Abdominal Migraine, Pediatric

Basics

Description

Paroxysmal disorder of an acute onset, severe, noncolicky, periumbilical abdominal pain accompanied variably with nausea, vomiting, anorexia, headache, and pallor

Epidemiology

Incidence

Occurs mostly in children; mean onset at age 7 years (3-10 years)
Peak symptoms 10-12 years of age
More common in girls (3:2)

Prevalence

May affect as many as 1-4% of children at some point in their lives
Declining frequency toward adulthood

Risk Factors

Genetics
Parents of affected children often have history of migraine headaches and motion sickness.

Etiology

May involve neuronal activity originating in the hypothalamus with involvement of the cortex and autonomic nervous system
Serotonin is implicated, and blockade of serotonin receptors may prevent abdominal migraine.
Recent studies suggest involvement of local intestinal vasomotor factors

Diagnosis

Rome III criteria-2 episodes within 12 months meeting all of the following criteria:

Paroxysmal intense periumbilical pain that lasts >1 hour
Intervening episodes of health between episodes
Pain that interferes with activity
Pain associated with ≥2 of the following: anorexia, nausea, vomiting, headache, photophobia, or pallor
No evidence of inflammatory, anatomic, metabolic, or neoplastic process
No evidence of elevated intracranial pressure (tumor, hydrocephalus)

History

Ask about a family history of migraine headache or unexplained bouts of abdominal pain as children.
Pain typically lasts <6 hours.
Generalized abdominal pain; can often be localized to upper quadrants
No abdominal pain between episodes
Repetition of identical abdominal crises, anywhere from 1 time per week to several times a year
Migraine in the history of patient or relatives
Occasionally, other migraine phenomena such as nausea, vomiting, perspiration, body temperature changes, focal paresthesias, radiation of pain to a limb, visual disturbances, or general malaise
Associated fatigue, lethargy, or impairment of consciousness

Physical Exam

Physical exam, including complete neurologic and abdominal exam, is usually unremarkable.
Complete eye exam including funduscopic exam should be done to evaluate for papilledema (elevated intracranial pressure).

Diagnostic Tests & Interpretation

Abdominal migraine is a diagnosis of exclusion.
Even if a patient meets most or all diagnostic criteria for abdominal migraine, studies as outlined below should be strongly considered to ensure that another serious disorder does not exist.

Lab

CBC with differential
ESR and CRP
Urinalysis
Pregnancy test
Amylase and lipase
Stool Hemoccult
Stool culture
Lactose breath test for lactose intolerance
Lead level
Evaluation for porphyria or familial Mediterranean fever
Metabolic evaluation (obtain during symptomatic episode, not during quiescence): urine organic acids, plasma amino acids, ammonia, lactate, blood gas, acylcarnitine profile, imaging

Diagnostic Procedures/Other

Obstruction series to assess for intermittent or partial bowel obstruction
Upper GI to rule out anatomic abnormalities
US or CT scan to rule out mass lesion or chronic appendicitis
Renal US during episodes to rule out ureteropelvic junction (UPJ) obstruction
Barium enema (during painful crisis) to rule out intussusception
EEG may help differentiate between abdominal migraine and epilepsy.
Visual evoked response (VER) to red and white flashlight: Children with abdominal migraine may display a specific fast-wave activity response.
Rarely, brain imaging with CT or MRI may be useful for evaluating causes of intermittent hydrocephalus.

Differential Diagnosis

Infection
- Giardia
Environmental
- Lead intoxication
Tumors
Metabolic
- Porphyria, lactose intolerance, female carriers of (X-linked) ornithine transcarbamylase (OTC) gene mutation, organic acidemias
Psychosocial
- Functional abdominal pain/irritable bowel syndrome
Surgical
- Appendicitis, intussusception, biliary colic
Inflammation
- Inflammatory bowel disease, peptic ulcer disease, mesenteric adenitis
GI
- Irritable bowel syndrome, gastroesophageal reflux, wandering spleen, cyclical vomiting syndrome, recurrent abdominal pain, functional abdominal pain, constipation, superior mesenteric artery (SMA) syndrome, recurrent pancreatitis
Anatomic
- Meckel diverticulum, UPJ obstruction
Neurologic
- Abdominal epilepsy-but has a shorter duration of pain (minutes), altered consciousness during event, abrupt onset, abnormal discharges in EEG in 80%
- Temporal lobe epilepsy
- Intermittent hydrocephalus (possibly secondary to a 3rd ventricle colloid cyst)

Alert
Because it is usually a diagnosis of exclusion, many patients go through a large workup to rule out other causes of pain, sometimes including laparotomy.

Treatment

Medication

Medications can be used to abort acute attacks or be taken as daily prophylaxis.
For most patients, risks of side effects and complications from the use of these medications may outweigh the relief of pain, especially in children who are experiencing infrequent episodes.
Limited data exist on abortive agents for abdominal migraines; however, several agents have shown benefit in specialty-based clinical practice, including metoclopramide, steroids, intranasal sumatriptan, and NSAIDs (although the latter may be avoided if there are clinical concerns for gastritis or peptic ulcer disease). Consider benzodiazepines (i.e., lorazepam) and antiemetics (i.e., ondansetron) for vomiting-predominant symptoms.
Suggested prophylactic treatments are similar to those for migraine headaches and include tricyclic antidepressants (e.g., amitriptyline), topiramate, propranolol, cyproheptadine, and valproic acid. If EEG or other data point to possible epilepsy, empiric treatment with anticonvulsants may be considered.

Additional Treatment

General Measures

Trigger avoidance
- An event diary should be kept to identify possible migraine triggers.
- Avoiding triggers is the most optimal strategy for preventing recurrent attacks:
  - Common triggers include caffeine, nitrites, amines, emotional arousal, travel, prolonged fasting, altered sleep, exercise, and/or flickering lights.
Cognitive therapies
- Behavioral therapies and lifestyle modification (regular sleep, hydration, and exercise) may also be of benefit. Biofeedback in conjunction with other cognitive therapies and/or relaxation programs may be helpful. Assistance from a trained pediatric mental health professional may be useful.

Ongoing Care

Follow-up Recommendations

Most children outgrow abdominal migraine symptoms (~60%) by early adolescence.
A substantial percentage of patients (~70%) may develop more typical migraine headaches during adulthood.
Although nonspecific EEG changes are seen more commonly in this condition, very few children go on to develop epilepsy.
10% of children who have a diagnosis of migraine headaches have previously suffered from unexplained recurrent abdominal pain.
Adult migraine headache sufferers experience abdominal pain more frequently than do tension headache sufferers.

Patient Education

To help child during bouts of pain, allow the child to do whatever makes him or her comfortable-rest, positioning, being quiet.
Whether the patient should be excused from school depends on various factors:
- Frequency, severity, and duration of pain
- Age, maturity, and coping skills of the child

Additional Reading

Catto-Smith AG, Ranuh R. Abdominal migraine and cyclical vomiting. Semin Pediatr Surg. 2003;12(4):254-258. [View Abstract]
Lewis DW. Pediatric migraine. Neurol Clin. 2009;27(2):481-501. [View Abstract]
Li BU, Balint JP. Cyclic vomiting syndrome: evolution in our understanding of a brain-gut disorder. Adv Pediatr. 2000;47:117-160. [View Abstract]
Popovich DM, Schentrup DM, McAlhany AL. Recognizing and diagnosing abdominal migraines. J Pediatr Health Care. 2010;24(6):372-377. [View Abstract]
Rasquin A, Di Lorenzo C, Forbes D, et al. Childhood functional gastrointestinal disorders: child/adolescent. Gastroenterology. 2006;130(5):1527-1537. [View Abstract]
Russell G, Abu-Arafeh I, Symon DN. Abdominal migraine: evidence for existence and treatment options. Paediatr Drugs. 2002;4(1):1-8. [View Abstract]
Tan V, Sahami AR, Peebes R, et al. Abdominal migraine and treatment with intravenous valproic acid. Psychosomatics. 2006;47(4):353-355. [View Abstract]
Weydert JA, Ball TM, Davis MF. Systematic review of treatments for recurrent abdominal pain. Pediatrics. 2003;111(1):e1-e11. [View Abstract]

Codes

ICD09

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
346.22 Variants of migraine, not elsewhere classified, without mention of intractable migraine with status migrainosus
346.23 Variants of migraine, not elsewhere classified, with intractable migraine, so stated, with status migrainosus

ICD10

G43.D0 Abdominal migraine, not intractable
G43.D1 Abdominal migraine, intractable

SNOMED

75879005 Abdominal migraine (disorder)

FAQ

Q: Does this diagnosis mean my child will develop migraine headaches?
A: There is no accurate way to predict whether your child will develop migraine headaches.
Q: I have 2 younger children. What chance do they have of developing abdominal migraines?
A: Although migraine headaches do tend to run in families, there is no known mendelian inheritance pattern.
Q: What can I do to help my child during bouts of pain?
A: First, allow the child to do whatever makes him or her comfortable. This may mean resting, positioning, or being quiet. Acetaminophen or NSAID-based pain relievers may help to a certain degree. Whether the patient should be excused from school depends on various factors such as the frequency, severity, and duration of the pain as well as the age, maturity, and coping skills of the child.
Q: My child is having frequent episodes that are affecting his/her quality of life. What can we do?
A: It may be appropriate to trial him/her on prophylactic daily medications.

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