Emergency department approach to nontraumatic headache in children

INTRODUCTION — This topic will discuss the emergency department (ED) approach to nontraumatic headache in otherwise healthy children.

Children with nontraumatic headaches who present non-urgently and those with well-established histories of migraine or tension-type headaches are discussed separately. (See "Headache in children: Approach to evaluation and general management strategies" and "Pathophysiology, clinical features, and diagnosis of migraine in children" and "Tension-type headache in children".)

EPIDEMIOLOGY — Headache is the most common neurologic complaint in the pediatric population and a common presenting complaint in the emergency department (ED). The prevalence of headache in children is increasing, as are ED visits and hospitalizations [1-4]. Caretakers may bring children to the ED or a primary care provider for headaches that are more severe or have not responded to nonprescription medications. In the pediatric ED, nontraumatic headaches account for up to 2.5 percent of visits [5-7].

CLASSIFICATION — The terms "primary" and "secondary" are used to describe headache etiology:

●Primary headaches – Primary headaches comprise 90 percent of all pediatric headaches [8] (ie, migraine, tension-type, or cluster headaches), are typically self-limited, and are diagnosed based on symptom profiles and patterns of headache attacks (table 1).

●Secondary headaches – Secondary headaches have identifiable underlying conditions. Although the vast majority of secondary headaches in children have benign etiologies (eg, acute febrile viral illness), the goal of the emergency evaluation of children with headaches is to identify as a first priority those with serious or life-threatening causes such as central nervous system infection, brain tumor, intracranial hemorrhage, or severe hypertension [8-12]. In most patients, this task can be accomplished with a careful history and physical examination and selected ancillary testing. The prevalence of serious or life-threatening intracranial conditions in children presenting to the ED with nontraumatic headache ranges from 1 to 10 percent, depending upon the clinical findings [13,14].

Headache can be the symptom of life-threatening complications for children with various underlying conditions (eg, those with indolent, progressive, or unsuspected head trauma; immunodeficiencies who develop opportunistic infections; or patients with ventriculoperitoneal shunts that malfunction, causing obstruction). Evaluation of headache in these children is often guided by specific protocols that include neuroimaging and consultation with a specialist (eg, neurosurgeon, infectious disease specialist, or oncologist). (See "Hydrocephalus in children: Management and prognosis", section on 'Shunt complications' and "Pathogenesis, clinical manifestations, and diagnosis of brain abscess".)

CAUSES — The most important consideration in the evaluation is to differentiate between primary and secondary headache. The challenge for the emergency clinician is to identify patients with secondary headaches who require rapid diagnosis and treatment (table 2). The majority of children who undergo emergency evaluations for headaches are diagnosed with viral illnesses or migraine headaches [5,6,15,16]. This epidemiology is similar to the causes of headache among children seen in primary care settings. (See "Headache in children: Approach to evaluation and general management strategies".)

Life-threatening conditions — Life-threatening causes of headache are those that may result in brain injury or death from various mechanisms including brain tumor, inflammation, increased intracranial pressure (ICP), and/or hypoxia.

Infection — Children with life-threatening infections as the cause of headache typically have other worrisome clinical features, such as altered mental status or focal neurologic findings. These manifestations may be subtle in younger children who cannot describe their symptoms.

●Bacterial meningitis – The irritable or lethargic child with fever, neck pain/stiffness, and/or headache may have bacterial meningitis. The incidence of bacterial meningitis among children has declined since the introduction of conjugate vaccines against Haemophilus influenza type B and Streptococcus pneumoniae. (See "Bacterial meningitis in children older than one month: Clinical features and diagnosis", section on 'Clinical features'.)

●Other non-viral meningitis – The differential diagnosis of headache is not complete without consideration of rare causes of meningitis:

•Tuberculous meningitis occurs in patients who have or have had tuberculosis and should be considered in the context of a history of possible exposure to Mycobacterium tuberculosis and signs of meningitis, including fever, headache, and nuchal rigidity. (See "Central nervous system tuberculosis: An overview".)

•Fungal meningitis is considered in the immunocompromised host who presents with signs of meningitis, including headache. (See "Candida infections of the central nervous system" and "Coccidioidal meningitis" and "Epidemiology, clinical manifestations, and diagnosis of Cryptococcus neoformans meningoencephalitis in patients with HIV".)

●Viral encephalitis – Progressive symptoms of fever, headache, and abrupt onset of altered sensorium (eg, bizarre behavior, visual or auditory hallucinations) are associated with viral encephalitis. In otherwise healthy children, herpes simplex virus causes 10 to 20 percent of sporadic cases of encephalitis and is the only treatable pathogen in this condition. Infection with specific arthropod-borne viruses may be suggested by seasonal patterns (eg, Eastern equine encephalitis or West Nile virus) or recent travel. (See "Herpes simplex virus type 1 encephalitis" and "Arthropod-borne encephalitides".)

●Orbital or cerebral abscess – Children with focal infections, such as orbital or cerebral abscesses, typically have focal findings on ocular and/or neurologic examination. Conditions that may be associated with central nervous system abscess formation include immunocompromise and congenital heart disease with right-to-left intracardiac shunting. Cerebral abscess and cerebral venous thrombosis are uncommon but life-threatening complications of orbital, sinus, ear, or dental infections [17,18]. (See "Orbital cellulitis" and "Bacterial meningitis in children: Neurologic complications" and "Cerebral venous thrombosis: Etiology, clinical features, and diagnosis", section on 'Risk factors and associated conditions'.)

Tumor — Chronic progressive headache (increasing in frequency and severity over time) is a common presenting symptom among children with brain tumors. Nocturnal or morning headache, especially when associated with vomiting, is also prevalent in children with brain tumors (see "Brain tumor headache", section on 'Clinical features'). However, very few children evaluated in the ED for headache have brain tumors as the cause. In a large multicenter pediatric series describing children with brain tumors, 62 percent of patients had headaches at the time of diagnosis [19]. In comparison, in a small prospective series, less than 3 percent of children presenting to the ED with headaches had newly diagnosed brain tumors [15].

The early symptoms of brain tumors are often nonspecific, and diagnosis may be delayed [20]. At the time of diagnosis, the overwhelming majority of children with brain tumors as the cause have other symptoms in addition to headache [21]. Over 99 percent of children in the multicenter report and all of the children in the ED series had at least one other symptom or sign (eg, nausea/vomiting, visual disturbance, ataxia, or abnormal eye movements) [15,19].

Subarachnoid and intracranial hemorrhage — Nontraumatic intracranial hemorrhage (typically subarachnoid) can develop in association with an aneurysm, vascular malformation, coagulopathy, or hemoglobinopathy. The patient may report the abrupt onset of headache, which reaches maximal intensity in less than one minute. In one retrospective series that described children with nontraumatic intracranial hemorrhage, more than half presented with headache and vomiting [22]. Frequent signs included hemiparesis and seizures. Among patients with sickle cell disease, intracranial hemorrhage accounts for about one-third of cerebrovascular events. (See "Acute stroke (ischemic and hemorrhagic) in children and adults with sickle cell disease".)

Intracranial hemorrhage is an uncommon but life-threatening cause of headache among children. Headache is rarely the sole manifestation of hemorrhage that occurs as the result of trauma (ie, epidural or subdural hematoma). (See "Severe traumatic brain injury (TBI) in children: Initial evaluation and management" and "Child abuse: Evaluation and diagnosis of abusive head trauma in infants and children".)

Acute obstructive hydrocephalus — Acute obstructive hydrocephalus causes an abrupt rise in intracranial pressure with one or more of the following findings:

●Headache

●Vomiting

●Lethargy or coma

●Hypertension with bradycardia or tachycardia

●Signs of transtentorial herniation (table 3)

Acute obstructive hydrocephalus may occur in patients with central nervous system (CNS) infection, space-occupying lesions such as tumor or hemorrhage, hydrocephalus, and cerebrospinal fluid shunt malfunction (see "Hydrocephalus in children: Management and prognosis", section on 'Shunt malfunction'), and, less commonly, as a presenting feature of selected brain malformations (eg, Chiari type I or Dandy-Walker malformation). (See "Chiari malformations" and "Prenatal diagnosis of CNS anomalies other than neural tube defects and ventriculomegaly", section on 'Dandy-Walker malformation'.)

Carbon monoxide poisoning — Symptoms of mild to moderate carbon monoxide (CO) poisoning are nonspecific but typically include headache [23]. Children may develop CO poisoning from occult sources, such as improperly vented home heating systems, space heaters, or automobile exhaust fumes. (See "Carbon monoxide poisoning".)

Hypertensive urgency or emergency — Patients with hypertensive crisis may present with headache as one manifestation of hypertensive encephalopathy. Other symptoms include visual changes from retinal hemorrhage or exudates, altered mental status, and seizures. Hypertensive encephalopathy is uncommon among children and is typically caused by systemic illness or renovascular disease. Pheochromocytoma is an exceptionally rare pediatric tumor that usually presents with headache, pallor, diaphoresis, and hypertension. (See "Approach to hypertensive emergencies and urgencies in children", section on 'Initial stabilization' and "Pheochromocytoma and paraganglioma in children".)

Common conditions

Infection — Among children evaluated in the ED for headache, infection is the most common cause [5,15,16].

●Fever – Children with fever (related almost entirely to infection) frequently complain of headache. Headaches associated with fever are thought to be due to vasodilation [24].

●Viral meningitis – Headache, with or without neck discomfort, is a typical presentation of viral meningitis among older children and adolescents. Other clinical manifestations (eg, rash or mouth lesions with enterovirus) may suggest a specific virus. (See "Viral meningitis in children: Clinical features and diagnosis", section on 'Clinical features'.)

●Lyme meningitis – In endemic regions (figure 1), Lyme meningitis is an important diagnostic consideration in children with headache. The presentation of Lyme meningitis is similar to that of aseptic meningitis. Erythema migrans (by history or examination), cranial nerve palsy, and papilledema (from increased ICP) are strongly associated with Lyme meningitis but uncommon in patients with viral meningitis. (See "Lyme disease: Clinical manifestations in children", section on 'Meningitis'.)

●Pharyngitis – Headache, sore throat, and abdominal pain are the classic symptoms of streptococcal pharyngitis. Influenza virus infection can cause similar symptoms, including headache. (See "Group A streptococcal tonsillopharyngitis in children and adolescents: Clinical features and diagnosis", section on 'Clinical features'.)

●Other – Otitis media, sinusitis, and dental infections can cause headache. Localized pain and signs of inflammation suggest the specific diagnosis. (See "Acute bacterial rhinosinusitis in children: Clinical features and diagnosis", section on 'Complications' and "Acute otitis media in children: Clinical manifestations and diagnosis", section on 'Clinical presentation'.)

Migraine — In several observational series describing children evaluated in EDs for acute headache, migraine was second only to viral illness as the cause [2,3,15,16,25]. Migraine headaches are typically recurrent and episodic, with characteristic patterns that are easily described by patients or parents/caregivers (table 1 and table 4). A family history of migraine or headaches is common. Children with significant changes in the quality, severity, or timing of headaches should be carefully evaluated for other causes. (See "Pathophysiology, clinical features, and diagnosis of migraine in children".)

Tension-type headache — Tension-type headache is the term designated by the International Headache Society to describe what were previously called tension, muscle-contraction, stress, or psychogenic headaches. Tension-type headaches occur in 10 to 25 percent of schoolchildren and adolescents, making them as common as migraine headaches [26]. Among children, they appear to be associated with depression, oromandibular dysfunction, and muscle tension due to stress [27].

In contrast with headaches related to brain tumors, pain is typically intermittent and recurring (but nonprogressive), and the neurologic examination is unremarkable (table 5). (See "Tension-type headache in children", section on 'Differential diagnosis'.)

Other conditions

Temporomandibular joint dysfunction — Temporomandibular joint (TMJ) dysfunction occurs in children with primary and mixed dentition. Children typically report increased pain while chewing and have point tenderness over the mandibular condyle [28]. Headache occurs more frequently among older children [29,30].

Idiopathic intracranial hypertension — Patients with idiopathic intracranial hypertension (IIH) have papilledema, increased ICP with normal cerebrospinal fluid (CSF) content, normal neuroimaging, the absence of neurologic signs except cranial nerve VI palsy, and no known cause. Headache is the most common chief complaint among children who are old enough to describe their symptoms. Although IIH has been described in young children, most patients are adolescents and typically female. (See "Idiopathic intracranial hypertension (pseudotumor cerebri): Clinical features and diagnosis".)

Trigeminal autonomic cephalalgia (eg, cluster headache) — Cluster headaches make up the most common trigeminal autonomic cephalalgia. This group of headaches is characterized by trigeminal location and association with autonomic features. Cluster headaches are typically unilateral and frontal-periorbital in location (table 6). The pain of cluster headaches is severe and lasts less than three hours, but multiple headaches occur in a very short period of time (hence "cluster"). Cluster headaches usually are associated with ipsilateral autonomic findings, which may include one or more of the following: lacrimation, rhinorrhea, ophthalmic injection, and occasionally Horner syndrome (ipsilateral miosis, ptosis, and facial anhidrosis).

Cluster headaches have been reported in children as young as three years of age, but they are rare in children younger than 10 years of age and uncommon in older patients. They become more apparent between the ages of 10 and 20 years, although they remain infrequent. Cluster headaches are discussed separately. (See "Cluster headache: Epidemiology, clinical features, and diagnosis", section on 'Clinical features'.)

Visual refractive errors — Although a rare cause of headache, visual refractive errors (anisometropia, myopia, and hyperopia) can cause chronic, fronto-orbital headaches that progress throughout the day in children [31,32]. Optical correction results in resolution in most cases. However, visual refractive errors remain a diagnosis of exclusion for chronic headaches in children; more urgent evaluation of signs or symptoms suggesting increased intracranial pressure should not be delayed while waiting for an ophthalmologic consultation.

Vision screening and detection of visual refractive errors in children are discussed in greater detail separately. (See "Vision screening and assessment in infants and children" and "Refractive errors in children".)

EVALUATION — Patients with underlying conditions, such as ventriculoperitoneal shunts or immunodeficiencies, who develop headaches must receive thorough evaluations in accordance with specialized protocols developed for those conditions. For most other children receiving emergency evaluation for acute headache, the combination of history and physical findings, with selective laboratory testing and neuroimaging, generally identifies those patients with significant underlying conditions (eg, meningitis or intracranial pathology) [8,15,16,25].

The remainder of this discussion will focus on the emergency evaluation specific to children with headache, including identification of those patients with serious causes of headache.

Stabilization — Rapid recognition of life-threatening causes of nontraumatic headache is important because these patients often require stabilization and rapid treatment of the underlying cause as described in the following algorithms and/or rapid overview tables:

●Intracranial hypertension (algorithm 1) (see "Elevated intracranial pressure (ICP) in children: Management")

●Bacterial meningitis (table 7) (see "Bacterial meningitis in children older than one month: Treatment and prognosis")

●Carbon monoxide poisoning (table 8 and algorithm 2) (see "Carbon monoxide poisoning")

●Hypertensive urgency or emergency (algorithm 3 and algorithm 4) (see "Initial management of hypertensive emergencies and urgencies in children")

History — An accurate history is an essential tool in the initial approach to nontraumatic headache in children.

Patient age — Headaches in children younger than six years of age are more likely to be caused by an underlying condition, most commonly a febrile viral illness.

Headache characteristics — Characteristics of the headache that may help to identify a cause include the following:

●Mode of onset – The abrupt onset of an extremely painful headache (thunderclap, "worst headache of my life") may represent an intracranial hemorrhage (eg, from an arteriovenous malformation or aneurysm). However, when this symptom occurs in isolation (ie, without neck pain or stiffness), it is nonspecific [13]. Thunderclap headache is rare in children, accounting for <1 percent of pediatric emergency department (ED) presentations for headache in one retrospective study [33]. (See 'Subarachnoid and intracranial hemorrhage' above and "Overview of thunderclap headache".)

In addition, precipitation or worsening of the headache with a Valsalva maneuver or exercise has been proposed as a potential symptom of increased intracranial pressure [8].

●Timing – Tension-type headaches typically develop late in the day and rarely cause a patient to awaken from sleep.

●Positional – A positional headache is one that presents or worsens upon lying down, awakens the child from sleep, or occurs soon after awakening in the morning [13]. A positional headache should raise concern about increased intracranial pressure or a space-occupying brain lesion, especially if it is accompanied by morning vomiting.

●Severity/quality – Descriptions from children of the severity and quality of headache pain may not be as useful for identifying a cause as those obtained from older patients. This was demonstrated in one prospective study describing children evaluated in an ED for headache in which 98 percent of children selected the most severe rating on a pain scale to describe the severity of their headaches [3]. In this study, children with upper respiratory infections were more likely to be able to describe the quality of headache pain than those with brain tumors and ventriculoperitoneal shunt malfunctions.

When able to describe the headache, the following characteristics are helpful in identifying an etiology:

•Laterality – Unilaterality suggests migraine headache, although in children under age 10 years, the pain in this condition may be bilateral. Also, unilateral headaches occur with focal infections, such as sinusitis.

•Location – Cluster headaches are usually temporal or retro-orbital in location. Pain may also localize to specific regions in secondary headaches, such as with sinusitis or dental abscess.

●Pattern of previous headaches – The following temporal headache patterns often suggest a specific diagnosis:

•Acute – This pattern describes a single episode of headache without a history of previous events. Acute headaches are typically secondary headaches, often caused by febrile viral infections.

•Acute recurrent – These headaches typically have consistent characteristics that are easily described by patients or parents/caregivers. They are usually primary headaches (ie, migraine or tension-type) (table 5) (see 'Common conditions' above). Children with significant changes in the quality, severity, or timing of headaches should be carefully evaluated for other causes.

•Chronic progressive – Chronic progressive headaches increase in frequency and severity over time. This is a worrisome pattern associated with space-occupying lesions such as brain tumors, abscesses, or hemorrhage. (See 'Life-threatening conditions' above.)

•Chronic nonprogressive – Frequent persistent headaches that have not changed in character are usually tension-type headaches.

Associated symptoms — The following additional historical features may suggest a specific diagnosis:

●Fever – The majority of children who are evaluated in EDs for headache have some type of infection (viral upper respiratory infections, sinusitis, or streptococcal pharyngitis) [15,16].

●Neck pain or altered mental status – Meningeal signs and/or an abrupt change in mental status suggest subarachnoid hemorrhage (typically with abrupt onset of "worst headache of my life") or, when accompanied by fever, life-threatening infections such as meningitis or encephalitis. (See 'Life-threatening conditions' above.)

●Localized pain – Children with localized pain may have specific infections, such as pharyngitis, otitis media, sinusitis, or dental infections. (See 'Common conditions' above.)

●Symptoms associated with brain tumors – The features of brain tumor headache are generally nonspecific and vary widely with tumor location, size, and rate of growth. The headache is usually bilateral but can be on the side of the tumor. Supratentorial tumors impinging on structures innervated by the ophthalmic division of the trigeminal nerve may produce a frontotemporal headache, while posterior fossa tumors compressing the glossopharyngeal and vagus nerves can cause occipitonuchal pain. There is generally little radiation of pain, except in posterior fossa tumors. Among children with headache who have brain tumors, additional symptoms are almost always present at the time of diagnosis, and funduscopic or neurologic examination is often abnormal. In addition to a chronic progressive pattern, the following symptoms suggest the possibility of an intracranial mass, such as a brain tumor (see "Brain tumor headache", section on 'Clinical features'):

•Headache pain that wakes the patient from sleep or occurs on waking in the morning

•Association with vomiting, especially progressive vomiting

•Behavioral changes

•Polydipsia/polyuria and/or visual field deficits (suggestive of craniopharyngioma)

•Other neurologic symptoms, such as ataxia, change in coordination, "clumsiness," blurred vision, or diplopia

●Trauma – Intracranial hemorrhage may cause headache for a child who has had a head injury, although this history may be absent in cases of abusive head trauma, especially in children younger than five years of age. (See 'Subarachnoid and intracranial hemorrhage' above.)

●Environmental exposure – Abrupt onset of headache and nausea in several members of one household (or headache and syncope in a child) may be the result of carbon monoxide (CO) poisoning. (See 'Carbon monoxide poisoning' above.)

●Change in visual acuity – Patients with idiopathic intracranial hypertension (IIH) may report a deterioration in visual acuity along with severe, unrelenting headache. (See "Elevated intracranial pressure (ICP) in children: Clinical manifestations and diagnosis", section on 'Clinical manifestations'.)

●Autonomic symptoms – Sweating or other autonomic symptoms may accompany cluster headaches or malignant hypertension caused by a pheochromocytoma. (See "Cluster headache: Epidemiology, clinical features, and diagnosis", section on 'Clinical features' and "Pheochromocytoma and paraganglioma in children", section on 'Clinical presentation'.)

●Aura – Migraine headache, particularly in children over 10 years of age, may involve an aura, such as scintillations or scotomata. (See "Pathophysiology, clinical features, and diagnosis of migraine in children", section on 'Clinical features'.)

Past medical history — In addition to children with immunodeficiencies or ventriculoperitoneal shunts, headaches in those with the following conditions may be life threatening (see 'Life-threatening conditions' above):

●Malignancy – Life-threatening conditions that can cause headache among children with known malignancies include infection, hemorrhage, and metastatic disease.

●Coagulopathy – Intracranial hemorrhage must always be considered as a cause of headache for patients with coagulation defects, including those with acquired coagulopathy (eg, immune thrombocytopenia, disseminated intravascular coagulopathy) or taking anticoagulant medication.

●Sickle cell disease – Cerebrovascular accident is the leading cause of death among children with sickle cell disease. Intracranial hemorrhage accounts for about one-third of these cerebrovascular events. (See "Acute stroke (ischemic and hemorrhagic) in children and adults with sickle cell disease".)

●Cyanotic heart disease – Headache may be the manifestation of brain abscess caused by septic emboli among children with cyanotic heart disease and a right-to-left intracardiac shunt who develop endocarditis. (See "Pathogenesis, clinical manifestations, and diagnosis of brain abscess".)

Physical examination — A complete physical examination should be performed, including vital signs (with temperature and blood pressure [BP] measurements) and a thorough neurologic examination.

●General appearance – Most children with serious causes for their headaches are ill appearing or have altered mental status (eg, confused, lethargic, or comatose).

●Vital signs – The vital signs must include temperature, heart rate, and BP measurements. Fever suggests infection or, rarely, intracranial hemorrhage. Elevated BP alone (table 9 and table 10) may result in headache symptoms or may be a response to increased intracranial pressure (ICP) along with bradycardia or, in children, tachycardia as part of Cushing triad. (See "Elevated intracranial pressure (ICP) in children: Clinical manifestations and diagnosis", section on 'Acutely elevated ICP'.)

●Head and neck – The head and neck should be examined for evidence of trauma. Other findings on examination that may suggest a diagnosis include:

•The diagnosis of otitis media can be determined with otoscopy. (See "Acute otitis media in children: Clinical manifestations and diagnosis", section on 'Diagnosis'.)

•Children with maxillary or frontal sinusitis may have facial tenderness to palpation or purulent rhinorrhea.

•Streptococcal pharyngitis as a cause of headache may be evident with swelling, erythema, and exudates of the tonsillar pillars. Pharyngitis due to other etiologies may present with pharyngeal enanthem, simple erythema, or erythema with exudates. (See "Group A streptococcal tonsillopharyngitis in children and adolescents: Clinical features and diagnosis", section on 'Clinical features' and "Evaluation of sore throat in children", section on 'Common conditions'.)

•The teeth and gingiva should be examined for evidence of inflammation or abscess.

•Tenderness over the temporomandibular joint (TMJ) and/or masseter muscles suggests TMJ dysfunction.

•Nuchal rigidity can be a sign of migraine headache, meningitis, intracranial hemorrhage, or, less commonly, brain tumor [34].

●Skin – Children with neurocutaneous syndromes (ie, neurofibromatosis or tuberous sclerosis) may have brain lesions causing headaches. Skin should be carefully examined for characteristic features, such as café au lait spots or ash leaf spots [12]. (See "Tuberous sclerosis complex: Clinical features", section on 'Brain lesions' and "Neurofibromatosis type 1 (NF1): Pathogenesis, clinical features, and diagnosis", section on 'Clinical manifestations'.)

Bruising characteristics that should raise concern for child abuse are provided in the table and discussed in detail separately (table 11). (See "Physical child abuse: Recognition".)

Funduscopic examination — Funduscopic examination should be performed for all children who are being evaluated for headache. Adequate visualization of disks may be challenging in young or uncooperative patients. Papilledema, hemorrhages, exudates, and abnormal vessels are important manifestations of serious intracranial pathology, but the absence of these findings does not exclude such conditions [35].

Neurologic examination — The majority of children with headaches who have serious neurologic conditions have abnormalities on neurologic examination [24,36-38]. The following findings are significant for children with headaches:

●Altered mental status may be the result of encephalitis, intracranial hemorrhage, elevated ICP, or hypertensive encephalopathy. Seizures that are focal or associated with a prolonged postictal period (but not including febrile seizures) raise concern for an intracranial focus such as an intracranial hemorrhage or brain tumor.

●Extraocular muscle palsies or nystagmus may be the result of elevated ICP or direct compression by a mass lesion.  

●Motor asymmetry, gait disturbance, or difficulty with fine motor coordination suggests a focal intracranial lesion.

●Some children with migraine headaches develop focal neurologic abnormalities (eg, ophthalmoplegia, motor weakness, or ataxia) as part of their migraine syndromes (table 5) [39]. Caretakers can generally confirm that the pattern is typical for the child's headaches.

The elements of the pediatric neurologic examination are discussed in detail separately. (See "Detailed neurologic assessment of infants and children", section on 'Neurologic examination'.)

Ancillary studies — The need for ancillary studies is determined by the patient's clinical findings. Patients with no red flag findings on history and a normal neurologic examination should not undergo routine lab studies, lumbar puncture, electroencephalography (EEG), or neuroimaging [25,36].

Neuroimaging

Indications — Indications and timing of neuroimaging are determined by clinical findings:

●Red-flag findings – Patients at the highest risk for life-threatening intracranial conditions and for whom we recommend emergency neuroimaging include patients with any one of the following red-flag findings [8,13,24,36,40]:

•Focal neurologic examination (eg, cranial nerve deficits, visual field cuts, focal motor weakness, asymmetric reflexes, or focal seizure other than seizures consistent with febrile seizures), prolonged altered mental status (>60 minutes), ataxia, or dysmetria

•Papilledema or other signs of increased intracranial pressure (eg, headache precipitated or worsening by a prolonged period of lying down [especially when associated with vomiting], hypertension with brady- or tachycardia, or progressive vomiting)

•Abrupt onset of "worst headache of life (“thunderclap” headache), especially if associated with neck pain or stiffness

●Chronic, progressive headache and normal examination – In patients with a chronic progressive headache but a normal neurologic examination and no findings of intracranial hypertension, including a normal funduscopic examination, we suggest either an emergency fast MRI, if available, or an expedited outpatient MRI (within 24 to 48 hours) and timely follow-up with their primary care provider or pediatric neurologist. This approach avoids the radiation exposure of an emergency CT scan.

●Cutaneous findings – Patients with skin lesions suggestive of neurocutaneous syndromes warrant prompt pediatric neurology consultation and should receive MRI as part of a diagnostic evaluation. (See "Neurofibromatosis type 1 (NF1): Pathogenesis, clinical features, and diagnosis" and "Tuberous sclerosis complex: Evaluation and diagnosis".)

Children with a nontraumatic headache with no worrisome features, normal fundoscopy, and a normal neurologic examination have a low risk for a life-threatening intracranial abnormality (<0.4 percent) and do not require neuroimaging [36].

Many experts regard certain headache characteristics and patient history as worrisome and in need of further investigation by emergency neuroimaging (table 12) [13,36,41,42]. However, most of these findings are nonspecific, and the prevalence of life-threatening conditions in previously healthy patients with these features is low. For example, in a prospective observational study of almost 200 otherwise healthy children with one or more red-flag findings on history, 73 underwent emergency imaging (either fast magnetic resonance imaging [MRI] or computed tomography [CT]), and one patient had an emergency intracranial abnormality (brain tumor) [13]. No emergency conditions were identified in the patients who did not receive emergency imaging as determined by follow-up at four to six months. (See 'MRI versus CT' below.)

MRI versus CT — For stable, previously healthy patients with a nontraumatic headache who warrant emergency imaging, we suggest fast MRI whenever available, as long as rapid pediatric radiology interpretation is assured [4,43]. This approach avoids significant radiation exposure [41]. (See "Ischemic stroke in children: Clinical presentation, evaluation, and diagnosis", section on 'CT safety considerations'.)

In unstable patients, or if MRI with rapid interpretation is not available, CT of the brain should be performed. CT does not require sedation in most children and generally identifies any condition that requires immediate treatment. If a CT is performed, some children may require an MRI at a later time to provide clearer definition of abnormalities noted on CT or to identify lesions that may not be seen on CT (eg, some infections, hemorrhagic processes, or cerebral venous thrombosis).

Lumbar puncture — The emergency evaluation of a child with headache should include a lumbar puncture (LP) in the following situations:

●Suspected nonfocal infection (meningitis, encephalitis)

●Concern for subarachnoid hemorrhage not diagnosed on neuroimaging

●To measure opening pressure for suspected idiopathic IIH (after neuroimaging)

Patients with focal neurologic examinations, a change in level of consciousness, significant concern for increased ICP, or papilledema should have an emergency CT scan or fast MRI performed before LP. There is a risk of herniation syndrome when LP is performed in patients with increased ICP and an abnormal ICP gradient (eg, tumor or abscess with a midline shift, obstructive hydrocephalus). (See "Lumbar puncture in children", section on 'Cerebral herniation'.)

Antibiotic therapy should not be delayed for children with suspected meningitis who are unstable or must receive neuroimaging before an LP is performed (table 7). (See "Bacterial meningitis in the neonate: Clinical features and diagnosis", section on 'Lumbar puncture'.)

The appropriate cerebrospinal fluid (CSF) studies to obtain include CSF cell count and differential, glucose, protein, Gram stain, and bacterial culture. CSF results in bacterial and viral infection are provided in the table (table 13). Depending upon the clinical suspicion and patient presentation, the clinician may also include specialized viral testing. (See "Viral meningitis in children: Clinical features and diagnosis", section on 'Detection of virus'.)

Borrelia burgdorferi-specific antibodies can help establish the diagnosis of central nervous system Lyme disease, but a negative test does not exclude it. Blood testing for Lyme disease should also be performed. Lyme PCR has low sensitivity, and false positives have been frequently reported. Thus, Lyme PCR is not recommended for routine testing of CSF in Lyme disease. (See "Nervous system Lyme disease", section on 'Meningitis'.)

Other studies — In patients whose history and/or physical examination suggest a life-threatening etiology, other studies may be indicated depending upon the suspected cause:

●A complete blood count, C-reactive protein, erythrocyte sedimentation rate, electrolytes, serum glucose, blood urea nitrogen, serum creatinine, and blood cultures are warranted for a child with possible bacterial meningitis. (See "Bacterial meningitis in children older than one month: Clinical features and diagnosis", section on 'Blood tests'.)

●In addition to testing as above for bacterial meningitis, patients with possible viral meningitis may warrant serologic tests for specific pathogens based upon clinical suspicion. (See "Viral meningitis in children: Clinical features and diagnosis", section on 'Blood tests'.)

●An elevated carboxyhemoglobin by direct cooximetry on a venous or arterial blood gas establishes the diagnosis of CO poisoning. (See "Carbon monoxide poisoning", section on 'Diagnosis'.)

●Recommended studies for children with hypertensive emergencies are provided in the table and discussed separately (table 14). (See "Approach to hypertensive emergencies and urgencies in children", section on 'Ancillary studies'.)

●Patients who reside in or have visited an endemic area for Lyme, have a risk factor for tick exposure, and have clinical findings consistent with Lyme meningitis should undergo serologic testing for Lyme disease. Diagnosis and treatment of Lyme meningitis differs from bacterial meningitis in that CSF studies are not always necessary and oral antibiotic therapy can be an appropriate treatment method. (See "Diagnosis of Lyme disease", section on 'Serologic tests' and "Treatment of Lyme disease", section on 'Acute neurologic manifestations'.)

DIAGNOSTIC APPROACH — A systematic approach to the emergency evaluation of children with nontraumatic headaches (including a focused history, careful physical examination, and selected ancillary studies) generally identifies those who have conditions that require rapid diagnosis and treatment (table 2 and algorithm 5A-B). Previously healthy children with no red-flag findings on history and a normal neurologic examination should not undergo routine lab studies, lumbar puncture, electroencephalography (EEG), or neuroimaging [25,36].  

Children with headaches who are immunocompromised, have ventriculoperitoneal shunts, or have been injured require specific evaluations that frequently include ancillary studies, such as neuroimaging. (See "Severe traumatic brain injury (TBI) in children: Initial evaluation and management" and "Minor blunt head trauma in infants and young children (<2 years): Clinical features and evaluation" and "Hydrocephalus in children: Management and prognosis", section on 'Shunt complications'.)

Chronic or recurrent headaches — For older children and adolescents who present with typical headache patterns and no additional findings, the diagnosis of migraine or tension headache can often be made following a careful history, family history, and thorough physical examination [36]. Those with a change in the headache pattern or new clinical features (eg, fever or stiff neck) may require ancillary studies as directed by the history and physical examination.

Normal neurologic examination — Children with headache who have no worrisome historical features (table 12) and normal neurologic examinations, including funduscopic examination, generally do not require neuroimaging [36]. (See 'Neuroimaging' above.)

Fever — For children with headache, fever, and meningeal signs, cerebrospinal fluid (CSF) evaluation for glucose and protein levels, cell count, and culture should be performed. Central nervous system infection can usually be diagnosed by CSF evaluation (table 13). (See "Bacterial meningitis in children older than one month: Clinical features and diagnosis", section on 'Interpretation'.)

Children with normal CSF studies and those with headache and fever without meningeal signs (who typically do not require CSF evaluation) likely have infectious causes for headache, such as viral syndrome, sinusitis, or dental abscess. (See 'Common conditions' above.)

No fever — A child with headache, a normal neurologic examination, and no fever, who has no other abnormal features identified on history or physical examination, may be experiencing a first migraine or a tension-type headache. (See 'Common conditions' above.)

Clinical features identified by history or physical examination that suggest a specific diagnosis include the following:

●Exposure to improperly vented home heating systems, space heaters, or automobile exhaust fumes suggests carbon monoxide (CO) poisoning. (See 'Carbon monoxide poisoning' above.)

●Hypertensive encephalopathy may be the cause of headache for children with elevated blood pressure (BP) measurements (table 9 and table 10). (See 'Hypertensive urgency or emergency' above.)

●Children with tenderness to palpation over sinuses, teeth, or temporomandibular joints (TMJs) may have focal infections or TMJ syndrome. (See 'Common conditions' above and 'Temporomandibular joint dysfunction' above.)

Focal neurologic examination — The emergency evaluation of children with headache who have focal findings on neurologic examination (including focal seizures in afebrile children), or papilledema includes neuroimaging. Computed tomography (CT) is readily available, requires no sedation, and generally identifies any condition that requires emergency treatment. Thus, it is the preferred imaging modality in unstable patients. Stable patients may undergo CT or, if reliable interpretation is rapidly available, fast MRI. (See 'MRI versus CT' above.)

Computed tomography abnormal — Conditions that cause headache and focal neurologic findings that may be identified with CT include mass lesions (eg, brain tumors, cerebral abscesses or hematomas), hydrocephalus, and intracranial hemorrhage. Patients with abnormal CT scans require emergency neurosurgical evaluation. (See 'Life-threatening conditions' above.)

Computed tomography normal — In patients who have normal head CTs, lumbar puncture (LP) for CSF evaluation and opening pressure should be performed for those patients with extremely severe headaches and/or neck stiffness.

The following abnormalities suggest a specific diagnosis:

●Red blood cells in CSF (with a nontraumatic LP) may indicate subarachnoid hemorrhage. (See 'Subarachnoid and intracranial hemorrhage' above.)

●An elevated white blood cell count is consistent with central nervous system inflammation, such as occurs with infection (table 13). In children with focal neurological findings and CSF pleocytosis, herpes simplex virus should be strongly considered, as some patients may benefit from treatment with acyclovir. (See "Bacterial meningitis in children older than one month: Clinical features and diagnosis", section on 'Interpretation' and "Herpes simplex virus type 1 encephalitis".)

●Children with papilledema, normal head CT scans, and elevated opening pressures on LP may have idiopathic increased intracranial hypertension (IIH). Rarely, patients with these findings have cerebral venous thromboses. Magnetic resonance imaging (MRI) is required to distinguish cerebral venous thrombosis from IIH. Consequently, children with apparent IIH who are asymptomatic (except for headache) and are well enough to be discharged from the emergency department (ED) should be evaluated by a neurologist within the next several days. (See "Idiopathic intracranial hypertension (pseudotumor cerebri): Clinical features and diagnosis".)

●Patients with apparent papilledema who have normal CT scans, normal CSF evaluations, and normal opening pressures on LP may have pseudopapilledema with headaches from unrelated etiologies. (See "Overview and differential diagnosis of papilledema", section on 'Bilateral disc abnormalities' and "Overview and differential diagnosis of papilledema".)

A child with a moderately severe headache, no neck stiffness, focal neurologic findings that have resolved, and a normal head CT may have a migraine with aura.

The International Headache Society notes that aura associated with a migraine headache typically lasts for less than 60 minutes. A patient with a new or atypical headache and focal findings that last for longer than one hour may have a more serious cause for headache. Children in this situation who are ill appearing should undergo emergency MRI. Those whose symptoms have improved and who look well may be scheduled for MRI as an outpatient within several days. (See "Types of migraine and related syndromes in children", section on 'Complications of migraine'.)

INFORMATION FOR PATIENTS — UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics." The Basics patient education pieces are written in plain language, at the 5^th to 6^th grade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are written at the 10^th to 12^th grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon.

Here are the patient education articles that are relevant to this topic. We encourage you to print or e-mail these topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on "patient info" and the keyword(s) of interest.)

●Basics topic (see "Patient education: Headaches in children (The Basics)")

SUMMARY AND RECOMMENDATIONS

●Epidemiology – The majority of children who undergo emergency evaluation for nontraumatic headaches have self-limited conditions such as a minor febrile illness or a primary headache syndrome (table 5). However, a significant minority of previously healthy patients have life-threatening causes that require urgent diagnosis and treatment (table 2). (See 'Epidemiology' above and 'Causes' above.)

●Stabilization – Rapid recognition and treatment of life-threatening causes of nontraumatic headache in children is described in the following algorithms and rapid overview tables:

•Intracranial hypertension (algorithm 1) (see "Elevated intracranial pressure (ICP) in children: Management")

•Bacterial meningitis (table 7) (see "Bacterial meningitis in children older than one month: Treatment and prognosis")

•Carbon monoxide poisoning (table 8 and algorithm 2) (see "Carbon monoxide poisoning")

•Hypertensive urgency or emergency (algorithm 3 and algorithm 4) (see "Initial management of hypertensive emergencies and urgencies in children")

●Evaluation – Once stabilized, a systematic approach to the emergency evaluation of children with headaches generally identifies the underlying cause (algorithm 5A-B). Previously healthy children with no red-flag findings on history and a normal neurologic examination should not undergo routine lab studies, lumbar puncture, electroencephalography (EEG), or neuroimaging. (See 'Diagnostic approach' above.)

•History – Nontraumatic headache characteristics that raise concern for a life-threatening cause include any one of the following (table 12) (see 'History' above):

-Abrupt onset that is very painful (thunderclap, "worst headache of my life")

-Positional (ie, presents or worsens upon lying down)

-Awakens the child from sleep

-Occurs soon after awakening in the morning

-Precipitated or worsened by a Valsalva maneuver or exercise

-Chronic and progressive (ie, increased frequency and severity over time)

-Associated with vomiting (especially progressive and/or morning vomiting), behavioral changes, vision changes, polydipsia and polyuria, clumsiness, and/or ataxia,

-Occurring in patients with shunted hydrocephalus, malignancy, sickle cell disease, immunodeficiency, coagulopathy, or cyanotic heart disease

•Physical examination – Concerning findings include any one of the following (see 'Physical examination' above):

-Hypertension with brady- or tachycardia suggestive of increased intracranial pressure (ICP)

-Prolonged altered mental status (>60 minutes)

-Papilledema

-Neck stiffness

-Ataxia or dysmetria

-Focal neurologic deficits

•Neuroimaging – Indications and timing of neuroimaging in previously healthy children are determined by clinical findings (see 'Indications' above):

-Red flag findings – For children with a focal neurologic examination, findings of increased ICP, or "thunderclap" headache (especially if associated with neck pain or stiffness), we recommend stabilization, as needed, and emergency neuroimaging. For stable, previously healthy patients, we suggest fast magnetic resonance imaging (MRI) if rapid pediatric radiology interpretation is assured. Unstable patients or patients for whom MRI with rapid interpretation is not available require brain computed tomography (CT). (See 'MRI versus CT' above.)

-Chronic, progressive headache and normal examination – In patients with chronic, progressive headache with a normal physical examination, we suggest either an emergency fast MRI if rapid pediatric radiology interpretation is assured, or an expedited outpatient MRI (within 24 to 48 hours) followed by timely follow-up with their primary care provider or pediatric neurologist.

-Cutaneous findings – Patients with skin lesions suggestive of neurocutaneous syndromes warrant prompt pediatric neurology consultation and should receive MRI as part of a diagnostic evaluation.

•Lumbar puncture – The emergency evaluation of a child with nontraumatic headache should include a lumbar puncture (LP) in the following situations (see 'Lumbar puncture' above):

-Suspected nonfocal infection (meningitis, encephalitis)

-Concern for subarachnoid hemorrhage not diagnosed on neuroimaging

-To measure opening pressure for suspected idiopathic intracranial hypertension (IIH; after neuroimaging)

Patients with these indications and a focal neurologic examination, significant concern for increased ICP, or papilledema should have emergency neuroimaging performed before LP (table 7). Antimicrobial therapy should not be delayed for children with suspected meningitis who are unstable or must receive neuroimaging before an LP is performed.

•Other studies – In previously healthy children whose history and/or physical examination suggest a life-threatening etiology, other studies may be indicated depending upon the suspected cause. (See 'Other studies' above.)

ACKNOWLEDGMENT — The UpToDate editorial staff acknowledges Christopher King, MD, FACEP, now deceased, who contributed to an earlier version of this topic review.