Abdominal Migraine in Children: Is It All in Their Heads?

Article Outline

• Abstract
• Scope of the Problem
• Case Study
• Diagnostic Criteria
• Pathophysiology
• Differential Diagnosis
• Prevention
• Treatment
• Conclusion
• References
• Copyright

Abstract 

Abdominal migraine in children is a migraine variant, described as isolated, paroxysmal attacks of severe periumbilical abdominal pain associated with nausea, vomiting, pallor, anorexia, headache, and photophobia, with intervening periods of normality. Abdominal migraine is a diagnosis of exclusion, as presenting symptomatology can also be characteristic of other disease processes. An extensive history and physical examination are necessary to differentiate between abdominal migraine and acute abdominal pain. Limited studies have been conducted on the management of children with an acute abdominal migraine attack. Treatment and prophylaxis of acute attacks is essential to reduce recurrence, severity, and extent of pain.

Keywords:  abdominal migraine , childhood periodic syndromes , chronic abdominal pain , functional abdominal pain , migraine subtypes , migraine syndrome

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“I feel like I have a headache in my stomach,” KM, 11 years old

Abdominal migraine (AM) is one of the most challenging diagnoses in children. Despite the increased recognition of AM in children in the past decade, the diagnosis remains controversial. The correlation between headache and abdominal pain has been a well-known phenomenon since the early 1900s. Buchanan in 1921 and Brams in 1922 were the first to propose the term AM to explain abdominal pain attacks not associated with headaches yet with analogous features to migraine headaches.¹ In 1988 the diagnosis of AM was established by the International Headache Society (IHS).

A study conducted in 1993 by Mortimer, Kay, and Jaron² was one of the first to follow the IHS criteria for AM. They studied the epidemiology of AM in children with a history of recurrent abdominal pain in an urban general practice. They found that AM occurred in 2%-4% of schoolchildren between the ages of 3 and 10, was significantly higher in girls, had a peak prevalence between the ages of 5 and 7 (particularly in children with a maternal history of migraine), and was associated with travel sickness.

After this study, in 1995 Abu-Arafeh and Russell³ studied the incidence and etiology of headache and abdominal pain among 2,165 Aberdeen schoolchildren 5-15 years old. They found that 10.6% of these children met the IHS criteria for the diagnosis of migraine, 4.1% for the diagnosis of AM, and 24% for both. They also found that the clinical features of AM recurrence, common triggers, alleviating factors, associated symptomatology, and duration of symptoms were similar to those with migraine headache. Based on these findings, they concluded that a common pathogenesis exists between migraine headache and AM. They recommended adherence to the IHS diagnostic criteria for the diagnosis of AM for children who present with recurrent abdominal pain. This research has been cited in the childhood periodic syndromes literature as one of the first studies to follow the initial AM criteria described by IHS, which was a precursor to the current criteria.

Beginning in 1995, Bentley et al⁴ conducted a 10-year prospective study of 150 children who presented with recurrent abdominal pain, 46% of whom had symptoms consistent with AM. Of those, 90% had a first-degree relative with a history of migraine headache. This longitudinal study further contributed to the development of diagnostic criteria for the diagnosis of AM.

Despite the controversy surrounding the classification of idiopathic, periodic, and severe attacks of abdominal pain in children as AM, it was incorporated into the International Classification of Headache Disorders (ICHD) by the IHS (with revised criteria) in 2004.⁵ In 2006 the term was also incorporated to the Rome III Pediatric Criteria for functional gastrointestinal disorders (FGID) in children.⁷

Since its integration to the ICHD and the Rome III,⁶ there has been extensive disagreement on the number of events essential for diagnosis of AM. A Brazilian study⁹ was conducted among 1,113 children 5-12 years old and registered in public elementary school. The investigators reviewed the incidence of childhood periodic syndromes and their correlation with headache, migraine headache, and tension-type headaches. Arruda and colleagues⁹ reported that recurrent abdominal pain was the most common symptom (32.9% prevalence), more than double any of the other complaints. They reported significant association between symptoms seen in periodic syndromes and the 3 tested migraine subtypes. They also observed that interictal symptoms were independently associated with all 3 types of headaches. They concluded that childhood periodic syndromes in children are associated with migraine and migraine subtypes and tension headache.

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Scope of the Problem 

The incidence of AM ranges from 2.4% to 4.1% among children 7-12 years old, is more common in girls, and occasionally continues into adult years.⁸ AM is considered to be a diagnosis of exclusion.

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Case Study 

An 11-year-old girl presented to the emergency department (ED) with acute onset of severe abdominal pain in the periumbilical area, nausea, vomiting, and pallor. According to the mother, she was doing well until 2 hours before presentation to the ED, when unexpectedly she started to complain of vague abdominal pain that quickly escalated in intensity to the point of doubling over, throwing herself to the ground, vomiting, and developing a “ghostly” appearance. Review of symptoms was otherwise unremarkable.

Past medical history revealed recurring attacks of severe abdominal pain since age 7, prompting extensive laboratory and radiological investigations with negative results, including multiple endoscopies with no evidence of gastrointestinal diseases. The family gastrointestinal history was unremarkable, with no reports of inflammatory bowel disease, celiac disease, or renal or metabolic disorders. However, the patient's mother and several maternal aunts reported a history of migraine headaches.

On examination in the ED the patient was afebrile, in no acute respiratory distress, and had a completely normal physical examination. Workup included abdominal X-ray, abdominal ultrasound, computerized tomography (CT) scan of the abdomen and pelvis, blood cell count, complete metabolic panel, amylase, lipase, liver enzymes, C-reactive protein, and urinalysis, which were all normal. She was given IV ondansetron (Zofran®; GlaxoSmithKline, Triangle Park, NC) for the nausea, intravenous fluid bolus, and IV toradol for the pain. After several hours in the ED, her pain resolved, and she was discharged home to be followed by primary care.

However, the patient returned to the ED 12 hours later with worsening abdominal pain, nausea, vomiting, and headache. She was hospitalized for further evaluation and treatment. Once again, extensive investigative workup was negative. She was treated with IV toradol and famotidine with some relief. She continued to complain of severe, paroxysmal pain, and when asked about the pain, she said, “I feel like I have a headache in my stomach.” This prompted a neurology consult. She was diagnosed with AM, treated with almotriptan with resolution of headache and abdominal pain, and discharged home on nasal sumatripan for preventive treatment.

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Diagnostic Criteria 

AM attacks are diagnosed by the presenting symptoms of paroxysmal, periodic episodes of recurrent, severe abdominal pain of variable duration and intensity in otherwise healthy children with a noncontributory physical examination and completely negative workup.¹⁰ The characteristic pain may last from hours to several days, with intensity that varies from moderate to severe. It may be coupled with dramatic reactions, such as holding the abdomen, doubling over, or falling to the floor. The abdominal pain may be associated with other migraine prodromes, such as tiredness and sleepiness.¹⁰

Although AM pain is usually located in the periumbilical area, midline pain or poorly localized pain described as either dull or sore in nature can also occur.¹, ¹¹ The pain is associated with any 2 additional features that may include anorexia, nausea, vomiting, headache, photophobia, and pallor.¹⁰ Pain onset and resolution are unpredictable; they hinder daily activities and school attendance during attacks and disrupt family life. Each episode self-resolves. Complete resolution of symptoms between attacks is an important criterion to differentiate from chronic abdominal pain.

In addition, while chronic abdominal pain has been associated with psychological dysfunction or psychiatric comorbidities, this is not the case in AM. A systematic literature review by Brujin and colleagues¹² demonstrated that somatic complains of children with AM, such as headache, nausea, vomiting, and abdominal pain, are an outcome of their illness, not an indication of psychological or psychiatric problems.

As it is a diagnosis of exclusion, differentiating the diagnosis of AM is challenging and must include a thorough history, detailed review of symptoms, complete physical examination, and appropriate investigative studies that rule out any other disorders.¹³ The history must include a detailed account of recurring abdominal pain and associated symptomatology, exacerbating and alleviating factors (including medications), state of health between attacks, laboratory and radiological studies done in the past that have excluded any other processes potentially explaining the child's symptoms, and family medical history of migraine. A thorough physical examination should be essentially negative except for abdominal tenderness, which (if present) is usually diffuse and mild. Inflammatory, infectious, organic, or neoplastic causes and any other probable causes of recurrent abdominal pain, either relating to the gastrointestinal tract or extra-abdominal causes, should be ruled out by appropriate studies. It is vital that the diagnostic criteria for AM, as established by ICHD-II, be met 2 or more times in the prior year.

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Pathophysiology 

The pathophysiology of AM in children is not fully understood. Several theories have attempted to explain the pathways that trigger migraine variant attacks. Recent studies have recognized migraine variants as a familial disease. It has been suggested that the processing of pain signals by the central nervous system (CNS) is a genetically inherited phenomenon. It has also been postulated that the susceptibility to the cellular excitability of the trigeminal-vascular system leads to hyperexcitability of neuropeptides that regulate pain receptors, both in the CNS and in the abdomen.¹⁴

The constant interaction of genetic and environmental factors contributes to the development of central and enteric nervous systems.¹⁵ Opiates, the neuropeptides involved in the regulation of pain information, are considered to play a central role in the pathogenesis of abdominal pain. As these neuropeptides are stimulated by innocuous stimuli, such as stress, generalized visceral hyperalgesia occurs, contributing to gastrointestinal dysmotility and pain.¹⁵ Transient mucosal inflammation as a response to mechanical or chemical stimuli may also intensify enteric nerve sensitivity, which could also contribute to the characteristic AM pain.

The events responsible for associated symptoms seen in AM are thought to be caused by the hypothalamic-pituitary-adrenal axis system response to stress, which causes the stereotypical attacks and associated symptoms, such as pallor, nausea, vomiting, and sensory-motor abnormalities.¹⁵

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Differential Diagnosis 

AM is a diagnosis of exclusion based on the stereotypical clinical presentation, in the absence of underlying disorders for abdominal pain and with 2 or more associated features as established by the ICHD-II and the Rome III criteria guidelines¹³, ¹⁶ (Table 1, Table 2).

Table 1. 2004 International Classification of Headache Disorders II Criteria for Abdominal Migraine¹

Table 2. 2006 Rome III Diagnostic Criteria^a for Abdominal Migraine⁸

The abdominal pain in AM must be differentiated from chronic or functional abdominal pain. Chronic abdominal pain, in the absence of organic causes, is often related to emotional stress; the pain is “constant” and localized in the periumbilical area, the child is reluctant to attend school, and physical examination is usually negative except for mild generalized tenderness the exam.¹⁵ In contrast, AM attacks are sporadic in nature, with complete resolution of pain between attacks, the pain in the periumbilical region is intense, it interferes with daily activities, and there is a positive family history for migraine headaches.¹⁸

The initial differential diagnosis of AM in children must include a thorough evaluation to identify any underlying process of abdominal pain for which prompt management may change the outcome. The 2 main diagnostic categories that must be ruled out before diagnosing AM are organic disorders and functional disorders.

Organic disorders that contribute to chronic abdominal pain in children include inflammatory bowel disease, Crohn's disease, malabsorption, celiac disease, food allergies, infectious processes, drug triggers, musculoskeletal pain, and upper gastrointestinal abnormalities (gastritis, esophagitis, gastroesophageal reflux, peptic ulcers), Helicobacter pylori, gallbladder disease, kidney stones, urologic disorders, chronic pancreatitis, duodenal obstruction, sickle cell anemia crisis, gynecologic disorders (such as Mittelschmerz, congenital uterine anomalies, recurrent ovarian cysts), posterior fossa tumors, and seizure disorders if the child presents with recurrent syncope or loss of consciousness.¹, ⁴, ⁶

Functional gastrointestinal disorders are conditions in which the child suffers from recurrent abdominal pain either as a neurobiological disorder, somatic response to stress, or secondary gain.¹⁷ These disorders include functional abdominal pain, chronic abdominal pain syndrome, irritable bowel syndrome, functional dyspepsia, chronic constipation, and aerophagia. The abdominal pain in AM is differentiated from chronic abdominal pain by the sporadic nature of the pain, location and duration, interference with daily activities during the attack, and family history of migraine headaches.¹⁸ When acute abdominal pain with an established diagnosis transforms to a chronic pattern, further investigation is warranted.

AM attacks can overlap with acute abdominal processes and may be precipitated by acute illnesses. For instance, emesis accompanied by severe abdominal pain with or without fever may indicate superimposed disease process, such as appendicitis, Meckle's diverticulitis, bowel obstruction, volvulus from malrotation, acute cholelithiasis, ureteropelvic junction obstruction, urolithiasis, pyelonephritis, new onset diabetes mellitus in diabetic ketoacidosis, and Munchausen syndrome by proxy.¹⁹ Any emesis accompanied by acute changes in neurological condition as evidenced by focal neurologic findings indicates increased intracranial pressure, seizures, or metabolic disorder, requiring immediate action.

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Prevention 

Parents must be made aware that the cornerstone of treatment is preventing recurrent attacks by identifying and avoiding trigger factors. Daily diaries of child's activities, diet, stressful situations, exercise, and symptoms can help to identify triggers. Parental guidance on alternative prophylactic remedies should include lifestyle modifications, avoidance of ill-coping mechanisms to deal with attacks, stress management, travel, sleeping habits, and environmental factors, such as very bright or iridescent/strobe lights, diet, and exercise. The STRESS mnemonic (Table 3) was developed by one of this article's authors as an educational guide for providers and a practical handout for parents. This guide was formulated based on current literature and internet sources.

Table 3. STRESS Mnemonic for Prevention of Abdominal Migraine in Children

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Treatment 

Once underlying or acute processes have been ruled out and the diagnosis of AM has been established, therapeutic approaches should include both pharmacological and non-pharmacological measures. To date, limited research has been conducted in the treatment of AM in children. Current treatment modes for acute attacks focus on lessening symptoms and are based on anecdotal experience with the disease, rather than evidence from clinical trials.

The treatment goal of AM is to elicit prompt symptom relief and reintegration of the child to normality as soon as possible without further setbacks. An effective therapeutic approach is adapted to the child and family based on the child's symptomatology, prior response to treatment, and the family's sociocultural health values, which may guide their adherence to the treatment recommended. An appropriate multidisciplinary team approach should include counseling the parents and child while formulating a plan for school reintegration, along with referral for psychological counseling if maladaptive coping mechanisms are present.¹⁹ Parental assurance, reassurance, and education are vital components of the treatment. Russell and colleagues³ reported successful outcomes when parents were assured that there was no severe abdominal pathology in the child.

Parents of children diagnosed with AM should be reassured that complementary and alternative therapies during an acute pain exacerbation may help and should be tried before seeking pharmacologic treatment.¹ In helping a child suffering from AM, providers can teach parents and children helpful coping strategies to deal with pain, such as relaxation techniques through deep-breathing and guided imagery to lessen stress. During an attack, the parent should allow the child to rest in a quiet, dark room until symptoms subside and reassure the child that this will be helpful.

Table 4 outlines medications for AM in children. Over-the-counter analgesics and antiemetics often provide sufficient relief.¹, ¹⁸, ²⁰ If the pain escalates, prescription pharmacological treatment might be necessary. Limited data were found concerning appropriate prescription pharmacological management of AM attacks in children. Current pharmacological treatment is based on expert opinion and experience with medications used for migraine headaches, given the lack of well-controlled clinical trials.¹¹ Additionally, data on the use of migraine headache drugs to treat abdominal migraines have been limited to children whose symptoms are not relieved by alternative therapies, when symptoms are severe and disabling.

Table 4. Medications for Management of Abdominal Migraine in Children

Anecdotal evidence reveals that AM attacks have been treated successfully in the pediatric population with triptans.¹⁸ Almotriptan, intranasal sumatriptan, and zolmitriptan are the most widely used drugs for migraine attacks and the only medications approved for acute attacks in adolescents.²⁰ To date, almotriptan is the only medication approved by the Food and Drug Administration for the acute treatment of migraine in 12- to 17-year-olds,²⁰, ²¹ and intranasal sumatriptan and zolmitriptan have been approved by the European Medicines Agency (EMEA).

Intranasal sumatriptan, one of the 7 available triptans studied in the United States, has been considered efficacious and safe for the acute treatment phase, based on the 2004 American Academy of Neurology management guidelines for acute migraine management in children.²² Nasal sumatriptan has been reported to be more effective than placebo in relieving headache, nausea, and vomiting during an acute migraine attack in the pediatric population²⁰, ²¹ and has been anecdotally used for AM attacks with similar success.

The use of intranasal sumatriptan in children with AM is an off-label use and requires caution as serious adverse effects and death have been reported in children after its use.²³ Adverse reactions to sumatriptan include acute cardiovascular effects, such as myocardial infarction, hypertension, life-threatening arrhythmias, stroke; gastrointestinal discomfort, such as nausea and vomiting; and neuromuscular and skeletal effects, such as weakness, myalgia, numbness, hyperreflexia, and meningismus.²³

Lewis and colleagues²⁴ examined the use of zolmitriptan nasal spray in 171 adolescents during a migraine attack. Intranasal zolmitriptan was well-tolerated and showed statistically significantly greater headache relief and sustained resolution of associated migraine symptoms than placebo. The use of this spray for AM is also off-label, and its adverse effects are similar to those in intranasal sumatriptan.

Tricyclic antidepressants, selective serotonin reuptake inhibitors, and anticholigernics are among the most common medications for AM prophylaxis.²⁰, ²¹ The most common prophylactic medications in adolescents and adults reported in the literature are amitriptyline, propranolol, cyproheptadine, valproic acid, and topiramate.¹, ²⁰, ²² There are limited studies in children regarding prophylaxis therapy for AM. Until further studies are conducted and standardized criteria established, pharmacological prophylaxis treatment of AM in children should be deferred to pediatric neurologists.

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Conclusion 

The incidence and significance of AM in children is underappreciated, although the disease has been firmly established among headache authorities. Limited studies have been conducted on the management of AM in children, and recent findings advise the use of a multidisciplinary approach that includes pharmacologic and non-pharmacologic measures for prevention of AM in children and for treatment during AM exacerbations. Parental reassurance and education are critical components of treatment. The ultimate goal is to find the best treatment mode to reduce exacerbations, with the aim of improving the quality of life for children and their families.

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