INTRODUCTION AND EPIDEMIOLOGY
Headache is pain in the scalp and cranium. Headaches in children can be mild, refractory, or life threatening, and can represent an acute, subacute, or chronic process. Sustained or recurrent headaches can greatly impact school performance and may even induce behavioral disturbances.1 Headache accounts for approximately 1% of all pediatric ED visits.2,3 Headaches increase in prevalence as a child ages; 30% to 60% of children through adolescence experience headaches.4,5 The most common causes of headache are viral and respiratory illnesses (28.5%),2,6 posttraumatic headache (20%), possible ventriculoperitoneal shunt malfunction (11.5%), and migraine (8.5%).3 Serious causes of headache are reported in 4% to 6.9% of children and include subdural hematoma, epidural hematoma, proven ventriculoperitoneal shunt malfunction, brain abscess, pseudotumor cerebri, and aseptic meningitis.2,3 Factors correlated with dangerous conditions include preschool age, recent onset of pain, occipital location, and the child’s inability to describe the quality of the headache. Emergent neurosurgical conditions in children with headache are generally predicted by the presence of focal neurologic signs.7
PATHOPHYSIOLOGY
The pathophysiology of headaches is complex and varies according to cause. The cranium, most of the overlying meninges, brain, ependymal lining, and choroid plexus do not possess pain receptors.6,8,9 Extracranial pain may arise from cervical nerve roots, cranial nerves, or extracranial arteries, and intracranial pain may arise from intracranial venous, arterial, or dural structures. Cranial nerve or root pain can radiate to the occiput, ear, retroauricular areas, or throat.9,10
Headaches are classified as primary or secondary based on the underlying cause. Primary headaches are physiologic or functional and are typically self-limited. They are often recurrent and are usually associated with normal findings on physical examination. Their diagnosis is typically based on recurrent symptoms, and they include migraine, tension, cluster, and chronic daily headaches. Migraine headaches are common and account for about 75% of primary pediatric headache disorders seen in the ED.7
Specific underlying causes are identifiable for secondary headaches (Table 136-1), which are usually, but not always, anatomic in nature. Causes include brain tumors, vascular malformations, and intracranial abscesses; craniofacial problems, such as sinusitis, dental abscesses, or otitis; systemic disorders, such as lupus cerebritis; and exposure to toxic substances, such as carbon monoxide, lead, or cocaine. Although primary headaches can be disabling, secondary headaches result in morbidity and mortality if not treated.
Historical Description | Physical Findings |
---|---|
Abrupt onset | Altered mental status |
First or worst ever | Septic or toxic appearance |
Posttraumatic | External evidence of head trauma |
Awakens from sleep | Bradycardia, hypertension, or irregular respirations |
Present with fever or a stiff neck | |
Aggravated by sneezing, coughing, Valsalva maneuver, lying down | Diaphoresis |
Facial herpes zoster | |
Vomiting and/or worsening pain in the morning | Petechiae |
Café au lait or ash-leaf spots | |
Altered mental status or focal neurologic symptoms | Asymmetry of pupillary response |
Ptosis | |
Change in behavior | Visual field defect |
Change in pattern (if chronic) or worsening over time | Retinal hemorrhage or optic disc distortion |
Toxic exposure | Asymmetry of motor or sensory responses |
Family history of subarachnoid hemorrhage | Thyromegaly |
Nuchal rigidity | |
Head tilt |
The International Headache Society provides one method for headache classification (http://ihs-classification.org/en/). Table 136-2 associates temporal patterns of headache with possible diagnoses.
Type | Temporal Pattern | Causes |
---|---|---|
Acute headache | Single episode of head pain without history of previous events | Upper respiratory tract infection, sinusitis, first migraine, medication use, trauma |
Acute recurrent headache | Pattern of head pain separated by symptom-free intervals | Migraine |
Chronic progressive headache | Gradual increase in frequency and severity; may be worse in the morning or awaken at night | Space-occupying lesion, hematoma, pseudotumor cerebri |
Chronic nonprogressive headache (chronic daily) | Frequent or constant headache | Tension headache, cluster headache |
Mixed headache | Acute recurrent headache superimposed on chronic daily background pattern; variant of chronic daily headache | Typically migraine (acute recurrent headache) superimposed on chronic daily headache |
HISTORICAL FEATURES
Obtain a thorough history from all possible sources (child, parents, and other caretakers available) in order to help identify or exclude more worrisome secondary causes of headache (Table 136-1). Enquire about the child’s personal and family medical histories. Elicit characteristic features of the headache. Obtain details as to whether the child or family has a history of headaches and whether the current headache is similar to past attacks.
The prevalence of migraine headaches increases with the child’s age, and a pattern of prior occurrences suggests primary headache.3,4,11 Although migraine headaches are unusual in preschool-age or younger children (<5 years old), they are becoming increasingly recognized in this young age group.12 Complex migraines (hemiplegic, confusional, or basilar type) may have their onset at a young age as well; however, incapacitating headache in a young child, especially when associated with vomiting or gait changes, suggests an intracranial mass with an infratentorial location being the most common location.13 Predictors of a surgical space-occupying lesion include headache of <6 months’ duration, sleep-related headache, vomiting, confusion, absence of visual symptoms, absence of family history of migraine, and abnormal findings on neurologic examination.14
Viral illnesses and fever are among the most common causes of headache in children, and the associated headaches are most frequently frontal or temporal.2,6,8 A history of trauma may suggest posttraumatic headache or traumatic brain injury. Posttraumatic headaches may be chronic as well.15 Migraines, more common and better studied in adults and adolescents, may be accompanied by premonitory symptoms (prodromes such as fatigue, mood changes, or GI symptoms) and have identifiable triggers.16 Children with prodromes tend to have more characteristic triggers as a whole, which may include specific foods (e.g., chocolate or monosodium glutamate), stress, light, specific odors, and weather changes.8,12 Headaches are among the most commonly reported symptoms in toxic exposures such as carbon monoxide poisoning.17 Additional precipitants include medications (e.g., methylphenidate, steroids, oral contraceptives, and anticonvulsants), infection (e.g., sinusitis, pharyngitis, or meningitis), hypertension, anemia, and substance abuse (e.g., cocaine).18
Headache coinciding with the onset of fever suggests inflammation of some sort, typically infectious (e.g., sinusitis, pharyngitis, otitis, or meningitis), or may be associated with a more general viral syndrome. Abrupt occurrence of severe headache due to a serious underlying condition, such as a brain tumor or intracranial hemorrhage, is typically associated with one or more objective findings on neurologic examination (e.g., altered mental status, ataxia, nuchal rigidity, papilledema, or hemiparesis).7,8 Cluster headaches also tend to develop acutely, whereas tension headaches have a more subacute onset. Hormonal cycles can trigger migraine headaches in adolescent females. Migraine headaches in children typically start relatively abruptly, intensify over several minutes, and then reach full intensity in about an hour.19 Young children often have headaches that begin in the late afternoon.
In a cohort of children with headaches presenting to a pediatric ED, only 27.5% of patients could identify a precise location of the pain. Among children with intracranial diseases, most either were unable to indicate the location of the pain or had an occipital headache.7,20 Medications, hypertension, and basilar-type migraines can also cause headaches in the occipital region. Pain at the vertex can be seen with sphenoid sinusitis. Ethmoid, maxillary, and frontal sinus infections tend to cause retro-orbital pain, as does meningitis (along with fever and neck stiffness) and dural sinus thrombosis. Pain seemingly in, around, or in front of the ear (or entire temporal region) is often seen with temporomandibular joint dysfunction and can be reproducible on exam. Migraine headaches are usually unilateral and involve the frontal or temporal region in adolescents. However, in younger children, they are usually bifrontal or generalized. Only about a third of children have unilateral migraines.19 Tension headaches tend to have the greatest variability in location. They may be generalized, frontal, or even occipital/posterior cervical. Occipital location in children is a red flag that should be investigated further before attributing to a primary headache disorder.
Younger children and many developmentally normal, otherwise healthy children may have a difficult time describing the quality of their headaches. An ability to describe pain quality or a description of the headache as having a pulsating quality is more frequently associated with benign headaches. An inability to describe the pain or a description of the headache as constrictive indicates a greater likelihood of a more serious cause.7,8,20 Many different qualities of pain can be identified: stabbing or hyperesthetic pain has been associated with herpes zoster; aching pain with tension headaches, meningitis, or encephalitis; and constant pain with sinusitis in all locations. A pulsating quality is one of the diagnostic criteria for migraine headache set forth by the International Headache Society but can also be seen with headaches caused by hypertension or intracerebral hemorrhage.21 The International Headache Society criteria for migraine were developed for the adult population, but children present differently. In particular children, may have shorter headaches and in different locations. Diagnostic criteria for pediatric migraine are presented in Tables 136-3 and 136-4.22 Although these diagnostic criteria rely on recurrent attacks, in an ED, a child may present with a first migraine headache due to intractable pain. One study investigated the utility of applying the International Headache Society criteria in the ED without the “recurrent” requirement (“Irma Criteria”) and found the criteria to be quite sensitive in diagnosing first-time migraines when followed long term using the original International Headache Society criteria as the gold standard.23
I | At least 5 attacks with features (II–IV) below |
II | Headache between 1 and 48 h |
III | At least 2 of the following: Bilateral or unilateral location (not to include posterior location) Pulsating Moderate-to-severe pain Made worse with activity |
IV | At least one associated symptom: Nausea/vomiting Photophobia/phonophobia |
The severity of a headache is neither a sensitive nor a specific characteristic in determining cause. Patients with tension headaches can complain of terrible pain, whereas a child with a brain tumor may complain of mild to moderate pain. Nonetheless, complaints of very intense pain should be taken seriously and assessed in context with other historical elements.7 Ask about and document presenting pain assessments in children with primary headache disorders because treatment end points will be dictated by improved pain scores in the ED.
Although the duration of a headache is not particularly useful in assessing the majority of headaches, the International Headache Society definition of migraines requires a duration of symptoms of 4 to 72 hours21 in adults, but the duration may be less (1 to 48 hours)22 in children (Tables 136-3 and 136-4). A migraine that lasts >72 hours is known as status migrainosus. Children can sometimes come to the ED with this condition and should be treated appropriately.
Patients with a sense of restlessness or agitation are more likely to have cluster headaches (more rare in children; Table 136-5).21 They may pace about the room or rock back and forth in a chair. In contrast, patients with migraines typically prefer silence and darkness because the lack of stimulation provides some relief, and photophobia/phonophobia are part of the diagnostic criteria for migraine headache (Table 136-3).21,22 Tension headaches can be frequent and frustrating but tend not