Many children complain of headache at one time or another, and headache is the chief complaint for thousands of clinic and emergency department visits each year. Headaches often worry families and providers, as the causes of a headache can range from life threatening to relatively benign. A population-based study found that 17% of children in the United States reported frequent or severe headaches in the past year,1 and a systemic review of pediatric headache burden found that 58% of children experienced headache sometime during childhood.2 Fortunately, most pediatric headaches are associated with a primary headache disorder such as migraine, or a self-limited, relatively benign illness such as a viral infection. However, disability related to migraine is underrecognized and many children with recurrent headaches do not receive optimal treatment.3 This article reviews the epidemiology and definitions of different types of pediatric headaches, provides recommendations for the evaluation of children and teens with headache, and describes treatment options for the management of pediatric migraine.
Epidemiology of Pediatric Headache
Headaches are common in the pediatric population; however, there is significant variability in the results from studies of the prevalence of migraine and other pediatric headache syndromes. A study found that between 24% and 90% of children report headaches, with an overall prevalence of 58.4% (95% confidence interval [CI], 58.1%–58.8%) in childhood.2 In school-aged children, a period in which approximately 5% of children complain of headaches, researchers have found that the prevalence of headache in school-aged children is similar in males and females.2 Although the prevalence of headache increases with age in both sexes, it increases more sharply in girls than boys during puberty. In adolescence, 8% of girls and 5% of boys report a migraine, and 27% of girls and 20% of boys report frequent or severe headaches within a 1-year time period.1,4 By adulthood, the prevalence of headache and migraine is even higher, as 60% of men and 80% of women report headache, and 6% of men and 15% of women state they have had a migraine within a 1-year time period.1,2,5,6
In the emergency department (ED), the most common causes of childhood headache are viral illness (39%–57%) and migraine (16%–18%).7 Acute recurrent and chronic headaches are also common causes of visits to primary care providers. Families are often worried that a catastrophic illness, such as a brain tumor, is the cause of their child's headaches. Once this fear has been addressed it is easier to discuss management of the headache, no matter the cause.
Headache phenotypes may be divided into four basic categories: (1) acute, (2) episodic, (3) chronic progressive, and (4) chronic nonprogressive.8 Episodic and chronic nonprogressive headaches are most likely related to a primary headache disorder, although secondary causes are possible. However, providers should be concerned about headaches that are continuing to increase in frequency and severity over time (ie, chronic progressive headache). Patients with this phenotype should have complete testing, including neuroimaging, for secondary causes of headache. A single severe but brief headache is most often benign; however, other more nefarious disorders may cause a sudden severe headache. Thus, it is important to consider other potential causes of a headache before making the diagnosis of migraine or simple illness-associated headache, although this evaluation may take place over days or weeks as symptoms evolve.
History. The single most important factor in the evaluation of a child with headache is the history. This should include complete characterization of the headache (Table 1) and associated symptoms, past medical history, family history, stressors, sleep, mood, diet, hydration, activity, and triggers. It can be helpful to develop a “headache template” with these questions so patients can quickly provide basic information and the results can be reviewed during the visit.
Headaches may be associated with systemic illnesses and medications, so it is important to obtain a full medical history (eg, concern for a secondary headache would be higher in a patient with leukemia or who recently started minocycline for acne). A review of other symptoms is also important, as there may be clues to an underlying cause of the headache (eg, tachycardia, tremor, and heat intolerance suggestive of hyperthyroidism or a history of episodic torticollis consistent with migraine.)
Migraines often run in families, and other disorders related to headache may also be genetically determined, so eliciting a detailed family history is essential. Information about headaches in secondary relatives can be helpful. In my experience, the female relatives of the father of a female patient with migraine often have migraine even if he does not. In can also be helpful to learn which treatments have been most effective for family members with migraine, as these may be more successful for the patient was well.
It is also critical to discuss social history, as stress at school, home, online, or with friends may activate headaches. This could include a HEADS (Home, Education/employment, peer group Activities, Drugs, Sexuality, and Suicide/depression) assessment, discussion about struggles with family, school, or friends, drug or alcohol use, mood changes, financial stressors, cutting behavior, eating disorder, or history of abuse, all of which may exacerbate headache. In addition, headache specialists have found that poor sleep, inadequate hydration or food intake, inappropriate exercise (too much or too little), and caffeine intake more than 2 to 3 days per week may also be associated with headache.9 It is often helpful to construct a headache diary to recognize headache triggers or patterns, and now patients and families can record data easily using their choice of headache diary “apps” on their cell phone (eg, iHeadache, Headache Buddy).
Examination. In my experience, the examination should include measurement of vital signs and a thorough physical examination, noting changes that could be signs of systemic disease related to headaches. Palpation of the scalp, head, face, neck and shoulders should be done to identify evidence of limitation of nuchal movement, muscular tenderness, trigger points, occipital neuralgia, or allodynia. Signs of systemic disease, cutting behavior, or stigmata of neurocutaneous syndromes may be seen on a thorough skin examination. Dental problems should be evaluated via oral examination, with attention to teeth and temporomandibular joint function. A thorough neurological examination should be performed to screen for evidence of altered mental status, as well as abnormalities in speech, vision, eye movements, sensation, strength, reflexes, gait, or coordination. Any new focal abnormalities require further evaluation. A fundoscopic examination should be done to screen for evidence of elevated intracranial pressure (ICP) or other abnormalities that could be related to headache.
Red Flags and Neuroimaging
In the population of children who come to the ED for evaluation of headache, several factors have been associated with an intracranial space occupying lesion, including sleep-related headache, absence of family history of migraine, headache fewer than 6 months in duration, confusion, abnormal neurological examination, lack of visual aura symptoms, and vomiting.10 Children with more risk factors have a higher risk of having a brain lesion that may require surgery.10 Other symptoms that are concerning for an intracranial lesion include recurrent and focal headache, headache with increasing severity and frequency, headache with Valsalva maneuver, exclusively occipital headache, or new severe headache. In my experience, brain magnetic resonance imaging should be considered for the small number of children with concerning symptoms noted above. Computed tomography scans are best for detecting hemorrhage or fracture, but may miss posterior fossa lesions or small tumors.
The American Academy of Neurology (AAN) practice parameter for neuroimaging in the evaluation of children and adolescents with recurrent headaches states that:
Obtaining a neuroimaging study on a routine basis is not indicated in children with recurrent headaches and a normal neurologic examination.
Neuroimaging should be considered in children with an abnormal neurologic examination, the coexistence of seizures, or both.
Neuroimaging should be considered in children in whom there are historical features to suggest the recent onset of severe headache, change in the type of headache, or if there are associated features that suggest neurologic dysfunction.11
In my experience, further testing is usually not helpful unless there are signs of, or concern for, another disorder such as thyroid disease, anemia, iron deficiency, meningitis, autoimmune disease, sleep apnea, celiac disease, or other systemic illness. When this is the case, one may consider other evaluations as appropriate for the clinical scenario, as identification of a potential cause of recurrent headaches is essential. Formal ophthalmological evaluation will screen for elevated ICP and other central nervous system disorders. One may perform a lumbar puncture with measurement of opening pressure and cerebrospinal fluid indices if the patient has symptoms concerning for intracranial hypertension and brain magnetic resonance imaging/magnetic resonance venography is normal.
Primary Headache Syndromes
Migraine is the most common form of primary headache disorder that causes disability in childhood.15,16 Migraine is likely underrecognized and underdiagnosed, but it is relatively common in childhood with an overall prevalence of 7.7% (95% CI, 7.6–7.8).2 The definition of migraine as defined in the International Classification of Headache Disorders, third edition, beta version,17 (ICHD-3beta) is “recurrent headache disorder manifesting in attacks lasting 2 to 72 hours. Typical characteristics of the headache are unilateral location, pulsating quality, moderate or severe intensity, aggravation by routine physical activity, and association with nausea and/or photophobia.” However, “in young children, photophobia and phonophobia may be inferred from their behavior. Migraine attacks can be associated with cranial autonomic symptoms and symptoms of cutaneous allodynia. Occipital headache in children is rare and calls for diagnostic caution.”17 In most cases, a thorough history and examination without neuroimaging will enable providers to make an appropriate diagnosis of migraine.
In young children, vomiting or vertigo may be the most obvious symptoms of migraine and may be more prominent than headache, making the diagnosis of migraine difficult to attain in some cases.17 In children, migraine pain may be unilateral or bilateral, but is often temporal or frontal and is typically a pulsing or pounding type of pain. Pediatric migraines are usually briefer than adult attacks, often lasting only 30 to 60 minutes. Exacerbation of symptoms with light or sound may be inferred from a child's behavior (eg, the child seeks out a quiet, dark place). Attacks are often associated with decreased appetite, nausea, or vomiting. However, many other symptoms can be associated with migraine, including autonomic symptoms such as flushing and sweating, or pallor, dizziness, blurry vision, and stomach pain. The most common triggers for an attack include poor sleep, illness, fever, stress, fasting, and dehydration. Although many families are concerned about allergies and food sensitivities, in my experience these issues are likely a factor for only a minority of children with migraine.18
Migraine with Aura
Approximately one-fourth of patients with migraine will have migraine with aura.19 Auras usually begin fewer than 30 minutes before the headache starts and typically last fewer than 20 minutes, but prolonged aura may last up to 60 minutes. The most common aura are visual changes such as scotomata or scintillations, but more complex visual changes can occur. Sensory aura (numbness or tingling) is also common, but more complex auras that may include confusion, weakness, amnesia, or aphasia can be associated with migraine as well. These symptoms can be frightening, but in migraine the onset of symptoms is typically gradual rather than abrupt. In addition, the aura should completely resolve and usually lasts fewer than 30 minutes. Also, if symptoms progress from one side of the body to the other, without alteration of mental status, migraine aura is much more likely than stroke or seizure. However, thorough examination and testing, most often including neuroimaging, is warranted for any focal or acute neurological symptoms that are prolonged or not completely reversible.17
Other Migraine Syndromes
Other subtypes of migraine include migraine with brainstem aura (previously basilar migraine) and hemiplegic migraine. A brainstem aura includes at least two of the following symptoms: vertigo, ataxia, dysarthria, tinnitus, hyperacusis, diplopia, or decreased level of consciousness,17 and the accompanying headache is often occipital. Familial hemiplegic migraine is a relatively rare autosomal dominant syndrome that is characterized by prolonged hemiplegia, aphasia, numbness, and confusion with migraine. Mutations in three genes, CACN1A, ATP1A2, and SCN1A, have been associated with familial hemiplegic migraine, and penetrance is estimated to be approximately 80%.20 However, affected members of the same family may have different symptoms, and some with the HFM gene may have migraine without hemiplegia.20 In general, vasoconstricting agents should be avoided and calcium channel blockers and acetazolamide should be considered as preventive medications.
Although there may be a spectrum of symptom severity between tension-type headaches and migraine, tension headaches are much more common, usually cause much less disability, and generate fewer medical visits than migraine. The pain of tension-type headaches is mild to moderate, is often described as a “band-like” pressure around the head, and can last for hours or days. Precipitating factors include fatigue, illness, and stress (similar to migraine), but can also include muscular discomfort and tension, particularly in the cervical and shoulder region. These headaches may be episodic (<15 days per month) or chronic (≥15 days per month). As with any headache type, a full history to identify factors associated with the headaches is essential.17
The ICHD-3beta defines chronic headache as headache 15 or more days each month for 3 or more months.11 The overall prevalence of chronic migraine in adolescents is 0.78%, but rises to 1.75% when including those with medication overuse.21 The vast majority of these adolescents describe significant disability related to headache, but fewer than 20% reported taking preventive medications in the last month.21 The typical pattern preceding chronic migraine is an episodic migraine that gradually becomes more and more frequent until it is occurring more than 15 days per month. As the migraines become more frequent, the more severe symptoms, such as vomiting or aura, often become less frequent although patients often still describe spikes of severe pain accompanied by their old migraine symptoms at times. Thus, it can be challenging to classify daily headaches as chronic tension-type versus chronic migraine, as the headache characteristics may be quite similar once the headaches become daily. However, patients with chronic tension-type headaches usually do not have a history of episodic migraines.22,23
Less Common Primary Headache Syndromes
Trigeminal autonomic cephalagias (TACs), such as cluster headaches and paroxysmal hemicranias, are unusual in children, but treatment of the TACs is often different than the treatments used for migraine, so it is important for providers to be able to recognize symptoms of these disorders. These paroxysmal, often severe headaches are typically associated with significant autonomic symptoms such as ipsilateral conjunctival injection, tearing, rhinorrhea, eyelid swelling, facial sweating, meiosis, or ptosis. It is important to note that although there are few controlled trials, some TACs have been shown to be responsive to indomethacin when other treatments are not effective.17
Primary stabbing headache or ice-pick headache is another rare but important headache to recognize. These patients typically describe a severe, quick stabbing pain around the orbit, temple, and/or parietal regions that lasts for a few seconds and repeats irregularly, and an individual can experience a few stabs per week or many per hour. The symptoms may be difficult to differentiate from trigeminal neuralgia, but stabbing headaches are typically responsive to indomethacin whereas trigeminal neuralgia is not.17
Children with symptoms of TACs or primary stabbing headaches should have neuroimaging, as secondary causes of these symptoms have been described (although most will have normal scans).24
Treatment of Primary Headaches
Once serious secondary causes of headache have been excluded and one has been able to establish an appropriate primary headache diagnosis, the work of family education and headache management can begin. I have found that three steps are important for successful headache management: (1) education of the family and patient about primary headaches, (2) acceptance that there is no intracranial lesion or other underlying systemic disease, and (3) agreement of the patient, family, and provider on a final, multifaceted treatment strategy. A headache diary is often helpful to recognize headache triggers and patterns, and to assess treatment response. The four major domains of headache treatment include (1) lifestyle modifications, (2) abortive therapy, (3) complementary therapies and (4) preventive treatment. In my experience, many children will do well once they learn to recognize and avoid their headache triggers, and begin proper abortive therapy using an appropriate medication at the right dose at the onset of a migraine. However, those with chronic migraine often require more time and a multilayered approach using elements from all four treatment domains.
Routines that can aggravate headaches need to be investigated and modified when it seems likely they are contributing to pain and disability. Some of the most common triggers include inadequate sleep, poor nutrition or fasting, stress from school, family, or friends, poor hydration, and inappropriate or insufficient exercise.25,26 Thus, those with migraine should aim to achieve regular and adequate sleep, to eat regular and nutritious meals (including breakfast), to limit caffeine intake to fewer than three servings per week, to maintain good hydration, to appropriately manage stress, and to exercise regularly in appropriate amounts (Table 4). If there is concern for anxiety, depression, or other significant stress, consultation with a mental health provider is often beneficial as these issues must be addressed in conjunction with the headaches. If these issues are neglected it will be difficult to achieve reasonable headache control.9,25,27
SMART Lifestyle Changes to Promote Headache Health
Early intervention is an essential feature of the successful abortive treatment of migraine or tension headache, as treatment is most effective early in the course of the headache. At the onset of even a mild headache, the child should drink a glass of water and rest. Medications that don't require a prescription, such as naproxen, ibuprofen, and acetaminophen, are often effective for children with migraine (Table 5). For more severe headaches, nonsteroidal anti-inflammatory drugs (NSAIDs) may be combined with acetaminophen and/or caffeine as long as the child is not overusing caffeine. If NSAIDs are not effective, one may consider using triptans (5-HT1 receptor agonists) in appropriate circumstances. Although many different triptans are available for use in adults, only a few triptans have been approved by the US Food and Drug Administration (FDA) for migraine abortive therapy in children, but others have been studied.28 For younger children with migraines that are not responsive to over-the-counter medications, rizatriptan may be used, as it has been approved for children age 6 years and older. Almotriptan has been approved for use in adolescents, and a combination of sumatriptan and naproxen was recently approved for use in adolescents. There are data28 to support the use of zolmitriptan (oral and nasal) and sumatriptan (subcutaneous and nasal) in children age 12 years and older (Table 5). However, to prevent medication-overuse headaches, abortive therapies should be used no more than 2 to 3 days per week (<10–15 days per month for NSAIDs and <8 days per month for triptans or caffeine).23 Opiates and barbiturates should not be used for the management of childhood headache.29 These treatments can alter pain response at the cellular level, lowering the pain threshold and increasing the risk of chronification of pain over time, and both can lead to dependency and addiction.29
Abortive Therapies for Pediatric Migraine
For those with significant nausea and vomiting associated with migraine, antinausea medications can be used in conjunction with abortive medications to manage these symptoms. Studies30 have shown that intravenous prochlorperazine and metaclopromide are effective in the ED setting, and thus may be of use in the outpatient setting. However, given the risk of dystonic reaction, it is usually best to give diphenhydramine with these medications. They also provide an additional sedating treatment, which can be beneficial for patients who need to sleep to treat their migraines. I have found that ondansetron has a better side-effect profile than the options listed above, and can be used as a simple antiemetic, particularly if vomiting is disabling or interferes with the ingestion of other medications (Table 6).
Antinausea Medication Options in Pediatric Migraine
Complementary therapies play an important role in the management of chronic or recurrent episodic headache. Biobehavioral techniques that may improve headache disability include cognitive-behavioral therapy, biofeedback therapy, yoga, relaxation techniques, acupuncture, hypnosis. Cognitive-behavioral therapy has the most scientific evidence to support its use for the management of chronic migraine in adolescents, and should be discussed as an option for any patient with chronic migraine.31 In my experience, physical therapy may be beneficial for some patients, particularly if the child or teen has muscular pain that is related to headache or if she is deconditioned due to chronic pain and subsequent inactivity.
There has been consensus that when patients report 4 days or more of disabling headaches per month, one should consider initiation of daily preventive therapy. It is important to discuss expectations, as preventive therapies may take 6 to 12 weeks to become optimally effective, and the child with chronic headaches is unlikely to have an immediate cessation of their headaches. However, recent data from the Childhood and Adolescent Migraine Prevention (CHAMP) study,32 a National Institutes of Health-sponsored randomized, controlled study of topiramate versus amitriptyline versus placebo, found that all three treatments, including placebo, decreased headache frequency, but there was no significant difference in the percentage of children who had a 50% reduction in headache frequency (approximately 50%–60% in each group responded). There was also no difference in the change in headache days between the treatment groups, which dropped from 11 to 5 days per month during the study.32 This study raises question about how we should manage recurrent migraine in childhood. It may be reasonable to consider using these options as they were helpful for many children; however, if side effects develop, the offending treatment should be quickly discontinued. Cognitive-behavioral therapy has been shown to be effective for the management of chronic migraine and should be an important element of the management of chronic migraine,15 although it can be challenging to find accessible providers comfortable with this treatment.
Although there are only a small number of controlled trials of supplements for the management of headaches in children, the results from the CHAMP study32 may increase the use of supplements, as there are several supplements that may provide benefit and have a relatively short list of benign potential side effects. Riboflavin (vitamin B2) may be effective for the treatment of adult migraine with daily doses ranging from 25 to 400 mg/day.33 Magnesium has also been used for the management of migraine for both acute and preventive treatment. One pediatric study using 9 mg/kg per day of magnesium oxide found that those taking magnesium experienced a significant decrease in headache frequency, whereas those taking placebo did not.34 Coenzyme Q10 is another supplement that could be helpful for the management of migraine and has few side effects. Butterbur extract (Petasites hybridus) has been shown to be as effective as many prescription medications for the management of adult migraine.35 However, one needs to use a safe source of butterbur, as this plant naturally contains pyrrolizidine alkaloids that can cause liver injury if not removed, so using a reputable source is essential, and products made from unprocessed roots or leaves should not be ingested. Melatonin has been studied for the treatment of chronic daily headaches in teens36 and can help initiate sleep, so it may be particularly beneficial for teens with headache who suffer from insomnia (Table 7).
Selected Preventive Agents Used in Pediatric Migraine
Prescription Preventive Medications
Unfortunately, relatively few preventive medications have been studied in randomized, controlled studies in pediatric populations. In 2004, the AAN practice parameter37 concluded that flunarizine (not available in the US) is “probably effective,” but that there was “conflicting or insufficient data” regarding the use of other medications for the prevention of pediatric migraine. Since that time, several pharmaceutical industry-sponsored studies have found that daily use of topiramate is more effective than placebo for the prevention of migraine in children.38 However, the most rigorous study of preventive therapy for pediatric migraine, the CHAMP study, was unable to detect a difference between topiramate, amitriptyline or placebo.32 Thus, it seems likely that other factors, such as pill-taking behavior, extra fluids, placebo effect, or interaction with study personnel are important in the response of migraine to any treatment. Other medications that have been used for migraine include beta-blockers such as propranolol, and tricyclic antidepressants such as amitriptyline and nortriptyline. There are some open-label studies39 on the use of valproic acid for migraine prevention in children, but valproic acid is teratogenic so is not an optimal option for teenage girls. For younger children, a nightly dose of cyproheptadine may help to decrease migraine burden, and it has a relatively benign side-effect profile. Although this regimen has not been well studied, it has been used for many years. Gabapentin and verapamil have been used for prevention of pediatric migraine. Although there is little evidence that they are effective in pediatric patients, they may be of use certain clinical situations, such as a patient with migraine with family history of hemiplegic migraine, or a teen with migraines and restless leg syndrome or neuropathic pain40 (Table 7).
Most headache specialists believe that when preventive medications are used, the initial dose should be low and then increased slowly to a reasonable amount or to the maximum tolerated dose, whichever is higher, to minimize side effects and maximize the chance of treatment success.
Given the results of the CHAMP study,32 any significant side effects associated with treatment should prompt discontinuation of the medication. The choice of preventive medication is often based on comorbid factors. For example, low-dose propranolol could be an option for a slightly anxious girl who has postural orthostatic tachycardia syndrome, but shouldn't be used for patients with asthma or depression; amitriptyline may be a reasonable choice for a teen who has difficulty sleeping at night but would be contraindicated for a patient with prolonged QT syndrome; and topiramate might be a good choice for an overweight adolescent with chronic migraine but should not be an option for a thin adolescent girl with a history of anorexia nervosa. Once headaches are under control for 3 to 6 months, I advise most patients that they can try slowly tapering off the daily medication, but this plan will vary depending on pretreatment disability, adverse effects, and treatment efficacy.
Although most pediatric headaches are due to a primary headache syndrome or acute illness, we need to be able to detect the signs and symptoms of secondary headaches. If we fail to identify and treat the headache's cause, whether it is migraine, illness, brain tumor, intracranial hypertension, or serious systemic disease, then we may contribute to the burden of headache-related disability. In pediatric headache, a stepwise approach is essential to avoid missing secondary headaches, to make the correct diagnosis as soon as possible, and to develop a multilayered treatment approach. The stepwise approach I recommend is as follows:
History: obtain a complete history detailing headache history and characteristics, family and social history, and risk factors for other systemic illness, as well as symptoms or exposures that are associated with the headache.
Examination: complete a comprehensive physical and neurological examination with attention to abnormalities that could be associated with a secondary cause of headache.
Testing: neuroimaging and other testing only if there is a concern for secondary cause of headache on history or examination.
Management: create a multifaceted treatment plan with the child and family that is reasonable and suitable for the patient's headache and lifestyle and is endorsed by the patient and family.
Revise and revisit: new symptoms and reactions to treatment should prompt review of the initial plan and revision as appropriate for the situation.
I hope that these steps will provide a framework that will enable medical practitioners to provide confident and competent care for their pediatric patients with headache to alleviate the pain and disability caused by this common disorder.