Cyclical vomiting syndrome

What is cyclical vomiting syndrome?¹

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 emicranie; around 80% of children and 25% of adults who develop CVS also have migraines.⁴

• 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.⁵

• 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.

  • Dolore addominale.

  • Diarrea.

  • Vertigini.

  • Fotofobia.

  • Mal di testa.

• 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:

  • Stress emotivo.

  • Ansia.

  • Infections, especially sinusitis.

  • Certain foods (eg, chocolate, cheese, monosodium glutamate).

  • Having long periods of time without food.

  • Disidratazione.

  • Hot weather.

  • Menstrual periods.

  • Excess exercise.

  • Privazione del sonno.

• Following an episode the patient is symptom-free for several weeks or months.

Nei bambini

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.

Negli adulti

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.

Questo include:

• Gastroesophageal reflux disease.

• Gastritis, duodenitis.

• morbo di Crohn.

• Colite ulcerosa.

• Emicranie.

• Acute intermittent porphyria.

• Morbo di Addison.

• Diabetes mellitus with ketoacidosis.

• Feocromocitoma.

Cannabinoid hyperemesis syndrome is an increasingly recognised similar illness that has been associated with chronic cannabis use.

• 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.²

• 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:⁹

  • 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.¹⁰

• Supportive care during acute episodes - eg, intravenous fluids, analgesia.

• Sostegno familiare.

NB: assessment and treatment of anxiety in children and adolescents with CVS may have a positive impact on health-related quality of life.¹¹

• Full recovery is usual in the majority of cases.

• Dehydration can occur in more severe or prolonged cases.

• Oesophagitis or a Lacerazione di Mallory-Weiss 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.¹²

• Parents and children with CVS have lower health-related quality of life compared to those children with irritable bowel syndrome.¹³

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.