Epidemiology & Risk Factors

  • Often diagnosed in children (peak age 2–7 years), but increasingly recognized in adults.
  • Strongly associated with a personal or family hx of migraines (hypothesized mitochondrial DNA mutations or dysautonomia).
  • Triggers: Psychological stress (positive or negative), infections (e.g., URI), physical exertion, sleep deprivation, or specific dietary triggers (chocolate, cheese).

Clinical Features

  • Stereotypical episodes: Each episode is identical in onset, symptoms, and duration within a single patient. c
  • Divided into 3 phases:
    • Prodrome: Intense nausea, pallor, diaphoresis, lethargy.
    • Emetic phase: Acute, severe, relentless vomiting/retching (often beginning in early morning, lasting hours to days).
    • Recovery phase: Gradual return of appetite and energy.
  • Symptom-free intervals between episodes (lasting weeks to months).
  • Accompanying symptoms: Abdominal pain, photophobia, headache.

Diagnosis

  • Primarily a clinical diagnosis of exclusion; no single diagnostic test is available.
  • Rome IV Criteria:
    • discrete episodes in the past year (or in past 6 months), occurring week apart.
    • Stereotypic onset and duration (< 1 week).
    • Complete absence of vomiting between episodes.
  • Initial Workup (essential to rule out organic/anatomical causes):
    • Key Labs: BMP (assess for hypokalemia, metabolic alkalosis), LFTs, amylase/lipase (to rule out pancreatitis/biliary pathology).
    • Imaging: Abdominal US or CT (exclude structural abnormalities like malrotation, intermittent bowel obstruction, or gallstones).
    • Upper GI Endoscopy: To exclude severe GERD, gastritis, or PUD.

Differential Diagnostics

  • Cannabinoid Hyperemesis Syndrome (CHS):
    • Diff: Chronic, daily marijuana use; vomiting episodes temporarily relieved by hot showers/baths; symptoms resolve completely within weeks of cannabis cessation.
  • Intestinal Malrotation / Volvulus:
    • Diff: Acute-onset bilious vomiting, abdominal distension; diagnosed via upper GI contrast series (corkscrew sign) or abdominal US; represents a surgical emergency.
  • Inborn Errors of Metabolism (e.g., urea cycle disorders, organic acidemias):
    • Diff: Typically presents in infancy/early childhood; associated with developmental delay, hyperammonemia, or metabolic acidosis triggered by fasting or illness.
  • Abdominal Migraine:
    • Diff: Paroxysmal abdominal pain is the predominant symptom rather than vomiting; also associated with migraine FHx.

Management

  • Acute Phase (Abortive & Supportive):
    1. IV Fluid (IVF) resuscitation: Aggressive hydration with normal saline or D5NS + electrolyte replacement (especially K+ and Cl-).
    2. Antiemetics: IV ondansetron (5-HT3 antagonist) or IV aprepitant (NK1 receptor antagonist).
    3. Migraine Abortives: IV/SC triptans (e.g., sumatriptan) if patient has a personal/family hx of migraines or severe prodromal headache.
    4. Supportive: Keep patient in a quiet, dark room; consider sedation with diphenhydramine or lorazepam for severe agitation.
  • Prophylactic Therapy (Indicated if episodes are frequent, severe, or cause significant loss of school/work days):
    1. First-Line (Age ≥ 5 / Adults): Amitriptyline (TCA).
    2. First-Line (Age < 5): Cyproheptadine (first-generation antihistamine/antiserotonergic).
    3. Alternative Prophylaxis: Propranolol (beta-blocker) or Topiramate.
    4. Supplements: Coenzyme Q10 and L-carnitine are frequently used as adjunctive therapies.

Complications

  • Severe dehydration and hypovolemic shock.
  • Electrolyte abnormalities: Hypokalemia, hypochloremic metabolic alkalosis (due to continuous loss of gastric HCl).
  • Mallory-Weiss tears (mucosal lacerations at the gastroesophageal junction from repeated retching).
  • Peptic esophagitis or esophageal perforation (Boerhaave syndrome).
  • Dental caries/enamel erosion (chronic acid exposure).