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Emergency And Acute Medicine – Hellp Syndrome
Basics And Description
HELLP syndrome is defined by hemolysis, elevated liver enzymes, and low platelet count. It exists on a continuum with severe preeclampsia, as most affected patients are hypertensive, although a subset may have normal blood pressure. Hepatic involvement is the hallmark feature, but other organs such as the brain, kidneys, and lungs may also be affected.
The condition is categorized into three severity classes based on the platelet nadir. Class 1 represents the most severe form with platelet counts below 50,000/μL and accounts for most maternal deaths. Class 2 involves platelet counts between 50,000 and 100,000/μL, while Class 3 is the least severe with platelet counts between 100,000 and 150,000/μL. Increased mortality is associated with hepatic hemorrhage and central nervous system or cardiopulmonary complications. HELLP syndrome occurs in approximately 0.2% of all pregnancies, affects 20% of pregnancies with severe preeclampsia or eclampsia, and may present antepartum, intrapartum, or postpartum.
Risk Factors And Epidemiology
Patients are often white, multiparous, and of older maternal age. Infant mortality is higher in pregnancies complicated by HELLP syndrome.
Etiology And Pathophysiology
The exact cause is unclear, but widespread vasospasm is central to disease development. Fetal–placental debris enters the maternal circulation, triggering a systemic inflammatory response. Vasoconstriction increases vascular resistance and contributes to hypertension. Endothelial injury leads to intercellular leakage, platelet activation, fibrin deposition, and microvascular thrombosis. These processes result in tissue hypoxia, hemorrhage, necrosis, and end-organ damage.
Clinical Presentation And Symptoms
Patients may present with nonspecific flulike symptoms including fatigue and malaise. Common complaints include nausea, vomiting, right upper quadrant or epigastric pain that worsens with disease severity, and headache often accompanied by visual changes.
High-risk symptoms include dyspnea or signs of fluid overload suggesting pulmonary edema, chest pain concerning for myocardial ischemia, altered mental status, seizures, focal neurologic deficits, peripheral edema, ascites, hematuria, and reduced urine output. Determining gestational age and fetal viability is critical.
Physical Examination Findings
Vital signs require close attention to blood pressure, which may be normal or elevated. Right upper quadrant tenderness may suggest hepatic involvement or subcapsular hematoma. Signs of fluid overload, neurologic abnormalities, and fetal heart tones should be carefully assessed.
Essential Initial Evaluation
Immediate laboratory evaluation includes a complete blood count with platelet count and smear, liver function tests, blood urea nitrogen, creatinine, coagulation profile, magnesium level, and urinalysis for protein. Patient weight should be recorded to assess recent fluid shifts.
Diagnostic Tests And Interpretation
Laboratory findings typically reveal anemia, thrombocytopenia, and evidence of microangiopathic hemolytic anemia on peripheral smear. Elevated lactate dehydrogenase, bilirubin, and reticulocyte count indicate hemolysis. Liver enzymes are elevated, commonly with AST and ALT above 40 IU/L, LDH above 600 IU/L, and bilirubin above 1.2 mg/dL.
Imaging may include chest radiography for suspected pulmonary edema, head CT for altered mental status or focal deficits, and pelvic ultrasound to assess fetal and placental status.
Differential Diagnosis
Gastrointestinal conditions include cholecystitis, pancreatitis, hepatitis, acute fatty liver of pregnancy, and severe reflux disease. Hematologic considerations include gestational thrombocytopenia, idiopathic thrombocytopenic purpura, thrombotic thrombocytopenic purpura, and hemolytic uremic syndrome. Neurologic causes include epilepsy, meningitis, encephalopathy, brain tumor, or intracranial hemorrhage. Other considerations include sepsis, pyelonephritis, and substance use.
Prehospital And Early Management Considerations
Patients should be transported in the left lateral decubitus position to avoid inferior vena cava compression. IV access should be established, and seizures managed preferentially with magnesium sulfate. Transport to a facility capable of high-risk obstetric care is essential.
Initial Stabilization And Monitoring
Management begins with airway, breathing, and circulation support. High-flow oxygen is administered, and maternal cardiac and oxygen monitoring initiated. Continuous fetal monitoring and uterine activity assessment are required.
Emergency Department Management
Hypertension is controlled with appropriate antihypertensives while avoiding ACE inhibitors. Magnesium sulfate is administered for seizure prophylaxis or treatment, not for blood pressure control. Obstetric consultation is mandatory, with consideration of neonatology involvement and emergent delivery when indicated.
IV fluids are restricted to prevent worsening capillary leak, typically limited to 60 mL/hr unless dehydration is present. Platelet transfusion is indicated for counts below 20,000/μL or higher thresholds if delivery or surgery is planned. Blood products are administered as needed for anemia or coagulopathy.
Medications
First-line antihypertensives include IV hydralazine or labetalol, titrated to maintain diastolic blood pressure below 110 mm Hg. Magnesium sulfate is given as a loading dose followed by continuous infusion, with monitoring for toxicity. Nitroprusside is reserved for refractory hypertension. Calcium gluconate is available as an antidote for magnesium toxicity.
Disposition And Follow-Up
All patients with HELLP syndrome require admission to an obstetric service for continuous maternal and fetal monitoring. ICU admission is indicated for severe complications such as pulmonary edema, respiratory failure, cerebral edema, or hemodynamic instability. Discharge decisions are made exclusively by obstetric specialists.
Clinical Pearls And Common Errors
Pregnant patients with abdominal pain, elevated liver enzymes, and thrombocytopenia require immediate evaluation for HELLP syndrome, even in the absence of hypertension. Normal blood pressure does not exclude the diagnosis. Prompt stabilization and transfer to a high-risk obstetric facility are critical to reducing maternal and fetal morbidity and mortality.
Basics And Description
HELLP syndrome is defined by hemolysis, elevated liver enzymes, and low platelet count. It exists on a continuum with severe preeclampsia, as most affected patients are hypertensive, although a subset may have normal blood pressure. Hepatic involvement is the hallmark feature, but other organs such as the brain, kidneys, and lungs may also be affected.
The condition is categorized into three severity classes based on the platelet nadir. Class 1 represents the most severe form with platelet counts below 50,000/μL and accounts for most maternal deaths. Class 2 involves platelet counts between 50,000 and 100,000/μL, while Class 3 is the least severe with platelet counts between 100,000 and 150,000/μL. Increased mortality is associated with hepatic hemorrhage and central nervous system or cardiopulmonary complications. HELLP syndrome occurs in approximately 0.2% of all pregnancies, affects 20% of pregnancies with severe preeclampsia or eclampsia, and may present antepartum, intrapartum, or postpartum.
Risk Factors And Epidemiology
Patients are often white, multiparous, and of older maternal age. Infant mortality is higher in pregnancies complicated by HELLP syndrome.
Etiology And Pathophysiology
The exact cause is unclear, but widespread vasospasm is central to disease development. Fetal–placental debris enters the maternal circulation, triggering a systemic inflammatory response. Vasoconstriction increases vascular resistance and contributes to hypertension. Endothelial injury leads to intercellular leakage, platelet activation, fibrin deposition, and microvascular thrombosis. These processes result in tissue hypoxia, hemorrhage, necrosis, and end-organ damage.
Clinical Presentation And Symptoms
Patients may present with nonspecific flulike symptoms including fatigue and malaise. Common complaints include nausea, vomiting, right upper quadrant or epigastric pain that worsens with disease severity, and headache often accompanied by visual changes.
High-risk symptoms include dyspnea or signs of fluid overload suggesting pulmonary edema, chest pain concerning for myocardial ischemia, altered mental status, seizures, focal neurologic deficits, peripheral edema, ascites, hematuria, and reduced urine output. Determining gestational age and fetal viability is critical.
Physical Examination Findings
Vital signs require close attention to blood pressure, which may be normal or elevated. Right upper quadrant tenderness may suggest hepatic involvement or subcapsular hematoma. Signs of fluid overload, neurologic abnormalities, and fetal heart tones should be carefully assessed.
Essential Initial Evaluation
Immediate laboratory evaluation includes a complete blood count with platelet count and smear, liver function tests, blood urea nitrogen, creatinine, coagulation profile, magnesium level, and urinalysis for protein. Patient weight should be recorded to assess recent fluid shifts.
Diagnostic Tests And Interpretation
Laboratory findings typically reveal anemia, thrombocytopenia, and evidence of microangiopathic hemolytic anemia on peripheral smear. Elevated lactate dehydrogenase, bilirubin, and reticulocyte count indicate hemolysis. Liver enzymes are elevated, commonly with AST and ALT above 40 IU/L, LDH above 600 IU/L, and bilirubin above 1.2 mg/dL.
Imaging may include chest radiography for suspected pulmonary edema, head CT for altered mental status or focal deficits, and pelvic ultrasound to assess fetal and placental status.
Differential Diagnosis
Gastrointestinal conditions include cholecystitis, pancreatitis, hepatitis, acute fatty liver of pregnancy, and severe reflux disease. Hematologic considerations include gestational thrombocytopenia, idiopathic thrombocytopenic purpura, thrombotic thrombocytopenic purpura, and hemolytic uremic syndrome. Neurologic causes include epilepsy, meningitis, encephalopathy, brain tumor, or intracranial hemorrhage. Other considerations include sepsis, pyelonephritis, and substance use.
Prehospital And Early Management Considerations
Patients should be transported in the left lateral decubitus position to avoid inferior vena cava compression. IV access should be established, and seizures managed preferentially with magnesium sulfate. Transport to a facility capable of high-risk obstetric care is essential.
Initial Stabilization And Monitoring
Management begins with airway, breathing, and circulation support. High-flow oxygen is administered, and maternal cardiac and oxygen monitoring initiated. Continuous fetal monitoring and uterine activity assessment are required.
Emergency Department Management
Hypertension is controlled with appropriate antihypertensives while avoiding ACE inhibitors. Magnesium sulfate is administered for seizure prophylaxis or treatment, not for blood pressure control. Obstetric consultation is mandatory, with consideration of neonatology involvement and emergent delivery when indicated.
IV fluids are restricted to prevent worsening capillary leak, typically limited to 60 mL/hr unless dehydration is present. Platelet transfusion is indicated for counts below 20,000/μL or higher thresholds if delivery or surgery is planned. Blood products are administered as needed for anemia or coagulopathy.
Medications
First-line antihypertensives include IV hydralazine or labetalol, titrated to maintain diastolic blood pressure below 110 mm Hg. Magnesium sulfate is given as a loading dose followed by continuous infusion, with monitoring for toxicity. Nitroprusside is reserved for refractory hypertension. Calcium gluconate is available as an antidote for magnesium toxicity.
Disposition And Follow-Up
All patients with HELLP syndrome require admission to an obstetric service for continuous maternal and fetal monitoring. ICU admission is indicated for severe complications such as pulmonary edema, respiratory failure, cerebral edema, or hemodynamic instability. Discharge decisions are made exclusively by obstetric specialists.
Clinical Pearls And Common Errors
Pregnant patients with abdominal pain, elevated liver enzymes, and thrombocytopenia require immediate evaluation for HELLP syndrome, even in the absence of hypertension. Normal blood pressure does not exclude the diagnosis. Prompt stabilization and transfer to a high-risk obstetric facility are critical to reducing maternal and fetal morbidity and mortality.
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Emergency And Acute Medicine – Heart Murmur
Basics And Description
Heart murmurs are sounds produced by normal physiologic flow or by functional and structural abnormalities of the heart. Stenotic lesions result in pressure overload of the chamber proximal to the valve, leading to compensatory hypertrophy. Regurgitant lesions cause volume overload of the preceding chamber, resulting in chamber dilatation. Genetic abnormalities and congenital defects may also produce abnormal cardiac blood flow and murmurs.
Etiology
Aortic stenosis may result from rheumatic heart disease, congenital bicuspid valve, calcification, or prosthetic valves. Aortic regurgitation is associated with rheumatic disease, endocarditis, aortic dissection, and prosthetic valves. Mitral stenosis is commonly caused by rheumatic heart disease, rheumatologic disorders such as systemic lupus erythematosus, calcification, atrial myxoma, congenital abnormalities, or prosthetic valves.
Acute mitral regurgitation may occur due to endocarditis, papillary muscle rupture or dysfunction, rupture of chordae tendineae, or prosthetic valve failure, while chronic mitral regurgitation is associated with rheumatic disease, mitral valve prolapse, and connective tissue disorders such as Marfan syndrome. Mitral valve prolapse itself may be congenital or related to connective tissue disease.
Tricuspid stenosis is usually rheumatic in origin, whereas tricuspid regurgitation may result from rheumatic disease, endocarditis, or pulmonary hypertension. Pericardial friction rubs arise from pericarditis or pericardial effusion. Ventricular septal defects may be congenital, traumatic, or postinfarction. Ventricular assist devices produce mechanical flow sounds due to implantable pumps supporting ventricular function.
Pediatric And Congenital Causes
Pulmonic stenosis is often congenital and may be associated with maternal–fetal rubella exposure or rheumatic disease. Pulmonic regurgitation may be congenital or related to pulmonary hypertension. Atrial septal defects and patent ductus arteriosus are congenital, with PDA also associated with prematurity and maternal rubella exposure. Coarctation of the aorta is congenital and linked to Turner syndrome. Hypertrophic cardiomyopathy, also known as idiopathic hypertrophic subaortic stenosis, is a congenital condition with a genetic predisposition.
Clinical Features And Murmur Characteristics
Aortic stenosis presents with a systolic crescendo–decrescendo murmur radiating to the carotids, diminished and delayed carotid upstroke (parvus et tardus), angina, exertional dyspnea, and syncope. Aortic regurgitation produces a diastolic blowing murmur along the left sternal border with findings such as widened pulse pressure, pulmonary edema, tachycardia, chest pain, Austin Flint murmur, Corrigan pulse, Quincke pulse, and de Musset sign.
Mitral stenosis causes a diastolic rumbling murmur at the apex with a loud S1 and opening snap, accompanied by dyspnea, orthopnea, hemoptysis, pulmonary edema, atrial fibrillation, and systemic emboli. Acute mitral regurgitation presents with a harsh systolic murmur at the apex and pulmonary edema, while chronic mitral regurgitation produces a holosystolic murmur radiating to the axilla with dyspnea, fatigue, and atrial fibrillation.
Mitral valve prolapse is characterized by an early- to mid-systolic click followed by a systolic murmur and may cause palpitations and chest pain. Tricuspid stenosis causes a diastolic murmur with peripheral edema, hepatosplenomegaly, ascites, atrial fibrillation, and prominent jugular venous A waves. Tricuspid regurgitation presents with a holosystolic murmur along the left sternal border and large V waves in the jugular venous pulse.
Patent ductus arteriosus produces a continuous machinery murmur and may lead to heart failure. Pericardial friction rubs are intermittent with systolic and/or diastolic components. Ventricular septal defects cause a harsh holosystolic murmur at the lower left sternal border. Ventricular assist devices generate a continuous or pulsatile mechanical hum, often with adequate perfusion despite absent palpable pulses.
Pediatric Murmur Features
Pulmonic stenosis presents with a systolic ejection murmur at the left upper sternal border, possible thrill, widely split S2, exertional dyspnea, and signs of right heart failure in severe cases. Pulmonic regurgitation causes a high-pitched early diastolic murmur with a widely split S2 and may be associated with pulmonary hypertension. Atrial septal defects often present with a systolic ejection murmur and wide fixed S2, while patent foramen ovale produces no murmur. Coarctation of the aorta may cause a continuous or late systolic murmur with differential upper and lower extremity pulses. Hypertrophic cardiomyopathy presents with a harsh systolic murmur that increases with Valsalva and may cause dyspnea, chest pain, syncope, or sudden death.
Physical Examination Focus
Evaluation includes careful auscultation of the heart and lungs, assessment of peripheral pulses, perfusion, edema, and jugular venous findings.
Essential Evaluation
Further assessment is guided by suspected pathology, with reference to specific valvular or congenital heart disease entities when appropriate.
Diagnostic Testing And Interpretation
Electrocardiography and chest radiography are commonly obtained. Echocardiography is essential for evaluating valves, chamber size, and flow patterns. CT imaging may be required to exclude aortic dissection. Acute regurgitant lesions may require cardiac catheterization.
Differential Diagnosis
Differential considerations mirror the underlying etiologies of murmurs and associated cardiac conditions.
Initial And Emergency Management
Prehospital care includes cautious IV fluid administration, especially in critical aortic stenosis, and oxygen as needed. In the emergency department, management focuses on oxygen, IV access, cardiac monitoring, and treatment of associated conditions such as heart failure or arrhythmias. Fluids and medications must be used cautiously in patients with aortic stenosis.
Medications
Therapy may include digoxin for rate control, calcium channel blockers or beta blockers for arrhythmias, diuretics for volume overload, anticoagulation when indicated, and vasodilators such as nitroglycerin or nitroprusside with careful monitoring. Medication choice depends on the underlying lesion and hemodynamic status.
Disposition And Follow-Up
Admission is required for patients with ischemia, syncope, pulmonary edema, hemodynamic instability, endocarditis, or significant arrhythmias. Asymptomatic and hemodynamically stable patients may be discharged with follow-up. New murmurs warrant referral to a primary provider or cardiologist.
Follow-Up Advice
Patients should inform all medical and dental providers of their heart murmur. Antibiotic prophylaxis may be necessary before certain procedures to prevent endocarditis.
Clinical Insights And Pitfalls
New heart murmurs accompanied by fever must prompt evaluation for infective endocarditis.
Basics And Description
Heart murmurs are sounds produced by normal physiologic flow or by functional and structural abnormalities of the heart. Stenotic lesions result in pressure overload of the chamber proximal to the valve, leading to compensatory hypertrophy. Regurgitant lesions cause volume overload of the preceding chamber, resulting in chamber dilatation. Genetic abnormalities and congenital defects may also produce abnormal cardiac blood flow and murmurs.
Etiology
Aortic stenosis may result from rheumatic heart disease, congenital bicuspid valve, calcification, or prosthetic valves. Aortic regurgitation is associated with rheumatic disease, endocarditis, aortic dissection, and prosthetic valves. Mitral stenosis is commonly caused by rheumatic heart disease, rheumatologic disorders such as systemic lupus erythematosus, calcification, atrial myxoma, congenital abnormalities, or prosthetic valves.
Acute mitral regurgitation may occur due to endocarditis, papillary muscle rupture or dysfunction, rupture of chordae tendineae, or prosthetic valve failure, while chronic mitral regurgitation is associated with rheumatic disease, mitral valve prolapse, and connective tissue disorders such as Marfan syndrome. Mitral valve prolapse itself may be congenital or related to connective tissue disease.
Tricuspid stenosis is usually rheumatic in origin, whereas tricuspid regurgitation may result from rheumatic disease, endocarditis, or pulmonary hypertension. Pericardial friction rubs arise from pericarditis or pericardial effusion. Ventricular septal defects may be congenital, traumatic, or postinfarction. Ventricular assist devices produce mechanical flow sounds due to implantable pumps supporting ventricular function.
Pediatric And Congenital Causes
Pulmonic stenosis is often congenital and may be associated with maternal–fetal rubella exposure or rheumatic disease. Pulmonic regurgitation may be congenital or related to pulmonary hypertension. Atrial septal defects and patent ductus arteriosus are congenital, with PDA also associated with prematurity and maternal rubella exposure. Coarctation of the aorta is congenital and linked to Turner syndrome. Hypertrophic cardiomyopathy, also known as idiopathic hypertrophic subaortic stenosis, is a congenital condition with a genetic predisposition.
Clinical Features And Murmur Characteristics
Aortic stenosis presents with a systolic crescendo–decrescendo murmur radiating to the carotids, diminished and delayed carotid upstroke (parvus et tardus), angina, exertional dyspnea, and syncope. Aortic regurgitation produces a diastolic blowing murmur along the left sternal border with findings such as widened pulse pressure, pulmonary edema, tachycardia, chest pain, Austin Flint murmur, Corrigan pulse, Quincke pulse, and de Musset sign.
Mitral stenosis causes a diastolic rumbling murmur at the apex with a loud S1 and opening snap, accompanied by dyspnea, orthopnea, hemoptysis, pulmonary edema, atrial fibrillation, and systemic emboli. Acute mitral regurgitation presents with a harsh systolic murmur at the apex and pulmonary edema, while chronic mitral regurgitation produces a holosystolic murmur radiating to the axilla with dyspnea, fatigue, and atrial fibrillation.
Mitral valve prolapse is characterized by an early- to mid-systolic click followed by a systolic murmur and may cause palpitations and chest pain. Tricuspid stenosis causes a diastolic murmur with peripheral edema, hepatosplenomegaly, ascites, atrial fibrillation, and prominent jugular venous A waves. Tricuspid regurgitation presents with a holosystolic murmur along the left sternal border and large V waves in the jugular venous pulse.
Patent ductus arteriosus produces a continuous machinery murmur and may lead to heart failure. Pericardial friction rubs are intermittent with systolic and/or diastolic components. Ventricular septal defects cause a harsh holosystolic murmur at the lower left sternal border. Ventricular assist devices generate a continuous or pulsatile mechanical hum, often with adequate perfusion despite absent palpable pulses.
Pediatric Murmur Features
Pulmonic stenosis presents with a systolic ejection murmur at the left upper sternal border, possible thrill, widely split S2, exertional dyspnea, and signs of right heart failure in severe cases. Pulmonic regurgitation causes a high-pitched early diastolic murmur with a widely split S2 and may be associated with pulmonary hypertension. Atrial septal defects often present with a systolic ejection murmur and wide fixed S2, while patent foramen ovale produces no murmur. Coarctation of the aorta may cause a continuous or late systolic murmur with differential upper and lower extremity pulses. Hypertrophic cardiomyopathy presents with a harsh systolic murmur that increases with Valsalva and may cause dyspnea, chest pain, syncope, or sudden death.
Physical Examination Focus
Evaluation includes careful auscultation of the heart and lungs, assessment of peripheral pulses, perfusion, edema, and jugular venous findings.
Essential Evaluation
Further assessment is guided by suspected pathology, with reference to specific valvular or congenital heart disease entities when appropriate.
Diagnostic Testing And Interpretation
Electrocardiography and chest radiography are commonly obtained. Echocardiography is essential for evaluating valves, chamber size, and flow patterns. CT imaging may be required to exclude aortic dissection. Acute regurgitant lesions may require cardiac catheterization.
Differential Diagnosis
Differential considerations mirror the underlying etiologies of murmurs and associated cardiac conditions.
Initial And Emergency Management
Prehospital care includes cautious IV fluid administration, especially in critical aortic stenosis, and oxygen as needed. In the emergency department, management focuses on oxygen, IV access, cardiac monitoring, and treatment of associated conditions such as heart failure or arrhythmias. Fluids and medications must be used cautiously in patients with aortic stenosis.
Medications
Therapy may include digoxin for rate control, calcium channel blockers or beta blockers for arrhythmias, diuretics for volume overload, anticoagulation when indicated, and vasodilators such as nitroglycerin or nitroprusside with careful monitoring. Medication choice depends on the underlying lesion and hemodynamic status.
Disposition And Follow-Up
Admission is required for patients with ischemia, syncope, pulmonary edema, hemodynamic instability, endocarditis, or significant arrhythmias. Asymptomatic and hemodynamically stable patients may be discharged with follow-up. New murmurs warrant referral to a primary provider or cardiologist.
Follow-Up Advice
Patients should inform all medical and dental providers of their heart murmur. Antibiotic prophylaxis may be necessary before certain procedures to prevent endocarditis.
Clinical Insights And Pitfalls
New heart murmurs accompanied by fever must prompt evaluation for infective endocarditis.
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Emergency And Acute Medicine – Migraine Headache
Overview And Pathophysiology
Migraine is a chronic, episodic primary headache disorder and accounts for the majority of headache-related emergency department visits, with approximately one million visits annually. It is three times more common in women, and prevalence peaks in the fourth decade of life. Migraine is no longer considered a primarily vascular disorder. Instead, it reflects a neurovascular process characterized by abnormal trigeminal nerve activation, stimulation of nociceptive pathways within the brainstem, disordered sensory processing, and autonomic dysfunction. Vascular dilation is reactive rather than causative. Cortical spreading depression is believed to underlie migraine aura.
Diagnostic Criteria And Subtypes
Migraine without aura is defined by established clinical criteria requiring at least five attacks lasting 4–72 hours, with headaches demonstrating at least two of the following features: unilateral location, pulsating quality, moderate to severe intensity that interferes with daily activity, and worsening with or avoidance of routine physical activity. During attacks, patients experience nausea and/or vomiting and photophobia with phonophobia, and symptoms are not attributable to another disorder.
Migraine with aura is less common and consists of reversible neurologic symptoms that typically precede headache but may occur simultaneously or afterward. Aura most often involves visual or sensory disturbances. Less common subtypes include basilar-type migraine, which may present with dysarthria, vertigo, ataxia, diplopia, or decreased level of consciousness; hemiplegic migraine, characterized by fully reversible motor weakness; and retinal migraine, involving recurrent monocular visual disturbances.
Special Population Considerations
In children, migraines more often present with bilateral pain and shorter duration. Associated symptoms may be difficult to verbalize and are often inferred from behavior. Cyclical vomiting syndrome is associated with pediatric migraine, and placebo response rates are high. Migraine has a strong genetic basis with variable penetrance and is influenced by environmental factors.
Clinical Presentation
History often reveals triggers such as chocolate, cheese, nuts, alcohol, sulfites, monosodium glutamate, stress, or menstruation. A prodrome may precede the headache by several days and can include yawning, drowsiness, or cognitive and emotional changes. Aura typically precedes headache by up to one hour and most commonly consists of visual phenomena such as scintillating scotoma or fortification spectra, as well as sensory symptoms like numbness or tingling.
The headache itself is usually unilateral and throbbing, although it may be bilateral, and is sufficiently severe to impair function. Symptoms typically develop gradually and are accompanied by nausea, vomiting, photophobia, phonophobia, and sometimes osmophobia. Patients frequently report similar headaches in the past.
Physical Examination Findings
Physical examination is generally normal. Allodynia, or pain from normally nonpainful stimuli, may be present and suggests a more refractory migraine. Funduscopic examination and assessment of visual fields should be performed. Elevated blood pressure or sinus tenderness does not exclude migraine as the diagnosis.
Initial Evaluation Strategy
Migraine is a clinical diagnosis based on history and examination. Patients presenting with a new headache syndrome or atypical features may require further evaluation, including neuroimaging or cerebrospinal fluid analysis, to exclude secondary causes.
Diagnostic Testing Considerations
No routine laboratory studies, imaging, or procedures are required for patients with a typical migraine presentation. Testing is reserved for cases with red flags or diagnostic uncertainty.
Differential Diagnosis
Conditions to consider include cluster headache, medication overuse headache, tension-type headache, allergic or viral rhinosinusitis, idiopathic intracranial hypertension, and reversible cerebral vasoconstriction syndrome.
Prehospital And Early Emergency Care
Patients benefit from a calm, dark environment. Supplemental oxygen may provide symptomatic relief. Early goals include exclusion of secondary headache causes and rapid, effective analgesia.
Emergency Department Management
A detailed history usually excludes secondary pathology. Treatment should prioritize nonopioid analgesia. Intravenous saline hydration is often beneficial. Clear communication of the diagnosis and education regarding trigger avoidance are important aspects of care. In pregnancy, metoclopramide and prochlorperazine are preferred treatment options.
Pharmacologic Therapy
First-line abortive therapy in the emergency department includes dopamine antagonists such as prochlorperazine or droperidol administered with diphenhydramine to prevent akathisia, as well as metoclopramide or trimethobenzamide. Triptans, such as subcutaneous sumatriptan, are effective but should be avoided in patients with significant cardiac risk factors. Ergot derivatives, including dihydroergotamine, may be used with antiemetics in appropriate patients. Nonsteroidal anti-inflammatory drugs such as ketorolac are commonly effective. Corticosteroids, including dexamethasone, may be used to reduce post-discharge headache recurrence. Opioids should be reserved only for refractory cases when other therapies have failed.
Disposition And Follow-Up Planning
Admission is indicated for patients with persistent severe headache, focal neurologic deficits, intractable vomiting, electrolyte abnormalities, inability to tolerate oral intake, or coexisting medication overuse headache. Patients may be discharged once pain relief is achieved and secondary causes have been excluded. Individuals with chronic or frequent episodic migraine should be referred for specialist care.
Outpatient Care And Prevention
Patients should be encouraged to maintain a headache diary to identify triggers and patterns. Ongoing primary care or specialty follow-up is essential to establish effective outpatient abortive and preventive therapies.
Clinical Pearls And Common Pitfalls
Opioids should not be used as first-line therapy for migraine in the emergency department. Migraine commonly recurs after ED discharge, so patients should leave with an appropriate prescription and management plan. It is critical to distinguish migraine, a chronic recurrent disorder, from new-onset or progressively worsening headaches that may signal serious underlying disease.
Overview And Pathophysiology
Migraine is a chronic, episodic primary headache disorder and accounts for the majority of headache-related emergency department visits, with approximately one million visits annually. It is three times more common in women, and prevalence peaks in the fourth decade of life. Migraine is no longer considered a primarily vascular disorder. Instead, it reflects a neurovascular process characterized by abnormal trigeminal nerve activation, stimulation of nociceptive pathways within the brainstem, disordered sensory processing, and autonomic dysfunction. Vascular dilation is reactive rather than causative. Cortical spreading depression is believed to underlie migraine aura.
Diagnostic Criteria And Subtypes
Migraine without aura is defined by established clinical criteria requiring at least five attacks lasting 4–72 hours, with headaches demonstrating at least two of the following features: unilateral location, pulsating quality, moderate to severe intensity that interferes with daily activity, and worsening with or avoidance of routine physical activity. During attacks, patients experience nausea and/or vomiting and photophobia with phonophobia, and symptoms are not attributable to another disorder.
Migraine with aura is less common and consists of reversible neurologic symptoms that typically precede headache but may occur simultaneously or afterward. Aura most often involves visual or sensory disturbances. Less common subtypes include basilar-type migraine, which may present with dysarthria, vertigo, ataxia, diplopia, or decreased level of consciousness; hemiplegic migraine, characterized by fully reversible motor weakness; and retinal migraine, involving recurrent monocular visual disturbances.
Special Population Considerations
In children, migraines more often present with bilateral pain and shorter duration. Associated symptoms may be difficult to verbalize and are often inferred from behavior. Cyclical vomiting syndrome is associated with pediatric migraine, and placebo response rates are high. Migraine has a strong genetic basis with variable penetrance and is influenced by environmental factors.
Clinical Presentation
History often reveals triggers such as chocolate, cheese, nuts, alcohol, sulfites, monosodium glutamate, stress, or menstruation. A prodrome may precede the headache by several days and can include yawning, drowsiness, or cognitive and emotional changes. Aura typically precedes headache by up to one hour and most commonly consists of visual phenomena such as scintillating scotoma or fortification spectra, as well as sensory symptoms like numbness or tingling.
The headache itself is usually unilateral and throbbing, although it may be bilateral, and is sufficiently severe to impair function. Symptoms typically develop gradually and are accompanied by nausea, vomiting, photophobia, phonophobia, and sometimes osmophobia. Patients frequently report similar headaches in the past.
Physical Examination Findings
Physical examination is generally normal. Allodynia, or pain from normally nonpainful stimuli, may be present and suggests a more refractory migraine. Funduscopic examination and assessment of visual fields should be performed. Elevated blood pressure or sinus tenderness does not exclude migraine as the diagnosis.
Initial Evaluation Strategy
Migraine is a clinical diagnosis based on history and examination. Patients presenting with a new headache syndrome or atypical features may require further evaluation, including neuroimaging or cerebrospinal fluid analysis, to exclude secondary causes.
Diagnostic Testing Considerations
No routine laboratory studies, imaging, or procedures are required for patients with a typical migraine presentation. Testing is reserved for cases with red flags or diagnostic uncertainty.
Differential Diagnosis
Conditions to consider include cluster headache, medication overuse headache, tension-type headache, allergic or viral rhinosinusitis, idiopathic intracranial hypertension, and reversible cerebral vasoconstriction syndrome.
Prehospital And Early Emergency Care
Patients benefit from a calm, dark environment. Supplemental oxygen may provide symptomatic relief. Early goals include exclusion of secondary headache causes and rapid, effective analgesia.
Emergency Department Management
A detailed history usually excludes secondary pathology. Treatment should prioritize nonopioid analgesia. Intravenous saline hydration is often beneficial. Clear communication of the diagnosis and education regarding trigger avoidance are important aspects of care. In pregnancy, metoclopramide and prochlorperazine are preferred treatment options.
Pharmacologic Therapy
First-line abortive therapy in the emergency department includes dopamine antagonists such as prochlorperazine or droperidol administered with diphenhydramine to prevent akathisia, as well as metoclopramide or trimethobenzamide. Triptans, such as subcutaneous sumatriptan, are effective but should be avoided in patients with significant cardiac risk factors. Ergot derivatives, including dihydroergotamine, may be used with antiemetics in appropriate patients. Nonsteroidal anti-inflammatory drugs such as ketorolac are commonly effective. Corticosteroids, including dexamethasone, may be used to reduce post-discharge headache recurrence. Opioids should be reserved only for refractory cases when other therapies have failed.
Disposition And Follow-Up Planning
Admission is indicated for patients with persistent severe headache, focal neurologic deficits, intractable vomiting, electrolyte abnormalities, inability to tolerate oral intake, or coexisting medication overuse headache. Patients may be discharged once pain relief is achieved and secondary causes have been excluded. Individuals with chronic or frequent episodic migraine should be referred for specialist care.
Outpatient Care And Prevention
Patients should be encouraged to maintain a headache diary to identify triggers and patterns. Ongoing primary care or specialty follow-up is essential to establish effective outpatient abortive and preventive therapies.
Clinical Pearls And Common Pitfalls
Opioids should not be used as first-line therapy for migraine in the emergency department. Migraine commonly recurs after ED discharge, so patients should leave with an appropriate prescription and management plan. It is critical to distinguish migraine, a chronic recurrent disorder, from new-onset or progressively worsening headaches that may signal serious underlying disease.
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Emergency And Acute Medicine – Cluster Headache
Overview And Key Features
Cluster headache is an excruciating primary headache disorder and represents an infrequent cause of emergency department visits, affecting approximately 0.1% of the population. Attacks often resolve before ED presentation. Individual headache episodes last 15–180 minutes, with about 75% resolving within 60 minutes. The disorder is more common in men at a ratio of approximately 3:1, with typical onset between 30 and 50 years of age. Headaches occur in clusters lasting weeks to months, followed by remission periods longer than one month. During a cluster period, attacks commonly occur 1–3 times per day over 2–3 months, often at the same time of day and during the same season, most frequently in spring and fall. A chronic form exists in about 10% of patients, characterized by remission periods shorter than one month or no remission at all. Cluster headache shares clinical and pathophysiologic similarities with migraine variants and often follows a trigeminal nerve dermatome.
Pathophysiology
The condition is mediated by activation of the trigeminovascular reflex. This reflex arc amplifies trigeminal nociceptive input and cranial autonomic symptoms through positive feedback mechanisms, resulting in severe unilateral pain and associated autonomic features.
Clinical Presentation
History typically reveals strictly unilateral pain that usually does not alternate sides between attacks. The pain is described as sharp, stabbing, or boring, with abrupt onset and rapid escalation to peak intensity within 5–15 minutes. Attacks end suddenly, and patients are often exhausted afterward. Pain is most commonly located in the eye or temple and may radiate to the ear, cheek, jaw, teeth, nose, or ipsilateral neck. Episodes frequently occur at night. Attacks are often triggered by alcohol ingestion, nitroglycerin, or histamine-containing substances and are more likely during periods of stress, prolonged strain, overwork, or emotional distress. There is no prodrome or aura.
Physical Examination Findings
Patients appear agitated and restless, often pacing or standing rather than lying quietly, which contrasts with migraine behavior. Prominent ipsilateral cranial autonomic symptoms accompany the headache, including nasal congestion or rhinorrhea, conjunctival injection or lacrimation, facial flushing, eyelid edema, partial Horner syndrome (ptosis and/or miosis), and sweating of the face or forehead.
Initial Evaluation
Diagnosis is primarily clinical and based on a characteristic history and physical examination. Life-threatening secondary causes of headache must be excluded, particularly when presentation is atypical.
Diagnostic Testing
Laboratory studies are generally unnecessary unless alternative diagnoses are suspected. Lumbar puncture is indicated if meningitis or subarachnoid hemorrhage is a concern. ESR should be obtained when temporal arteritis is suspected. Neuroimaging with CT or MRI is warranted if there is concern for intracranial hemorrhage, mass lesion, or other secondary pathology.
Differential Diagnosis
Important considerations include migraine headache, trigeminal neuralgia, meningitis, temporal arteritis, intracranial mass lesions, herpes zoster, intracerebral hemorrhage, dental pathology, orbital or ocular disease such as acute angle-closure glaucoma, and temporomandibular joint disorders.
Prehospital Care
Prehospital priorities include recognizing and excluding life-threatening causes of headache. Administration of high-flow oxygen by face mask may provide symptom relief during transport.
Emergency Department Management
Initial management focuses on ruling out secondary causes and administering supplemental oxygen. High-flow 100% oxygen via nonrebreather mask is the preferred first-line therapy.
Pharmacologic Treatment
First-line acute therapies include 100% oxygen at 12 L/min via nonrebreather mask for 15 minutes, increasing to 15 L/min if symptoms persist. Subcutaneous sumatriptan and intravenous dihydroergotamine are also effective.
Second-line options include narcotic analgesics and corticosteroids. Additional medications that may be used include ketorolac, prochlorperazine, somatostatin, fentanyl, or morphine for refractory cases. Verapamil (immediate-release, starting at 80 mg three times daily) is the preventive agent of choice.
Disposition And Follow-Up
Admission is indicated for persistent headaches unresponsive to standard therapy or when the diagnosis remains uncertain. Patients with moderate to complete pain relief, a normal neurologic examination, and a confident diagnosis may be discharged. Consider prescribing home oxygen and/or subcutaneous sumatriptan for outpatient management. Neurology follow-up should be arranged for ongoing preventive therapy and cluster management.
Clinical Pearls And Pitfalls
A careful history is essential, as pain may have improved by the time of ED evaluation. High-flow 100% oxygen should be initiated immediately as first-line therapy. Cluster headaches can be so severe that they are associated with suicidal ideation; appropriate counseling and follow-up are critical. Ongoing follow-up is essential because cluster periods may persist for months.
Overview And Key Features
Cluster headache is an excruciating primary headache disorder and represents an infrequent cause of emergency department visits, affecting approximately 0.1% of the population. Attacks often resolve before ED presentation. Individual headache episodes last 15–180 minutes, with about 75% resolving within 60 minutes. The disorder is more common in men at a ratio of approximately 3:1, with typical onset between 30 and 50 years of age. Headaches occur in clusters lasting weeks to months, followed by remission periods longer than one month. During a cluster period, attacks commonly occur 1–3 times per day over 2–3 months, often at the same time of day and during the same season, most frequently in spring and fall. A chronic form exists in about 10% of patients, characterized by remission periods shorter than one month or no remission at all. Cluster headache shares clinical and pathophysiologic similarities with migraine variants and often follows a trigeminal nerve dermatome.
Pathophysiology
The condition is mediated by activation of the trigeminovascular reflex. This reflex arc amplifies trigeminal nociceptive input and cranial autonomic symptoms through positive feedback mechanisms, resulting in severe unilateral pain and associated autonomic features.
Clinical Presentation
History typically reveals strictly unilateral pain that usually does not alternate sides between attacks. The pain is described as sharp, stabbing, or boring, with abrupt onset and rapid escalation to peak intensity within 5–15 minutes. Attacks end suddenly, and patients are often exhausted afterward. Pain is most commonly located in the eye or temple and may radiate to the ear, cheek, jaw, teeth, nose, or ipsilateral neck. Episodes frequently occur at night. Attacks are often triggered by alcohol ingestion, nitroglycerin, or histamine-containing substances and are more likely during periods of stress, prolonged strain, overwork, or emotional distress. There is no prodrome or aura.
Physical Examination Findings
Patients appear agitated and restless, often pacing or standing rather than lying quietly, which contrasts with migraine behavior. Prominent ipsilateral cranial autonomic symptoms accompany the headache, including nasal congestion or rhinorrhea, conjunctival injection or lacrimation, facial flushing, eyelid edema, partial Horner syndrome (ptosis and/or miosis), and sweating of the face or forehead.
Initial Evaluation
Diagnosis is primarily clinical and based on a characteristic history and physical examination. Life-threatening secondary causes of headache must be excluded, particularly when presentation is atypical.
Diagnostic Testing
Laboratory studies are generally unnecessary unless alternative diagnoses are suspected. Lumbar puncture is indicated if meningitis or subarachnoid hemorrhage is a concern. ESR should be obtained when temporal arteritis is suspected. Neuroimaging with CT or MRI is warranted if there is concern for intracranial hemorrhage, mass lesion, or other secondary pathology.
Differential Diagnosis
Important considerations include migraine headache, trigeminal neuralgia, meningitis, temporal arteritis, intracranial mass lesions, herpes zoster, intracerebral hemorrhage, dental pathology, orbital or ocular disease such as acute angle-closure glaucoma, and temporomandibular joint disorders.
Prehospital Care
Prehospital priorities include recognizing and excluding life-threatening causes of headache. Administration of high-flow oxygen by face mask may provide symptom relief during transport.
Emergency Department Management
Initial management focuses on ruling out secondary causes and administering supplemental oxygen. High-flow 100% oxygen via nonrebreather mask is the preferred first-line therapy.
Pharmacologic Treatment
First-line acute therapies include 100% oxygen at 12 L/min via nonrebreather mask for 15 minutes, increasing to 15 L/min if symptoms persist. Subcutaneous sumatriptan and intravenous dihydroergotamine are also effective.
Second-line options include narcotic analgesics and corticosteroids. Additional medications that may be used include ketorolac, prochlorperazine, somatostatin, fentanyl, or morphine for refractory cases. Verapamil (immediate-release, starting at 80 mg three times daily) is the preventive agent of choice.
Disposition And Follow-Up
Admission is indicated for persistent headaches unresponsive to standard therapy or when the diagnosis remains uncertain. Patients with moderate to complete pain relief, a normal neurologic examination, and a confident diagnosis may be discharged. Consider prescribing home oxygen and/or subcutaneous sumatriptan for outpatient management. Neurology follow-up should be arranged for ongoing preventive therapy and cluster management.
Clinical Pearls And Pitfalls
A careful history is essential, as pain may have improved by the time of ED evaluation. High-flow 100% oxygen should be initiated immediately as first-line therapy. Cluster headaches can be so severe that they are associated with suicidal ideation; appropriate counseling and follow-up are critical. Ongoing follow-up is essential because cluster periods may persist for months.
- Published on
Emergency And Acute Medicine – High-Altitude Illness
Basics
Description
High-altitude illness occurs due to hypobaric hypoxia following rapid ascent to higher elevations, typically above 8,000 ft (≈2,500 m), without adequate acclimatization. The incidence depends on the rate of ascent, final altitude reached, sleeping altitude, and duration of exposure.
Acute mountain sickness (AMS) occurs in up to 67% of individuals who ascend rapidly (1–2 days) to elevations above 14,000 ft. Among skiers sleeping at altitude, AMS occurs in approximately 22% at 7,000–9,000 ft and 40% at 10,000 ft.
High-altitude pulmonary edema (HAPE) occurs in fewer than 1–2% of exposed individuals, and high-altitude cerebral edema (HACE) occurs in less than 1%. HAPE and HACE are uncommon below 13,000 ft (≈4,000 m).
Risk Factors
Risk factors for AMS include a prior history of high-altitude illness, rapid ascent, physical exertion, younger age (<50 yr), obesity, and preexisting lung disease. physical fitness does not confer protection.< />pan>
Pregnancy Considerations
The relationship between pregnancy and high-altitude illness is not well defined. Pregnancy-induced hypertension, proteinuria, and peripheral edema are more common at altitude, likely related to maternal hypoxemia. There is no evidence of increased risk of spontaneous abortion or placental complications at moderate altitude. Travel above 13,000 ft should be approached cautiously, especially in complicated pregnancies.
Geriatric Considerations
Although older adults often have comorbidities such as hypertension, COPD, or coronary artery disease, individuals older than 55 years appear to have a lower risk of AMS than younger adults.
Etiology
The primary cause of high-altitude illness is rapid ascent without acclimatization. Susceptibility varies between individuals, and illness is influenced by ascent rate, altitude attained, sleeping altitude, and physiologic response to hypoxia.
Diagnosis
Signs and Symptoms
Acute Mountain Sickness (AMS)
AMS is defined by headache plus at least one of the following: nausea or vomiting, fatigue or lassitude, dizziness, or difficulty sleeping. Symptoms typically begin 4–12 hours after ascent and are usually self-limited but may become debilitating.
High-Altitude Pulmonary Edema (HAPE)
HAPE usually develops 2–4 days after ascent, often on the second night. It is potentially life-threatening and presents with dry cough progressing to productive cough, dyspnea at rest, and decreased exercise tolerance.
High-Altitude Cerebral Edema (HACE)
HACE is life-threatening and typically occurs in patients with AMS and/or HAPE. Onset usually requires 2–4 days but may occur as early as 12 hours after AMS symptoms. Findings include severe headache, nausea and vomiting, altered mental status, and ataxia.
Pediatric Considerations
In infants and young children, AMS may present as irritability, decreased playfulness, reduced appetite, vomiting, and sleep disturbances. HAPE occurs more frequently in individuals younger than 20 years, and cases have not been reported in children younger than 4 years.
Physical Examination
Essential Workup
Diagnosis is primarily clinical in the setting of recent altitude gain.
Diagnostic Tests and Interpretation
Differential Diagnosis
Treatment
Prehospital Care
Severe cases require immediate descent. Further ascent is contraindicated in symptomatic individuals. Supplemental oxygen or simulated descent using a portable hyperbaric chamber (e.g., Gamow bag) may be lifesaving when evacuation is delayed.
Initial Stabilization and Therapy
For HAPE and HACE, prioritize airway, breathing, and circulation. Provide supplemental oxygen, establish IV access, and monitor closely. Endotracheal intubation may be required for respiratory failure or airway protection. CPAP may be beneficial in HAPE.
Emergency Department Treatment and Procedures
AMS
Mild cases are usually self-limited. Management includes halting ascent, symptomatic treatment, acetazolamide for moderate to severe symptoms, analgesics for headache, antiemetics for nausea, and oxygen for severe cases. Descent is required if symptoms persist or worsen.
HAPE
Immediate descent is mandatory for moderate to severe disease. Mild cases may be managed at altitude only if oxygen and close monitoring are available. Bed rest, high-flow supplemental oxygen, and avoidance of exertion are critical. Nifedipine may be used when oxygen or descent is unavailable. Hyperbaric therapy may be used when descent is impossible.
HACE
Immediate evacuation to lower altitude is essential. Administer oxygen and dexamethasone. Maintain bed rest with head elevation and manage elevated intracranial pressure aggressively in severe cases.
Medications
First-Line Therapy
Acetazolamide for AMS, nifedipine for HAPE
Second-Line Therapy
Dexamethasone
Use of multiple medications concurrently is not supported by current evidence.
Follow-Up and Disposition
Admission Criteria
HAPE, HACE, or persistent symptoms after descent and observation require admission.
Discharge Criteria
Patients may be discharged after clinical improvement with oxygen saturation >95% on room air at sea level or appropriate baseline saturation at altitude.
Issues for Referral
Patients with recurrent AMS may benefit from acetazolamide prophylaxis for future ascents. Those with prior HAPE may benefit from nifedipine prophylaxis.
Clinical Pearls and Pitfalls
High-altitude illness often mimics viral syndromes, leading to missed diagnosis. Once symptoms develop, further ascent is contraindicated until complete resolution. Ataxia and dyspnea at rest are early warning signs of HACE and HAPE, respectively. When descent is not possible, hyperbaric therapy and adjunctive medications should be considered promptly.
Basics
Description
High-altitude illness occurs due to hypobaric hypoxia following rapid ascent to higher elevations, typically above 8,000 ft (≈2,500 m), without adequate acclimatization. The incidence depends on the rate of ascent, final altitude reached, sleeping altitude, and duration of exposure.
Acute mountain sickness (AMS) occurs in up to 67% of individuals who ascend rapidly (1–2 days) to elevations above 14,000 ft. Among skiers sleeping at altitude, AMS occurs in approximately 22% at 7,000–9,000 ft and 40% at 10,000 ft.
High-altitude pulmonary edema (HAPE) occurs in fewer than 1–2% of exposed individuals, and high-altitude cerebral edema (HACE) occurs in less than 1%. HAPE and HACE are uncommon below 13,000 ft (≈4,000 m).
Risk Factors
Risk factors for AMS include a prior history of high-altitude illness, rapid ascent, physical exertion, younger age (<50 yr), obesity, and preexisting lung disease. physical fitness does not confer protection.< />pan>
Pregnancy Considerations
The relationship between pregnancy and high-altitude illness is not well defined. Pregnancy-induced hypertension, proteinuria, and peripheral edema are more common at altitude, likely related to maternal hypoxemia. There is no evidence of increased risk of spontaneous abortion or placental complications at moderate altitude. Travel above 13,000 ft should be approached cautiously, especially in complicated pregnancies.
Geriatric Considerations
Although older adults often have comorbidities such as hypertension, COPD, or coronary artery disease, individuals older than 55 years appear to have a lower risk of AMS than younger adults.
Etiology
The primary cause of high-altitude illness is rapid ascent without acclimatization. Susceptibility varies between individuals, and illness is influenced by ascent rate, altitude attained, sleeping altitude, and physiologic response to hypoxia.
Diagnosis
Signs and Symptoms
Acute Mountain Sickness (AMS)
AMS is defined by headache plus at least one of the following: nausea or vomiting, fatigue or lassitude, dizziness, or difficulty sleeping. Symptoms typically begin 4–12 hours after ascent and are usually self-limited but may become debilitating.
High-Altitude Pulmonary Edema (HAPE)
HAPE usually develops 2–4 days after ascent, often on the second night. It is potentially life-threatening and presents with dry cough progressing to productive cough, dyspnea at rest, and decreased exercise tolerance.
High-Altitude Cerebral Edema (HACE)
HACE is life-threatening and typically occurs in patients with AMS and/or HAPE. Onset usually requires 2–4 days but may occur as early as 12 hours after AMS symptoms. Findings include severe headache, nausea and vomiting, altered mental status, and ataxia.
Pediatric Considerations
In infants and young children, AMS may present as irritability, decreased playfulness, reduced appetite, vomiting, and sleep disturbances. HAPE occurs more frequently in individuals younger than 20 years, and cases have not been reported in children younger than 4 years.
Physical Examination
- AMS: Often normal examination; mild cases may mimic viral illness or alcohol hangover.
- HAPE: Tachypnea, rales, cyanosis, possible fever, and signs of respiratory distress.
- HACE: Ataxia, altered mental status, papilledema, retinal hemorrhages, seizures (rare), and coma in severe cases.
Essential Workup
Diagnosis is primarily clinical in the setting of recent altitude gain.
Diagnostic Tests and Interpretation
- AMS: No laboratory or imaging studies required.
- HAPE:
- Arterial blood gas: Hypoxemia (PaO₂ 30–50 mm Hg) with respiratory alkalosis
- Chest radiograph: Patchy alveolar infiltrates with areas of sparing; cardiomegaly and classic signs of cardiogenic pulmonary edema are typically absent
- ECG: Tachycardia, possible right-heart strain
- HACE:
- CT or MRI: Vasogenic edema of the cerebral white matter
Differential Diagnosis
- AMS: Viral syndrome, alcohol hangover, carbon monoxide poisoning, meningitis, encephalitis, exhaustion
- HAPE: Pneumonia, pulmonary embolism (typically more acute with pleuritic chest pain), high-altitude bronchitis
- HACE: Stroke or transient ischemic attack (focal deficits suggest vascular etiology)
Treatment
Prehospital Care
Severe cases require immediate descent. Further ascent is contraindicated in symptomatic individuals. Supplemental oxygen or simulated descent using a portable hyperbaric chamber (e.g., Gamow bag) may be lifesaving when evacuation is delayed.
Initial Stabilization and Therapy
For HAPE and HACE, prioritize airway, breathing, and circulation. Provide supplemental oxygen, establish IV access, and monitor closely. Endotracheal intubation may be required for respiratory failure or airway protection. CPAP may be beneficial in HAPE.
Emergency Department Treatment and Procedures
AMS
Mild cases are usually self-limited. Management includes halting ascent, symptomatic treatment, acetazolamide for moderate to severe symptoms, analgesics for headache, antiemetics for nausea, and oxygen for severe cases. Descent is required if symptoms persist or worsen.
HAPE
Immediate descent is mandatory for moderate to severe disease. Mild cases may be managed at altitude only if oxygen and close monitoring are available. Bed rest, high-flow supplemental oxygen, and avoidance of exertion are critical. Nifedipine may be used when oxygen or descent is unavailable. Hyperbaric therapy may be used when descent is impossible.
HACE
Immediate evacuation to lower altitude is essential. Administer oxygen and dexamethasone. Maintain bed rest with head elevation and manage elevated intracranial pressure aggressively in severe cases.
Medications
- Acetazolamide:
- AMS treatment: 250–500 mg PO BID (peds: 5 mg/kg PO BID)
- AMS prophylaxis: 250 mg PO BID starting 24 hr before ascent
- Dexamethasone: 8 mg IV, then 4 mg PO/IV QID
- Ibuprofen: 800 mg PO TID (peds: 5–10 mg/kg)
- Nifedipine: 10 mg PO, then 30 mg sustained release PO daily
- Promethazine: 12.5–25 mg PO/IM/PR q4–6h
First-Line Therapy
Acetazolamide for AMS, nifedipine for HAPE
Second-Line Therapy
Dexamethasone
Use of multiple medications concurrently is not supported by current evidence.
Follow-Up and Disposition
Admission Criteria
HAPE, HACE, or persistent symptoms after descent and observation require admission.
Discharge Criteria
Patients may be discharged after clinical improvement with oxygen saturation >95% on room air at sea level or appropriate baseline saturation at altitude.
Issues for Referral
Patients with recurrent AMS may benefit from acetazolamide prophylaxis for future ascents. Those with prior HAPE may benefit from nifedipine prophylaxis.
Clinical Pearls and Pitfalls
High-altitude illness often mimics viral syndromes, leading to missed diagnosis. Once symptoms develop, further ascent is contraindicated until complete resolution. Ataxia and dyspnea at rest are early warning signs of HACE and HAPE, respectively. When descent is not possible, hyperbaric therapy and adjunctive medications should be considered promptly.
- Published on
Emergency And Acute Medicine – Hiccups
Basics
Description
Hiccups are sudden, involuntary contractions of the diaphragm (usually unilateral) and other inspiratory muscles, abruptly terminated by closure of the glottis. The medical term is singultus. Episodes typically occur at a frequency of 4–60 per minute and result from stimulation of the hiccup reflex arc involving irritation of the vagus and phrenic nerves. The hiccup center is believed to be located in the upper spinal cord or brainstem.
Classification includes hiccup bouts lasting less than 48 hours, persistent hiccups lasting 48 hours to 1 month, and intractable hiccups persisting longer than 1 month. Hiccups are more common in males than females (approximately 4:1).
Etiology
Gastrointestinal causes include gastric distention, overeating, rapid eating, gastroesophageal reflux disease, achalasia, candida esophagitis, esophageal or gastric cancer, ulcers, hepatitis, hepatoma, pancreatitis, pancreatic pseudocyst or cancer, bowel obstruction, inflammatory bowel disease, cholelithiasis, cholecystitis, appendicitis, abdominal aortic aneurysm, and postoperative or post–abdominal procedures.
Diaphragmatic irritation may result from hiatal hernia, intra-abdominal mass, pericarditis, diaphragmatic eventration, splenomegaly, hepatomegaly, or peritonitis.
Central nervous system causes include ischemic or hemorrhagic stroke, head trauma, arteriovenous malformations, encephalitis, meningitis, abscess, malignancy, Parkinson disease, multiple sclerosis, hydrocephalus, and ventriculoperitoneal shunts.
Thoracic causes include pneumonia, tuberculosis, myocardial infarction, pericarditis, aortic aneurysm, malignancy, and mediastinal lymphadenopathy.
Head and neck causes include otic foreign bodies irritating the tympanic membrane, pharyngitis, laryngitis, goiter, retropharyngeal or peritonsillar abscess, and neck masses.
Metabolic causes include uremia, hyponatremia, hypocalcemia, gout, and diabetes mellitus.
Toxic and drug-related causes include alcohol, tobacco, α-methyldopa, benzodiazepines, steroids, barbiturates, narcotics, chemotherapeutic agents, antibiotics, and general anesthesia.
Psychogenic causes include stress, excitement, grief, malingering, and conversion disorder. Many cases remain idiopathic.
Diagnosis
Signs And Symptoms
Hiccups produce a characteristic sound that abruptly ends an inspiratory effort. Attacks usually occur at brief intervals and last seconds to minutes. Episodes persisting longer than 48 hours or continuing during sleep strongly suggest an underlying organic cause.
History
A targeted history should assess the severity and duration of the current episode, prior episodes, previous treatment attempts, and associated symptoms that may suggest an underlying etiology.
Physical Examination
A focused examination should evaluate the head and neck, chest, abdomen, and neurologic system to identify potential causes.
Essential Workup
For persistent or intractable hiccups, further evaluation is guided by findings from the history and physical examination.
Diagnostic Tests And Interpretation
Laboratory evaluation may include a complete blood count with differential and measurement of electrolytes, blood urea nitrogen, and creatinine. A chest radiograph is commonly obtained. Additional imaging or testing should be based on clinical suspicion and is often appropriate for outpatient evaluation.
Differential Diagnosis
Eructation (belching).
Treatment
Emergency Department Treatment And Procedures
Specific causes should be treated when identified, such as removal of an ear foreign body or decompression of gastric distention with a nasogastric tube.
Nonpharmacologic maneuvers include posterior pharyngeal stimulation with a catheter or cotton swab, direct uvular stimulation, supraorbital pressure, carotid sinus massage, digital rectal massage, and suboccipital release with gentle traction and pressure to the posterior neck.
Pharmacologic therapy includes chlorpromazine, the only FDA-approved medication for hiccups. Other agents used include gabapentin, metoclopramide, baclofen, haloperidol, nebulized lidocaine, amitriptyline, and phenytoin.
Medications
Amitriptyline 10 mg PO TID
Baclofen 10 mg PO TID
Chlorpromazine 25–50 mg IV or IM, or 25–50 mg PO BID–TID
Gabapentin 100 mg PO TID–QID
Haloperidol 2–5 mg IM
Lidocaine 4% solution, 3 mL nebulized, may repeat
Metoclopramide 10 mg IV or IM, or 10–20 mg PO QID
Phenytoin 200 mg IV
Follow-Up And Disposition
Admission Criteria
Admission is indicated when hiccups interfere with daily activities or lead to complications such as poor oral intake, aspiration, insomnia, or wound dehiscence.
Discharge Criteria
Patients may be discharged if hiccups last less than 48 hours and evaluation does not suggest an underlying organic cause.
Issues For Referral
Referral is recommended for intractable hiccups requiring further investigation or advanced therapies such as phrenic nerve block or transection, hypnosis, behavioral modification, acupuncture, or psychiatric intervention.
Follow-Up Recommendations
Patients may try home remedies for recurrence, including swallowing a spoonful of sugar, sucking on hard candy, swallowing peanut butter, breath holding or Valsalva maneuver, biting a lemon, tongue traction, lifting the uvula with a cold spoon, drinking from the far side of a glass, applying noxious stimuli, or rebreathing into a paper bag.
Clinical Insights And Common Pitfalls
Prolonged or persistent hiccups are strongly suggestive of an underlying organic disease and should not be dismissed as benign without appropriate evaluation.
Basics
Description
Hiccups are sudden, involuntary contractions of the diaphragm (usually unilateral) and other inspiratory muscles, abruptly terminated by closure of the glottis. The medical term is singultus. Episodes typically occur at a frequency of 4–60 per minute and result from stimulation of the hiccup reflex arc involving irritation of the vagus and phrenic nerves. The hiccup center is believed to be located in the upper spinal cord or brainstem.
Classification includes hiccup bouts lasting less than 48 hours, persistent hiccups lasting 48 hours to 1 month, and intractable hiccups persisting longer than 1 month. Hiccups are more common in males than females (approximately 4:1).
Etiology
Gastrointestinal causes include gastric distention, overeating, rapid eating, gastroesophageal reflux disease, achalasia, candida esophagitis, esophageal or gastric cancer, ulcers, hepatitis, hepatoma, pancreatitis, pancreatic pseudocyst or cancer, bowel obstruction, inflammatory bowel disease, cholelithiasis, cholecystitis, appendicitis, abdominal aortic aneurysm, and postoperative or post–abdominal procedures.
Diaphragmatic irritation may result from hiatal hernia, intra-abdominal mass, pericarditis, diaphragmatic eventration, splenomegaly, hepatomegaly, or peritonitis.
Central nervous system causes include ischemic or hemorrhagic stroke, head trauma, arteriovenous malformations, encephalitis, meningitis, abscess, malignancy, Parkinson disease, multiple sclerosis, hydrocephalus, and ventriculoperitoneal shunts.
Thoracic causes include pneumonia, tuberculosis, myocardial infarction, pericarditis, aortic aneurysm, malignancy, and mediastinal lymphadenopathy.
Head and neck causes include otic foreign bodies irritating the tympanic membrane, pharyngitis, laryngitis, goiter, retropharyngeal or peritonsillar abscess, and neck masses.
Metabolic causes include uremia, hyponatremia, hypocalcemia, gout, and diabetes mellitus.
Toxic and drug-related causes include alcohol, tobacco, α-methyldopa, benzodiazepines, steroids, barbiturates, narcotics, chemotherapeutic agents, antibiotics, and general anesthesia.
Psychogenic causes include stress, excitement, grief, malingering, and conversion disorder. Many cases remain idiopathic.
Diagnosis
Signs And Symptoms
Hiccups produce a characteristic sound that abruptly ends an inspiratory effort. Attacks usually occur at brief intervals and last seconds to minutes. Episodes persisting longer than 48 hours or continuing during sleep strongly suggest an underlying organic cause.
History
A targeted history should assess the severity and duration of the current episode, prior episodes, previous treatment attempts, and associated symptoms that may suggest an underlying etiology.
Physical Examination
A focused examination should evaluate the head and neck, chest, abdomen, and neurologic system to identify potential causes.
Essential Workup
For persistent or intractable hiccups, further evaluation is guided by findings from the history and physical examination.
Diagnostic Tests And Interpretation
Laboratory evaluation may include a complete blood count with differential and measurement of electrolytes, blood urea nitrogen, and creatinine. A chest radiograph is commonly obtained. Additional imaging or testing should be based on clinical suspicion and is often appropriate for outpatient evaluation.
Differential Diagnosis
Eructation (belching).
Treatment
Emergency Department Treatment And Procedures
Specific causes should be treated when identified, such as removal of an ear foreign body or decompression of gastric distention with a nasogastric tube.
Nonpharmacologic maneuvers include posterior pharyngeal stimulation with a catheter or cotton swab, direct uvular stimulation, supraorbital pressure, carotid sinus massage, digital rectal massage, and suboccipital release with gentle traction and pressure to the posterior neck.
Pharmacologic therapy includes chlorpromazine, the only FDA-approved medication for hiccups. Other agents used include gabapentin, metoclopramide, baclofen, haloperidol, nebulized lidocaine, amitriptyline, and phenytoin.
Medications
Amitriptyline 10 mg PO TID
Baclofen 10 mg PO TID
Chlorpromazine 25–50 mg IV or IM, or 25–50 mg PO BID–TID
Gabapentin 100 mg PO TID–QID
Haloperidol 2–5 mg IM
Lidocaine 4% solution, 3 mL nebulized, may repeat
Metoclopramide 10 mg IV or IM, or 10–20 mg PO QID
Phenytoin 200 mg IV
Follow-Up And Disposition
Admission Criteria
Admission is indicated when hiccups interfere with daily activities or lead to complications such as poor oral intake, aspiration, insomnia, or wound dehiscence.
Discharge Criteria
Patients may be discharged if hiccups last less than 48 hours and evaluation does not suggest an underlying organic cause.
Issues For Referral
Referral is recommended for intractable hiccups requiring further investigation or advanced therapies such as phrenic nerve block or transection, hypnosis, behavioral modification, acupuncture, or psychiatric intervention.
Follow-Up Recommendations
Patients may try home remedies for recurrence, including swallowing a spoonful of sugar, sucking on hard candy, swallowing peanut butter, breath holding or Valsalva maneuver, biting a lemon, tongue traction, lifting the uvula with a cold spoon, drinking from the far side of a glass, applying noxious stimuli, or rebreathing into a paper bag.
Clinical Insights And Common Pitfalls
Prolonged or persistent hiccups are strongly suggestive of an underlying organic disease and should not be dismissed as benign without appropriate evaluation.
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Emergency And Acute Medicine – Genital Herpes
Overview And Definition
Genital herpes is a lifelong, recurrent viral infection caused by herpes simplex virus (HSV). Approximately one in four Americans older than 30 years are seropositive for HSV-2, though most are asymptomatic. The infection is characterized by periods of latency and reactivation, with viral shedding that can occur even in the absence of visible lesions, contributing to ongoing transmission.
Disease Course And Natural History
Primary infection has an incubation period of approximately 2–12 days, with symptoms typically peaking 8–10 days after onset and lesions healing within about three weeks. Primary disease may present with a more severe clinical syndrome and complications such as meningitis, encephalitis, or hepatitis. Importantly, more than half of first recognized episodes are not true primary infections, as earlier asymptomatic exposure is common.
Recurrent infection results from viral reactivation in the dorsal root ganglia. The average patient experiences about four recurrences per year. Recurrences are generally milder, involve fewer lesions, and resolve within 10 days. HSV-1 genital infections recur significantly less often than HSV-2.
Asymptomatic infection is common, with intermittent viral shedding that frequently leads to transmission by individuals without symptoms or lesions.
Etiology And Risk Factors
Most cases (70–90%) of genital herpes are caused by HSV-2, with the remainder caused by HSV-1. Genital HSV-1 infections are increasing in prevalence, likely due to higher rates of oral–genital contact and reduced childhood exposure to HSV-1, leaving more adolescents and adults susceptible. Acquisition of HSV-2 in individuals with prior HSV-1 infection tends to be less symptomatic, whereas acquisition of HSV-1 in those with HSV-2 is uncommon.
Genital herpes is strongly associated with HIV and other sexually transmitted infections. HSV infection increases both susceptibility to HIV and HIV viral shedding during HSV reactivation. Despite extensive research, no effective HSV vaccine is currently available.
Clinical Manifestations
Patients commonly report local pain, itching, or burning. A prodrome of tingling, itching, or pain may occur 1–2 days before lesion eruption and can sometimes mimic radicular pain such as sciatica. Lesions typically begin as macules and papules, progress to vesicles and pustules, and then ulcerate by approximately day five. Skin lesions crust over, whereas mucosal lesions heal without crusting.
Associated manifestations may include herpetic cervicitis, vaginitis, or urethritis, presenting with dysuria, urinary hesitancy or retention, vaginal discharge, or pelvic pain. Systemic symptoms such as fever, malaise, headache, myalgias, photophobia, anorexia, and lymphadenopathy are more common during primary infection.
Physical Examination Findings
Examination may reveal grouped vesicles on an erythematous base involving the vulva, vagina, cervix, perineum, buttocks, penile shaft, or glans. On moist mucosal surfaces, ulcers may predominate. Atypical findings include localized edema, erythema, fissures, or crusts.
Special Populations
Pediatric patients with genital herpes require careful evaluation, as neonatal infection is often disseminated or involves the central nervous system and carries high morbidity and mortality. Congenital HSV infection without vesicles may resemble other congenital infections. Sexual abuse must be considered in children with genital HSV, and evaluation for other sexually transmitted infections is recommended.
Pregnancy is a high-risk context, particularly with primary infection, which is associated with significant neonatal morbidity. Suppressive antiviral therapy after 36 weeks’ gestation reduces lesion recurrence at delivery and lowers cesarean section rates.
Diagnostic Approach
Diagnosis is usually clinical, based on history and examination. Laboratory testing is useful when confirmation is needed or in atypical or severe cases. Viral culture from vesicle fluid or ulcer base is positive in most early lesions but loses sensitivity as lesions heal. PCR testing is more sensitive and is the diagnostic test of choice, especially for central nervous system involvement. Serologic testing detects prior exposure but is not useful for diagnosing acute disease, as it cannot distinguish recent from chronic infection.
Differential Diagnosis
Important considerations include syphilis, chancroid, lymphogranuloma venereum, granuloma inguinale, candidiasis, and Behçet syndrome.
Emergency Management
Treatment reduces symptom severity and duration but does not eradicate latent virus or prevent future recurrences once therapy is stopped.
Episodic therapy for recurrences shortens lesion duration if started during the prodrome or within one day of lesion onset.
Suppressive therapy, recommended for patients with frequent recurrences (six or more per year), reduces recurrence frequency by approximately 75% and decreases viral shedding.
Severe disease, systemic involvement, or infection in immunocompromised patients requires intravenous antiviral therapy. Resistance to acyclovir occurs in a minority of immunocompromised patients, in which case foscarnet may be effective. Women with urinary retention may require bladder catheterization due to sacral nerve involvement.
Pharmacologic Treatment
First-episode, recurrent, suppressive, and HIV-associated genital herpes are treated with oral or intravenous acyclovir, valacyclovir, or famciclovir, with regimen selection based on severity, immune status, and recurrence pattern. Dosing adjustments may be necessary in renal impairment.
Disposition And Counseling
Hospital admission is indicated for patients with central nervous system involvement, disseminated disease, severe pain or urinary retention, or significant immunosuppression. Immunocompetent patients without systemic involvement can be managed as outpatients.
Discharge counseling is essential and should emphasize avoidance of sexual contact during prodrome and active lesions, consistent use of barrier protection even when asymptomatic, and the lifelong nature of infection. Patients should be informed about the likelihood of recurrence and the option of suppressive therapy.
Key Clinical Pearls
Primary genital herpes may be severe and should be treated promptly. Always consider sexual abuse in children presenting with genital HSV. Genital herpes is lifelong, with intermittent asymptomatic shedding that plays a major role in transmission.
Overview And Definition
Genital herpes is a lifelong, recurrent viral infection caused by herpes simplex virus (HSV). Approximately one in four Americans older than 30 years are seropositive for HSV-2, though most are asymptomatic. The infection is characterized by periods of latency and reactivation, with viral shedding that can occur even in the absence of visible lesions, contributing to ongoing transmission.
Disease Course And Natural History
Primary infection has an incubation period of approximately 2–12 days, with symptoms typically peaking 8–10 days after onset and lesions healing within about three weeks. Primary disease may present with a more severe clinical syndrome and complications such as meningitis, encephalitis, or hepatitis. Importantly, more than half of first recognized episodes are not true primary infections, as earlier asymptomatic exposure is common.
Recurrent infection results from viral reactivation in the dorsal root ganglia. The average patient experiences about four recurrences per year. Recurrences are generally milder, involve fewer lesions, and resolve within 10 days. HSV-1 genital infections recur significantly less often than HSV-2.
Asymptomatic infection is common, with intermittent viral shedding that frequently leads to transmission by individuals without symptoms or lesions.
Etiology And Risk Factors
Most cases (70–90%) of genital herpes are caused by HSV-2, with the remainder caused by HSV-1. Genital HSV-1 infections are increasing in prevalence, likely due to higher rates of oral–genital contact and reduced childhood exposure to HSV-1, leaving more adolescents and adults susceptible. Acquisition of HSV-2 in individuals with prior HSV-1 infection tends to be less symptomatic, whereas acquisition of HSV-1 in those with HSV-2 is uncommon.
Genital herpes is strongly associated with HIV and other sexually transmitted infections. HSV infection increases both susceptibility to HIV and HIV viral shedding during HSV reactivation. Despite extensive research, no effective HSV vaccine is currently available.
Clinical Manifestations
Patients commonly report local pain, itching, or burning. A prodrome of tingling, itching, or pain may occur 1–2 days before lesion eruption and can sometimes mimic radicular pain such as sciatica. Lesions typically begin as macules and papules, progress to vesicles and pustules, and then ulcerate by approximately day five. Skin lesions crust over, whereas mucosal lesions heal without crusting.
Associated manifestations may include herpetic cervicitis, vaginitis, or urethritis, presenting with dysuria, urinary hesitancy or retention, vaginal discharge, or pelvic pain. Systemic symptoms such as fever, malaise, headache, myalgias, photophobia, anorexia, and lymphadenopathy are more common during primary infection.
Physical Examination Findings
Examination may reveal grouped vesicles on an erythematous base involving the vulva, vagina, cervix, perineum, buttocks, penile shaft, or glans. On moist mucosal surfaces, ulcers may predominate. Atypical findings include localized edema, erythema, fissures, or crusts.
Special Populations
Pediatric patients with genital herpes require careful evaluation, as neonatal infection is often disseminated or involves the central nervous system and carries high morbidity and mortality. Congenital HSV infection without vesicles may resemble other congenital infections. Sexual abuse must be considered in children with genital HSV, and evaluation for other sexually transmitted infections is recommended.
Pregnancy is a high-risk context, particularly with primary infection, which is associated with significant neonatal morbidity. Suppressive antiviral therapy after 36 weeks’ gestation reduces lesion recurrence at delivery and lowers cesarean section rates.
Diagnostic Approach
Diagnosis is usually clinical, based on history and examination. Laboratory testing is useful when confirmation is needed or in atypical or severe cases. Viral culture from vesicle fluid or ulcer base is positive in most early lesions but loses sensitivity as lesions heal. PCR testing is more sensitive and is the diagnostic test of choice, especially for central nervous system involvement. Serologic testing detects prior exposure but is not useful for diagnosing acute disease, as it cannot distinguish recent from chronic infection.
Differential Diagnosis
Important considerations include syphilis, chancroid, lymphogranuloma venereum, granuloma inguinale, candidiasis, and Behçet syndrome.
Emergency Management
Treatment reduces symptom severity and duration but does not eradicate latent virus or prevent future recurrences once therapy is stopped.
Episodic therapy for recurrences shortens lesion duration if started during the prodrome or within one day of lesion onset.
Suppressive therapy, recommended for patients with frequent recurrences (six or more per year), reduces recurrence frequency by approximately 75% and decreases viral shedding.
Severe disease, systemic involvement, or infection in immunocompromised patients requires intravenous antiviral therapy. Resistance to acyclovir occurs in a minority of immunocompromised patients, in which case foscarnet may be effective. Women with urinary retention may require bladder catheterization due to sacral nerve involvement.
Pharmacologic Treatment
First-episode, recurrent, suppressive, and HIV-associated genital herpes are treated with oral or intravenous acyclovir, valacyclovir, or famciclovir, with regimen selection based on severity, immune status, and recurrence pattern. Dosing adjustments may be necessary in renal impairment.
Disposition And Counseling
Hospital admission is indicated for patients with central nervous system involvement, disseminated disease, severe pain or urinary retention, or significant immunosuppression. Immunocompetent patients without systemic involvement can be managed as outpatients.
Discharge counseling is essential and should emphasize avoidance of sexual contact during prodrome and active lesions, consistent use of barrier protection even when asymptomatic, and the lifelong nature of infection. Patients should be informed about the likelihood of recurrence and the option of suppressive therapy.
Key Clinical Pearls
Primary genital herpes may be severe and should be treated promptly. Always consider sexual abuse in children presenting with genital HSV. Genital herpes is lifelong, with intermittent asymptomatic shedding that plays a major role in transmission.
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Emergency And Acute Medicine – Herpes Zoster
Definition And Overview
Herpes zoster, commonly known as shingles, is characterized by a unilateral eruption of painful vesicles distributed along a single dermatome. It results from reactivation of latent varicella zoster virus (VZV) in dorsal root ganglia. Dissemination is rare in immunocompetent hosts but occurs more frequently in immunocompromised patients. The disease is most common in individuals with impaired cell-mediated immunity, particularly adults older than 50 years, patients with malignancy, and those receiving immunosuppressive therapy.
Etiology And Risk Factors
Herpes zoster is caused by reactivation of VZV, a DNA virus in the Herpesviridae family. Most cases occur in individuals with a history of primary varicella (chickenpox), and only rarely in vaccinated individuals. Declining cell-mediated immunity with aging or immunosuppression is the primary risk factor.
Pregnancy is not associated with an increased risk of congenital varicella syndrome. In children, herpes zoster may occur following in utero exposure or primary varicella infection during the first six months of life.
Clinical Features
Dermatomal zoster typically begins with a prodrome of pain, paresthesias, or pruritus in the affected dermatome, occurring in approximately 75% of patients. Pain may be sharp, burning, tingling, or severe. The classic rash consists of grouped vesicles on an erythematous base, which progress to crusting over 7–10 days, with complete resolution in 2–3 weeks. Thoracic and lumbar dermatomes are most commonly involved, followed by trigeminal and cervical distributions.
Zoster sine herpete presents with dermatomal pain without rash and can be diagnostically challenging.
Herpes zoster ophthalmicus results from involvement of the ophthalmic division of the trigeminal nerve. Hutchinson sign, defined as vesicles on the tip of the nose, suggests nasociliary nerve involvement and increased risk of ocular complications. Ocular findings may include punctate keratitis or corneal pseudodendrites, which are less ulcerative and show less fluorescein uptake than HSV dendrites.
Ramsay Hunt syndrome is caused by involvement of cranial nerves VII and VIII and presents with vesicles in the external auditory canal, peripheral facial palsy, vertigo, and altered sensation of the anterior two-thirds of the tongue.
Disseminated disease may occur, particularly in immunocompromised patients, and can involve the central nervous system, liver, or lungs, leading to complications such as meningoencephalitis, myelitis, hepatitis, pneumonitis, and peripheral neuropathy.
Postherpetic neuralgia is defined as pain persisting at the site of zoster lesions for more than three months after cutaneous healing. It occurs in 10–70% of patients, with risk increasing in those older than 50 years and in patients with severe initial rash or pain.
Diagnostic Evaluation
Diagnosis is primarily clinical in typical presentations. Laboratory testing is reserved for atypical cases, disseminated disease, or immunocompromised patients. Tzanck smear may show multinucleated giant cells but cannot distinguish VZV from HSV and has low sensitivity. PCR testing from vesicle fluid, blood, cerebrospinal fluid, or bronchoalveolar lavage is the preferred diagnostic method due to high sensitivity and specificity. Serologic testing is less reliable in the acute setting, and viral culture is slow and insensitive.
Differential Diagnosis
Conditions to consider include primary varicella, herpes simplex infection, cellulitis, allergic contact dermatitis, bullous impetigo, molluscum contagiosum, insect bites, trigeminal neuralgia, radiculopathy, biliary or renal colic, Bell palsy, peripheral vertigo, conjunctivitis, and HSV keratitis.
Emergency Management
Herpes zoster is usually self-limited. Management focuses on reducing pain, shortening disease duration, and preventing postherpetic neuralgia. Lesions should be covered, and universal precautions maintained, as zoster can transmit varicella to nonimmune individuals.
In immunocompetent patients, oral antiviral therapy should be initiated within 72 hours of rash onset, though treatment may still be beneficial if new lesions are forming. Valacyclovir is preferred for ease of dosing, though acyclovir or famciclovir are acceptable alternatives. Analgesia should be tailored to pain severity, ranging from nonsteroidal anti-inflammatory drugs to opioids. Corticosteroids remain controversial and may modestly improve acute pain and rash healing but do not prevent postherpetic neuralgia.
Immunocompromised patients require intravenous acyclovir and close monitoring.
Herpes zoster ophthalmicus requires urgent ophthalmology consultation. Oral antivirals are indicated, with intravenous therapy for immunocompromised patients or those with cranial nerve involvement.
Postherpetic neuralgia is managed with analgesics, tricyclic antidepressants, gabapentin or pregabalin, and topical lidocaine; antivirals are not effective once PHN is established.
Postexposure Prophylaxis And Prevention
Varicella zoster immune globulin (VariZIG) is recommended within 72 hours of exposure for immunocompromised patients, pregnant individuals, and certain neonates. The live-attenuated zoster vaccine is recommended for adults over 60 years of age but is contraindicated in pregnancy and immunocompromised patients. Vaccination does not treat active disease or prevent postherpetic neuralgia in those with established zoster.
Disposition And Follow-Up
Most patients can be managed as outpatients. Admission is indicated for immunocompromised patients, disseminated disease, severe or intractable pain, neonatal infection, or herpes zoster ophthalmicus with cranial nerve involvement. Patients should be advised to avoid contact with pregnant or immunocompromised individuals until all lesions have crusted. Long-term follow-up may be required for management of postherpetic neuralgia.
Clinical Pearls And Pitfalls
Always assess for ocular involvement when lesions appear on the tip of the nose. Consider herpes zoster in patients with unexplained dermatomal pain, even in the absence of rash. Fully expose the skin in patients presenting with chest or abdominal pain. Counsel patients early about the risk and chronic nature of postherpetic neuralgia.
Definition And Overview
Herpes zoster, commonly known as shingles, is characterized by a unilateral eruption of painful vesicles distributed along a single dermatome. It results from reactivation of latent varicella zoster virus (VZV) in dorsal root ganglia. Dissemination is rare in immunocompetent hosts but occurs more frequently in immunocompromised patients. The disease is most common in individuals with impaired cell-mediated immunity, particularly adults older than 50 years, patients with malignancy, and those receiving immunosuppressive therapy.
Etiology And Risk Factors
Herpes zoster is caused by reactivation of VZV, a DNA virus in the Herpesviridae family. Most cases occur in individuals with a history of primary varicella (chickenpox), and only rarely in vaccinated individuals. Declining cell-mediated immunity with aging or immunosuppression is the primary risk factor.
Pregnancy is not associated with an increased risk of congenital varicella syndrome. In children, herpes zoster may occur following in utero exposure or primary varicella infection during the first six months of life.
Clinical Features
Dermatomal zoster typically begins with a prodrome of pain, paresthesias, or pruritus in the affected dermatome, occurring in approximately 75% of patients. Pain may be sharp, burning, tingling, or severe. The classic rash consists of grouped vesicles on an erythematous base, which progress to crusting over 7–10 days, with complete resolution in 2–3 weeks. Thoracic and lumbar dermatomes are most commonly involved, followed by trigeminal and cervical distributions.
Zoster sine herpete presents with dermatomal pain without rash and can be diagnostically challenging.
Herpes zoster ophthalmicus results from involvement of the ophthalmic division of the trigeminal nerve. Hutchinson sign, defined as vesicles on the tip of the nose, suggests nasociliary nerve involvement and increased risk of ocular complications. Ocular findings may include punctate keratitis or corneal pseudodendrites, which are less ulcerative and show less fluorescein uptake than HSV dendrites.
Ramsay Hunt syndrome is caused by involvement of cranial nerves VII and VIII and presents with vesicles in the external auditory canal, peripheral facial palsy, vertigo, and altered sensation of the anterior two-thirds of the tongue.
Disseminated disease may occur, particularly in immunocompromised patients, and can involve the central nervous system, liver, or lungs, leading to complications such as meningoencephalitis, myelitis, hepatitis, pneumonitis, and peripheral neuropathy.
Postherpetic neuralgia is defined as pain persisting at the site of zoster lesions for more than three months after cutaneous healing. It occurs in 10–70% of patients, with risk increasing in those older than 50 years and in patients with severe initial rash or pain.
Diagnostic Evaluation
Diagnosis is primarily clinical in typical presentations. Laboratory testing is reserved for atypical cases, disseminated disease, or immunocompromised patients. Tzanck smear may show multinucleated giant cells but cannot distinguish VZV from HSV and has low sensitivity. PCR testing from vesicle fluid, blood, cerebrospinal fluid, or bronchoalveolar lavage is the preferred diagnostic method due to high sensitivity and specificity. Serologic testing is less reliable in the acute setting, and viral culture is slow and insensitive.
Differential Diagnosis
Conditions to consider include primary varicella, herpes simplex infection, cellulitis, allergic contact dermatitis, bullous impetigo, molluscum contagiosum, insect bites, trigeminal neuralgia, radiculopathy, biliary or renal colic, Bell palsy, peripheral vertigo, conjunctivitis, and HSV keratitis.
Emergency Management
Herpes zoster is usually self-limited. Management focuses on reducing pain, shortening disease duration, and preventing postherpetic neuralgia. Lesions should be covered, and universal precautions maintained, as zoster can transmit varicella to nonimmune individuals.
In immunocompetent patients, oral antiviral therapy should be initiated within 72 hours of rash onset, though treatment may still be beneficial if new lesions are forming. Valacyclovir is preferred for ease of dosing, though acyclovir or famciclovir are acceptable alternatives. Analgesia should be tailored to pain severity, ranging from nonsteroidal anti-inflammatory drugs to opioids. Corticosteroids remain controversial and may modestly improve acute pain and rash healing but do not prevent postherpetic neuralgia.
Immunocompromised patients require intravenous acyclovir and close monitoring.
Herpes zoster ophthalmicus requires urgent ophthalmology consultation. Oral antivirals are indicated, with intravenous therapy for immunocompromised patients or those with cranial nerve involvement.
Postherpetic neuralgia is managed with analgesics, tricyclic antidepressants, gabapentin or pregabalin, and topical lidocaine; antivirals are not effective once PHN is established.
Postexposure Prophylaxis And Prevention
Varicella zoster immune globulin (VariZIG) is recommended within 72 hours of exposure for immunocompromised patients, pregnant individuals, and certain neonates. The live-attenuated zoster vaccine is recommended for adults over 60 years of age but is contraindicated in pregnancy and immunocompromised patients. Vaccination does not treat active disease or prevent postherpetic neuralgia in those with established zoster.
Disposition And Follow-Up
Most patients can be managed as outpatients. Admission is indicated for immunocompromised patients, disseminated disease, severe or intractable pain, neonatal infection, or herpes zoster ophthalmicus with cranial nerve involvement. Patients should be advised to avoid contact with pregnant or immunocompromised individuals until all lesions have crusted. Long-term follow-up may be required for management of postherpetic neuralgia.
Clinical Pearls And Pitfalls
Always assess for ocular involvement when lesions appear on the tip of the nose. Consider herpes zoster in patients with unexplained dermatomal pain, even in the absence of rash. Fully expose the skin in patients presenting with chest or abdominal pain. Counsel patients early about the risk and chronic nature of postherpetic neuralgia.
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Emergency And Acute Medicine – Herpes Simplex
Definition And Overview
Herpes simplex is a viral disease characterized by recurrent, painful vesicular lesions involving mucocutaneous surfaces. Commonly affected sites include the lips, oral cavity, genitalia, rectum, hands, and eyes. Infection occurs in two phases: a primary phase during which the virus establishes latency within sensory nerve ganglia, and a secondary phase marked by recurrent reactivation at the same anatomic site. The incubation period is approximately four days after exposure. Viral shedding lasts about 7–10 days in primary infection (up to 23 days) and 3–4 days during recurrences. Neonatal infection may occur in utero, intrapartum (most commonly), or postnatally, with an incidence of approximately 1 in 3,500 births annually in the United States. Transmission is human-to-human, and 60–90% of the population is infected with herpes simplex virus type 1 (HSV-1) or type 2 (HSV-2). Infection is more common in Black populations than White populations under 40 years of age, and females are affected more frequently than males.
Etiology And Virology
HSV-1 and HSV-2 are DNA viruses belonging to the Herpesviridae family. Transmission occurs through contact with infected secretions via mucous membranes or abraded skin, as well as through respiratory droplets. Recurrent asymptomatic mucosal shedding can transmit infection. HSV-1 most commonly causes oral infections, while HSV-2 more frequently causes genital disease, though either virus may infect oral or genital mucosa. Recurrence rates vary depending on viral type and anatomic location; HSV-2 genital infections recur more frequently than HSV-1 genital infections, and HSV-1 oral infections recur more often than genital HSV-1 infections.
Clinical Manifestations
Many primary infections are subclinical and detected only by serologic evidence of antibodies. Symptomatic disease typically presents with grouped 1–2 mm vesicles on an erythematous base, containing clear or cloudy fluid or appearing pustular.
Orofacial Disease
Primary orofacial infection often manifests as gingivostomatitis or pharyngitis with ulcerative lesions of the gingiva and mucosa. Associated symptoms include fever, malaise, irritability, headache, myalgias, and cervical lymphadenopathy. Symptoms may last two to four weeks and usually heal without scarring. Pain may limit oral intake, increasing the risk of dehydration.
Recurrent disease typically involves the lips, especially the vermilion border, and is often preceded by a prodrome of tingling, itching, burning, or throbbing. Triggers include sunlight, stress, heat, trauma, and immunosuppression. Lesions progress from erythema to vesicle, ulcer, crusting, and healing. Transmission can occur even without visible lesions.
Cutaneous Disease
Herpetic whitlow involves infection of the fingers, most commonly caused by HSV-2, and presents with vesicles on the pulp or lateral finger. It may occur from autoinoculation or occupational exposure and can last three to four weeks.
Traumatic herpes may develop after cosmetic, surgical, dental procedures, sun exposure, or burns.
Herpes gladiatorum is a cutaneous infection seen in athletes, particularly wrestlers, affecting the face, chest, and hands.
Eczema herpeticum is a disseminated HSV infection occurring in patients with atopic dermatitis, often associated with fever, headache, fatigue, and risk of secondary bacterial infection.
Ocular Disease
HSV is the most common cause of corneal blindness in developed countries. Infection may result from direct inoculation or spread from facial lesions. Symptoms include eye pain, photophobia, blurry vision, conjunctivitis, chemosis, and periauricular lymphadenopathy. Dendritic corneal lesions are seen on fluorescein examination. Vesicles on the tip of the nose (Hutchinson sign) suggest involvement of the nasociliary nerve and increased risk of ocular disease.
Central Nervous System Involvement
HSV encephalitis is the most common cause of severe sporadic encephalitis in the Western world, usually due to HSV-1 reactivation. Patients may present with altered mental status, seizures, focal neurologic deficits, or coma, with or without a history of mucocutaneous herpes.
Special Populations
In neonates, up to 60–80% of infected infants are born to mothers without known genital herpes. Vesicular lesions may be absent initially. Primary maternal genital infection carries the highest risk of neonatal transmission.
In children, primary infection often occurs early in life, with gingivostomatitis being the most common presentation in those under five years of age.
In pregnancy, suppressive antiviral therapy near term may reduce recurrence and viral shedding.
Diagnostic Evaluation
Diagnosis is often clinical based on history and examination. Definitive testing is indicated in severe disease, immunocompromised patients, suspected abuse, ocular involvement, or CNS disease. PCR testing of vesicular fluid is the most sensitive and specific diagnostic method. Viral culture, fluorescent antibody detection, and Tzanck smear may also be used.
Ocular disease requires fluorescein examination, and CNS disease requires lumbar puncture with CSF PCR, along with neuroimaging.
Differential Diagnosis
Conditions to consider include bacterial pharyngitis, Stevens–Johnson syndrome, herpes zoster, varicella, impetigo, syphilis, contact dermatitis, conjunctivitis, corneal abrasion, glaucoma, and other causes of encephalitis.
Emergency Management
Supportive care with hydration and analgesia is sufficient for mild primary infections in immunocompetent patients. Antiviral therapy is indicated for severe primary disease, recurrent infections with significant symptoms, immunocompromised patients, ocular disease, neonatal infection, and encephalitis. Incision and drainage of herpetic lesions should be avoided. Ocular involvement requires urgent ophthalmology consultation, and corticosteroids should not be used.
Pharmacologic Therapy
First-line treatment includes acyclovir, valacyclovir, or famciclovir. Acyclovir is preferred in children and severe disease. Encephalitis requires high-dose IV acyclovir for 14–21 days. Long-term suppressive therapy may be used for frequent recurrences. Antiviral dosing should be adjusted in renal impairment.
Disposition And Follow-Up
Hospital admission is required for encephalitis, neonatal infection, disseminated disease, severe dehydration, immunocompromised patients, and significant ocular involvement. Uncomplicated localized disease may be managed on an outpatient basis. Patients should be counseled on transmission risk, avoidance of contact with lesions, and indications for suppressive therapy.
Clinical Pearls And Pitfalls
Failure to recognize HSV encephalitis or ocular herpes can result in significant morbidity. Transmission may occur without visible lesions. Patients should be warned about autoinoculation and spread to others, especially during outbreaks. Avoid topical or systemic steroids unless specifically indicated and supervised.
Definition And Overview
Herpes simplex is a viral disease characterized by recurrent, painful vesicular lesions involving mucocutaneous surfaces. Commonly affected sites include the lips, oral cavity, genitalia, rectum, hands, and eyes. Infection occurs in two phases: a primary phase during which the virus establishes latency within sensory nerve ganglia, and a secondary phase marked by recurrent reactivation at the same anatomic site. The incubation period is approximately four days after exposure. Viral shedding lasts about 7–10 days in primary infection (up to 23 days) and 3–4 days during recurrences. Neonatal infection may occur in utero, intrapartum (most commonly), or postnatally, with an incidence of approximately 1 in 3,500 births annually in the United States. Transmission is human-to-human, and 60–90% of the population is infected with herpes simplex virus type 1 (HSV-1) or type 2 (HSV-2). Infection is more common in Black populations than White populations under 40 years of age, and females are affected more frequently than males.
Etiology And Virology
HSV-1 and HSV-2 are DNA viruses belonging to the Herpesviridae family. Transmission occurs through contact with infected secretions via mucous membranes or abraded skin, as well as through respiratory droplets. Recurrent asymptomatic mucosal shedding can transmit infection. HSV-1 most commonly causes oral infections, while HSV-2 more frequently causes genital disease, though either virus may infect oral or genital mucosa. Recurrence rates vary depending on viral type and anatomic location; HSV-2 genital infections recur more frequently than HSV-1 genital infections, and HSV-1 oral infections recur more often than genital HSV-1 infections.
Clinical Manifestations
Many primary infections are subclinical and detected only by serologic evidence of antibodies. Symptomatic disease typically presents with grouped 1–2 mm vesicles on an erythematous base, containing clear or cloudy fluid or appearing pustular.
Orofacial Disease
Primary orofacial infection often manifests as gingivostomatitis or pharyngitis with ulcerative lesions of the gingiva and mucosa. Associated symptoms include fever, malaise, irritability, headache, myalgias, and cervical lymphadenopathy. Symptoms may last two to four weeks and usually heal without scarring. Pain may limit oral intake, increasing the risk of dehydration.
Recurrent disease typically involves the lips, especially the vermilion border, and is often preceded by a prodrome of tingling, itching, burning, or throbbing. Triggers include sunlight, stress, heat, trauma, and immunosuppression. Lesions progress from erythema to vesicle, ulcer, crusting, and healing. Transmission can occur even without visible lesions.
Cutaneous Disease
Herpetic whitlow involves infection of the fingers, most commonly caused by HSV-2, and presents with vesicles on the pulp or lateral finger. It may occur from autoinoculation or occupational exposure and can last three to four weeks.
Traumatic herpes may develop after cosmetic, surgical, dental procedures, sun exposure, or burns.
Herpes gladiatorum is a cutaneous infection seen in athletes, particularly wrestlers, affecting the face, chest, and hands.
Eczema herpeticum is a disseminated HSV infection occurring in patients with atopic dermatitis, often associated with fever, headache, fatigue, and risk of secondary bacterial infection.
Ocular Disease
HSV is the most common cause of corneal blindness in developed countries. Infection may result from direct inoculation or spread from facial lesions. Symptoms include eye pain, photophobia, blurry vision, conjunctivitis, chemosis, and periauricular lymphadenopathy. Dendritic corneal lesions are seen on fluorescein examination. Vesicles on the tip of the nose (Hutchinson sign) suggest involvement of the nasociliary nerve and increased risk of ocular disease.
Central Nervous System Involvement
HSV encephalitis is the most common cause of severe sporadic encephalitis in the Western world, usually due to HSV-1 reactivation. Patients may present with altered mental status, seizures, focal neurologic deficits, or coma, with or without a history of mucocutaneous herpes.
Special Populations
In neonates, up to 60–80% of infected infants are born to mothers without known genital herpes. Vesicular lesions may be absent initially. Primary maternal genital infection carries the highest risk of neonatal transmission.
In children, primary infection often occurs early in life, with gingivostomatitis being the most common presentation in those under five years of age.
In pregnancy, suppressive antiviral therapy near term may reduce recurrence and viral shedding.
Diagnostic Evaluation
Diagnosis is often clinical based on history and examination. Definitive testing is indicated in severe disease, immunocompromised patients, suspected abuse, ocular involvement, or CNS disease. PCR testing of vesicular fluid is the most sensitive and specific diagnostic method. Viral culture, fluorescent antibody detection, and Tzanck smear may also be used.
Ocular disease requires fluorescein examination, and CNS disease requires lumbar puncture with CSF PCR, along with neuroimaging.
Differential Diagnosis
Conditions to consider include bacterial pharyngitis, Stevens–Johnson syndrome, herpes zoster, varicella, impetigo, syphilis, contact dermatitis, conjunctivitis, corneal abrasion, glaucoma, and other causes of encephalitis.
Emergency Management
Supportive care with hydration and analgesia is sufficient for mild primary infections in immunocompetent patients. Antiviral therapy is indicated for severe primary disease, recurrent infections with significant symptoms, immunocompromised patients, ocular disease, neonatal infection, and encephalitis. Incision and drainage of herpetic lesions should be avoided. Ocular involvement requires urgent ophthalmology consultation, and corticosteroids should not be used.
Pharmacologic Therapy
First-line treatment includes acyclovir, valacyclovir, or famciclovir. Acyclovir is preferred in children and severe disease. Encephalitis requires high-dose IV acyclovir for 14–21 days. Long-term suppressive therapy may be used for frequent recurrences. Antiviral dosing should be adjusted in renal impairment.
Disposition And Follow-Up
Hospital admission is required for encephalitis, neonatal infection, disseminated disease, severe dehydration, immunocompromised patients, and significant ocular involvement. Uncomplicated localized disease may be managed on an outpatient basis. Patients should be counseled on transmission risk, avoidance of contact with lesions, and indications for suppressive therapy.
Clinical Pearls And Pitfalls
Failure to recognize HSV encephalitis or ocular herpes can result in significant morbidity. Transmission may occur without visible lesions. Patients should be warned about autoinoculation and spread to others, especially during outbreaks. Avoid topical or systemic steroids unless specifically indicated and supervised.
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Emergency And Acute Medicine – Hernias
Definition And Classification
A hernia is the protrusion of an organ or bodily structure through a defect in the tissues that normally contain it. Hernias are classified as external when the protrusion is visible outside the body, internal when herniated contents remain within a body cavity, and interparietal when the hernial sac lies within the abdominal wall layers. Abdominal wall hernias result from weakness or disruption of the fibromuscular layers of the abdominal wall. Groin hernias include inguinal (direct and indirect) and femoral hernias, while ventral hernias include epigastric, umbilical, spigelian, and incisional hernias.
Types Of Hernias
Indirect inguinal hernias arise from persistence of the processus vaginalis, allowing peritoneal contents to pass through the internal inguinal ring. They are more common on the right side and carry a lifetime repair risk of approximately 27% in men and 3% in women.
Direct inguinal hernias occur due to weakness in the transversalis fascia within Hesselbach’s triangle, bounded by the inguinal ligament inferiorly, inferior epigastric vessels laterally, and the rectus abdominis medially.
Femoral hernias result from herniation through the femoral canal beneath the inguinal ligament and have a high risk of incarceration due to their narrow neck.
Incisional hernias occur from failure of prior surgical fascial closure.
Umbilical hernias arise from congenital failure of the umbilical ring to close or acquired weakness at the umbilicus; they frequently incarcerate in adults but often close spontaneously in infants.
Epigastric hernias occur along the midline between the xiphoid and umbilicus.
Spigelian hernias protrude through the oblique fascia lateral to the rectus abdominis muscle.
Obturator hernias pass through the obturator membrane and may compress the obturator nerve, causing medial thigh pain.
Lumbar hernias occur through defects in the posterior abdominal wall and have a relatively high incarceration rate.
Epidemiology
Herniorrhaphy is one of the most common general surgical procedures, with over 750,000 repairs performed annually in the United States. Hernias affect approximately 5% of the population. Groin and femoral hernias account for about 85% of cases, while umbilical and incisional hernias comprise most of the remainder.
Pathophysiology And Hernia States
A reducible hernia can be manually returned to the abdominal cavity. An incarcerated hernia cannot be reduced and may lead to obstruction. A strangulated hernia involves compromised blood supply to entrapped bowel, resulting in ischemia, necrosis, and potential gangrene. Hernias with small necks and large sacs are at particularly high risk. Symptoms of bowel obstruction or ischemia, including nausea, vomiting, fever, and leukocytosis, may be present.
Clinical Presentation
Patients typically report localized pain and swelling at the hernia site. Pain may worsen with straining or positional changes and improve with rest. Persistent pain, vomiting, or fever suggests incarceration or strangulation. Vital signs are often normal but may show tachycardia, hypotension, or fever in complicated cases. Physical examination may reveal skin color changes, tenderness, or irreducibility. Inguinal hernias may present as scrotal swelling in men or a groin or labial bulge in women. Femoral hernias appear inferior to the inguinal ligament. Obturator hernias may present with intermittent bowel obstruction and medial thigh pain worsened by hip extension.
Special Populations
In children, hernias may be intermittent and difficult to detect, with incarceration rates as high as 10–20%, particularly in infants younger than six months. Umbilical hernias in children usually close spontaneously.
During pregnancy, hernias are uncommon but may pose diagnostic challenges; emergent surgical consultation is required if incarceration or strangulation is suspected.
Older adults have a higher risk of bowel resection and postoperative complications when hernias become incarcerated.
Diagnostic Evaluation
Diagnosis is primarily clinical, based on careful history and physical examination, often aided by examination during standing or Valsalva maneuver. Laboratory studies may reveal leukocytosis in strangulation or electrolyte abnormalities with dehydration. Imaging is reserved for unclear cases or suspected complications. Ultrasound is useful for groin and abdominal wall hernias, while CT is preferred for obturator or spigelian hernias and in obese or complex cases.
Emergency Management
Initial management includes assessment of airway, breathing, and circulation, with intravenous fluid resuscitation for dehydration, obstruction, or sepsis. Incarcerated or strangulated hernias require urgent surgical consultation. Nasogastric decompression is indicated for bowel obstruction. Manual reduction may be attempted in selected patients without signs of strangulation, using gentle, sustained pressure with adequate analgesia and sedation. Reduction should not be attempted in patients with fever, leukocytosis, or signs of ischemia.
Medications
Analgesia is provided with opioids such as morphine or fentanyl. Sedation with benzodiazepines may be used to facilitate reduction when appropriate. Broad-spectrum antibiotics are considered in cases of suspected strangulation or sepsis.
Disposition And Follow-Up
Strangulated hernias require immediate surgical intervention and admission. Incarcerated hernias generally require admission for urgent surgery. Patients may be discharged after successful reduction if asymptomatic, with clear instructions and surgical follow-up. All patients should be referred for elective surgical evaluation.
Clinical Pearls And Pitfalls
Failure to recognize incarceration or strangulation can lead to bowel ischemia and sepsis. Forcing reduction in the presence of strangulation may return necrotic bowel to the abdomen, delaying diagnosis and worsening outcomes. Early surgical consultation is essential when complications are suspected.
Definition And Classification
A hernia is the protrusion of an organ or bodily structure through a defect in the tissues that normally contain it. Hernias are classified as external when the protrusion is visible outside the body, internal when herniated contents remain within a body cavity, and interparietal when the hernial sac lies within the abdominal wall layers. Abdominal wall hernias result from weakness or disruption of the fibromuscular layers of the abdominal wall. Groin hernias include inguinal (direct and indirect) and femoral hernias, while ventral hernias include epigastric, umbilical, spigelian, and incisional hernias.
Types Of Hernias
Indirect inguinal hernias arise from persistence of the processus vaginalis, allowing peritoneal contents to pass through the internal inguinal ring. They are more common on the right side and carry a lifetime repair risk of approximately 27% in men and 3% in women.
Direct inguinal hernias occur due to weakness in the transversalis fascia within Hesselbach’s triangle, bounded by the inguinal ligament inferiorly, inferior epigastric vessels laterally, and the rectus abdominis medially.
Femoral hernias result from herniation through the femoral canal beneath the inguinal ligament and have a high risk of incarceration due to their narrow neck.
Incisional hernias occur from failure of prior surgical fascial closure.
Umbilical hernias arise from congenital failure of the umbilical ring to close or acquired weakness at the umbilicus; they frequently incarcerate in adults but often close spontaneously in infants.
Epigastric hernias occur along the midline between the xiphoid and umbilicus.
Spigelian hernias protrude through the oblique fascia lateral to the rectus abdominis muscle.
Obturator hernias pass through the obturator membrane and may compress the obturator nerve, causing medial thigh pain.
Lumbar hernias occur through defects in the posterior abdominal wall and have a relatively high incarceration rate.
Epidemiology
Herniorrhaphy is one of the most common general surgical procedures, with over 750,000 repairs performed annually in the United States. Hernias affect approximately 5% of the population. Groin and femoral hernias account for about 85% of cases, while umbilical and incisional hernias comprise most of the remainder.
Pathophysiology And Hernia States
A reducible hernia can be manually returned to the abdominal cavity. An incarcerated hernia cannot be reduced and may lead to obstruction. A strangulated hernia involves compromised blood supply to entrapped bowel, resulting in ischemia, necrosis, and potential gangrene. Hernias with small necks and large sacs are at particularly high risk. Symptoms of bowel obstruction or ischemia, including nausea, vomiting, fever, and leukocytosis, may be present.
Clinical Presentation
Patients typically report localized pain and swelling at the hernia site. Pain may worsen with straining or positional changes and improve with rest. Persistent pain, vomiting, or fever suggests incarceration or strangulation. Vital signs are often normal but may show tachycardia, hypotension, or fever in complicated cases. Physical examination may reveal skin color changes, tenderness, or irreducibility. Inguinal hernias may present as scrotal swelling in men or a groin or labial bulge in women. Femoral hernias appear inferior to the inguinal ligament. Obturator hernias may present with intermittent bowel obstruction and medial thigh pain worsened by hip extension.
Special Populations
In children, hernias may be intermittent and difficult to detect, with incarceration rates as high as 10–20%, particularly in infants younger than six months. Umbilical hernias in children usually close spontaneously.
During pregnancy, hernias are uncommon but may pose diagnostic challenges; emergent surgical consultation is required if incarceration or strangulation is suspected.
Older adults have a higher risk of bowel resection and postoperative complications when hernias become incarcerated.
Diagnostic Evaluation
Diagnosis is primarily clinical, based on careful history and physical examination, often aided by examination during standing or Valsalva maneuver. Laboratory studies may reveal leukocytosis in strangulation or electrolyte abnormalities with dehydration. Imaging is reserved for unclear cases or suspected complications. Ultrasound is useful for groin and abdominal wall hernias, while CT is preferred for obturator or spigelian hernias and in obese or complex cases.
Emergency Management
Initial management includes assessment of airway, breathing, and circulation, with intravenous fluid resuscitation for dehydration, obstruction, or sepsis. Incarcerated or strangulated hernias require urgent surgical consultation. Nasogastric decompression is indicated for bowel obstruction. Manual reduction may be attempted in selected patients without signs of strangulation, using gentle, sustained pressure with adequate analgesia and sedation. Reduction should not be attempted in patients with fever, leukocytosis, or signs of ischemia.
Medications
Analgesia is provided with opioids such as morphine or fentanyl. Sedation with benzodiazepines may be used to facilitate reduction when appropriate. Broad-spectrum antibiotics are considered in cases of suspected strangulation or sepsis.
Disposition And Follow-Up
Strangulated hernias require immediate surgical intervention and admission. Incarcerated hernias generally require admission for urgent surgery. Patients may be discharged after successful reduction if asymptomatic, with clear instructions and surgical follow-up. All patients should be referred for elective surgical evaluation.
Clinical Pearls And Pitfalls
Failure to recognize incarceration or strangulation can lead to bowel ischemia and sepsis. Forcing reduction in the presence of strangulation may return necrotic bowel to the abdomen, delaying diagnosis and worsening outcomes. Early surgical consultation is essential when complications are suspected.
