Cyclical vomiting syndrome (CVS) is a migraine syndrome that includes predictable, recurring episodes of vomiting and intense nausea with periods of no symptoms. During attacks, vomiting typically occurs at least 4 times per hour but may be as frequent as 10 times an hour. Pallor and lethargy are associated with this syndrome and can last from one hour to ten days, but usually are one to two days in duration. These symptoms begin early in the morning and the episodes are at least one week apart but tend to be roughly 2-4 weeks apart as predictable intervals. [1, 2]
The onset of CVS is usually about age 5, but on average the diagnosis is often delayed by 2-3 years. [3-5] CVS is the second most common cause of recurrent vomiting in children, while gastroesophageal reflux is the first. Most children will outgrow their symptoms by age 10 [1] in up to 82% of patients. A family history of migraine is reported. [3, 4-6] A significant proportion of patients with CVS will continue to have symptoms through adolescence and into adulthood. It’s important to note that CVS is often a reliable predictor of migraine in adulthood, especially when the symptoms first occurred at an age of less than 6 years. [2] Episodes can be triggered by both significant physical and psychological stress. [3]
Diagnosis
The diagnosis of CVS requires the presence of a clinical history associated with the symptoms of this migraine syndrome. Additionally, for a proper diagnosis to be made, any gastrointestinal and neurological conditions which may have similar symptoms must be excluded. A patient with a history of severe abdominal pain and/or severe vomiting needs to be evaluated for the possibility of: intermittent bowel obstruction from malrotation with volvulus, acute cholecystitis, hepatitis, pancreatitis, abdominal adhesions and ureteropelvic junction obstructions. [3]
There are other potential causes of vomiting not associated with CVS which need to be ruled out before a diagnosis is made. These include vomiting due to fasting, acute illness, or a metabolic disorder. Other potential causes may include respiratory alkalosis, mitochondrial disorders, and increased intracranial pressure.
Treatment and Prevention
There are currently a number of acute treatment and preventative options for cyclical vomiting syndrome. Acute attacks are treated with aggressive hydration, sedation and anti-emetic agents. The triptans in the subcutaneous or nasal formulations may prove effective, especially in patients with a family history of migraine. [1,3] There is currently no specific triptan approved for the treatment of CVS by the FDA for this age group. The medications cyproheptadine or propranolol have been recommended as preventative treatments for CVS for children age 5 and under. The drugs amitriptyline or propranolol have been recommended as first-line agents for older children. Other preventative treatments that should be considered for children older than 5 years include cyproheptadine, topiramate, and divalproex sodium. [1,3]
To best manage the symptoms of cyclical vomiting syndrome, it is important for both the patient and family to become familiar with the syndrome as well as its methods for treatment and prevention.
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References:
- Lewis DW, Bigal ME, Winner P. Migraine and the Childhood Periodic Syndromes. In: Winner P, Lewis DW, Rothner AD, editors. Headache in Children and Adolescence, 2 ed. Ontario, Canada: B.C. Decker, 2008: 37-55.
- Lin YP, Ni YH, Weng WC, Lee WT. Cyclic Vomiting Syndrome and Migraine in Children. J Formos Med Assoc. 2011 Jun:110(6):382-7.
- Cuvellier JC, Lepine A. Childhood Periodic Syndromes. Pediatr Neurol 2010;42:1-11
- Li BU, Misiewicz L. Cyclic vomiting syndrome: A brain-gut disorder. Gastroenterol Clin North Am 2003;32:997-1019.
- Prakash C, Staiano A, Rothbaum RJ, Clouse RE. Similarities in cyclic vomiting syndrome across age groups. Gastroenterology 2001;96:684-8.
- Abell TL, Adams KA, Boles RG, et al. Cyclic vomiting syndrome in adults. Neurogastroenterol Motil 2008;20:269-84.