Cyclic Vomiting Syndrome

Aka: Cyclic Vomiting Syndrome, Cyclical Vomiting Syndrome, Periodic Vomiting Disorder

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II. Epidemiology

  1. Incidence in chronic unexplained Vomiting
    1. Adults: 3 to 14% (mean diagnosis age 25 years)
    2. Children: <2% (mean diagnosis age 5 years)
  2. Ethnicity
    1. More common in caucasian patients
  3. Gender
    1. Slightly more common in males
  4. Associated with frequent Emergency Department visits (mean 15 before diagnosis)
    1. Delayed Diagnosis is common
    2. Venkatesan (2010) BMC Emerg Med10:4 +PMID: 20181253 [PubMed]

III. Definitions

  1. Chronic Nausea and Vomiting
    1. Nausea and Vomiting persisting longer than one month
  2. Cyclic Vomiting Syndrome
    1. Discrete, recurrent Vomiting episodes
    2. Episodes are often preceded by Nausea, and may be accompanied with Diarrhea, dipahoresis
    3. Vomiting follows Nausea and last up to 7 days

IV. Pathophysiology

  1. Unknown cause and multiple hypotheses
  2. Cyclic Vomiting Syndrome is highly associated with Migraine Headaches
    1. Children: Up to 75% will develop Migraine Headaches
    2. Adults: Up to 36% have Migraines
  3. Images
    1. vomitingPathway.png

V. Symptoms

  1. Triggers
    1. Emotional stress
    2. Sleep deprivation
    3. Menstruation
    4. Tyramine-Vasoactive Amines (e.g. Chocolate, cheese, monosodium Glutamate)
    5. Daily Cannabinoid use (see Cannabinoid Hyperemesis Syndrome)
  2. Phases
    1. Prodromal: Aura (variably present, lasts minutes to days)
      1. Anorexia
      2. Abdominal Pain
      3. Lethargy
      4. Pallor
      5. Autonomic Symptoms (Sweating, Salivation)
    2. Acute Vomiting and Hyperemesis
      1. Episodic intense Nausea and Vomiting
        1. Children with 9 to 10 episodes per year on average
        2. Adults with 14 to 15 episodes per year on average
      2. Vomiting may occur as often as 8 times every hour
      3. Onset typically in early morning or middle of the day
      4. Duration 1 to 5 days
        1. Children average 3 to 4 days
        2. Adults average 6 days
    3. Recovery
      1. Vomiting ceases
      2. Energy recovers
      3. Appetite returns
    4. Inter-episode Remission
      1. Asymptomatic between episodes

VI. Associated Conditions

  1. Migraine Headaches (frequently cited)
  2. Menstrual periods (catamenial Cyclic Vomiting Syndrome)
  3. Diabetes Mellitus
  4. Pregnancy
    1. See Vomiting in Pregnancy

VII. Differential Diagnosis

  1. See Vomiting Causes in Children
  2. See Vomiting in Pregnancy (Morning Sickness)
  3. See Vomiting in Cancer
  4. See Psychogenic Vomiting
  5. See Medication Induced Vomiting
  6. Cannabinoid Hyperemesis Syndrome
    1. See Cannabinoid Hyperemesis
    2. May be a subset of Cyclic Vomiting Syndrome
  7. Chronic Nausea Vomiting Syndrome
    1. Bothersome Nausea or Vomiting weekly for 3 months or more AND
    2. No other organic or psychological cause identified despite thorough evaluation including endoscopy
  8. Other rare conditions with similar presentations
    1. Acute Intermittent Porphyria (associated neurologic findings)
    2. Disorders of Fatty Acid Metabolism
      1. Medium-chain-acyl-CoA Dehydrogenase Deficiency
      2. Carnitine palmityltransferase deficiency (deficiency of the mitochondrial translocation)

VIII. Diagnosis: Cyclic Vomiting Syndrome (CVS)

  1. Episodic, stereotypical Vomiting
    1. At least 2 acute episodes in past 6 months
      1. Rome IV only requires 2 episodes for CVS Diagnosis
      2. International Headache Society requires 5 episodes for CVS diagnosis
    2. Each episode occurs at least 1 week apart
    3. Each episode lasts <1 week
  2. Vomiting is absent between episodes
    1. However, more mild symptoms may be present between episodes

IX. Evaluation

  1. Exclude serious causes of Chronic Vomiting
  2. See Chronic Vomiting for red flag findings and recommended laboratory testing

X. Management

  1. See Antiemetic
  2. See Vomiting Management in Children
  3. See Vomiting in Pregnancy
  4. See Postoperative Nausea and Vomiting
  5. See Vomiting in Cancer
  6. Trigger Avoidance
    1. See triggers above (includes Cannabinoids)
  7. Abortive management
    1. See Cannabinoid Hyperemesis Syndrome
    2. Background
      1. Little evidence for emergency department management for Cyclic Vomiting Syndrome
      2. Author has had success in the Emergency Department with Dopaminergic Antiemetics
        1. Recommends slow infusion over 15 minutes in a piggy back bag to prevent Dystonic Reaction
        2. Authors greatest success (anecdotal) has been with Droperidol or Olanzapine
        3. Author has had poor success with Ondansetron in the emergency department for CVS
    3. Emergency Department
      1. Intravenous Fluids (e.g. D5LR)
      2. Dopaminergic agents
        1. Droperidol (Inapsine)
        2. Olanzapine (Zyprexa)
        3. Metoclopramide (Reglan)
        4. Promethazine (Phenergan)
      3. Other agents
        1. Diphenhydramine
        2. Lorazepam
    4. Home abortive management
      1. 5-HT3 Receptor Antagonists (e.g. Ondansetron)
      2. Triptans (e.g. Sumatriptan, Rizatriptan)
      3. NK1 Receptor Antagonist (Aprepitant)
  8. Prophylactic Medications
    1. Efficacy
      1. Effective for chronic remission (or reduction of episodes) in 70% of cases
      2. Worse prognosis for remission in several sub-groups
        1. Poorly controlled Migraine Headaches
        2. Psychiatric Disorders
        3. Chronic Opioid use
        4. Cannabinoid Hyperemesis Syndrome
    2. Prophylactic agents also commonly used in Migraine Headache Prophylaxis
      1. Tricyclic Antidepressants (e.g. Amitriptyline, Nortriptyline)
        1. Hejazi (2010) J Clin Gastroenterol 44(1):18-21 +PMID: 20027010 [PubMed]
      2. Topiramate
        1. Sezer (2016) J Neurogastroenterol Motil 22(4):656-60 +PMID: 27302967 [PubMed]
      3. Propranolol
        1. Haghighat (2007) World J Gastroenterol 13(12):1833-6 +PMID: 17465476 [PubMed]
    3. Supplements with evidence of benefit
      1. Coenzyme Q10
        1. Boles (2010) BMC Neurol 10:10 +PMID: 20109231 [PubMed]
      2. L-carnitine
        1. Boles (2011) BMC Neurol 11:102 +PMID: 21846334 [PubMed]

XI. References

  1. Hayes (2018) Clin Exp Gastroenterol 11:77-84 +PMID: 29520160 [PubMed]
  2. Raucci (2020) Front Neurol 11:583425 +PMID: 33224097 [PubMed]

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