Dr. Shapiro. On the other hand, men were much more likely to stigmatize a man or a woman with migraine than women were.

In addition, the researchers found that the woman with chronic migraine who missed 10 workdays per year was much more likely to be stigmatized than migraineurs who missed fewer workdays. People who missed more workdays were less likely to be seen as trying hard, less likely to be interviewed, more likely to be considered malingerers, and less likely to be considered trustworthy.

Fear of Pain and Social Distance

As part of the same study, respondents completed instruments that measured fear of pain and empathy, and also provided demographic information, including migraine status. Overall, fear of pain was similar between migraineurs and nonmigraineurs. Migraineurs feared migraine as much as they feared falling down a flight of stairs or having a car door slammed on the hand. Nonmigraineurs, however, considered migraine to be less severe than migraineurs did. Among nonmigraineurs, greater fear of pain was associated with greater social distance from migraineurs. But in the same group, greater fear of migraine was associated with less social distance from migraineurs.

Furthermore, Dr. Shapiro’s group noted that the more migraine is part of a person’s experience, the less social distance that person maintains from migraineurs. Similarly, they found that as empathy increased, the social distance to migraine decreased.

Other findings included that younger people were more likely to stigmatize migraine than older people, and that non-Caucasians were more likely to stigmatize migraine than Caucasians. The gender of the stigmatizer was a dominant influence on the amount of stigma.

Reasons for Migraine Stigma

Various hypotheses offer potential reasons for stigmatizing migraine. Approximately 75% of migraineurs are women, and migraine changes with hormonal fluctuations. Hence, sexism against women may be one cause of stigma.

Also, migraine has been considered a behavior problem or conversion disorder for decades, said Dr. Shapiro. One illustration of this point is that a monograph published in 1926 identified migraine as a neurosis. In 1894, Freud described migraine as the result of a failure to find release after sexual stimulation.

Migraine is associated with headache, and headache has various connotations. The type of headache with which most people are familiar is tension-type headache, thus the general public may be likely to minimize the severity or importance of migraine. The word “headache” also connotes “concern” or “annoyance,” which may contribute to a minimization of migraine’s severity.

Whatever its origin, the stigma associated with migraine often is overlooked, said Dr. Shapiro. Neurologists should consider the potential effects of stigma on health outcomes as they treat patients with headache, he concluded.

—Erik Greb

Suggested Reading

Caspermeyer JJ, Sylvester EJ, Drazkowski JF, et al. Evaluation of stigmatizing language and medical errors in neurology coverage by US newspapers. Mayo Clin Proc. 2006;81(3):300-306.

Evans RW, Evans RE. A survey of neurologists on the likeability of headaches and other neurological disorders. Headache. 2010;50(7):1126-1129.

Evans RW, Evans RE, Kell HJ. A survey of family doctors on the likeability of migraine and other common diseases and their prevalence of migraine. Cephalalgia. 2010;30(5):620-623.

Young WB, Park JE, Tian IX, Kempner J. The stigma of migraine. PLoS One. 2013;8(1):e54074.

It’s the final day of the Vascular Annual Meeting – and there is plenty on the agenda to keep you busy.

6:30 to 8:00 a.m. – Three Breakfast Sessions: “Beyond the Basics of Hemodialysis Access,” “The Treatment of Chronic Venous Leg Ulcers,” and “How to Write a Paper and Have it Accepted in the JVS or EJVES.”

8:00 to 10:00 a.m. – Plenary 7 and Plenary 8. With so many high-quality abstracts submitted, two plenary sessions are set for Saturday; Plenary 8 includes late-breaking abstracts and the Vascular Quality Initiative. SDCC, Room 6 A/B

9:00 a.m. to 1:00 p.m. – It’s your last chance to visit the Exhibit Hall to see what’s new and improved. Exhibit Hall B.

10:30 a.m. to 12:00 p.m. – Four concurrent sessions, on the modern VA vascular practice and three joint sessions with an alphabet soup of allied societies, on medical management of vascular disease (SVM), collaboration of vascular surgeons and nurses (SVN), and building and managing a vascular laboratory (SVU).

10:30 a.m. to 12:00 p.m. – Top 10 papers relevant to vascular surgery that were NOT published in the Journal of Vascular Surgery. SDCC, Room 6 A/B.

12:00 p.m. – SVS members, see the passing of the gavel and other business issues, as well as award presentations. SDCC, Room 6 D/E.

1:30 to 3:30 p.m. – Sometimes seeing makes more of an impact than listening. The “How I do it” Video Session will feature 11 video abstracts on a variety of topics. SDCC, Room 6C.

1:30 to 5:00 p.m. – RPVI exam preparation, in collaboration with the Society for Vascular Ultrasound. SDCC, Room 4.

1:30 to 5:15 p.m. – Aortic Summit, with the Society of Thoracic Surgeons. SDCC, Room 3.

3:30 to 4:30 p.m. – Poster Runoff: Championship Round. From 120 posters to just 10, with three winners selected by the audience. SDCC, Room 6C.

It’s the final day of the Vascular Annual Meeting – and there is plenty on the agenda to keep you busy.

6:30 to 8:00 a.m. – Three Breakfast Sessions: “Beyond the Basics of Hemodialysis Access,” “The Treatment of Chronic Venous Leg Ulcers,” and “How to Write a Paper and Have it Accepted in the JVS or EJVES.”

8:00 to 10:00 a.m. – Plenary 7 and Plenary 8. With so many high-quality abstracts submitted, two plenary sessions are set for Saturday; Plenary 8 includes late-breaking abstracts and the Vascular Quality Initiative. SDCC, Room 6 A/B

9:00 a.m. to 1:00 p.m. – It’s your last chance to visit the Exhibit Hall to see what’s new and improved. Exhibit Hall B.

10:30 a.m. to 12:00 p.m. – Four concurrent sessions, on the modern VA vascular practice and three joint sessions with an alphabet soup of allied societies, on medical management of vascular disease (SVM), collaboration of vascular surgeons and nurses (SVN), and building and managing a vascular laboratory (SVU).

10:30 a.m. to 12:00 p.m. – Top 10 papers relevant to vascular surgery that were NOT published in the Journal of Vascular Surgery. SDCC, Room 6 A/B.

12:00 p.m. – SVS members, see the passing of the gavel and other business issues, as well as award presentations. SDCC, Room 6 D/E.

1:30 to 3:30 p.m. – Sometimes seeing makes more of an impact than listening. The “How I do it” Video Session will feature 11 video abstracts on a variety of topics. SDCC, Room 6C.

1:30 to 5:00 p.m. – RPVI exam preparation, in collaboration with the Society for Vascular Ultrasound. SDCC, Room 4.

1:30 to 5:15 p.m. – Aortic Summit, with the Society of Thoracic Surgeons. SDCC, Room 3.

3:30 to 4:30 p.m. – Poster Runoff: Championship Round. From 120 posters to just 10, with three winners selected by the audience. SDCC, Room 6C.

It’s the final day of the Vascular Annual Meeting – and there is plenty on the agenda to keep you busy.

6:30 to 8:00 a.m. – Three Breakfast Sessions: “Beyond the Basics of Hemodialysis Access,” “The Treatment of Chronic Venous Leg Ulcers,” and “How to Write a Paper and Have it Accepted in the JVS or EJVES.”

8:00 to 10:00 a.m. – Plenary 7 and Plenary 8. With so many high-quality abstracts submitted, two plenary sessions are set for Saturday; Plenary 8 includes late-breaking abstracts and the Vascular Quality Initiative. SDCC, Room 6 A/B

9:00 a.m. to 1:00 p.m. – It’s your last chance to visit the Exhibit Hall to see what’s new and improved. Exhibit Hall B.

10:30 a.m. to 12:00 p.m. – Four concurrent sessions, on the modern VA vascular practice and three joint sessions with an alphabet soup of allied societies, on medical management of vascular disease (SVM), collaboration of vascular surgeons and nurses (SVN), and building and managing a vascular laboratory (SVU).

10:30 a.m. to 12:00 p.m. – Top 10 papers relevant to vascular surgery that were NOT published in the Journal of Vascular Surgery. SDCC, Room 6 A/B.

12:00 p.m. – SVS members, see the passing of the gavel and other business issues, as well as award presentations. SDCC, Room 6 D/E.

1:30 to 3:30 p.m. – Sometimes seeing makes more of an impact than listening. The “How I do it” Video Session will feature 11 video abstracts on a variety of topics. SDCC, Room 6C.

1:30 to 5:00 p.m. – RPVI exam preparation, in collaboration with the Society for Vascular Ultrasound. SDCC, Room 4.

1:30 to 5:15 p.m. – Aortic Summit, with the Society of Thoracic Surgeons. SDCC, Room 3.

3:30 to 4:30 p.m. – Poster Runoff: Championship Round. From 120 posters to just 10, with three winners selected by the audience. SDCC, Room 6C.

Reader inquires about coding for McCall culdoplasty

It is difficult to know what CPT code to use for billing when my practice’s physicians do a McCall culdoplasty during a vaginal or laparoscopic hysterectomy. They often do a McCall procedure when a rectocele is present. One provider said it is CPT 57283. But I read an article that said a McCall repairs an “enterocele” and code 58263 would be used if doing one during a vaginal hysterectomy. Do you have a recommendation?

Sonia Pap, CPC, COBGC
Linville/Boone, North Carolina

Melanie Witt responds

Preventing vaginal vault prolapse by supporting the vaginal cuff is an essential part of hysterectomy, whether abdominal or vaginal. The McCall culdoplasty procedure is performed to support the vaginal cuff at the time of a vaginal hysterectomy by attaching the uterosacral and cardinal ligaments to the peritoneal surface with suture material such that, when tied, it draws toward the midline, helping to close off the cul-de-sac. This procedure not only supports the vaginal cuff but also closes off the cul-de-sac, thus preventing the formation of an enterocele.

As such it would be considered integral to the normal vaginal hysterectomy procedure and is not separately billable. However, in some cases where the patient has stage 1 to stage 4 uterovaginal prolapse, adjunct vaginal apex support is necessary. If the patient has this documented prior to the surgery, she will likely need more than the included uterosacral-cardinal ligament attachment to the vaginal membrane. This is where a colpopexy comes into play, and traditionally, sacrospinous fixation has been performed to accomplish this. In recent years, the uterosacral ligaments have been used instead, which is why we now have 2 codes for vaginal approach colpopexy: 57283 (uterosacral) and 57282 (sacrospinous). Both of these procedures will eliminate an existing enterocele and therefore could potentially be billed with a vaginal hysterectomy unless a more comprehensive code exits that describes the total surgery.

If the purpose of the colpopexy is to repair an existing enterocele, you would not itemize, but rather would report a vaginal hysterectomy with enterocele repair code (58263, 58270, 58292, or 58294) for that complete surgery. The codes do not specify the type of enterocele repair performed and so by definition would include “any method” including a colpopexy. You will note that the colpopexy codes 57283 and 57282 are bundled into all vaginal hysterectomy codes, and although you can use a modifier -59 to bypass this edit, you must meet the criteria for doing so. But especially, 57283 and 57282 are permanently bundled with the vaginal hysterectomy codes that include enterocele repair. Since there already exists a code that describes a vaginal hysterectomy with enterocele repair, you cannot report the modifier -59 for a separate colpopexy if the reason for doing it was to repair an enterocele. You could, however, use it if the sole reason was to do an adjunct vaginal vault repair due to documented uterovaginal prolapse.

Share your thoughts! Send your Letter to the Editor to [email protected]. Please include your name and the city and state in which you practice.

Reader inquires about coding for McCall culdoplasty

It is difficult to know what CPT code to use for billing when my practice’s physicians do a McCall culdoplasty during a vaginal or laparoscopic hysterectomy. They often do a McCall procedure when a rectocele is present. One provider said it is CPT 57283. But I read an article that said a McCall repairs an “enterocele” and code 58263 would be used if doing one during a vaginal hysterectomy. Do you have a recommendation?

Sonia Pap, CPC, COBGC
Linville/Boone, North Carolina

Melanie Witt responds

Preventing vaginal vault prolapse by supporting the vaginal cuff is an essential part of hysterectomy, whether abdominal or vaginal. The McCall culdoplasty procedure is performed to support the vaginal cuff at the time of a vaginal hysterectomy by attaching the uterosacral and cardinal ligaments to the peritoneal surface with suture material such that, when tied, it draws toward the midline, helping to close off the cul-de-sac. This procedure not only supports the vaginal cuff but also closes off the cul-de-sac, thus preventing the formation of an enterocele.

As such it would be considered integral to the normal vaginal hysterectomy procedure and is not separately billable. However, in some cases where the patient has stage 1 to stage 4 uterovaginal prolapse, adjunct vaginal apex support is necessary. If the patient has this documented prior to the surgery, she will likely need more than the included uterosacral-cardinal ligament attachment to the vaginal membrane. This is where a colpopexy comes into play, and traditionally, sacrospinous fixation has been performed to accomplish this. In recent years, the uterosacral ligaments have been used instead, which is why we now have 2 codes for vaginal approach colpopexy: 57283 (uterosacral) and 57282 (sacrospinous). Both of these procedures will eliminate an existing enterocele and therefore could potentially be billed with a vaginal hysterectomy unless a more comprehensive code exits that describes the total surgery.

If the purpose of the colpopexy is to repair an existing enterocele, you would not itemize, but rather would report a vaginal hysterectomy with enterocele repair code (58263, 58270, 58292, or 58294) for that complete surgery. The codes do not specify the type of enterocele repair performed and so by definition would include “any method” including a colpopexy. You will note that the colpopexy codes 57283 and 57282 are bundled into all vaginal hysterectomy codes, and although you can use a modifier -59 to bypass this edit, you must meet the criteria for doing so. But especially, 57283 and 57282 are permanently bundled with the vaginal hysterectomy codes that include enterocele repair. Since there already exists a code that describes a vaginal hysterectomy with enterocele repair, you cannot report the modifier -59 for a separate colpopexy if the reason for doing it was to repair an enterocele. You could, however, use it if the sole reason was to do an adjunct vaginal vault repair due to documented uterovaginal prolapse.

Share your thoughts! Send your Letter to the Editor to [email protected]. Please include your name and the city and state in which you practice.

Reader inquires about coding for McCall culdoplasty

It is difficult to know what CPT code to use for billing when my practice’s physicians do a McCall culdoplasty during a vaginal or laparoscopic hysterectomy. They often do a McCall procedure when a rectocele is present. One provider said it is CPT 57283. But I read an article that said a McCall repairs an “enterocele” and code 58263 would be used if doing one during a vaginal hysterectomy. Do you have a recommendation?

Sonia Pap, CPC, COBGC
Linville/Boone, North Carolina

Melanie Witt responds

Preventing vaginal vault prolapse by supporting the vaginal cuff is an essential part of hysterectomy, whether abdominal or vaginal. The McCall culdoplasty procedure is performed to support the vaginal cuff at the time of a vaginal hysterectomy by attaching the uterosacral and cardinal ligaments to the peritoneal surface with suture material such that, when tied, it draws toward the midline, helping to close off the cul-de-sac. This procedure not only supports the vaginal cuff but also closes off the cul-de-sac, thus preventing the formation of an enterocele.

As such it would be considered integral to the normal vaginal hysterectomy procedure and is not separately billable. However, in some cases where the patient has stage 1 to stage 4 uterovaginal prolapse, adjunct vaginal apex support is necessary. If the patient has this documented prior to the surgery, she will likely need more than the included uterosacral-cardinal ligament attachment to the vaginal membrane. This is where a colpopexy comes into play, and traditionally, sacrospinous fixation has been performed to accomplish this. In recent years, the uterosacral ligaments have been used instead, which is why we now have 2 codes for vaginal approach colpopexy: 57283 (uterosacral) and 57282 (sacrospinous). Both of these procedures will eliminate an existing enterocele and therefore could potentially be billed with a vaginal hysterectomy unless a more comprehensive code exits that describes the total surgery.

If the purpose of the colpopexy is to repair an existing enterocele, you would not itemize, but rather would report a vaginal hysterectomy with enterocele repair code (58263, 58270, 58292, or 58294) for that complete surgery. The codes do not specify the type of enterocele repair performed and so by definition would include “any method” including a colpopexy. You will note that the colpopexy codes 57283 and 57282 are bundled into all vaginal hysterectomy codes, and although you can use a modifier -59 to bypass this edit, you must meet the criteria for doing so. But especially, 57283 and 57282 are permanently bundled with the vaginal hysterectomy codes that include enterocele repair. Since there already exists a code that describes a vaginal hysterectomy with enterocele repair, you cannot report the modifier -59 for a separate colpopexy if the reason for doing it was to repair an enterocele. You could, however, use it if the sole reason was to do an adjunct vaginal vault repair due to documented uterovaginal prolapse.

Share your thoughts! Send your Letter to the Editor to [email protected]. Please include your name and the city and state in which you practice.

“MORE THAN ONE-THIRD OF TUMORS FOUND ON BREAST CANCER SCREENING REPRESENT OVERDIAGNOSIS”

ANDREW M. KAUNITZ, MD (MARCH 2017)

Refutes concept of overdiagnosis of breast cancer

I read with interest and serious concern the commentary and conclusions of “overdiagnosed” breast cancer. Let us revisit a few time-honored principles. Are we throwing away the valued concept of the early diagnosis of node-negative breast cancer? Is it still true that 5-year and long-term survivals are markedly better for stage I and II disease as opposed to stage III and IV disease? Is it still true that treatments designed for cure are substantially less involved, more successful, and more likely to conserve the breast and require less chemotherapy in early stage disease? Is it still true that the majority of women diagnosed with breast cancer are in the lowest risk category, ie, no family history and negative for the BRCA gene? If so, then who can explain the statement that “an invasive breast cancer detected by any means is overdiagnosis”? Would this imply that screening and the biopsy required to make the diagnosis was time poorly spent, the breast cancer should not be treated, and/or we should simply wait for a lump to be found by the patient deep in a large breast most likely at that point representing advanced disease?

The last paragraph notes the current US Preventive Services Task Force (USPSTF) guidance: wait until 50 years of age to start biennial screening. If so, what do we say to women in their 40s who, through screening with mammography and/or ultrasound, were diagnosed with early node-negative invasive breast cancer? That all of that was unnecessary and would not have led to symptoms? Would extreme morbidity from advanced or recurrent disease and the horrors of treatment just to extend a few months of life qualify as a symptom to these investigators? Lax protocols are not for me, my colleagues, or patients that I know. One of the most common reasons for a lawsuit to be brought against a primary care or ObGyn provider is failure to diagnose breast cancer!

John T. Armstrong, MD
Napa, California

Dr. Kaunitz responds

I thank Dr. Armstrong for his interest in my commentary on screening mammography and overdiagnosis. As I indicated in my commentary, I continue to recommend screening mammography for my patients, encouraging average-risk women to begin biennial screens at age 50 (consistent with USPSTF guidance), when the likelihood that tumors found with mammograms representing overdiagnosis is lower. I also indicated that I recognize that some patients prefer to begin screening at a younger age and to be screened more frequently. Dr. Armstrong’s letter refers to the “horrors of treatment” of breast cancer. From my perspective, the most “horrible” treatment is that which is administered to a woman diagnosed with a tumor destined to not cause clinical problems during her lifetime (overdiagnosis). You also refer to a statement, “an invasive breast cancer detected by any means is overdiagnosis.” That statement does not appear in my commentary.

My commentary’s point is that overdiagnosis is common among tumors diagnosed by screening mammography, and likely explains why, in contrast with cervical cancer screening, screening mammography has failed to reduce the incidence of breast cancers presenting as advanced (metastatic) disease. Although this represents a confusing and disquieting reality for our patients, and for us their clinicians, I agree with Dr. Otis Brawley, Chief Medical and Scientific Officer of the American Cancer Society, that we must acknowledge to our patients that overdiagnosis is common, the benefits of screening have been overstated, and that some patients considered as “cured” from breast cancer have in fact been harmed by unneeded treatment.¹

Share your thoughts!  Send your Letter to the Editor to [email protected]. Please include your name and the city and state in which you practice.

“MORE THAN ONE-THIRD OF TUMORS FOUND ON BREAST CANCER SCREENING REPRESENT OVERDIAGNOSIS”

ANDREW M. KAUNITZ, MD (MARCH 2017)

Refutes concept of overdiagnosis of breast cancer

I read with interest and serious concern the commentary and conclusions of “overdiagnosed” breast cancer. Let us revisit a few time-honored principles. Are we throwing away the valued concept of the early diagnosis of node-negative breast cancer? Is it still true that 5-year and long-term survivals are markedly better for stage I and II disease as opposed to stage III and IV disease? Is it still true that treatments designed for cure are substantially less involved, more successful, and more likely to conserve the breast and require less chemotherapy in early stage disease? Is it still true that the majority of women diagnosed with breast cancer are in the lowest risk category, ie, no family history and negative for the BRCA gene? If so, then who can explain the statement that “an invasive breast cancer detected by any means is overdiagnosis”? Would this imply that screening and the biopsy required to make the diagnosis was time poorly spent, the breast cancer should not be treated, and/or we should simply wait for a lump to be found by the patient deep in a large breast most likely at that point representing advanced disease?

The last paragraph notes the current US Preventive Services Task Force (USPSTF) guidance: wait until 50 years of age to start biennial screening. If so, what do we say to women in their 40s who, through screening with mammography and/or ultrasound, were diagnosed with early node-negative invasive breast cancer? That all of that was unnecessary and would not have led to symptoms? Would extreme morbidity from advanced or recurrent disease and the horrors of treatment just to extend a few months of life qualify as a symptom to these investigators? Lax protocols are not for me, my colleagues, or patients that I know. One of the most common reasons for a lawsuit to be brought against a primary care or ObGyn provider is failure to diagnose breast cancer!

John T. Armstrong, MD
Napa, California

Dr. Kaunitz responds

I thank Dr. Armstrong for his interest in my commentary on screening mammography and overdiagnosis. As I indicated in my commentary, I continue to recommend screening mammography for my patients, encouraging average-risk women to begin biennial screens at age 50 (consistent with USPSTF guidance), when the likelihood that tumors found with mammograms representing overdiagnosis is lower. I also indicated that I recognize that some patients prefer to begin screening at a younger age and to be screened more frequently. Dr. Armstrong’s letter refers to the “horrors of treatment” of breast cancer. From my perspective, the most “horrible” treatment is that which is administered to a woman diagnosed with a tumor destined to not cause clinical problems during her lifetime (overdiagnosis). You also refer to a statement, “an invasive breast cancer detected by any means is overdiagnosis.” That statement does not appear in my commentary.

My commentary’s point is that overdiagnosis is common among tumors diagnosed by screening mammography, and likely explains why, in contrast with cervical cancer screening, screening mammography has failed to reduce the incidence of breast cancers presenting as advanced (metastatic) disease. Although this represents a confusing and disquieting reality for our patients, and for us their clinicians, I agree with Dr. Otis Brawley, Chief Medical and Scientific Officer of the American Cancer Society, that we must acknowledge to our patients that overdiagnosis is common, the benefits of screening have been overstated, and that some patients considered as “cured” from breast cancer have in fact been harmed by unneeded treatment.¹

Share your thoughts!  Send your Letter to the Editor to [email protected]. Please include your name and the city and state in which you practice.

“MORE THAN ONE-THIRD OF TUMORS FOUND ON BREAST CANCER SCREENING REPRESENT OVERDIAGNOSIS”

ANDREW M. KAUNITZ, MD (MARCH 2017)

Refutes concept of overdiagnosis of breast cancer

I read with interest and serious concern the commentary and conclusions of “overdiagnosed” breast cancer. Let us revisit a few time-honored principles. Are we throwing away the valued concept of the early diagnosis of node-negative breast cancer? Is it still true that 5-year and long-term survivals are markedly better for stage I and II disease as opposed to stage III and IV disease? Is it still true that treatments designed for cure are substantially less involved, more successful, and more likely to conserve the breast and require less chemotherapy in early stage disease? Is it still true that the majority of women diagnosed with breast cancer are in the lowest risk category, ie, no family history and negative for the BRCA gene? If so, then who can explain the statement that “an invasive breast cancer detected by any means is overdiagnosis”? Would this imply that screening and the biopsy required to make the diagnosis was time poorly spent, the breast cancer should not be treated, and/or we should simply wait for a lump to be found by the patient deep in a large breast most likely at that point representing advanced disease?

The last paragraph notes the current US Preventive Services Task Force (USPSTF) guidance: wait until 50 years of age to start biennial screening. If so, what do we say to women in their 40s who, through screening with mammography and/or ultrasound, were diagnosed with early node-negative invasive breast cancer? That all of that was unnecessary and would not have led to symptoms? Would extreme morbidity from advanced or recurrent disease and the horrors of treatment just to extend a few months of life qualify as a symptom to these investigators? Lax protocols are not for me, my colleagues, or patients that I know. One of the most common reasons for a lawsuit to be brought against a primary care or ObGyn provider is failure to diagnose breast cancer!

John T. Armstrong, MD
Napa, California

Dr. Kaunitz responds

I thank Dr. Armstrong for his interest in my commentary on screening mammography and overdiagnosis. As I indicated in my commentary, I continue to recommend screening mammography for my patients, encouraging average-risk women to begin biennial screens at age 50 (consistent with USPSTF guidance), when the likelihood that tumors found with mammograms representing overdiagnosis is lower. I also indicated that I recognize that some patients prefer to begin screening at a younger age and to be screened more frequently. Dr. Armstrong’s letter refers to the “horrors of treatment” of breast cancer. From my perspective, the most “horrible” treatment is that which is administered to a woman diagnosed with a tumor destined to not cause clinical problems during her lifetime (overdiagnosis). You also refer to a statement, “an invasive breast cancer detected by any means is overdiagnosis.” That statement does not appear in my commentary.

My commentary’s point is that overdiagnosis is common among tumors diagnosed by screening mammography, and likely explains why, in contrast with cervical cancer screening, screening mammography has failed to reduce the incidence of breast cancers presenting as advanced (metastatic) disease. Although this represents a confusing and disquieting reality for our patients, and for us their clinicians, I agree with Dr. Otis Brawley, Chief Medical and Scientific Officer of the American Cancer Society, that we must acknowledge to our patients that overdiagnosis is common, the benefits of screening have been overstated, and that some patients considered as “cured” from breast cancer have in fact been harmed by unneeded treatment.¹

Share your thoughts!  Send your Letter to the Editor to [email protected]. Please include your name and the city and state in which you practice.

Though the Society for Vascular Surgery has long published clinical practice guidelines, its 2017 annual meeting marks the first seminar dedicated to guidelines in progress, allowing for more interaction, debate, and feedback across the vascular community before these guidelines become final.

At Friday afternoon’s update on SVS clinical practice guidelines, speakers will present on four that are in advanced development, including new global vascular guidelines.

Friday, June 2

3:30 p.m. - 5:00 p.m., SDCC, Room 4

C9: Update on Society for Vascular Surgery Clinical Practice Guidelines

Though the Society for Vascular Surgery has long published clinical practice guidelines, its 2017 annual meeting marks the first seminar dedicated to guidelines in progress, allowing for more interaction, debate, and feedback across the vascular community before these guidelines become final.

At Friday afternoon’s update on SVS clinical practice guidelines, speakers will present on four that are in advanced development, including new global vascular guidelines.

Friday, June 2

3:30 p.m. - 5:00 p.m., SDCC, Room 4

C9: Update on Society for Vascular Surgery Clinical Practice Guidelines

Though the Society for Vascular Surgery has long published clinical practice guidelines, its 2017 annual meeting marks the first seminar dedicated to guidelines in progress, allowing for more interaction, debate, and feedback across the vascular community before these guidelines become final.

At Friday afternoon’s update on SVS clinical practice guidelines, speakers will present on four that are in advanced development, including new global vascular guidelines.

Friday, June 2

3:30 p.m. - 5:00 p.m., SDCC, Room 4

C9: Update on Society for Vascular Surgery Clinical Practice Guidelines

Over the past year, the Journal of Vascular Surgery and its two affiliated journals have undergone a number of important changes.

Editorial boards have been revamped, disadvantageous limits on the number of contributing authors and on references have been scrapped, and editors’ videos accompany every new issue. New short abstracts, take-home messages, and extended tables of contents allow even the most time-crunched clinicians to get up to speed.

Even the look of the journals has changed – with a red color scheme for JVS, a blue one for JVS-VL, the venous and lymphatic disorders journal, and a mixed red and blue one for the open-access publication dedicated to cases and techniques.

Saturday, June 3

6:30 a.m. - 8:00 a.m.

Breakfast Session B9: How to Write a Paper and Have it Accepted in the JVS or EJVES

Over the past year, the Journal of Vascular Surgery and its two affiliated journals have undergone a number of important changes.

Editorial boards have been revamped, disadvantageous limits on the number of contributing authors and on references have been scrapped, and editors’ videos accompany every new issue. New short abstracts, take-home messages, and extended tables of contents allow even the most time-crunched clinicians to get up to speed.

Even the look of the journals has changed – with a red color scheme for JVS, a blue one for JVS-VL, the venous and lymphatic disorders journal, and a mixed red and blue one for the open-access publication dedicated to cases and techniques.

Saturday, June 3

6:30 a.m. - 8:00 a.m.

Breakfast Session B9: How to Write a Paper and Have it Accepted in the JVS or EJVES

Over the past year, the Journal of Vascular Surgery and its two affiliated journals have undergone a number of important changes.

Editorial boards have been revamped, disadvantageous limits on the number of contributing authors and on references have been scrapped, and editors’ videos accompany every new issue. New short abstracts, take-home messages, and extended tables of contents allow even the most time-crunched clinicians to get up to speed.

Even the look of the journals has changed – with a red color scheme for JVS, a blue one for JVS-VL, the venous and lymphatic disorders journal, and a mixed red and blue one for the open-access publication dedicated to cases and techniques.

Saturday, June 3

6:30 a.m. - 8:00 a.m.

Breakfast Session B9: How to Write a Paper and Have it Accepted in the JVS or EJVES

Prescribing rheumatoid arthritis patients a biologic agent rather than triple therapy, when they fail to respond adequately to methotrexate, costs more than half a million dollars per quality-adjusted life year gained over a lifetime but provides only a minimal health benefit beyond triple therapy, according to a report published online May 30 in Annals of Internal Medicine.

The evidence is clear that triple therapy using sulfasalazine, hydroxychloroquine, and methotrexate is at least as effective as methotrexate plus an anti–tumor necrosis factor biologic (etanercept, adalimumab, infliximab, golimumab, or certolizumab pegol). Nevertheless, few RA patients are transitioned to triple therapy before escalating to biologics. This occurred in only 2.5% of patients in one Veterans Affairs study, said Nick Bansback, PhD, of the University of British Columbia and St. Paul’s Hospital, Vancouver, and his associates.

Dr. Bansback and his colleagues performed a cost-effectiveness study of proceeding directly to etanercept when RA fails to respond to methotrexate, using data from the Rheumatoid Arthritis Comparison of Active Therapies (RACAT) trial. The RACAT study, a large international, randomized, double-blind trial, confirmed the clinical noninferiority of triple therapy versus etanercept plus methotrexate. In their analysis, Dr. Bansback and his associates calculated the costs and QALYs for both treatment strategies for 324 RACAT participants.

Switching directly to etanercept-methotrexate instead of triple therapy provided only a marginal advantage in QALYs, a difference of only 0.004 QALY over 24 weeks of treatment (0.358 with etanercept-methotrexate vs. 0.353 with triple therapy) and of only 0.016 QALY over 48 weeks of treatment (0.743 with etanercept-methotrexate vs. 0.726 with triple therapy). The associated costs were $11,295 for 24 weeks of etanercept-methotrexate, vs. $343 for 24 weeks of triple therapy, and $19,634 for 48 weeks of etanercept-methotrexate, vs. $3,680 for 48 weeks of triple therapy.

“The resultant ICER [incremental cost-effectiveness ratio] for first-line etanercept-methotrexate, vs. triple therapy, was $2.7 million per QALY gained over 24 weeks,” the investigators said (Ann Intern Med. 2017 May 29. doi: 10.7326/M16-0713).

When they extrapolated the data to determine the cost-effectiveness of the two treatment strategies over the course of the average patient’s lifetime, the model predicted an ICER of $521,520 per QALY gained for etanercept-methotrexate instead of triple therapy. These findings remained robust in numerous sensitivity and scenario analyses. For example, when the model assumed that radiographic and quality of life benefits of triple therapy were far lower than observed in the RACAT trial and far lower than what has been reported in the literature and that the tolerability of triple therapy was far worse than observed in the RACAT trial and reported in the literature, switching to etanercept-methotrexate instead of triple therapy still predicted an ICER of $350,000 per QALY per patient.

All of these ICERs far exceed the standard cost-effectiveness acceptability threshold ICER of $100,000 per QALY, Dr. Bansback and his associates noted.

This study demonstrates the substantial cost savings of prescribing triple therapy before a biologic. It shows that “for every patient who tries triple therapy before a biologic, payers will save an average of $78,000 over the patient’s lifetime, and most of the savings will accrue within the first 10 years,” the investigators wrote.

“Patients who receive triple therapy before a biologic will miss out on a benefit of approximately 0.15 QALY over their lifetime or a benefit of approximately 0.05 HAQ [Health Assessment Questionnaire] point at any point in time. To put these numbers in perspective, total hip arthroplasty in a patient with osteoarthritis who is approximately the same age as an average patient with RA provides an additional 6.9 QALYs. In terms of HAQ score, only differences greater than 0.2 points are considered minimally important to patients,” Dr. Bansback and his associates wrote.

Changing health care policy to require, rather than to just recommend, that triple therapy be prescribed before biologics would save millions of dollars in health care expenditures, they added.

This study was supported by the U.S. Department of Veterans Affairs Office of Research and Development, the Canadian Institutes for Health Research, the National Institutes of Health, and the American Recovery and Reinvestment Act. Dr. Bansback reported having no relevant financial disclosures. His associates reported ties to numerous industry sources.
 

[email protected]

Prescribing rheumatoid arthritis patients a biologic agent rather than triple therapy, when they fail to respond adequately to methotrexate, costs more than half a million dollars per quality-adjusted life year gained over a lifetime but provides only a minimal health benefit beyond triple therapy, according to a report published online May 30 in Annals of Internal Medicine.

The evidence is clear that triple therapy using sulfasalazine, hydroxychloroquine, and methotrexate is at least as effective as methotrexate plus an anti–tumor necrosis factor biologic (etanercept, adalimumab, infliximab, golimumab, or certolizumab pegol). Nevertheless, few RA patients are transitioned to triple therapy before escalating to biologics. This occurred in only 2.5% of patients in one Veterans Affairs study, said Nick Bansback, PhD, of the University of British Columbia and St. Paul’s Hospital, Vancouver, and his associates.

Dr. Bansback and his colleagues performed a cost-effectiveness study of proceeding directly to etanercept when RA fails to respond to methotrexate, using data from the Rheumatoid Arthritis Comparison of Active Therapies (RACAT) trial. The RACAT study, a large international, randomized, double-blind trial, confirmed the clinical noninferiority of triple therapy versus etanercept plus methotrexate. In their analysis, Dr. Bansback and his associates calculated the costs and QALYs for both treatment strategies for 324 RACAT participants.

Switching directly to etanercept-methotrexate instead of triple therapy provided only a marginal advantage in QALYs, a difference of only 0.004 QALY over 24 weeks of treatment (0.358 with etanercept-methotrexate vs. 0.353 with triple therapy) and of only 0.016 QALY over 48 weeks of treatment (0.743 with etanercept-methotrexate vs. 0.726 with triple therapy). The associated costs were $11,295 for 24 weeks of etanercept-methotrexate, vs. $343 for 24 weeks of triple therapy, and $19,634 for 48 weeks of etanercept-methotrexate, vs. $3,680 for 48 weeks of triple therapy.

“The resultant ICER [incremental cost-effectiveness ratio] for first-line etanercept-methotrexate, vs. triple therapy, was $2.7 million per QALY gained over 24 weeks,” the investigators said (Ann Intern Med. 2017 May 29. doi: 10.7326/M16-0713).

When they extrapolated the data to determine the cost-effectiveness of the two treatment strategies over the course of the average patient’s lifetime, the model predicted an ICER of $521,520 per QALY gained for etanercept-methotrexate instead of triple therapy. These findings remained robust in numerous sensitivity and scenario analyses. For example, when the model assumed that radiographic and quality of life benefits of triple therapy were far lower than observed in the RACAT trial and far lower than what has been reported in the literature and that the tolerability of triple therapy was far worse than observed in the RACAT trial and reported in the literature, switching to etanercept-methotrexate instead of triple therapy still predicted an ICER of $350,000 per QALY per patient.

All of these ICERs far exceed the standard cost-effectiveness acceptability threshold ICER of $100,000 per QALY, Dr. Bansback and his associates noted.

This study demonstrates the substantial cost savings of prescribing triple therapy before a biologic. It shows that “for every patient who tries triple therapy before a biologic, payers will save an average of $78,000 over the patient’s lifetime, and most of the savings will accrue within the first 10 years,” the investigators wrote.

“Patients who receive triple therapy before a biologic will miss out on a benefit of approximately 0.15 QALY over their lifetime or a benefit of approximately 0.05 HAQ [Health Assessment Questionnaire] point at any point in time. To put these numbers in perspective, total hip arthroplasty in a patient with osteoarthritis who is approximately the same age as an average patient with RA provides an additional 6.9 QALYs. In terms of HAQ score, only differences greater than 0.2 points are considered minimally important to patients,” Dr. Bansback and his associates wrote.

Changing health care policy to require, rather than to just recommend, that triple therapy be prescribed before biologics would save millions of dollars in health care expenditures, they added.

This study was supported by the U.S. Department of Veterans Affairs Office of Research and Development, the Canadian Institutes for Health Research, the National Institutes of Health, and the American Recovery and Reinvestment Act. Dr. Bansback reported having no relevant financial disclosures. His associates reported ties to numerous industry sources.
 

[email protected]

Prescribing rheumatoid arthritis patients a biologic agent rather than triple therapy, when they fail to respond adequately to methotrexate, costs more than half a million dollars per quality-adjusted life year gained over a lifetime but provides only a minimal health benefit beyond triple therapy, according to a report published online May 30 in Annals of Internal Medicine.

The evidence is clear that triple therapy using sulfasalazine, hydroxychloroquine, and methotrexate is at least as effective as methotrexate plus an anti–tumor necrosis factor biologic (etanercept, adalimumab, infliximab, golimumab, or certolizumab pegol). Nevertheless, few RA patients are transitioned to triple therapy before escalating to biologics. This occurred in only 2.5% of patients in one Veterans Affairs study, said Nick Bansback, PhD, of the University of British Columbia and St. Paul’s Hospital, Vancouver, and his associates.

Dr. Bansback and his colleagues performed a cost-effectiveness study of proceeding directly to etanercept when RA fails to respond to methotrexate, using data from the Rheumatoid Arthritis Comparison of Active Therapies (RACAT) trial. The RACAT study, a large international, randomized, double-blind trial, confirmed the clinical noninferiority of triple therapy versus etanercept plus methotrexate. In their analysis, Dr. Bansback and his associates calculated the costs and QALYs for both treatment strategies for 324 RACAT participants.

Switching directly to etanercept-methotrexate instead of triple therapy provided only a marginal advantage in QALYs, a difference of only 0.004 QALY over 24 weeks of treatment (0.358 with etanercept-methotrexate vs. 0.353 with triple therapy) and of only 0.016 QALY over 48 weeks of treatment (0.743 with etanercept-methotrexate vs. 0.726 with triple therapy). The associated costs were $11,295 for 24 weeks of etanercept-methotrexate, vs. $343 for 24 weeks of triple therapy, and $19,634 for 48 weeks of etanercept-methotrexate, vs. $3,680 for 48 weeks of triple therapy.

“The resultant ICER [incremental cost-effectiveness ratio] for first-line etanercept-methotrexate, vs. triple therapy, was $2.7 million per QALY gained over 24 weeks,” the investigators said (Ann Intern Med. 2017 May 29. doi: 10.7326/M16-0713).

When they extrapolated the data to determine the cost-effectiveness of the two treatment strategies over the course of the average patient’s lifetime, the model predicted an ICER of $521,520 per QALY gained for etanercept-methotrexate instead of triple therapy. These findings remained robust in numerous sensitivity and scenario analyses. For example, when the model assumed that radiographic and quality of life benefits of triple therapy were far lower than observed in the RACAT trial and far lower than what has been reported in the literature and that the tolerability of triple therapy was far worse than observed in the RACAT trial and reported in the literature, switching to etanercept-methotrexate instead of triple therapy still predicted an ICER of $350,000 per QALY per patient.

All of these ICERs far exceed the standard cost-effectiveness acceptability threshold ICER of $100,000 per QALY, Dr. Bansback and his associates noted.

This study demonstrates the substantial cost savings of prescribing triple therapy before a biologic. It shows that “for every patient who tries triple therapy before a biologic, payers will save an average of $78,000 over the patient’s lifetime, and most of the savings will accrue within the first 10 years,” the investigators wrote.

“Patients who receive triple therapy before a biologic will miss out on a benefit of approximately 0.15 QALY over their lifetime or a benefit of approximately 0.05 HAQ [Health Assessment Questionnaire] point at any point in time. To put these numbers in perspective, total hip arthroplasty in a patient with osteoarthritis who is approximately the same age as an average patient with RA provides an additional 6.9 QALYs. In terms of HAQ score, only differences greater than 0.2 points are considered minimally important to patients,” Dr. Bansback and his associates wrote.

Changing health care policy to require, rather than to just recommend, that triple therapy be prescribed before biologics would save millions of dollars in health care expenditures, they added.

This study was supported by the U.S. Department of Veterans Affairs Office of Research and Development, the Canadian Institutes for Health Research, the National Institutes of Health, and the American Recovery and Reinvestment Act. Dr. Bansback reported having no relevant financial disclosures. His associates reported ties to numerous industry sources.
 

[email protected]

Tafamidis, an oral non-NSAID and highly specific stabilizer of the TTR protein, delays neurologic progression in early-stage transthyretin familial amyloid polyneuropathy (ATTR-FAP), according to research published online ahead of print April 10 in Amyloid.

ATTR-FAP is a progressive condition caused by mutations in the TTR gene that destabilize the TTR protein, thereby facilitating misfolding and aggregation. The neuropathy may be accompanied by cardiac, gastrointestinal, renal, or ocular symptoms, and death occurs at an average of 10 years after symptom onset.

A pivotal phase III trial compared tafamidis with placebo over 18 months in 128 patients with early-stage ATTR-FAP. Researchers observed a trend toward a smaller increase in Neuropathy Impairment Score for Lower Limbs (NIS-LL) with tafamidis, compared with placebo, but the result was not statistically significant. Baseline NIS-LL values were higher among controls than among actively treated patients, however, and many participants dropped out of the study for liver transplantation.

In a post hoc analysis, the investigators took these factors into account and reanalyzed change from baseline in NIS-LL in the trial. They also analyzed change in NIS-LL plus three small-fiber nerve tests (NIS-LL+Σ3) and NIS-LL plus seven nerve tests (NIS-LL+Σ7) without baseline measurements as covariates.

The analysis indicated a significant benefit of tafamidis, compared with placebo, at Months 12 and 18. The baseline-adjusted mean increase in NIS-LL from baseline to Month 12 was 1.5 points in the tafamidis group versus 4.5 points in the placebo group. By Month 18, the mean change from baseline was 2.9 points in the tafamidis group versus 5.6 points in the placebo group.

When the researchers applied sensitivity analysis based on multiple imputation for missing data by treatment group, the mean estimates of the increase in NIS-LL total score from baseline to Month 18 were significantly lower for tafamidis, compared with placebo. A more conservative model based on multiple imputation showed a benefit of tafamidis, but results did not reach statistical significance.

The researchers also found a statistically significant benefit of tafamidis, compared with placebo, on NIS-LL+Σ3 at Months 12 and 18. Significant differences favoring tafamidis also were observed on NIS-LL+Σ7 at Months 12 and 18. Four of the study’s five authors are employees of Pfizer, which owns tafamidis and sponsored the study.

—Erik Greb

Suggested Reading

Keohane D, Schwartz J, Gundapaneni B, et al. Tafamidis delays disease progression in patients with early stage transthyretin familial amyloid polyneuropathy: additional supportive analyses from the pivotal trial. Amyloid. 2017 Apr 10 [Epub ahead of print].

Tafamidis, an oral non-NSAID and highly specific stabilizer of the TTR protein, delays neurologic progression in early-stage transthyretin familial amyloid polyneuropathy (ATTR-FAP), according to research published online ahead of print April 10 in Amyloid.

ATTR-FAP is a progressive condition caused by mutations in the TTR gene that destabilize the TTR protein, thereby facilitating misfolding and aggregation. The neuropathy may be accompanied by cardiac, gastrointestinal, renal, or ocular symptoms, and death occurs at an average of 10 years after symptom onset.

A pivotal phase III trial compared tafamidis with placebo over 18 months in 128 patients with early-stage ATTR-FAP. Researchers observed a trend toward a smaller increase in Neuropathy Impairment Score for Lower Limbs (NIS-LL) with tafamidis, compared with placebo, but the result was not statistically significant. Baseline NIS-LL values were higher among controls than among actively treated patients, however, and many participants dropped out of the study for liver transplantation.

In a post hoc analysis, the investigators took these factors into account and reanalyzed change from baseline in NIS-LL in the trial. They also analyzed change in NIS-LL plus three small-fiber nerve tests (NIS-LL+Σ3) and NIS-LL plus seven nerve tests (NIS-LL+Σ7) without baseline measurements as covariates.

The analysis indicated a significant benefit of tafamidis, compared with placebo, at Months 12 and 18. The baseline-adjusted mean increase in NIS-LL from baseline to Month 12 was 1.5 points in the tafamidis group versus 4.5 points in the placebo group. By Month 18, the mean change from baseline was 2.9 points in the tafamidis group versus 5.6 points in the placebo group.

When the researchers applied sensitivity analysis based on multiple imputation for missing data by treatment group, the mean estimates of the increase in NIS-LL total score from baseline to Month 18 were significantly lower for tafamidis, compared with placebo. A more conservative model based on multiple imputation showed a benefit of tafamidis, but results did not reach statistical significance.

The researchers also found a statistically significant benefit of tafamidis, compared with placebo, on NIS-LL+Σ3 at Months 12 and 18. Significant differences favoring tafamidis also were observed on NIS-LL+Σ7 at Months 12 and 18. Four of the study’s five authors are employees of Pfizer, which owns tafamidis and sponsored the study.

—Erik Greb

Suggested Reading

Keohane D, Schwartz J, Gundapaneni B, et al. Tafamidis delays disease progression in patients with early stage transthyretin familial amyloid polyneuropathy: additional supportive analyses from the pivotal trial. Amyloid. 2017 Apr 10 [Epub ahead of print].

Tafamidis, an oral non-NSAID and highly specific stabilizer of the TTR protein, delays neurologic progression in early-stage transthyretin familial amyloid polyneuropathy (ATTR-FAP), according to research published online ahead of print April 10 in Amyloid.

ATTR-FAP is a progressive condition caused by mutations in the TTR gene that destabilize the TTR protein, thereby facilitating misfolding and aggregation. The neuropathy may be accompanied by cardiac, gastrointestinal, renal, or ocular symptoms, and death occurs at an average of 10 years after symptom onset.

A pivotal phase III trial compared tafamidis with placebo over 18 months in 128 patients with early-stage ATTR-FAP. Researchers observed a trend toward a smaller increase in Neuropathy Impairment Score for Lower Limbs (NIS-LL) with tafamidis, compared with placebo, but the result was not statistically significant. Baseline NIS-LL values were higher among controls than among actively treated patients, however, and many participants dropped out of the study for liver transplantation.

In a post hoc analysis, the investigators took these factors into account and reanalyzed change from baseline in NIS-LL in the trial. They also analyzed change in NIS-LL plus three small-fiber nerve tests (NIS-LL+Σ3) and NIS-LL plus seven nerve tests (NIS-LL+Σ7) without baseline measurements as covariates.

The analysis indicated a significant benefit of tafamidis, compared with placebo, at Months 12 and 18. The baseline-adjusted mean increase in NIS-LL from baseline to Month 12 was 1.5 points in the tafamidis group versus 4.5 points in the placebo group. By Month 18, the mean change from baseline was 2.9 points in the tafamidis group versus 5.6 points in the placebo group.

When the researchers applied sensitivity analysis based on multiple imputation for missing data by treatment group, the mean estimates of the increase in NIS-LL total score from baseline to Month 18 were significantly lower for tafamidis, compared with placebo. A more conservative model based on multiple imputation showed a benefit of tafamidis, but results did not reach statistical significance.

The researchers also found a statistically significant benefit of tafamidis, compared with placebo, on NIS-LL+Σ3 at Months 12 and 18. Significant differences favoring tafamidis also were observed on NIS-LL+Σ7 at Months 12 and 18. Four of the study’s five authors are employees of Pfizer, which owns tafamidis and sponsored the study.

—Erik Greb

Suggested Reading

Keohane D, Schwartz J, Gundapaneni B, et al. Tafamidis delays disease progression in patients with early stage transthyretin familial amyloid polyneuropathy: additional supportive analyses from the pivotal trial. Amyloid. 2017 Apr 10 [Epub ahead of print].