Brief
in
Cyclic Vomiting Syndrome
Michael S. Foreman, MD,* Tammy Camp, MD*
*Texas Tech University Health Sciences Center, Lubbock, TX
AUTHOR DISCLOSURE Drs Foreman and
Camp have disclosed no financial
relationships relevant to this article. This
commentary does not contain a discussion
of an unapproved/investigative use of a
commercial product/device.
Treatment of Cyclic Vomiting Syndrome
with Co-enzyme Q10 and Amitriptyline, a
Retrospective Study. Boles RG, Lovett-Barr
MR, Preston A, Li BU, Adams K. BMC Neurol.
2010;10:10
The Management of Cyclic Vomiting
Syndrome: A Systematic Review. Lee LY,
Abbott L, Mahlangu B, Moodie SJ, Anderson S.
Eur J Gastroenterol Hepatol. 2012;24(9):
1001–1006
North American Society for Pediatric
Gastroenterology, Hepatology, and
Nutrition Consensus Statement on the
Diagnosis and Management of Cyclic
Vomiting Syndrome. Li BU, Lefevre F,
Chelimsky GG, et al. J Pediatr Gastroenterol
Nutr. 2008;47(3):379–393
Cyclic Vomiting Syndrome: What a
Gastroenterologist Needs to Know. Pareek
N, Fleisher DR, Abell T. Am J Gastroenterol.
2007;102(12):2832–2840
100 Pediatrics in Review
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Cyclic vomiting syndrome (CVS) is characterized by recurring, intense episodes
of nausea and vomiting lasting hours to days, separated by weeks to months of
symptom-free, normal health. The prevalence of CVS is approximately 1% to 2%,
and it affects multiple ethnicities and ages, most frequently white school-aged
children. From the onset of symptoms, diagnosis can be delayed by an average of
1 to 4 years. Episodes result in approximately a month’s worth of missed school
days annually and an estimated $17,000 in yearly health-care costs, multiple
emergency department visits, and repetitive testing.
Cyclic vomiting syndrome and migraines seem to be linked in both patho-
physiology and treatment, and familial history of migraine is often noted. Several
etiologic hypotheses exist, but none are proved. The potential causative fac-
tors include mitochondrial DNA polymorphisms/variants, excessive secretion
of adrenocorticotropin-releasing factor, dysmotility of the gastrointestinal system,
and autonomic (particularly sympathetic) nervous system dysregulation.
With approximately 90% specificity, the North American Society for Pediatric
Gastroenterology, Hepatology, and Nutrition has published a consensus state-
ment that includes the criteria to diagnose CVS. All of the following criteria must
be met: at least 5 attacks in any interval, or a minimum of 3 attacks during a
6-month period; episodic attacks of intense nausea and vomiting lasting 1 hour to
10 days occurring at least 1 week apart; stereotypical patterns and symptoms in
individual patients; vomiting during attacks occurring at least 4 times per hour
for at least 1 hour; a return to baseline health between episodes; and vomiting
cannot be attributable to any other disorder. Although not included in the
criteria, 75% of patients have been noted to have episodes early in the morning,
and symptoms are often triggered by excitement, illness, or stress and lead to
severe dehydration and lethargy.
Other acute, urgent disorders causing vomiting must be ruled out and can
usually be excluded by history, physical examination, and basic laboratory studies,
including a complete blood cell count, complete metabolic panel, urinalysis, and
upper gastrointestinal series with small-bowel follow-through. A pregnancy test
may be applicable. Blood samples should be collected before administration of
intravenous fluids. Further investigations depend on specific history and phys-
ical examination findings, with particular attention given to red flag symptoms.
In patients with severe abdominal pain, tenderness, or bilious vomiting, pro-
viders must consider in the differential diagnosis an acute abdomen, biliary disease,
hepatitis, pancreatitis, hydronephrosis, or ureteropelvic junction obstruction. In
these cases, abdominal ultrasonography or computed tomographic scanning and
appropriate laboratory tests (aspartate aminotransferase, alanine aminotransfer-
ase, gamma-glutamyltransferase amylase, lipase) may be justified. Hematemesis
or chronicity of unremitting symptoms may warrant upper endoscopy to evaluate
for ulcers, bleeds, celiac disease, or inflammatory bowel disease. In addition, a
urea breath test or biopsy to rule out Helicobacter pylori
infection may be considered. Other suspicious symptoms
deserving further evaluation include attacks with fasting,
illness, or high-protein meals. Measurement of levels of
serum glucose, lactate, ammonia, amino acids, plasma
carnitine, acylcarnitine, urine ketones, and organic acids
in this scenario may reveal a metabolic disorder. In patients
with progressive neurologic symptoms or focal physical
examination findings, magnetic resonance imaging of the
brain should be performed. Although rare, acute intermit-
tent porphyria also may be considered in the differential
diagnosis in adolescents with concomitant anxiety, depres-
sion, or hallucinations and recurrent abdominal pain with
vomiting. Urinary d-aminolevulinic acid and porphobilinogen
levels would aid in such diagnosis.
Divided into well and episodic phases, with episodic
phases subdivided into prodromal, vomiting, and recovery
phases, optimal treatment depends on the phase of illness.
Some experts support the use of oral nonsteroidal anti-
inflammatory drugs during the prodromal phase before
vomiting begins. During the episodic vomiting phase, staples
of treatment are supportive and abortive. Treatment includes
a quiet, dark, less-stimulating environment; age-appropriate
inhaled sumatriptan as abortive therapy; intravenous
dextrose-containing fluids; and antiemetics (ondansetron),
with the addition of sedatives such as diphenhydramine or
lorazepam and analgesics such as ketorolac as needed. If
these management strategies do not provide some relief to
the patient, the clinician should reassess for an underly-
ing surgical etiology. The recovery phase follows the last
emesis and typically lasts a few hours as the patient states
that he or she is hungry and begins to tolerate a slowly
advancing oral diet.
Prophylactic treatment during periods of wellness is an
option for frequent episodes or severe episodes likely to
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hospitalize the patient. Triggers such as excessive excite-
ment, fatigue, stress, or fasting should be avoided. When
medication is warranted, cyproheptadine is the preferred
drug for prophylaxis in patients 5 years and younger, and
amitriptyline for patients older than 5 years. Propranolol,
an effective second-line drug, may be an alternative with a
more acceptable adverse effect profile. Anticonvulsants, used
for migraines, are also effective. Recent studies suggest that
coenzyme Q and/or L-carnitine supplementation may be
beneficial, although only limited case series and cross-
sectional studies exist.
Younger age at onset of symptoms seems to correlate to
longer duration of illness. However, 67% to 75% of CVS
cases eventually resolve. Unfortunately, a large proportion
of patients may develop migraine headaches by their late
teens or adulthood.
COMMENT: Having cared for several hospitalized patients
with the diagnosis of cyclic vomiting, I was struck by the
intensity of the symptoms and the true misery of the pa-
tients during the duration of the emesis. Hence, it is critical
to make the diagnosis, support the patient in both treatment
and prevention, and try to eliminate unnecessary repeti-
tive testing. The heterogeneity of symptoms can lead to the
challenges of diagnosis, but the consensus statement by
the North American Society for Pediatric Gastroenterology,
Hepatology, and Nutrition aids clinicians to make the diag-
nosis in a more timely manner. The critical need for pa-
tient and family support is apparent, and families should be
guided to organizations that can provide resources, such as
the Cyclic Vomiting Syndrome Association and the Gas-
troparesis and Dysmotilities Association, to name a few.
– Janet R. Serwint, MD
Associate Editor, In Brief
Vol. 39 No. 2 FEBRUARY 2018 101
 Cyclic Vomiting Syndrome
Michael S. Foreman and Tammy Camp
Pediatrics in Review 2018;39;100
DOI: 10.1542/pir.2016-0137

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 Cyclic Vomiting Syndrome
Michael S. Foreman and Tammy Camp
Pediatrics in Review 2018;39;100
DOI: 10.1542/pir.2016-0137

The online version of this article, along with updated information and services, is
located on the World Wide Web at:
http://pedsinreview.aappublications.org/content/39/2/100

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