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Cyclic vomiting syndrome within the pediatric population is a is a rare chronic functional gastrointestinal disorder characterized by reoccurring episodes of nausea, vomiting, and/or retching, separated by symptom free periods, resulting in frequent hospitalizations, associations with multiple comorbidities, and a reduced quality of life (Li, 2018; Bhandari, Jha, Thakur, Kar, Gerdes, and Venkatesan, 2018). Its pathophysiology is uncertain but it is thought to be associated with other episodic conditions (e.g.: migraine headaches), mitochondraiopathy, and autonomic and hypothalamic-pituitary-adrenal axis hyperreactivity (Li, 2018; Garzon-Maaks, Barber-Star, Gaylord, Driessnack, & Duderstadt, 2020). Physical exam findings are generally normal but may reveal associated signs and symptoms of dehydration (e.g.: pallor, tachycardia) or other symptoms associated with other complications (e.g.: hematemesis) (Garzon-Makks et al., 2020).

As many different conditions may cause nausea, vomiting and abdominal pain similar to cyclic vomiting disorder, there are several differentials for this condition such as hydronephrosis, cholelithiasis, peptic disorders, peptic disorders, pancreatitis or appendicitis, viral gastroenteritis, Addison disease, and brain tumors (Garzon-Makks et al., 2020; National Organization of Rare Disorders). There is no definitive treatment proven to be able to manage cyclic vomiting syndrome (Garzon-Makks et al., 2020). Thus, patients are treated empirically using evidenced-based guidelines provided by professional organizations and pediatric experts such as the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition and experts opinions from the Consensus Statement on cyclic vomiting syndrome.

Current guidelines recommendations include prophylactic treatment with things such as lifestyle changes modifications, avoidance of triggers, stress reduction techniques, adequate sleep, prophylactic medications such as anti-migraine (amitriptyline and propranolol for children older than five and cyproheptadine for children younger than five), anti-convulsant medications (e.g.: Phenobarbital), tricyclic antidepressants (e.g.: nortriptyline, doxepin), and supplements (coenzyme Q10 and L-carnitine) (Li, 2018; Garzon-Makks et al., 2020). Abortive medications include antimigraine medications (e.g.: sumatriptan, amitriptyline) or a 5-hydroxytryptamine-1 receptor 1B/1D agonist, given intranasally or subcutaneously (Bhandari et al, 2018).

When prophylactic and abortive measures fail, acute and supportive pharmacologic treatment recommendations include things such as antiemetics (e.g.: Zofran), sedatives (lorazepam), PPI or H2RAs (for epigastric pain), analgesics or ACE inhibitors (for hypertension), IVF and electrolyte replacement (Garzon Makks et al., 2020; National Organization for Rare Disorders. (2020). If the child fails to respond to these treatment methods or has red flag symptoms (e.g.: bilious vomiting, severe abdominal pain, hematemesis, abnormal neurologic examination, etc.) the child should be referred (Garzon Makks et al., 2020).

References:

Bhandari, S., Jha, P., Thakur, A., Kar, A., Gerdes, H., & Venkatesan, T. (2018). Cyclic vomiting syndrome: epidemiology, diagnosis, and treatment. Official Journal of the Clinical Autonomic Research Society, 28(2), 203–209. https://doi.org/10.1007/s10286-018-0506-2

Garzon-Maaks, D., Barber-Star, N., Gaylord, N., Driessnack, M., & Duderstadt, K. (2020). Burns’ pediatric primary care (7th ed.). St Louis, Missouri: Elsevier.

Li, B. (2018). Managing cyclic vomiting syndrome in children: beyond the guidelines. European Journal of Pediatrics, 177(10), 1435–1442. https://doi.org/10.1007/s00431-018-3218-7

National Organization for Rare Disorders. (2020). Cyclic vomiting syndrome. Retrieved from https://rarediseases.org/rare-diseases/cyclic-vomiting-syndrome/

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