Cyclic vomiting syndrome in children

Supportive therapy

Once diagnosis is made, family physicians can recommend supportive measures to reduce suffering among children. Sleep is often the only relief and a quiet, dark room is a nonstimulating environment that can help patients sleep. Frequently, a combination of antiemetics or sedatives, such as diphenhydramine, chlorpromazine, ondansetron, and lorazepam, provides supportive measures during a vomiting episode.

Early recognition of dehydration, at times based on the anticipated course of the episode in view of prior incidents, should prompt use of intravenous fluids and monitoring of electrolyte imbalance and possible ketosis.

Families need support to deal with the frustration of coping with this unpredictable, disruptive, and unexplained illness that is not only typically misdiagnosed but for which there are few definitive answers.⁸

Prophylactic treatment

While CVS episodes are typically self-limited, the long nature of episodes in some children and increased frequency of episodes in others warrant the use of prophylactic treatment. Current options are described only for the adult population and include tricyclic antidepressants such as amitriptyline, as well as topiramate as second-line therapy.¹⁰ Adverse events associated with amitriptyline therapy, such as fatigue, weight gain, QT prolongation, and hallucinations, limit topiramate’s use.¹⁰

In a large cohort of adults from Milwaukee, Wis, 65% of patients (88 out of 136) responded to topiramate in an intent-to-treat analysis, with a decreased annual number of CVS episodes (18.1 vs 6.2; P < .0001), CVS-related emergency department visits (4.3 vs 1.6; P = .0029), and CVS-related hospitalizations (2.0 vs 1.0; P = .035).¹¹ Response to treatment was associated with higher dose, prophylactic use for a year or more, and use of topiramate as monotherapy. However, 55% experienced side effects, resulting in a third of patients discontinuing treatment.¹¹

Pediatric data are very limited. In 2011, Boles reported results of implementing a “loose protocol” in which a few children avoided fasting, and received coenzyme Q10 and l-carnitine with the addition of amitriptyline (for those older than 5 years) in refractory cases of CVS.¹² In a retrospective study from Turkey, the efficacy of topiramate compared with propranolol for CVS episode prophylaxis was examined in 16 treatment-naïve children. The study reported that topiramate led to a significant decrease in the number of CVS episodes per year (P = .001).¹³ Most (81%) children in the topiramate group reported freedom from attacks and 13% had a decrease of more than 50% in attacks per year. Minor adverse effects (drowsiness, nervousness, and dizziness) were observed in a few patients.¹³

In the recent Italian study of 57 children, investigators found no differences in age of onset, gender, duration of follow-up, severity, medication, family history, or trigger factors between children who undertook resolution of vomiting and those who did not, or between children who suffered from migraine and those who did not at follow-up.⁷ When discharged from the hospital, 47 of the children (82%) were started on long-term prophylactic treatment with flunarizine, cyproheptadine, topiramate, or proton pump inhibitors.

Early recognition

Family physicians, with early recognition, evaluation, prompt management during episodes, and referral to further care when intravenous rehydration is needed, can help improve outcomes among children. Understanding the triggers, neuroendocrine mechanism, relationship to migraine, and ways to abort vomiting early in the course of illness will help children cope with CVS.