Authors: Michael Plewa, MD, FACEP, Director of Research, Department of Emergency Medicine, St. Vincent Mercy Medical Center, Toledo, OH; Michael Neeki, DO, Resident, Department of Emergency Medicine, St. Vincent Mercy Medical Center, Toledo, OH.
Peer Reviewer: Paul Ishimine, MD, FACEP, FAAP, Attending Physician, Department of Emergency Medicine, University of California, San Diego Medical Center; Attending Physician, Division of Pediatric Emergency Medicine, Children’s Hospital and Health Center, San Diego; Assistant Clinical Professor of Medicine and Pediatrics, University of California, San Diego School of Medicine, San Diego, CA.
Although headache is a common symptom in children and adolescents, only a very small percentage of patients present to the emergency department (ED) for evaluation of this complaint. The majority of these headaches are benign and are either primary, such as migraine or tension-type headaches, or secondary to a viral etiology. Parents and children themselves are most concerned about the possibility of a brain tumor, whereas ED physicians are also on the alert for carbon monoxide toxicity, subarachnoid hemorrhage, meningitis, and increased intracranial pressure.
The authors review the causes, diagnostic testing, and treatment of the common headache, as well as some unusual causes of non-traumatic headache.
Note: This issue contains information about an off-label use. — The Editor
Headache is a frequent symptom in childhood and is the chief complaint for approximately 1% of pediatric ED visits.1,2 The most frequently identified etiology is viral infections (2939%), followed by the primary headache disorders: migraine (921%), tension-type (229%), sinus disease (916%), meningitis (29%), shunt malfunction or hydrocephalus (21%), and tumor (34%).3 Approximately 20% of the children in the United States have chronic head-aches.4 The ED physician must be adept at evaluating and treating the various causes of head-ache in the pediatric age group.
Etiology and Pathophysiology
Various causes of headache include fever (the most common cause of benign paroxysmal headache in the setting of upper respiratory infection),3 anemia, altitude changes, hypoxia, hypercarbia, hypertension, and increased or decreased cerebrospinal fluid (CSF) pressure. Refractory error, eye-strain, or squint are rarely the cause of headache in children.5
Tension, traction, or inflammation of the pain-sensitive intracranial structures, such as the dura, great venous sinuses, and major intracranial arteries, results in pain messages transmitted by the trigeminal nerve (with resulting frontal headache) or spinal nerves (with resulting occipital headache). Inflammation of adjacent head and neck structures including skin, muscle, skull, eyes, ears, teeth, nasal cavities, and sinuses also can cause headache. Although brain tissue may participate in the spreading depression of neuronal activity of migraine, it is not itself a pain-sensitive structure.
Numerous vasoactive mediators are involved in migraine, including nitric oxide, calcitonin gene-related peptide, neurokinin A, substance P, and prostaglandins resulting in stimulation of endothelial cells, mast cells, and platelets, with subsequent dural vasodilation and perivascular extravasation.6 Serotonin levels are diminished between attacks, increase during the aura phase, and then diminish during the headache.6, 7 Despite various etiologies, the final common pathway for headache involves substance P and the trigeminal and spinal nerves.
History. The history is essential to accurate diagnosis in the headache patient.5 Determine the location, quality, and degree or intensity of headache. Occipital location is less common than frontal, and might suggest posterior fossa tumor.8 The inability to describe the headache (present in 25% of children and adolescents with benign headache)9 may be predictive of intracranial pathology; half of brain tumor patients and two-thirds of ventriculoperitoneal (VP) shunt malfunction patients are unable to describe their pain.8 Intensity of pain is important to monitor pain relief, but is not predictive of etiology.8 Ask about frequency, duration, timing, and rapidity of onset. Headaches occurring suddenly (within seconds) suggest subarachnoid hemorrhage (SAH) or seizure. Headaches present in the morning upon awakening from bed suggest increased intracranial pressure (ICP). Document alleviating or exacerbating factors and response to medications, as well as history or pattern of headaches, family history of migraine or cluster headaches, aneurysm, arteriovascular malformation (AVM), Chiari malformation, tumor, hydrocephalus, or seizure. Toxin exposures, such as lead or carbon monoxide, caffeine use, and medication exposure or use should be excluded. Many chronic headaches are related to caffeine withdrawal or analgesic overuse. Determine if the patient had recent head or neck trauma, or a recent lumbar puncture (LP).
Then, focus on associated symptoms such as nausea, vomiting, photophobia, vertigo, fever, stiff neck, myalgias, cough, sore throat, nasal congestion, alteration in vision, toothache, and jaw pain. Vomiting is the most commonly associated symptom in patients with intracranial pathology, present in 74%.1 Is the headache worse with coughing, bending over, or Valsalva maneuver (suggesting sinusitis or increased pressure from mass or fluid), or is it worse with chewing or yawning (suggesting temporomandibular joint [TMJ] syndrome)? Are there associated psychological or behavioral changes? Irritability, temper tantrums, anxiety, mood swings, poor concentration, and drowsiness can occur with migraine and increased ICP. Proceed to exclude abnormal neurologic symptoms, such as aura, ataxia, vertigo, diplopia, weakness, amnesia, seizure, and episodes of loss of consciousness. Disorders of perception, such as micropsia, where images appear smaller than normal, or macropsia, where images appear larger than normal, occur in the Alice-in-Wonderland migraine variant.
The past medical history should address sickle cell disease (which can result in stroke or SAH), coagulopathy, and a history of human immunodeficiency virus (HIV) infection (which would prompt concern for intracranial infections such as, toxoplasmosis, herpes simplex virus, cytomegalovirus, atypical mycobacteria, fungal, tumor, or lymphoma).
The temporal pattern of headache can be a useful method of classification.5 Acute headaches without prior history are typically related to fever, upper respiratory tract infection, sinusitis, mild trauma or stress. Acute recurrent headaches are isolated headache episodes separated by symptom-free intervals, and the differential includes migraine and its variants, tension-type headache, cluster, and neuralgias. Chronic non-progressive headaches are frequent or constant headaches without an increase in frequency or severity. This category includes transformed migraine, chronic daily headache, chronic sinusitis, post-concussive headache, and psychological or stress-related head-ache. Children with recurrent headaches for more than 6 months without neurologic complaints or signs can be reassured of not having intracranial pathology.10 The chronic progressive category of headache exhibits gradual increase in frequency and severity of headache and is most worrisome. This category includes the most serious diagnoses, such as brain tumor, idiopathic intracranial hypertension, hydrocephalus, chronic meningitis, chronic subdural hematoma, brain abscess, AVM, angiomas, arachnoid cyst, lead toxicity, medication side effects and intracranial malformations (e.g., Chiari, Dandy-Walker syndrome).
Physical Examination. The greatest information in the physical exam of the headache patient is obtained from a careful analysis of the vital signs, head and neck exam, and neurologic and eye exam. Vital signs should include temperature, blood pressure, respiration, and heart rate measurements and a pain score. Carefully assess the child’s mental status, level of alertness, interaction, and playfulness. Infants with irritability or lethargy and fever may indicate meningitis.11 When considering meningitis, assess the child for neck stiffness and Kernig’s and Brudzinski’s signs, but remember that their absence does not exclude meningitis.11-13 Other signs of meningeal irritation include jolt accentuation of headache14, 15 and tenderness with ocular globe compression.
The child’s head circumference may provide helpful information. Before suture closure, a markedly enlarged cranium can suggest hydrocephalus or an intracranial mass. Palpate for a bulging fontanel, which may be indicative of increased intracranial pressure from hydrocephalus or meningitis. Auscultate over the orbits and temporal bones for bruits suggestive of AVM. Examine the head and neck for sinus tenderness, purulent nasal discharge, tonsillar hypertrophy, cervical lymphadenopathy, TMJ mobility and tenderness, dental tenderness and tympanic membrane opacity and mobility.
The neurologic examination has high sensitivity for intracranial pathology. In a review of more than 3,000 children with brain tumors, 98% had abnormalities on a focused neurologic exam of mental status, coordination, deep tendon reflexes, sensory, motor, eye movement and funduscopic exam;16 implying that a careful neurologic and eye exam will exclude a brain tumor in 98% of cases.
The eye exam should assess visual acuity, visual fields, pupil response, extraocular movements, and include a funduscopic exam. A relative afferent papillary defect with swinging flashlight test may be present with idiopathic intracranial hypertension. In the funduscopic exam, look for papilledema, hemorrhages and spontaneous venous pulsations. Papilledema is pathologic, and indicative of increased ICP, but may be absent in some cases of shunt malfunction. Subhyaloid and retinal hemorrhages are nearly diagnostic of SAH. Spontaneous venous pulsations are normal when present, but may be unreliable because they can be absent in normal individuals. Eye findings, such as papilledema, visual field defect, 3rd, 4th, or 6th cranial nerve palsy, internuclear ophthalmoplegia, or upward gaze paresis can precede headache, vomiting, and head computerized tomography (CT) findings in patients with increased ICP.17
Inspect the skin for evidence of neurocutaneous syndromes such as tuberous sclerosis (adenoma sebaceum, Shagreen patches, and amelanotic nevi called "ash-leaf spots") and neurofibromatosis (> 5 café au lait spots, axillary freckling and subcutaneous nodules), both of which can be associated with brain tumors. Look for facial hemangiomas, such as a facial port-wine nevus in the 5th cranial nerve distribution suggestive of Sturge-Weber syndrome, which may be associated with intracranial angiomas.
For patients with a CSF shunt, palpate the burr hole, the extracranial proximal catheter, the valve and the distal catheter tubing all along the track assessing for fluid collections, tenderness, warmth, or lack of tubing integrity. Fluid collection along the shunt track can be present in 36% of cases of shunt malfunction.18 Most, but not all, patients with shunts have pumpable valves.19 Pumping the shunt valve has limited sensitivity (39%) and specificity (85%), for shunt obstruction.19 Avoid repeated pumping, which may damage the shunt or valve mechanisms, entrap the choroids plexus resulting in obstruction, or occasionally result in retrograde flow into the ventricle.20
The majority of headache patients do not require diagnostic testing in the ED. The most useful tests for headache in the pediatric age group are lumbar puncture (LP), head CT, and carboxyhemoglobin level measurement.
Lumbar Puncture. Indications for LP include suspicion for meningitis, subarachnoid hemorrhage, and/or idiopathic intracranial hypertension. LP is contraindicated in patients with coagulopathy, overlying cellulitis, or substantial risk of herniation. Consider omitting the LP in patients with CSF shunts suspected of a shunt infection.20 In this situation, CSF should be obtained from the shunt by a neurosurgeon.
Measure the CSF pressure, when possible, in the lateral decubitus position with legs relaxed in extension; the fetal position can falsely elevate pressure. Normal pressure is less than 200 mmHg. Send fluid for cell count, differential, glucose, and protein measurements and bacterial culture. Consider bacterial antigen testing if the patient already has been treated with antibiotics or if there is a high prevalence of antibiotic-resistant Streptococcus pneumoniae. An LP for SAH is best performed 6 hours or more after the onset of symptoms, and is most sensitive with spectrophotometric determination of xanthochromia.21
CT scans prior to LP have less sensitivity than clinical assessment for impending herniation,22 and patients without papilledema, altered mental status, focal neurologic exam, or clinical impression of impending herniation do not require a screening CT scan prior to LP.23 Although there is controversy about whether LP is causative or even temporally related,24, 25 herniation can occur in as many as 5% of children with meningitis, especially if purulent, and can occur with normal head CT scan also.25, 26 Delayed LP after presumptive antibiotics, resuscitation, and stabilization in patients who appear too ill for immediate LP is appropriate.
Head CT Scan. Although the list of indications for head CT is extensive (See Table 1.), head CT scan is certainly not indicated in the majority of children with headache. Head CT with contrast should be considered when venous sinus thrombosis is highly suspected. Quite often, however, structural or anatomic abnormalities are identified (as often as 21%), which are unrelated to the headaches themselves.27
Indications for Neuroimaging in Children with Headache
Brain tumor is less common in children than adults, despite being the largest cause of solid tumors in children, with an annual incidence of 3 per 100,000.16 Imaging for brain tumor is unnecessary in the absence of neurologic findings, alteration in consciousness or personality, morning headache, or positional or exertional changes.28 Children with non-migraine headache episodes lasting more than 6 months and normal neurologic exam are at low risk for brain tumor, with baseline risk of 0.01%; close clinical follow-up without imaging is the most cost-effective strategy.29 Although children with migraine symptoms and normal neurologic exam are considered at intermediate risk of brain tumor of 0.4%, most would agree that imaging is not necessary.30 The most cost-effective strategy—head CT followed by magnetic resonance imaging (MRI) for positive results—is quite expensive (more than $1 million per quality-adjusted life year [QALY] saved)29 and can be pursued by the child’s primary care physician if necessary. Children with headache and clinical symptoms of a space-occupying lesion, such as headache of less than 6 months duration, sleep-related headache, vomiting, confusion, absence of visual symptoms, absence of family history of migraine or abnormal neurologic exam, have the highest risk of tumor at 4% and might benefit from MRI scan, with costs of $113,800 per QALY saved.29
Hydrocephalus can be communicating (non-obstructive), where all ventricles are enlarged, or non-communicating (ob-structive), where only part of the ventricular system is enlarged.
Narrowing of the cortical sulci, ventricles, and basal cisterns may indicate cerebral edema. Signs of shift are more indicative of potential herniation than increased CSF pressure.30,31 (See Table 2.) When assessing a head CT scan for potential herniation, look not only for evidence of focal space occupying lesion, but also compartmentalization or obstruction of CSF flow.
Contraindications to Lumbar Puncture Based on Head CT Findings
In the evaluation of a child with a CSF shunt, ventricular size in comparison with previous head CT scans should be assessed. Enlarged ventricular size alone does not imply shunt obstruction; it also can occur in normally functioning shunts.32 Look for signs of increased CSF pressure, including effacement of cerebral cortical sulci, obscuration of the basal cisterns and transependymal CSF absorption.32
Not all shunt obstructions result in an increase in ventricular size. In some instances, the 4th ventricle becomes isolated and enlarged, and the lateral ventricles are normal or unchanged in size. In as many as 23% of shunt obstructions, ventricular size and anatomy are normal or unchanged,33 and in 13% the ventricles are slit-like32 despite elevations in pressure, perhaps secondary to increased brain turgor or ventricular rigidity.34 The sensitivity and specificity of head CT for shunt obstruction is approximately 83% (6492%) and 88%, respectively.33,35
In patients with SAH, blood in the subarachnoid space appears white on CT scans for the first several days and then becomes isodense 4-7 days after the bleed, and can be seen most frequently in the basal cisterns (often giving the appearance of narrowing of the cisterns),36 as well as the suprasellar cistern, posterior fossa cistern, Sylvian fissures and interhemispheric fissure. The sensitivity of CT for SAH is approximately 93%, even on new generation scanners. Although MRI and angiography are the imaging methods of choice, many AVMs, cavernous angiomas, and aneurysms may be seen on non-contrast CT as hyperdense serpiginously shaped lesions. Hyperdensity or enlargement of the vein of Galen, straight sinus or superior sagittal sinus or torcula (confluence) on non-contrast CT can indicate deep venous thrombosis.
Although MRI is recommended to accurately exclude any of the various Chiari malformations, Chiari II malformations may be evident on CT scan with hydrocephalus, diminished 4th ventricle size, widening of the foramen magnum and upper cervical canal, incomplete fusion of the posterior arch of C1, or protrusion of the vermis, 4th ventricle, medulla and cervical cord into the spinal canal space.37
Ultrasound. Although ultrasound is used routinely in the neonatal period, the acoustic windows are more difficult to penetrate as the child grows older. Some institutions, however, have found reasonable sensitivity and specificity of transcranial ultrasound to detect intracranial lesions for children 3-15 years of age (average 10.4) with isolated headaches, 75% and 99.7%, respectively,38 using every possible acoustic window, including over the supraorbital area. The advantages of this alternative screening method are increased availability, lower cost and lack of need for sedation in comparison with CT or MRI.
Electroencephalogram (EEG). Although EEGs performed in patients with migraine or recurrent headache frequently have nonspecific abnormalities, they do not provide useful diagnostic information or discriminate between headache types or causes.39 EEGs rarely are indicated, except when a concomitant seizure is suspected, such as with alteration of consciousness or abnormal involuntary movements.39
Shunt Evaluation Procedures. In addition to head CT, patients with possible shunt malfunction also may be evaluated by several other methods. The shunt series (plain films of the skull, chest, and abdomen) has low sensitivity (20%) for CSF shunt malfunction,33 as well as low yield (< 1%) for detecting unsuspected abnormalities not seen on CT, but occasionally may provide information not suspected on physical exam or head CT. Look for suture diastasis, disconnections, retraction out of the abdomen, discontinuity at the bulb, kinking, or no movement of the tip in comparison with previous radiographs (implying a possible loculation in the abdomen). Most shunts have barium impregnated silicone tubing, however, some tubing and connections are nonradioopaque, and can mimic shunt disconnection or breakage, and therefore, comparison with old films is critical.32
A shunt tap can be diagnostic of shunt infection. Defer this procedure to the neurosurgeon, except for extenuating circumstances. Complications are more likely when performed by inexperienced clinicians and include infection,40 damage to the valve or distal tubing requiring replacement surgery, local bleeding, and intraventricular or subdural bleeding if the CSF drains too rapidly.
Differential Diagnosis of Headache
The differential diagnosis of headache is extensive and can be divided into primary headache disorders (e.g., migraine, tension-type, cluster, idiopathic stabbing headache) and secondary head-ache disorders (See Table 3.). Primary headaches are disorders without systemic or verifiable pathology, and for which head pain is the predominant defining symptom. Secondary headaches are caused by organic disorders with verifiable pathology, with head pain as one component of several possible symptoms.
Table 3. Causes of Headache
Primary Headache Disorders. Migraine occurs in 1% of children by age 7 and in 5% by age 15 years.41 Until puberty, there is a male preponderance of childhood migraine.41 In adolescence, the prevalence of migraine is 3.8% in boys and 6.6% in girls.42 Children with migraine are absent from school more than 1.5 weeks/year more than their peers without migraines and may have impairments in communication, school functioning, and social activities. They experience more depression, anxiety, sleep and eating disorders, and emotional impairment than their peers,43 rate their quality-of-life scores as low as those of children with cancer or arthritis,43 and are nearly twice as likely to have multiple somatic complaints and psychiatric morbidity as adults.44
The International Headache Society (IHS) classification for migraine has been modified for children (See Table 4.). Children with migraines are more likely than adults to have bilateral pain, brief duration (1 4 hours), visual aura, nausea, vomiting, pallor, malaise, irritability, anorexia, or blurred vision7 and less likely to have as many as five attacks per year, photophobia, and phonophobia, or pulsating quality.41,45 Migraine variants are also more common, occurring in 10% of children, and include benign paroxysmal torticollis (10%), benign paroxysmal vertigo (38%), abdominal migraine/cyclical vomiting (19%), acephalgic migraine (29%), and acute confusional migraine (5%).46 Migraine may be without aura (common migraine) or with aura, such as scintillating scotoma, flashing lights, blurring, hemianopsia, focal symptoms such as weakness, sensory changes, aphasia, ophthalmoplegia, or altered mentation, such as fugue states or transient global amnesia. Complicated migraine results in neurologic deficits from prolonged ischemia lasting hours to days, often with hemiplegia or hemisensory changes, aphasia and paresthesias. Complicated migraine is a diagnosis of exclusion after other causes of persistent neurologic deficits such as stroke, tumor, or AVM, have been ruled out. Ophthalmoplegic migraine can cause unilateral eye pain and 3rd nerve palsy with ptosis, papillary dilation, and external eye deviation. Basilar artery migraine can result in ataxia, vertigo, dysarthria, weakness, syncope, and persistent scotoma or transient blindness. The acute confusional state results in altered personality or behavior or orientation that is sometimes restless, hyperactive, or combative, or sometimes less active or interactive.
Modified IHS Criteria for Diagnosis of Migraine in Children
Tension-type headaches are common and persistent, but usually of milder severity than migraine and not associated with photophobia, phonophobia, vomiting, or auras (See Table 5.). A variety of stressors can precipitate tension-type headache, which responds well to simple analgesics and behavioral therapies.
Table 5. Criteria for Tension-type Headache
Chronic daily headache is present in fewer than 1% of adolescents and may be caused by overuse of analgesics, even acetaminophen, excess caffeine intake,47 or may be considered a form of transformed migraine. These headaches typically last more than 4 hours and occur more than 15 times per month.
Cluster headaches are more common in boys, and cause severe periorbital pain with lacrimation, miosis, rhinorrhea, and conjunctival injection usually lasting about 3060 minutes.
Idiopathic stabbing headaches are rare, although associated with other primary headaches in 14% of cases, more common in girls, and are characterized as ultra-brief attacks lasting from a few seconds to 15 minutes in duration, typically in the orbital region, and without autonomic effects, conjunctival injection, tearing or rhinorrhea.48 These respond well to indomethacin.
Secondary Headache Disorders. The classic description of sinusitis headache is worse with coughing or positional changes, and is associated with purulent nasal discharge, fever, and cough. Frontal headache and facial pain occur with maxillary, frontal, or ethmoid sinusitis, whereas sphenoid sinusitis may cause headache in the vertex. Sphenoid sinusitis is rare in childhood because the sphenoid sinus does not develop until age 35 years, but can have serious complications, such as cavernous sinus thrombophlebitis, 3rd, 4th, 5th, or 6th cranial palsy, brain abscess, or meningitis.49
Brain abscess can occur in children with congenital heart disease, sinusitis, otitis, mastoiditis, or an immunocompromised state and typically results in progressive headache, fever, seizures, or focal neurologic symptoms.
In addition to progressive headache, dural venous sinus thrombophlebitis also may cause focal or neurologic findings, such as seizures or alterations in consciousness if sagittal sinus thrombosis, idiopathic intracranial hypertension or 6th nerve palsy if lateral sinus thrombosis, and chemosis, periorbital edema, and possibly 3rd, 4th, or 6th palsy if cavernous sinus thrombosis.
Carbon monoxide (CO) toxicity routinely causes headache (the most common symptom), along with dizziness, nausea, weakness, confusion, dyspnea, ataxia, and other neurologic deficits. The headache can be of any quality, location, or pattern, and may not correlate with the carboxyhemoglobin (COHb) level. If other family members or cohabitants have similar symptoms, carbon monoxide toxicity should be considered. Treat with high-flow normobaric oxygen, and consider hyperbaric oxygen treatment in severe cases (e.g., loss of consciousness, COHb > 25% or > 15% during pregnancy, serious neuropsychiatric, or cardiovascular findings).50
Vascular abnormalities include aneurysm, AVM, cavernous angioma, venous angioma, and capillary telangiectasia. Although aneurysm is more common (25% of the general population) than tumor (1%) and AVM (0.8%), presentation during childhood is extremely rare.28 Children with AVM headache typically have a migraine-like headache only on one side, may have a bruit, prolonged aura, lower incidence of family history of migraine and possibly abnormal neurologic exam.28 Subarachnoid hemorrhage classically results in sudden onset of headache, neck discomfort, photophobia, syncope, or neurologic symptoms and retinal hemorrhage.
Idiopathic intracranial hypertension (IIH) is defined by three major criteria: papilledema, increased ICP with normal CSF constituency, and normal brain imaging studies (normal or small ventricles).51 IIH is more common in girls than boys and associated with obesity. The headache is chronic and progressive, typically worse in the supine position or upon awakening, partially relieved in the upright position, associated with nausea, vomiting, neck, back shoulder and joint pains, transient monoocular, or binocular visual loss,52 pulsatile tinnitus (in 60%), diplopia from 6th nerve palsy, ataxia, or impaired concentration. Vision loss is the primary serious complication. There are numerous causes of IIH, including scarring of arachnoid granulations from SAH or infection, obesity, superior vena cava syndrome, Addison’s disease, hypoparathyroidism, steroid withdrawal, hypervitaminosis, AVM, and medication exposure (e.g., tetracyclines, sulfamethoxazole, and penicillin). Treatment involves discontinuation of the offending agent, weight loss of at least 6% if obese, administration of acetazolamide 14 gm/day, and rarely, surgical shunting or optic nerve sheath decompression procedures.
Post-lumbar puncture headache complicates approximately 10% (2 60%) of LPs and is unrelated to hydration status, amount of fluid removed, or bedrest following the procedure. It is more likely to occur with the use of large bore-cutting needles, repeated attempts, and changing orientation of the needle during LP; less likely with the use of small gauge (2224 ga) or pencil-tipped needles, or a bevel parallel to fibers.53 Symptoms including headache, nausea, dizziness, presyncope, and diaphoresis, are worsened in the upright position and partially relieved in the recumbent position. The syndrome is generally self-limited, lasting an average of 5 days, but can last weeks or even months. Conservative measures include analgesics, fluids, caffeine, and prolonged recumbency. Refractory cases are treated with an epidural blood patch,53 usually performed by an anesthesiologist.
Chiari malformations are rare congenital disorders of the posterior fossa with Chiari I malformation involving protrusion of the cerebellar tonsils into the foramen magnum;54 and Chiari II malformation, usually associated with hydrocephalus and myelomeningocele, involving herniation of the cerebellar vermis, brainstem, and the 4th ventricle into the foramen magnum.54 Symptoms are worsened by cough, Valsalva maneuver, and exertion, and consist of ataxia, vertigo, dysarthria, dysphagia, hearing loss, headache, nausea, vomiting, neck pain, and weakness. Neurosurgical decompression usually is recommended.
Evaluation of the child with headache and CSF shunt can be a diagnostic challenge. Shunts are prone to the complications of occlusion, overshunting, or infection. As many as 57% of shunt patients also may have chronic headaches or shunt migraines not related to shunt malfunction.53 Classic symptoms of shunt obstruction include a progressively worsening headache, worse with the supine position, coughing or Valsalva maneuver, and associated with any of the following: irritability, drowsiness, personality changes, poor feeding, vomiting, ataxia, periorbital puffiness, urinary incontinence, cranial nerve palsy, sunsetting eyes, lethargy, or coma. A child with overshunting might complain of low CSF pressure headache that is worse when upright and relieved in the recumbent position. Complications of missed shunt malfunction can include loss of vision, permanent neurologic deficit, refractory seizures, and death. Parent and physician suspicion for shunt malfunction has an approximately 50% positive predictive value and an 80% negative predictive value,56 and approximately 25% of patients evaluated for possible shunt malfunction will require surgical intervention.33
Shunt infection occurs at a rate of 813%,57,58 most commonly (90%) within 6 months of the latest procedure, and have a higher mortality rate (3040%) than shunt malfunction.59 Minor non-specific complaints are the most common, such as low-grade fever, lethargy, or minor headache. Fever is absent in 1058% of patients, 44% have no evidence of sepsis,18 and meningismus may be absent in 6675% of cases.20 The source of infection is usually from the initial procedure, rarely from bowel perforation, and usually not from transient bacteremia.20,58 The majority of organisms are Gram-positive, especially Staphylococcus epidermidis, but Gram-negative organisms can cause delayed, more serious infections, as can anaerobic organisms.
The majority (93%) of children with headache can be treated successfully both in the ED and as an outpatient with over-the-counter analgesics3 such as acetaminophen, ibuprofen, or naproxen sodium. These analgesics are the first-line agents recommended for outpatient treatment of recurrent headache, including migraine. An adequate dose (more than needed for fever) early in the course of headache and access to medication at all times, such as during school or activities, may lessen the intensity of the headache.39 The frequent or daily use of even simple analgesics should be avoided because of risk of chronic daily headache. There are several other abortive agents for migraine used for moderate to severe headaches and when simple analgesics fail. The use of abortive agents should be limited to two headaches per week to minimize analgesic-induced rebound headache.
Migraine-specific agents—triptans and ergotamines—or phenothiazines for moderate to severe migraine should be used, especially those refractory to simple analgesics. Many of the triptans and ergotamines are not FDA approved for children, though there are several clinical trials and extensive clinical experience to suggest they are safe and effective. For children weighing more than 50 kg, the full adult dose is recommended. Triptans should not be combined with ergotamines, and are contraindicated in pregnancy, cardiovascular disease, hypertension, and complicated migraine. Side effects include tachycardia, flushing, tingling, burning, disorientation, and chest tightness with subcutaneous administration and taste disturbance, dry mouth, dizziness, asthenia/fatigue, nausea, and somnolence with oral use. Nasal sumatriptan is considered effective,60-62 whereas oral sumatriptan and rizatriptan are less effective in children and adolescents than in adults,63,64 and may be no different than placebo. The newer nasal triptans—zolmatriptan and rizatriptan—are likely to be more effective than oral preparations.
Ergotamines usually are reserved for older children and adolescents. Complications may include nausea, vomiting, diarrhea, paresthesias, and muscle pain. Avoid ergotamine use in patients who have liver, kidney, or vascular disease; hypertension; complicated migraine; are pregnant; or use triptans concomitantly. Ergotamine can be administered sublingually (with or without caffeine), as a nasal spray, or by injection. Consider giving a 0.1 mg IV test dose of dihydroergotamine (DHE) prior to the full dose to watch for nausea, vomiting, hypertension, and abdominal pain. Most patients receiving DHE should be pretreated with an anti-emetic, such as metoclopramide, to prevent nausea and vomiting.
Other nonsteroidal anti-inflammatory agents (NSAIDs), such as ketorolac, (by injection or orally), and indomethacin are frequently effective. Data from clinical trials comparing the relative efficacy of various NSAIDS in pediatric headache are not available.
Opiates are useful rescue agents when prior use of simple analgesics or other agents have failed. Avoid prescriptions that encourage chronic outpatient opiate use; however, instructions for occasional use for severe refractory headaches can prevent recurrent ED or physician visits. Opiates can cause sedation, hypotension, constipation, altered mental status, respiratory depression, and habituation. Codeine, hydrocodone, and oxycodone used in combination with acetaminophen are prescribed most frequently in adolescents, but codeine and hydrocodone can be given alone in liquid form. Butorphanol, available as a nasal spray, is an agonist-antagonist without addictive potential, and can cause nausea, drowsiness, blurred vision, and headache.
Phenothiazines are effective agents, not only as antiemetics, but also for terminating migraine, but can cause akathisia or dystonia. Many clinicians treat concomitantly with diphenhydra-mine 0.5 mg/kg (up to 5 mg/kg in 24 hours) for 48 hours of therapy. Metoclopramide and prochlorperazine are used extensively in adults. Prochlorperazine is more effective than ketorolac in children65 and is effective in refractory status migrainosus.66 There is less clinical experience with the use of promethazine and chlorpromazine, both of which are more sedating. Droperidol, a butyrophenone, is also quite effective as an antiemetic and migraine abortive agent in adults,67 but also can cause arrhythmia or sudden death following QT-interval prolongation.
There are several oral combination agents (in tablet form) available for treatment of migraine, which can be sedating and result in chronic daily headache with frequent use. Acetaminophen-butalbital-caffeine and aspirin-butalbital-caffeine may be used for children older than 6 years of age, and also can be combined with codeine. Isometheptene mucate-dichioraiphenazone-acetaminophen, may be used for children older than 12 years.
Migraine recurrence within 2448 hours is common, especially in those without complete pain relief prior to ED discharge,68 and may vary between 18% and 87% depending upon the type and route of abortive agent used.69 Glucocorticosteroids are recommended for status migrainosus and may be useful in preventing migraine recurrence.70 The use of a single dose of dexamethasone (0.25 0.5 mg/kg IV or orally), or methylprednisolone (1 mg/kg orally) may be beneficial.
Prophylaxis is recommended when migraine headaches are severe and recurrent, more frequent than three episodes per month or interfere with school or social activities, especially if use of acute medications would increase the chance of drug-induced rebound headache. Allow sufficient time, usually 23 months, to demonstrate clinical improvement, starting with the smallest effective dose to avoid side effects. Numerous agents have been recommended in adults, each with their own unique side effects, although only propranolol and flunarizine are supported by adequate evidence in children,71 despite extensive clinical experience with others. Propranolol is the most frequently used prophylactic agent, but may have associated side effects of fatigue, orthostasis, insomnia, nausea, and diarrhea. Amitriptyline may be complicated by blurred vision, urinary retention, dry mouth, dizziness, sedation, and is not recommended in children younger than 12 years. Cyproheptadine can cause fatigue, dizziness, increased appetite, and cough. Flunarizine can cause dizziness, fatigue, and weight gain. Topiramate can result in paresthesias, fatigue, anorexia, difficulty with memory, and taste perversion. Valproic acid can cause hepatotoxicity, increased appetite, dizziness, and drowsiness.
One of the most important aspects of caring for the benign headache patient is reassurance (both for the child and the parents) that there is no evidence of a significant brain abnormality, such as tumor, hydrocephalus, stroke, or bleeding. Children with recurrent headaches should be encouraged to use a headache diary and record when, where, and why their headaches have returned. Allowing the child or adolescent to be involved in personal choices in their care, in learning what to watch for and what to do when headaches return, will increase compliance and success.
Instruct patients with migraines, using written discharge instructions, to avoiding triggers such as foods, medications (e.g., oral contraceptives), stress (e.g., loss, separation, let-down periods), schedule disruptions (e.g., sleep changes, skipping meals), sensory stimuli (e.g., odors, flickering lights, loud noises), and weather changes (e.g., barometric changes, altitude changes, lack of sunlight). Caffeine withdrawal, monosodium glutamate (e.g., Asian food), tyramine (e.g., aged dark or hard cheese), nitrites (e.g., processed lunchmeats, hot dogs), phenylethylamine (e.g., chocolate), octopamine (e.g., citrus fruits), alcohol, pickles, nuts, aspartame (Nutrasweet), and smoked or pickled meats and fish can precipitate migraines. Although a diet diary is recommended, total avoidance of all possible dietary triggers is not.72 Recommend eating balanced meals and avoid fasting or skipping meals. Supplements may be useful in prophylaxis of recurrent headaches.
Consider recommending relaxation training, biofeedback, or cognitive-behavioral therapy for prevention of recurrent headache.73-75 Each of those is effective in prophylaxis of migraine and tension-type headaches,75,76 although they require high motivation, typically at least 1015 visits with a skilled therapist,10 and may not be covered by insurance. Acupuncture, cervical manipulation, hypnotherapy, and massage may be effective in individual cases, although studies have not consistently demonstrated efficacy in recurrent headache.73
Disposition and Referral
Patients with headache secondary to serious intracranial abnormality, such as idiopathic intracranial hypertension, abscess, or venous sinus thrombosis, as well as those with persistent status migrainosus should be admitted for further treatment. Immediate neurosurgical consultation is essential for impending herniation, acute hydrocephalus, and potential shunt malfunction. Outpatient ophthalmologic follow-up is essential for patients with idiopathic intracranial hypertension. Outpatient neurologic follow-up is useful for patients with chronic progressive pattern of headache, children younger than 3 years, acute headache with focal signs or symptoms, as well as generally difficult cases.
A thorough history, focused physical exam, and judicious use of diagnostic tests will identify even the most serious causes of headache in the pediatric population. The ED physician aware of the head CT findings of SAH, increased ICP, and shunt malfunction can identify successfully the majority of serious intracranial pathology. Treatment of the majority of headaches in children, especially migraines, is effective with simple analgesics, prudent use of triptans, ergotamines, phenothiazines, and occasionally opiates for rescue use. Reassurance of the child and parents and adequate instructions can be a great help in the well being of children with severe or recurrent headaches.
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