1. Stereotypical episodes of vomiting regarding onset (acute) and duration (less than 1 week)
2. Three or more discrete episodes in the prior year
3. Absence of nausea and vomiting between episodes
Supportive (minor) criteria for the diagnosis would be a personal or family history of migraine headache
Table 9.2
Clinical features of cyclic vomiting in children and adults
Children | Adults | ||
|---|---|---|---|
Female: Male | Slight female preponderance | Similar | |
Mean age of onset | 4.8 years | 35 years | |
Symptoms | Vomiting | 6 times/h at peak, bilious (81 %), bloody (34 %) | Episodic stereotypical attacks |
Systemic | Lethargy (93 %), pallor (91 %), fever (30 %) | ||
GI | Salivation (27 %), nausea (82 %), abdominal pain (81 %), anorexia (81 %), retching (79 %), diarrhea (30 %) | Abdominal pain (71 %), diarrhea (19 %) | |
Neurological | Headache (42 %), photophobia (38 %), phonophobia (38 %), vertigo 26 % | None | |
Temporal pattern | Duration | 24 h | 3 days |
Prodrome, recovery | 1.5 h, 6 h | Nausea, epigastric pain | |
Periodic | 49 % have regular intervals, usually 2–4 weeks | 3 months | |
Circadian | Early morning onset (42 %) | Not applicable | |
Stereotypical | Usually | Usually | |
Precipitating events | Psychological stress (47 %), infection (31 %), exhaustion (24 %), dietary (23 %), menses (22 %)—1, or more triggers identified (76 %) | Menses (57 %) | |
Natural history | 3.6+ years, 28 % progress to migraine | Unknown | |
Complications | Secondary esophagitis | Secondary to recurrent vomiting | |
Family history of migraine | 82 % | 24 % | |
The initial testing approach that we recommend is a baseline blood panel which would include electrolytes, liver and pancreatic enzyme screening, and a urinalysis, and in children, an evaluation for metabolic disorders (which would include lactate, ammonia, and amino acids). Judicious use of imaging, limiting radiation exposure, and EGD should be considered. If there are no red flags and if the attacks follow a relatively predictable temporal pattern with identifiable triggers, then the diagnosis of CVS is established.
Treatment
Once diagnosed, the treatment approach to CVS should address both the emetic and the non-emetic phases of the cycle.
Emetic Phase
Limited evidence is available to guide the management of the acute emetic phase of CVS. The evidence—such as it is—is limited to small case series and anecdotal reports which advocate symptomatic relief, intravenous fluid, and sedation to control symptoms.
Supportive Therapy
Intravenous dextrose is the fluid of choice in CVS. Given the postulated role of mitochondrial dysfunction, it is thought that the administration of dextrose solution intravenously may buffer the energy cycle malfunction in a subset of pediatric patients with CVS. As psychological and physical stimuli can exacerbate the vomiting and nausea during this phase of CVS, benzodiazepine sedation and use of a low stimuli environment such as a single darkened room should be considered.
Antiemetic Therapy
5HT3 antagonists such as ondansetron or granisetron are generally more effective than dopamine antagonists such as prochlorperazine.
Antimigraine Therapy
Antimigraine medications can be used to terminate the acute emetic phase of the cycle. Triptans such as sumatriptan and zolmitriptan can be used at this time. If the acute migraine treatment does not settle the episode within a reasonable period of time (2–3 h), further supportive therapy should be considered.
Non-emetic Phase
In those patients—both pediatric and adult—who have a clear trigger, efforts should be made to remove these precipitants, but triggers are less common in adults. Cannabis must be stopped even if the patient believes its use is helping any nausea (they are often mistaken!). Ask about the role of sleep deprivation, over excitability, ingestion of certain foods such as cheese or chocolate, or menses.
In those patients who have a prior personal or family history of migraine headache, trialing antimigraine medications can be considered. Antimigraine medications are also sometimes considered in those patients with no specific personal or family history of headache. A systematic review of the management of CVS published in 2012 reviewed the management of 1,093 cases in 25 papers and found that TCAs and antimigraine therapies appeared to be helpful (Table 9.3).
Table 9.3
Response of pediatric cyclic vomiting syndrome to medications (uncontrolled studies)
Drug | No of patients | Response (%) |
|---|---|---|
Tricyclic antidepressants | 244 | 67.6 |
Propranolol | 91 | 86.8 |
l-Carnitine and amitriptyline | 30 | 76.7 |
Erythromycin | 20 | 65 |
Coenzyme Q | 18 | 66.7 |
Phenobarbital | 14 | 78.6 |
Valproate | 13 | 100 |
Pizotifen | 8 | 50 |
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