Cyclic Vomiting Syndrome Clinical Presentation

  • Author: Thangam Venkatesan, MBBS; Chief Editor: Carmen Cuffari, MD   more...
 
Updated: Apr 23, 2010
 

History

Cyclic vomiting syndrome (CVS) is characterized by recurrent, discrete, stereotypical episodes of rapid-fire vomiting between varying periods of completely normal health. This on-and-off stereotypic pattern of vomiting is nearly pathognomonic of cyclic vomiting syndrome. Although periods of complete normalcy typically occur between episodes, a pattern of coalescence has been described in many adult patients. Over time, these patients may lose the cyclic pattern of symptoms, and 63% of patients develop interepisodic symptoms (often nausea) between episodes, which is termed coalescence.[11]

The Rome III diagnostic criteria for cyclic vomiting syndrome in children includes all of the following:

  • Two or more periods of intense nausea and unremitting vomiting or retching, lasting hours to days
  • Return to usual state of health that lasts weeks to months

The cut off of 2 episodes resulted in a significant proportion of misdiagnosis; thus, the guidelines were modified and the criteria as per the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition (NASPGHAN) are as follows (all criteria must be met):[34]

  • At least 5 episodes, or a minimum of 3 over a 6-month period
  • Episodic attacks of intense nausea and vomiting that lasts 1 hour to 10 days, occurring at least one week apart
  • Stereotypical pattern and symptoms in the individual patient
  • Vomiting during episodes occurs at least 4 times an hour for at least one hour
  • A return to baseline health during episodes
  • Not attributed to another disorder

The Rome III criteria for cyclic vomiting syndrome in adults includes the following:

  • Stereotypical episodes of vomiting regarding onset (acute) and duration (< 1 wk)
  • Three or more discrete episodes in the prior year
  • Absence of nausea and vomiting between episodes
  • No metabolic, GI, or CNS structural or biochemical disorders

Supportive criteria include history of migraine headaches and family history of migraine headaches.

The vomiting in cyclic vomiting syndrome is typically much more severe and intermittent than is observed in gastroesophageal reflux. When children with cyclic vomiting syndrome were compared with children with chronic vomiting, children with cyclic vomiting syndrome had a much higher peak rate of emeses per hour (12.6 vs 1.9) but far fewer episodes per month (1.5 vs 36).[1] A cut-off criterion of at least 4 emeses per hour at peak and fewer than 2 episodes per week was 92% sensitive and 100% specific for the final diagnosis of cyclic vomiting syndrome.[35] With a larger cohort, the median peak rate of emeses is still 6 times per hour.[18] Only Bacillus cereus food poisoning matches this high intensity of emesis.[29] This singularly severe vomiting (so-called cyclic vomiting pattern) typifies patients with cyclic vomiting syndrome and helps point toward a disorder that is localized outside the GI tract.

Persons with the cyclic pattern (high-intensity, low-frequency) tend to have extraintestinal disorders (eg, neurologic, renal, metabolic, endocrine), whereas persons with a chronic vomiting pattern (low-grade, daily) tend to have upper GI injury, such as gastroesophageal reflux and gastritis.[29] The emesis is often projectile (48%) and frequently contains bile (81%), mucous (72%), and blood (34%).[1] Hematemesis is more often due to retrograde herniation of the gastric cardia through the gastroesophageal junction (ie, prolapse gastropathy) than to a classic Mallory-Weiss tear.[36]

The "on-off" stereotypical pattern often begins with a half-hour prodrome of nausea and pallor. Vomiting peaks in the first hour and then begins to decline over the ensuing 4-8 hours, lasting a mean of 24 hours (median, 43 h). Episodes commonly occur in the early morning (2-4 am) or upon awakening (6-8 am). The recovery period from the end of vomiting to the point of being able to eat and play lasts a mere 5 hours. Despite the label cyclic, only one half of patients have a stable periodicity; the rest have sporadic intervals.[29]

Besides vomiting, patients may also experience other GI symptoms. Abdominal pain is present in 80% of patients and may initially be severe enough to mimic acute abdomen and result in a laparotomy.[18] Patients may also have epigastric pain secondary to peptic injury of the esophagus.

Most patients experience retching (79%) and nausea (82%) and describe the nausea to be the most distressing symptom.[1] It is unrelenting, completely unrelieved by vomiting, and disappears only when the child is asleep or the episode is over.

Many of the behavioral symptoms commonly observed in patients with cyclic vomiting syndrome (eg, fetal positioning, social withdrawal, turning off lights and televisions) are attempts to lessen nausea.[29]

Approximately one third of patients also experience fever, diarrhea, or both, complicating differentiation from gastroenteritis; this is likely due to associated autonomic symptoms. Autonomic symptoms are also common, particularly lethargy (93%) and pallor (91%).[1] Lethargy may be profound, and patients may be unable to walk or talk or may appear comatose. Excess salivation (27%) can also be dramatic.[1]

Many patients with cyclic vomiting syndrome have neurologic symptoms, which supports the relationship between migraines and cyclic vomiting syndrome. Symptoms include headache (42%), photophobia (38%), phonophobia (30%), and vertigo (26%). Because fewer than one half of patients with cyclic vomiting syndrome have classic migraine symptoms, this precludes using the symptoms as diagnostic criteria for a migraine variant.[1]

Approximately 68% of families are able to identify events that appear to precipitate a patient's episode.[9, 37, 18, 21] The most common precipitating event is infection (41%), particularly chronic sinusitis.[29] Psychological stresses (34%) and food products, including chocolate, cheese, and monosodium glutamate (MSG), rank close behind chronic sinusitis.[29] Positive excitement, such as birthdays, holidays, vacations, and school outings, appear to trigger more episodes than do negative stresses. Others recognize physical exhaustion or lack of sleep (18%), atopic events (13%), motion sickness (9%), and menses (13%) as triggers.[29] Many patients experience remission in the summer, when the number of infections and school stressors decline.[38]

The terms cyclic vomiting syndrome and abdominal migraine have often been used interchangeably because of overlap in clinical criteria. Indeed, the key criteria, except vomiting, in abdominal migraines are identical to those in cyclic vomiting syndrome and include recurrent, stereotypical, and severe episodes of abdominal pain; punctuating well periods; autonomic symptoms (eg, pallor, lethargy); and a family history of migraine headaches. Because 80% of children with cyclic vomiting syndrome have abdominal pain, and 50% of those with abdominal pain vomit, many children can be diagnosed with either cyclic vomiting syndrome or abdominal migraine. When both symptoms occur, the authors use the predominant or most consistent symptom as the primary label.

The following 3 additional criteria help strengthen the diagnosis of cyclic vomiting syndrome:[29]

  • Negative screening test results to exclude common GI, hepatobiliary, renal, metabolic, and endocrine disorders
  • Subsequent development of migraine headaches
  • A positive response to antimigraine medications

Table 2. Clinical Features in Adults and Children with Cyclic Vomiting Syndrome[39] (Open Table in a new window)

Feature ChildrenAdults
Age of Onset4.8 y (Earliest, 6 d)35 y (Latest, 73 y)
Delay in Diagnosis2.6 y8 y
Female-to-Male Ratio57:4317:24
FrequencyEvery 2-4 wkEvery 3 mo
Duration (Mean)1-2 d (range, 1-10 d)6 d (range, 1-21 d)
Periodicity49%Not reported
Early Morning Onset42%50%
Stereotypical Episodes99%85%
Prodrome72%, 1.5 h93%
SymptomsNausea, anorexia, pallorNausea, epigastric pain
Recovery to Oral Feeding6 h24 h
Relieving FactorsDeep sleepHot bath/shower (56%)
Precipitating FactorsStress (47%), infection (31%)Menses (57%), anxiety
Comorbid conditionsAnxietyNot reported
Interepisodic nausea< 6%63%
Coalescence of EpisodesFew50%
Vomiting6/hr at peak, bile (81%)8.5/hr
Systemic SymptomsPallor, salivation, listlessnessIntense thirst (33%)
GI SymptomsAnorexia, nausea, diarrhea, abdominal painAbdominal pain, diarrhea
Neurologic SymptomsHeadache, photophobia, phonophobia, abdominal painIrritable, confused
Natural history≥ 28% progress to migraineNot reported
Family history82%57%
ComplicationsDehydration, esophagitisDehydration, esophagitis, laparotomy (18%)
Morbidity14-25 d of missed school/year32% completely disabled
Proceed to Differential Diagnoses
 
 
Contributor Information and Disclosures
Author

Thangam Venkatesan, MBBS  Assistant Professor, Department of Internal Medicine, Division of Gastroenterology and Hepatology, Medical College of Wisconsin

Thangam Venkatesan, MBBS is a member of the following medical societies: American Gastroenterological Association and Indian Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

B UK Li, MD  Professor of Pediatrics, Division of Gastroenterology, Hepatology and Nutrition, Director, Pediatric Fellowships and Gastroenterology Fellowship, Medical Director, Functional Gastrointestinal Disorders and Cyclic Vomiting Program, Medical College of Wisconsin; Attending Gastroenterologist, Children's Hospital of Wisconsin

B UK Li, MD is a member of the following medical societies: Alpha Omega Alpha, American Gastroenterological Association, and North American Society for Pediatric Gastroenterology and Nutrition

Disclosure: Nothing to disclose.

Seth Marcus, MD  Fellow, Department of Pediatric Gastroenterology, Hepatology and Nutrition, Children's Memorial Hospital, Northwestern University

Seth Marcus, MD is a member of the following medical societies: American Academy of Pediatrics and North American Society for Pediatric Gastroenterology and Nutrition

Disclosure: Nothing to disclose.

Shikha Sundaram, MD  Fellow, Department of Gastroenterology, Hepatology and Nutrition, Children's Memorial Hospital of Chicago and Northwestern University

Shikha Sundaram, MD is a member of the following medical societies: American Academy of Pediatrics and American Medical Association

Disclosure: Nothing to disclose.

Abhilasha Pandey, MBBS  Froedtert Hospital, Medical College of Wisconsin

Disclosure: Nothing to disclose.

Specialty Editor Board

Jayant Deodhar, MD  Associate Professor in Pediatrics, BJ Medical College, India; Honorary Consultant, Departments of Pediatrics and Neonatology, King Edward Memorial Hospital, India

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Pharmacy Editor, eMedicine

Disclosure: Nothing to disclose.

David A Piccoli, MD  Chief of Pediatric Gastroenterology, Hepatology and Nutrition, The Children's Hospital of Philadelphia; Professor, University of Pennsylvania School of Medicine

David A Piccoli, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American Gastroenterological Association, and North American Society for Pediatric Gastroenterology and Nutrition

Disclosure: Nothing to disclose.

Steven M Schwarz, MD, FAAP, FACN, AGAF  Professor of Pediatrics, Children's Hospital at Downstate, SUNY-Downstate Medical Center

Steven M Schwarz, MD, FAAP, FACN, AGAF is a member of the following medical societies: American Academy of Pediatrics, American College of Nutrition, American College of Physician Executives, American Gastroenterological Association, American Pediatric Society, Gastroenterology Research Group, New York Academy of Medicine, North American Society for Pediatric Gastroenterology and Nutrition, and Society for Pediatric Research

Disclosure: Curemark, LLC Consulting fee Board membership; Centocor, Inc. Grant/research funds Independent contractor; Johnson & Johnson, Inc. Grant/research funds Independent contractor

Chief Editor

Carmen Cuffari, MD  Associate Professor, Department of Pediatrics, Division of Gastroenterology/Nutrition, Johns Hopkins University School of Medicine

Carmen Cuffari, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, and Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

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Cyclic versus chronic temporal patterns of recurrent vomiting. The number of emeses is plotted over a 2-month period. The chronic pattern, represented by a thin dashed line, has low grade on nearly a daily basis (eg, gastroesophageal reflux). The cyclic pattern, represented by a heavy solid line, involves high-intensity episodes intermittently once every several weeks (eg, cyclic vomiting syndrome).
Table 1. Characteristics of Emergency Department Visits in Patients With Cyclic Vomiting Syndrome
CharacteristicAdults



(n = 104)



Children



(n = 147)



Number of ED visits per patient with cyclic vomiting syndrome (Median)15 (range, 1-200)10 (range, 1-175)
Number of ED visits prior to a diagnosis of cyclic vomiting syndrome (Median)7 (Range, 1-150)5 (Range, 0-65)
Diagnosis not made in the ED89 (93%)119 (93%)
Diagnosis not recognized in the ED in patients with an established diagnosis of cyclic vomiting syndrome84 (88%)97 (80%)
Number of different ED visited (Mean ± standard deviation)4.69 ± 4.722.6 ± 2.42
Table 2. Clinical Features in Adults and Children with Cyclic Vomiting Syndrome[39]
Feature ChildrenAdults
Age of Onset4.8 y (Earliest, 6 d)35 y (Latest, 73 y)
Delay in Diagnosis2.6 y8 y
Female-to-Male Ratio57:4317:24
FrequencyEvery 2-4 wkEvery 3 mo
Duration (Mean)1-2 d (range, 1-10 d)6 d (range, 1-21 d)
Periodicity49%Not reported
Early Morning Onset42%50%
Stereotypical Episodes99%85%
Prodrome72%, 1.5 h93%
SymptomsNausea, anorexia, pallorNausea, epigastric pain
Recovery to Oral Feeding6 h24 h
Relieving FactorsDeep sleepHot bath/shower (56%)
Precipitating FactorsStress (47%), infection (31%)Menses (57%), anxiety
Comorbid conditionsAnxietyNot reported
Interepisodic nausea< 6%63%
Coalescence of EpisodesFew50%
Vomiting6/hr at peak, bile (81%)8.5/hr
Systemic SymptomsPallor, salivation, listlessnessIntense thirst (33%)
GI SymptomsAnorexia, nausea, diarrhea, abdominal painAbdominal pain, diarrhea
Neurologic SymptomsHeadache, photophobia, phonophobia, abdominal painIrritable, confused
Natural history≥ 28% progress to migraineNot reported
Family history82%57%
ComplicationsDehydration, esophagitisDehydration, esophagitis, laparotomy (18%)
Morbidity14-25 d of missed school/year32% completely disabled
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