Basics
Description
- A chronic, idiopathic disorder characterized by recurrent, discrete episodes of disabling nausea and vomiting separated by symptom-free intervals lasting a few days to months
- Adult population " average age of diagnosis is 31:
- Average age of onset is 21
- Pediatric population " average age of diagnosis is 5
- General characteristics:
- Phase 1: Interepisodic phase:
- Phase 2: Prodrome:
- Varying intensity of nausea and diaphoresis
- Phase 3: Emetic phase:
- Intense nausea/vomiting/retching/dry heaving up to 7 days
- Phase 4: Recovery phase:
- Improvement of nausea and tolerance of PO intake
Epidemiology
Incidence and Prevalence Estimates
- True incidence and prevalence in adult general population unknown due to limited data and research, increasing recognition in syndrome
- In pediatric population, cyclic vomiting syndrome affects 0.04 " 2% of population with estimated new cases 3/100,000 annually
Etiology
- Etiology unknown
- Pathophysiology is also unknown and is under research:
- Limited research suggests multifactorial factors such as autonomic, central, and environmental to be involved
Diagnosis
Signs and Symptoms
Commonly present to ED with unexplained onset of nausea/vomiting and abdominal pain.
History
- History of similar prior episodes
- No preceding trigger identified at times but typically when asked specifically may identify
- Will complain of abdominal pain, usually epigastric
Physical Exam
May have benign physical exam or various findings based on degree of dehydration:
- Normal vital signs or abnormal vital signs demonstrating:
- Tachycardia
- Hypotension (including orthostatic hypotension)
- Tachypnea
- Cool extremities and/or delayed (>2 s) capillary refill indicating shock
- Varying degrees of consciousness:
- Alert, lethargic, or obtunded
- Dry mucous membranes:
- Sunken eyes
- Dry/sticky or cracked mouth
- Poor skin turgor
- Oliguria or anuria
May present with above in addition to refusal to eat/drink, reduced or lack of tear production, sunken fontanels, reduced or absent urine output (reduced wet diapers)
Essential Workup
Must rule out other potentially serious conditions (see Differential Diagnosis)
Diagnosis Tests & Interpretation
- Perform necessary exam and lab or radiographic tests necessary to rule out other conditions with similar presenting signs and symptoms
- Cyclic vomiting has no specific diagnostic feature nor specific biochemical marker
- Extensive list of other diagnostic possibilities
- Diagnosis of adult cyclic vomiting is based on Rome III criteria:
- Stereotypical episodes of vomiting regarding onset (acute) and duration (<1 wk)
- At least 3 episodes in the past year
- Absence of nausea/vomiting between episodes
Lab
- CBC
- Electrolytes, BUN/Cr, glucose
- Liver enzyme, liver profile
- Lipase
- Lactate
- Urinalysis
- Pregnancy test
- Toxicology screen/drug levels:
- Acetaminophen
- Salicylic acid
- Alcohols:
- Ethanol, isopropanol, methanol, ethylene glycol
- Digoxin
Imaging
Atypical severity or atypical episodes should raise suspicion of underlying disorder not due to cyclic vomiting:
- Tailor imaging to individual patient presentation
Diagnostic Procedures/Surgery
Outpatient gastric emptying study should be done to r/o gastroparesis or other gut motility disorders as cause of frequent emesis.
Differential Diagnosis
- Infectious:
- Appendicitis
- Pyelonephritis
- Pneumonia
- Cholecystitis
- Metabolic/endocrine:
- Renal failure/uremia
- Electrolyte disorder
- Diabetic ketoacidosis
- Thyroid disorder
- Adrenal insufficiency
- Pheochromocytoma
- Pregnancy or hyperemesis gravidarum
- Renal:
- Nephroureterolithiasis
- UVJ obstruction/hydronephrosis
- GI:
- Gastroparesis
- Bowel obstruction
- Peptic ulcer disease
- Cholelithiasis
- Pancreatitis
- Malrotation with volvulus
- Inflammatory bowel disease
- CNS:
- Intracranial hemorrhage
- Brain tumor
- Hydrocephalus
- CVA
- Cardiovascular:
- Anginal equivalent
- STEMI/NSTEMI
- Toxicology (examples):
- Cannabinoid hyperemesis
- Mushroom toxicity:
- Acute alcohol/toxic alcohol ingestion:
- Ethanol, isopropanol, methanol, ethylene glycol
- Alcohol withdrawal
- Heroin withdrawal
- Any acute/subacute ingestion; consider:
- Acetaminophen
- Salicylic acid
- Digoxin
- Psychiatric:
- Self induced
- Bulimia
- Anorexia
- Anxiety
Munchausen by proxy
Treatment
Pre-Hospital
- Address airway/breathing/circulation
- Initiate IV, oxygen (if indicated), place on cardiac monitor
- Start IV fluids if presenting with vomiting and/or abnormal vital signs
Initial Stabilization/Therapy
- Address airway/breathing/circulation
- Continue IV/O2 (as indicated), cardiac monitor
- Address abnormal vital signs specifically hypotension and tachycardia:
- Adults: 500 to 1000 mL bolus 0.9% NS
- Pediatric: 20 mL/kg bolus 0.9% NS
Ed Treatment/Procedures
- Supportive care in acute phase
- Abort emetic phase of nausea/vomiting with antiemetics
- IV 0.9 normal saline:
- Add dextrose after initial boluses
- Correct electrolyte abnormalities
- Treat pain with analgesics
- Provide light sedation for very symptomatic patients
- Administer gastric acid suppressants:
- H2 receptor antagonist
- Proton pump inhibitors
- Consider antimigraine triptans
Medication
Antiemetics
- Ondansetron 4 " 8 mg IV/PO/ODT q4 " 8h prn
- Metoclopramide 10 mg IV/IM q2 " 3h prn 4 " 8 mg IV/PO/ODT q4 " 8h prn
- Prochlorperazine 5 " 10 mg IV/PO/IM (peds: 0.1 mg/kg/dose PO/IM/PR) q6 " 8h prn
- Promethazine 12.5/25 mg PO/IM/PR q4 " 6h (IV use common but not approved) (peds: 0.25 " 1 mg/kg PO/IM/PR q4 " 6h prn if >2 yr)
Pain/Sedation
- Ketorolac 15 " 30 mg IV
- Lorazepam 0.5 " 1 mg IV/IM/PO
- Morphine 0.1 mg/kg IV
- Sumatriptan 4 " 6 mg SC-repeat in 1 hr prn
Gastric Acid Suppressants
- Cimetidine (H2-blocker): 800 mg PO at bedtime nightly (peds: 20 " 40 mg/kg/24 h)
- Famotidine 20 mg IV q12h
- Pantoprazole 40 mg IV q24h
- Ranitidine 50 mg IV/IM q8h
Follow-Up
Disposition
Admission Criteria
- Vital signs/lab or physical exam findings suggestive of moderate to severe dehydration
- Inability to tolerate PO fluids
Discharge Criteria
- Stable vital signs
- Cessation of vomiting and pain control
- Able to tolerate PO fluids and keep self hydrated
Issues for Referral
- GI consult for further outpatient workup when symptom free
- Consider additional referral to specialist managing this syndrome
Follow-Up Recommendations
- Prophylaxis:
- Identification and avoidance of triggers:
- Emotional stress, poor sleep, fasting, illness, marijuana, specific foods (chocolate, cheeses, etc.)
- Management of coexisting conditions:
- Migraine headaches, psychiatric disorders, chronic narcotic, and marijuana use
- Medications (outpatient-in active research):
- Tricyclic antidepressants (amitriptyline)
- Propranolol
- Coenzyme Q-10
- Antihistamines
- Antianxiety medications
Pearls and Pitfalls
- Obtain good history about prior cyclic episodes and similarities to prior episodes
- Manage active coexisting conditions if applicable
- Exclude other disorders with similar presentations of nausea/vomiting/abdominal pain
Additional Reading
- Abell TL, Adams KA, Boles RG, et al. Cyclic vomiting syndrome in adults. Neurogastroenterol Motil. 2008;20:269 " 284.
- Fleisher DR, Gornowicz B, Adam K, et al. Cyclic Vomiting Syndrome in 41 adults: The illness, the patients, and problems of management. BMC Med. 2005;3:20.
- Hejazi RA, McCallum RW. Review article: Cyclic vomiting syndrome in adults " rediscovering and redefining an old entity. Aliment Pharmacol Ther. 2011;34:263 " 273.
- Venkatesan T, Prieto T, Barboi A, et al. Autonomic nerve function in adults with cyclic vomiting syndrome: A prospective study. Neurogastroenterol Motil. 2010;22:1303 " 1307.
- Venkatesan T, Tarbell S, Adams K, et al. A survey of emergency department use in patients with cyclic vomiting syndrome. BMC Emerg Med. 2010;10:4.
Codes
ICD9
- 346.20 Variants of migraine, not elsewhere classified, without mention of intractable migraine without mention of status migrainosus
- 346.21 Variants of migraine, not elsewhere classified, with intractable migraine, so stated, without mention of status migrainosus
- 536.2 Persistent vomiting
ICD10
- G43.A Cyclical vomiting
- G43.A0 Cyclical vomiting, not intractable
- G43.A1 Cyclical vomiting, intractable
SNOMED
- 18773000 cyclical vomiting syndrome (disorder)