Headache is a common condition in children and adolescents. Patients can be hospitalized primarily for headaches or develop headaches during their hospital stay. In either situation, it is important to determine whether the headache represents a primary headache disorder, such as migraine or tension headache, or a secondary disorder resulting from potentially serious intracranial or systemic processes (Table 119-1).

Epidemiologic studies in large cohorts of children reveal estimated 1-year prevalence rates of 6% to 20% for migraine and 11% to 18% for tension headache.^1-7 In general, post-pubertal girls have the highest prevalence rates of both migraine and tension headache. The most recent classification criteria established by the International Headache Society (IHS) in 2013⁸ provide a detailed classification of headaches in children.

Head pain (cephalgia) is a symptom, and the diagnosis of a headache disorder or migraine is a diagnosis of exclusion. Evaluation of headache consists chiefly of a detailed history and thorough physical examination. Any abnormalities on neurologic examination in a previously healthy child or changes from neurologic baseline in a child with previous abnormalities warrant expeditious evaluation. In the absence of worrisome symptoms and abnormalities in the physical (including neurologic) examination, the majority of headaches in children are not caused by serious pathology. The physician should consider the following key questions during the evaluation:

• 
  

  Are there any warning signs of symptoms suggesting increased intracranial pressure (ICP) or focal neurologic abnormalities?

  
  
• 
  

  Do the symptoms fit into the pattern of a typical headache syndrome?

  
  
• 
  

  Are there any predisposing factors to secondary headaches such as a ventriculoperitoneal (VP) shunt?

Although the majority of headaches in children do not represent life-threatening emergencies, the pain experienced by the child and the ensuing anxiety of the family can be great. Once a patient with headache is deemed stable and relevant secondary causes have been considered and ruled out, the focus should shift to providing symptomatic relief and reassurance.

The typical presentations of headaches caused by increased ICP, other secondary headaches, and common primary headache disorders will be reviewed. Familiarity with the particular symptoms or symptom combination for each of these categories is important in guiding differential diagnosis.

Although less common than primary headaches, secondary headaches are of greater immediate clinical concern, especially for the physician evaluating a child in the emergency department or the inpatient hospital setting.

HEADACHES ASSOCIATED WITH INCREASED INTRACRANIAL PRESSURE

Symptoms and signs that should alert the physician to the possibility of a headache secondary to increased ICP are reviewed in Table 119-2.

In general, a “first or worst” headache warrants immediate evaluation for the possibility of a mass lesion causing increased ICP, but further historical details are required to better assess the likelihood of this diagnosis.

A sudden “thunderclap” headache or the report of the “worst headache of my life” may suggest acute intracranial hemorrhage as the underlying diagnosis. These can be caused by either spontaneous aneurysmal rupture (rare) or arterial-venous malformation (more common). A severe headache in the setting of recent head trauma or in a patient with a bleeding diathesis may suggest acute or subacute hemorrhage into the epidural, subarachnoid, or subdural space.

Headaches caused by mass lesions and slowly increasing ICP are often worse in the morning or after a period of lying in the supine position. There may be associated vomiting, visual disturbance (e.g. blurring or enlargement of the blind spot caused by optic nerve swelling), or other complaints reflecting focal neurologic deficits. Children with brain tumors in the posterior fossa, which account for only a small minority of children with headache, may present with ataxia and other symptoms referable to the brainstem, such as diplopia and difficulty swallowing. A child with such symptoms will also most likely have corresponding abnormalities on neurologic examination. The presence of all three features of the Cushing triad (hypertension, bradycardia, and irregular respirations) is a late sign of increased ICP and represents impending coma or a comatose state.

Headaches from increased ICP should also be considered in several special circumstances as listed in Table 119-3.

These circumstances include a known bleeding diathesis or disseminated intravascular coagulation. Patients with a clotting tendency are at risk of developing cerebral venous sinus thrombosis. Among such patients are those factor V Leiden or antithrombin III deficiency and patients with secondary coagulopathies as a result of rheumatologic disease (e.g. systemic lupus erythematosus associated with lupus anticoagulant), inflammatory bowel disease, or cancer. Medications such as cyclosporine and methotrexate are associated with a posterior leukoencephalopathy syndrome presenting with headache, seizures, cortical blindness, and hallucinations.

Another important history detail is the presence of a VP shunt. Not every headache in a child with a VP shunt represents a neurosurgical emergency, but one must be suspicious of shunt malfunction and increased (or increasing) ICP. This is particularly true if the headache is severe, if it is a first or new type of headache for the child, or if there are any other symptoms or signs suggesting increased ICP. Of note, early in the process of VP shunt obstruction, accompanying abnormalities may include subtle mood changes or decreased energy. Papilledema and other signs of increased ICP may not be present in the first several hours of presentation. For all these reasons, headache in a patient with a VP shunt should be considered secondary to shunt obstruction until proved otherwise, unless it is a typical headache for a given child and there are no other worrisome features in the history or physical examination to suggest otherwise.

OTHER SECONDARY HEADACHE PRESENTATIONS

The history and physical examination assist in identifying secondary headaches not associated with increased ICP. For example, headache may be a presenting feature of meningitis, but there are typically other features, such as fever, to suggest this diagnosis.

Headache with or without neck pain in the setting of head or neck trauma, even relatively trivial trauma, can be caused by dissection of the carotid or vertebral arteries. Although less common in children than adults, this entity deserves consideration due to the risk of embolic stroke from the clot that forms in response to the dissection.

Secondary headaches may herald otherwise occult head and neck conditions, such as dental abscess, otitis media, or sinusitis. Pain may not be well localized in these conditions.

Other causes of secondary headaches, particularly in the inpatient setting, include metabolic disturbances (listed in Table 119-1 as disorders of homeostasis), intracranial hypotension (or a “low-pressure” or “post lumbar puncture” headache) caused by a cerebrospinal fluid (CSF) leak after lumbar puncture (LP), and opiate withdrawal in patients receiving analgesia for other conditions or for headache itself.

Pseudotumor cerebri, or benign idiopathic intracranial hypertension, is considered a secondary headache, although the underlying cause is often not determined. Patients with this condition have symptoms suggesting increased ICP. They may also report transient visual obscurations, a large blind spot or diplopia. Typical examination findings include papilledema and enlargement of the blind spot. Occasionally, weakness of the lateral rectus muscle is noted as a result of decreased ability to abduct the eye because of pressure on the sixth cranial nerves. Findings on computed tomography (CT) can be normal or small ventricles may be seen, and CSF opening pressure at LP is high. Often the patient experiences relief of symptoms transiently after LP. Although not a life-threatening condition, pseudotumor cerebri can lead to permanent loss of vision if not treated expeditiously.