Cyclic Vomiting Syndrome in Children and Adolescents
Diagnosis
Diagnose CVS using Rome IV criteria: stereotypical episodes of acute-onset vomiting lasting <7 days, with ≥3 discrete episodes in a year (≥2 in the prior 6 months), separated by at least 1 week of baseline health between episodes. 1, 2
Key Diagnostic Features
- Stereotypical pattern is essential: each episode must be identical in timing, duration, and associated symptoms for that individual patient 1
- Prodromal symptoms occur in ~65-70% of cases: look for impending sense of doom, panic, anxiety, restlessness, diaphoresis, flushing, mental fog, headache, or bowel urgency—these typically last a median of 1 hour before vomiting begins 1, 2
- Episodes most commonly begin in early morning hours (pre-dawn) 1
- Abdominal pain is present in most patients and should not exclude the diagnosis 1
- Personal or family history of migraine is present in 20-30% of patients and supports the diagnosis 1, 2
Critical Diagnostic Distinction
Screen all patients for cannabis use before confirming CVS diagnosis: use >4 times weekly for >1 year suggests cannabinoid hyperemesis syndrome (CHS) rather than CVS, requiring 6 months of cessation to differentiate. 1, 2, 3
- Hot water bathing is NOT pathognomonic for CHS: this behavior occurs in 48% of CVS patients who don't use cannabis 2, 3
Red Flags Requiring Alternative Workup
- Bilious vomiting is a surgical emergency: requires urgent upper GI series to exclude malrotation with volvulus, even with normal initial exam 3
- Fewer than 3 lifetime episodes: do not diagnose CVS, as viral gastroenteritis is far more common 3
Acute Episode Management
Prodromal Phase (Abortive Therapy)
The highest probability of aborting an episode occurs when medications are taken immediately at prodromal symptom onset—educate caregivers to recognize these warning signs. 1, 2
Standard abortive regimen:
- Sumatriptan 20 mg intranasal spray (can repeat once after 2 hours, maximum 2 doses per 24 hours; administer in head-forward position to optimize nasal receptor contact) 1
- PLUS Ondansetron 8 mg sublingual every 4-6 hours 1
Additional abortive agents if needed:
- Promethazine 12.5-25 mg oral/rectal every 4-6 hours 1
- Prochlorperazine 5-10 mg every 6-8 hours or 25 mg suppository every 12 hours 1
- Sedatives (alprazolam, lorazepam, diphenhydramine) to truncate the episode—use caution in adolescents with substance abuse risk 1, 2
Emetic Phase (Emergency Department Management)
If home abortive therapy fails, immediate ED interventions include:
- Place patient in quiet, dark room to minimize sensory stimulation, as patients are often agitated and unable to communicate 1, 2
- Aggressive IV fluid replacement with dextrose-containing fluids for rehydration and metabolic support 1, 2
- Ondansetron 8 mg IV every 4-6 hours as first-line antiemetic 1, 2
- IV ketorolac 15-30 mg every 6 hours (maximum 5 days, daily maximum 120 mg) as first-line non-narcotic analgesia—avoid opioids as they worsen nausea and carry addiction risk 1, 2
- IV benzodiazepines for sedation 1, 2
- Check and correct electrolyte abnormalities immediately 2
For refractory cases:
- Droperidol or haloperidol as dopamine antagonists 1, 2
- Multiple concurrent agents with different mechanisms administered around-the-clock rather than PRN 2
Recovery Phase
- Rehydration with electrolyte-rich fluids (sports drinks) with small, frequent sips as tolerated 2
- Gradual introduction of nutrient drinks 2
Prophylactic Therapy
Disease Severity Classification
Classify CVS severity to determine treatment intensity: 1, 2
- Mild CVS: <4 episodes/year, each lasting <2 days, no ED visits or hospitalizations → abortive therapy only
- Moderate-severe CVS: ≥4 episodes/year, lasting >2 days, requiring ED visits or hospitalizations → both prophylactic AND abortive therapy
First-Line Prophylactic Agent
Amitriptyline is first-line prophylaxis with 67-75% response rate: 1, 2
- Starting dose: 25 mg at bedtime
- Goal dose: 1-1.5 mg/kg at bedtime (typically 75-150 mg nightly)
- Monitor baseline ECG due to QTc prolongation risk 1
Second-Line Prophylactic Agents
If amitriptyline fails or is contraindicated, use: 1
- Topiramate: start 25 mg daily, titrate to 100-150 mg daily in divided doses; monitor electrolytes and renal function twice yearly
- Levetiracetam: start 500 mg twice daily, titrate to 1000-2000 mg daily in divided doses; monitor CBC
- Zonisamide: start 100 mg daily, titrate to 200-400 mg daily; monitor electrolytes and renal function twice yearly
Adjunctive Prophylactic Therapy
Aprepitant (neurokinin-1 antagonist): 1
- 80 mg 2-3 times weekly for adolescents 40-60 kg
- 125 mg 2-3 times weekly for adolescents >60 kg
Essential Lifestyle Modifications and Trigger Management
All patients require the following interventions regardless of disease severity: 1, 2
- Regular sleep schedule and avoiding sleep deprivation 1
- Avoiding prolonged fasting 1
- Stress management techniques 1
- Identifying and avoiding individual triggers—identifiable triggers are found in 70-80% of children with CVS 1
- Common triggers in children: infections, psychological stress (including positive events), sleep deprivation, prolonged fasting, travel-related disruptions 1
Comorbidity Screening and Management
Screen all patients for anxiety, depression, and panic disorder: these psychiatric comorbidities are present in 50-60% of CVS patients, and treating underlying anxiety can decrease CVS episode frequency. 1, 2
- Consider cognitive-behavioral therapy to manage psychological triggers 2
- Referral to psychiatry, psychology, or counseling may be necessary 2
Common Pitfalls to Avoid
- Missing the prodromal window dramatically reduces abortive therapy effectiveness—caregiver education is critical 1, 2
- Do not misinterpret self-soothing behaviors (excessive water intake, self-induced vomiting) as malingering—these are characteristic coping responses in CVS 1, 2
- Do not overlook retching and nausea: these symptoms are equally disabling as vomiting itself and require aggressive treatment 1, 2
- Do not underestimate CVS severity: approximately one-third of adults with CVS become disabled 2
- Younger children cannot verbalize prodromal symptoms: look for behavioral changes, restlessness, or anxiety 1
- Do not assume hot water bathing equals CHS: nearly half of CVS patients without cannabis use exhibit this behavior 2, 3