Cyclic Vomiting Syndrome in Children

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-schoo...

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Main Authors: Umberto Raucci, Osvaldo Borrelli, Giovanni Di Nardo, Renato Tambucci, Piero Pavone, Silvia Salvatore, Maria Elisabetta Baldassarre, Duccio Maria Cordelli, Raffaele Falsaperla, Enrico Felici, Michela Ada Noris Ferilli, Salvatore Grosso, Saverio Mallardo, Diego Martinelli, Paolo Quitadamo, Licia Pensabene, Claudio Romano, Salvatore Savasta, Alberto Spalice, Caterina Strisciuglio, Agnese Suppiej, Massimiliano Valeriani, Letizia Zenzeri, Alberto Verrotti, Annamaria Staiano, Maria Pia Villa, Martino Ruggieri, Pasquale Striano, Pasquale Parisi
Format: Article
Language:English
Published: Frontiers Media S.A. 2020-11-01
Series:Frontiers in Neurology
Subjects:
Online Access:https://www.frontiersin.org/articles/10.3389/fneur.2020.583425/full
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author Umberto Raucci
Osvaldo Borrelli
Giovanni Di Nardo
Renato Tambucci
Piero Pavone
Silvia Salvatore
Maria Elisabetta Baldassarre
Duccio Maria Cordelli
Raffaele Falsaperla
Enrico Felici
Michela Ada Noris Ferilli
Salvatore Grosso
Saverio Mallardo
Diego Martinelli
Paolo Quitadamo
Licia Pensabene
Claudio Romano
Salvatore Savasta
Alberto Spalice
Caterina Strisciuglio
Agnese Suppiej
Massimiliano Valeriani
Letizia Zenzeri
Alberto Verrotti
Annamaria Staiano
Maria Pia Villa
Martino Ruggieri
Pasquale Striano
Pasquale Striano
Pasquale Parisi
author_facet Umberto Raucci
Osvaldo Borrelli
Giovanni Di Nardo
Renato Tambucci
Piero Pavone
Silvia Salvatore
Maria Elisabetta Baldassarre
Duccio Maria Cordelli
Raffaele Falsaperla
Enrico Felici
Michela Ada Noris Ferilli
Salvatore Grosso
Saverio Mallardo
Diego Martinelli
Paolo Quitadamo
Licia Pensabene
Claudio Romano
Salvatore Savasta
Alberto Spalice
Caterina Strisciuglio
Agnese Suppiej
Massimiliano Valeriani
Letizia Zenzeri
Alberto Verrotti
Annamaria Staiano
Maria Pia Villa
Martino Ruggieri
Pasquale Striano
Pasquale Striano
Pasquale Parisi
author_sort Umberto Raucci
collection DOAJ
description 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.
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spelling doaj.art-b4a369d72a89414b9909c247ebe48f842022-12-21T19:21:43ZengFrontiers Media S.A.Frontiers in Neurology1664-22952020-11-011110.3389/fneur.2020.583425583425Cyclic Vomiting Syndrome in ChildrenUmberto Raucci0Osvaldo Borrelli1Giovanni Di Nardo2Renato Tambucci3Piero Pavone4Silvia Salvatore5Maria Elisabetta Baldassarre6Duccio Maria Cordelli7Raffaele Falsaperla8Enrico Felici9Michela Ada Noris Ferilli10Salvatore Grosso11Saverio Mallardo12Diego Martinelli13Paolo Quitadamo14Licia Pensabene15Claudio Romano16Salvatore Savasta17Alberto Spalice18Caterina Strisciuglio19Agnese Suppiej20Massimiliano Valeriani21Letizia Zenzeri22Alberto Verrotti23Annamaria Staiano24Maria Pia Villa25Martino Ruggieri26Pasquale Striano27Pasquale Striano28Pasquale Parisi29Pediatric Emergency Department, Bambino Gesù Children's Hospital, Institute for Research, Hospitalization and Health Care (IRCCS), Rome, ItalyDivision of Neurogastroenterology and Motility, Department of Pediatric Gastroenterology, University College London (UCL) Institute of Child Health and Great Ormond Street Hospital, London, United KingdomChair of Pediatrics, Department of Neuroscience, Mental Health and Sense Organs (NESMOS), Faculty of Medicine & Psychology, Sant'Andrea Hospital, Sapienza University of Rome, Rome, ItalyDigestive Endoscopy and Surgery Unit, Bambino Gesù Children's Hospital, Institute for Research, Hospitalization and Health Care (IRCCS), Rome, ItalySection of Pediatrics and Child Neuropsychiatry, Department of Clinical and Experimental Medicine, University of Catania, Catania, ItalyPediatric Department, Ospedale “F. Del Ponte,” University of Insubria, Varese, ItalyDepartment of Biomedical Science and Human Oncology Aldo Moro University of Bari, Bari, ItalyChild Neurology Unit, University of Bologna, Bologna, ItalyNeonatal Intensive Care and Pediatric Units, S. Marco Hospital, Vittorio Emanuele Hospital, Catania, Italy0Unit of Pediatrics, The Children Hospital, Azienda Ospedaliera SS Antonio e Biagio e Cesare Arrigo, Alessandria, Italy1Division of Neurology, Bambino Gesù Children's Hospital, Institute for Research, Hospitalization and Health Care (IRCCS), Rome, Italy2Clinical Pediatrics, Department of Molecular Medicine and Development, University of Siena, Siena, Italy3Pediatric Department, Santa Maria Goretti Hospital, Sapienza University of Rome, Latina, Italy4Division of Metabolism, Department of Pediatric Specialties, Bambino Gesù Children's Hospital, Institute for Research, Hospitalization and Health Care (IRCCS), Rome, Italy5Department of Pediatrics, A.O.R.N. Santobono-Pausilipon, Naples, Italy6Pediatric Unit, Department of Medical and Surgical Sciences, University “Magna Graecia” of Catanzaro, Catanzaro, Italy7Pediatric Gastroenterology Unit, Department of Human Pathology in Adulthood and Childhood “G. Barresi”, University of Messina, Messina, Italy8Pediatric Unit “Hospital ASST of Crema,” Crema, Italy9Child Neurology Division, Department of Pediatrics, “Sapienza,” University of Rome, Rome, Italy0Department of Woman, Child, General and Specialistic Surgery, University of Campania “Luigi Vanvitelli,” Naples, Italy1Pediatric Section, Department of Medical Sciences, University of Ferrara, Ferrara, Italy1Division of Neurology, Bambino Gesù Children's Hospital, Institute for Research, Hospitalization and Health Care (IRCCS), Rome, Italy2Emergency Pediatric Department, Santobono-Pausilipon Children's Hospital, Naples, Italy3Department of Pediatrics, University of L'Aquila, L'Aquila, Italy4Section of Pediatrics, Department of Translational Medical Science, “Federico II” University of Naples, Naples, ItalyChair of Pediatrics, Department of Neuroscience, Mental Health and Sense Organs (NESMOS), Faculty of Medicine & Psychology, Sant'Andrea Hospital, Sapienza University of Rome, Rome, Italy5Unit of Rare Diseases of the Nervous System in Childhood, Section of Pediatrics and Child Neuropsychiatry, Department of Clinical and Experimental Medicine, University of Catania, Catania, Italy6Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health, University of Genoa, Genova, Italy7Institute for Research, Hospitalization and Health Care (IRCCS) “G. Gaslini” Institute, Genova, ItalyChair of Pediatrics, Department of Neuroscience, Mental Health and Sense Organs (NESMOS), Faculty of Medicine & Psychology, Sant'Andrea Hospital, Sapienza University of Rome, Rome, ItalyCyclic 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.https://www.frontiersin.org/articles/10.3389/fneur.2020.583425/fullfunctional gastrointestinal disordersmigrainevomitingantiemeticsanticonvulsantscyclic vomiting syndrome
spellingShingle Umberto Raucci
Osvaldo Borrelli
Giovanni Di Nardo
Renato Tambucci
Piero Pavone
Silvia Salvatore
Maria Elisabetta Baldassarre
Duccio Maria Cordelli
Raffaele Falsaperla
Enrico Felici
Michela Ada Noris Ferilli
Salvatore Grosso
Saverio Mallardo
Diego Martinelli
Paolo Quitadamo
Licia Pensabene
Claudio Romano
Salvatore Savasta
Alberto Spalice
Caterina Strisciuglio
Agnese Suppiej
Massimiliano Valeriani
Letizia Zenzeri
Alberto Verrotti
Annamaria Staiano
Maria Pia Villa
Martino Ruggieri
Pasquale Striano
Pasquale Striano
Pasquale Parisi
Cyclic Vomiting Syndrome in Children
Frontiers in Neurology
functional gastrointestinal disorders
migraine
vomiting
antiemetics
anticonvulsants
cyclic vomiting syndrome
title Cyclic Vomiting Syndrome in Children
title_full Cyclic Vomiting Syndrome in Children
title_fullStr Cyclic Vomiting Syndrome in Children
title_full_unstemmed Cyclic Vomiting Syndrome in Children
title_short Cyclic Vomiting Syndrome in Children
title_sort cyclic vomiting syndrome in children
topic functional gastrointestinal disorders
migraine
vomiting
antiemetics
anticonvulsants
cyclic vomiting syndrome
url https://www.frontiersin.org/articles/10.3389/fneur.2020.583425/full
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