THE CASE

A 38-year-old Hispanic man was brought to the emergency department (ED) after losing consciousness and falling at home, striking his elbow, head, and neck. For the past week, he’d had palpitations, shortness of breath, mild swelling of the lower extremities, fever, nausea, and fatigue. He had also been experiencing squeezing chest pain that worsened with exertion and was only partially relieved by nitroglycerin.

The patient did not have any rashes and denied having contact with anyone who was sick. He said that he’d been bitten by mosquitos during recent outdoor activities. His medical history included hypertension, hemorrhagic basal ganglia stroke, hyperlipidemia, sleep apnea, metabolic syndrome, and gout. The patient denied smoking or using illicit drugs.

In the ED, his temperature was 101°F, heart rate was 112 beats/min, blood pressure was 175/100 mm Hg, and respiratory rate was 18 breaths/min. His head and neck exam was normal, with no neck stiffness. A lung exam revealed bilateral basal crackles, and a neurologic exam showed residual right-sided weakness due to the hemorrhagic stroke one year ago.

Lab test results revealed the following: white blood cell (WBC) count, 13,000/mm³ with relative monocytosis (14%); lymphocytosis (44%) with normal neutrophils and no bands; hemoglobin, 12 g/dL; hematocrit, 36/mm³; and platelets, 300,000/mm³. Liver function tests were within normal limits. Urinalysis was unremarkable. His troponin I level was elevated at 1.385 ng/dL. In addition to the tachycardia, his electrocardiogram (EKG) showed left axis deviation, left atrial enlargement, left anterior fascicular block, and diffuse nonspecific ST and T wave abnormalities. Chest x-ray was unremarkable except for cardiomegaly. A computed tomography (CT) scan of his head showed residual changes from the previous stroke.

The patient was admitted with a provisional diagnosis of systemic inflammatory response syndrome (SIRS), syncope, non–ST elevation myocardial infarction (NSTEMI), and acute heart failure. The patient had continuous EKG monitoring and serial assessments of his troponin levels. He was also given aspirin, metoprolol 25 mg BID, lisinopril 10 mg/d, furosemide 40 mg IV, isosorbide mononitrate 60 mg/d, and atorvastatin 40 mg/d.

The patient’s cardiac enzymes subsequently decreased. A left heart catheterization was performed, which showed minimum irregularities of the left anterior descending artery (< 20% narrowing) and an ejection fraction (EF) of 35%, without any evidence of obstructive coronary artery disease (CAD). An echocardiogram revealed systolic dysfunction, with an EF of 35% to 40% and global hypokinesis without any apical ballooning or pericardial effusion. (An echocardiogram performed 6 months earlier had shown normal systolic function, an EF of 60% to 65%, and no wall motion abnormalities.) Blood, urine, and fungal cultures were negative; stool studies for ova and parasites were also negative. A lower extremity venous Doppler was negative for deep vein thrombosis.

THE DIAGNOSIS

Because our patient had SIRS, troponinemia, acute systolic dysfunction, and global hypokinesis without any evidence of obstructive CAD, we considered a diagnosis of viral myocarditis. Serologic studies for echovirus, coxsackievirus B, parvovirus B19, adenovirus, and human herpesvirus 6 (HHV-6) all came back negative. However, an enzyme-linked immunosorbent assay (ELISA) for West Nile virus (WNV) was positive. WNV infection was confirmed with a positive plaque reduction neutralization test and a positive qualitative polymerase chain reaction (PCR) assay, which established a diagnosis of WNV myocarditis.

DISCUSSION

While most individuals infected with WNV are asymptomatic, 20% to 40% of patients will exhibit symptoms.^1-4 Typical presentations of WNV infection include West Nile fever and neuroinvasive disease. West Nile fever is a self-limited illness characterized by a low-grade fever, headache, malaise, back pain, myalgia, and anorexia for 3 to 6 days.² Neuroinvasive disease caused by WNV may present as encephalitis, meningitis, or flaccid paralysis.⁵ Atypical presentations of the virus include rhabdomyolysis,⁶ fatal hemorrhagic fever with multi-organ failure and palpable purpura,⁷ hepatitis,⁸ pancreatitis,⁹ central diabetes insipidus,¹⁰ and myocarditis.¹¹

Although WNV has been linked to myocarditismin animals,¹² few human cases of WNV myocarditis^11,13 or cardiomyopathy¹⁴ have been reported. Viral myocarditis often leads to the development of dilated cardiomyopathy, and myocardial damage may result from direct virus-induced cytotoxicity, T cell-mediated immune response to the virus, or apoptosis.¹⁵ Some research suggests that immune-mediated mechanisms play a primary role in myocardial damage. Caforio et al¹⁶ found that anti-alpha myosin antibodies were present in 34% of myocarditis patients. In a follow-up study, these antibodies were shown to persist for up to 6 months, which far surpasses the viral cardiac replication timeline of 2 to 3 weeks,¹⁷ suggesting that damage occurring after that time is primarily an autoimmune process.

The differential diagnosis for WNV myocarditis includes myocardial stunning from demand ischemia related to SIRS, Takotsubo cardiomyopathy (stress cardiomyopathy), and Dressler’s syndrome. For our patient, myocardial stunning from demand ischemia was less likely because he had no obstructive coronary disease or focal hypokinesis. In addition, the persistence of left ventricular systolic dysfunction and global hypokinesis demonstrated in a repeat echocardiogram during follow-up 6 months later reinforced the likelihood of myocarditis.

Although West Nile virus has been linked to myocarditis in animals, few human cases of WNV myocarditis or cardiomyopathy have been reported.

The patient’s chest pain with syncope, elevated troponin level, and nonspecific EKG changes in the absence of obstructive CAD raised the possibility of Takotsubo cardiomyopathy. The characteristic echocardiogram finding in Takotsubo cardiomyopathy is transient apical ballooning with akinesis or hypokinesis in the apical and/or mid ventricular regions (typical variant) or isolated midventricular hypokinesis (apical sparing variant). Our patient’s echocardiogram did not show any of these focal wall motion abnormalities, but instead showed global hypokinesis. In addition, the persistence of systolic dysfunction during the repeat echocardiogram and the patient’s lack of psychological distress made the diagnosis of Takotsubo cardiomyopathy unlikely.

Dressler’s syndrome, which is also known as post-myocardial infarction (MI) syndrome, typically presents weeks to months after MI as pleuritic chest pain with a pericardial rub, elevated inflammatory markers, typical EKG changes (diffuse ST-segment elevation and PR-segment depression), and pericardial effusion. This did not fit our patient’s presentation.

Supportive care for heart failure is the mainstay of treatment

The standard treatment for WNV myocarditis is supportive care. Diuretics are used as needed for fluid overload, along with angiotensin-converting enzyme inhibitors and beta-blockers for cardiomyopathy with decreased EF.

Our patient’s dyspnea improved with treatment of furosemide 40 mg IV BID, and his blood pressure was controlled with metoprolol 25 mg BID and lisinopril 10 mg BID. His chest pain and fever resolved when his blood pressure improved. He was discharged home after 7 days on the furosemide, metoprolol, and lisinopril, in addition to isosorbide mononitrate 30 mg/d, atorvastatin 40 mg/d, and aspirin 325 mg/d. An echocardiogram performed 6 months later showed persistent systolic dysfunction, with an EF of 35% and global wall motion abnormalities.

THE TAKEAWAY

In addition to acute coronary syndrome, consider alternate etiologies in patients who present with chest pain and elevated cardiac biomarkers, particularly if diagnostic work-up is negative for obstructive coronary artery disease. WNV myocarditis should be considered as a diagnosis when a patient’s symptoms suggest acute coronary syndrome but are accompanied by fever, headache, and other constitutional symptoms, especially during mosquito season or a WNV outbreak.

THE CASE

A 38-year-old Hispanic man was brought to the emergency department (ED) after losing consciousness and falling at home, striking his elbow, head, and neck. For the past week, he’d had palpitations, shortness of breath, mild swelling of the lower extremities, fever, nausea, and fatigue. He had also been experiencing squeezing chest pain that worsened with exertion and was only partially relieved by nitroglycerin.

The patient did not have any rashes and denied having contact with anyone who was sick. He said that he’d been bitten by mosquitos during recent outdoor activities. His medical history included hypertension, hemorrhagic basal ganglia stroke, hyperlipidemia, sleep apnea, metabolic syndrome, and gout. The patient denied smoking or using illicit drugs.

In the ED, his temperature was 101°F, heart rate was 112 beats/min, blood pressure was 175/100 mm Hg, and respiratory rate was 18 breaths/min. His head and neck exam was normal, with no neck stiffness. A lung exam revealed bilateral basal crackles, and a neurologic exam showed residual right-sided weakness due to the hemorrhagic stroke one year ago.

Lab test results revealed the following: white blood cell (WBC) count, 13,000/mm³ with relative monocytosis (14%); lymphocytosis (44%) with normal neutrophils and no bands; hemoglobin, 12 g/dL; hematocrit, 36/mm³; and platelets, 300,000/mm³. Liver function tests were within normal limits. Urinalysis was unremarkable. His troponin I level was elevated at 1.385 ng/dL. In addition to the tachycardia, his electrocardiogram (EKG) showed left axis deviation, left atrial enlargement, left anterior fascicular block, and diffuse nonspecific ST and T wave abnormalities. Chest x-ray was unremarkable except for cardiomegaly. A computed tomography (CT) scan of his head showed residual changes from the previous stroke.

The patient was admitted with a provisional diagnosis of systemic inflammatory response syndrome (SIRS), syncope, non–ST elevation myocardial infarction (NSTEMI), and acute heart failure. The patient had continuous EKG monitoring and serial assessments of his troponin levels. He was also given aspirin, metoprolol 25 mg BID, lisinopril 10 mg/d, furosemide 40 mg IV, isosorbide mononitrate 60 mg/d, and atorvastatin 40 mg/d.

The patient’s cardiac enzymes subsequently decreased. A left heart catheterization was performed, which showed minimum irregularities of the left anterior descending artery (< 20% narrowing) and an ejection fraction (EF) of 35%, without any evidence of obstructive coronary artery disease (CAD). An echocardiogram revealed systolic dysfunction, with an EF of 35% to 40% and global hypokinesis without any apical ballooning or pericardial effusion. (An echocardiogram performed 6 months earlier had shown normal systolic function, an EF of 60% to 65%, and no wall motion abnormalities.) Blood, urine, and fungal cultures were negative; stool studies for ova and parasites were also negative. A lower extremity venous Doppler was negative for deep vein thrombosis.

THE DIAGNOSIS

Because our patient had SIRS, troponinemia, acute systolic dysfunction, and global hypokinesis without any evidence of obstructive CAD, we considered a diagnosis of viral myocarditis. Serologic studies for echovirus, coxsackievirus B, parvovirus B19, adenovirus, and human herpesvirus 6 (HHV-6) all came back negative. However, an enzyme-linked immunosorbent assay (ELISA) for West Nile virus (WNV) was positive. WNV infection was confirmed with a positive plaque reduction neutralization test and a positive qualitative polymerase chain reaction (PCR) assay, which established a diagnosis of WNV myocarditis.

DISCUSSION

While most individuals infected with WNV are asymptomatic, 20% to 40% of patients will exhibit symptoms.^1-4 Typical presentations of WNV infection include West Nile fever and neuroinvasive disease. West Nile fever is a self-limited illness characterized by a low-grade fever, headache, malaise, back pain, myalgia, and anorexia for 3 to 6 days.² Neuroinvasive disease caused by WNV may present as encephalitis, meningitis, or flaccid paralysis.⁵ Atypical presentations of the virus include rhabdomyolysis,⁶ fatal hemorrhagic fever with multi-organ failure and palpable purpura,⁷ hepatitis,⁸ pancreatitis,⁹ central diabetes insipidus,¹⁰ and myocarditis.¹¹

Although WNV has been linked to myocarditismin animals,¹² few human cases of WNV myocarditis^11,13 or cardiomyopathy¹⁴ have been reported. Viral myocarditis often leads to the development of dilated cardiomyopathy, and myocardial damage may result from direct virus-induced cytotoxicity, T cell-mediated immune response to the virus, or apoptosis.¹⁵ Some research suggests that immune-mediated mechanisms play a primary role in myocardial damage. Caforio et al¹⁶ found that anti-alpha myosin antibodies were present in 34% of myocarditis patients. In a follow-up study, these antibodies were shown to persist for up to 6 months, which far surpasses the viral cardiac replication timeline of 2 to 3 weeks,¹⁷ suggesting that damage occurring after that time is primarily an autoimmune process.

The differential diagnosis for WNV myocarditis includes myocardial stunning from demand ischemia related to SIRS, Takotsubo cardiomyopathy (stress cardiomyopathy), and Dressler’s syndrome. For our patient, myocardial stunning from demand ischemia was less likely because he had no obstructive coronary disease or focal hypokinesis. In addition, the persistence of left ventricular systolic dysfunction and global hypokinesis demonstrated in a repeat echocardiogram during follow-up 6 months later reinforced the likelihood of myocarditis.

Although West Nile virus has been linked to myocarditis in animals, few human cases of WNV myocarditis or cardiomyopathy have been reported.

The patient’s chest pain with syncope, elevated troponin level, and nonspecific EKG changes in the absence of obstructive CAD raised the possibility of Takotsubo cardiomyopathy. The characteristic echocardiogram finding in Takotsubo cardiomyopathy is transient apical ballooning with akinesis or hypokinesis in the apical and/or mid ventricular regions (typical variant) or isolated midventricular hypokinesis (apical sparing variant). Our patient’s echocardiogram did not show any of these focal wall motion abnormalities, but instead showed global hypokinesis. In addition, the persistence of systolic dysfunction during the repeat echocardiogram and the patient’s lack of psychological distress made the diagnosis of Takotsubo cardiomyopathy unlikely.

Dressler’s syndrome, which is also known as post-myocardial infarction (MI) syndrome, typically presents weeks to months after MI as pleuritic chest pain with a pericardial rub, elevated inflammatory markers, typical EKG changes (diffuse ST-segment elevation and PR-segment depression), and pericardial effusion. This did not fit our patient’s presentation.

Supportive care for heart failure is the mainstay of treatment

The standard treatment for WNV myocarditis is supportive care. Diuretics are used as needed for fluid overload, along with angiotensin-converting enzyme inhibitors and beta-blockers for cardiomyopathy with decreased EF.

Our patient’s dyspnea improved with treatment of furosemide 40 mg IV BID, and his blood pressure was controlled with metoprolol 25 mg BID and lisinopril 10 mg BID. His chest pain and fever resolved when his blood pressure improved. He was discharged home after 7 days on the furosemide, metoprolol, and lisinopril, in addition to isosorbide mononitrate 30 mg/d, atorvastatin 40 mg/d, and aspirin 325 mg/d. An echocardiogram performed 6 months later showed persistent systolic dysfunction, with an EF of 35% and global wall motion abnormalities.

THE TAKEAWAY

In addition to acute coronary syndrome, consider alternate etiologies in patients who present with chest pain and elevated cardiac biomarkers, particularly if diagnostic work-up is negative for obstructive coronary artery disease. WNV myocarditis should be considered as a diagnosis when a patient’s symptoms suggest acute coronary syndrome but are accompanied by fever, headache, and other constitutional symptoms, especially during mosquito season or a WNV outbreak.

THE CASE

A 38-year-old Hispanic man was brought to the emergency department (ED) after losing consciousness and falling at home, striking his elbow, head, and neck. For the past week, he’d had palpitations, shortness of breath, mild swelling of the lower extremities, fever, nausea, and fatigue. He had also been experiencing squeezing chest pain that worsened with exertion and was only partially relieved by nitroglycerin.

The patient did not have any rashes and denied having contact with anyone who was sick. He said that he’d been bitten by mosquitos during recent outdoor activities. His medical history included hypertension, hemorrhagic basal ganglia stroke, hyperlipidemia, sleep apnea, metabolic syndrome, and gout. The patient denied smoking or using illicit drugs.

In the ED, his temperature was 101°F, heart rate was 112 beats/min, blood pressure was 175/100 mm Hg, and respiratory rate was 18 breaths/min. His head and neck exam was normal, with no neck stiffness. A lung exam revealed bilateral basal crackles, and a neurologic exam showed residual right-sided weakness due to the hemorrhagic stroke one year ago.

Lab test results revealed the following: white blood cell (WBC) count, 13,000/mm³ with relative monocytosis (14%); lymphocytosis (44%) with normal neutrophils and no bands; hemoglobin, 12 g/dL; hematocrit, 36/mm³; and platelets, 300,000/mm³. Liver function tests were within normal limits. Urinalysis was unremarkable. His troponin I level was elevated at 1.385 ng/dL. In addition to the tachycardia, his electrocardiogram (EKG) showed left axis deviation, left atrial enlargement, left anterior fascicular block, and diffuse nonspecific ST and T wave abnormalities. Chest x-ray was unremarkable except for cardiomegaly. A computed tomography (CT) scan of his head showed residual changes from the previous stroke.

The patient was admitted with a provisional diagnosis of systemic inflammatory response syndrome (SIRS), syncope, non–ST elevation myocardial infarction (NSTEMI), and acute heart failure. The patient had continuous EKG monitoring and serial assessments of his troponin levels. He was also given aspirin, metoprolol 25 mg BID, lisinopril 10 mg/d, furosemide 40 mg IV, isosorbide mononitrate 60 mg/d, and atorvastatin 40 mg/d.

The patient’s cardiac enzymes subsequently decreased. A left heart catheterization was performed, which showed minimum irregularities of the left anterior descending artery (< 20% narrowing) and an ejection fraction (EF) of 35%, without any evidence of obstructive coronary artery disease (CAD). An echocardiogram revealed systolic dysfunction, with an EF of 35% to 40% and global hypokinesis without any apical ballooning or pericardial effusion. (An echocardiogram performed 6 months earlier had shown normal systolic function, an EF of 60% to 65%, and no wall motion abnormalities.) Blood, urine, and fungal cultures were negative; stool studies for ova and parasites were also negative. A lower extremity venous Doppler was negative for deep vein thrombosis.

THE DIAGNOSIS

Because our patient had SIRS, troponinemia, acute systolic dysfunction, and global hypokinesis without any evidence of obstructive CAD, we considered a diagnosis of viral myocarditis. Serologic studies for echovirus, coxsackievirus B, parvovirus B19, adenovirus, and human herpesvirus 6 (HHV-6) all came back negative. However, an enzyme-linked immunosorbent assay (ELISA) for West Nile virus (WNV) was positive. WNV infection was confirmed with a positive plaque reduction neutralization test and a positive qualitative polymerase chain reaction (PCR) assay, which established a diagnosis of WNV myocarditis.

DISCUSSION

While most individuals infected with WNV are asymptomatic, 20% to 40% of patients will exhibit symptoms.^1-4 Typical presentations of WNV infection include West Nile fever and neuroinvasive disease. West Nile fever is a self-limited illness characterized by a low-grade fever, headache, malaise, back pain, myalgia, and anorexia for 3 to 6 days.² Neuroinvasive disease caused by WNV may present as encephalitis, meningitis, or flaccid paralysis.⁵ Atypical presentations of the virus include rhabdomyolysis,⁶ fatal hemorrhagic fever with multi-organ failure and palpable purpura,⁷ hepatitis,⁸ pancreatitis,⁹ central diabetes insipidus,¹⁰ and myocarditis.¹¹

Although WNV has been linked to myocarditismin animals,¹² few human cases of WNV myocarditis^11,13 or cardiomyopathy¹⁴ have been reported. Viral myocarditis often leads to the development of dilated cardiomyopathy, and myocardial damage may result from direct virus-induced cytotoxicity, T cell-mediated immune response to the virus, or apoptosis.¹⁵ Some research suggests that immune-mediated mechanisms play a primary role in myocardial damage. Caforio et al¹⁶ found that anti-alpha myosin antibodies were present in 34% of myocarditis patients. In a follow-up study, these antibodies were shown to persist for up to 6 months, which far surpasses the viral cardiac replication timeline of 2 to 3 weeks,¹⁷ suggesting that damage occurring after that time is primarily an autoimmune process.

The differential diagnosis for WNV myocarditis includes myocardial stunning from demand ischemia related to SIRS, Takotsubo cardiomyopathy (stress cardiomyopathy), and Dressler’s syndrome. For our patient, myocardial stunning from demand ischemia was less likely because he had no obstructive coronary disease or focal hypokinesis. In addition, the persistence of left ventricular systolic dysfunction and global hypokinesis demonstrated in a repeat echocardiogram during follow-up 6 months later reinforced the likelihood of myocarditis.

Although West Nile virus has been linked to myocarditis in animals, few human cases of WNV myocarditis or cardiomyopathy have been reported.

The patient’s chest pain with syncope, elevated troponin level, and nonspecific EKG changes in the absence of obstructive CAD raised the possibility of Takotsubo cardiomyopathy. The characteristic echocardiogram finding in Takotsubo cardiomyopathy is transient apical ballooning with akinesis or hypokinesis in the apical and/or mid ventricular regions (typical variant) or isolated midventricular hypokinesis (apical sparing variant). Our patient’s echocardiogram did not show any of these focal wall motion abnormalities, but instead showed global hypokinesis. In addition, the persistence of systolic dysfunction during the repeat echocardiogram and the patient’s lack of psychological distress made the diagnosis of Takotsubo cardiomyopathy unlikely.

Dressler’s syndrome, which is also known as post-myocardial infarction (MI) syndrome, typically presents weeks to months after MI as pleuritic chest pain with a pericardial rub, elevated inflammatory markers, typical EKG changes (diffuse ST-segment elevation and PR-segment depression), and pericardial effusion. This did not fit our patient’s presentation.

Supportive care for heart failure is the mainstay of treatment

The standard treatment for WNV myocarditis is supportive care. Diuretics are used as needed for fluid overload, along with angiotensin-converting enzyme inhibitors and beta-blockers for cardiomyopathy with decreased EF.

Our patient’s dyspnea improved with treatment of furosemide 40 mg IV BID, and his blood pressure was controlled with metoprolol 25 mg BID and lisinopril 10 mg BID. His chest pain and fever resolved when his blood pressure improved. He was discharged home after 7 days on the furosemide, metoprolol, and lisinopril, in addition to isosorbide mononitrate 30 mg/d, atorvastatin 40 mg/d, and aspirin 325 mg/d. An echocardiogram performed 6 months later showed persistent systolic dysfunction, with an EF of 35% and global wall motion abnormalities.

THE TAKEAWAY

In addition to acute coronary syndrome, consider alternate etiologies in patients who present with chest pain and elevated cardiac biomarkers, particularly if diagnostic work-up is negative for obstructive coronary artery disease. WNV myocarditis should be considered as a diagnosis when a patient’s symptoms suggest acute coronary syndrome but are accompanied by fever, headache, and other constitutional symptoms, especially during mosquito season or a WNV outbreak.

ANSWER
The radiograph shows a fracture dislocation of the ankle. The distal tibia is dislocated medially relative to the talus, as evidenced by the widened joint space. There is also an oblique fracture of the distal fibula.

Since the patient was experiencing neurovascular compromise, the dislocation was promptly reduced in the emergency department. Subsequently, he was taken to the operating room for open reduction and internal fixation of his fibula fracture.   

ANSWER
The radiograph shows a fracture dislocation of the ankle. The distal tibia is dislocated medially relative to the talus, as evidenced by the widened joint space. There is also an oblique fracture of the distal fibula.

Since the patient was experiencing neurovascular compromise, the dislocation was promptly reduced in the emergency department. Subsequently, he was taken to the operating room for open reduction and internal fixation of his fibula fracture.   

ANSWER
The radiograph shows a fracture dislocation of the ankle. The distal tibia is dislocated medially relative to the talus, as evidenced by the widened joint space. There is also an oblique fracture of the distal fibula.

Since the patient was experiencing neurovascular compromise, the dislocation was promptly reduced in the emergency department. Subsequently, he was taken to the operating room for open reduction and internal fixation of his fibula fracture.   

At a New Jersey hospital, a pregnant woman underwent an ultrasound examination with results suggesting a possible fetal abnormality. In response, DNA testing of the patient and her husband was ordered to investigate for a suspected hormonal disorder. But the wrong test was ordered, and the results of that test were negative.

A baby girl was born with congenital adrenal hyperplasia, a condition causing ambiguous genitalia due to exposure to high concentrations of androgens in utero. She underwent genital reconstructive surgery at age 4 months and is expected to require additional surgery, lifelong hormone replacement therapy, and lifelong monitoring.

The parents claimed that they would have elected to terminate the pregnancy if they had been properly informed of the child’s condition.

What was the outcome? >>

OUTCOME
A jury returned a ruling of 75% liability to the hospital and 25% liability to a hospital lab technician. The verdict was for $1 million, comprising $625,000 for the child and $375,000 for her parents.

COMMENT
The controversial legal theory of recovery in this case is known as “wrongful life” or “wrongful birth.” To prevail on these tort actions, one must prove that the defendant’s negligence led to the birth of an infant following a pregnancy that would have been terminated, had the parents been given all the prenatal screening information required by the standard of care.

The goal of any prenatal screening program should be to provide parents with information that is adequate, accurate, and timely. In this case, after the suspicious sonographic findings were encountered, the wrong test was ordered and the diagnosis was missed. Each practice providing prenatal screening should have a checklist to confirm that the correct test was ordered, completed, and documented—not to mention discussed with the patient in a timely manner.

In this case, the clinician ordered the wrong test, which left the patient with inadequate information. From the facts given, it is unclear if the ordering clinician became aware of this fact and what information, if any, the patient was given regarding the error. Importantly, information must also be given in a timely manner, leaving the patient adequate time to make an informed decision regarding termination—before fetal viability. But how is viability defined?

Although a detailed discussion of the constitutional principles of fetal viability is beyond the scope of this commentary, three US Supreme Court cases paved the way for successful wrongful life/wrongful birth actions. In Griswold v Connecticut (1965), the court held that decisions regarding birth control were protected by the right to privacy. In Roe v Wade (1973), the court held that a constitutionally protected right to privacy exists with regard to pregnancy terminations until the point of “viability,” originally defined as between 24 and 28 gestational weeks. Planned Parenthood v Casey (1991) held that advances in neonatal care required a revised definition of viability to a point “somewhat earlier,” without establishing a specific bright-line rule for viability.

To complicate matters, in recent years, at least 14 states (Alabama, Arizona, Arkansas, Georgia, Idaho, Indiana, Kansas, Louisiana, Mississippi, Nebraska, North Carolina, North Dakota, Oklahoma, and Texas) have redefined viability and passed laws banning therapeutic abortion beyond week 20 (although some of these bans have been judicially blocked). In states with this type of legislation, whether a clinician could be held legally responsible for failing to provide information necessary to permit an informed decision prior to the 20-week mark is unclear.

Questions as to whether these state laws were in conflict with Roe v Wade led to a constitutional challenge. In 2013, the US Court of Appeals for the Ninth Circuit (the highest level before the Supreme Court) ruled that a 20-week cutoff was unconstitutional because it violated the “viability rule” established by Roe and Casey. The Supreme Court declined to review that decision.¹

Damage awards in wrongful life/wrongful birth cases are often substantial. The verdict in this case was relatively restrained.

Without doubt, this is a sensitive issue, and respect for our fellow clinicians’ opinions is warranted. However, from a liability standpoint, the safest course of action is to provide patients with all the necessary information—including prenatal testing results—as soon as possible, ­allowing them to make an informed decision before viability (however that is defined in your state). —DML

REFERENCE
1. Isaacson v. Horne, 716 F.3d 1213, 1225 (9th Cir. 2013), cert denied, 134 S. Ct. 905 (2014).

At a New Jersey hospital, a pregnant woman underwent an ultrasound examination with results suggesting a possible fetal abnormality. In response, DNA testing of the patient and her husband was ordered to investigate for a suspected hormonal disorder. But the wrong test was ordered, and the results of that test were negative.

A baby girl was born with congenital adrenal hyperplasia, a condition causing ambiguous genitalia due to exposure to high concentrations of androgens in utero. She underwent genital reconstructive surgery at age 4 months and is expected to require additional surgery, lifelong hormone replacement therapy, and lifelong monitoring.

The parents claimed that they would have elected to terminate the pregnancy if they had been properly informed of the child’s condition.

What was the outcome? >>

OUTCOME
A jury returned a ruling of 75% liability to the hospital and 25% liability to a hospital lab technician. The verdict was for $1 million, comprising $625,000 for the child and $375,000 for her parents.

COMMENT
The controversial legal theory of recovery in this case is known as “wrongful life” or “wrongful birth.” To prevail on these tort actions, one must prove that the defendant’s negligence led to the birth of an infant following a pregnancy that would have been terminated, had the parents been given all the prenatal screening information required by the standard of care.

The goal of any prenatal screening program should be to provide parents with information that is adequate, accurate, and timely. In this case, after the suspicious sonographic findings were encountered, the wrong test was ordered and the diagnosis was missed. Each practice providing prenatal screening should have a checklist to confirm that the correct test was ordered, completed, and documented—not to mention discussed with the patient in a timely manner.

In this case, the clinician ordered the wrong test, which left the patient with inadequate information. From the facts given, it is unclear if the ordering clinician became aware of this fact and what information, if any, the patient was given regarding the error. Importantly, information must also be given in a timely manner, leaving the patient adequate time to make an informed decision regarding termination—before fetal viability. But how is viability defined?

Although a detailed discussion of the constitutional principles of fetal viability is beyond the scope of this commentary, three US Supreme Court cases paved the way for successful wrongful life/wrongful birth actions. In Griswold v Connecticut (1965), the court held that decisions regarding birth control were protected by the right to privacy. In Roe v Wade (1973), the court held that a constitutionally protected right to privacy exists with regard to pregnancy terminations until the point of “viability,” originally defined as between 24 and 28 gestational weeks. Planned Parenthood v Casey (1991) held that advances in neonatal care required a revised definition of viability to a point “somewhat earlier,” without establishing a specific bright-line rule for viability.

To complicate matters, in recent years, at least 14 states (Alabama, Arizona, Arkansas, Georgia, Idaho, Indiana, Kansas, Louisiana, Mississippi, Nebraska, North Carolina, North Dakota, Oklahoma, and Texas) have redefined viability and passed laws banning therapeutic abortion beyond week 20 (although some of these bans have been judicially blocked). In states with this type of legislation, whether a clinician could be held legally responsible for failing to provide information necessary to permit an informed decision prior to the 20-week mark is unclear.

Questions as to whether these state laws were in conflict with Roe v Wade led to a constitutional challenge. In 2013, the US Court of Appeals for the Ninth Circuit (the highest level before the Supreme Court) ruled that a 20-week cutoff was unconstitutional because it violated the “viability rule” established by Roe and Casey. The Supreme Court declined to review that decision.¹

Damage awards in wrongful life/wrongful birth cases are often substantial. The verdict in this case was relatively restrained.

Without doubt, this is a sensitive issue, and respect for our fellow clinicians’ opinions is warranted. However, from a liability standpoint, the safest course of action is to provide patients with all the necessary information—including prenatal testing results—as soon as possible, ­allowing them to make an informed decision before viability (however that is defined in your state). —DML

REFERENCE
1. Isaacson v. Horne, 716 F.3d 1213, 1225 (9th Cir. 2013), cert denied, 134 S. Ct. 905 (2014).

At a New Jersey hospital, a pregnant woman underwent an ultrasound examination with results suggesting a possible fetal abnormality. In response, DNA testing of the patient and her husband was ordered to investigate for a suspected hormonal disorder. But the wrong test was ordered, and the results of that test were negative.

A baby girl was born with congenital adrenal hyperplasia, a condition causing ambiguous genitalia due to exposure to high concentrations of androgens in utero. She underwent genital reconstructive surgery at age 4 months and is expected to require additional surgery, lifelong hormone replacement therapy, and lifelong monitoring.

The parents claimed that they would have elected to terminate the pregnancy if they had been properly informed of the child’s condition.

What was the outcome? >>

OUTCOME
A jury returned a ruling of 75% liability to the hospital and 25% liability to a hospital lab technician. The verdict was for $1 million, comprising $625,000 for the child and $375,000 for her parents.

COMMENT
The controversial legal theory of recovery in this case is known as “wrongful life” or “wrongful birth.” To prevail on these tort actions, one must prove that the defendant’s negligence led to the birth of an infant following a pregnancy that would have been terminated, had the parents been given all the prenatal screening information required by the standard of care.

The goal of any prenatal screening program should be to provide parents with information that is adequate, accurate, and timely. In this case, after the suspicious sonographic findings were encountered, the wrong test was ordered and the diagnosis was missed. Each practice providing prenatal screening should have a checklist to confirm that the correct test was ordered, completed, and documented—not to mention discussed with the patient in a timely manner.

In this case, the clinician ordered the wrong test, which left the patient with inadequate information. From the facts given, it is unclear if the ordering clinician became aware of this fact and what information, if any, the patient was given regarding the error. Importantly, information must also be given in a timely manner, leaving the patient adequate time to make an informed decision regarding termination—before fetal viability. But how is viability defined?

Although a detailed discussion of the constitutional principles of fetal viability is beyond the scope of this commentary, three US Supreme Court cases paved the way for successful wrongful life/wrongful birth actions. In Griswold v Connecticut (1965), the court held that decisions regarding birth control were protected by the right to privacy. In Roe v Wade (1973), the court held that a constitutionally protected right to privacy exists with regard to pregnancy terminations until the point of “viability,” originally defined as between 24 and 28 gestational weeks. Planned Parenthood v Casey (1991) held that advances in neonatal care required a revised definition of viability to a point “somewhat earlier,” without establishing a specific bright-line rule for viability.

To complicate matters, in recent years, at least 14 states (Alabama, Arizona, Arkansas, Georgia, Idaho, Indiana, Kansas, Louisiana, Mississippi, Nebraska, North Carolina, North Dakota, Oklahoma, and Texas) have redefined viability and passed laws banning therapeutic abortion beyond week 20 (although some of these bans have been judicially blocked). In states with this type of legislation, whether a clinician could be held legally responsible for failing to provide information necessary to permit an informed decision prior to the 20-week mark is unclear.

Questions as to whether these state laws were in conflict with Roe v Wade led to a constitutional challenge. In 2013, the US Court of Appeals for the Ninth Circuit (the highest level before the Supreme Court) ruled that a 20-week cutoff was unconstitutional because it violated the “viability rule” established by Roe and Casey. The Supreme Court declined to review that decision.¹

Damage awards in wrongful life/wrongful birth cases are often substantial. The verdict in this case was relatively restrained.

Without doubt, this is a sensitive issue, and respect for our fellow clinicians’ opinions is warranted. However, from a liability standpoint, the safest course of action is to provide patients with all the necessary information—including prenatal testing results—as soon as possible, ­allowing them to make an informed decision before viability (however that is defined in your state). —DML

REFERENCE
1. Isaacson v. Horne, 716 F.3d 1213, 1225 (9th Cir. 2013), cert denied, 134 S. Ct. 905 (2014).

Q) I’ve heard a lot of talk about all the kidney problems that the sugarcane workers in Central America have. Does anyone know why this is happening?

The unusually high rates of chronic kidney disease (CKD) among sugarcane workers in Central America have been a subject of great interest since National Public Radio (NPR) aired a special on this topic.³ There has been a rising epidemic of CKD in otherwise healthy male farm workers (ages 20 to 50), particularly those who harvest sugarcane.^4,5 It has been hypothesized that recurrent episodes of acute kidney injury (AKI)—related to dehydration, volume depletion, pollutants, and rhabdomyolysis with inflammatory stress—are the underlying cause.⁵

Sugarcane harvesters typically work nine-hour days, six days per week, in extremely high temperatures and while wearing heavy, hot clothing. Each worker cuts approximately 10 tons of sugarcane daily, since they are paid based on cutting volume. Workers drink between five and 10 L of water during their shifts.

Santos et al designed a study to prospectively examine the effects of burnt sugarcane harvesting on renal function in healthy male farm workers. Twenty-eight men (ages 19 to 39) with no CKD risk factors (diabetes, smoking, obesity, hypertension, illicit drug or alcohol use) were followed for eight months from preharvest to postharvest. Blood samples were collected at the beginning and at the end of the workday and preharvest and postharvest season.⁵

Preseason lab values were normal in all 28 men. But postseason, all workers had elevated creatinine levels, with five meeting the criteria for AKI (see Table at left).^5,6

Santos and colleagues identified potential causes for AKI in this population. These included
• Dehydration and volume depletion (episodes of tachycardia, increased urine density, lower urinary/serum sodium, higher hematocrit)
• Rhabdomyolysis (increased creatine kinase at the end of each workday) 
• Systemic inflammation (increased white blood count, neutrophils, lymphocytes, and monocytes during the workday—possibly indicative of an inflammatory burst)
• Other factors (burning of the sugarcane releasing unknown nephrotoxic substances; unreported NSAIDs use)⁵

Compared to workers who showed early signs of CKD, those who developed frank AKI were more likely to have hyponatremia. Recommendations to reduce the problem include consumption of water/salt hydrating drinks, use of appropriate clothing, work-hour limitations, and changes to payment structures (ie, from a volume system to an hourly or daily system). Furthermore, education on the need to avoid alcohol, illicit drugs, and NSAIDs during the harvest season should help to decrease incidence of AKI among these workers.

Elizabeth C. Evans, RN, MSN, CNP, DNP
Renal Medicine Associates, Albuquerque, New Mexico

REFERENCES
1. Ayuk J, Gittoes N. Contemporary view of the clinical relevance of magnesium homeostasis. Ann Clin Biochem. 2014;51(Pt 2):179-188.
2. Firouzi A, Maadani M, Kiani R, et al. Intravenous magnesium sulfate: new method in prevention of contrast-induced nephropathy in primary percutaneous coronary intervention. Int Urol Nephrol. 2015;47(3):521-525.
3. Beaubien J. Mysterious kidney disease slays farm workers in central America. National Public Radio; 2014. www.npr.org/blogs/health/2014/04/30/306907097/mysterious-kidney-disease-slays-farmworkers-in-central-america. Accessed April 1, 2015.
4. Almaguer M, Herrera R, Orantes CM. Chronic kidney disease of unknown etiology in agricultural communities. MEDICC Rev. 2014;16(2):9-15.
5. Santos UP, Zanetta DMT, Burdmann EA. Burnt sugarcane harvesting is associated with acute renal dysfunction. Kidney Int. 2015;87(4):792-799.
6. Kidney Disease: Improving Global Outcomes (KDIGO) Acute Kidney Injury Work Group. KDIGO Clinical Practice Guideline for Acute Kidney Injury. Kidney Int Suppl. 2012;2:1-138.

Q) I’ve heard a lot of talk about all the kidney problems that the sugarcane workers in Central America have. Does anyone know why this is happening?

The unusually high rates of chronic kidney disease (CKD) among sugarcane workers in Central America have been a subject of great interest since National Public Radio (NPR) aired a special on this topic.³ There has been a rising epidemic of CKD in otherwise healthy male farm workers (ages 20 to 50), particularly those who harvest sugarcane.^4,5 It has been hypothesized that recurrent episodes of acute kidney injury (AKI)—related to dehydration, volume depletion, pollutants, and rhabdomyolysis with inflammatory stress—are the underlying cause.⁵

Sugarcane harvesters typically work nine-hour days, six days per week, in extremely high temperatures and while wearing heavy, hot clothing. Each worker cuts approximately 10 tons of sugarcane daily, since they are paid based on cutting volume. Workers drink between five and 10 L of water during their shifts.

Santos et al designed a study to prospectively examine the effects of burnt sugarcane harvesting on renal function in healthy male farm workers. Twenty-eight men (ages 19 to 39) with no CKD risk factors (diabetes, smoking, obesity, hypertension, illicit drug or alcohol use) were followed for eight months from preharvest to postharvest. Blood samples were collected at the beginning and at the end of the workday and preharvest and postharvest season.⁵

Preseason lab values were normal in all 28 men. But postseason, all workers had elevated creatinine levels, with five meeting the criteria for AKI (see Table at left).^5,6

Santos and colleagues identified potential causes for AKI in this population. These included
• Dehydration and volume depletion (episodes of tachycardia, increased urine density, lower urinary/serum sodium, higher hematocrit)
• Rhabdomyolysis (increased creatine kinase at the end of each workday) 
• Systemic inflammation (increased white blood count, neutrophils, lymphocytes, and monocytes during the workday—possibly indicative of an inflammatory burst)
• Other factors (burning of the sugarcane releasing unknown nephrotoxic substances; unreported NSAIDs use)⁵

Compared to workers who showed early signs of CKD, those who developed frank AKI were more likely to have hyponatremia. Recommendations to reduce the problem include consumption of water/salt hydrating drinks, use of appropriate clothing, work-hour limitations, and changes to payment structures (ie, from a volume system to an hourly or daily system). Furthermore, education on the need to avoid alcohol, illicit drugs, and NSAIDs during the harvest season should help to decrease incidence of AKI among these workers.

Elizabeth C. Evans, RN, MSN, CNP, DNP
Renal Medicine Associates, Albuquerque, New Mexico

REFERENCES
1. Ayuk J, Gittoes N. Contemporary view of the clinical relevance of magnesium homeostasis. Ann Clin Biochem. 2014;51(Pt 2):179-188.
2. Firouzi A, Maadani M, Kiani R, et al. Intravenous magnesium sulfate: new method in prevention of contrast-induced nephropathy in primary percutaneous coronary intervention. Int Urol Nephrol. 2015;47(3):521-525.
3. Beaubien J. Mysterious kidney disease slays farm workers in central America. National Public Radio; 2014. www.npr.org/blogs/health/2014/04/30/306907097/mysterious-kidney-disease-slays-farmworkers-in-central-america. Accessed April 1, 2015.
4. Almaguer M, Herrera R, Orantes CM. Chronic kidney disease of unknown etiology in agricultural communities. MEDICC Rev. 2014;16(2):9-15.
5. Santos UP, Zanetta DMT, Burdmann EA. Burnt sugarcane harvesting is associated with acute renal dysfunction. Kidney Int. 2015;87(4):792-799.
6. Kidney Disease: Improving Global Outcomes (KDIGO) Acute Kidney Injury Work Group. KDIGO Clinical Practice Guideline for Acute Kidney Injury. Kidney Int Suppl. 2012;2:1-138.

Q) I’ve heard a lot of talk about all the kidney problems that the sugarcane workers in Central America have. Does anyone know why this is happening?

The unusually high rates of chronic kidney disease (CKD) among sugarcane workers in Central America have been a subject of great interest since National Public Radio (NPR) aired a special on this topic.³ There has been a rising epidemic of CKD in otherwise healthy male farm workers (ages 20 to 50), particularly those who harvest sugarcane.^4,5 It has been hypothesized that recurrent episodes of acute kidney injury (AKI)—related to dehydration, volume depletion, pollutants, and rhabdomyolysis with inflammatory stress—are the underlying cause.⁵

Sugarcane harvesters typically work nine-hour days, six days per week, in extremely high temperatures and while wearing heavy, hot clothing. Each worker cuts approximately 10 tons of sugarcane daily, since they are paid based on cutting volume. Workers drink between five and 10 L of water during their shifts.

Santos et al designed a study to prospectively examine the effects of burnt sugarcane harvesting on renal function in healthy male farm workers. Twenty-eight men (ages 19 to 39) with no CKD risk factors (diabetes, smoking, obesity, hypertension, illicit drug or alcohol use) were followed for eight months from preharvest to postharvest. Blood samples were collected at the beginning and at the end of the workday and preharvest and postharvest season.⁵

Preseason lab values were normal in all 28 men. But postseason, all workers had elevated creatinine levels, with five meeting the criteria for AKI (see Table at left).^5,6

Santos and colleagues identified potential causes for AKI in this population. These included
• Dehydration and volume depletion (episodes of tachycardia, increased urine density, lower urinary/serum sodium, higher hematocrit)
• Rhabdomyolysis (increased creatine kinase at the end of each workday) 
• Systemic inflammation (increased white blood count, neutrophils, lymphocytes, and monocytes during the workday—possibly indicative of an inflammatory burst)
• Other factors (burning of the sugarcane releasing unknown nephrotoxic substances; unreported NSAIDs use)⁵

Compared to workers who showed early signs of CKD, those who developed frank AKI were more likely to have hyponatremia. Recommendations to reduce the problem include consumption of water/salt hydrating drinks, use of appropriate clothing, work-hour limitations, and changes to payment structures (ie, from a volume system to an hourly or daily system). Furthermore, education on the need to avoid alcohol, illicit drugs, and NSAIDs during the harvest season should help to decrease incidence of AKI among these workers.

Elizabeth C. Evans, RN, MSN, CNP, DNP
Renal Medicine Associates, Albuquerque, New Mexico

REFERENCES
1. Ayuk J, Gittoes N. Contemporary view of the clinical relevance of magnesium homeostasis. Ann Clin Biochem. 2014;51(Pt 2):179-188.
2. Firouzi A, Maadani M, Kiani R, et al. Intravenous magnesium sulfate: new method in prevention of contrast-induced nephropathy in primary percutaneous coronary intervention. Int Urol Nephrol. 2015;47(3):521-525.
3. Beaubien J. Mysterious kidney disease slays farm workers in central America. National Public Radio; 2014. www.npr.org/blogs/health/2014/04/30/306907097/mysterious-kidney-disease-slays-farmworkers-in-central-america. Accessed April 1, 2015.
4. Almaguer M, Herrera R, Orantes CM. Chronic kidney disease of unknown etiology in agricultural communities. MEDICC Rev. 2014;16(2):9-15.
5. Santos UP, Zanetta DMT, Burdmann EA. Burnt sugarcane harvesting is associated with acute renal dysfunction. Kidney Int. 2015;87(4):792-799.
6. Kidney Disease: Improving Global Outcomes (KDIGO) Acute Kidney Injury Work Group. KDIGO Clinical Practice Guideline for Acute Kidney Injury. Kidney Int Suppl. 2012;2:1-138.

ANSWER

This ECG shows marked sinus bradycardia with a first-degree atrioventricular block and nonspecific T-wave abnormality. The QT interval of 524 ms is consistent with prolonged QT interval but is normal when corrected for rate.

These findings were consistent with previous ECGs. Since the patient’s bradycardia is asymptomatic, no intervention (ie, placement of a permanent pacemaker) is indicated.

ANSWER

This ECG shows marked sinus bradycardia with a first-degree atrioventricular block and nonspecific T-wave abnormality. The QT interval of 524 ms is consistent with prolonged QT interval but is normal when corrected for rate.

These findings were consistent with previous ECGs. Since the patient’s bradycardia is asymptomatic, no intervention (ie, placement of a permanent pacemaker) is indicated.

ANSWER

This ECG shows marked sinus bradycardia with a first-degree atrioventricular block and nonspecific T-wave abnormality. The QT interval of 524 ms is consistent with prolonged QT interval but is normal when corrected for rate.

These findings were consistent with previous ECGs. Since the patient’s bradycardia is asymptomatic, no intervention (ie, placement of a permanent pacemaker) is indicated.

Stomach pain chalked up to flu; patient suffers fatal cardiac event

A 40-YEAR-OLD MAN went to the emergency department (ED) after 2 days of stomach discomfort. The ED physician who evaluated him released him after 4 or 5 hours without testing for levels of troponin or other cardiac enzymes. The patient’s discomfort continued, and about 3 days later, he told his wife to call 911. He was transported to the ED but did not survive.

PLAINTIFF’S CLAIM The decedent had been suffering from an acute cardiac event during the first ED visit. Testing to rule out cardiac problems should have been performed.

THE DEFENSE The patient had been suffering from a stomach flu during his initial ED visit. Any testing performed at that time would have been normal. The patient’s death was unrelated to the symptoms he was experiencing when he was first seen.

VERDICT $4 million Alabama verdict.

COMMENT Many questions come to mind with this case: How careful was the history? Did the patient’s discomfort get worse with activity? What were the characteristics of his pain? What were the patient’s cardiac risk factors? A colleague of mine missed a very similar case several years ago in a 67-year-old. The patient even had vomiting and diarrhea, but clearly had a myocardial infarction when diagnosed a few days later.

Follow-up failure on PSA results costs patient valuable Tx time

A PATIENT AT A GROUP PRACTICE underwent prostate specific antigen (PSA) screening, which revealed an abnormal result (4.1 ng/mL). The physician circled this value on the lab report, wrote, “Discuss next visit,” and placed the report in the patient’s chart. However, the patient switched to another physician in the group and was not told of the abnormal result for more than 2 years. When the patient went to a medical center for back pain, magnetic resonance imaging of his spine revealed the presence of cancer in his spine, shoulder blades, pelvis, and ribs. A PSA test performed at that time came back at 100 ng/mL. Two days later, a biopsy confirmed the diagnosis of prostate cancer (Gleason score, 9).

PLAINTIFF’S CLAIM In addition to failing to inform the patient of his abnormal PSA test result, the physician did not perform digital rectal exams.

THE DEFENSE Earlier treatment would not have made a difference in the outcome.

VERDICT $934,000 Florida verdict.

COMMENT If you order a PSA, you must follow up on it. When a patient transfers to your care, be sure to obtain and review past testing and provide follow-up on abnormal results. We now send all test results directly to patients so they can serve as a safety check for their own care. Despite fears of being inundated with calls, most organizations that have instituted such a policy have not turned back.

Stomach pain chalked up to flu; patient suffers fatal cardiac event

A 40-YEAR-OLD MAN went to the emergency department (ED) after 2 days of stomach discomfort. The ED physician who evaluated him released him after 4 or 5 hours without testing for levels of troponin or other cardiac enzymes. The patient’s discomfort continued, and about 3 days later, he told his wife to call 911. He was transported to the ED but did not survive.

PLAINTIFF’S CLAIM The decedent had been suffering from an acute cardiac event during the first ED visit. Testing to rule out cardiac problems should have been performed.

THE DEFENSE The patient had been suffering from a stomach flu during his initial ED visit. Any testing performed at that time would have been normal. The patient’s death was unrelated to the symptoms he was experiencing when he was first seen.

VERDICT $4 million Alabama verdict.

COMMENT Many questions come to mind with this case: How careful was the history? Did the patient’s discomfort get worse with activity? What were the characteristics of his pain? What were the patient’s cardiac risk factors? A colleague of mine missed a very similar case several years ago in a 67-year-old. The patient even had vomiting and diarrhea, but clearly had a myocardial infarction when diagnosed a few days later.

Follow-up failure on PSA results costs patient valuable Tx time

A PATIENT AT A GROUP PRACTICE underwent prostate specific antigen (PSA) screening, which revealed an abnormal result (4.1 ng/mL). The physician circled this value on the lab report, wrote, “Discuss next visit,” and placed the report in the patient’s chart. However, the patient switched to another physician in the group and was not told of the abnormal result for more than 2 years. When the patient went to a medical center for back pain, magnetic resonance imaging of his spine revealed the presence of cancer in his spine, shoulder blades, pelvis, and ribs. A PSA test performed at that time came back at 100 ng/mL. Two days later, a biopsy confirmed the diagnosis of prostate cancer (Gleason score, 9).

PLAINTIFF’S CLAIM In addition to failing to inform the patient of his abnormal PSA test result, the physician did not perform digital rectal exams.

THE DEFENSE Earlier treatment would not have made a difference in the outcome.

VERDICT $934,000 Florida verdict.

COMMENT If you order a PSA, you must follow up on it. When a patient transfers to your care, be sure to obtain and review past testing and provide follow-up on abnormal results. We now send all test results directly to patients so they can serve as a safety check for their own care. Despite fears of being inundated with calls, most organizations that have instituted such a policy have not turned back.

Stomach pain chalked up to flu; patient suffers fatal cardiac event

A 40-YEAR-OLD MAN went to the emergency department (ED) after 2 days of stomach discomfort. The ED physician who evaluated him released him after 4 or 5 hours without testing for levels of troponin or other cardiac enzymes. The patient’s discomfort continued, and about 3 days later, he told his wife to call 911. He was transported to the ED but did not survive.

PLAINTIFF’S CLAIM The decedent had been suffering from an acute cardiac event during the first ED visit. Testing to rule out cardiac problems should have been performed.

THE DEFENSE The patient had been suffering from a stomach flu during his initial ED visit. Any testing performed at that time would have been normal. The patient’s death was unrelated to the symptoms he was experiencing when he was first seen.

VERDICT $4 million Alabama verdict.

COMMENT Many questions come to mind with this case: How careful was the history? Did the patient’s discomfort get worse with activity? What were the characteristics of his pain? What were the patient’s cardiac risk factors? A colleague of mine missed a very similar case several years ago in a 67-year-old. The patient even had vomiting and diarrhea, but clearly had a myocardial infarction when diagnosed a few days later.

Follow-up failure on PSA results costs patient valuable Tx time

A PATIENT AT A GROUP PRACTICE underwent prostate specific antigen (PSA) screening, which revealed an abnormal result (4.1 ng/mL). The physician circled this value on the lab report, wrote, “Discuss next visit,” and placed the report in the patient’s chart. However, the patient switched to another physician in the group and was not told of the abnormal result for more than 2 years. When the patient went to a medical center for back pain, magnetic resonance imaging of his spine revealed the presence of cancer in his spine, shoulder blades, pelvis, and ribs. A PSA test performed at that time came back at 100 ng/mL. Two days later, a biopsy confirmed the diagnosis of prostate cancer (Gleason score, 9).

PLAINTIFF’S CLAIM In addition to failing to inform the patient of his abnormal PSA test result, the physician did not perform digital rectal exams.

THE DEFENSE Earlier treatment would not have made a difference in the outcome.

VERDICT $934,000 Florida verdict.

COMMENT If you order a PSA, you must follow up on it. When a patient transfers to your care, be sure to obtain and review past testing and provide follow-up on abnormal results. We now send all test results directly to patients so they can serve as a safety check for their own care. Despite fears of being inundated with calls, most organizations that have instituted such a policy have not turned back.

Both of these treatments relieve redness and itching

A 2004 systematic review of 40 RCTs (total N not provided) assessed the efficacy of topical treatment with mast cell stabilizers and antihistamines, comparing each with the other and placebo.¹ Eleven trials that included 899 children and adults compared mast cell stabilizers (sodium cromoglycate, nedocromil, and lodoxamide tromethamine) with placebo. Follow-up periods ranged from 4 to 9 weeks.

Because of study heterogeneity, a random-effects model was used and showed that topical mast cell stabilizers relieved symptoms (ocular itching, burning, and lacrimation) 4.9 times more effectively than placebo (95% confidence interval [CI], 2.5-9.6). Possible publication bias was cited as a limitation.

In the same systematic review, 9 RCTs with 313 patients compared topical antihistamines (levocabastine, azelastine hydrochloride, emedastine, and antazoline phosphate) with placebo. Signs and symptoms (itching, redness, burning, and swelling) were graded using symptom severity scales. Follow-up ranged from 30 minutes to 24 hours. A meta-analysis wasn’t possible because most studies didn’t tabulate the mean scores and error associated with these scores. Most individual studies, however, showed improvement in the cardinal symptom of itchiness.

Finally, 8 RCTs compared topical mast cell stabilizers (sodium cromoglycate, lodoxamide, and nedocromil sodium) with levocabastine, a topical antihistamine. Two RCTs with 74 patients had follow-up periods of 15 minutes to 4 hours; the remaining 6 RCTs with 473 patients had follow-up periods of 14 days to 4 months. Subjective scoring of symptoms was done in 7 of the 8 studies.

Scores between treatment groups were reported as not statistically significant in the 6 longer-term studies. Meta-analysis wasn’t possible because most studies didn’t tabulate the mean scores and error associated with measures. The 2 short-term studies reported a statistically significant reduction in itching and redness (P<.05) in patients treated with the antihistamine (data not provided).

NSAIDs relieve itching but may sting when applied

A 2007 meta-analysis of 8 RCTs compared topical NSAIDs (ketorolac, diclofenac, aspirin, or steroid) with placebo for treating isolated allergic conjunctivitis in 712 children and adults.² Primary outcomes were measured as subjective reductions in conjunctival injection and itching measured at 2 to 6 weeks using a 0-to-3 severity scale.

Topical NSAIDs produced significantly greater relief of conjunctival itching (4 trials, N=231; mean difference [MD]=-0.54; 95% CI, -0.84 to -0.24) and conjunctival injection (4 trials, N=208; MD=-0.51; 95% CI, -0.97 to -0.05). NSAIDs weren’t superior to placebo in treating other ocular symptoms of eyelid swelling, ocular burning, photophobia, or foreign body sensation, and they had a higher rate of stinging on application (odds ratio=4.0; 95% CI, 2.7-5.9).

Guideline recommends topical antihistamines or mast cell stabilizers

The American Academy of Ophthalmology’s 2012 evidence-based guideline recommends treating allergic conjunctivitis with topical antihistamines (Level A-1 evidence, defined as important evidence supported by at least one RCT or a meta-analysis) and using topical mast cell stabilizers if the condition is recurrent.³

Both of these treatments relieve redness and itching

A 2004 systematic review of 40 RCTs (total N not provided) assessed the efficacy of topical treatment with mast cell stabilizers and antihistamines, comparing each with the other and placebo.¹ Eleven trials that included 899 children and adults compared mast cell stabilizers (sodium cromoglycate, nedocromil, and lodoxamide tromethamine) with placebo. Follow-up periods ranged from 4 to 9 weeks.

Because of study heterogeneity, a random-effects model was used and showed that topical mast cell stabilizers relieved symptoms (ocular itching, burning, and lacrimation) 4.9 times more effectively than placebo (95% confidence interval [CI], 2.5-9.6). Possible publication bias was cited as a limitation.

In the same systematic review, 9 RCTs with 313 patients compared topical antihistamines (levocabastine, azelastine hydrochloride, emedastine, and antazoline phosphate) with placebo. Signs and symptoms (itching, redness, burning, and swelling) were graded using symptom severity scales. Follow-up ranged from 30 minutes to 24 hours. A meta-analysis wasn’t possible because most studies didn’t tabulate the mean scores and error associated with these scores. Most individual studies, however, showed improvement in the cardinal symptom of itchiness.

Finally, 8 RCTs compared topical mast cell stabilizers (sodium cromoglycate, lodoxamide, and nedocromil sodium) with levocabastine, a topical antihistamine. Two RCTs with 74 patients had follow-up periods of 15 minutes to 4 hours; the remaining 6 RCTs with 473 patients had follow-up periods of 14 days to 4 months. Subjective scoring of symptoms was done in 7 of the 8 studies.

Scores between treatment groups were reported as not statistically significant in the 6 longer-term studies. Meta-analysis wasn’t possible because most studies didn’t tabulate the mean scores and error associated with measures. The 2 short-term studies reported a statistically significant reduction in itching and redness (P<.05) in patients treated with the antihistamine (data not provided).

NSAIDs relieve itching but may sting when applied

A 2007 meta-analysis of 8 RCTs compared topical NSAIDs (ketorolac, diclofenac, aspirin, or steroid) with placebo for treating isolated allergic conjunctivitis in 712 children and adults.² Primary outcomes were measured as subjective reductions in conjunctival injection and itching measured at 2 to 6 weeks using a 0-to-3 severity scale.

Topical NSAIDs produced significantly greater relief of conjunctival itching (4 trials, N=231; mean difference [MD]=-0.54; 95% CI, -0.84 to -0.24) and conjunctival injection (4 trials, N=208; MD=-0.51; 95% CI, -0.97 to -0.05). NSAIDs weren’t superior to placebo in treating other ocular symptoms of eyelid swelling, ocular burning, photophobia, or foreign body sensation, and they had a higher rate of stinging on application (odds ratio=4.0; 95% CI, 2.7-5.9).

Guideline recommends topical antihistamines or mast cell stabilizers

The American Academy of Ophthalmology’s 2012 evidence-based guideline recommends treating allergic conjunctivitis with topical antihistamines (Level A-1 evidence, defined as important evidence supported by at least one RCT or a meta-analysis) and using topical mast cell stabilizers if the condition is recurrent.³

Both of these treatments relieve redness and itching

A 2004 systematic review of 40 RCTs (total N not provided) assessed the efficacy of topical treatment with mast cell stabilizers and antihistamines, comparing each with the other and placebo.¹ Eleven trials that included 899 children and adults compared mast cell stabilizers (sodium cromoglycate, nedocromil, and lodoxamide tromethamine) with placebo. Follow-up periods ranged from 4 to 9 weeks.

Because of study heterogeneity, a random-effects model was used and showed that topical mast cell stabilizers relieved symptoms (ocular itching, burning, and lacrimation) 4.9 times more effectively than placebo (95% confidence interval [CI], 2.5-9.6). Possible publication bias was cited as a limitation.

In the same systematic review, 9 RCTs with 313 patients compared topical antihistamines (levocabastine, azelastine hydrochloride, emedastine, and antazoline phosphate) with placebo. Signs and symptoms (itching, redness, burning, and swelling) were graded using symptom severity scales. Follow-up ranged from 30 minutes to 24 hours. A meta-analysis wasn’t possible because most studies didn’t tabulate the mean scores and error associated with these scores. Most individual studies, however, showed improvement in the cardinal symptom of itchiness.

Finally, 8 RCTs compared topical mast cell stabilizers (sodium cromoglycate, lodoxamide, and nedocromil sodium) with levocabastine, a topical antihistamine. Two RCTs with 74 patients had follow-up periods of 15 minutes to 4 hours; the remaining 6 RCTs with 473 patients had follow-up periods of 14 days to 4 months. Subjective scoring of symptoms was done in 7 of the 8 studies.

Scores between treatment groups were reported as not statistically significant in the 6 longer-term studies. Meta-analysis wasn’t possible because most studies didn’t tabulate the mean scores and error associated with measures. The 2 short-term studies reported a statistically significant reduction in itching and redness (P<.05) in patients treated with the antihistamine (data not provided).

NSAIDs relieve itching but may sting when applied

A 2007 meta-analysis of 8 RCTs compared topical NSAIDs (ketorolac, diclofenac, aspirin, or steroid) with placebo for treating isolated allergic conjunctivitis in 712 children and adults.² Primary outcomes were measured as subjective reductions in conjunctival injection and itching measured at 2 to 6 weeks using a 0-to-3 severity scale.

Topical NSAIDs produced significantly greater relief of conjunctival itching (4 trials, N=231; mean difference [MD]=-0.54; 95% CI, -0.84 to -0.24) and conjunctival injection (4 trials, N=208; MD=-0.51; 95% CI, -0.97 to -0.05). NSAIDs weren’t superior to placebo in treating other ocular symptoms of eyelid swelling, ocular burning, photophobia, or foreign body sensation, and they had a higher rate of stinging on application (odds ratio=4.0; 95% CI, 2.7-5.9).

Guideline recommends topical antihistamines or mast cell stabilizers

The American Academy of Ophthalmology’s 2012 evidence-based guideline recommends treating allergic conjunctivitis with topical antihistamines (Level A-1 evidence, defined as important evidence supported by at least one RCT or a meta-analysis) and using topical mast cell stabilizers if the condition is recurrent.³

PRACTICE CHANGER
Consider prescribing oral naltrexone (50 mg/d) for patients with alcohol use disorder who wish to maintain abstinence after a brief period of detoxification.¹

STRENGTH OF RECOMMENDATION
A: Based on a meta-analysis of 95 randomized controlled trials.¹

ILLUSTRATIVE CASE
Your patient, a 42-year-old man with alcohol use disorder (AUD), detoxifies from alcohol during a recent hospitalization. He doesn’t want to resume drinking but reports frequent cravings. Are there any medications you can prescribe to help prevent relapse?

Excessive alcohol consumption is responsible for one of every 10 deaths among US adults ages 20 to 64.² About 20% to 36% of patients seen in a primary care office have AUD.³ Up to 70% of people who quit with psychosocial support alone will relapse.³

The US Preventive Services Task Force gives a grade B recommendation to screening all adults for AUD, indicating that clinicians should provide this service.⁴ For patients with AUD who wish to abstain but struggle with cravings and relapse, the National Institute on Alcohol Abuse and Alcoholism (NIAAA) recommends considering medication as an adjunct to brief behavioral counseling.⁵

Continue for study summary >>

STUDY SUMMARY
Evidence shows naltrexone can prevent a return to drinking
In a meta-analysis, Jonas et al¹ reviewed 123 studies (N = 22,803) of pharmacotherapy for AUD. After excluding 28 studies (seven were the only study of a given drug, one was a prospective cohort, and 20 had insufficient data), 95 randomized controlled trials were included in the analysis. Twenty-­two were placebo-controlled for acamprosate (1,000 to 3,000 mg/d), 44 for naltrexone (50 mg/d oral, 100 mg/d oral, or injectable) and four compared the two drugs. Additional studies evaluated disulfiram as well as 23 other off-­label medications, such as valproic acid and topiramate.

Two investigators independently reviewed the studies, checking for completeness and accuracy. Studies were also analyzed for bias using predefined criteria; those with high or unclear risk for bias were excluded from the main analysis but included in the sensitivity analysis. Funnel plots showed no evidence of publication bias. 

Participants were primarily recruited as inpatients, and in most studies the mean age was in the 40s. Most patients were diagnosed with alcohol dependence based on criteria in the Diagnostic and Statistical Manual of Mental Disorders, 4th edition, text revision (DSM-IV-TR); this diagnosis translates to likely moderate to severe AUD in DSM-5. Prior to starting medications, participants underwent detoxification or achieved at least three days of sobriety. Most studies included psychosocial intervention in addition to medication, but the types of intervention varied. The duration of the trials ranged from 12 to 52 weeks.

Researchers analyzed five drinking outcomes—return to any drinking, return to heavy drinking (defined as ≥ 4 drinks/d for women and ≥ 5 drinks/d for men), number of drinking days, number of heavy drinking days, and drinks per drinking day. They also evaluated health outcomes (accidents, injuries, quality of life, function, and mortality) and adverse effects.

Acamprosate and oral naltrexone (50 mg/d) significantly decreased return to any drinking, with a number needed to treat (NNT) of 12 for acamprosate and 20 for naltrexone. Oral naltrexone (50 mg/d) also decreased return to heavy drinking (NNT, 12), while acamprosate did not. Neither medication showed a decrease in heavy drinking days.

In a post hoc subgroup analysis of acamprosate for return to any drinking, the drug appeared to be more effective in studies with a higher risk for bias and less effective in studies with a lower risk for bias. The two studies with the lowest risk for bias found no significant effect.

Disulfiram had no effect on any of the outcomes analyzed.

Of the off-label medications, topiramate showed a decrease in drinking days (weighted mean difference [WMD], –6.5%), heavy drinking days (WMD, –9.0%), and drinks per drinking day (WMD, –1.0).

There were no significant differences in health outcomes for any of the medications. Adverse events were greater in treatment groups than placebo groups. Acamprosate was associated with increased risk for diarrhea (number needed to harm [NNH], 11), vomiting (NNH, 42), and anxiety (NNH, 7). Naltrexone was associated with increased risk for nausea (NNH, 9), vomiting (NNH, 24), and dizziness (NNH, 16).

WHAT’S NEW
Consider prescribing naltrexone to prevent relapse
While previous studies suggested that pharmacotherapy could help patients with AUD remain abstinent, this methodologically rigorous meta-analysis compared the efficacy of several commonly used medications and found clear evidence favoring oral nal­trexone. Prescribe oral naltrexone (50 mg/d) to help patients with moderate to severe AUD avoid returning to any drinking or heavy drinking after alcohol detoxification. Acamprosate may also decrease return to drinking, although the evidence is not as strong (the studies with low bias showed no effect).

Next page: Caveats >>

CAVEATS
Medication should be used with psychosocial treatments
Pharmacotherapy for AUD should be reserved for patients who want to quit drinking and should be used in conjunction with psychosocial intervention.³ Only one of the studies analyzed by Jonas et al¹ was conducted in primary care. That said, many of the psychosocial interventions—such as regular follow-up visits to encourage adherence and monitor for adverse effects, in conjunction with attendance at Alcoholics Anonymous meetings—could be done in primary care settings.

Comorbidities may limit therapy options. Naltrexone is contraindicated in acute hepatitis and liver failure and in combination with opioids.⁵ Acamprosate is contraindicated in renal disease.⁵

CHALLENGES TO IMPLEMENTATION
Cost, adherence may be factors for some patients
Perhaps the greatest hurdle in pharmacotherapy for AUD in primary care is a lack of familiarity with these medications. For clinicians who are comfortable with prescribing these medications, implementation may be hindered by a lack of available psychosocial resources for successful abstinence.

Additionally, the medications are expensive. The branded version of naltrexone (50 mg) costs approximately $118 for a 30-day supply,⁶ and the branded version of acamprosate costs approximately $284 for a 30-day supply.⁷

As is the case with any chronic medical condition, medication adherence is a challenge. Naltrexone is taken once daily, while acamprosate is taken three times a day. The risk for relapse is high until six to 12 months of sobriety is achieved and then wanes over several years.⁵ The NIAAA recommends treatment for a minimum of three months.⁵

REFERENCES
1. Jonas DE, Amick HR, Feltner C, et al. Pharmacotherapy for adults with alcohol use disorders in outpatient settings: a systematic review and meta-analysis. JAMA. 2014;311:1889-1900.

2. CDC. Fact sheets - Alcohol use and your health. www.cdc.gov/alcohol/fact-sheets/alcohol-use.htm. Accessed April 13, 2015.

3. Johnson BA. Pharmacotherapy for alcohol use disorder. UpToDate. www.uptodate.com/contents/pharmacotherapy-for-alcohol-use-disorder. Accessed April 13, 2015.

4. US Preventive Services Task Force. Final recommendation statement: Alcohol misuse: Screening and behavioral counseling interventions in primary care. www.uspreventiveservicestaskforce.org/Page/Document/RecommendationStatementFinal/alcohol-misuse-screening-and-behavioral-counseling-interventions-in-primary-care. Accessed April 13, 2015.

5. US Department of Health and Human Services; National Institutes of Health; National Institute on Alcohol Abuse and Alcoholism. Excerpt from Helping Patients Who Drink Too Much: A Clinician’s Guide. http://pubs.niaaa.nih.gov/publications/Practitioner/Clinicians Guide2005/PrescribingMeds.pdf. Accessed April 13, 2015.

6. Drugs.com. Revia prices, coupons and patient assistance programs. www.drugs.com/price-guide/revia. Accessed April 13, 2015.

7. Drugs.com. Campral prices, coupons and patient assistance programs. www.drugs.com/price-guide/campral. Accessed April 13, 2015.

ACKNOWLEDGEMENT
The PURLs Surveillance System was supported in part by Grant Number UL1RR024999 from the National Center For Research Resources, a Clinical Translational Science Award to the University of Chicago. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Center For Research Resources or the National Institutes of Health. 

Copyright © 2015. The Family Physicians Inquiries Network. All rights reserved. 

Reprinted with permission from the Family Physicians Inquiries Network and The Journal of Family Practice. 2015;64(4):238-240.

PRACTICE CHANGER
Consider prescribing oral naltrexone (50 mg/d) for patients with alcohol use disorder who wish to maintain abstinence after a brief period of detoxification.¹

STRENGTH OF RECOMMENDATION
A: Based on a meta-analysis of 95 randomized controlled trials.¹

ILLUSTRATIVE CASE
Your patient, a 42-year-old man with alcohol use disorder (AUD), detoxifies from alcohol during a recent hospitalization. He doesn’t want to resume drinking but reports frequent cravings. Are there any medications you can prescribe to help prevent relapse?

Excessive alcohol consumption is responsible for one of every 10 deaths among US adults ages 20 to 64.² About 20% to 36% of patients seen in a primary care office have AUD.³ Up to 70% of people who quit with psychosocial support alone will relapse.³

The US Preventive Services Task Force gives a grade B recommendation to screening all adults for AUD, indicating that clinicians should provide this service.⁴ For patients with AUD who wish to abstain but struggle with cravings and relapse, the National Institute on Alcohol Abuse and Alcoholism (NIAAA) recommends considering medication as an adjunct to brief behavioral counseling.⁵

Continue for study summary >>

STUDY SUMMARY
Evidence shows naltrexone can prevent a return to drinking
In a meta-analysis, Jonas et al¹ reviewed 123 studies (N = 22,803) of pharmacotherapy for AUD. After excluding 28 studies (seven were the only study of a given drug, one was a prospective cohort, and 20 had insufficient data), 95 randomized controlled trials were included in the analysis. Twenty-­two were placebo-controlled for acamprosate (1,000 to 3,000 mg/d), 44 for naltrexone (50 mg/d oral, 100 mg/d oral, or injectable) and four compared the two drugs. Additional studies evaluated disulfiram as well as 23 other off-­label medications, such as valproic acid and topiramate.

Two investigators independently reviewed the studies, checking for completeness and accuracy. Studies were also analyzed for bias using predefined criteria; those with high or unclear risk for bias were excluded from the main analysis but included in the sensitivity analysis. Funnel plots showed no evidence of publication bias. 

Participants were primarily recruited as inpatients, and in most studies the mean age was in the 40s. Most patients were diagnosed with alcohol dependence based on criteria in the Diagnostic and Statistical Manual of Mental Disorders, 4th edition, text revision (DSM-IV-TR); this diagnosis translates to likely moderate to severe AUD in DSM-5. Prior to starting medications, participants underwent detoxification or achieved at least three days of sobriety. Most studies included psychosocial intervention in addition to medication, but the types of intervention varied. The duration of the trials ranged from 12 to 52 weeks.

Researchers analyzed five drinking outcomes—return to any drinking, return to heavy drinking (defined as ≥ 4 drinks/d for women and ≥ 5 drinks/d for men), number of drinking days, number of heavy drinking days, and drinks per drinking day. They also evaluated health outcomes (accidents, injuries, quality of life, function, and mortality) and adverse effects.

Acamprosate and oral naltrexone (50 mg/d) significantly decreased return to any drinking, with a number needed to treat (NNT) of 12 for acamprosate and 20 for naltrexone. Oral naltrexone (50 mg/d) also decreased return to heavy drinking (NNT, 12), while acamprosate did not. Neither medication showed a decrease in heavy drinking days.

In a post hoc subgroup analysis of acamprosate for return to any drinking, the drug appeared to be more effective in studies with a higher risk for bias and less effective in studies with a lower risk for bias. The two studies with the lowest risk for bias found no significant effect.

Disulfiram had no effect on any of the outcomes analyzed.

Of the off-label medications, topiramate showed a decrease in drinking days (weighted mean difference [WMD], –6.5%), heavy drinking days (WMD, –9.0%), and drinks per drinking day (WMD, –1.0).

There were no significant differences in health outcomes for any of the medications. Adverse events were greater in treatment groups than placebo groups. Acamprosate was associated with increased risk for diarrhea (number needed to harm [NNH], 11), vomiting (NNH, 42), and anxiety (NNH, 7). Naltrexone was associated with increased risk for nausea (NNH, 9), vomiting (NNH, 24), and dizziness (NNH, 16).

WHAT’S NEW
Consider prescribing naltrexone to prevent relapse
While previous studies suggested that pharmacotherapy could help patients with AUD remain abstinent, this methodologically rigorous meta-analysis compared the efficacy of several commonly used medications and found clear evidence favoring oral nal­trexone. Prescribe oral naltrexone (50 mg/d) to help patients with moderate to severe AUD avoid returning to any drinking or heavy drinking after alcohol detoxification. Acamprosate may also decrease return to drinking, although the evidence is not as strong (the studies with low bias showed no effect).

Next page: Caveats >>

CAVEATS
Medication should be used with psychosocial treatments
Pharmacotherapy for AUD should be reserved for patients who want to quit drinking and should be used in conjunction with psychosocial intervention.³ Only one of the studies analyzed by Jonas et al¹ was conducted in primary care. That said, many of the psychosocial interventions—such as regular follow-up visits to encourage adherence and monitor for adverse effects, in conjunction with attendance at Alcoholics Anonymous meetings—could be done in primary care settings.

Comorbidities may limit therapy options. Naltrexone is contraindicated in acute hepatitis and liver failure and in combination with opioids.⁵ Acamprosate is contraindicated in renal disease.⁵

CHALLENGES TO IMPLEMENTATION
Cost, adherence may be factors for some patients
Perhaps the greatest hurdle in pharmacotherapy for AUD in primary care is a lack of familiarity with these medications. For clinicians who are comfortable with prescribing these medications, implementation may be hindered by a lack of available psychosocial resources for successful abstinence.

Additionally, the medications are expensive. The branded version of naltrexone (50 mg) costs approximately $118 for a 30-day supply,⁶ and the branded version of acamprosate costs approximately $284 for a 30-day supply.⁷

As is the case with any chronic medical condition, medication adherence is a challenge. Naltrexone is taken once daily, while acamprosate is taken three times a day. The risk for relapse is high until six to 12 months of sobriety is achieved and then wanes over several years.⁵ The NIAAA recommends treatment for a minimum of three months.⁵

REFERENCES
1. Jonas DE, Amick HR, Feltner C, et al. Pharmacotherapy for adults with alcohol use disorders in outpatient settings: a systematic review and meta-analysis. JAMA. 2014;311:1889-1900.

2. CDC. Fact sheets - Alcohol use and your health. www.cdc.gov/alcohol/fact-sheets/alcohol-use.htm. Accessed April 13, 2015.

3. Johnson BA. Pharmacotherapy for alcohol use disorder. UpToDate. www.uptodate.com/contents/pharmacotherapy-for-alcohol-use-disorder. Accessed April 13, 2015.

4. US Preventive Services Task Force. Final recommendation statement: Alcohol misuse: Screening and behavioral counseling interventions in primary care. www.uspreventiveservicestaskforce.org/Page/Document/RecommendationStatementFinal/alcohol-misuse-screening-and-behavioral-counseling-interventions-in-primary-care. Accessed April 13, 2015.

5. US Department of Health and Human Services; National Institutes of Health; National Institute on Alcohol Abuse and Alcoholism. Excerpt from Helping Patients Who Drink Too Much: A Clinician’s Guide. http://pubs.niaaa.nih.gov/publications/Practitioner/Clinicians Guide2005/PrescribingMeds.pdf. Accessed April 13, 2015.

6. Drugs.com. Revia prices, coupons and patient assistance programs. www.drugs.com/price-guide/revia. Accessed April 13, 2015.

7. Drugs.com. Campral prices, coupons and patient assistance programs. www.drugs.com/price-guide/campral. Accessed April 13, 2015.

ACKNOWLEDGEMENT
The PURLs Surveillance System was supported in part by Grant Number UL1RR024999 from the National Center For Research Resources, a Clinical Translational Science Award to the University of Chicago. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Center For Research Resources or the National Institutes of Health. 

Copyright © 2015. The Family Physicians Inquiries Network. All rights reserved. 

Reprinted with permission from the Family Physicians Inquiries Network and The Journal of Family Practice. 2015;64(4):238-240.

PRACTICE CHANGER
Consider prescribing oral naltrexone (50 mg/d) for patients with alcohol use disorder who wish to maintain abstinence after a brief period of detoxification.¹

STRENGTH OF RECOMMENDATION
A: Based on a meta-analysis of 95 randomized controlled trials.¹

ILLUSTRATIVE CASE
Your patient, a 42-year-old man with alcohol use disorder (AUD), detoxifies from alcohol during a recent hospitalization. He doesn’t want to resume drinking but reports frequent cravings. Are there any medications you can prescribe to help prevent relapse?

Excessive alcohol consumption is responsible for one of every 10 deaths among US adults ages 20 to 64.² About 20% to 36% of patients seen in a primary care office have AUD.³ Up to 70% of people who quit with psychosocial support alone will relapse.³

The US Preventive Services Task Force gives a grade B recommendation to screening all adults for AUD, indicating that clinicians should provide this service.⁴ For patients with AUD who wish to abstain but struggle with cravings and relapse, the National Institute on Alcohol Abuse and Alcoholism (NIAAA) recommends considering medication as an adjunct to brief behavioral counseling.⁵

Continue for study summary >>

STUDY SUMMARY
Evidence shows naltrexone can prevent a return to drinking
In a meta-analysis, Jonas et al¹ reviewed 123 studies (N = 22,803) of pharmacotherapy for AUD. After excluding 28 studies (seven were the only study of a given drug, one was a prospective cohort, and 20 had insufficient data), 95 randomized controlled trials were included in the analysis. Twenty-­two were placebo-controlled for acamprosate (1,000 to 3,000 mg/d), 44 for naltrexone (50 mg/d oral, 100 mg/d oral, or injectable) and four compared the two drugs. Additional studies evaluated disulfiram as well as 23 other off-­label medications, such as valproic acid and topiramate.

Two investigators independently reviewed the studies, checking for completeness and accuracy. Studies were also analyzed for bias using predefined criteria; those with high or unclear risk for bias were excluded from the main analysis but included in the sensitivity analysis. Funnel plots showed no evidence of publication bias. 

Participants were primarily recruited as inpatients, and in most studies the mean age was in the 40s. Most patients were diagnosed with alcohol dependence based on criteria in the Diagnostic and Statistical Manual of Mental Disorders, 4th edition, text revision (DSM-IV-TR); this diagnosis translates to likely moderate to severe AUD in DSM-5. Prior to starting medications, participants underwent detoxification or achieved at least three days of sobriety. Most studies included psychosocial intervention in addition to medication, but the types of intervention varied. The duration of the trials ranged from 12 to 52 weeks.

Researchers analyzed five drinking outcomes—return to any drinking, return to heavy drinking (defined as ≥ 4 drinks/d for women and ≥ 5 drinks/d for men), number of drinking days, number of heavy drinking days, and drinks per drinking day. They also evaluated health outcomes (accidents, injuries, quality of life, function, and mortality) and adverse effects.

Acamprosate and oral naltrexone (50 mg/d) significantly decreased return to any drinking, with a number needed to treat (NNT) of 12 for acamprosate and 20 for naltrexone. Oral naltrexone (50 mg/d) also decreased return to heavy drinking (NNT, 12), while acamprosate did not. Neither medication showed a decrease in heavy drinking days.

In a post hoc subgroup analysis of acamprosate for return to any drinking, the drug appeared to be more effective in studies with a higher risk for bias and less effective in studies with a lower risk for bias. The two studies with the lowest risk for bias found no significant effect.

Disulfiram had no effect on any of the outcomes analyzed.

Of the off-label medications, topiramate showed a decrease in drinking days (weighted mean difference [WMD], –6.5%), heavy drinking days (WMD, –9.0%), and drinks per drinking day (WMD, –1.0).

There were no significant differences in health outcomes for any of the medications. Adverse events were greater in treatment groups than placebo groups. Acamprosate was associated with increased risk for diarrhea (number needed to harm [NNH], 11), vomiting (NNH, 42), and anxiety (NNH, 7). Naltrexone was associated with increased risk for nausea (NNH, 9), vomiting (NNH, 24), and dizziness (NNH, 16).

WHAT’S NEW
Consider prescribing naltrexone to prevent relapse
While previous studies suggested that pharmacotherapy could help patients with AUD remain abstinent, this methodologically rigorous meta-analysis compared the efficacy of several commonly used medications and found clear evidence favoring oral nal­trexone. Prescribe oral naltrexone (50 mg/d) to help patients with moderate to severe AUD avoid returning to any drinking or heavy drinking after alcohol detoxification. Acamprosate may also decrease return to drinking, although the evidence is not as strong (the studies with low bias showed no effect).

Next page: Caveats >>

CAVEATS
Medication should be used with psychosocial treatments
Pharmacotherapy for AUD should be reserved for patients who want to quit drinking and should be used in conjunction with psychosocial intervention.³ Only one of the studies analyzed by Jonas et al¹ was conducted in primary care. That said, many of the psychosocial interventions—such as regular follow-up visits to encourage adherence and monitor for adverse effects, in conjunction with attendance at Alcoholics Anonymous meetings—could be done in primary care settings.

Comorbidities may limit therapy options. Naltrexone is contraindicated in acute hepatitis and liver failure and in combination with opioids.⁵ Acamprosate is contraindicated in renal disease.⁵

CHALLENGES TO IMPLEMENTATION
Cost, adherence may be factors for some patients
Perhaps the greatest hurdle in pharmacotherapy for AUD in primary care is a lack of familiarity with these medications. For clinicians who are comfortable with prescribing these medications, implementation may be hindered by a lack of available psychosocial resources for successful abstinence.

Additionally, the medications are expensive. The branded version of naltrexone (50 mg) costs approximately $118 for a 30-day supply,⁶ and the branded version of acamprosate costs approximately $284 for a 30-day supply.⁷

As is the case with any chronic medical condition, medication adherence is a challenge. Naltrexone is taken once daily, while acamprosate is taken three times a day. The risk for relapse is high until six to 12 months of sobriety is achieved and then wanes over several years.⁵ The NIAAA recommends treatment for a minimum of three months.⁵

REFERENCES
1. Jonas DE, Amick HR, Feltner C, et al. Pharmacotherapy for adults with alcohol use disorders in outpatient settings: a systematic review and meta-analysis. JAMA. 2014;311:1889-1900.

2. CDC. Fact sheets - Alcohol use and your health. www.cdc.gov/alcohol/fact-sheets/alcohol-use.htm. Accessed April 13, 2015.

3. Johnson BA. Pharmacotherapy for alcohol use disorder. UpToDate. www.uptodate.com/contents/pharmacotherapy-for-alcohol-use-disorder. Accessed April 13, 2015.

4. US Preventive Services Task Force. Final recommendation statement: Alcohol misuse: Screening and behavioral counseling interventions in primary care. www.uspreventiveservicestaskforce.org/Page/Document/RecommendationStatementFinal/alcohol-misuse-screening-and-behavioral-counseling-interventions-in-primary-care. Accessed April 13, 2015.

5. US Department of Health and Human Services; National Institutes of Health; National Institute on Alcohol Abuse and Alcoholism. Excerpt from Helping Patients Who Drink Too Much: A Clinician’s Guide. http://pubs.niaaa.nih.gov/publications/Practitioner/Clinicians Guide2005/PrescribingMeds.pdf. Accessed April 13, 2015.

6. Drugs.com. Revia prices, coupons and patient assistance programs. www.drugs.com/price-guide/revia. Accessed April 13, 2015.

7. Drugs.com. Campral prices, coupons and patient assistance programs. www.drugs.com/price-guide/campral. Accessed April 13, 2015.

ACKNOWLEDGEMENT
The PURLs Surveillance System was supported in part by Grant Number UL1RR024999 from the National Center For Research Resources, a Clinical Translational Science Award to the University of Chicago. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Center For Research Resources or the National Institutes of Health. 

Copyright © 2015. The Family Physicians Inquiries Network. All rights reserved. 

Reprinted with permission from the Family Physicians Inquiries Network and The Journal of Family Practice. 2015;64(4):238-240.

Both approaches resulted in function and pain improvement

A 2013 multicenter RCT evaluated 351 adults, 45 years and older, with a meniscal tear and mild to moderate osteoarthritis confirmed by imaging, for functional improvement by physical therapy alone compared with arthroscopic partial meniscectomy and physical therapy.¹

At the beginning of the study and 6 and 12 months after treatment, researchers assessed symptoms using the Western Ontario and McMaster Universities (WOMAC) Osteoarthritis Index physical-function score (0-100, with higher scores indicating more severe symptoms), the Knee Injury and Osteoarthritis Outcome Score (KOOS) for pain (0-100, with higher numbers correlating with less pain), and the 36-item Short Form Health Survey (SF-36) for physical activity (0-100, with higher scores indicating greater physical activity).

Modified intention to treat analysis showed no significant difference in function and pain improvement at 6 and 12 months between patients with meniscal injury who underwent arthroscopic repair and physical therapy and patients who underwent physical therapy alone (TABLE¹). A limitation of the study was the crossover of 30% of patients from the nonoperative group to the operative group.

No differences found in Tx outcomes for nontraumatic tears

A 2007 prospective RCT evaluated 90 adults ages 45 to 64 with nontraumatic meniscal tears confirmed by magnetic resonance imaging for improvement in knee pain and function with arthroscopic treatment and supervised exercise (AE) or supervised exercise (E) alone.² Knee pain and function were assessed before intervention, after 8 weeks, and after 6 months of treatment using 3 surveys: the KOOS, the Lysholm Knee Scoring Scale (LKSS; 0-100, with higher scores correlating with good knee function), and the Visual Analogue Scale (VAS) for knee pain (0-10, with 0 indicating no pain and 10 indicating maximum pain).

The KOOS revealed that at 8 weeks and 6 months both groups had significant improvement from the initial evaluation in all subscale scores. In the AE group, the 8-week pain score increased from a baseline of 56 to 89 (P<.001) and remained at 89 at 6 months (P<.001). For the E group, the 8-week pain score improved from a baseline of 62 to 86 (P<.001) and continued at 86 after 6 months (P<.001).

The LKSS score for both groups showed significant improvement from baseline at 8 weeks: 34% of the AE group and 42% of the E group scored higher than 91 (P<.001).

VAS scores showed a significant decrease in pain at 8 weeks for both the AE and E groups: beginning median value for both groups was 5.5 and decreased to 1.0 at 8 weeks and 6 months (P<.001).

The authors concluded that both groups improved significantly from initial evaluation regardless of treatment method and that no statistically significant difference existed between treatment results.

Both approaches resulted in function and pain improvement

A 2013 multicenter RCT evaluated 351 adults, 45 years and older, with a meniscal tear and mild to moderate osteoarthritis confirmed by imaging, for functional improvement by physical therapy alone compared with arthroscopic partial meniscectomy and physical therapy.¹

At the beginning of the study and 6 and 12 months after treatment, researchers assessed symptoms using the Western Ontario and McMaster Universities (WOMAC) Osteoarthritis Index physical-function score (0-100, with higher scores indicating more severe symptoms), the Knee Injury and Osteoarthritis Outcome Score (KOOS) for pain (0-100, with higher numbers correlating with less pain), and the 36-item Short Form Health Survey (SF-36) for physical activity (0-100, with higher scores indicating greater physical activity).

Modified intention to treat analysis showed no significant difference in function and pain improvement at 6 and 12 months between patients with meniscal injury who underwent arthroscopic repair and physical therapy and patients who underwent physical therapy alone (TABLE¹). A limitation of the study was the crossover of 30% of patients from the nonoperative group to the operative group.

No differences found in Tx outcomes for nontraumatic tears

A 2007 prospective RCT evaluated 90 adults ages 45 to 64 with nontraumatic meniscal tears confirmed by magnetic resonance imaging for improvement in knee pain and function with arthroscopic treatment and supervised exercise (AE) or supervised exercise (E) alone.² Knee pain and function were assessed before intervention, after 8 weeks, and after 6 months of treatment using 3 surveys: the KOOS, the Lysholm Knee Scoring Scale (LKSS; 0-100, with higher scores correlating with good knee function), and the Visual Analogue Scale (VAS) for knee pain (0-10, with 0 indicating no pain and 10 indicating maximum pain).

The KOOS revealed that at 8 weeks and 6 months both groups had significant improvement from the initial evaluation in all subscale scores. In the AE group, the 8-week pain score increased from a baseline of 56 to 89 (P<.001) and remained at 89 at 6 months (P<.001). For the E group, the 8-week pain score improved from a baseline of 62 to 86 (P<.001) and continued at 86 after 6 months (P<.001).

The LKSS score for both groups showed significant improvement from baseline at 8 weeks: 34% of the AE group and 42% of the E group scored higher than 91 (P<.001).

VAS scores showed a significant decrease in pain at 8 weeks for both the AE and E groups: beginning median value for both groups was 5.5 and decreased to 1.0 at 8 weeks and 6 months (P<.001).

The authors concluded that both groups improved significantly from initial evaluation regardless of treatment method and that no statistically significant difference existed between treatment results.

Both approaches resulted in function and pain improvement

A 2013 multicenter RCT evaluated 351 adults, 45 years and older, with a meniscal tear and mild to moderate osteoarthritis confirmed by imaging, for functional improvement by physical therapy alone compared with arthroscopic partial meniscectomy and physical therapy.¹

At the beginning of the study and 6 and 12 months after treatment, researchers assessed symptoms using the Western Ontario and McMaster Universities (WOMAC) Osteoarthritis Index physical-function score (0-100, with higher scores indicating more severe symptoms), the Knee Injury and Osteoarthritis Outcome Score (KOOS) for pain (0-100, with higher numbers correlating with less pain), and the 36-item Short Form Health Survey (SF-36) for physical activity (0-100, with higher scores indicating greater physical activity).

Modified intention to treat analysis showed no significant difference in function and pain improvement at 6 and 12 months between patients with meniscal injury who underwent arthroscopic repair and physical therapy and patients who underwent physical therapy alone (TABLE¹). A limitation of the study was the crossover of 30% of patients from the nonoperative group to the operative group.

No differences found in Tx outcomes for nontraumatic tears

A 2007 prospective RCT evaluated 90 adults ages 45 to 64 with nontraumatic meniscal tears confirmed by magnetic resonance imaging for improvement in knee pain and function with arthroscopic treatment and supervised exercise (AE) or supervised exercise (E) alone.² Knee pain and function were assessed before intervention, after 8 weeks, and after 6 months of treatment using 3 surveys: the KOOS, the Lysholm Knee Scoring Scale (LKSS; 0-100, with higher scores correlating with good knee function), and the Visual Analogue Scale (VAS) for knee pain (0-10, with 0 indicating no pain and 10 indicating maximum pain).

The KOOS revealed that at 8 weeks and 6 months both groups had significant improvement from the initial evaluation in all subscale scores. In the AE group, the 8-week pain score increased from a baseline of 56 to 89 (P<.001) and remained at 89 at 6 months (P<.001). For the E group, the 8-week pain score improved from a baseline of 62 to 86 (P<.001) and continued at 86 after 6 months (P<.001).

The LKSS score for both groups showed significant improvement from baseline at 8 weeks: 34% of the AE group and 42% of the E group scored higher than 91 (P<.001).

VAS scores showed a significant decrease in pain at 8 weeks for both the AE and E groups: beginning median value for both groups was 5.5 and decreased to 1.0 at 8 weeks and 6 months (P<.001).

The authors concluded that both groups improved significantly from initial evaluation regardless of treatment method and that no statistically significant difference existed between treatment results.

ANSWER
The correct answer is nevus sebaceous (choice “a”). This benign hamartomatous lesion is derived from local tissue and grows at the same rate.

It differs considerably from the other items in the differential, including aplasia cutis congenita (choice “b”). In this condition, a focal area of epidermis simply fails to develop, leaving a permanent hairless scar that contrasts sharply with the raised, mammillated plaque of nevus sebaceous.

Epidermal nevus (choice “c”) is usually a collection of tan to brown superficial nevoid papules that can be linear, agminated, or plaque-like. These lesions lack the color and mammillated surface of those seen in nevus sebaceous.

Neonatal lupus (choice “d”) can present at birth with hairless, cicatricial inflamed lesions. However, these tend to resolve quickly, often leaving focal scarring alopecia but no plaque formation.

DISCUSSION
Nevus sebaceous (NS), first described by Jadassohn in 1895, has long been recognized as an unusual but by no means rare congenital lesion. Occurring equally in both sexes and comprising ­sebaceous glands in a nevoid morphologic context, NS is considered a variant of sebaceous nevi and verrucous epidermal nevi in some circles. All three are derived from overgrowth of local, normal tissues that typically grow at the same rate as surrounding structures.

The vast majority of NS lesions are found in the scalp, although they can also develop on the ear or neck and, rarely, elsewhere on the body. This patient’s plaque—with its uniform surface; tiny, smooth, shiny papules; and (perhaps most important) total lack of hair—is typical. Other classic features are congenital onset and permanent nature, which distinguish them from the rest of the differential.

Focal malignant transformation of NS lesions has been reported—in fact, this author has seen two such cases in 30 years. Both were small basal cell carcinomas, although cases of melanoma and other malignancies have been reported.

Such changes are rare enough that most experts consider prophylactic removal to be unwarranted. Watching the lesions for change over the years is certainly reasonable, as is protecting them from sun exposure.

Surgical removal—usually performed by a plastic surgeon—is occasionally necessary for cosmetic reasons. This is particularly so when NS covers a portion of the face, or when the cosmetic implications of having a hairless plaque in the scalp are sufficiently distressing.

This patient and her parents were educated about the nature of the diagnosis and apprised of their options.

Editor's note: For a similar presentation with a very different diagnosis, see the March 2015 DermaDiagnosis case (http://bit.ly/1ye69Ym).

ANSWER
The correct answer is nevus sebaceous (choice “a”). This benign hamartomatous lesion is derived from local tissue and grows at the same rate.

It differs considerably from the other items in the differential, including aplasia cutis congenita (choice “b”). In this condition, a focal area of epidermis simply fails to develop, leaving a permanent hairless scar that contrasts sharply with the raised, mammillated plaque of nevus sebaceous.

Epidermal nevus (choice “c”) is usually a collection of tan to brown superficial nevoid papules that can be linear, agminated, or plaque-like. These lesions lack the color and mammillated surface of those seen in nevus sebaceous.

Neonatal lupus (choice “d”) can present at birth with hairless, cicatricial inflamed lesions. However, these tend to resolve quickly, often leaving focal scarring alopecia but no plaque formation.

DISCUSSION
Nevus sebaceous (NS), first described by Jadassohn in 1895, has long been recognized as an unusual but by no means rare congenital lesion. Occurring equally in both sexes and comprising ­sebaceous glands in a nevoid morphologic context, NS is considered a variant of sebaceous nevi and verrucous epidermal nevi in some circles. All three are derived from overgrowth of local, normal tissues that typically grow at the same rate as surrounding structures.

The vast majority of NS lesions are found in the scalp, although they can also develop on the ear or neck and, rarely, elsewhere on the body. This patient’s plaque—with its uniform surface; tiny, smooth, shiny papules; and (perhaps most important) total lack of hair—is typical. Other classic features are congenital onset and permanent nature, which distinguish them from the rest of the differential.

Focal malignant transformation of NS lesions has been reported—in fact, this author has seen two such cases in 30 years. Both were small basal cell carcinomas, although cases of melanoma and other malignancies have been reported.

Such changes are rare enough that most experts consider prophylactic removal to be unwarranted. Watching the lesions for change over the years is certainly reasonable, as is protecting them from sun exposure.

Surgical removal—usually performed by a plastic surgeon—is occasionally necessary for cosmetic reasons. This is particularly so when NS covers a portion of the face, or when the cosmetic implications of having a hairless plaque in the scalp are sufficiently distressing.

This patient and her parents were educated about the nature of the diagnosis and apprised of their options.

Editor's note: For a similar presentation with a very different diagnosis, see the March 2015 DermaDiagnosis case (http://bit.ly/1ye69Ym).

ANSWER
The correct answer is nevus sebaceous (choice “a”). This benign hamartomatous lesion is derived from local tissue and grows at the same rate.

It differs considerably from the other items in the differential, including aplasia cutis congenita (choice “b”). In this condition, a focal area of epidermis simply fails to develop, leaving a permanent hairless scar that contrasts sharply with the raised, mammillated plaque of nevus sebaceous.

Epidermal nevus (choice “c”) is usually a collection of tan to brown superficial nevoid papules that can be linear, agminated, or plaque-like. These lesions lack the color and mammillated surface of those seen in nevus sebaceous.

Neonatal lupus (choice “d”) can present at birth with hairless, cicatricial inflamed lesions. However, these tend to resolve quickly, often leaving focal scarring alopecia but no plaque formation.

DISCUSSION
Nevus sebaceous (NS), first described by Jadassohn in 1895, has long been recognized as an unusual but by no means rare congenital lesion. Occurring equally in both sexes and comprising ­sebaceous glands in a nevoid morphologic context, NS is considered a variant of sebaceous nevi and verrucous epidermal nevi in some circles. All three are derived from overgrowth of local, normal tissues that typically grow at the same rate as surrounding structures.

The vast majority of NS lesions are found in the scalp, although they can also develop on the ear or neck and, rarely, elsewhere on the body. This patient’s plaque—with its uniform surface; tiny, smooth, shiny papules; and (perhaps most important) total lack of hair—is typical. Other classic features are congenital onset and permanent nature, which distinguish them from the rest of the differential.

Focal malignant transformation of NS lesions has been reported—in fact, this author has seen two such cases in 30 years. Both were small basal cell carcinomas, although cases of melanoma and other malignancies have been reported.

Such changes are rare enough that most experts consider prophylactic removal to be unwarranted. Watching the lesions for change over the years is certainly reasonable, as is protecting them from sun exposure.

Surgical removal—usually performed by a plastic surgeon—is occasionally necessary for cosmetic reasons. This is particularly so when NS covers a portion of the face, or when the cosmetic implications of having a hairless plaque in the scalp are sufficiently distressing.

This patient and her parents were educated about the nature of the diagnosis and apprised of their options.

Editor's note: For a similar presentation with a very different diagnosis, see the March 2015 DermaDiagnosis case (http://bit.ly/1ye69Ym).