Cyclical vomiting syndrome: diagnosis and management

Cyclical vomiting syndrome is characterised by episodes of sudden onset nausea and vomiting in an otherwise healthy child. Dr Louise Newson discusses diagnostic criteria and management.

Case scenario

George, a 6 year old boy, came to see me one afternoon with his mother. He had a history of three separate episodes of sudden onset nausea and vomiting which lasted for a couple of hours and then he was well afterwards.

No other family members had similar symptoms so his mother was concerned about this. She said that before these episodes he had become very pale and was not himself. He was otherwise a very healthy boy and was fully up to date with his relevant immunisations. He was happy at school and did not appear to have any emotional issues.

General examination, including abdominal examination, was completely normal. His mother was very anxious and concerned. In view of this rather unusual history, I referred him to one of the local paediatricians.

When he attended the clinic appointment two months later, George had experienced three more of these episodes which were very similar in nature and duration than the previous ones. All these episodes had occurred on days that he had played football either at school or for his local club and he admitted that he had not eaten or drunk much on these days.

On further questioning, the paediatrician learnt that George’s maternal aunt and his older sister suffered with occasional migraines. He had blood tests including full blood count, renal function, liver function tests and blood glucose undertaken which were all normal.

He was subsequently diagnosed with cyclical vomiting syndrome and was given advice about eating and drinking regularly, especially on days when he participated in sport. He continued to have more episodes, however, so was given propranolol to take on a regular basis. Over the following three months he did not have any further episodes.

Background

The actual incidence of CVS is not known. It is thought to occur in around 3 out of 100,000 children although it may be more common than this.1 It occurs more commonly in those people who have migraines and also those in whom there is a family history of migraines.

There is an overlap between CVS and migraines and this condition is thought to belong to a spectrum of cyclical disorders which may have a genetic link.2 The most common age of presentation is around 5 years. However, around 20% of cases are adults.

Presentation

The main symptoms of CVS are sudden onset severe nausea and vomiting which lasts for anything from a few hours to several days. There are four phases of this condition; prodromal, vomiting, recovery and well phases.

During the prodromal phase the patient often looks very pale and can have sweating and also some nausea. Abdominal pain can sometimes occur. This phase can last for up to a few hours.

The vomiting phase then occurs and the nausea, vomiting and retching can last for 20 to 30 minutes at a time. Some patients also experience intense abdominal pain. Other symptoms can include lack of appetite, diarrhoea, headache, photophobia and dizziness.

Once the vomiting has subsided, then the recovery phase begins. The patient often notices that their energy levels return and also that their appetite returns. Finally, the wellness phase follows during which the patient is completely well again.

Each episode of cyclical vomiting syndrome is usually similar to previous ones, so that 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. This condition can affect an individual for months, years or even decades.

Complications such as oesophagitis, Mallory-Weiss tear and dehydration can occur in the more severe cases.

Triggers

Although some attacks occur spontaneously, there are some triggers that can precipitate an attack. These include dehydration, lack of food, stress or anxiety, hot weather, excessive exercise or sleep deprivation.

Diagnosis

The diagnosis is usually made clinically. Investigations are sometimes undertaken to exclude other conditions.

The diagnosis can be made in children with the following3:

  • 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 between episodes
  • Symptoms cannot be attributed to another disorder

The diagnosis can be made in adults with the following:

  • 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

Treatment

It is important to identify and avoid any potential triggers such as those listed above. Patients need to be advised to get adequate sleep, avoid foods that trigger episodes and address any stress or anxiety. It is important that patients are advised to eat a healthy, balanced diet and also eat their meals regularly. Relatives of patients need to be given education about this condition.

Preventative medications are often considered for those patients who have more than one episode a month. Prophylactic treatments include amitriptyline, propranolol and topiramate. Benefits of treatment need to be balanced against potential side effects.

Medications used for aborting acute episodes include ondansetron, prochlorperazine and triptans. They need to be taken early in the prodromal phase and can help to stop an episode becoming worse or even occurring. The addition of erythromycin to propranolol has been shown to improve the response to treatment in children.4

Supportive care is important during acute episodes, such as analgesia, anti-emetics and also intravenous fluids in the more severe cases.

Outlook

The majority of patients recover completely with time. This can be several years. Around a half of patients with CVS develop migraines.5

  • Dr Louise Newson is a GP in the West Midlands

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References

  1. Kaul A, Kaul KK. Cyclic Vomiting Syndrome: A Functional Disorder. Pediatr Gastroenterol Hepatol Nutr. 2015 Dec;18(4):224-9.
  2. Gelfand AA. Migraine and childhood periodic syndromes in children and adolescents. Curr Opin Neurol. 2013 Jun;26(3):262-8.
  3. 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 Sep;47(3):379-93
  4. 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 Fall;8(4):270-7
  5. 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

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