Headaches are common in children; while most are caused by a benign problem or primary headache disorder, headaches can be a sign of a serious underlying condition. Pediatricians must be aware of the most recent recommendations for evaluating and managing headaches.After reading this article, readers should be able to:Headaches are common in children and adolescents and are a frequent chief complaint in office and emergency department visits. The vast majority of childhood headaches are due to a primary headache disorder, such as migraine, or an acute, relatively benign process, such as viral infection. However, clinicians also need to consider other causes of headaches in children. Even when headaches are benign, they may cause significant dysfunction for the child and family and must be managed appropriately to minimize disability and optimize function. In this review, we discuss the epidemiology of childhood headache, evaluation of the child who has headaches, when to consider secondary headache syndromes, and the diagnosis and management of primary headache disorders such as migraine and tension-type headaches.Acute and chronic headaches are relatively common in children and adolescents, although estimates of the precise prevalence of headache and migraine vary widely. Depending on the study definition of headache, population involved, and time periods studied, 17% to 90% of children report headaches, with an overall prevalence of 58% reporting some form of headache in the past year. (1)(2) Headaches are slightly more common in young boys than girls (age <7 years), but around the time of puberty, this ratio begins to change. Although the prevalence of headache increases with age in both genders, the prevalence of headache increases much more sharply in girls until it reaches adult levels in late adolescence, when the prevalence of headache is significantly higher in women than in men. In adolescence, 27% of girls and 20% of boys describe frequent or severe headaches, and 8% of girls and 5% of boys have had a migraine in the past year. (1)(3) In adults, over 80% of women and 60% of men have had a headache, and 15% of women and 6% of men report having had a migraine in the past year. (1)(2)(4)(5)Although the majority of children who have headaches do not seek medical care, severe or recurrent acute headaches and chronic headaches are common causes of office and emergency department visits for families who are concerned about the cause of the child’s headache (often worried about a brain tumor) and are looking for ways to prevent or manage the headaches. Although serious secondary causes of headache are not common, it is important to ensure that there is no significant underlying disorder that is causing the headaches.Headaches can be divided into four basic patterns, the recognition of which facilitates the evaluation and diagnosis of the headache: 1) acute; 2) acute recurrent (or episodic); 3) chronic progressive; and 4) chronic nonprogressive (Fig). (6) A thorough history and examination are essential to classify and manage the headache appropriately. Acute recurrent and chronic nonprogressive headaches are most likely related to a primary headache disorder, although other secondary causes of headaches should be considered in the appropriate circumstances. Chronic progressive headaches are the most worrisome type of headache and deserve a thorough evaluation, most often including neuroimaging. A single acute headache is most often benign, usually triggered by an underlying primary headache disorder or viral infection; however, other serious disorders can cause acute severe headaches (Table 1). (7)Migraine is common in childhood, with a prevalence of 1% to 3% in children age 3 to 7 years and 8% to 23% in adolescence, (3) when migraine is much more common in girls than in boys. Migraine is also a common cause of acute headaches that lead to evaluation by a medical professional. The definition of migraine is outlined in Table 2; migraine typically is diagnosed via history and examinations without neuroimaging.Migraine headaches without aura are more common than those with aura, but both can affect children. It may be difficult to diagnose migraine in very young children because symptoms of vomiting or vertigo may be more prominent than headache. Migraine headache pain may be unilateral or bilateral in children, often is frontal or temporal, and typically is a pounding or pulsing pain. Exclusively occipital pain is unusual and should raise suspicions for another disorder.Migraine is a primary neurologic disorder. The pathophysiology of migraine is presumed to be the same in children and adults. It is believed that the mechanisms of migraine are based on complex interactions between the neural and vascular systems, including cortical spreading depression, abnormal neuronal excitability, serotonin activity, inflammatory response, and trigeminal neurovascular activation with signal transmission through the thalamus to the cortex. (8) Migraine is no longer believed to be a simple phenomenon of isolated vasoconstriction-triggering pain but a complex cascade of events. We know that mutations in a calcium channel gene (CACNA1A), a sodium/potassium pump (ATP1A2) gene, and a sodium channel (SCN1A) gene and mitochondrial dysfunction all can result in migraine. There are likely to be other genetic differences, yet to be revealed, that can alter neuronal or glial function and lead to the clinical syndrome of migraine. However, migraine is multifactorial; although migraine has a strong genetic component, the heritability pattern is not simple, the clinical manifestations can be different in various family members, and migraine has a very strong environmental component. Although migraines have been described for thousands of years and studied for over 50 years, the exact pathophysiologic mechanisms of migraine remain unknown.Migraine headaches in children typically are shorter than adult migraine attacks and may last only 30 to 60 minutes. Children often seek a quiet dark place to go during an attack due to phonophobia or photophobia. Anorexia, nausea, and vomiting also are common during a migraine attack. Sleep often relieves the headache.Other symptoms that may be associated with a migraine attack include dizziness, blurry vision, difficulty reading, stomach pain, flushing, sweating, pallor, and dark circles around the eyes. Children may have difficulty describing the pain or associated symptoms; asking the child to draw his or her headache can help to define the headache. A family history of migraine or headaches is common, although the pattern of headaches may be different in other family members. Common triggers for a migraine attack include stress, “let-up” from stress, fatigue and poor sleep, illness, fasting, and dehydration. An obvious food trigger is not common but may be a factor for some children who experience migraine.Most children do not have aura with migraine, and many of those who do have aura sometimes have headaches without aura. Auras usually occur less than 30 minutes before the headache and last only 5 to 20 minutes. A typical visual aura may consist of scotomata, transient blurry vision, zig-zag lines, or scintillations, but more complex visual changes such as those seen in Alice in Wonderland syndrome (visual distortions that include sensation that objects are bigger or smaller than they are; objects appear to be moving when they are still; or objects have shattered like glass) can occur. Other types of aura also occur, including sensory changes (numbness or tingling), confusion, weakness, amnesia, or aphasia. These symptoms are notable, but the onset of symptoms in migraine typically occurs over a longer time period than the symptoms of stroke or seizure. The symptoms of aura are completely reversible, usually last <30 minutes, and often are recurrent over time. Patients who have neurologic symptoms that are prolonged, not related to headache, or not completely reversible should have an evaluation to rule out other underlying conditions.Less common subtypes of migraine include basilar, confusional, and hemiplegic. In basilar migraine, the aura is characterized by vertigo, ataxia, nystagmus, dysarthria, tinnitus/hyperacusis, bilateral paresthesias, diplopia, or visual disturbance. The aura can be unilateral or bilateral but does not involve motor weakness; the accompanying headache often is occipital.Confusional migraine is characterized by altered mental status, often accompanied by aphasia or impaired speech and followed by a headache. This state can be triggered by relatively mild head trauma, and the initial episode warrants a complete evaluation to rule out other disorders and intoxication. Hemiplegic migraine is a rare migraine variant that can be familial or sporadic and is characterized by prolonged hemiplegia, numbness, aphasia, and confusion. Genetic testing is available to identify mutations in three genes (CACN1A, ATP1A2, and SCN1A) that have been associated with familial hemiplegic migraine.Tension-type headache probably is the most common type of headache in childhood but generally is less disabling than migraine. In contrast to migraine, the pain is mild to moderate, may last for 1 hour or for several days, and often is described as “band-like,” pressure, or tightening. Triggers may be similar to migraine and include stress, fatigue, and illness but also include muscle pain and tension, particularly in the neck and shoulders.Tension headaches may be episodic (<15 days per month) or chronic (≥15 days per month). A thorough history to identify stressors, depression, or other factors associated with these headaches is important. Little is known about the pathophysiology of tension-type headaches. Some believe that nociceptive input from cranial/cervical myofascial components triggers these headaches initially, and if this noxious input is sustained, central sensitization can occur, so that an individual becomes more sensitive to these impulses and develops chronic headaches.Chronic daily headache is defined as ≥15 headache days per month. There are three major categories of chronic daily headaches in children: chronic migraine, chronic tension-type headaches, and new daily persistent headache (NDPH).Chronic or transformed migraine is not uncommon in adolescents. These children typically have a history of episodic migraine that becomes more and more frequent until they have more than 15 days per month of headache. Often they have few or no headache-free days. Typically, the serious migraine symptoms that were associated initially with the headache, such as vomiting and severe head pain or aura, diminish somewhat as the headaches become more frequent, although patients still may have “spikes” of severe head pain at times.Chronic tension-type headaches may share similarities with chronic migraines once the headaches become daily, and it can be difficult to classify daily headaches as tension or migraine. However, those who have tension-type headaches should not have a history of episodic migraine. Preventive treatment should be considered when the child is having 4 or more days of disabling headache per month. It is also critical to address lifestyle issues such as inadequate or irregular sleep, stress, inadequate or inappropriate food or caffeine intake, inadequate exercise, and poor hydration. Depression and anxiety are common issues for patients who have chronic headaches and may contribute to the headache.NDPH is characterized by the occurrence of a new headache that becomes daily within 3 days of onset and is not caused by another disorder. Given the abrupt onset of this headache, children who have this type of headache should have an evaluation for secondary disorders. NDPH often is triggered by a viral illness but may be caused by mild head trauma or surgery, or a trigger may be absent.Trigeminal autonomic cephalalgias (TACs) are rare in children. The treatments for migraine or tension headaches may not be effective for TACs, and recognition of these headaches is therefore important. This diagnostic group includes cluster headaches, paroxysmal hemicranias, and SUNCT (short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing). These paroxysmal headaches typically are accompanied by autonomic symptoms, such as ipsilateral eye redness, tearing, nasal congestion, rhinorrhea, eyelid swelling, forehead or facial sweating, miosis, or ptosis. Another rare headache that is important to recognize is primary stabbing headache. These patients have stabbing pain in the first division of the trigeminal nerve (orbit, temple, and parietal area) that lasts for a few seconds and recurs in an irregular pattern. Given that secondary causes of TACs and stabbing headaches have been reported, children who have these symptoms should undergo neuroimaging, although most will have normal scan results.Elevated intracranial pressure (ICP) is an uncommon but important cause of pediatric headaches and has various causes. Hydrocephalus may result from a space-occupying lesion, blockage of cerebrospinal fluid (CSF) flow via aqueductal stenosis, or impaired CSF absorption. Increasing the volume of tissue or fluids in the cranial vault (eg, mass lesions, edema, inflammation, hemorrhage) also can lead to a dramatic increase in ICP.Headaches are the most common presenting symptom of elevated ICP. Typically, these headaches are progressive, may cause nighttime wakening, and are worse with the Valsalva maneuver or exertion. Children who have elevated ICP often experience persistent vomiting, neurologic deficits, lethargy, or personality change. Other signs of elevated ICP include papilledema and palsies of the third, fourth, or sixth cranial nerves, resulting in eye movement or pupillary abnormalities.Low ICP also can cause headaches. Intracranial hypotension should be considered if there is a risk for CSF leak (eg, spinal surgery, trauma, connective tissue disease). Meningeal enhancement on brain magnetic resonance imaging may be seen with intracranial hypotension.Idiopathic intracranial hypertension (IIH), sometimes called pseudotumor cerebri, is elevated ICP without evidence of a specific cause. Daily headache is the most common symptom of IIH and may be associated with nausea and vomiting and other migrainous features, but the headache often is poorly characterized. Classic symptoms of IIH include transient obscuration of vision, tinnitus, and diplopia due to cranial nerve dysfunction. In young children, the most common complaints are headache, stiff neck, strabismus, irritability, apathy, somnolence, dizziness, and ataxia.Children are more likely to have an underlying condition associated with IIH than adults (Table 3); a thorough evaluation in addition to accurate assessment of opening pressure is thus essential when IIH is suspected in a child. The patterns of IIH in adolescence are similar to adult patterns; more female patients than male patients are affected, and obesity is associated with IIH. However, in younger children, when IIH is less common, the genders are affected equally, and obesity is not strongly associated with IIH.Acute viral illness with fever is the most common cause of pediatric headache evaluated in the emergency department. (9) Typically, these children will have an acute onset of headache, and the headache resolves as the other viral symptoms dissipate. Many other systemic infections can be associated with headache, including streptococcal pharyngitis, sepsis, Lyme disease, Bartonella infection, rickettsial diseases, and human immunodeficiency virus infection, but headache rarely is the only symptom. Viral infection, particularly with Epstein-Barr virus, can be associated with the onset of NDPH.Although sinusitis may cause or trigger headaches in some children, the majority of patients diagnosed as having “sinus headaches” have some form of primary headache syndrome. Sinus-related pain generally is pressure-like and dull periorbital pain, worse in the morning, associated with nasal congestion, and lasts for days at a time. It is not associated with nausea, visual changes, phonophobia, or photophobia.Meningitis or encephalitis are the causes of acute headaches in 2% to 9% of children evaluated for headache in the emergency department. (9) Headaches due to meningitis or encephalitis often are associated with photophobia, nausea, vomiting, and pain with eye movements. These patients typically also have symptoms such as fever, altered mental status, and nuchal rigidity, although fungal meningitis may be more indolent.Although families and patients worry that a brain tumor is causing the headaches, tumors are uncommon causes of headache in children. Tumors and other space-occupying lesions, such as large arachnoid cysts or vascular malformations, can cause headache via hydrocephalus, mass effect, or hemorrhage. One should consider a space-occupying lesion if the child has “red flag” symptoms noted in the following discussion (Table 4) or if the child has a history of exposure to ionizing radiation or a syndrome (such as tuberous sclerosis or neurofibromatosis) that is associated with tumors.Chiari I malformation, characterized by the herniation of the cerebellar tonsils >5 mm below the foramen magnum, may cause headache. The classic symptoms include occipital headaches, cough headaches or syncope, sensory disturbance, weakness, ataxia, vertigo, or other cranial nerve dysfunction. Confirming that headaches are due to a Chiari malformation can be challenging because more than 30% of patients with Chiari I malformation on magnetic resonance imaging are asymptomatic, radiologic findings often do not correlate with clinical symptoms, and other causes of headache not related to Chiari malformations are common.Spontaneous intracranial hemorrhage (ICH) and ischemic stroke are rare causes of headache in children. Although an acute “thunderclap” headache is the classic presenting symptom of ICH, most children who have ICH or ischemic stroke have additional signs or symptoms by the time they present to medical care. ICH should be considered in patients who have an acute onset of severe headache, particularly if the patient has an abnormal result on neurologic examination or a disorder that places him or her at risk for hemorrhage.In ischemic stroke, neurologic symptoms come on abruptly and persist, typically do not progress from one side of the body to the other, and typically are not recurrent. In contrast, the symptoms of migraine aura usually last less than 30 minutes, may involve both sides of the body or progress from one side of the body to the other, and often are recurrent over months to years.Sinus venous thrombosis (SVT) is another uncommon cause of secondary headaches in children. The most common presenting symptoms of SVT in children are headache, focal neurologic signs, seizures, decreased level of consciousness, and papilledema. The vast majority have some risk factor for SVT, including head or neck infection, chronic systemic disease, or other prothrombotic state. Thus, SVT should be considered in patients who have headaches and other neurologic symptoms, particularly in those who have underlying conditions that place them at risk for thrombosis.Children who have severe or progressive headache or altered mental status after head injury should be evaluated emergently. Headaches after a head injury may be due to traumatic ICH or fracture but more commonly are due to posttraumatic headache without significant structural injury. One study concluded that children older than age 2 years who have normal mental status, no signs of skull fracture, no loss of consciousness, no vomiting, nonsevere mechanism of injury, and nonsevere headache do not need a computed tomography scan after head trauma. (10)Posttraumatic headaches develop within 1 week of head trauma, concussion, or whiplash. These headaches may have qualities of migraine or tension headaches and often are associated with other postconcussive symptoms, including sleep disturbance, balance abnormalities, cognitive changes, and mood changes. The vast majority of posttraumatic headaches resolve within 2 weeks.Children and teenagers who have posttraumatic headache (indeed, all who have sustained a concussion) should not return to sports or vigorous exercise until they are symptom-free at rest and while active and have been cleared by a trained medical provider. Once symptom-free, they should return to their regular activities in a step-wise fashion, as outlined in the 2009 Zurich Consensus Statement on Concussion in Sport. (11) Adolescents who have chronic posttraumatic headaches may benefit from returning to low-level “subthreshold” exercise (aerobic exercise that does not trigger worsening of symptoms), with supervision by a trained medical provider. (12)There are many substances that can cause headaches, including overuse or withdrawal of caffeine, alcohol use, illicit drug use, carbon monoxide poisoning, and lead toxicity. Medications also can trigger headaches due to the medication’s primary mechanism of action, an idiosyncratic response to the medication, or medication withdrawal. Overuse of medications to treat headaches, especially analgesics, caffeine, opioids, ergotamines, and 5-hydroxytryptamine 1 (5-HT1) receptor agonists (ie, the triptans), is associated with transformation from episodic to chronic headaches. Some medications that have been associated with headache are listed in Table 5. (13)Fasting is a relatively common cause of headaches in children. Eating disorders also can trigger headaches but may be concealed by the patient. Hypothyroidism can cause headache and should be considered when evaluating for refractory headaches. Hypercapnia and hypoxia occur together in sleep apnea or hypopnea due to neuromuscular disease and are associated with headache. A sleep study may be indicated in a patient who has morning headaches and symptoms suggestive of sleep apnea.Peri-ictal headaches are common in children who have epilepsy. Typically, the association between the headache and seizure will be obvious. However, some seizures are characterized by episodes of altered mental status or visual disturbances followed by headache, which may be confused with migraine. The visual hallucinations in epilepsy typically are colored and rounded objects, rather than the jagged or scintillating impressions seen in migraine aura.Headache and migraine are frequent findings in mitochondrial disease. However, children who have significant mitochondrial disease typically also have problems with other organ systems and additional neurologic symptoms.Temporomandibular disorders have been associated with migraine, tension-type headache, and chronic daily headache. Malocclusion of the jaw and other dental problems also can cause headache.Children who have sickle cell disease are at risk for multiple serious causes of headache, including ischemic stroke, intracerebral hemorrhage, thrombosis, and chronic anemia. However, they also may have migraine or tension headaches, and young children who have sickle cell disease are more likely to have idiopathic headaches than age-matched controls. (14)Acute, severe headaches may be the initial sign of a hypertensive crisis. However, mild to moderate hypertension typically does not cause significant headaches.Children who have rheumatologic disease often have headache. The causes of headache in rheumatologic disease can include aseptic meningitis, intracranial hypertension, SVT, vasculitis, intracerebral hemorrhage, ischemia, or headache without underlying pathology. Immunosuppressive agents and nonsteroidal anti-inflammatory drugs (NSAIDs) used to treat rheumatologic disorders also can cause headaches. Thus, children who have diagnosed or suspected rheumatologic disease and headache should have a thorough evaluation for secondary causes of headache.Although depression and anxiety disorders are common in patients who have headaches, psychiatric disease usually is an exacerbating rather than a causative factor. Screening for depression or anxiety is beneficial because successful treatment of the headache will be difficult if psychiatric issues remain unaddressed. Headaches should be attributed to somatization disorder, psychotic disorder, major depressive disorder, or anxiety if those symptoms are prominent and if the headaches remit with treatment of the psychiatric disorder.The patient’s history is the single most important factor in the evaluation of headache. Both the patient and the parents should be included in this discussion to gather a complete picture of the patient and his or her symptoms. The history should include characterization of the headache:Adapted from Rothner AD. The evaluation of headaches in children and adolescents. Semin Pediatr Neurol. 1995;2(2):109–118.It is important also to obtain a detailed medical history because headaches may be associated with systemic illnesses and medications. For example, one would have a higher suspicion for secondary headache in a patient who has neurofibromatosis who is at risk for central nervous system tumors, or in a patient who started minocycline just before headache onset and is at risk for intracranial hypertension. Review of other symptoms related or seemingly unrelated to headache is important because they may suggest systemic disease as a secondary cause of the headache (eg, a malar rash suggestive of systemic lupus erythematosus, cold intolerance and skin changes suggestive of hypothyroidism, a history of episodic torticollis or vertigo as a young child consistent with migraine precursors).Information regarding any family history of headaches, pain, and other medical disorders is important because migraine has a strong genetic component and other disorders related to headache may also run in the family. It is important to ask about family history of any type of headaches because other forms may be mentioned by the family when “migraine” is denied. It is helpful to get information about headaches in grandparents, siblings, aunts, and uncles because migraines may skip the parent (often the father) and manifest in the child (often the daughter), and the headache pattern often is different in different family members.Discussion of social history also is critical because stressors at home, at school, or with friends can trigger or exacerbate headaches. A private conversation with an adolescent often is helpful because the teenager may not wish to discuss some issues in a parent’s presence. This dialogue might include questions about conflict with friends or family, sexual activity, pregnancy, drugs, cutting behavior, physical or sexual abuse, bullying, depression, family finances, alcohol use, or eating disorders, all of which may affect headache.Lifestyle factors often affect a headache pattern; it is therefore important to ask about sleep, diet, exercise, caffeine intake, and other activities. A headache diary can help to identify headache triggers or patterns. Inadequate sleep, poor hydration, and poor food choices are common, particularly in teenagers, and these factors often exacerbate or trigger headaches. Children and teenagers who have chronic headaches frequently do not get enough exercise, and regular appropriate exercise is an essential part of a headache management plan (Table 6). Caffeine intake more than 2 to 3 days per week may be a cause of rebound or medication overuse headaches. Clinicians should be aware of the presence of caffeine in soda and energy drinks.The evaluation should include measurement of heart rate, blood pressure, weight, and height in the context of a thorough physical examination looking for signs of systemic disease or focal findings that could be related to headaches. This procedure should include palpation of the face, neck, and shoulders, looking for nuchal rigidity, muscular or bony tenderness, trigger points, or allodynia (abnormal pain sensation with light touch, often associated with migraine); skin examination looking for signs of systemic disease, cutting behavior, or neurocutaneous syndromes; and oral evaluation looking for signs of dental disease.A thorough neurological examination is essential to look for abnormalities in mental status, vision, eye movements, speech, sensation, strength, reflexes, gait, or coordination, particularly noting any focal abnormalities, significant asymmetries, or cranial nerve palsies. Funduscopic examination looking for evidence of papilledema, optic atrophy, or other abnormalities must be included in the examination.Several risk factors are associated with an intracranial space–occupying lesion in children who have headache, including sleep-related headache, absence of family history of migraine, headache <6 months’ duration, confusion, abnormal neurologic findings, lack of visual aura symptoms, and vomiting. Children who have more risk factors have a higher risk of having a brain lesion requiring surgery. (15) Other worrisome symptoms include headache associated with cough, urination, or defecation; recurrent and focal headache; exclusively occipital headache; change in headache type; and progressive increase in headache frequency or severity. Table 4 lists factors associated with serious secondary headache.One should consider neuroimaging to rule out a structural intracranial lesion if the child has symptoms noted here. Although there are no guidelines regarding the risks of serious secondary causes of headaches in very young children (less than age 3-5), because of the possibility of secondary headaches, neuroimaging should be considered thoughtfully in these young children who have significant recurrent headaches.Brain magnetic resonance imaging is the modality of choice to investigate potential structural abnormalities, infection, inflammation, and
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Dec 1, 2023
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