Approach
Headache may be primary (due to an underlying headache disorder) or secondary (symptomatic of an underlying systemic or neurological condition).[17]
The diagnosis of a primary headache disorder requires exclusion of secondary headache aetiologies.[18] Children with abrupt onset of headache, other neurological or medical problems, or focal symptoms or signs, are at higher risk for having a secondary headache aetiology.
All children presenting with headache require a thorough history and examination, with judicious use of neuroimaging.[1][2][5][7][9] Blood tests and lumbar puncture may be indicated dependent on clinical assessment.
Headache diary as diagnostic aid
Consider asking the patient (or their carer) to keep a detailed headache diary to assess the true frequency and description of primary headache.[3][10][19] The diary may also be useful for headache management, and determining whether treatment produces a quantitative change in headache occurrence.[20] A headache calendar supports follow up.[19] Note that psychological comorbidities may co-exist and are important to consider.
History
Time course
Recent-onset headache or progressively worsening headache: concerning for an acute symptomatic aetiology (e.g., intracranial lesion, subdural haematoma).
Abrupt-onset headache (thunderclap headache): concerning for an acute event (e.g., subarachnoid haemorrhage, parenchymal haemorrhage, sinovenous thrombosis, arterial dissection, pituitary apoplexy, intracranial hypotension, intermittent hydrocephalus).
Context
Onset with activity: concerning for intracranial haemorrhage.
Head or neck trauma: consider Intracranial haemorrhage or arterial dissection.
Recent back trauma: consider intracranial hypotension.
Localisation[10]
Site of pain: for example, tension headaches commonly result in mild, bilateral pain.
Prior headaches
Previous history of similar headache: suggests primary headache, unless there is evidence of progressive or intermittent elevated intracranial pressure, suggested by headache worsening when the patient remains in certain positions.
Headache changed in nature: patients with primary headache disorders can develop other acute aetiologies for headache that must be considered if this headache is different to the patient's typical recurring headaches.
Frequent headaches, or headaches of increasing frequency: worsening intracranial aetiology.
Provocation
Change in headache with a position change suggests intracranial hypertension or hypotension.
Worse when lying down: suggestive of intracranial hypertension.
Worse when standing up: suggestive of intracranial hypotension.
Worse following a bowel movement: suggestive of intracranial hypertension worsened with Valsalva.
Associated conditions or medical history[21]
Other neurological symptoms or signs: suggestive of a secondary aetiology of headache.
Visiovestibular symptoms following history of traumatic brain injury: balance disturbances, dizziness, or visual changes suggest post-traumatic headache.
Vision changes: suggestive of optic nerve oedema related to intracranial hypertension; diplopia due to focal lesions or elevated intracranial pressure; or visual strokes/transient ischaemic attacks.
Initial survey
Vital signs
Elevated blood pressure: suggests possible elevated intracranial pressure or hypertensive encephalopathy.
Raised temperature: possible infectious aetiology that may be intracranial or extracranial.
Cushing's triad (hypertension, bradycardia, and irregular breathing pattern): suggests elevated intracranial pressure.
Mental status
Abnormality suggests encephalopathic process.
Skin
Neurocutaneous lesions: hyper- or hypo-pigmented skin lesions, oro-buccal telangiectasias, axillary or inguinal freckles may suggest intracranial lesions.
Head and neck examination
Cranial and neck auscultation
Bruits suggest vascular malformation or dissection.
Sinuses
Tenderness to palpation, inflamed mucosa, and purulent nasal discharge may suggest sinusitis.
Temporomandibular joint (TMJ) dysfunction[22]
Tenderness to palpation at TMJ or temples.
Clicks at TMJ when opening and/or closing jaw.
Reduced jaw movement: maximal mandibular opening may be reduced to <35 mm.
Oral/dental examination
May reveal dental caries, gingival disease, or oral abscess that could cause head pain.
Meningeal signs
Meningismus, photophobia: meningeal inflammation due to inflammatory, infectious, or neoplastic disease.
Head circumference (in all children)
Abnormal rate of increase in head circumference suggests an intracranial lesion.
Neurological examination
A full neurological examination is required for all patients. Identifying abnormal signs suggests an underlying brain lesion and a secondary headache aetiology. Focal findings may suggest specific localisation of the lesion, based on the examination sign. Non-focal findings, such as optic nerve oedema or bilateral abducens (6th cranial nerve) nerve palsies, may be non-specific signs of elevated intracranial pressure. Neurological examination abnormalities generally suggest further evaluation is needed urgently, often beginning with a non-contrast head CT.
Cranial nerve (CN) II: optic nerve
Optic nerve oedema: suggests elevated intracranial pressure.
Visual fields reduction: consider elevated intracranial pressure.
Pupil function or asymmetry
An abnormally dilated pupil in a bright room: suggests herniation syndrome.
An abnormally constricted pupil in a dark room: suggests Horner's syndrome with a carotid artery dissection.
Dilated pupils with oculomotor palsy (eye gazes out and down): suggests CN III palsy and compressive lesions including acute herniation.
Extra-ocular movements
Lateralising nystagmus, disconjugate gaze, or limitations in horizontal or vertical gaze: suggest a brainstem lesion.
Eye gazing out and down combined with dilated pupil: suggests CN III palsy and possible compressive lesion including acute herniation.
CN IV trochlear nerve palsy
Presents with head tilt and chin tuck.
May occur with head trauma or brainstem lesions.
CN VI abducens nerve palsy
Presents with head turn or horizontal diplopia.
Suggests elevated intracranial pressure or brainstem lesions.
CN VIII vestibulocochlear nerve[21]
Deficits such as gaze instability, tandem gait, or hearing loss may occur with concussion.
Strength, tone, reflex, and sensory asymmetries and abnormalities
Suggests focal lesions.
Babinski sign
Abnormal plantar reflex with upgoing toes: suggests an upper motor neuron lesion in the brain or spinal cord.
Cerebellar signs
Suggest a posterior fossa lesion (i.e., tumour, cerebellitis).
Gait that is worse than baseline in any child, wide based (if older than a toddler), ataxic, or abnormal due to unilateral dysfunction: red flag for intracranial pathology.
Urgent neuroimaging
Most children with headache do not require urgent neuroimaging. Imaging yield in paediatric patients presenting with primary headache is typically low.[9]
Indications for urgent neuroimaging include:[9][24]
A very severe headache with acute onset (worst headache of one's life; e.g., thunderclap headache, primary cough headache)
New focal neurological signs
Meningismus
Optic nerve oedema
History suggestive of elevated intracranial pressure (rapidly worsening headache, vomiting, diplopia, optic nerve oedema if not acutely elevated)
Possible ventriculoperitoneal shunt infection or malfunction
Headache with known or possibly metastatic cancer
Headache in an immunocompromised patient
One systematic review identified several clinical features associated with the presence of significant intracranial abnormality that warrant neuroimaging:[25]
Cluster-type headache
Abnormal findings on neurological examination
Undefined headache
Headache with aura or vomiting
Headache aggravated by exertion or valsalva-like manoeuvres
Preferred neuroimaging modalities
If intracranial hypertension or hypotension is suspected, magnetic resonance imaging (MRI) is preferred over computed tomography (CT) unless MRI is not available or will lead to delay in care. MRI is also indicated to detect small tumours, vascular malformation, inflammatory changes, or abnormalities in the posterior fossa and cervical cord.
In thunderclap headache, or when haemorrhage is suspected, non-contrast head CT may be used for speed and sensitivity in detecting a bleed.[9] A normal neurological examination and a normal brain CT within 6 hours of headache onset are extremely sensitive in ruling out aneurysmal subarachnoid haemorrhage in a patient presenting with thunderclap headache.[13]
Non-urgent neuroimaging
Most children with headache do not require urgent neuroimaging. Imaging yield in paediatric patients presenting with primary headache is typically low.[9]
Indications for non-urgent neuroimaging include:
New onset of headaches
Chronic-progressive pattern
Unvarying headache location
Headache that awakens child from sleep or is present on awakening
Child who cannot provide a clear history
If a child presenting with recurrent headaches is otherwise well, a CT might be deferred to avoid radiation exposure and an MRI obtained as an outpatient. A practice parameter from the American Academy of Neurology addresses the evaluation of recurrent headache in children and adolescents.[26]
Lumbar puncture
If infection or elevated intracranial pressure is suspected, treatment should not be delayed in order to obtain a lumbar puncture.
Lumbar puncture should be considered for:
Acute onset of severe headache
Headache associated with fever and/or meningismus, altered mental status, or seizure
Immunocompromised patients
History suggestive of idiopathic intracranial hypertension (pseudotumour cerebri)
Accompanying studies
If elevated intracranial pressure is suspected, neuroimaging should be performed prior to lumbar puncture to avoid the rare but real possibility of herniation.
If high or low pressure is suspected, opening pressure should be measured with the patient in a relaxed lateral recumbent position.
If a subarachnoid bleed is suspected, examination of the centrifuged supernatant cerebrospinal fluid for xanthochromia (yellow colouration) is the most sensitive method and is best identified and quantified in the laboratory, rather than visually. Xanthochromia can persist up to several weeks following a subarachnoid haemorrhage. If this method is not available, sending tubes 1 and 4 for a cell count can also enable differentiation between true subarachnoid blood and blood from a traumatic tap.
Ancillary tests
Additional tests that may help narrow the differential further in specific cases include the following:
Sinovenous thrombosis: FBC, prothrombin time (PT), and activated partial thromboplastin time (APTT) to elucidate a clotting problem.
Ventriculoperitoneal shunt dysfunction: shunt series x-rays (imaging of the shunt in the neck, chest, and abdomen may reveal disconnection as the aetiology of malfunction); all patients with suspected ventriculoperitoneal shunt dysfunction should be referred for neurosurgical evaluation and possible shunt tapping.
Brain tumour: biopsy to confirm pathology.
Indomethacin-responsive headache: can include trigeminal-autonomic cephalgias, Valsalva-induced headaches, and primary stabbing headache (ice-pick headache or jabs and jolts syndrome). Paroxysmal and continuous hemicranias invariably respond in an absolute manner to indomethacin; Valsalva-induced and ice-pick headaches may respond in an equally dramatic, but less consistent fashion. Hypnic headache may also respond to indomethacin.[27]
Cluster headaches: therapeutic trial of oxygen supplementation; resolution of headache is diagnostic. Cluster headache is rare in children and may be symptomatic of a brain lesion, so MRI is indicated.
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