Overview
This national survey examined diagnostic and treatment practices for cyclic vomiting syndrome (CVS) in children across Italian pediatric centers, highlighting significant variability between specialties and suggesting a need for standardized national guidelines.
Epidemiology and Clinical Burden
- CVS is a rare disorder characterized by recurrent episodes of intense vomiting and nausea, impacting children's quality of life and causing frequent hospital admissions.
- Prevalence is estimated between 1.9–2.3%, likely underestimated due to under-diagnosis.
Survey Design and Demographics
- Cross-sectional web-based survey conducted among members of SIGENP and SINP, covering 67 clinics in 51 centers.
- Most clinics cared for fewer than 20 CVS patients, with a higher concentration of respondents from northern Italy.
Diagnostic Approaches
- Rome IV criteria used by 61.2% of clinics, more by gastroenterologists; ICHD-III beta criteria used by 23.9%, more by neurologists.
- Significant differences in diagnostic criteria choice were found between clinic types.
- A broad differential diagnosis was reported, often requiring multidisciplinary evaluation.
Time to Diagnosis and Referral
- Majority of patients referred after 3–5 episodes; one third after more than 5 episodes.
- No significant differences in referral timing by clinic type.
Comorbidities and Family History
- Headache, anxiety, sleep disorders, and irritable bowel syndrome were common comorbidities.
- Family history of migraine reported in 86.6% of clinics, supporting a migraine association with CVS.
Triggers and Screening
- Stress most frequently identified as a trigger, followed by sleep deprivation and infections.
- Nearly all clinics performed baseline investigations to rule out organic causes; some test preferences differed by specialty.
Treatment Practices
- Ondansetron most commonly used during prodromal and emetic phases.
- Supportive care (quiet room, IV glucose) commonly provided during emetic episodes.
- Prophylactic treatments included cyproheptadine, pizotifen, amitriptyline, and mitochondrial supplements.
- Marked preference for certain drugs during emetic phases based on clinic specialty.
Long-Term Outcomes
- Outcomes ranged from progression to migraine, symptom persistence, to complete resolution.
- Neurologists more often reported progression to migraine; gastroenterologists noted prolonged symptom remission phases.
Study Limitations
- Survey design prioritized breadth over depth, possibly affecting data precision.
- Not all Italian centers treating CVS may have participated.
Conclusions and Recommendations
- Significant variation exists in CVS diagnosis and management among Italian clinicians.
- Lack of standardized guidelines results in inconsistent care.
- A national consensus guideline and multicenter studies are needed to improve and standardize practices.
Action Items
- TBD – National pediatric societies: Develop a national consensus guideline for CVS diagnosis and management.
- TBD – Research teams: Conduct multicenter prospective studies to identify CVS phenotypes and treatment responses.