Patient Presentation
A 13-year-old female came to clinic after a 12 hour emergency room visit for severe emesis, nausea, headache and abdominal pain 2 days previously. She had about 2 hours of nausea, milder abdominal pain and headache that was frontal with radiation to bilateral temples. She had no photophobia or phonophobia. She then began to have multiple episodes of emesis over the next 2-3 hours and her nausea, headache and abdominal pain became worse. After another 4 hours, she began to feel better and was much improved after sleeping. The emergency room diagnosed her with a severe migraine as she didn’t have or develop any infectious symptoms and her laboratory testing showed electrolytes that were consistent with emesis and dehydration. Her abdominal and head computed tomographic studies were negative. The first day after the emergency room visit she was more tired, but in the office she and her mother deny any problems. The past medical history showed that she had some intermittent headaches and these mainly occurred with illnesses or with poor sleep. She had started her menses about 7 months previously and they were intermittent. The family history is strongly positive for migraines on the maternal side including her mother, older sister and maternal aunt. This maternal aunt’s daughter has cyclic vomiting syndrome, and her other son had migraines.
The pertinent physical exam showed normal vital signs and her weight was back to her pre-emesis weight. Her examination was unremarkable.
The diagnosis of a likely first migraine was made. Her mother had many questions about potential treatment and also cyclic vomiting syndrome. “They only recently diagnosed her cousin with the cyclic vomiting after a long time having lots of problems. They are looking at my nephew too. I just want to be proactive about this if she is going to have a lot more problems,” her mother stated. The pediatrician acknowledged the mother and patient’s concerns by saying, “With this family history we’ll need to be really aware. Maybe you’ll never have another episode like this one, but we need to start keeping track of the symptoms early on, so we can see if there are any patterns. If you have more it may be migraine but it could be cyclic vomiting. I’m going to go over using a symptom diary. It’s an important part of the evaluation for these types of problems. It’s just like keeping track of sugar measurements for people with diabetes. It helps us figure out problems and how to treat them. Also treatment for migraines and cyclic vomiting is similar. Healthy lifestyle changes like making sure you have consistent sleep, exercise, and don’t get dehydrated are really important. If you have more episodes we can talk about maybe starting a medicine to prevent them. If you start to feel that nausea, headache or abdominal pain again, then you can use a medicine to try to stop it before it starts like your mom does. It’s called a triptan and I’ll go over how to use it,” she counseled.
The patient’s clinical course showed another episode in the next 4 months that responded to triptan use and did not require an emergency room visit.
Discussion
Cyclic vomiting syndrome (CVS) “…is characterized by episodic attacks of intense nausea and emesis, with predictably cyclic timing of episodes, and complete resolution of symptoms between attacks.” It can be very difficult to diagnose and likely is underreported. Incidence is thought to be around 3.5/1000,000 persons. There are four phases to the clinical syndrome:
- Prodromal phase is often brief (1-2 hours) where patients can have intensive nausea, abdominal pain, pallor and tiredness
- Other symptoms may include headache, mood changes, phono- or photophobic, yawning, and systemic autonomic nervous symptoms. These can continue through the vomiting and recovery phases as well.
- Vomiting phase
- Lasts usually hours
- First hour has the most emesis (6+ times) that usually wanes over the next 4-8 hours
- May need intravenous hydration in many cases or hospitalizations
- Recovery phase begins with when the nausea remits and continues until the patient has recovered their appetite, body weight lost during vomiting phase and strength. Patients usually sleep during this period. This is also usually a brief period of about 6 hours, but symptoms can linger for up to 1 week.
- Interepisodic phase where patients are symptom free
It can be difficult to tell if the episodes are recurrent emesis or an episodic attack. While the symptoms patients experience and the timing and duration are different, they are often stereotypical for an individual patient. “Attacks can last from hours to days (between 1h and 10 days, mean 2 days). Typically, attacks have a predictable periodicity…[and t]his periodicity is the discriminating criterion for …classification [as CVS]. This periodicity is variable for each patient.”
Symptom absence between episodes is also a key feature.
Some patients may be able to recognize potential triggers such as lack of sleep, exercise, excitement/stress, menstruation, and potentially certain foods (i.e. cheeses, chocolate, acidic or salty foods).
Natural history is that symptoms may resolve within 10 years (about 60% of children). About 50% go on to have a migraine syndrome. CVS is thought to be linked to migraine by several mechanisms mainly as both are primary brain disorders. The differential diagnosis of CVS includes:
- Central nervous system
- Migraine
- Epilepsy/seizure
- Intracranial masses
- Cannabis-induced hyperemesis syndrome
- Autonomic dysfunction
- Gastrointestinal
- Gastroesophageal reflux disease
- Obstruction
- Inflammatory bowel disease
- Celiac disease
- Cholecystitis
- Peptic ulcer disease
- Metabolic
- Inborn errors of metabolism
- Mitochondrial disease gastrointestinal problems including gastroesophageal reflux disease, obstruction, inflammatory bowel disease
- Renal
-
Hydronephrosis
If the patient does not have resolution of their symptoms between attacks or has other central nervous system (CNS) problems such as developmental or intellectual problems, seizures, evidence of encephalopathy, or it appears that the attacks may precipitate CNS problems, or that there are additional gastrointestinal symptoms (i.e. gastrointestinal bleeding) then these are red flags that should be evaluated.
Learning Point
Treatment for CVS includes:
- Lifestyle changes – consistent food and fluid intake, sleep, and exercise
- Interepisodic phase
- Prophylactic medication for those that are severe (> 2 day duration, hospitalization) or frequent (> every 4-6 weeks)
- < 5 years old = cyproheptadine
- > 5 years old = amitriptyline. This is the most effective agent overall
- Consider propranolol as second line
- There are other options if these are not tolerated or give insufficient control
- Prodromal phase
- Triptans used as an abortive medication – sumatriptan, rizatriptan, zolmitripan
- Benzodiazepines may be helpful for panic anxiety or anticipation of vomiting phase
- Vomiting phase
- Intravenous fluids with glucose to provide energy
- Intravenous, rectal or dermal formulations of
- Antiemetics – ondanstron, granisetron
- Analgesics – ketorolac
- Sedatives – z, diphenhydramine
- Environment that is dark, quiet
- Recovery phase
- Management of symptoms as needed
Questions for Further Discussion
1. Explain the physiology that causes emesis? A review can be found here
2. What are different types of headaches? A review can be found here
3. What causes vomiting? A review can be found here
Related Cases
- Disease: Cyclic Vomiting Syndrome | Nausea and Vomiting | Migraine
- Symptom/Presentation: Vomiting | Headaches | Abdominal Pain
- Specialty: Emergency Medicine | Neurology / Neurosurgery
- Age: Teenager
To Learn More
To view pediatric review articles on this topic from the past year check PubMed.
Evidence-based medicine information on this topic can be found at SearchingPediatrics.com and the Cochrane Database of Systematic Reviews.
Information prescriptions for patients can be found at MedlinePlus for these topics: Nausea and Vomiting and Migraine.
To view current news articles on this topic check Google News.
To view images related to this topic check Google Images.
To view videos related to this topic check YouTube Videos.
Donnet A, Redon S. Cyclic Vomiting Syndrome in Children. Curr Pain Headache Rep. 2018;22(4):30. doi:10.1007/s11916-018-0684-6
Li BUK. Managing cyclic vomiting syndrome in children: beyond the guidelines. Eur J Pediatr. 2018;177(10):1435-1442. doi:10.1007/s00431-018-3218-7
Kovacic K, Li BUK. Cyclic vomiting syndrome: A narrative review and guide to management. Headache. 2021;61(2):231-243. doi:10.1111/head.14073
Author
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa
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