Cyclic Vomiting Syndrome in Children.

Umberto Raucci,Pasquale Parisi,Silvia Salvatore,Duccio Maria Cordelli,Alberto Verrotti,Salvatore Savasta,Annamaria Staiano,Enrico Felici,Saverio Mallardo,Paolo Quitadamo,Salvatore Grosso,Massimiliano Valeriani,Piero Pavone,Maria Elisabetta Baldassarre,Martino Ruggieri,Diego Martinelli,Letizia Zenzeri,Pasquale Striano,Licia Pensabene,Alberto Spalice,Raffaele Falsaperla,Maria Pia Villa,Claudio Romano,Giovanni Di Nardo,Agnese Suppiej,Caterina Strisciuglio,Osvaldo Borrelli,Michela Ada Noris Ferilli,Renato Tambucci

doi:10.3389/fneur.2020.583425

Abstract

Cyclic Vomiting Syndrome (CVS) is an underdiagnosed episodic syndrome characterized by frequent hospitalizations, multiple comorbidities, and poor quality of life. It is often misdiagnosed due to the unappreciated pattern of recurrence and lack of confirmatory testing. CVS mainly occurs in pre-school or early school-age, but infants and elderly onset have been also described. The etiopathogenesis is largely unknown, but it is likely to be multifactorial. Recent evidence suggests that aberrant brain-gut pathways, mitochondrial enzymopathies, gastrointestinal motility disorders, calcium channel abnormalities, and hyperactivity of the hypothalamic-pituitary-adrenal axis in response to a triggering environmental stimulus are involved. CVS is characterized by acute, stereotyped and recurrent episodes of intense nausea and incoercible vomiting with predictable periodicity and return to baseline health between episodes. A distinction with other differential diagnoses is a challenge for clinicians. Although extensive and invasive investigations should be avoided, baseline testing toward identifying organic causes is recommended in all children with CVS. The management of CVS requires an individually tailored therapy. Management of acute phase is mainly based on supportive and symptomatic care. Early intervention with abortive agents during the brief prodromal phase can be used to attempt to terminate the attack. During the interictal period, non-pharmacologic measures as lifestyle changes and the use of reassurance and anticipatory guidance seem to be effective as a preventive treatment. The indication for prophylactic pharmacotherapy depends on attack intensity and severity, the impairment of the QoL and if attack treatments are ineffective or cause side effects. When children remain refractory to acute or prophylactic treatment, or the episode differs from previous ones, the clinician should consider the possibility of an underlying disease and further mono- or combination therapy and psychotherapy can be guided by accompanying comorbidities and specific sub-phenotype. This review was developed by a joint task force of the Italian Society of Pediatric Gastroenterology Hepatology and Nutrition (SIGENP) and Italian Society of Pediatric Neurology (SINP) to identify relevant current issues and to propose future research directions on pediatric CVS.

Highlights

EPIDEMIOLOGYCyclic Vomiting Syndrome (CVS) is identified by acute, stereotyped and recurrent episodes of intense nausea with incoercible vomiting, lasting from a few hours to a few days; both children and adults are affected, the clinical presentation and natural history vary somewhat with age [1]
If a child does not respond to one of the discussed regimens or the episode differs from previous ones by greater severity, longer duration, or different symptoms, the clinician should consider the possibility of an underlying disease and the need for new or to repeat diagnostic testing [79]
If a patient does not respond to the first-line therapy the following options should be considered: [1] presence of persisting triggers and comorbid conditions or missed underlying disorders and toxic exposure; [2] inadequate compliance which is common in adolescents and can be documented by testing blood levels for amitriptyline; [3] response to specific medications in CVS is quite variable and often requires serial medication trials and dose escalation before efficacy is achieved; [4] use of combination therapy with 2 drugs; [5] use of complementary therapy such as carnitine, coenzyme Q, estrogens, acupuncture, or psychotherapy

Summary

INTRODUCTION

Cyclic Vomiting Syndrome (CVS) is identified by acute, stereotyped and recurrent episodes of intense nausea with incoercible vomiting, lasting from a few hours to a few days; both children and adults are affected, the clinical presentation and natural history vary somewhat with age [1]. Common physical and psychological stressors might initiate the emetic cascade by stimulating dysfunctional hypothalamic neurons, characterized by high intrinsic energy demands, and activating the autonomic nervous system and HPA axis with CRF release. About 90% of patients experience a prodromal phase [63], that is characterized mainly by signs and symptoms of autonomic dysfunction such as pallor, sweating, lethargy, hot flashes and rarely temperature change and drooling [25, 65] It generally occurs a few hours before the vomit onset, and it might resemble a panic attack; this premonitory phase is similar to that of migraine headache attack [26]. Stereotypical in the individual patient and recurring with predictable periodicity

All of the following

GENETICS FINDINGS IN CVS

CONCLUSION

DATA AVAILABILITY STATEMENT