Introduction

Headache is a common symptom in children. Recent population-based studies have found that headaches generally increased from childhood to adolescence and there is an estimated overall mean prevalence of headache in children and adolescents of about 50%–60%. Accompanying eye or vision signs and symptoms may lead these children to the ophthalmologist. Since serious neurologic morbidity and mortality can be heralded by head pain, it is important for pediatric ophthalmologists to understand the causes of headache in children as well as recognize headache syndromes to help guide the diagnostic work-up and management.

Classification and Etiology

Headaches in children can be classified as primary or secondary. The International Classification of Headache Disorders (ICHD-3) defines the headache syndromes, and pediatric criteria were first included in 2004. In adults and children, the most common type of primary headache is migraine with and without aura, and less common syndromes include new persistent daily headache (NPDH), trigeminal autonomic cephalalgia (TAC), and tension-type headache (TTH). Medication-overuse headache can often complicate a primary headache syndrome or be a secondary headache unassociated with a headache syndrome. Other primary headaches are listed in Box 58.1 .

Box 58.1
Causes of primary headaches in children

Migraine without aura

Migraine with aura

Tension-type headache

Trigeminal autonomic cephalalgia

Primary stabbing headache

Primary cough/exercise/cold-stimulus/hypnic headache

New daily persistent headache

Migraine

Migraine is common in children; its frequency increases throughout childhood and peaks in adolescence, with a prevalence of 1.2%–3.2% in 3–7-year-olds, 4%–11% in 7–11-year-olds, and 8%–23% by age 15. Migraines are present in boys and girls with similar incidence in the prepubertal ages, but the gender predominance shifts towards girls in the adolescent years. There are three main groups of pediatric migraine: migraine without aura (formerly common migraine); migraine with aura (formerly classic migraine); and childhood periodic syndromes that may be associated with headaches.

Migraine without aura is the most frequent form of migraine in children and adolescents, and accounts for 60%–85% of migraine in children. Migraine is defined by recurrent episodes of intense disabling headache separated by symptom-free intervals. The ICHD-3 criteria are included in Box 58.2 , and typical characteristics in children are bilaterality, a pulsating quality, moderate or severe intensity, aggravation by routine physical activity, and association with autonomic symptoms such as nausea and/or photophobia and/or phonophobia (may need to be inferred from behavior, as in retreating to a quiet dark room). Migraine in children is different than in adults ( Table 58.1 ). For instance, cranial autonomic symptoms such as aural fullness (feeling that the ears are plugged), facial flushing/sweating, lacrimation, conjunctival injection, ptosis, gritty eye symptoms, nasal congestion/rhinorrhea, and periorbital edema have been well described in children and occur more often in children than in adults. At least one cranial autonomic symptom was noted in 62% of pediatric migraineurs in one study. Aural fullness was found to be the most common symptom.

Box 58.2
Criteria for pediatric migraine without aura
Source: The International Classification of Headache Disorders, 3rd edition. Cephalalgia 2018;38(1):1–211.

Diagnostic criteria:

  • A.

    At least five attacks fulfilling criteria B through D

  • B.

    Headache attacks lasting 2–72 hours (untreated or unsuccessfully treated). Sleep is considered part of the headache duration

  • C.

    Headache has at least two of the following characteristics:

    • 1.

      Bifrontal/bitemporal or unilateral location (adults unilateral head pain)

    • 2.

      Pulsating/throbbing quality (which may need inference from child behavior)

    • 3.

      Moderate or severe pain intensity (numerical or FACES scale)

    • 4.

      Aggravation by or causing avoidance of routine physical activity

  • D.

    During headache at least one of the following:

    • 1.

      Nausea and/or vomiting

    • 2.

      Photophobia or phonophobia (may need inference from child behavior)

  • E.

    Not better accounted for by another disorder

Table 58.1
Differences between adult and childhood migraine
Feature Childhood Adult
Laterality Bilateral (but may start unilaterally) Unilateral
Duration of headache >2 hours >4 hours
Systemic symptoms Variable but often few Common
Positive family history Common Common
Non-visual aura Common Infrequent
Visual aura without headache Very unusual Common

Typically, migraine in children is episodic with symptom-free intervals; however, when the migraine symptom frequency increases to 15 or more days per month for 3 months, chronic migraine is diagnosed. Status migrainosus occurs when there is a debilitating unremitting headache lasting for more than 72 hours.

Red flags of migraine without aura

The risks of radiation exposure from computerized tomography (CT) imaging and the risk of anesthesia and sedation needed for magnetic resonance imaging (MRI) can often discourage neuroimaging a child with headache. However, the red flag signs and symptoms of a first or isolated headache listed in Box 58.3 should prompt consideration of urgent imaging of a child with headache. In a child with recurrent headaches, the findings of an abnormal neurologic exam should prompt imaging the brain. Emergent and urgent imaging in children or adolescents with headache is often with a CT scan of the head, but a brain MRI in a child is ideal if promptly available because of the superior neuroanatomic detail and lack of radiation.

Box 58.3

Red flags of pediatric headache

  • Focal neurological signs

  • Seizures (focal or generalized)

  • Alteration of consciousness/confusion

  • Papilledema

  • Fever and/or meningeal signs

  • Change or deterioration of personality/behavior

  • Age under 4 (especially with increasing head circumference)

  • Headaches that are always unilateral (rare in pediatric migraine)

  • Headaches that wake from sleep or upon wakening

  • Headaches with coughing, straining, or changing position

A study of 1562 children with recurrent headaches presenting to nine pediatric neurology clinics in tertiary hospitals found that 77% of these children had brain imaging, but only 9.3% had abnormal findings on the scans. In those with an abnormal neurologic exam, however, 50% of the scans identified a cause. A new-onset headache during pregnancy in an adolescent female is a red flag symptom, given the increased risk for an underlying etiology such as cerebral venous thrombosis in this setting. Occipital headaches in children in the past were considered a red flag, however, more recent data suggests that in the absence of an atypical history or an abnormal exam, occipital headaches are usually benign. Occipital location of head pain was found in 20% of children with migraine presenting to the emergency room.

Migraine with aura

In 30% of patients with migraine, the migraine attacks are preceded or accompanied by an “aura” characterized by transient focal neurologic symptoms. Migraine with aura ( Box 58.4 ) is defined by recurrent (≥2) attacks, lasting 5–60 minutes, with fully reversible visual, sensory, or other central nervous system symptoms. The aura usually develops gradually over minutes and is followed by associated migraine symptoms and headache. It typically starts before the pain phase begins. However, the aura may begin after the pain starts or continue into the pain phase. If a child meets criteria for typical aura, but has no subsequent headache, the child is diagnosed with typical aura without headache, previously referred to as “acephalgic migraine” or “ocular migraine.”

Box 58.4
Criteria for pediatric migraine with aura
Source: The International Classification of Headache Disorders, 3rd edition. Cephalalgia 2018;38(1):1–211.

Diagnostic criteria

  • A.

    At least two attacks fulfilling criteria B and C

  • B.

    One or more of the following fully reversible aura symptoms

    • 1.

      Visual

    • 2.

      Sensory

    • 3.

      Speech and/or language

    • 4.

      Motor

    • 5.

      Brainstem

    • 6.

      Retinal

  • C.

    At least one of the following four characteristics:

    • 1.

      At least one aura symptom spreads gradually over ≥5 minutes, and/or two or more symptoms occur in succession

    • 2.

      Each aura symptoms lasts 5–60 minutes (multiple aura symptoms have cumulative time, i.e. 3 symptom is allotted 3 × 60 minutes for resolution)

    • 3.

      At least one aura symptom is unilateral

    • 4.

      The aura is accompanied, or followed within 60 minutes by headache

  • D.

    Not better accounted for by another ICHD-3 or other neurologic diagnosis

Visual aura is the most common type of aura in adults and children, and found in 93% of children and adolescents diagnosed with migraine with aura. Positive visual symptoms are most often described as an arc of colored or white zig-zag, jagged, or serrated lines moving to the periphery; however, flashes of light or shapes are also described. The visual symptoms usually disappear after 15–20 minutes. Atypical visual complaints in migraine, but not aura per se , such as complex hallucinations, visual snow, and Alice in Wonderland syndrome, are well described in children.

Other non-visual auras

Sensory disturbance is the next most common aura, found in 5.5% of children with migraine with aura, and is usually described as pins and needles or numbness that slowly spreads (seconds to minutes) away from a point of origin. Migraine with brainstem aura, previously known as basilar migraine, must include at least two brainstem features (dysarthria, vertigo, tinnitus, hyperacusis, diplopia, ataxia, or decreased consciousness). Hemiplegic migraine has an aura that includes motor weakness. If there is a first- or second-degree relative with migraine aura involving motor weakness, then familial hemiplegic migraine from causative gene mutations CACNA1A , SCN1A , or ATP1A2 should be considered.

Migraine visual aura complications

The visual aura typically lasts less than 1 hour (although motor aura can last weeks) and is fully reversible; however, on rare occasions an aura can persist for more than 1 week and last for months. If there is no ischemia found on neuroimaging, persistent aura without infarction is diagnosed. Persistent positive visual phenomena (PPVP) differs from persistent aura without infarction in that the visual phenomena are usually continuous, full-field without vision loss, and not visually disabling. Another rare complication of aura is migrainous infarction, defined as a migraine aura lasting more than 1 hour and associated with infarction in the appropriate territory on neuroimaging. In a recent study, however, no increase in hemorrhagic or ischemic stroke risk in children with migraine was found.

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