’
Pediatric Headache: An Overview
Raquel Langdon, MD, and Marc T. DiSabella, DO
Headache represents the most common neurologic disorder in multiple primary and secondary headache types in children
the general population including children and is increasingly are discussed using the International Headache Criteria (IHCD-
being recognized as a major source of morbidity in youth 3) as a guide, and a summary provided of the various
related to missed school days and activities. In this article, we treatment modalities employed for pediatric headache includ-
take a holistic approach to the child presenting with headache ing lifestyle modifications, behavioral techniques, and abortive
with a focus on the detailed headache history, physical and and preventive medications.
neurologic examinations, and diagnostic evaluation of these
patients. Clinical presentations and classification schema of Curr Probl Pediatr Adolesc Health Care 2017;47:44-65
Part I: Introduction/Epidemiology of based on duration and temporal profile, severity,
Pediatric Headache associated symptoms, and neurologic examination. A
thorough history, including questions regarding fever,
eadache is the most common neurologic dis-
H order in children, affecting as many as 88% of
the pediatric and adolescent population.1 Head
recent illnesses, travel, and toxin or medication expo-
sures is helpful to determine if a secondary headache
disorder is likely.
pain may result in significant disability, including
missed school days and extracurriculars, in addition
to suboptimal participation in these types of regular Part II: Approach to Pediatric
activities for children. Chronic daily headache and Headache
chronic migraine has been shown to affect between 1%
and 4% of the adolescent population.1,2 Suicidal Headache History
ideation among adolescents with more than seven
The approach to the child with headache begins with
migraines per month is elevated to 1.69 times the
a comprehensive history and physical examination.
general population risk,3 demonstrating the morbidity
Throughout this process, clinicians will be attempting
and potential for mortality associated with this very
to define whether a headache is primary or secondary
common condition.
in nature in order to help guide subsequent evaluation
Headache is routinely classified as primary, meaning
and management of the patient. It is important to note
due to a complex interaction of genetics and environ-
that many children suffer from multiple headache
ment, versus secondary, meaning due to another
types, and, therefore, a detailed history of each differ-
process resulting in head pain such as increased intra-
ent headache type becomes essential.
cranial pressure, infection or inflammation, or very
A series of helpful headache questions has been
often due to alteration of nociceptor activity as seen in
previously proposed by Rothner4 and can be seen in
medication overuse headache. Determining this differ-
Table 1.4 As any type of pain including headaches is
ence is key to headache classification and management,
subjective, the emphasis should be placed on having
and supersedes all else in headache patients. Key
the child report the headache history, with parents and
clinical factors that help determine this difference are
other historians adding their comments only after the
From the Center for Neurosciences and Behavioral Health, Children's child's account has been taken.
National Medical Center, Washington, DC. However, clinicians caring for children with head-
Curr Probl Pediatr Adolesc Health Care 2017;47:44-65 aches may face challenges in that it is often difficult for
1538-5442/$ - see front matter
young children and adolescents to verbally describe
& 2017 Elsevier Inc. All rights reserved.
their symptoms. If this is the case, previous studies
[Link] have demonstrated that the adjunctive use of children's
44 Curr Probl PediatrAdolesc Health Care, March 2017
TABLE 1. Helpful headache questions (Reprinted with permission from Dooley JM4)
Helpful questions
1. When did the headache begin? Chronic headaches are unlikely to reflect intracranial pathology. New-onset worsening headaches
are more likely to be due to a space-occupying lesion and require neuroimaging.
2. How did the headache begin? Look for precipitants, such as head injury or social stresses.
3. What is the temporal pattern of the Intermittent headaches separated by intervals of well-being are most likely to be migraines.
headaches? Progressively more severe headaches are more likely to reflect pathology and require further
investigations. Tension-type headaches (TTH) are usually chronic and non-progressive.
4. What is the headache frequency? Migraines typically occur weekly or less often. TTH occur daily or several times per week. Headache
syndromes in childhood, such as cluster headache, may have their own unique pattern, occurring in
clusters of two to three per week over a few weeks or months, followed by long periods of headache
freedom. Headaches due to increased intracranial pressure (ICP) often occur nightly.
5. How long does the headache typically last? Migraines are often brief, lasting 30–120 min. Although the International Headache Society criteria
define pediatric migraine as lasting up to 72 h, few pediatric patients have regular migraines that
last this long. TTH often lasts “all day”. Cluster headaches are brief.
6. Do the headaches happen at any particular Headaches that occur at night or in the early morning are more likely to reflect increased ICP,
time or circumstance? although as many as 25% of migraine episodes occur at night. Children with TTH may describe
waking with their headache, although this is typically after the child arises in contrast with
increased ICP, which may wake up the child. Occasionally, headaches occur exclusively in one
situation or circumstance (e.g., school, when hungry or with changes in weather). Children with
chronic morning headaches and a history of bruxism should be examined for temporomandibular
joint dysfunction.
7. Is there an aura or prodrome? Children with migraines may be able to describe or draw their aura. If the aura is persistently on the
same side, a structural lesion should be excluded. Parents may predict a migraine hours before it
occurs because their child may show a prodrome of lethargy, mood change, thirst or food cravings,
yawning, or pallor.
8. Where is the pain? Migraine is bifrontal in more than 55%. TTH is usually more diffusely located. The severity of pain is
not helpful in identifying serious causes of headaches.15 An inability to describe the quality of the
headache is much more likely to distinguish those with brain tumors or ventriculoperitoneal shunt
malfunctions; occipital headaches are more likely to occur in children with brain tumors.15
Persistent unilateral headaches should be considered to be suspicious.53
9. What is the pain like? Offering choices helps to determine the quality of the pain. Migraines are typically throbbing, but
may be described as heavy or pressing. An inability to describe the pain is more significant than the
actual choice of adjective. Historical concepts of throbbing equating to migraine and band-like to
TTH are probably inaccurate.
10. Are there associated symptoms? Migraines are usually accompanied by nausea, vomiting, anorexia, photophobia, phonophobia, or
osmophobia. Vomiting without accompanying nausea is suspicious. Migraine with aura may be
associated with aphasia, vertigo, visual, sensory, or other associated symptoms. If symptoms
persist beyond the headache or if the associated phenomenon is persistent from one headache to
the next, thought should be given to possible underlying pathology.
11. What do you do during the headache? What a child does if a headache begins during play is often more informative than asking what they
do if a headache begins at school. Those with migraines will usually interrupt their activity to return
home. Children with TTH will often watch television or play video games. In comparison, those with
migraines usually seek refuge in a quiet and darkened bedroom.
12. Would I know you had a headache if I saw The child with migraines usually looks ill. Those with TTH usually appear normal.
you?
13. What makes the headache better and Details on medication use can provide insight into both the headache and the patient/family's
worse? preferences for headache management. Many report using large doses of medication despite its
lack of benefit. Migraineurs often describe benefit from sleep or simple analgesics taken early in
the headache course. Aggravating factors in migraine include activity, light, noise, and smells.
Those with increased ICP will often find increased discomfort on lying down. Headaches due to low
ICP are usually worse on sitting or standing up.
14. Are there symptoms between headaches? Patients with migraines or TTH are asymptomatic between headaches. Ongoing symptoms, such as
forgetfulness, confusion, or localizing neurological symptoms suggest a structural lesion. Brain
tumors may manifest as lethargy, personality changes, or recent school failure. Difficulties with
concentration may persist beyond the headaches in those who have suffered a concussion.
In the setting of chronic daily headache, comorbid symptoms of depression may be present.
Underlying psychosocial factors are common and may relate to learning difficulties, bullying,
parental conflict, grieving reactions, and drug or alcohol abuse. In a population-based study,54
school-related factors, lifestyle, and mental health were predictive of headaches in adolescence.
15. Are there any other health problems? Children with chronic illnesses often feel stressed by their prognosis, they need to attend hospital
visits and take medications. Those with hypertension may have “migraine-like” headaches.
16. Are you taking medications? Headaches may occur as an adverse effect to medications used to treat other conditions or to treat
the headaches themselves. It is important to understand the attitudes of the patient and parents
Curr Probl PediatrAdolesc Health Care, March 2017 45
TABLE 1. (continued )
toward medication. Quantifying the child's use of non-prescription analgesics will identify those at
risk for rebound analgesic headaches. A medication history may also reveal exposure to
medications associated with idiopathic intracranial hypertension, such as oral contraceptives,
vitamin A, isotretinoin, tetracycline, and corticosteroids.
17. Is there a family history of headaches? Many children with migraine or TTH have first-degree family members with similar headaches. In
these families, educational efforts should be directed toward all those in the family with
headaches.
18. What do you think is causing the This is usually a very valuable question. Some children will identify a particular stressor of which the
headaches? parents are often unaware. Both children and parents are also afforded the opportunity to discuss
their fears of underlying pathology. A number of families will demonstrate a remarkable
misunderstanding of the potential causes of their child's headaches. Many believe the headaches
are caused by chronic sinusitis. There is no evidence to support chronic headaches as a result of
chronic sinusitis.
headache drawings may aid in the clinical diagnosis of through April 2013 and found the following risk factors
headache type. Stafstrom et al. evaluated 226 children for headache or its chronification in children and
consecutively seen for headache and asked all children adolescents (up to 5.8-fold elevation of risk): a dysfunc-
to draw a picture of how their headache felt prior to tional family situation, the regular consumption of
history taking. The drawings were then scored as alcohol, caffeine ingestion, smoking, a low level of
migraine or non-migraine by pediatric neurologists physical activity, physical or emotional abuse, bullying
blinded to the clinical history. A clinical diagnosis of by peers, unfair treatment in school, and insufficient
headache type was then independently determined by a leisure time. Teenagers should be interviewed independ-
different pediatric neurologist using the patient's clin- ently and asked specifically about substance use. Social
ical history and examination findings. Compared with history may be particularly revealing with respect to a
the gold standard clinical diagnosis, the headache patient's functional disability due to headaches, and use of
drawings were found to have a sensitivity of 93.1%, validated questionnaires such as the PedMIDAS may be
specificity of 82.7%, and a positive predictive value helpful to this end.8
(PPV) of 87.1% for migraine.5 Based on the history, headaches can be divided into
The headache history should also be obtained in the four basic temporal patterns, which can help to further
broader context of a patient's general medical history shape differential diagnosis and patient evaluation.
including birth history, past medical history, and These are summarized in Figure 19 and include acute,
development with special emphasis placed on histor- acute recurrent (episodic), chronic progressive, and
ical features that may increase headache risk such as chronic non-progressive headache types. Papetti
head trauma, major illnesses, prior surgeries, or pres- et al.10 have nicely reviewed common causes of
ence of comorbid medical conditions (i.e., chronic headache by temporal pattern (Table 2).
diseases, malignancy, hypercoagulability states, and
neurocutaneous disorders). In addition, questions
should be asked pertaining to lifestyle factors that
may predispose a child to headaches such as sleep
habits, nutrition, hydration, physical activity, and
screen use. Family history of headaches is equally
important, and certain primary headache disorders such
as migraine have been found to have associated genetic
risk. Social history should focus on looking at family life,
school, relationships, activities, and presence of any
stressors. For example, a meta-analysis conducted by
Gini et al.6 in 2014 found that bullied children and
adolescents had a significantly higher risk for headaches
compared to non-bullied peers. Similarly, Straube et al.7 FIG 1. Temporal headache patterns. (Reprinted with permission
performed a retrospective literature review of PubMed from Blume HK.9)
46 Curr Probl PediatrAdolesc Health Care, March 2017
Physical Examination suggestive of increased intracranial pressure (i.e.,
altered mental status, cranial nerve abnormalities,
The comprehensive pediatric headache examination papilledema, vomiting, or headaches disrupting sleep).
was first proposed by Linder in 2005.11 The clinical The American Academy of Neurology previously
examination of the child with headache will largely be published a practice parameter on the evaluation of
driven by the patient history and will also be used to children and adolescents with recurrent headaches in
look for signs suggestive of 2002. The current guidelines
secondary headache etiologies. recommend against routine
Assessment should begin with The American Academy of lab studies, lumbar puncture,
evaluation of vital signs includ- Neurology previously published EEG, or neuroimaging in
ing head circumference to screen a practice parameter on the patients with no red flags on
for presence of abnormalities
evaluation of children and ado- history and a normal neuro-
such as fever, hypertension, or logic examination.12 More-
macrocephaly. A thorough head lescents with recurrent head- over, benign neuroimaging
and neck examination should aches in 2002. The current findings are common and have
follow including cervical spine guidelines recommend against been reported in up to 20% of
assessment to look for signs of routine lab studies, lumbar pediatric headache patients
trauma, meningeal irritation, who are imaged.13 Therefore,
areas of tenderness, and cranial puncture, EEG, or neuroimaging with routine use of neuroimag-
auscultation for bruits. A Muel- in patients with no red flags on ing in children with headaches,
ler's maneuver may be utilized to history and a normal neurologic some families may believe
detect increased pressure in the examination. these incidental findings are
sinuses, whereby a child is asked causative, when in fact they
to hold their nose while counting are likely unrelated to the head-
to three and then cough, inducing a brief increase in ache.4 The AAN found that presence of the following
sinus pressure that may indicate an underlying sinus- were predictive of the presence of a space-occupying
itis. Oral examination to rule out dental disease may lesion: (1) headache of less than 1-month duration, (2)
also be helpful, and skin examination should be absence of family history of migraine, (3) abnormal
focused on stigmata suggestive of neurocutaneous neurologic findings on examination, (4) gait abnormal-
disorders or signs of systemic disease such as rash. ities, and (5) occurrence of seizures.12
Neurologic examination will include evaluation of Table 3 presents a summary of risk factors to consider
mental status and speech, vision, funduscopic examination when requesting imaging in children with headache.14
to rule out papilledema, cranial nerves, motor and strength,
reflexes, sensation, coordination, and gait. Particular note Part III: Primary Headaches in
should be made to any asymmetries or focal findings Pediatrics
appreciated.4,11
The two most common primary headache types in
pediatric patients includes both migraine and tension-
Diagnostic Testing type headaches (TTH). The following section will
In children with recurrent headaches, diagnostic review disease-specific definitions, genetics, patho-
studies are rarely required unless risk factors or so- physiology, clinical manifestations, and diagnostic
called “Red Flag” or worrisome headache symptoms testing for each of these respective headache types.
are present. Concerning features may include head- For a detailed discussion of treatment approaches in
aches that are worsening in frequency or severity, a pediatric headache, please refer to the section Part VI:
significant change in a patient's baseline headache Treatment.
pattern, sudden onset of a “worst ever” headache,
headaches in the setting of systemic symptoms or
Clinical Vignette #1
comorbid conditions known to increase headache risk,
headaches with associated abnormalities on physical A 15-year-old female presents to your office with
examination, and headaches associated with signs eight headaches per month over the last 6 months that
Curr Probl PediatrAdolesc Health Care, March 2017 47
typically last hours to 1 day. The headache is in significant disability, with 19% having some dis-
described as bifrontal, throbbing, and severe, with ruption in participation in school and extracurricular
associated light sensitivity and nausea. She denies activities.17
vision changes or other neurologic symptoms with
headaches. She states that her headaches typically Genetics
resolve with use of OTC pain relievers and she will The genetics of migraine appear to be extremely
also lie down in her room with the lights off and sleep. complex, with numerous studies implicating more than
She has a past medical history notable for motion 30 potential genes associated. Factors complicating the
sickness, and there is a family history of maternal discovery of single gene alleles implicated in migraine
relatives with headaches attributed to sinusitis. Her include epigenetic phenomena, variable penetrance,
neurologic examination is non-focal. What is the most and the possibility of multiple gene effects on one
likely diagnosis based on the patient's clinical history another as seen in polygenetic conditions. Syndromes
and examination? (Refer to clinical case discussion associated with migraine include cerebral autosomal
section at the end of the section for answer). dominant arteriopathy with subcortical infarcts and
leukoencephalopathy (CADASIL), mitochondrial
Migraine With and Without Aura encephalopathy, lactic acidosis, and stroke-like epi-
sodes (MELAS), and have genetic mutations that have
Disease Definitions and Epidemiology been fairly well established, including NOTCH3 and
Migraine is a common headache disorder character- most frequently MTTL1, respectively.18 The best
ized by recurrent episodes of moderate-to-severe studied monogenetic disorder in migraine is familial
throbbing head pain resulting in a decrease in activity hemiplegic migraine, which has been shown to have a
level, and associated symptoms that include gastro- variety of mutations in genes including CACNA1A,
intestinal symptoms and/or a heightened sensitivity to SCN1A, and ATP1A2.19 These genes are responsible
environmental stimuli, such as light or sound. Migraine for protein products that result in neuronal modulation
with aura is associated with through calcium, sodium, and
further neurologic symptoms potassium gated channels result-
including visual, sensory, speech, Recent studies have suggested ing in both gain and loss of
or motor disturbances, which add that infant colic may in fact be a function. A number of mutations
to headache disability. manifestation of headache or in serotonin, dopamine, and gluta-
Migraine headaches are prev- 16 mate receptors have been shown
alent in both adult and pediatric
migraine. Manifestations of to be affected in population-based
patients, most often beginning headache in children can be studies demonstrating higher
in adolescence. Children and variable, and include episodes prevalence among the migraine
adolescents may experience of vertigo, vomiting, and groups using techniques of
head pain at a very early age,
abdominal pain as precursors to genome-wide associated studies
with a migraine prevalence of (GWAS). A variety of candidate
3% in children 3–7 years old, migraine. genes are currently being studied
and increasing to 4–11% for potential linkages to migraine,
15
between 7 and 11 years old. There is a shifting including neuronal, hormonal, vascular, and inflamma-
gender frequency in migraine, with males being more tory targets.18 Despite considerable interest in identifying
common at young ages, and females being more these targets, recent GWAS analyses have failed to show
common from teenage years through adulthood, which reproducible results in the 30 or so gene targets thought to
has been theorized to be related to puberty and the be involved in migraine, perhaps suggesting epigenetic or
effects of estrogen on smooth muscles, specifically population-specific variance.
intracranial blood vessels. Recent studies have sug-
gested that infant colic may in fact be a manifestation Pathophysiology
16
of headache or migraine. Manifestations of headache There have been a variety of theories over the past
in children can be variable, and include episodes of several decades regarding migraine pathophysiology. It
vertigo, vomiting, and abdominal pain as precursors to appears likely that a cascade of events occur in
migraine. Migraine in children and adolescents results migraine related to an activation of pain-sensitive
48 Curr Probl PediatrAdolesc Health Care, March 2017
TABLE 2. Differential diagnosis of headache by pattern (Adapted with cerebral vessels, likely the source of the throbbing
permission from Papetti et al10)
pain in migraine. The trigeminal nerve and its con-
Acute Acute infection
o Upper respiratory infection
nection to intravascular blood vessels has been
o Sinusitis thought to be a downstream effect of migraine cortical
o Meningitis spreading depression. Using a model of overall
o Encephalitis
central nervous system hypersensitivity, migraine
Hypertension
Substance/toxin induced can be viewed as a sequence of events resulting in
o Substance use trigeminal nucleus activation in the brainstem in
o Medications response to a variety of internal and external stressors,
o Intoxicants (lead and CO)
Increased intracranial pressure followed by cortical spreading depression and central
o Brain tumor nervous system vasodilation and activation of pain
o Hydrocephalus receptors mediated by the V1 subdivision of the
Vascular
o Intracranial hemorrhage
trigeminal nerve.
o Subarachnoid hemorrhage Cortical spreading depression has been well studied
o Cerebral venous sinus thrombosis as well and is the leading theory implicated in migraine
Migraine (first presentation)
onset, with animal and human models demonstrating a
Acute recurrent Primary headache type wave of spreading depolarization across the cortex,
o Migraine
o Tension-type headache
often beginning in the occipital lobe, explaining why
o Autonomic cephalgia visual changes such as scotoma may occur in many
Seizures patients with migraine aura. During the aura, there is a
Hypertension
relative hypoperfusion of the cortex that can be
Metabolic
o Hyperthyroidism demonstrated on conventional angiography, followed
o Electrolyte disturbance by the painful portion of migraine during which there
Medication induced is relative hyperperfusion of the cortex with resultant
MELAS
vasodilation.21 A variety of neurotransmitters and their
Chronic non-progressive Primary headache type receptors have been implicated in migraine patho-
o Chronic migraine
o Chronic tension-type headache physiology, mostly focusing on serotonin (5-HT) and
Medication overuse headache (MOH) calcitonin gene-related peptide (CGRP), both of which
Post-concussion syndrome are primary therapeutic targets and have shown prom-
Chronic progressive Increased intracranial pressure ising results.
o Brain tumor
o Hydrocephalus
o Pseudotumor cerebri Clinical Manifestations
Vascular Migraine without aura is defined by the ICHD-3 as a
o Vascular malformation
o Aneurysm
“recurrent headache disorder manifesting in attacks
o Hematoma lasting 4–72 h; typical characteristics are unilateral
Medications location, pulsating quality, moderate or severe inten-
sity, aggravation by routine physical activity and
intracranial structures, including the dural venous association with nausea and/or photophobia and phono-
sinuses, large intracranial cerebral vessels, and dura phobia”.22 Pediatric modifiers include duration as short as
mater, along with a decrease in endogenous pain 1 h, frontal or bilateral location, pulsating being defined as
control pathways.20 Pain within the head is mediated throbbing or varying with the heartbeat, and the implica-
by nociceptors that transmit information from the tion of light and sound sensitivity by the child's behavior,
anterior head regions, via the ophthalmic branch of such that the child lies in a dark and quiet room. Based on
the trigeminal nerve to the trigeminal ganglion, and a study by Hershey et al.23 in 2005, the sensitivity of
from the posterior head regions, via the upper cervical migraine diagnosis can be significantly increased from
roots, C1 and C2. This in turn activates the trigemi- 61.9% to 84.4% by eliminating the minimum duration
novascular system at the level of the raphe nucleus, criteria, the number of associated symptoms, and
resulting in release of neuropeptides, including sub- unilateral location. In terms of migraine symptoms,
stance P, calcitonin gene-related peptide, and nitric vomiting, family history of migraine, unilateral loca-
oxide, which in turn causes dilation of pain-sensitive tion, and nausea have been found to be most specific,
Curr Probl PediatrAdolesc Health Care, March 2017 49
TABLE 3. Risk factors for consideration of imaging in children with headache (Reprinted with permission from Kabbouche MA14)
1. Abnormal neurological examination
2. Atypical presentation of the headache: vertigo, intractable vomiting, and headache waking the child from sleep
3. Recent headache of less than 6 months
4. Child of less than 6 years of age
5. No family history of migraine and/or primary headache
6. Occipital headache
7. Change in type of headache
8. Subacute progressive headache severity
9. New-onset headache in an immunosuppressed child
10. First and/or worst headache
11. Systemic symptoms and signs
12. Headache associated with confusion, mental status changes, or focal neurological complaints
whereas moderate-to-severe intensity, photophobia, neurologic exam.12 Worrisome symptoms include
and phonophobia are most sensitive. When combin- occipital location of headache, persistent focal neuro-
ing the sensitivity and specificity of migraine symp- logic complaints, acute onset of headache in an
toms, the most predictive of migraine symptoms individual without prior history of migraine, or lack
include intensity, nausea, worsening of pain with of a family history of migraine. Exam findings such as
activity, photophobia, and pulsating quality.24 altered mental status, focal neurologic signs, seizure,
Migraine with aura is less common in children, and is fever, nuchal rigidity, and abnormal fundoscopic
defined by the presence of a focal neurologic symptom, examination warrant further studies, including possible
most often visual, sensory, or speech changes, which neuroimaging or lumbar puncture with opening pres-
occurs before or at the onset of a migraine, with aura sure. Indications for neuroimaging include: (1) head-
symptoms lasting less than 1 h in duration. Additional ache of less than 6-month duration, (2) absence of
clinical manifestations of migraine and migraine var- family history of migraine, (3) abnormal neurologic
iants include cyclic vomiting syndrome, characterized findings on examination, and (4) headaches associated
by recurrent spells of vomiting; abdominal migraine, with substantial periods of vomiting or confusion.25.
characterized by recurrent spells of achy peri-umbilical
abdominal pain; benign paroxysmal conditions includ-
Clinical Vignette #2
ing vertigo or torticollis; Alice in Wonderland syn-
drome, characterized by alterations in perception of An 11-year-old boy presents to your office with worsen-
objects in the environment in relation to their size or ing headaches over the last month. The family denies
speed; and acute confusional migraine, characterized clear triggers for the headaches, although mentions the
by spells of recurrent and often trauma-induced periods patient recently completed end of semester testing at
of confusion and encephalopathy. school and one of his best friends moved away. He
By definition, the examination of a child with a describes his headaches as bifrontal or diffuse with a
primary headache disorder such as migraine should be squeezing type of pain and phonophobia. He denies
normal. Subtle findings may include tenderness to photophobia, nausea, vomiting, visual disturbance, or
palpation of the V1 segment of the trigeminal nerve, other focal neurologic symptoms with his headaches. The
cutaneous allodynia with scalp tenderness, and muscle family history is unremarkable. His neurologic examina-
tension related to chronic neck pain from headache. tion is non-focal, but notable for neck muscle tension.
What is the most likely diagnosis based on the patient's
Diagnostic Testing clinical history and examination? (Refer to clinical case
The diagnosis of migraine is purely made based on discussion section at the end of the section for answer).
the clinical criteria set forth by the ICHD-3 in the
presence of a normal neurologic examination. The Tension-Type Headache
American Academy of Neurology Practice Parameter
regarding the workup for children and adolescents with Disease Definitions and Epidemiology
headache recommend against lab testing or EEG in Tension-type headache (TTH) is the most common
patients with no red flags on history and with a normal type of headache experienced in the population and it
50 Curr Probl PediatrAdolesc Health Care, March 2017
is thought that all individuals experience these head- Diagnostic Testing
aches at some time in their life. Tension headaches The majority of patients with TTH require no testing.
seem to be most common when individuals are under Similar to migraine, any signs of secondary headache
significant stress due to emotional distress, poor sleep, like fever or meningismus, or neurologic examination
or missed meals. Tension headaches are less likely to abnormalities should prompt a broader workup.
present to emergency departments or primary care
physicians due to their mild intensity and infrequent Clinical Case Discussion (Vignette #1)
impact on daily life. Unlike migraine, TTH is not The patient's presentation is consistent with a diag-
associated with gastrointestinal symptoms and does not nosis of migraine without aura. Migraine without aura
require an individual to rest because of the pain. TTH is defined as a headache fulfilling two major criteria of
is more common among younger patients and females bifrontal or unilateral, throbbing, moderate-to-severe
and has a decreasing frequency with age.26 intensity pain, and worsening with activity or relief
TTH is defined as a headache fulfilling at least two with rest; and one minor criteria of photophobia,
major criteria of bilateral location, pressing, tightening, phonophobia, nausea, and vomiting.
and non-throbbing quality, mild-to-moderate intensity,
and lack of exacerbation by activity; and both of the Clinical Case Discussion (Vignette #2)
minor criteria including no more than one of light This patient is presenting with a tension-type headache,
sensitivity or sound sensitivity, and cannot be associ- likely worsened in the setting of school-related stress and
ated with nausea or vomiting.22 TTH are as short at a transition with a close friend moving away. Recall that a
30 min, and in their intractable or chronic form, can TTH is defined as a headache fulfilling at least two major
last indefinitely. criteria of bilateral location, pressing, tightening, and non-
throbbing quality, mild-to-moderate intensity, and lack of
Genetics exacerbation by activity. Either photophobia or phono-
Based on studies of families with TTH, there appears phobia may be present, but not both, and nausea/vomiting
to be little clear genetic influence. Multiple studies is less typical.
have attempted to identify targeted genes in TTH with
little success, likely explaining why most everyone Part IV: Secondary Headaches in
experiences TTH throughout their life. Pediatrics
Pathophysiology Secondary headaches are commonly observed in
The pathophysiology of TTH appears to suggest a pediatric patients and include a diverse array of
muscle-based source for headache. Painful nociceptors potential etiologies as defined by the IHCD-3 and
have been shown to be sensitized in TTH, resulting in summarized in Table 4.
lower thresholds for activation and low levels of
headache often extending to the neck and shoulders. Head Trauma
Please refer to section Part V: Pediatric Post-
Clinical Manifestations
Traumatic Headache (PTH).
Patients with TTH typically report a precipitating
event including emotional distress at school including
Clinical Vignette #3
examinations, disruption of sleep, or social stressors
with relationships between friends and family.27 They An 11-year-old boy presents to the emergency depart-
experience low levels of pain across the front and back ment after he collapsed while running outside following
of the head, slowly increasing over time, or fluctuating the sudden onset of “the worst” headache of his life
with frequent ups and downs, often most prominent associated with vomiting and neck pain. There was no
when stressors increase and least prominent when witnessed head trauma. On examination, the boy is
distracted or relaxed. afebrile, though noted to be lethargic and difficult to
Patients with TTH should have a normal neurologic arouse with photophobia and neck stiffness. What is the
exam aside from tenderness to palpation of the forehead next best step in the management of this patient? (Refer to
and neck muscles, some tenderness over the occipital clinical case discussion section at the end of the section for
head region, and tightness in the neck and shoulders. answer).
Curr Probl PediatrAdolesc Health Care, March 2017 51
TABLE 4. Etiologies of secondary headaches (Adapted with permission from Headache Classification Committee of the International Headache Society22)
Secondary headaches
Headache attributed to trauma or injury to the head and/or neck Acute/persistent headache attributed to traumatic injury to the head
Acute/persistent headache attributed to whiplash
Acute/persistent headache attributed to craniotomy
Headache attributed to cranial or cervical vascular disorder Headache attributed to ischemic stroke or TIA
Headache attributed to non-traumatic intracranial hemorrhage
Headache attributed to unruptured vascular malformation
Headache attributed to arteritis
Headache attributed to cervical carotid or vertebral artery disorder
Headache attributed to cerebral venous thrombosis
Headache attributed to other acute intracranial arterial disorder
Headache attributed to genetic vasculopathy
Headache attributed to pituitary apoplexy
Headache attributed to non-vascular intracranial disorder Headache attributed to increased cerebrospinal fluid pressure
Headache attributed to low cerebrospinal fluid pressure
Headache attributed to non-infectious inflammatory disease
Headache attributed to intracranial neoplasia
Headache attributed to intrathecal injection
Headache attributed to epileptic seizure
Headache attributed to Chiari malformation type I
Headache attributed to other non-vascular intracranial disorder
Headache attributed to a substance or its withdrawal Headache attributed to use of or exposure to a substance
Medication overuse headache
Headache attributed to substance withdrawal
Headache attributed to infection Headache attributed to intracranial infection
Headache attributed to systemic infection
Headache attributed to disorder of homeostasis Headache attributed to hypoxia and/or hypercapnia
Dialysis headache
Headache attributed to arterial hypertension
Headache attributed to hypothyroidism
Headache attributed to fasting
Cardiac cephalalgia
Headache attributed to other disorder of homeostasis
Headache or facial pain attributed to disorder of the cranium, neck, eyes, Headache attributed to disorder of cranial bone
ears, nose, sinuses, teeth, mouth, or other facial or cervical structures Headache attributed to disorder of the neck
Headache attributed to disorder of the eyes
Headache attributed to disorder of the ears
Headache attributed to disorder of the nose or paranasal sinus
Headache attributed to disorder of the teeth or jaw
Headache attributed to temporomandibular disorder
Head or facial pain attributed to inflammation of the stylohyoid ligament
Headache or facial pain attributed to other disorder of cranium, neck,
eyes, ears, nose, sinuses, teeth, mouth, or other facial or cervical
structures
Headache attributed to psychiatric disorder Headache attributed to somatization disorder
Headache attributed to psychotic disorder
Vascular Disorders usually that of a “thunderclap” or “worst headache of
life,” with sudden, acute onset that may also be
Vascular disorders including hemorrhage, ischemia, associated with focal neurologic deficits, seizures, or
aneurysms, vascular malformations, dissections, or altered mental status.
vasculitis may in rare cases cause headaches in Emergent non-contrast HCT is essential to evaluate
children. The temporal pattern of the headache is for subarachnoid hemorrhage (SAH), intracranial
52 Curr Probl PediatrAdolesc Health Care, March 2017
hemorrhage, ischemic stroke, cerebral venous sinus children with papilledema without headaches may
thrombosis, tumor, or third ventricular colloid cyst. have worse long-term visual outcomes.32,36
Imaging with a head CT within 6 h of symptom onset Early identification to avoid vision loss is key as
has a sensitivity between 92% and 100% for detecting some have estimated that up to 10% of children
an aneurysmal subarachnoid hemorrhage28; however if diagnosed with pseudotumor cerebri may have more
the CT is negative, lumbar puncture should next be long-term ophthalmologic impairment.37 In asympto-
performed. CSF testing should include opening pres- matic children with mild papilledema and the absence
sure, cell counts with differential in tubes 1 and 4, of vision abnormalities and other symptoms, close
protein, glucose, closing pressure, analysis for xantho- follow-ups with vision assessments may suffice with-
chromia, and potentially infectious studies.29 out other treatments.38 Medical management for pseu-
dotumor cerebri may include weight loss for
Idiopathic Intracranial Hypertension overweight/obese youth or use of diuretic medications
such as Diamox or Topamax to modify CSF produc-
(Pseudotumor Cerebri)
tion and flow. Surgical treatments include CSF shunt-
Pseudotumor cerebri remains a diagnosis of exclu- ing procedures and optic nerve sheath fenestration,
sion, literally meaning “without tumor,” with its hall- though optic nerve sheath fenestration is typically
mark being elevated intracranial pressure in the reserved for children with acute, severe vision loss
absence of other CNS pathology identified by imaging who have failed other therapies. In children who have
or CSF studies. Friedman et al.30 in 2013 published completed optic nerve sheath fenestration procedures,
updated diagnostic criteria (Fig 2) for pediatric pseu- up to 80% will have improved visual functioning.39,40
dotumor cerebri.
Pseudotumor cerebri can be classified as primary or
Hydrocephalus
secondary, where secondary pseudotumor may be
caused by a number of etiologies such as medications, Hydrocephalus is defined as the excessive accumu-
systemic diseases, or venous sinus thrombosis. lation of cerebral spinal fluid leading to ventricular
Whereas adolescents with primary pseudotumor cere- enlargement and increased intracranial pressure.
bri syndrome are more likely to be obese and female, Hydrocephalus may be present at birth (congenital)
studies suggest that pre-pubescent children are less or acquired. There are two major types of hydro-
likely to be obese, may be male or female, and may cephalus: (1) communicating and (2) obstructive (non-
present without headaches or visual blurring.31 communicating). In a communicating hydrocephalus,
In children with pseudotumor cerebri, headache is the the ventricular system is enlarged but patent, and there
most common presenting symptom. Headaches are is a downstream blockage or impaired CSF absorption
often described as diffuse, constant, and may be present. In contrast, with an obstructive hydrocephalus,
associated with other features such as pulsatile tinnitus, there is a blockage to CSF flow within the ventricular
visual changes (transient visual obscurations, double system that leads to increased intracranial pressure
vision, and impaired visual acuity), neck, or back (opening pressure 4250 mm). The blockage may be,
pain.22 In some cases, complicating the diagnosis, for example, due to the presence of a colloid cyst,
patients may also complain of symptoms such as aqueductal stenosis, or mass.
photophobia, nausea, vomiting, imbalance, ataxia, or Headaches with hydrocephalus should develop in
worsening with Valsalva, which may initially raise direct temporal relation to the onset/worsening of the
suspicion for a primary headache disorder or posterior hydrocephalus and also demonstrate improvement with
fossa lesion.32–34 Papilledema is estimated in 80–100% resolution of the hydrocephalus.22 Headaches may be
of patients with pseudotumor cerebri, though is no progressive and associated with other signs or symp-
longer required to make a diagnosis,30 and sixth nerve toms suggestive of elevated intracranial pressure (i.e.,
palsies are more commonly seen in the pediatric age increasing head circumference, papilledema, nighttime
group in 9–48% of children.35 Furthermore, asympto- or early morning awakenings, vomiting, altered mental
matic pseudotumor cerebri has been described in the status, seizures, cranial nerve abnormalities, or other
pediatric population, often in school age (patients focal neurologic deficits).
under the age of 8 years) with papilledema detected Regarding treatment, there is now level II evidence to
during routine fundoscopy. Literature suggests that support the use of CSF shunts and endoscopic third
Curr Probl PediatrAdolesc Health Care, March 2017 53
ventriculostomy (ETV) procedures for pediatric hydro- hydrocephalus and elevated ICP. On headache history,
cephalus.41 Shunt event-free survival has been esti- “red flag symptoms” may be elicited such as early
mated to be about 70% at 1 year post-surgery and 40% morning or nocturnal awakenings with headache
at 10 years.42 In pediatric patients who present with worsening, nausea, vomiting, vision changes, person-
worsening headaches or new acute, severe headache in ality changes, gait disturbance, focal neurologic symp-
the setting of a known diagnosis of hydrocephalus toms, and seizures.44 Screening for family history of
post-shunt placement, differential diagnosis should neoplasia, radiation exposure, and comorbid diagnoses
also include the possibility of shunt malfunction or associated with tumor risk (i.e., neurofibromatosis and
shunt infection. Long-term sequela of hydrocephalus tuberous sclerosis) is also essential.
may include cognitive delay, school difficulties, seiz-
ures, impaired mobility/ambulation, abnormalities with
Intracranial Hypotension
hearing or vision, endocrine dysfunction, depression,
and chronic pain.42 Just as headaches may occur with elevated intracranial
pressure, headaches may also occur with low intracranial
pressure (CSF opening pressure o60 mm H2O). CSF
Intracranial Neoplasm
leak is the most common cause of intracranial hypo-
In children aged 0–19 years in the United States and tension, and is frequently seen post-procedurally secon-
Canada, tumors of the brain and central nervous system dary to lumbar puncture or spinal surgery. However,
are the second leading cause of cancer-related death.43 intracranial hypotension in rare cases, may also present
In children aged 0–14 years with CNS tumors, glial due to an underlying CSF fistula, or may even occur
cell tumors including pilocytic astrocytomas (17%) spontaneously.
and brainstem gliomas (10%) are the most common.43 The classic presentation of a headache with intra-
Ependymomas and embryonal tumor derivatives such cranial hypotension is a headache that worsens within
as medulloblastoma may also be seen. minutes of a patient obtaining an upright posture
The headache pattern with space-occupying intra- (sitting and standing) and improves when the patient
cranial neoplasms is typically chronic and progressive, is supine, with or without additional symptoms such as
with headache worsening over time, as most pediatric photophobia, nausea, tinnitus, hearing changes, or
lesions localize infratentorially and may lead to neck pain. Diffuse meningeal enhancement may be
FIG 2. Updated diagnostic criteria for PTCS (Reprinted with permission from Sheldon et al.31)
54 Curr Probl PediatrAdolesc Health Care, March 2017
seen on neuroimaging and help support the ectopia on MRI, the more likely the child is to be
diagnosis.22 symptomatic.48
The majority of post-dural puncture headaches self- Surgical treatments of symptomatic Chiari I malfor-
resolve, however, if symptoms fail to improve, epi- mations may include sub-occipital craniectomy or
dural blood patching may help to resolve symptoms foramen magnum decompression procedures, and
when a leak is present with an early effect related to MRI with CSF flow studies may be useful to help
volume replacement, and a later effect due to sealing determine surgical necessity.44 A recent literature
off the leak. Although most patients with post-dural review suggested that of 179 pediatric patients ana-
puncture headache will improve after a single epidural lyzed post-operatively, improvement/resolution of
blood patch, those patients with spontaneous CSF headache was noted in 157 (88%).49
leaks may require multiple blood patches.45
Clinical Vignette #4
A 17-year-old girl presents to your outpatient clinic
Chiari I Malformation
for evaluation of refractory migraines. She has expe-
Radiographic diagnosis of a Chiari I malformation rienced on average three migraines per month over the
can be made with evidence of herniation of the last 2 years; however over the last 4 months, with the
cerebellar tonsils below the foramen magnum, with a onset of the school year, her migraines have increased
herniation of 45 mm considered pathologic in to almost daily. She was previously seen 4 weeks ago
patients older than 15 years, and a herniation by another provider in the practice and had an MRI
46 mm considered pathologic in patients younger brain with and without contrast performed which was
than 15 years.46 normal. There is a strong maternal family history of
In a population-based retrospective cohort study of migraine without aura and anxiety. She has been
children under 20 years diagnosed with Chiari I alternating tylenol and ibuprofen every 6 h over this
malformation, there were 51 patients identified with time without relief. She denies any recent fever, illness,
Chiari, which represented 1% of all children with head or stressors. She denies history of substance use. Her
or spine MRI imaging performed during the time of the neurologic examination is non-focal. Which of the
study. Of the 51 patients identified, 32 (63%) were following is most likely contributing to this patient's
symptomatic. The most common symptoms reported headaches? (Refer to clinical case discussion section
were headache (55%) and neck pain (12%), and older at the end of the section for answer).
age at diagnosis was found to be associated with an
increased risk of headaches. Syringomyelia was
Substance Use/Withdrawal
present in 12% at diagnosis. It is worth noting that
37% of children with Chiari I were diagnosed inci- Headaches may be caused by multiple substances
dentally after having been referred for imaging for including prescription medications. Examples of med-
other unrelated symptoms.47 ications that may induce headaches include amphet-
Headaches due to an underlying Chiari I malforma- amines, opioids, anti-microbials, immunoglobulin,
tion are usually occipital or sub-occipital, brief, lasting corticosteroids, and oral contraceptives.9
minutes, and worsen with neck flexion or Valsalva Screening for alcohol and drug use in teenagers is
maneuvers, coughing, or sneezing. Evidence of brain- critical, as illicit use of these substances may induce
stem and lower cranial nerve dysfunction may be seen headaches or serve as a headache trigger for those with
in addition to symptoms such as sensory disturbance, a primary headache diagnosis such as migraine.
upper extremity weakness, dizziness, or ataxia.22 It is Population-based studies have previously indicated
important to note that many children with Chiari I increased incidence of headaches in youth alcohol
malformations may also suffer from other headache and tobacco users compared to non-users.50
types such as migraine, so a careful headache history is Excessive caffeine consumption and/or withdrawal
crucial to help clearly define whether a patient's head- may also cause headaches in children. Hering-Hanit
aches are attributable to the Chiari I malformation, or and Gadoth have published on their population in a
may be due to another etiology. Some sources have tertiary headache clinic, where they identified 36
suggested that the greater the degree of tonsillar patients over a 5-year period with daily to near daily
Curr Probl PediatrAdolesc Health Care, March 2017 55
headaches related to excessive consumption of cola viral infections are the most common cause of pediatric
drinks, with patients consuming at least 1.5 L of cola headache presenting to the emergency department.57
per day equating to 192.88 mg of caffeine daily. Headaches are often described with infections such as
Gradual withdrawal from caffeine led to complete pharyngitis, acute otitis media, dental caries, and sinus
headache resolution in 33 of the 36 subjects.51 A disease.
recent study in adults also suggests that caffeine Special attention should be paid to sinus headaches
cessation may improve the acute treatment of migraine, as many patients diagnosed with sinus-type headaches
where a caffeine abstinence group was shown to have may in actuality have migraines. Cady and Schreiber58
better efficacy of acute migraine treatment compared to have previously published in the adult literature on the
a non-abstinence group.52 overlap between sinus headaches and migraines, and
demonstrated in a self-described patient population
with sinus headaches, 98% experienced headaches
Medication Overuse Headache (MOH)
fulfilling IHS criteria for migraines. Headaches with
The prevalence of MOH in children has been sinus disease tend to be frontal in location, dull, or
estimated at 0.3–0.5%, though studies have also pressure-like with symptoms including fever, facial
suggested up to 20–30% of children with chronic and dental pain, congestion, nasal purulence, and
headaches have medication overuse.53,54 impaired sense of smell. Per ICHD-III criteria, there
Medication overuse headache can be defined by any also needs to be clinical, nasal endoscopic, or imaging
one of the following: evidence confirming rhinosinusitis and a temporal
relationship between the headache and the develop-
1) Use of triptans, ergot alkaloids, combination ment of the rhinosinusitis.22 Presence of migrainous
analgesics or opioids 410 days per month. features (photophobia, phonophobia, nausea, vomiting,
2) Use of analgesics such as non-steroidal anti- and visual changes) is less typical. The presence of a
inflammatory medications (NSAIDs) 415 days positive Mueller sign on physical examination may
per month. also help to further confirm the diagnosis.59
3) Use of two or more headache medications 410 Intracranial infections such as meningitis, encephalitis, or
days per month. cerebral abscess are less common causes of acute headache
in pediatrics, but should be strongly considered in patients
Though not specific to the youth population, multiple presenting with headache, fever, altered mental status,
risk factors have been identified for medication overuse nuchal rigidity, photophobia, nausea, vomiting, and other
headache such as low socioeconomic status, high body focal neurologic symptoms.
mass index, depression, anxiety, and sleep disturbance.22,55
The treatment approach for management of MOH
Headache Due to Fasting
headache has been widely debated, partly due to the
lack of randomized, controlled studies. A recent Headaches associated with fasting are typically
systematic review of the literature, however, suggests diffuse, non-pulsating, and improve with eating.22
treatment with discontinuation of the offending med- Pediatric patients may be especially prone to this type
ication with the addition of a daily preventive medi- of headache due to picky eating habits or irregular meal
cation.56 Early patient counseling and education on schedules with school and activities. Many children go
medication overuse are the keys to prevention. More- without breakfast, may have a late lunch at school, or
over, previously published studies have indicated that may skip school lunch altogether if the foods are not
the majority of patients with MOH are in fact not aware appetizing. Adolescents will frequently diet for weight
that medication overuse may lead to headache worsen- loss purposes as body image takes on a more central
ing/chronicity,53 making detailed medication reconci- role in identity.
liation a critical part of each patient encounter.
Headache Attributed to Psychiatric Disorder
Infectious Causes
It has been estimated that up to 80% of children with
Headaches may occur secondary to a variety of headaches have comorbid psychiatric disorders, com-
childhood infectious processes, and in fact, febrile monly related to anxiety or depression,44 but are also
56 Curr Probl PediatrAdolesc Health Care, March 2017
seen with chronic pain syndromes or somatization in specialty care clinics, illustrating that estimates of
disorders. Increased psychosocial stress is commonly concussion incidence based on ED visits alone likely
linked to headache-related disability, and school- vastly underestimates the true scope of injuries in
related stress is frequently described particularly in children.
the adolescent population. For example, a study of high
school students found that 80% complained of head-
Pathophysiology
aches and over 40% had less than two unplanned hours
per day.7 School-related bullying is also a well-known The pathophysiologic processes underlying concus-
cause of headaches in children.6 The pathophysiology sion are complex. Traumatic biomechanical forces
underlying headaches attributed to stress, anxiety, and interrupt cell membranes leading to alterations in ionic
depression is likely multi-factorial involving mood gradients, neuronal depolarization, glutamate release,
dysregulation, sleep disturbance, and deconditioning depletion of intracellular energy stores, and a resultant
(lack of activity or activity avoidance). Individual state of brain energy crisis. Simultaneously, cerebral
coping skills and familial responses to headaches and blood flow decreases and distortion of axons causes
pain may also be contributory. For patients with overall impaired transport and connectivity. Excito-
headaches due to stress or psychiatric disorders, a toxic cell damage, inflammation, genetics, and psycho-
multi-disciplinary treatment approach is often useful in logic influences have all also been postulated in the
order to promote functional ability and regular school development of post-traumatic clinical syndromes.
attendance. Ultimately, the summation of these factors has led to
concussion now being considered a “brain network
Clinical Case Discussion (Vignette #3) injury.”22,62–64
Onset of an acute, severe headache described as the
“worst headache of life”, is classically indicative of a Definitions
vascular headache. Urgent non-contrast head CT According to the ICHD-3, a classification of post-
should be performed in order to rule out the presence traumatic headache is made both according to injury
of subarachnoid or intracranial hemorrhage. severity (mild, moderate, or severe) and duration of
headaches. An acute PTH is defined as headache onset
Clinical Case Discussion (Vignette #4) within 7 days of injury, regaining consciousness, or the
ability to report pain, as opposed to a persistent PTH
Medication overuse is common in children and with headache lasting 43 months.22
should be screened for at each visit. This patient's
use of daily ibuprofen and tylenol for four consecutive
months is consistent with this diagnosis and discontin- Clinical Presentation
uation of the offending medications should be recom- Multiple phenotypes of post-traumatic headache
mended, and consideration made to initiation of a daily have been described; however, symptoms of post-
preventive headache medication. traumatic headaches will often mimic those of chronic
migraine or chronic tension-type headache. Kuczynzki
Part V: Pediatric Post-Traumatic and Barlow in a prospective cohort study published in
Headache (PTH) 2013 described that in an outpatient pediatric TBI
clinic, 61% had daily headaches, 39% migraine, and
In the United States, mild traumatic brain injury is 9% with tension-type headaches. Of those patients
estimated to occur in 692 of 100,000 children younger studied with headaches 3 months following mTBI,
than 15 years,60 making it one of the most common 44% had migraine, 44% had daily headaches, and 26%
presenting complaints to the emergency department. had onset of a new-onset migraine-like disorder
Additionally, a recent publication by Arbogast et al.61 attributed to the mTBI.65 When there are additional
evaluating the point of healthcare entry for children complaints in a child with post-traumatic headache
with concussion found that in their population 81.9% such as sleep disturbance, cognitive symptoms, mood
of patients had their first visit for concussion within changes, or balance issues, a diagnosis of post-
primary care as opposed to 11.7% in the ED and 5.2% concussion syndrome may be considered.
Curr Probl PediatrAdolesc Health Care, March 2017 57
Although the vast majority of children who sustain a performed. Finally, patients with post-traumatic head-
mild traumatic brain injury will recover within a few ache are at particular risk for medication overuse
weeks, there is a subset of patients who continue to headache (MOH) and it is imperative that screening
experience persistent symptoms. In a study conducted and counseling regarding medication overuse occur
by Barlow et al.66 in 2010, 670 patients presenting to with each clinical visit.62 A previous study identified
the ED with mTBI were compared to 197 patients 70% of adolescents with chronic post-traumatic head-
presenting with extracranial injury (ECI). At 3 months, aches of 43 months' duration also met criteria for
11% of mTBI patients remained symptomatic as probable MOH.72
opposed to 0.5% of the ECI group. Similarly, Babcock
et al.67 found 29.3% of children aged 5–18 years in
Treatment/Management
their population were still symptomatic 3 months post-
concussion, with headache, fatigue, and frustration To date, there are no established guidelines for the
representing the most common symptoms. Although treatment of post-traumatic headaches and no random-
there are no reliable predictors for acute or persistent ized controlled trials evaluating the efficacy of medi-
post-traumatic headaches in pediatrics, multiple studies cations in treating pediatric post-traumatic headaches.
now suggest that certain pre-injury factors such as age, In a 2012 systematic review of interventions for post-
sex, or pre-injury physical or psychological problems traumatic headache by Watanabe et al.,73 they found in
are most important for those with prolonged symptoms reviewing all articles published 1985–2009, there was
lasting 43 months. Female sex, significant headache insufficient evidence to recommend or refute the
prior to injury, prior concussion with prolonged effectiveness of treatment for post-traumatic headache.
recovery, family history of headache, pre-existing Currently, the standard approach is to treat post-
mood or learning disorder, and significant symptoms traumatic headache similar to how one would a
in the setting of a questionable or very mild injury have migraine, tension, or chronic daily headache with a
also been identified as risk factors for persistent post- focus on lifestyle modifications (nutrition, hydration,
traumatic headache.68,69 A study conducted by Kirk- sleep hygiene), and use of abortive and preventive
wood et al.70 has also suggested that utilization of medications as indicated. Data is emerging to suggest a
symptom validity scales may be beneficial, with a potential role for melatonin for the treatment of
reported 12% of children seen for outpatient neuro- persistent post-traumatic headache and the Play
psychological evaluation after mTBI who were found Game Trial is ongoing ([Link]
to be exaggerating or feigning symptoms, as measured Kuczynski et al.65 have previously published positive
by failing the Medical Symptom Validity Test. results with the use of melatonin in children with
persistent PTH in doses of 3–10 mg, with significant
headache improvement noted in 9 out of 12 children
Evaluation
(75%). Another area of interest includes the use of
In assessing the pediatric patient with head injury, a peripheral nerve blocks for treatment. A recent retro-
comprehensive history and neurologic examination are spective chart review of patients (mean age of 15
essential in ruling out potential life-threatening etiol- years) with PTH indicated 64% of patients reported a
ogies such as traumatic fracture, cerebral hemorrhage, long-term response, improved quality of life, and
hydrocephalus, cerebral venous thrombosis, or carotid decreased post-concussion symptom scores following
artery dissection. The majority of pediatric patients occipital nerve block.74,75
presenting with mild traumatic brain injury, do not While there is limited evidence on the management
require neuroimaging, and may be closely observed, of post-traumatic headache, early education regarding
and guidelines for the use of CT imaging have mild traumatic brain injury and expected clinical
previously been established.71 Assessment of post- course has been shown to improve outcome. Ponsford
traumatic headache should include a detailed headache et al. previously published a study evaluating 61
history as previously outlined, including information patients with mild TBI who were assessed 1 week
on pre-injury headaches, and how the headache pattern and 3 months post-injury, as compared to a group of 58
has changed following injury. Screening for potential children who were only evaluated at 3 months post-
risk factors indicative of possible prolonged recovery injury. Each of these groups in turn was then compared
or persistent post-traumatic headache should also be to two control groups with minor, non-head-related
58 Curr Probl PediatrAdolesc Health Care, March 2017
injuries. Those children who were seen at 1 week were synonymous with a “return to play” with resumption of
given information and education regarding TBI inju- high risk/contact activities, and this latter decision should
ries, expected symptoms, and coping strategies, be made in the greater context of an individual's overall
whereas the 3-month follow-up group were not. Results recovery trajectory, neurologic examination, symptom
indicated that overall children burden, functional status, and risk
with mTBI reported more symp- for re-injury.
toms at 1 week following injury The use of prescribed cognitive While return to activity is
than the non-head injury controls, and physical rest following important and often the focus
and that the mTBI 3-month fol- for many patients, it should not
low-up group without the educa- concussion has been widely supercede school re-integration
tional intervention reported more debated, and perhaps fueled by and so-called “return to learn.”
symptoms and was more stressed concerns for the development of A clearly defined plan for the
3 months after injury.76 the so-called second impact patient and school in how to
The use of prescribed cognitive approach this is key, such that
and physical rest following con-
syndrome. Second impact patients have academic and envi-
cussion has been widely debated, syndrome has been described as ronmental accommodations in
and perhaps fueled by concerns the development of severe, place that help to facilitate
for the development of the so- diffuse cerebral edema when a school attendance amidst symp-
called second impact syndrome. tomatic recovery without caus-
second concussive injury is
Second impact syndrome has ing undue stress and anxiety82–84
been described as the develop- sustained shortly following an (Table 5).
ment of severe, diffuse cerebral initial injury where the symptoms A multi-disciplinary treatment
77
edema when a second concussive have not fully resolved. There is approach is recommended in the
injury is sustained shortly follow- literature to suggest that a short setting of persistent post-
ing an initial injury where the traumatic headache including
symptoms have not fully period of rest in the acute phase physical rehabilitation strategies
resolved.77 There is literature to of concussion recovery may be and pain-focused cognitive
suggest that a short period of rest beneficial78; however, more pro- behavioral therapy.75,85
in the acute phase of concussion longed activity restriction beyond
recovery may be beneficial78;
however, more prolonged activity
the first few days may place
patients at risk for prolonged Part VI: Treatment
restriction beyond the first few
days may place patients at risk for symptoms and psychologic Goals of long-term migraine
prolonged symptoms and psycho- sequela such as depression treatment include “reduction of
headache frequency, severity, dura-
logic sequela such as depression
79 or anxiety.79 tion, and disability; reduction of
or anxiety. In a landmark study
conducted by Thomas et al. in reliance on poorly tolerated, inef-
2010, strict verse limited rest was evaluated in patients fective, or unwanted acute pharmacotherapies; improve-
aged 11–22 years presenting to the ED within 24 h ment in quality of life; avoidance of acute headache
following a concussion. Patients were randomized to strict medication escalation; education and enablement of
rest for 5 days or usual care (rest for 1–2 days followed by patients to manage their disease to enhance personal
gradual increase in activity). However, results indicated control of their migraine; and reduction of headache-
that the intervention group of strict rest reported more daily related distress and psychological symptoms”.86
post-concussive symptoms (total symptom score over 10
days, 187.9 vs 131.9, P o 0.03) and slower symptom
Clinical Vignette #5
resolution compared to the limited rest group.80 Studies
have also shown that individuals who engaged in a sub- A 17-year-old boy presents to your office with
threshold exercise program experienced overall improve- greater than 15 migraines per month causing signifi-
ment in post-concussive symptom scores.81 It should be cant disability related to missed school days and
noted, however, that a “return to activity” is not activities. He has been using ibuprofen 600 mg PRN
Curr Probl PediatrAdolesc Health Care, March 2017 59
for abortive therapy, however, is now having to take it reviewed. A brief explanation is provided that sleep
multiple days per week. Other past medical history is changes and missed meals may result in decreased
notable for obesity and asthma. His neurologic exami- availability of brain energy, which causes compensa-
nation is stable from previous assessments. What is the tory increased blood flow to the brain through vaso-
next best step in management for this patient? (Refer to dilation and resultant head pain.
clinical case discussion section at the end of the Exercise goals should be specified based on standard
section for answer). practice, including a focus on aerobic exercise to help
increase oxygen carrying capacity of red blood cells
and increasing tone in blood vessel walls.
Lifestyle Modification
In these authors' experience, these simple habits are
A variety of non-pharmacologic complementary and successful in the treatment of many children with
integrative treatments are utilized in the management migraine headaches and often will avoid the necessity
of migraine headaches in children and adolescents. All for additional prescription therapy. A simple strategy
patients should be educated about the basic underlying utilized by a number of tertiary headache specialty
mechanisms of migraine to provide a better under- centers in discussing migraine with families is listed in
standing as to why they experience headache recur- Figure 3.
rently. Concerns about the fear of brain tumors,
aneurysms, and other forms of secondary headache
Behavioral Strategies
should be addressed during the visit, and families
should be given time to ask questions because parents Many patients with headache experience significant
and adolescents often have significant anxiety about exacerbation of headache during times of emotional
having a more serious secondary headache condition. and cognitive stress, especially those with chronic
Healthy lifestyle habits should be a focus in migraine. Those patients with exacerbation of head-
the treatment plan, including adequate hydration, ache in stressful situations may have a robust response
avoidance of potential triggers to behavioral therapy. Behavio-
including caffeinated beverages ral therapies require a specialist
and missed meals, and regular Many patients with headache trained in pain medicine, most
sleep patterns and exercise. Spe- often a psychologist or thera-
experience significant
cific fluid goals based on patient pist, and must have a broad skill
weight and instructions about exacerbation of headache set that includes biofeedback,
drinking “maintenance fluids” during times of emotional and pain-focused cognitive behavio-
should be provided to act as a cognitive stress, especially those ral therapy (CBT), and relaxa-
guide to patients and families. with chronic migraine. Those tion therapy. Through the use of
An explanation is provided to self-administered treatment
the patient including a simple
patients with exacerbation of plans using techniques of bio-
theory that fluids may help by headache in stressful situations feedback and CBT, patients
increasing blood volume, result- may have a robust response to are able to slow heart rate and
ing in improved oxygen supply behavioral therapy. decrease sympathetic activa-
to the brain. Avoidance of caf- tion during painful crises,
feine and artificial sweeteners is allowing them reduce pain
standard practice in headache specialty centers due to and disability. Additional coping skills are taught
their potential to exacerbate headache conditions. to children and their families, allowing them to
The National Sleep Foundation guidelines on sleep participate in regular activities, avoid secondary
duration for children, based on age, should be pro- anxiety, and reduce overall stress and disability
vided, with a focus on specific sleep duration, avoid- related to migraine.88
ance of poor sleep hygiene including significant Alternative treatments including massage, chiroprac-
circadian changes on weekends or holidays, and sleep tic or osteopathic manipulation, and acupuncture show
timing should be addressed.87 Avoidance of skipped promising results; however, they have not been con-
meals and caloric deficits and instructions on main- sistently shown in randomized and controlled studies
taining a healthy and well-balanced diet should be in children demonstrating their efficacy.
60 Curr Probl PediatrAdolesc Health Care, March 2017
TABLE 5. Return to learn (Reprinted with permission from source Master et al83)
Stage Activity Objective
No activity Complete cognitive rest—no school, no homework, no Recovery
reading, no texting, no video games, and no computer work.
Gradual reintroduction of Relax previous restrictions on activities and add back for Gradual controlled increase in subsymptom threshold
cognitive activity short periods of time (5–15 min at a time). cognitive activities.
Homework at home Homework in longer increments (20–30 min at a time). Increase cognitive stamina by repetition of short periods of
before school work at self-paced cognitive activity.
school
School re-entry Part day of school after tolerating 1–2 cumulative hours of Re-entry into school with accommodations to permit
homework at home. controlled subsymptom threshold increase in cognitive load.
Gradual re-integration Increase to full day of school. Accommodations decrease as cognitive stamina improves.
into school
Resumption of full Introduce testing, catch up with essential work. Full return to school; may commence Return-to-Play protocol
cognitive workload (see Step 2 in Table 2).
Abortive Medications and reduce their activation and irritability.90 Alteration
of the inflammatory pain cascade in migraine via
All patients with migraine should be given instructions triptans has been shown to decrease its associated
on appropriate use of abortive medications regardless of severity, duration, and disability. Triptans have not
frequency. Abortive treatments for migraines in children been shown to decrease head pain in other headache
include the non-steroidal anti-inflammatory agents disorders, so its effectiveness is considered to be
(NSAIDs), analgesics, and 5-hydroxytryptamine receptor diagnostic of migraine. Their side effects are relatively
agonists, commonly referred to as triptans. Similar among common but typically well tolerated, including tight-
each agent is the necessity of early administration and ness of the chest, tingling hands and feet, and nausea.
appropriate dosage in order to be effective. Whenever Those individuals at risk for stroke or other vascular
providing an abortive agent, education should immediately events due to constricted vessels, such as atheroscle-
be provided about the risk for medication overuse head- rosis, should not be given triptans due to a theoretical
ache, which may occur in those individuals using NSAIDs risk of stroke. For reasons related to trial design rather
or analgesics Z15 days per month for a 3-month period, than scientific evidence, basilar-type migraine is con-
or those using triptans Z10 days per month for a sidered a contraindication to triptans but has not been
3-month period.22 proven in a systematic manner. In fact a small report in
The most commonly used agent as a migraine 2001 on 13 patients with basilar-type migraine dem-
abortive is the NSAID ibuprofen at a dose of 10 mg/ onstrated no adverse effect.91
kg/dose. NSAIDs are thought to be effective through In the emergency and inpatient hospital setting, many
the inhibition of cyclooxygenase, thereby preventing other agents are used intravenously for abortion of an
downstream inflammatory prostaglandin production. acute or prolonged migraine, including dopamine
Its onset of action is within 30 min of administration, receptor antagonists, such as prochlorperazine, ketor-
and ibuprofen results in a significant reduction in head olac, divalproex sodium, fluid boluses, and dihydroer-
pain at 2 h in 50–75% of migraine patients given a gotamine. The combination of prochlorperazine and
weight appropriate dose, compared to only 36–53% of ketorolac has been shown to be superior in reducing
patients on placebo.89 Acetaminophen at a dose of migraine intensity by more than 50% than either agent
15 mg/kg results in similar improvement to ibuprofen, alone.92 Divalproex sodium, when given in a rapid
possibly with a faster onset of action.89 infusion of 20 mg/kg to a maximum of 1 g over
Triptans are agents that activate 5-hydroxytryptamine 10–15 min, has been shown to reduce migraine intensity
1B and 1D receptors located in both the central 39% and 57% with one or two doses, respectively.93
trigeminal nucleus caudalis and peripheral trigeminal
afferents, resulting in cerebral vasoconstriction via
Preventive Medications
activation of smooth muscle cells in vessel walls.
Direct effects of triptans on brainstem trigeminal nuclei Preventative medications are indicated for patients
are also thought to centrally mediate pain nociceptors who have significant disability related to their
Curr Probl PediatrAdolesc Health Care, March 2017 61
headaches, whether due to interference with academic Amitriptyline, a tricyclic antidepressant, has been
or extracurricular activities, interference in activities of shown to be effective at doses of 1 mg/kg/day, result-
daily living such as socialization with family and ing in a statistically significant decrease in headache
friends, or due to significant neurologic symptoms severity and frequency when compared to pretreatment
associated with headache such as migraine with motor data.94 Most patients tolerate amitriptyline well, with
or speech aura. There is no consensus regarding the the most common side effects being dry mouth and
number of headaches that a patient should have before sleepiness, which is why it is often taken at bedtime to
being offered preventative medications due to varia- help with sleep maintenance, which appears to be
bility in parental, patient, and physician comfort with abnormal in many patients with migraine.
each medication and their potential side effects. The antiepileptic medications divalproex sodium and
A number of preventative medications have been topiramate are both frequently used for migraine and
studied and used for primary headache disorders such headache prevention. Valproic acid can be given in
as TTH and migraine; however, there is significant both oral and intravenous forms to abort or prevent
variability among studies regarding efficacy, primarily migraine, and data shows significant reduction in
due to high placebo rates and poor study design. The headache frequency, duration, and severity when
American Academy of Neurology Practice Parameter compared to baseline characteristics.93,95 Particular
for the treatment of migraine in children and adoles- caution must be taken in adolescent females who have
cents has compiled studies based on their quality, with an elevated risk of developing polycystic ovarian
prospective, randomized, double-blinded, controlled syndrome and due to divalproex sodium's teratogenic-
trials considered as the gold standard, known as level ity in patients who become pregnant.
A evidence.86 No medications approved by the FDA Topiramate has been shown in pediatric and adoles-
fulfill the criteria for level A evidence, although the cent patients to decrease migraine frequency, duration,
calcium channel blocker flunarizine, available outside severity, and headache disability when compared to
of the United States, has been shown to be effective in baseline characteristics.96 Based on pediatric data, the
migraine prevention at this level. most effective dose of topiramate is 100 mg per day,
The most commonly used agents in the United States which may be given once or divided twice daily, and
include amitriptyline, divalproex sodium, topiramate, requires at least 4 weeks on this dose to separate from
and levetiracetam, which the AAN considers to placebo in trials. The largest ever multi-center random-
have insufficient evidence to make recommendations ized, placebo-controlled trial comparing placebo to
for based on a lack of retrospective data showing topiramate and amitriptyline, the Childhood and Ado-
efficacy. lescent Migraine Prevention study, utilized topiramate
1. Discuss the criteria for migraine and specify which are fulfilled by the child’s symptoms. A
standardized handout may be utilized for this.
2. Discuss migraine pathophysiology to explain to the child and family that increased brain
needs, whether due to dehydration, sleep disruptions, poor caloric intake, barometric
weather fronts, emotional stress, increased cognitive demands, result in vasodilation to the
brain, which results in activation of the V1 subdivision of the trigeminal nerve causing head
pain.
3. Discuss the treatment strategy for the patients migraine, including separate foci on lifestyle
modification, behavioral strategies, abortive therapies, and preventative therapy when
indicated. A standardized handout with clear delineation of each of these areas can act as a
self-administered treatment plan at home.
4. Discuss the indications for diagnostic testing and whether the patient fulfills these or not. If
the patient does not require testing, discuss the historical finding and exam findings that are
reassuring and make the likelihood of more serious central nervous system pathology
unlikely.
5. Provide appropriate contact information for interim support for headaches. Consider
providing a specific emergency room protocol for status migrainosus.
6. Provide specific follow up timing.
FIG 3. Step-wise approach to explaining migraine and a treatment plan to patients and families.
62 Curr Probl PediatrAdolesc Health Care, March 2017
at 2 mg/kg dosing with results pending at the time of 10. Papetti L, Capuano A, Tarantino S, et al. Headache as an
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include weight loss, paresthesias, and cognitive Rep 2015;19(3):3.
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slowing.
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Although randomized controlled trials are of limited aches: report of the Quality Standards Subcommittee of the
quality or lacking or in terms of efficacy data, they are American Academy of Neurology and the Practice Committee
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