Faculty Collaboration Database

Cyclic vomiting syndrome: A narrative review and guide to management. Headache 2021 Feb;61(2):231-243

Date

02/24/2021

Pubmed ID

33619730

DOI

10.1111/head.14073

Scopus ID

2-s2.0-85101200568 (requires institutional sign-in at Scopus site)   17 Citations

Abstract

OBJECTIVES/BACKGROUND: Cyclic vomiting syndrome (CVS) is a disabling disorder of gut-brain interaction manifested by stereotypical and severe episodes of nausea and vomiting. Prevalence data indicate that CVS affects 1-2% of children and there has been a recent dramatic rise in diagnosed adults.

METHODS: This narrative review summarizes relevant literature pertaining to pediatric and adult CVS and provides a guide to management based on extensive clinical experience.

RESULTS: More timely diagnosis is facilitated by an expert consensus diagnostic approach and limited testing. Some diagnostic tests of exclusion remain essential. These include an upper gastrointestinal (GI) contrast study to exclude intestinal malrotation and basic laboratory screening. An abdominal ultrasound is recommended to exclude renal hydronephrosis in children and biliary disease in adults. Exclusion of metabolic/genetic conditions is warranted in those with specific warning signs, presentation in infants/toddler age, and in those with refractory disease. In the absence of chronic GI symptoms, referral to a GI specialist for upper endoscopy is generally not necessary in children but recommended in adults. A large subset termed migraine-equivalent CVS display strong clinical and genetic features of migraine. A unifying pathophysiologic core concept involves neuronal hyperexcitability and aberrant central modulation of autonomic signals. This is coupled with multiple susceptibility factors including mitochondrial dysfunction/cellular energy deficits, a hyper-responsive hypothalamic-pituitary-adrenal axis and many comorbidities that increase vulnerability to triggering events. CVS episodes are frequently triggered by stressors and intercurrent illnesses. Lifestyle and non-pharmacological interventions thus play a pivotal role in successful management. Pharmacological therapies are categorized into abortive, supportive/rescue, and prophylactic treatments. The majority respond particularly well to migraine-focused treatment strategies.

CONCLUSION: Despite improved characterization and understanding, CVS remains classified as a functional disorder of brain-gut interaction that is often disjointly managed by generalists and subspecialists. Early recognition, evaluation, and management will facilitate care and improve outcomes. Further research into its natural history with common progression to migraine headaches, neuroendocrine mechanisms, and the pathophysiologic relation to migraine diathesis is much needed.

Author List

Kovacic K, Li BUK

Author

Katja K. Karrento MD Associate Professor in the Pediatrics department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Child
Child, Preschool
Humans
Practice Guidelines as Topic
Vomiting