Etiology
Studies of children presenting to the ER with headache have demonstrated that the most common etiologies include primary headache (mostly migraine and rarely tension-type) and sinusitis-related headache.[1][2][6] Primary headache disorders require exclusion of secondary headache etiologies.
In 6% to 15% of children a serious neurologic condition is diagnosed. These include viral or bacterial meningitis, ventriculoperitoneal shunt malfunction, neoplasms, intracranial hemorrhages (epidural, subdural, intraparenchymal), and pseudotumor cerebri (idiopathic intracranial hypertension). It is recommended that patients with neurologic etiologies are diagnosed by thorough history and examination, with judicial use of neuroimaging.[1][2][6][7]
Traumatic
Head trauma may lead to cerebral contusion, post concussion headache, and hemorrhage (e.g., parenchymal, subdural, epidural, and subarachnoid). Urgent evaluation for secondary causes of headache is generally indicated when there is a history of head trauma preceding the headache. A detailed neurologic examination is essential. If one of these secondary etiologies is suspected, urgent neuroimaging is indicated.
Vascular
Headache may be symptomatic of underlying vascular conditions including:
Migraine
Typically unilateral; moderate-to-severe intensity; often associated with nausea, vomiting, and visual disturbances
Dissection (carotid, vertebral, or intracranial arteries)
May be a history of head or neck injury
The onset of symptoms may be delayed
Intracranial hemorrhage
Subarachnoid (can occur due to aneurysm rupture or in the context of traumatic brain injury)
Parenchymal (may be related to vascular malformations or there may be history of trauma)
Ischemic stroke
Acute onset of headache with focal seizures and symptoms of elevated intracranial pressure (headache, vomiting, depressed consciousness)
Sinovenous thrombosis
Gradual onset of headache made worse by bending over or squatting, or while straining with bowel movements (Valsalva).
If secondary vascular etiology is suspected, urgent neuroimaging with a noncontrast head CT is indicated (blood appears hyperdense).[7]
Infectious
Intracranial infections include:
Encephalitis (can be secondary to common but preventable childhood infections such as measles, mumps, and rubella)
Meningitis (Streptococcus pneumoniae and Neisseria meningitidis are the most common causes of bacterial meningitis in children; viral meningitis is usually milder)
Dental caries, abscess, and gingival disease
Sinusitis. Tenderness to palpation, inflamed mucosa, and purulent nasal discharge may suggest sinusitis. May be accompanied by dental or ear pain.
Patients with headache and fever or neck stiffness should generally undergo a lumbar puncture, after neuroimaging is performed to rule out any intracranial lesions. If lumbar puncture cannot be performed rapidly, antibiotics and antivirals may be initiated before CSF is evaluated. Dental pain requires an urgent dental referral.
Neoplastic
Brain tumors may lead to intracranial pressure that develops gradually as the tumor enlarges, or abruptly if there is an intratumor hemorrhage (with concomitant hydrocephalus).
Often these patients present with chronically worsening headache that may be more severe when lying supine and in the morning.
Of children with brain tumors, 62% have headache prior to diagnosis, and 98% have at least one neurologic symptom or abnormality on examination.[4]
Head CT may identify large tumors, but MRI without and with contrast is the optimal imaging study.[7]
Common brain tumors in children are astrocytoma, medulloblastoma, and ependymoma.
Medication-associated
Medication overuse
Overuse of many non-prescription and prescription headache medications (e.g., ergotamine, triptans, analgesics, opioids, or a combination of these medications) may cause chronic headache.
With transformation to chronic headache, features of the initial underlying headache condition may be reduced or absent, so the history must include a description of the initial headache that led to medication use.
Identification and withdrawal of culprit medication is an important component of headache treatment.
Indomethacin-responsive headache
Some of the trigeminal autonomic cephalgia forms of primary headache are responsive to indomethacin.
All are characterized by headache and autonomic symptoms.
Paroxysmal hemicrania (unilateral, severe, boring headache lasting 20 minutes occurring 10 to 40 times per day with autonomic symptoms such as nasal congestion, lacrimation, and conjunctival injection).
Hemicrania continua (unilateral headache lasting hours or days associated with milder autonomic or migrainous symptoms).
Musculoskeletal
Muscle tension and joint abnormalities (e.g., temporomandibular joint disorders) may result in headache pain. The headache examination must identify the exact site of the pain. Tenderness and positional exacerbations are important clues for entrapments. Examination of the oropharynx and teeth and palpation of neck muscles is an important component of the physical examination.
Other
Other important primary etiologies to consider include:
Cluster headache
Patients present with a stabbing excruciating headache often at the orbit that lasts 15 to 180 minutes and occurs 1 to 10 times per day
Autonomic features are present, including lacrimation, conjunctival injection, nasal congestion, ptosis, and eyelid edema
MRI is indicated, as although rare, it may be symptomatic of a brain lesion in children
New daily persistent headache
Characterized by a headache lasting for more than 3 months that is unremitting and occurs daily from within 3 days of onset
May be secondary to underlying pathology, so MRI with contrast, sometimes also with MR venography, and CSF evaluation including opening pressure is important.
Other important secondary etiologies include:
Hypertensive encephalopathy
Patients may present with blood pressure elevation
Pituitary apoplexy
May present with abnormalities on eye movement and/or altered mental status
Ventriculoperitoneal shunt dysfunction
Children with ventriculoperitoneal shunts may experience shunt dysfunction (proximal or distal etiologies)
Requires urgent intervention, especially if intracranial hypertension is also suspected (optic nerve edema, CN6 palsy with horizontal diplopia, headache worse when supine)
Pseudotumor cerebri
Optic nerve edema or use of provoking medications should prompt consideration of pseudotumor cerebri (idiopathic intracranial hypertension), and lumbar puncture must include an opening pressure
Intermittent hydrocephalus
May occur when a mass lesion intermittently obstructs CSF flow, especially near the narrow structure of the ventricular system
If pressure remains elevated only briefly, optic nerve edema may not develop
Diagnosis is important because future obstructions (especially during sleep) might not self-resolve, and can rapidly cause intracranial hypertension due to obstructive hydrocephalus.
A detailed history is important in identifying these rare but important secondary causes of headache.
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