eMedicine Specialties > Pediatrics: General Medicine > Gastroenterology

Cyclic Vomiting Syndrome: Differential Diagnoses & Workup

Author: Thangam Venkatesan, MBBS, Assistant Professor, Department of Internal Medicine, Division of Gastroenterology and Hepatology, Medical College of Wisconsin
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; Seth Marcus, MD, Fellow, Department of Pediatric Gastroenterology, Hepatology and Nutrition, Children's Memorial Hospital, Northwestern University; Shikha Sundaram, MD, Fellow, Department of Gastroenterology, Hepatology and Nutrition, Children's Memorial Hospital of Chicago and Northwestern University; Abhilasha Pandey, MBBS, Froedtert Hospital, Medical College of Wisconsin
Contributor Information and Disclosures

Updated: Oct 29, 2009

Differential Diagnoses

Appendicitis
Intestinal Malrotation
Cholelithiasis
Mood Disorder: Depression
Crohn Disease
Munchausen Syndrome by Proxy
Crohn Disease: Surgical Perspective
Pheochromocytoma
Diabetic Ketoacidosis
Porphyria, Acute
Eating Disorder: Bulimia
Sinusitis
Gastroesophageal Reflux
Ulcerative Colitis
Gastroesophageal Reflux: Surgical Perspective
Ulcerative Colitis: Surgical Perspective
Helicobacter Pylori Infection
Ureteropelvic Junction Obstruction
Hirschsprung Disease
Volvulus

Other Problems to Be Considered

  • GI disorders
  • Neurologic disorders
    • Abdominal migraine with vomiting
    • Migraine headaches with vomiting
    • Chronic sinusitis
    • Subtentorial neoplasm (cerebellar medulloblastoma, brainstem glioma)
    • Chiari malformation
    • Familial dysautonomia (Riley-Day syndrome)
  • Renal disorders
    • Acute hydronephrosis secondary to uteropelvic junction obstruction
    • Nephrolithiasis
  • Metabolic disorders
    • Disorders of fatty acid oxidation
    • Urea cycle defects
    • Mitochondriopathy
    • Acute intermittent porphyria
  • Endocrine disorders
    • Addison disease
    • Diabetes mellitus with ketoacidosis
    • Pheochromocytoma
    • Hyperemesis gravidum
  • Psychological disorders
  • Other - Pregnancy
  • Cannabinoid hyperemesis
    • Allen et al reported 19 cyclic vomiting syndrome cases associated with chronic cannibas use. In this study, 10 patients were not included in follow-up; most of the remaining 9 who abstained from marijuana had symptom relief.39 Other cases of cannabinoid hyperemesis have been reported, although no long-term follow-up is noted in most cases.40
    • Evidence implicating marijuana use as the actual cause of vomiting is insufficient; no clear temporal relationship between the onset of vomiting and the actual initiation of marijuana use has been established. These patients may have cyclic vomiting syndrome and are using marijuana for its antiemetic properties.
    • Although the role of marijuana in cyclic vomiting syndrome has not been well established, a recent report by McCallum et al shows chronic marijuana use is a risk factor for lack of response to tricyclic antidepressants used in the treatment of this disorder. Thus, complete abstinence from marijuana may be recommended in patients with cyclic vomiting syndrome.41

Workup

Laboratory Studies

  • A heterogeneous group of disorders can mimic cyclic vomiting syndrome (CVS), and these disorders must be excluded with systematic laboratory and radiographic testing. An analysis by Li and colleagues demonstrated 3 main categories to consider in the differential diagnoses: GI disorders, extraintestinal disorders, and idiopathic cyclic vomiting syndrome.42
  • When evaluating for GI diseases, screening blood work should include a CBC count with differential, erythrocyte sedimentation rate (ESR), and levels of hepatic transaminases, pancreatic amylase, and lipase.
  • Nonanatomic renal disease can be detected using serum BUN and creatinine tests, urinalysis, and urine calcium-to-creatinine ratio.43
  • Screening for multiple metabolic and endocrine disorders can be accomplished by assessing electrolytes, pH, glucose, lactic acid, ammonia, amino acids, ACTH, and antidiuretic hormone (ADH).
  • Urinary ketones, organic acids, ester-to-free carnitine ratio, porphobilinogen, and aminolevulinic acid may also guide diagnosis in the correct direction.43 These metabolic and endocrine tests must be obtained during the episode to detect intermittent disorders (eg, disorder of fatty acid oxidation) or heterozygote disorders (eg, partial ornithine transcarbamylase deficiency).
  • Blood and urine tests must be performed before starting intravenous fluids that contain glucose or other medical treatments.
  • In a postmenarchal girl, the physician must consider a beta human chorionic gonadotropin (bhCG) test for pregnancy.43

Imaging Studies

  • When evaluating for GI diseases, an upper GI (UGI) with small-bowel followthrough (SBFT) radiography, esophagogastroduodenoscopy (EGD), abdominal ultrasonography, or CT and gastric-emptying scanning may provide definitive information.43
  • When evaluating for neurologic or otolaryngologic diseases, a sinus CT scan or brain MRI should be considered.43 CT scans may not adequately visualize the subtentorial region.
  • Obstructive renal disease can be revealed with renal ultrasonography or CT imaging.43
  • With a broad array of possible diagnoses and possible diagnostic approaches, an extensive evaluation may appear cumbersome. Olson and Li reported that UGI radiography followed by empiric antimigraine therapy for 2 months is the most cost-effective approach ($1600) for the initial treatment of recurrent episodic vomiting in children ($3020 for extensive diagnostic evaluation, and $1830 for empiric treatment alone).44 Until prospective trials are conducted, the authors' current approach generally includes initial blood and urine screens, including metabolic screening and UGI at initial presentation.
  • The presence of specific symptoms such as hematemesis, bilious vomiting, persistent headache, flank pain, acidosis, or uncharacteristically severe or atypical vomiting episodes should raise the clinician's index of suspicion of an underlying disorder and should warrant a prompt and more extensive or repeat evaluation.44 The 4 tests with the highest yield include endoscopy, sinus radiography or CT imaging, small-bowel radiography, and head CT imaging or MRI.43

Other Tests

  • A psychological evaluation may reveal ongoing panic, anxiety, and eating disorders, and stress management may attenuate the stress triggers.43
  • In summary, because no biochemical markers have been identified, the recent NASPHAGN guidelines suggest that physicians must initially look for alarming symptoms and tailor their work up accordingly. Suspicious symptoms include the following:
    • Bilious vomiting, abdominal tenderness, and/or severe abdominal pain
    • Attacks precipitated by intercurrent illness, fasting, and/or high protein meal
    • Abnormal neurologic examination findings, including severe alteration of mental status, abnormal eye movements, papilledema, motor asymmetry, and/or gait abnormality (ataxia)
    • Progressively worsening episodes or conversion to a continuous or chronic pattern
  • Depending on the presenting symptoms and signs other than vomiting, different diagnostic approaches are recommended. In addition, certain subgroups of patients are thought to be at high risk for metabolic disorders; if the following conditions are met, early referral to a metabolic specialist or neurologist should be considered:
    • Presentation younger than age 2 years (with cyclic vomiting or comorbidities below)
    • Vomiting episodes associated with intercurrent illnesses, prior fasting, and/or increased protein intake
    • Any neurological finding, including ataxia, dystonia, or another gait disturbance; mental retardation; or seizure disorder or acute encephalopathy (including true lethargy, severe irritability, confusion, psychosis, or rapidly changing/unstable mental status)
    • Laboratory metabolic findings, including hypoglycemia, substantial anion gap metabolic acidosis, respiratory alkalosis, or hyperammonemia

More on Cyclic Vomiting Syndrome

Overview: Cyclic Vomiting Syndrome
Differential Diagnoses & Workup: Cyclic Vomiting Syndrome
Treatment & Medication: Cyclic Vomiting Syndrome
Follow-up: Cyclic Vomiting Syndrome
Multimedia: Cyclic Vomiting Syndrome
References
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Further Reading

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Keywords

cyclic vomiting syndrome, CVS, vomit, emesis, migraine, syndrome of mitochondrial myopathy, encephalopathy, lactic acidosis, strokelike episodes, MELAS syndrome, abdominal migraine, rotavirus gastroenteritis, gastroesophageal reflux

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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.

Medical Editor

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.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

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.

CME Editor

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: TAP Pharmaceuticals Honoraria Speaking and teaching; Curemark, LLC Consulting fee Board membership; Centocor, 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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