Secondary Headaches in the Pediatric Population

David Symon

Mirja L. Hämäläinen

Many parents who bring their children to medical attention with a complaint of headache are not merely seeking relief of the troublesome symptom of pain. They are worried that their child may have some serious intracranial disease, and in particular they are often worried about the possibility of a brain tumor. In the majority of cases these worries are unfounded. Most serious secondary headaches do not present with headache alone but with additional symptoms and physical signs that make the nature of the problem quite clear.

Headache is a common symptom in children and about two thirds of children will report that they have had headache in the past year (2). In only one third of these cases is the headache severe enough to interfere with normal activities, and the majority of these children will have migraine (2). In a large epidemiologic study, clinical interview and examination of the children with severe headache did not reveal a single serious underlying disorder that may have been missed (2). Similarly, a hospital-based study looking at children presenting to an emergency service with headache showed no children with brain tumor or bacterial meningitis in whom headache was the presenting complaint (3). A hospital clinic series of 800 children and adolescents found that only three children (0.375%) had significant neurologic cause for their headaches, and only one child (0.125%) had an unexpected diagnosis of brain tumor (1). Despite this, headache can occasionally be one of the symptoms of serious underlying pathology, and all children who present with headache must be carefully evaluated. This applies even in children in whom the diagnosis seems obvious from the clinical history, as there may be unexpected clinical findings on examination.

CEREBRAL TUMOR

International Headache Society (IHS) code and diagnosis: 7.4 Headache attributed to intracranial neoplasm

World Health Organization (WHO) code: G44.822

Children and their parents seeking medical help for headaches are often worried about the possibility of a brain tumor being present. Intracranial tumors are the second most common type of neoplasm in childhood and the most common solid tumors. The incidence is 2.4 per 100,000 in children younger than 15 years (5). When a brain tumor causes a headache, it is usually secondary to increased intracranial pressure, which may be due to the mass itself or a result of obstruction of cerebrospinal fluid (CSF) flow. The headache pattern is usually chronic and progressive and increases in frequency and severity over time. Increased intracranial pressure may cause symptoms that can be confused with migraine (Chapter 43). The headaches tend to be worse in the morning and are often accompanied by vomiting. The pain is usually intermittent and in some patients may be throbbing, although it is usually dull and steady. The pain is exacerbated by coughing, straining, or lying down. Third- or sixth-nerve palsies may cause strabismus, but children may have difficulty describing diplopia and this may be confused with the visual aura of migraine. The majority of cerebral tumors in children are located in the posterior fossa and in these, dizziness is a common complaint. This too may be accepted as a manifestation of migraine.

It may be difficult to detect signs of a cerebral tumor if the patient presents with a very short history of headache. The diagnosis can be suspected in certain genetic
syndromes, such as neurofibromatosis, and in those who have symptoms other than headache alone, including seizures, ataxia, weakness, visual abnormalities, and personality change. Children with cerebral tumors may have psychologic changes including behavioral problems, depression, apathy, inattentiveness or other problems at school, and slowing of intellectual development. If the history is of any significant length, the neurologic examination is almost always abnormal. Chronic or frequent headache is present in 62% of children with brain tumors before their first hospitalization (5). However, when headache is a presenting complaint in a child with a brain tumor, the child is likely to have at least one other symptom and one or more neurologic signs regardless of tumor location. Less than 1% of children with headache and brain tumor have no other symptoms. More than half of children with headache and brain tumor exhibit five or more neurologic deficits (5).

Careful neurologic examination is therefore required and must include examination of the optic fundi and of eye movements as well as other cranial nerves and the motor system in general, with emphasis on coordination. Increased intracranial pressure may also be associated with systemic hypertension or with bradycardia.

As delayed diagnosis or missing a cerebral tumor may have serious consequences, any child presenting with headache who is found to have positive neurologic signs on clinical examination requires neuroimaging. It is probably better to overinvestigate if there is any suspicion of cerebral tumor (8).

BENIGN INTRACRANIAL HYPERTENSION (PSEUDOTUMOR CEREBRI)

IHS code and diagnosis: 7.1.1 Headache attributed to idiopathic intracranial hypertension (IIH)

WHO code: G44.820

This is a condition in which there is increased intracranial pressure that may mimic the symptoms of brain tumor but where there is no evidence of tumor. The anatomy of the ventricular system and CSF are normal. Benign intracranial hypertension may be associated rarely with a wide variety of diseases, but the most common association is with obesity (12). The condition is rare. An annual incidence of symptomatic disease of 0.9 per 100,000 children was reported from Nova Scotia with a 2.7-fold female preponderance (9).

The typical patient with benign intracranial hypertension is an alert, conscious patient without localized clinical signs but with clinical features of increased intracranial pressure. The most common symptom is headache, although this is not always present in every patient (7). Other neurologic symptoms may be present. The main clinical finding on examination is bilateral papilledema, but this may occasionally be absent (7,11).

Neuroimaging is mandatory in any child with headache and papilledema, and until this has been performed, the diagnosis cannot be adequately made. A lumbar puncture must be performed and CSF pressure should be in excess of 150 mm of water.

There have been no randomized controlled trials of management. Current management approaches are discussed in Chapter 114.

The prognosis of benign intracranial hypertension is variable, with some patients recovering spontaneously after only a few days while others continue to have symptoms for many years. The main complication is that a proportion of children will develop visual impairment.

HEADACHE SECONDARY TO INFECTION

IHS codes and diagnoses:

9.2.2 Headache attributed to systemic viral infection

(WHO code: G44.881)

9.1.2 Headache attributed to lymphocytic meningitis

(WHO code: G44.821)

9.1.1 Headache attributed to bacterial meningitis

(WHO code: G44.821)

9.1.3 Headache attributed to encephalitis

(WHO code: G44.821)

9.1.4 Headache attributed to brain abscess

(WHO code: G44.821)

11.5 Headache attributed to rhinosinusitis

(WHO code: G44.845)

11.6 Headache attributed to disorder of teeth, jaws, or related structures

(WHO code: G44.846)

Headache associated with fever is almost always associated with infection. Headache is a common feature of the febrile illnesses of childhood and most are not of serious significance. Headache is one of the principal symptoms of many viral infections and during outbreaks of these, the diagnosis is usually obvious. In other cases it is usually impossible to make a precise etiologic diagnosis, and investigation is seldom indicated as most of these infections are self-limiting.

Some of the infections may, however, be more severe. In one emergency department study 5% of children presenting with severe headache had viral meningitis. Meningitis is a major worry of parents bringing their children to the emergency service with acute headache, and although
headache is a feature of nearly all children with meningitis, they will usually have other signs of meningeal irritation. If these signs are found, a lumbar puncture is indicated to establish the diagnosis.

Tuberculous meningitis is rare, and in most instances the onset is insidious and symptoms are nonspecific. It is a complication of primary tuberculosis with or without miliary spread. On occasion, headache may initially be the only significant symptom, perhaps accompanied by slight mood swing. After 1 or 2 weeks further symptoms will become apparent.

Encephalitis is an inflammatory process of the cerebral parenchyma of infectious cause. Viruses are frequently the causal agents, and this is one of the most serious life-threatening diseases of children and adolescents. Most patients will complain of headache, but this is only one of a constellation of symptoms.

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