Cyclical vomiting syndrome (CVS) is a condition in which there are repeated episodes of severe nausea, vomiting and physical exhaustion. This condition can be very disruptive and frightening for the individual and also for their families.
- This condition is more common in children, although it can present in adulthood.
- The true incidence of this condition is unknown. It is rare and thought to occur in around 3 out of 100,000 children although its true incidence may be greater than this.
- The average age of first presentation is 5 years.
- Females are affected slightly more than males.
- There is no known cause for this condition.
- It is more common in those who have migraines; around 80% of children and 25% of adults who develop CVS also have migraines.
- There is an overlap between CVS and abdominal migraine. Some research has suggested that this syndrome is a condition related to migraine.
- This condition is more common in those people with a family history of migraine.
- The pathogenesis of this syndrome is likely to be multifactorial, with multiple genetic, autonomic, central and environmental factors playing a role.
- This condition appears to belong to a spectrum of cyclical disorders which may have a genetic link.
- The clinical features of this syndrome resemble those found in association with migraine headaches.
- The main symptoms are severe nausea and sudden vomiting which can last from a few hours to a few days.
- There are four phases of the cycle - prodromal, vomiting, recovery and well phases.
- The prodromal phase can often be marked by intense sweating and nausea. The person may also look very pale. This phase generally lasts from a few minutes to several hours.
- The vomiting phase then follows which can last from hours to days. Nausea, vomiting and retching last for 20 to 30 minutes at a time.
- The recovery phase begins with cessation of vomiting and retching, improving appetite and return of energy.
- The final phase of this illness is a phase of wellness when the patient is symptom-free.
- The following symptoms may also occur:
- Lack of appetite
- Abdominal pain
- The severity of episodes varies between cases.
- The episodes tend to start at the same time of day, last the same length of time and occur with the same symptoms and level of intensity.
- Some people even need hospital admission during episodes.
- The following may trigger an episode:
- Emotional stress.
- Infections, especially sinusitis.
- Certain foods (eg, chocolate, cheese, monosodium glutamate).
- Having long periods of time without food.
- Hot weather.
- Menstrual periods.
- Excess exercise.
- Sleep deprivation.
- Following an episode the patient is symptom-free for several weeks or months.
For a diagnosis to be made in children the following criteria need to be met:
- At least five episodes, or a minimum of three over a six-month period.
- Episodic attacks of intense nausea and vomiting lasting one hour to ten days, occurring at least one week apart.
- Stereotypical pattern and symptoms in the individual patient.
- Vomiting during episodes occurring at least four times an hour for at least one hour.
- A return to baseline health during episodes.
- Symptoms cannot be attributed to another disorder.
For a diagnosis to be made in adults the following criteria need to be met:
- Stereotypical episodes of vomiting regarding onset (acute) and duration (less than one week).
- A minimum of three discrete episodes in the preceding year.
- Absence of nausea and vomiting between episodes.
- No metabolic, gastrointestinal, or central nervous system structural or biochemical disorders.
- Gastroesophageal reflux disease.
- Gastritis, duodenitis.
- Crohn's disease.
- Ulcerative colitis.
- Acute intermittent porphyria.
- Addison's disease.
- Diabetes mellitus with ketoacidosis.
- The diagnosis is usually made clinically.
- CVS should be considered in any child who has repeated episodes of vomiting with periods of wellness between episodes.
- Investigations may be undertaken but these are usually to exclude any underlying conditions. These may include FBC, renal function, LFTs, gastroscopy and abdominal ultrasound.
- A pregnancy test may be considered for some older girls and women.
The following treatment strategies are used:
- Avoidance of triggers - eg, certain foods, stress, sleep deprivation, avoiding dehydration.
- Prophylactic and abortive therapy:
- Preventative medications are usually considered for those patients with more than one episode a month.
- Prophylactic treatments include amitriptyline, propranolol and topiramate.
- Medications used for aborting acute episodes include ondansetron, prochlorperazine and triptans.
- The addition of erythromycin to standard propranolol treatment has been shown to improve the response to treatment in children.
- Supportive care during acute episodes - eg, intravenous fluids, analgesia.
- Family support.
NB: assessment and treatment of anxiety in children and adolescents with CVS may have a positive impact on health-related quality of life.
- Full recovery is usual in the majority of cases.
- Dehydration can occur in more severe or prolonged cases.
- Oesophagitis or a Mallory-Weiss tear can occur due to the excessive vomiting.
- Tooth decay can occur in some cases.
- Most cases resolve in late childhood or early adolescence.
- Around half of children with this condition develop migraines when they are older.
- Parents and children with CVS have lower health-related quality of life compared to those children with irritable bowel syndrome.
Prevention of further episodes
This is not always possible. However, the following should be recommended to patients:
- They should have adequate sleep and avoid exhaustion.
- Any stress or anxiety should be addressed and managed.
- Foods that trigger episodes should be avoided.
- Medications used for migraine prophylaxis can be beneficial for some cases.
Further reading and references
Kaul A, Kaul KK; Cyclic Vomiting Syndrome: A Functional Disorder. Pediatr Gastroenterol Hepatol Nutr. 2015 Dec18(4):224-9. doi: 10.5223/pghn.2015.18.4.224. Epub 2015 Dec 23.
Tepper SJ; Cyclic vomiting syndrome, inborn errors of metabolism, migraine variants, episodic syndromes that may be associated with migraine, and other unusual pediatric headache syndromes. Headache. 2016 Jan56(1):205. doi: 10.1111/head.12751. Epub 2015 Dec 21.
Gelfand AA; Migraine and childhood periodic syndromes in children and adolescents. Curr Opin Neurol. 2013 Jun26(3):262-8. doi: 10.1097/WCO.0b013e32836085c7.
Li BU, Lefevre F, Chelimsky GG, et al; North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition consensus statement on the diagnosis and management of cyclic vomiting syndrome. J Pediatr Gastroenterol Nutr. 2008 Sep47(3):379-93. doi: 10.1097/MPG.0b013e318173ed39.
Hejazi RA, McCallum RW; Cyclic vomiting syndrome: treatment options. Exp Brain Res. 2014 Aug232(8):2549-52. doi: 10.1007/s00221-014-3989-7. Epub 2014 May 28.
Haghighat M, Dehghani SM, Shahramian I, et al; Combination of erythromycin and propranolol for treatment of childhood cyclic vomiting syndrome: a novel regimen. Gastroenterol Hepatol Bed Bench. 2015 Fall8(4):270-7.
Tarbell SE, Li BU; Anxiety Measures Predict Health-Related Quality of Life in Children and Adolescents with Cyclic Vomiting Syndrome. J Pediatr. 2015 Sep167(3):633-8.e1. doi: 10.1016/j.jpeds.2015.05.032. Epub 2015 Jun 18.
Hikita T, Kodama H, Ogita K, et al; Cyclic Vomiting Syndrome in Infants and Children: A Clinical Follow-Up Study. Pediatr Neurol. 2016 Jan 7. pii: S0887-8994(15)30346-5. doi: 10.1016/j.pediatrneurol.2016.01.001.
Tarbell SE, Li BU; Health-related quality of life in children and adolescents with cyclic vomiting syndrome: a comparison with published data on youth with irritable bowel syndrome and organic gastrointestinal disorders. J Pediatr. 2013 Aug163(2):493-7. doi: 10.1016/j.jpeds.2013.01.025. Epub 2013 Feb 26.