Pediatric Headaches
December 1, 1999 35 minutes read
Pediatric Headaches
Author: Raymond D. Pitetti, MD, Assistant Professor, Division of Pediatric Emergency Medicine, Department of Pediatrics, Children’s Hospital of Pittsburgh and the University of Pittsburgh School of Medicine.
Peer reviewer: Martha S. Wright, MD, Associate Professor of Pediatrics, Case Western Reserve University School of Medicine, Cleveland, OH; Associate Director, Pediatric Emergency Medicine, Rainbow Babies and Children’s Hospital, Cleveland, OH.
Children frequently present to the emergency department complaining of a headache. Most parents fear their child’s headache is caused by a brain tumor. However, headaches rarely indicate a serious underlying illness in children. Careful attention to historical information, a thorough physical examination, and selective use of laboratory and radiographic tests will often diagnose the cause of the headache. At the very least, it should serve to persuade the child, the parents, and the clinician that no serious condition exists.
The author presents a comprehensive review of headaches in children, paying particular attention to the history and physical examination of the child with a headache. He describes common causes of headaches in children as well as recommended management.
— The Editor
Introduction
Children commonly present to the emergency department complaining of a headache. It has been estimated that by age 7, 40% of children will have experienced a headache.1,2 By age 15, close to 75% of children will have complained of a headache.1,2 Approximately 1 million children and adolescents suffer from migraine headaches, and several hundred thousand school days are missed each month as a result of pediatric migraine alone.3
A headache in a child can cause extreme anxiety in both parents and children. Parents will often bring their child to the emergency department seeking assurances that the headache is not a sign of intracranial disease. Although the concern may not be verbalized, in their minds, the headache must be "due to" a brain tumor. Even clinicians, who know that most headaches in children are not representative of a serious illness or condition, often share the same concern. Though it is sometimes difficult to identify the underlying cause of a headache in a child, particularly in the emergency department, a carefully obtained history and physical examination can be used, in the majority of cases, to assure the parents that serious intracranial pathology would be unlikely.
Other parents will come to the emergency department with a child who suffers from recurrent headaches. One of the most common referrals to a pediatric neurology clinic is the child with chronic headaches. Such headaches are rarely indicative of a serious underlying illness or condition, but can be frustrating for the child and the family.
It is important to remember that a headache is merely a symptom of an underlying problem. The clinician must determine, based on history, physical examination, and selective use of diagnostic studies, the most likely cause of the headache. He or she must distinguish between those headaches that are merely painful and those that represent a more serious concern. This review will describe the approach to the child with headache as well as the common types of headache in children and their management.
Sources of Pain
Understanding the pain sensitive structures located inside and outside the skull, and understanding where that pain will be felt, can aid the clinician in determining the underlying cause of a headache. The cerebral and dural arteries and the large veins and venous sinuses are the major pain sensitive structures located within the skull.2,4 Vasodilatation, inflammation, and traction-displacement of these structures will result in pain.4 The brain parenchyma, its ependymal lining, and the meninges are insensitive to pain.4
Painful impulses from supratentorial intracranial vessels are transmitted by the trigeminal nerve, while the first three cervical nerves transmit impulses from infratentorial vessels.1,4 The ophthalmic division of the trigeminal nerve innervates arteries in the superficial portion of the dura and refers pain to the eye and forehead.4 The second and third divisions of the trigeminal nerve innervate the middle meningeal artery and refer pain to the temple.4 All three divisions of the trigeminal nerve innervate the cerebral arteries and refer pain to the eye, forehead, and temple.4 Pain from structures located in the posterior fossa will often be referred to the occiput and neck.4
Major pain sensitive structures located outside the skull that can cause headache include the cervical roots, cranial nerves, extracranial arteries, the muscles attached to the skull, and the periosteum of the sinuses.4 Cervical root and cranial nerve pain is usually caused by mechanical traction due to injury or malformation and will follow the nerve distribution.4 Cervical root pain will refer to the neck and back of the head up to the vertex, while cranial nerve pain will refer to the face.4 Extracranial arteries, such as major scalp arteries found around the eye, forehead, and temple, can produce pain when dilated or stretched.4 Muscles attached to the skull, such as the neck extensors, the masseter muscles, and the frontalis muscle, can become painful when placed under prolonged contraction.4 While cranial bones are insensitive to pain, the periosteum of the sinuses and sockets of the teeth become painful when inflamed.4
History
Though historical clues are often diagnostic of the cause of headaches in adults, young children are rarely able to describe the type of pain they are experiencing or how often they are experiencing it. However, ascertaining how often the child has missed school or stopped playing because of headaches may provide a more objective measure of the frequency and severity of the headaches. In addition, asking how many different types of headaches the child has may also provide clues to their severity. Children often respond that they have two kinds of headaches: the "bad" kind of headache of which the child will complain spontaneously and the "mild" kind of headache of which the child will complain only when asked. Further questioning should focus on the bad headache. Children older than 10 years are more likely to provide helpful responses to traditional historical questioning about headaches.
Several headache patterns may emerge during questioning of parents and children that can help in diagnosing the source of the headache. Temporal pain is usually vascular, while a steady, tight pain, in a band-like distribution, or concentrated over the occiput, is usually indicative of a musculoskeletal origin. Cervical root and cranial nerve pain is often described as radiating or shooting in quality. Intermittent headaches associated with nausea and a complete recovery between attacks usually indicate migraine headaches.
Headaches of recent onset are of greatest concern. Acute onset of a severe headache, unlike anything experienced before, without a full recovery, is more likely to indicate intracranial disease. In contrast, a continuous low intensity chronic headache, in the absence of systemic signs and symptoms, is not likely to indicate intracranial disease.
Most headaches in children are bilateral. Unilateral headaches are more suggestive of either migraines or intracranial mass lesions. It is important to note that migraine headaches in younger children tend to be bilateral, unlike in adults, for whom most migraine headaches are unilateral.5
Noting the time of day the headache occurs can also be helpful. Tension headaches are usually experienced later in the day while migraines can occur at any time. Headaches caused by increased intracranial pressure are usually most severe in the morning before the child has risen and improve when the child stands. Noting when the headache first began may also be helpful. The date of onset of the headache may be associated with a stressful event in a child’s life. A headache that has lasted for months or years, has not changed in severity or frequency, and is not associated with neurologic abnormalities is unlikely to indicate a serious illness or condition.
Social and family histories can help identify significant stresses in the child’s life or provide further clues to the underlying cause of the headache. A complete history detailing medication use should be obtained — tetracycline, nitrofurantoin, hypervitaminosis A, retinoic acid and withdrawal of steroids have all been associated with pseudotumor cerebri — as well as the results of any previous diagnostic tests, such as electroencephalography or computed tomography. Historical questions that are most helpful in diagnosing the cause of headaches in children are shown in Table 1.
| Table 1. Historical Data |
| • Age at onset of headaches |
| • Number of different kinds of headaches |
| • Associated features |
| • Factors that precipitate the headaches |
| • Factors that relieve the headaches |
| • Frequency and duration of the headaches |
| • Length of illness |
| • Location of the headaches |
| • Time of day of onset of the headaches |
| • Associated neurologic signs or symptoms |
| • Medications and dosages used to relieve headaches |
| • Family history of headaches |
Physical Examination
A complete physical examination, including a detailed neurologic evaluation, should be performed in every child who presents with a complaint of a headache. However, the vast majority of children will have a normal physical examination, including a normal neurologic evaluation.
Measurements of growth parameters, head circumference, and blood pressure should be included in the physical examination. Particular attention should be paid to the teeth and sinuses, and a fundoscopic examination should be performed to look for papilledema. The temporal area should be auscultated with the bell of a stethoscope to listen for cranial bruits. A unilateral bruit in this region may indicate an arteriovenous malformation. Neurologic examination should include an assessment of the child’s affect, mood, development, gait, and level of awareness. Particular attention should be paid to the assessment of cerebellar function because of the prevalence of posterior fossa tumors in childhood. Visual acuity should be measured. The visual disturbances of migraine will usually precede a headache and normally last less than an hour.5 Defects in visual acuity or fields of vision that persist after the headache resolves should raise suspicion of serious pathology. Careful attention should be paid to the child’s level of concern about the headache, the family’s level of concern, and interactions between family members in the emergency department.
Laboratory and Radiological Evaluation
Laboratory tests are rarely indicated or helpful in the child with a headache. In the presence of an unremarkable history and normal physical examination, laboratory tests will rarely reveal significant organic disease. Laboratory tests should only be obtained if concerns for a mass lesion, an intracranial bleed, or increased intracranial pressure are raised during the history and physical examination.
Electroencephalograms are usually not helpful in diagnosing children with headaches. Some children with migraine headaches may have an abnormal electroencephalogram, but the findings are often nonspecific and of limited value. Electroencephalograms should be reserved for those children with a history of episodic loss of consciousness and headaches or in children with complicated migraine.
Sinus films may be helpful in the child with suspected sinusitis, although most cases of sinusitis can be diagnosed by examination alone. Computed tomographic (CT) examination of the sinuses has been recommended as the diagnostic procedure of choice to dilineate sinus disease. Lumbar puncture with measurement of opening pressure may be helpful if pseudotumor cerebri is suspected. However, in such cases, a CT scan of the head should be considered prior to lumbar puncture to identify patients with obstructive hydrocephalus, an intracranial mass lesion, or cerebral edema. Such patients are theoretically at increased risk for tonsillar herniation following lumbar puncture.
Neuroimaging is often obtained during the evaluation of a child with a headache, but is rarely useful. One study retrospectively reviewed the medical records of 133 children referred to a pediatric neurology clinic for evaluation of headache.6 Fifty-nine percent of patients had an MRI or CT scan. Cerebral abnormalities were found in four patients, but in each case the abnormality was deemed unrelated to the headache. Neuroimaging studies are not indicated in the child without signs or symptoms of intracranial pathology. Imaging studies may be indicated in children with migraine equivalents, when headaches are consistently on the same side of the head, or in chronic progressive headaches.2 In such cases, magnetic resonance imaging (MRI) is the study of choice because of its ability to detect arteriovenous malformations and low grade tumors.2 If increased intracranial pressure or an acute intracranial lesion is suspected, a non-contrast CT head scan should be performed. A CT scan of the head will demonstrate nearly all acute structural causes of headache.1 Table 2 lists those signs and symptoms which, if present in a child with a headache, should lead to further diagnostic testing.
| Table 2. Signs or Symptoms that May Require Neuroimaging |
| • Papilledema |
| • Sixth nerve palsy |
| • Ataxia |
| • Other signs of elevated intracranial pressure |
| • Severe nocturnal headaches |
| • Recurrent occipital headaches of increasing frequency and intensity |
| • Occurrence of seizures |
| • Changes in severity of pain with coughing or changes in body position |
| • Onset following head trauma |
| • Periodic obtundation |
| • Rapid increase in pain over days or weeks |
| • Sudden onset of severe headache |
Treatment
In treating a child with a headache, emphasis should be placed on excluding serious intracranial pathology, reassurance, the removal of precipitating factors, and simple analgesics. Aggressive pharmacological management should be avoided whenever possible. Often providing parents and children with reassurance that the headache does not represent a brain tumor or any other serious disorder will decrease family anxiety and symptoms. This review will provide more specific treatment options as each cause of headache is discussed.
Classification of Headaches
Headaches can be classified based on etiology, pathology, and symptom complex. However, classifying headaches according to their temporal pattern may be the most useful to the clinician. This review will focus on five recognized temporal patterns of headaches in the pediatric patient: acute, acute recurrent, chronic progressive, chronic nonprogressive, and mixed.
Acute Headaches
An acute headache is defined as a single event with no history of a previously similar event. While an acute headache may be the first manifestation of a migraine, the most frequent cause of such a headache is an infection, such as a viral illness or sinusitis. In a patient who is critically ill or presents with abnormal neurologic symptoms or signs, meningitis and subarachnoid hemorrhage should be considered. Other causes of an acute headache include dental infections, systemic hypertension, and seizures.
Febrile Illness
Fever is the most common cause of a vascular headache. Bacterial and viral infections stimulate the release of vasoactive substances (cytokines, prostaglandins, etc.), that can cause painful vasodilatation.7 The degree of vasodilatation will parallel the rise in body temperature and occurs both intracranially and extracranially. The headache is often described as bitemporal or diffuse and throbbing in quality. With return of normal body temperature, the headache will often resolve.
Intracranial Infections
Children with meningitis or encephalitis may present with a headache. Infectious meningitis is often one of the first diagnostic considerations in the febrile child who presents with a headache. A diagnostic lumbar puncture should be considered if a child has fever, meningismus, lethargy, photophobia, an altered level of consciousness, signs of systemic infection, or vomiting. Lumbar puncture should be deferred in patients with papilledema, focal neurologic deficits, or cardiac pathology with left to right shunts (these patients are at increased risk for septic embolic events leading to cerebral abscess formation) pending neuroimaging studies. Appropriate antimicrobial therapy should be started immediately and should not be delayed in order to obtain a lumbar puncture.
Encephalitis may present in a similar manner to meningitis. Children may complain of a generalized headache of rapid onset that is associated with fever, confusion, altered level of consciousness, meningismus, focal neurologic signs, or seizures. Following appropriate neuroimaging, a lumbar puncture should be performed in these patients and antimicrobial therapy initiated pending culture results.
Dental Infections
Dental problems can present as a temporal headache. The periosteum near the teeth can become extremely painful when inflamed. Often, patients will complain of localized tenderness at the site of inflammation and a frontal or temporal headache. Dental caries or gingivitis can be diagnosed on physical examination. Such patients should be treated with simple analgesics or acetaminophen with codeine, and placed on an appropriate antimicrobial agent until they can be seen by a dentist.
Sinus Infections
Sinusitis is a localized inflammatory disorder of the sinuses, often due to bacterial infection. In older children, sinusitis is typically associated with fever, cough that worsens at night, focal tenderness over a sinus, and purulent rhinorrhea. In younger children, the diagnosis of sinusitis may be more difficult to make. The clinician should look for more subtle signs such as persistence of rhinorrhea for more than 10 days, malodorous breath, or morning eye swelling. Sinusitis is frequently blamed for frontal headaches in children. However, frontal headaches occur in only a small group of children with sinus infection. Treatment of sinusitis consists of appropriate antimicrobial agents and simple analgesics.
Subarachnoid Hemorrhage
Subarachnoid hemorrhage is a rare but serious cause of headache in children. Subarachnoid hemorrhages can result from head trauma, rupture of an arterial aneurysm, or bleeding from an arteriovenous malformation.8
Head trauma is a major cause of intracranial hemorrhage throughout childhood.8 It can be associated with intracerebral hemorrhage, subarachnoid hemorrhage, subdural hematoma, and epidural hematoma. Increased intracranial pressure is a constant feature of intracranial hemorrhage, and children will present with related signs and symptoms.
Arterial aneurysms represent vestiges of the embryonic circulation and are present in a rudimentary form before birth.8 Arterial aneurysms rarely rupture during infancy. Most children with an arterial aneurysm become symptomatic after age 10.8 The first manifestation of an arterial aneurysm is often a subarachnoid hemorrhage. Children can present with catastrophic symptoms, such as loss of consciousness, hypotension, and evidence of increased intracranial pressure, or more commonly with a warning leak.8 Severe headache, stiff neck, and low-grade fever characterize a subarachnoid hemorrhage or warning leak. Occasionally, neurologic signs can be present due to pressure exerted on adjacent cranial nerves. The oculomotor nerve is most frequently affected.8
Arteriovenous malformations (AVM) account for 9% of subarachnoid hemorrhages at all ages but make up a larger share in childhood.8 Approximately 90% of AVMs are supratentorial and 10% are infratentorial.8 Symptoms of bleeding from an AVM may begin as early as age 5.8 Hemorrhage is into the parenchyma of the brain in two-thirds of cases and into the subarachnoid space in one-third.8 Symptoms due to bleeding from an arteriovenous malformation often occur over a period of hours and are characterized by sudden severe headache, neck stiffness, and vomiting. Fever is often present along with focal neurologic deficits.
Children who present with signs and symptoms suggestive of subarachnoid hemorrhage should first undergo noncontrast/contrast CT scan of the head. Since a small percentage of subarachnoid hemorrhages may be missed on CT, a subsequent LP is required. Lumbar puncture will reveal grossly bloody fluid. However, clinicians will often attribute blood in the spinal fluid to a "bloody tap." In such cases, centrifuged cerebrospinal fluid should be examined for the presence of xanthochromia. Children suspected of having an AVM should undergo a neurosurgical evaluation and subsequent MRI/4-vessel arteriography.
The initial goals of therapy in patients with subarachnoid hemorrhage due to a ruptured arterial aneurysm include the prevention of early rebleeding and cerebral ischemia due to arterial spasm. Surgery is recommended to prevent rebleeding. Calcium channel blockers can be administered to prevent arterial spasm. A combination of embolization and surgical ligation of feeding arteries are used to manage children who have a subarachnoid hemorrhage due to bleeding from an arteriovenous malformation.8
Systemic Hypertension
There is no evidence chronic hypertension results in a persistent headache. However, an acute rise in systemic blood pressure can result in headache.4 The headache is often characterized as explosive and throbbing. Controlling systemic blood pressure may result in symptomatic relief, however, if the patient has intracranial hypertension and their cerebral perfusion pressure is dependent upon the elevated blood pressure, a sudden drop in blood pressure could result in cerebral ischema.
Seizures
Headache as the sole manifestation of a seizure is uncommon.2 Less than 1% of children with seizures will report headache as their only symptom of seizures.9 However, children will often complain of a headache during the postictal phase following a generalized tonic-clonic seizure. This is thought to result from vasodilatation of the cerebral arteries.
An association between seizures and migraine headaches has been observed.2 Seizures have been reported in 3-11% of children with migraine and an increased incidence of seizures in patients with migraine over the general population has been reported.2,10 In some patients who suffer from both epilepsy and migraine, one disorder can trigger the other.
Acute-Recurrent Headaches
Acute-recurrent headaches appear and disappear and recur at a later time. Most children who present with acute-recurrent headaches have migraines. Other causes of acute-recurrent headaches include hypertension, vascular malformations, cluster headaches, and sinusitis.
Migraine
Migraine headache is an autosomal dominant hereditary disorder which is inherited with incomplete penetrance. Migraine headaches are the most common cause of acute recurrent headaches in children.1,4,11 A history of migraine is often documented in at least one parent in 90% of cases. A family history of motion sickness is also common, as well as a history of the child suffering from motion sickness, fainting spells, sleep talking, or sleep walking.12,13 The prevalence of migraine headache in children younger than age 7 has been estimated to be 2.5%, with both sexes equally affected.14,15 The prevalence of migraine headache increases to 5% in children aged 7 years to puberty, with a female to male ratio of 3:2. About 5% of postpubertal boys and 10% of postpubertal girls are thought to suffer from migraine headaches.14,15 Prevalence rates are thought to be higher in postpubertal girls than in boys because of the triggering effect of the menstrual cycle on migraine headaches.4
Children with a predisposition to migraine often have triggering factors that provoke attacks. Such triggers include stress, exercise, head trauma, and the premenstrual decline in circulating estrogen.4 Other factors include humidity, changes in altitude, and changes in the weather or barometric pressure.16 Certain medications, foods, upper respiratory illnesses, distinctive odors, and allergies may also trigger migraine headaches.16 The diagnosis of migraine headache in children is based on an interpretation of clinical signs and symptoms.
Classic Migraine
While classic migraine is the best known form of migraine, it occurs in less than one-third of children with migraine headaches.17 Historically, classic migraine has been described as a biphasic event. It has been postulated that, during the initial phase of a migraine headache, a wave of cortical excitation followed by cortical depression spreads over both hemispheres from back to front, in association with decreased regional blood flow and transitory neurologic disturbances.4 The second phase is thought to consist of increased blood flow in both the internal and external carotid circulation. Headache, nausea, and vomiting occur during the second phase.
Symptoms may occur: 1) only during the first phase, resulting in what is termed a migraine equivalent; 2) during the second phase, resulting in headache and vomiting; or 3) during both phases. The most common symptoms to occur during the first phase of a migraine headache consist of visual aberrations, such as the perception of dancing lights, blind spots, blurred vision, visual hallucinations, or transitory blindness. Visual symptoms tend to be specific for each child suffering from migraine headaches.
Migraine headaches may terminate after the first phase without headache or proceed to the second phase with symptoms of headache and nausea. Headaches tend to be dull at first, becoming throbbing, pulsating, or pounding. The headache is unilateral in two-thirds of older children and adult patients and most intense in the region of the eye, forehead, or temple. However, younger patients with migraine headaches will often complain of bilateral temporal pain. Eventually, the headache becomes constant and diffuse, lasting a variable length of time. Anorexia and photophobia are often present.
Recent investigators have proposed a neuronal hypothesis to describe the pathogenesis of migraine headache. Most current investigators of migraine headaches accept this theory.18,19,20 The hypothesis considers migraine headache as an inherited sensitivity of the trigeminal vascular system. Cortical, thalamic, or hypothalamic mechanisms will initiate an attack due to some internal or external stimuli. The locus ceruleus and nucleus raphe dorsalis are stimulated, which in turn stimulate the cortex via serotoninergic and noradrenergic pathways, producing a spreading wave of neuronal depression. The cranial vasculature is also stimulated, resulting in neurogenic inflammation and secondary vascular reactivity. Released vasoactive peptides stimulate endothelial cells, mast cells, and platelets, creating a cascade that results in sterile inflammation of dural and pial blood vessels from which nociceptive afferents transmit centrally via the trigeminal nerve. Gaining a better understanding of the pathogenesis of migraine headaches has direct implications for abortive and prophylactic treatment of migraine headaches.
Most children who suffer from migraine headaches will have 1-4 attacks a month. However, children may be symptom free for long intervals and then suffer from a cluster of attacks.
Common Migraine
Common migraine is the most common form of migraine in children. Common migraines differ from classic migraine in that the symptoms do not regularly develop in a biphasic mode of neurologic aura and headache. Children with common migraine will often present with malaise, dizziness, nausea, and vomiting that is followed by either a unilateral or bilateral, pounding headache. The child will appear sick, will often want to lie down, and is extremely sensitive to light and sound. The headache will often end when the child falls asleep.
Migraine Equivalents
Migraine equivalents or complicated migraines are migraines that are associated with transient neurologic deficits or alterations in states of consciousness. Migraine equivalents are thought to occur due to prolonged vasoconstriction and ischemia of affected cerebral areas. Neurologic deficits will often precede a headache, but may follow instead. In some instances, a headache may not occur. Migraine equivalents are difficult to diagnose and should be thought of as a diagnosis of exclusion. Migraine equivalents are usually benign and many children will go on to develop typical migraine headaches later in life.
Hemiplegic or hemisensory migraine equivalents are more common in young children than in adults and are characterized by the sudden onset of hemiparesis or hemisensory loss followed by contralateral headache. Symptoms can last hours to days. Unilateral eye pain, headache, and transient ipsilateral third nerve palsy characterize ophthalmoplegic migraines. Ophthalmoplegic migraines have been known to occur in infants younger than 1 year. Symptoms can last minutes to a month. Third nerve involvement occurs in 80% of cases.21 Basilar artery (or Bickerstaff) migraine consists of a combination of visual symptoms, including blindness, vertigo, ataxia, loss of consciousness, and drop attacks. Basilar artery migraine is the most common form of migraine equivalent and occurs more frequently in females.22 Acute confusional migraines rarely occur in children. Difficult to diagnose, acute confusional migraines are characterized by restlessness, combative or hyperactive behavior, and occasional loss of consciousness. Attacks are not often associated with a headache. Micropsia, metamorphosia, olfactory, auditory or gustatory hallucinations, distortion of body image, spatial relations, and time sense characterize the Alice in Wonderland syndrome. Headache may or may not occur with this syndrome.
Migraine Variants
Migraine variants are characterized by the occurrence of episodic complaints not associated with headache in children who later go on to develop typical migraine attacks. Typical complaints include cyclical nausea, vomiting, and abdominal pain or episodes of vertigo. A family history of migraine may be present. Difficult to diagnose, the relationship with migraine is often made after typical migraine symptoms develop later in life.
A number of distinct migraine variants have been recognized. These include benign paroxysmal vertigo, cyclic vomiting, abdominal migraine, and benign torticollis. Children with benign paroxysmal vertigo will present with monthly attacks that decrease in frequency as the child grows older. Attacks will last several minutes and are characterized by the child appearing frightened, grabbing something for stability, nystagmus, and nausea and vomiting. Benign paroxysmal vertigo is the most common cause of vertigo in young children.23 Attacks will typically resolve in 1-2 years and be supplanted by more typical migraine headaches.24
Cyclic vomiting usually occurs in children between the ages of 4 and 8 years. Episodes often occur monthly and are characterized by episodes of nausea and vomiting. Between episodes, the child appears well. Abdominal migraine is characterized by episodes of crampy or colicky abdominal pain located in the periumbilical or epigastric areas. Attacks can last minutes to hours and can be accompanied by nausea and vomiting. In between attacks, the child appears well. The disorder typically resolves in 1-2 years, only to be replaced by more typical migraine headaches. Because both cyclic vomiting and abdominal migraines respond similarly to blockade of serotonin receptors, as well as share similar historic and clinical features, they probably represent related conditions. Evaluation should focus on eliminating possible gastrointestinal or metabolic causes. Treatment consists largely of intravenous hydration and the administration of antiemetic medications, such as ondansetron.
Benign torticollis consists of recurring episodes of head tilt in a child younger than 1 year of age. The side of the torticollis will often vary. The condition is short lived and resolves spontaneously. The child will appear well between episodes.
Cluster Headaches
Cluster headaches are uncommon in children younger than age 10.4 Cluster headaches are more common in males.4 The majority of children with cluster headaches will have multiple attacks of headache occurring over a period of weeks or months separated by intervals of as much as one to two years. Headache is the initial symptom, often described as unilateral. The same side of the head will be affected with each attack. The headache is intense, either throbbing or constant, and the child will not want to lie down, but will instead pace back and forth across the floor. The scalp may appear edematous and tender. Nausea and vomiting do not occur but symptoms of hemicranial autonomic dysfunction, such as Horner syndrome, flushing of the face, and tearing of an eye may develop on the same side as the headache.4 Attacks may be as brief as 10 minutes, but may last for hours. The majority of children will have one attack a day. A family history of migraine headache is often absent, and children with cluster headaches do not go on to develop typical migraine symptoms. Cluster headaches do not often remit later in life and do not respond to typical migraine therapy. Methysergide, lithium, and corticosteroids have been found to be effective in preventing cluster headaches.1 Acute attacks can be treated with sumatriptan or by inhalation of 100% oxygen at a rate of 8-10 L/min.1
Treatment
Parents are often surprised to learn that children can have migraine headaches. Educating parents and children about migraine headaches will often help decrease anxiety and lead to a decrease in symptoms. Sleep is often effective in relieving most migraine attacks.
Parents of children with suspected migraine headaches should be asked to complete a headache diary in an attempt to identify potential triggers. The elimination of potential triggers should be a primary goal of migraine prophylaxis. It is often helpful to ask parents to begin a headache diary after their child has been seen in the emergency department. However, pharmacological agents used in migraine prophylaxis should be started only in collaboration with the child’s primary care physician.
Relaxation techniques, self-hypnosis, and biofeedback have all been evaluated as possible treatment options for pediatric migraine.25-29 All three treatment modalities have shown some success in relieving or preventing symptoms of pediatric migraine. Simple analgesics should be prescribed in adequate dosages at the beginning of an attack. Once an attack is established, gastric motility and absorption are often reduced, diminishing the effectiveness of oral medications.1 A number of pharmacological agents can be used to abort an attack that has already been established.
Non-steroidal agents are the most common first-line medication used for children with migraine headaches. Many theories have been proposed as to the role of NSAIDs in this setting, but most investigators believe that NSAIDs act via their inhibition of prostaglandins and platelet aggregation. In the initial phase of migraine attacks, plasma serotonin levels will increase, and as the attack progresses, decrease. Serotonin is carried almost exclusively by platelets. NSAIDs may act in treating migraine headaches by inhibiting platelet aggregability and decreasing serotonin levels.30 Ketorolac has been found to be highly effective in the treatment of adults with migraine headaches.31,32 It has not been well studied in the treatment of children with migraine headaches and is not approved for use in children younger than 16 years of age. Naproxen sodium has been found to reduce the severity and duration of headache and photophobia in adult patients with migraine headache without aura.33 However, naproxen sodium has not been extensively evaluated in the treatment of children with migraine headaches.
Opiates should be prescribed rarely, if at all, for the management of migraine headaches in children.
Sumatriptan has been used in the treatment of pediatric migraine. Sumatriptan selectively stimulates vascular 5-HT1 receptors that constrict affected vessels. This results in a blockade of nociceptive impulses and the subsequent inflammatory response.34 Approved for use only in adolescents, some investigators have recommended its use in younger children at a dose of 0.06 mg/kg.35 Side effects include flushing, chest pain, and scalp burning. Sumatriptan is relatively expensive and is contraindicated in those who are intolerant of its smooth muscle stimulating properties, such as those with a history of reactive airways disease.36
Dihydroergotamine (DHE) is an ergot derivative that causes vasoconstriction of the external carotid arteries by directly affecting serotonin receptors and through alpha-adrenergic blockade.37 Given parenterally in combination with an antiemetic, DHE has been found to be safe and effective in treating children with migraine headache.38 The addition of an antiemetic in combination with DHE will help to reduce the symptoms of nausea and enhances the effect of DHE.22 One side effect noted with administration of DHE in children is a sense of terror on the part of the child and extreme agitation.25 These side effects usually resolve within 5 minutes after administration.
Phenothiazines are centrally acting dopamine antagonists that produce an antiemetic effect at the chemoreceptor trigger zone. Both chlorpromazine and prochlorperizine have been used to treat migraine headaches. Both exert neuroleptic effects. Chlorpromazine has been shown to significantly improve symptoms in adults with migraine headache.39 Chlorpromazine may act either by altering serotonin levels through inhibition of monoamine reuptake or by a direct effect at serotonergic receptors, or through its neuroleptic effects.40 A prospective, randomized, double-blind, placebo-controlled trial found prochlorperazine to be superior to metoclopramide in the emergency department treatment of migraine headaches in adults.36 Intravenous prochlorperazine was shown to be superior to metoclopramide in relieving headache and decreasing symptoms of nausea. Prochlorperazine has been used in the treatment of children with migraine headaches. Promethazine hydrochloride suppositories have been found to be effective in relieving the symptoms of migraine headache in children. A significant side effect which may be associated with the use of chlorpromazine is a dystonic reaction.
Metoclopramide increases the absorption of analgesics by promoting gastric motility and has commonly been used with other pharmacological agents in the treatment of migraine headaches.41 Metoclopramide has recently been studied as a single agent in the treatment of adults with migraine headaches.42 One study evaluated metoclopramide alone and in combination with ibuprofen in a randomized, prospective, double-blinded, placebo-controlled study of the treatment of migraine headaches.42 Metoclopramide was found to be highly effective compared with placebo. It was found to be equally effective with or without ibuprofen.
Intranasal lidocaine has been used in the treatment of adult patients with migraine headaches. Intranasal lidocaine is thought to act at the sphenopalatine ganglion. In one randomized, double-blind, controlled trial of intranasal lidocaine in the treatment of adult patients with migraine headache, 55% of patients had relief from their headache.43 However, relapse of headache occurred often and early after the treatment. Intranasal lidocaine has not been evaluated in the treatment of children with migraine headaches.
Table 3 lists common medications used in the treatment of migraine headaches in children.
| Table 3. Common Medications Used to Treat Migraine Headaches in Children | ||
| Medication | Pediatric dose | Adult dose |
| Dihydroergotamine | Not established | 1 2 mg/dose or 6 mg/d IM or IV |
| Metoclopramide | 1 2 mg/kg/dose IV | 10 20 mg/dose IV |
| Prochloperazine | 0.1 0.15 mg/kg/dose IM | 5 10 mg/dose IM |
| (> 2y/o or 10 kg) 25 mg/dose PR | 0.1 mg/kg/dose PO or PR | 5 10 mg/dose PO |
| Promethazine | 0.25 0.5 mg/kg/dose PO, IV, IM, or PR | 12.5 25 mg/dose |
| Sumatriptan | Not established | 6 mg/dose SQ 100 mg/dose PO |
| Acetaminophen | 10 15 mg/kg/dose PO | 325 650 mg/dose PO |
| Ibuprofen | 10 mg/kg/dose PO | 400 800 mg/dose PO |
| Naproxen | 5 7 mg/kg/dose PO | 250 500 mg/dose PO |
| Ketorolac | 0.5 mg/kg/dose IM, IV Max 120 mg/d | 30 mg/dose IV or IM 10 mg/dose PO |
Chronic-Progressive Headaches
Chronic-progressive headaches are the least common type of headache in children, however, they are the most serious type and require immediate evaluation. Chronic-progressive headaches generally increase in severity and frequency over a period of months. When abnormal neurologic signs or symptoms accompany the headache, such as lethargy, focal weakness, or personality changes, the practitioner should be concerned about the presence of an intracranial process such as hydrocephalus or a brain tumor. The majority of headaches due to intracranial abnormalities result from traction placed on pain-sensitive structures. Traction can result from a mass lesion such as a tumor, abscess, or subdural hematoma, by distortion of intracranial structures due to a rise in intracranial pressure (ICP), or due to the weight of the brain following removal of cerebrospinal fluid during a lumbar puncture.
Increased ICP
Headache due to increased ICP is characteristically worse at night or immediately upon waking.1 When a history of nighttime headaches is obtained, it is important to differentiate between those headaches that wake the child up and those that are noticed when the child has awakened normally.1 Headaches due to increased ICP typically become worse when the child is lying flat or is asked to perform maneuvers that increase venous pressure, such as coughing, sneezing, or straining.1 The headache may be associated with vomiting. A fundoscopic examination may reveal papilledema and obliteration of venous pulsations. If increased ICP is suspected, a non-contrast CT scan of the head should be obtained immediately.
Pseudotumor cerebri is a disorder characterized by increased intracranial pressure with a normal CT scan or MRI.44,45 Also termed idiopathic intracranial hypertension, pseudotumor cerebri is characterized by headache, blurred vision, diplopia, transient visual obscurations, and dizziness. Papilledema is nearly always present. Field defects, 6th nerve palsies, and loss of visual acuity (especially impairment of peripheral vision) may also occur. The diagnosis can be confirmed by measuring an elevated opening pressure during lumbar puncture. However, lumbar puncture should only be performed after a CT scan or MRI has shown no intracranial abnormalities.
Neoplasm
Brain tumors are an uncommon cause of headache in children. Although headache can be the first symptom of a brain tumor, it is rarely the only symptom. Headache due to neoplasm is usually caused by raised intracranial pressure. As a result, other symptoms such as seizures, weakness, or cognitive changes are often present.45
One study reviewed the history, physical examination findings, and skull films of 72 children with headaches secondary to brain tumors.46 A history of recurrent morning headaches, of headaches that repeatedly awaken the child from sleep, of intense, prolonged, incapacitating headaches, or of changes in the quality, frequency, and pattern of headache was associated with brain tumors. Abnormal neurologic or ocular findings on physical examination occurred early in the course of children with a headache from a brain tumor. Eighty-five percent of children had an abnormal examination within two months of the onset of headache. The authors recommended neuroimaging studies for a child with a headache and an age younger than 3 years, a history of neurofibromatosis, the presence of diabetes insipidus, the presence of short stature, a decrease in linear growth, and the presence of neurologic abnormalities or ocular abnormalities.
If acute hydrocephalus or raised intracranial pressure is of immediate concern in the pediatric patient who presents with a headache, a non-contrast CT scan of the head should be obtained immediately. However, in most other cases, an MRI would be considered the study of choice in the evaluation of a pediatric headache.
Chronic-Nonprogressive Headaches
Chronic-nonprogressive headaches are the most common type of headache seen in children. The most common cause is sustained tension or muscle contraction headache. Other causes include functional or psychogenic headaches, postdural puncture headache, post-traumatic headache, ocular disorders, and chronic sinusitis.
Muscular Contraction
Muscle contraction or tension headaches result from sustained contraction of the muscles of the neck and scalp. Although common in adolescents and adults, such headaches are rare in younger children.17 Typical symptoms include a sensation of tightness or pressure in a band like distribution about the head. The headache is constant, but symptom-free periods may occur. Physical examination may reveal tenderness or tightness of the muscles in the occipital region. Treatment consists of simple analgesics. Biofeedback techniques have been used in the treatment of tension headaches in children.50
Postdural Puncture Headache
Headache is the most common complication of lumbar puncture.47 However, postdural puncture headache (PDPH) more often occurs in adolescents and adults than in the young child.48 PDPH is thought to occur due to a continuous loss of cerebrospinal fluid (CSF) through a dural hole. The loss of CSF is thought to decrease the normal cushioning of intracranial contents causing these same contents to drop downward when the patient assumes an upright position. When the intracranial contents descend downward, they stretch pain-sensitive structures in the dura and intracranial vessels. Risk factors associated with PDPH include the size of the needle used to perform the procedure (larger needles are more likely to be associated with PDPH), the design of the needle tip, the number of punctures, the orientation of the needle (orienting the needle sagittally will result in a smaller hole), the angle of needle insertion, age (older patients are more likely to develop PDPH), and sex (females are 2.5 times more likely to develop PDPH).47-49 Symptoms of PDPH frequently begin 24-48 hours following the procedure and consist primarily of a headache that becomes more severe when the patient assumes an upright position. The headache is commonly located over the occipital area and may radiate to the neck, forehead, or eyes. Associated symptoms include neck pain, blurred vision, and nausea. Pressure applied to the right upper abdominal quadrant will often relieve the pain of PDPH. The majority of PDPH will resolve spontaneously within 3-7 days. Symptomatic relief for pain and nausea can be provided with simple analgesics and by maintaining an adequate hydration status.47 Continued symptoms should be treated by directly repairing the hole in the dura. This can be accomplished either by using a blood patch, by placing pieces of catgut into the epidural space, or by direct closure during laminectomy.
Psychogenic
Chronic headache may be a sign of childhood depression.2 The headache is described as a dull, constant headache which is generalized or localized to the occipital region. Other symptoms of childhood depression such as mood changes, withdrawal, poor school performance, sleep disturbances, lack of energy, and weight loss are usually present. Headache, however, is rarely the primary symptom of childhood depression and must be distinguished from muscle contraction or migraine headaches that have increased in frequency and severity due to stress.51
Ocular
Refractive errors and eye muscle imbalance, though common in children, rarely cause headache and should never be considered the etiology of a headache.1 However, children with refractive errors may complain of a dull ache localized to the periorbital or frontal area. Correction of the visual deficit will often result in resolution of symptoms.
Post-Traumatic
A vascular headache is experienced by close to 40% of patients following a closed head injury.4 The headache will often be described as diffuse, pounding, and exacerbated by head movements, coughing, or straining. Such headaches resolve spontaneously, usually within a few weeks.2 Prolonged post-traumatic headaches, lasting months or years, are not fully understood but are not thought to be vascular in origin.4
Of concern to both clinicians and to parents of children with a headache following a closed head injury is the likelihood of an underlying subdural or epidural hematoma. Headache is the single most common symptom in patients with subdural hematomas, making it extremely difficult to decide which child should have a neuroimaging procedure. The headache of subdural hematoma is often described as bitemporal or generalized. However, other signs and symptoms are often present in children with subdural hematomas. These include changes in personality or cognitive abilities, vomiting, focal weakness, seizures, sensory changes, excessive sleepiness, lethargy, or a decreasing level of consciousness. It is the presence or absence of these signs and symptoms that should help the clinician when deciding whether to obtain a CT scan of the head. One reasonable approach to the neurologically intact child with an isolated complaint of headache following a closed head injury is close observation in the emergency department.
A recent investigation found that a history of motion sickness, migraine headaches, and a family history of migraine were highly predictive of vomiting in a child following a mild head injury.52 Parents of children who present to the emergency department with headache and vomiting following a closed head injury should be asked about a history of migraine headaches and motion sickness in both the parents and the child.
Mixed Headaches
Mixed headaches consist of a combination of acute-recurrent and chronic-nonprogressive headaches. The acute-recurrent headaches are often migrainous in nature, while the chronic-nonprogressive headaches often occur daily and are not associated with neurologic symptoms. Stress is often a precipitant for both types of headaches. History and physical examination of the patient are often normal, as are laboratory and imaging studies. Treatment of such patients includes the use of counseling, biofeedback, and antidepressant medications.53
Summary
Headaches occur commonly in children. The vast majority of headaches are benign and do not represent serious intracranial pathology. A thorough history and physical examination can often determine the underlying cause of the headache and rule out the presence of a serious illness or condition. Laboratory tests and neuroimaging studies are rarely indicated in the evaluation of most children with headaches. However, the presence of a number of distinct signs and symptoms on history and physical examination should prompt further investigation.
Most children with headaches can be managed with reassurance and simple analgesics. Pharmacologic agents exist for treating more severe cases of headache, in particular migraines. The prognosis for most children with headaches is favorable. Most headaches will remit spontaneously later in life, although some children will develop chronic headaches that last into adulthood.
References
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Physician CME Questions
38. What is the most common cause of vascular headaches in children?
a. Migraines
b. Vasculitis
c. Food additives
d. Fever
39. A computed tomography scan of the head should be considered in any child who presents with a headache and signs or symptoms of:
a. papilledema.
b. sixth nerve palsy.
c. ataxia.
d. all of the above.
40. Which of the following is associated with migraine headaches?
a. Motion sickness
b. A relative with migraine headaches
c. Sleepwalking
d. Sleeptalking
e. All of the above
41. Risk factors associated with the occurrence of postdural puncture headache include:
a. size of the spinal needle.
b. number of attempts.
c. age of the patient.
d. type of needle.
e. all of the above.
42. A history of unilateral eye pain, headache, and transient ipsilateral third nerve palsy is characteristic of which of the following migraine equivalents?
a. Basilar artery migraine
b. Hemiplegic or hemisensory migraine
c. Ophthalmoplegic migraine
d. Benign paroxysmal vertigo
43. Headaches following a closed head injury:
a. are common.
b. are vascular in origin.
c. are typically diffuse and throbbing and made worse by coughing or straining.
d. will often resolve spontaneously.
e. all of the above.
44. Headaches secondary to increased intracranial pressure are usually:
a. worse in the afternoon.
b. improved following lumbar puncture.
c. associated with hypotension.
d. aggravated by maneuvers that increase venous pressure.
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