Headache

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8.6 Headache

Alistair Murray, John Ryan

Essentials

1 The primary role of an emergency physician is to exclude a significant underlying cause for a headache, most urgently that of infection, raised intracranial pressure or an intra-cerebral bleed.
2 While the majority of children with headaches presenting to the emergency department (ED) are likely to be benign, appropriate investigation and follow up of patients will ensure that serious causes are unlikely to be missed.
3 A thorough history and examination is likely to provide a diagnosis in the majority of children presenting to an ED with a headache.
4 Chronic headaches need not necessarily be investigated on an emergent basis unless they are progressive.
5 Red flags prompting further investigation of headache include: occipital headache; meningism; focal neurological signs; seizures; progressive nature; papilloedema; persistent vomiting; ataxia; presence of a ventriculoperitineal shunt; age younger than 3 years; or early morning headaches, especially those that wake the child from sleep.

Introduction

The aim of this chapter is to provide the reader with a basic understanding of the aetiology of headaches in a paediatric population presenting to an ED while offering a functional and safe approach to the management of paediatric patients with headaches. Some key points should be remembered from the outset:

• Children may require different approaches depending on factors such as age, ethnicity or socioeconomic status.
• Children under three years rarely complain of headache. This should prompt consideration of an organic cause.
• The classical history of migraine from a teenager may be more easily elicited than the history of symptoms from a shy 5-year-old.

Incidence

Approximately 1% of all presentations to emergency departments have headache as the presenting complaint.1,2. Headaches in children are very common with up to 75% of children having had a headache of some form by the age of fifteen.3 Despite the frequency, very few paediatric patients with headaches ever consult their family physician or an ED. However this does not take account of patients who present with a different complaint such as a temperature who might also have a headache as part of a concomitant illness.

Pathophysiology

The causes of headache are myriad, but the primary aim of the emergency physician should be to differentiate the patient with a headache which will run a relatively benign course, from that which may be a symptom of significant underlying pathology with immediate health implications.

The overwhelming majority of headaches will be diagnosed on history and examination alone, with little additional information arising from investigations.3–5. Furthermore, the vast majority of children that present to the emergency department with headaches are likely to be benign, but those that are not, have the potential to be life threatening.

The classification of headaches is based on the underlying aetiology.6 The International Headache Society has developed a classification of headache, the second edition of which was published in 2004 in Cephalgia and is also available on their website.7 This classifies headache into three broad categories most notably, primary or secondary headaches and cranial neuralgias central and primary facial pain and other headaches (Table 8.6.1).

Table 8.6.1 Summary of International Headache Society classification of headaches (ICHD-2)

Primary headaches Migraine Tension-type headache Cluster headaches and other trigeminal-autonomic cephalgias Other primary headaches Secondary headaches Headache attributed to head and/or neck trauma Headache attributed to cranial and/or cervical vascular disorders Headache attributed to non-vascular intracranial disorder Headache attributed to a substance or its withdrawal Headache attributed to infection Headache attributed to disorder of homeostasis Headache or facial pain attributed to disorder of cranium, neck, eyes, ears, nose, sinuses, teeth or other facial or cranial structures. Headache attributed to psychiatric disorder Cranial neuralgias, central and primary facial pain and other headaches Cranial neuralgias and central causes of facial pain Other headache, cranial neuralgia, central or primary facial pain

The causes of some headaches will be dealt with in other chapters, e.g. Chapter 8.7 on meningitis, while some of the primary headache disorders will be discussed in more detail later in this chapter. We recommend an approach whereby the emergency doctor approaches each case by initially excluding the most sinister causes of the headache (Tables 8.6.2 and 8.6.3).

Table 8.6.2 Causes of headache in children
Infection
• Meningitis, encephalitis, abscess
• Influenza
• Systemic infection
• Sinusitis
• Dental infection
Vascular
• Migraine
• Intracranial haemorrhage
• Hypertensive encephalopathy
Post lumbar puncture
Raised intracranial pressure
• Brain tumour
• Hydrocephalus
• Benign intracranial hypertension
• Trauma – SDH, concussion
Toxic
• Carbon monoxide
• Lead
Functional
• Tension or cluster headache
Psychogenic
Table 8.6.3 Important causes of non-benign headache
• Brain tumours and hydrocephalus
• Benign intracranial hypertension
• Intracranial haemorrhage
• Meningitis encephalitis or abscess
• Hypertensive encephalopathy

Clinical assessment

History

The first step in any medical assessment is the history and this is no less important in the case of headache. Depending on the age of the child, the history may be taken

Onset of the headache

Sudden onset headaches can be considered differently in children compared to adults. The classical history of sudden onset headache being suggestive of subarachnoid haemorrhage in an adult is less relevant in the case of the paediatric patient. In children, the most frequent underlying cause is an upper respiratory tract infection or primary headache.2–4 There is a significantly higher proportion of underlying pathology in cases of acute headache, compared to chronic headaches. It should also be noted that the investigation of headache of acute onset is more properly the role of the emergency physician, while chronic headaches may be best investigated by the child’s general practitioner or paediatrician.

Site

Classic migraine is typically unilateral. However, the case of childhood migraine is a little different, often being bilateral. The headache of meningeal irritation due to meningitis may involve neck pain. It should also be noted that occipital headaches are relatively uncommon in children and have been reported to be an independent predictor of underlying pathology, particularly of the posterior fossa.

Pyrexia

The presence of a fever will often be reported by a parent and should immediately raise the suspicion of an infectious origin. Other inflammatory disorders may also cause a pyrexia. Equally, the absence of pyrexia does not equal the absence of an infection. In the ED, a febrile child with a headache requires consideration of the possibility of meningitis or encephalitis.

Progression

The temporal progression is of relevance in children with headaches. For example, a classic migraine will last between 1 and 72 hours in a child, while a patient with a chronic headache that is becoming progressively more severe may well have an underlying organic cause. This should prompt the emergency physician who encounters a child with such a pattern, even if incidentally, to ensure that neuroimaging is performed and that urgent appropriate follow up is arranged.5

Nature

Headache of a throbbing nature is suggestive of migraine, while band-like headaches are often tension in type. It has also been suggested that the inability of a child to describe the nature of the headache may in itself be a predictor of underlying pathology.3

Behavioural change and avoidance behaviour

This is often noted in a collateral history. While entirely non-specific, it is particularly important in raising suspicion of other causes of a headache such as a school phobia, drug misuse in the adolescent or indeed may be a pointer towards sexual assault.8

Sleep disturbance

Benign causes of headache rarely wake patients from their sleep and if a child is being woken by a headache this should prompt the consideration of further investigation or referral.

Postural symptoms

A headache that is worse on lying flat or exacerbated by coughing may be related to raised intracranial pressure. Early morning headaches after recumbency of sleep may occur with raised intracranial pressure or space occupying lesions.

Neurological deficit

A history of neurological deficit, albeit temporary, should be considered highly significant and should always be sought. This is particularly important, as some children may have subtle objective neurological findings that could easily be overlooked in a hurried examination. Most mothers will have noticed an unusual posture or limp but may not immediately mention it unless prompted. The importance of this is re-inforced by the evidence that it may take an average of 7 months for a brain tumour to be diagnosed, with as many as three different consultations with a physician. This is despite the fact that a significant proportion of children with tumours have abnormal neurological examinations.9

Family history (especially of migraine)

Approximately 70–90% of diagnosed cases of migraine have a positive family history. Therefore diagnosing a migraine in the absence of a family history should be done with caution.

Analgesic use

Abolition of a headache with simple analgesics or restoration of normal activities is more common in those with upper respiratory tract associated headaches. It should also be noted that chronic daily use of analgesics is in itself a cause of headaches.10

Examination

A number of specific points should be considered when examining the child with a headache. Headache may be a secondary symptom of a more generalised illness. A comprehensive physical examination is therefore mandatory. Observation of a child from a distance may be valuable. A child lying quietly, unresponsive to the environment, should prompt earlier assessment than an active child who interacts normally with healthcare staff. One must also consider other causes of headache that are not readily discernible. In some cases there may be few clinical signs, such as with carbon monoxide poisoning from houses with poorly ventilated gas boilers.11 Heavy metal poisoning is another rare cause.

A focused examination should be directed on the basis of the history. Vital signs may provide valuable keys to the aetiology. A significant proportion of children who present with headache will have a diagnosis of respiratory tract infection, hence the importance of a thorough ear, nose and throat examination. Occasionally a child with tonsillitis will present with headache, without any complaint of a sore throat. Dental examination may reveal percussion tenderness of an infection that may cause a referred temporal headache. The presence of nasal discharge or respiratory symptoms should prompt the clinician to seek evidence of sinus tenderness. Likewise, it should be noted that, in most children, headaches due to uncomplicated upper respiratory tract infections can be expected to settle with simple analgesics. The persistence of headache should prompt the consideration of further investigation. The temperature should be recorded on more than one occasion. Likewise, it is important to measure the blood pressure as headache may be the presenting feature of coarctation of the aorta or underlying hypertension. The blood glucose is relevant if the history suggests hypoglycaemia. It is useful to measure head circumference and plot this on centile charts, even for the older child. A disproportionately large head should prompt further investigation5 and the tension should be palpated in infants who have an open anterior fontanelle.

A careful examination may reveal subtle evidence of trauma, which may or may not have been sustained accidentally. A mild post-concussion headache may persist for a number of days after a head injury, but may require imaging to exclude a possible subdural haematoma, particularly where the headache is severe, responds poorly to analgesia, is associated with vomiting or is prolonged. Headache has been retrospectively described as being more common in victims of sexual abuse. The febrile, toxic-appearing child should be examined for evidence of nuchal rigidity and other signs of meningeal irritation, which may be present in meningitis. In the child who has a ventriculoperitoneal shunt, the reservoir should be assessed by palpation (see Chapter 8.1). 8

Ophthalmological examination including fundoscopy is particularly important as it may influence immediate management. The presence or absence of papilloedema should be specifically sought as this may be the only objective finding in cases of benign intracranial hypertension (BIH). In this condition the computerised tomography (CT) scan may be normal and the diagnosis based on an elevated opening pressure >20 cmH2O on lumbar puncture. This valuable test is frequently neglected in lumbar punctures performed in the ED, but should be done if one is considering benign intracranial hypertension. The cerebrospinal fluid (CSF) protein, glucose and cell counts are normal in BIH. These children may present with intermittent headache, vomiting, blurred vision or diplopia.

Retinal haemorrhages should be sought on ophthalmoscopy. Their presence is considered as evidence of significant trauma, in the absence of any other causes such as hypertension or diabetes mellitus. It is difficult, if not at times impossible, to achieve successful examination of the fundi and retina of the younger child. Occasionally one may require mydriatic eye drops to dilate the pupils or seek the opinion of a suitably qualified senior colleague. A deferred formal retinal assessment by an ophthalmologist may be required in cases where the possibility of non-accidental injury exists. Eye movements must be carefully examined as subtle nerve palsies may be apparent early in children presenting with a space-occupying lesion or hydrocephalus.

The main consideration in a cardiovascular examination should be directed at the exclusion of hypertension and the palpation of femoral pulses, checking for evidence of coarctation of the aorta.

Abdominal examination is usually normal but is nevertheless important, as nausea, vomiting and abdominal pain are frequently associated with headache in children. It is recognised by the International Headache Society that ‘cyclical vomiting’ and ‘abdominal migraine’ are distinct entities and not infrequently progress to the typical pattern of migraine in adulthood. Hypertension of renal origin may be suggested by the presence of polycystic kidneys.

A standard neurological examination should be performed. Specific features that should be sought include papilloedema, ataxia, hemiparesis, abnormal eye movements and abnormal tendon reflexes.4

The child should be fully undressed. In particular, a rash or abnormality of pigmentation should specifically be sought. The most urgent of these is the search for the petechial rash of meningococcal disease, but examination may also reveal neurofibromas and café-au-lait spots associated with neurofibromatosis or a pigmented patch associated with tuberous sclerosis.

Investigation

The issue of how to investigate a child with a headache in the ED is not clearly defined and remains contentious. It is clear that in the majority of cases the cause of children presenting to the emergency department with a headache is a respiratory tract infection or primary headache syndrome. The key role of the emergency physician is to diagnose those headaches that may have significant underlying pathology. Investigation should be driven by the history and by findings on examination, as the majority of diagnoses should be suggested by these and confirmed by appropriate diagnostic tests.

CT scanning

Numerous studies have assessed the value of investigating a child with headache, with most focusing on the relevance of neuroimaging.3,12,13

A CT scan cannot be considered a benign test as it carries with it a risk in relation to the sedation and transfer of a child and with regard to radiation exposure. A CT head scan carries a 1:1500 risk of developing a subsequent cancer directly related to that radiation exposure.14

In the setting of an acute atraumatic headache, the absence of focal neurology or other ‘red flags’ (as listed later in this chapter), a CT scan is unlikely to be of significant value (Table 8.6.4).

Table 8.6.4 Red flags prompting further investigation of headache

Occipital headache Meningism Focal neurological signs Chronic progressive headaches Persistent vomiting Seizures Papilloedema Focal neurological symptoms Ataxia Presence of a VP shunt Age younger than 3 years Abnormal eye movements Early morning headaches especially those that wake the child from sleep.

VP, ventriculoperitoneal.

A guide to CT scanning in the context of trauma is considered separately.

In the context of a chronic, non-progressive headache with a normal neurological examination, CT is unlikely to be of value. This is different to a chronic progressive headache, which is a concerning feature and more likely to harbour underlying pathology. However, this artificially selects a cohort of patients that are unlikely to be seen in the emergency department. The investigation of chronic headaches is not the role of the emergency physician, and in the absence of an acute deterioration patients may be better served by redirection to their family physician or paediatrician.

Blood tests

Laboratory-based testing has a very low yield in children with headaches. Occasionally they can alert the clinician to more serious underlying pathology, but their value is diagnostically limited in the emergency setting. They should not delay the progression to more specific investigation and treatment. Indeed, with the increasing use of near patient testing much of the useful information in this regard can be obtained with the use of a venous blood gas, which provides information on pH, electrolytes, haemoglobin and even carboxyhaemoglobin levels.

Lumbar puncture

Lumbar puncture is mandatory in any case of suspected meningitis or encephalitis. It is a reassuring test when normal and often diagnostic when abnormal. It should be noted, however, that post lumbar puncture headache is also a recognised complication in children, as it is in adults.15 Such an invasive procedure may often be emotionally traumatic for a child and should not be ordinarily recommended as a routine investigation in the diagnosis of headache. A lumbar puncture with measurement of an elevated opening pressure is diagnostic in children with benign intracranial hypertension.

Other investigations

Magnetic resonance imaging (MRI) is an investigation that may show increasing utility in the future as its availability becomes more widespread. It has the advantage of no radiation dose and provides more detailed images of the brain.

Other investigations such as electroencephalogram, electromyogram, SPECT, positron emission tomography, autonomic testing and transcranial Doppler have not clearly demonstrated their utility in the acute setting.13

Management

Symptomatic treatment in the ED of most headaches in children can be achieved by use of oral analgesics such as paracetamol, codeine or ibuprofen. Headaches that require stronger analgesics to control pain are an indication for admission for further evaluation.

Disposition

The disposition of a patient from the ED should be considered on an individual basis, though some guidelines may be helpful in minimising the risk of missing a significant diagnosis. The increasing use of observation wards can prove helpful in selected cases. Such a facility is most useful in the case where an infective cause of the headache is suspected.

All patients who present to the ED with headache should have a follow-up appointment in the short term with either their family physician or a paediatrician, depending on each case. This will ensure that not only are high standards of care maintained, but unnecessary acute investigations can be avoided that would otherwise be ordered in order to exclude every possible cause of headache. Furthermore, there may be psychosocial aspects of relevance to childhood headaches that may be more appropriately dealt with by the family physician.

Migraine

Essentials

1 Family history of migraine is very common and a diagnosis of migraine in the absence of a family history should be carefully considered.
2 Unlike adult migraine, simple analgesics are often effective in the context of childhood migraine.
3 Childhood migraine is often bilateral.
4 Childhood migraine may be shorter-lived than in adults (1–72 hours).
5 Migraine is frontotemporal. Occipital headache suggests a sinister cause of headache.

Childhood migraine is a common condition presenting to the ED. The diagnosis is often based on an adequate history but the diagnosis can be a little more difficult to make in the younger age group. The emergency physician should not diagnose migraine in children less than 3 years old. Childhood migraine also has a wider spectrum of symptoms than adult migraine and should be considered in a patient who has recurrent abdominal pain, with normal investigations. It is important to remember that although the diagnosis of migraine is common, it is principally a diagnosis of exclusion.

Pathophysiology

The exact mechanism of migraine is complex and it has only recently become better understood. More recently, it is felt to be related to a hyperexcitable cerebral cortex, leading to cortical spreading depression (CSD), leading in turn to activation of the trigeminal nerve and its associated vessels. CSD can lead to areas of oligaemia of the cortex and may be responsible for the aura associated with migraine. Following this, the trigeminal nerve afferents appear to be sensitised and may account for the fact that ordinary activities can significantly exacerbate the migraine.16

It is also apparent that serotonin is integrally involved, hence the increasing popularity of the use of serotonin agonists such as sumatriptan in the acute treatment of adult migraine.

Clinical features

Migraine without aura is more common than migraine with aura in children.17 A number of key features are listed below:

International Headache Society diagnostic criteria of migraine without aura:7

A. History of at least five attacks, fulfilling criteria B–D
B. Last 1–72 hours (untreated or unsuccessfully treated)
C. Headache with at least two of the following:

Headaches pulsating in quality
Headaches may be unilateral or bilateral (unlike adult migraine which is typically unilateral), not occipital
Headaches exacerbated by routine activities (walking or climbing stairs)
The headache is moderate or severe

D. At least one of the following

Nausea and/or vomiting
Photophobia or phonophobia (may be inferred from child’s behaviour).

The presence of an occipital headache suggests a structural abnormality and not migraine.

In migraine with aura the headache is preceded by a period of altered perception that may take numerous forms. This period of altered perception can last up to 60 minutes. Examples are positive visual symptoms (flickering lights, spots, lines), negative visual symptoms (loss of vision, including partial), sensory symptoms (pins and needles) or fully reversible speech disturbances (dysphasia). This period of altered perception can last up to 60 minutes prior to the onset of the headache.7

Assessing the severity of headache in a child can prove challenging. Children almost universally point to the unhappiest face on a visual analogue scale when describing a headache. More reliable answers may be achieved by asking about quality of life issues such as ability to continue playing. Children who can continue playing during a headache appear to have a more benign cause to their headache.

Up to 90% of patients with migraine have a positive family history, although it is noteworthy that only one migraine gene has been identified and this is associated with only 50% of the cases of familial hemiplegic migraine, a particularly rare condition.5

A number of other conditions are considered to be migraine disorders in childhood, most notably cyclical vomiting and abdominal migraine. In these conditions there may be recurrent episodes of severe abdominal pain with associated vomiting but the examination and investigation of the children is often normal. These conditions are a diagnosis of exclusion, but their consideration may lead to appropriate referral and follow up as they carry significant morbidity.

Migraine variant disorders may cause transient abnormal neurological findings, such as hemiplegia, ophthalmoplegia or confusional state. These conditions are rare and an abnormal examination should prompt the emergency physician to investigate further.

Investigation

Migraine as a distinct entity has no specific diagnostic test. Key to the diagnosis is the history, as described above, with a normal neurological examination. Investigations should be requested on the basis of excluding other pathology as the underlying cause of headache. It is notable that although a significant number of migraine sufferers have abnormal EEGs, these add little diagnostic value and on current evidence should remain a research tool.13

Treatment

Most cases of childhood migraine can be adequately managed with simple analgesics such as paracetamol (15 mg kg−1) and ibuprofen (10 mg kg−1), although ibuprofen appears superior.18,19 As nausea can often be a significant feature, prochlorperazine appears to be of benefit.20

At present the triptan group of drugs remain unlicensed for paediatric use in many countries but there is now evidence for the use of nasal sumatriptan in adolescents and possibly even in those as young as 8.18,21 The evidence behind the use of other triptans, or routes of administration, is currently limited in the paediatric population.

There are very limited data regarding prophylactic treatment of migraine in children and hence no clear recommendations can be given, other than ensuring appropriate follow up of these cases.

Disposition

All patients with a discharge diagnosis of migraine should be followed up to ensure that the headache resolves within the expected timeframe and also to ensure that it does not progress. Migraine can carry significant impairment to lifestyle, if unrecognised. Follow up should be arranged with the primary care physician, with review of medication and parental understanding of the condition. Children with frequent migraine warrant referral to a paediatrician.

Conclusions

Patients with headaches commonly present to paediatric EDs. Those children presenting with new onset symptoms, significant neurological symptoms/signs or evidence of abnormal physiology should be rapidly assessed, with early referral for diagnostic facilities and neuroimaging being a priority. Children with chronic symptoms or recurrence of chronic symptoms should have a thorough examination and treatment. Follow up should be arranged with the patient’s paediatrician or general practitioner.

References

1 Conicella E.M.R.U., Vanacore N., Vigevano F., et al. The child with headache in a pediatric emergency department. Headache. 2008;48(7):1005-1011.

2 Burton L.J., Quinn B., Pratt-Cheney J.L., Pourani M. Headache etiology in a pediatric emergency department. Pediatr Emerg Care. 1997;13(1):1-4.

3 Kan L., Nagelberg J., Maytal J. Headaches in a pediatric emergency department: etiology, imaging, and treatment. Headache. 2000;40(1):25-29.

4 Lewis D.W., Qureshi F. Acute headache in children and adolescents presenting to the emergency department. Headache. 2000;40(3):200-203.

5 Lewis D.W. Headaches in children and adolescents. Am Fam Physician. 2002;65(4):625-632.

6 Society I.H. International Headache Classification (ICHD-2). Cephalalgia. 2004;24:1-160.

7 IHS. International Classification of Headache Disorders 2 (ICHD-2). 2006. http://ihs-classificationorg/en/

8 Golding J.M. Sexual assault history and headache: five general population studies. J Nerv Ment Dis. 1999;187(10):624-629.

9 Mehta V., Chapman A., McNeely P.D., et al. Latency between symptom onset and diagnosis of pediatric brain tumors: an Eastern Canadian geographic study. Neurosurgery. 2002;51(2):365-372. discussion 72–3

10 Vasconcellos E., Pina-Garza J.E., Millan E.J., Warner J.S. Analgesic rebound headache in children and adolescents. J Child Neurol. 1998;13(9):443-447.

11 Hampson N.B., Hampson L.A. Characteristics of headache associated with acute carbon monoxide poisoning. Headache. 2002;42(3):220-223.

12 Lewis D.W., Dorbad D. The utility of neuroimaging in the evaluation of children with migraine or chronic daily headache who have normal neurological examinations. Headache. 2000;40(8):629-632.

13 Sandrinia G.F.L., Jänigc W., Jensend R., et al. Neurophysiological tests and neuroimaging procedures in non-acute headache: guidelines and recommendations. Eur J Neurol. 2004;11:217-224.

14 Brenner D., Elliston C., Hall E., Berdon W. Estimated risks of radiation-induced fatal cancer from pediatric rCT. Am J Radiol. 2001;176:289-296.

15 Janssens E., Aerssens P., Alliet P., et al. Post-dural puncture headaches in children. A literature review. Eur J Pediatr. 2003;162(3):117-121.

16 Lewis D. Toward the definition of childhood migraine. Curr Opin Pediatr. 2004;16:628-636.

17 Bulloch B.T.M. Emergency department management of pediatric migraine. Pediatr Emerg Care. 2000;16(3):196-201.

18 Lewis D., Ashwal S., Hershey A., et al. Practice Parameter: Pharmacological treatment of migraine headache in children and adolescents. Report of the American Academy of Neurology Quality Standards Subcommittee and the Practice Committee of the Child Neurology Society. Neurology. 2004;63:2215-2224.

19 Lewis D.W., Kellstein D., Dahl G., et al. Children’s ibuprofen suspension for the acute treatment of pediatric migraine headache. Headache. 2002;42:780-786.

20 Bailey B., Cummins McManus B. Treatment of children with migraine in the Emergency Department. A qualitative systematic review. Pediatr Emerg Care. 2008;24(5):321-330.

21 Ahonen K., Hamalainen M., Rantala H., Hoppu K. Nasal sumatriptan is effective in the treatment of migraine attacks in children. Neurology. 2004;62:883-887.

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