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896 SECTION 12: Pediatrics ED evaluation and management of head injury increasingly focus on strategies for risk stratification in order to avoid the potential long-term effects of ionizing radiation and are discussed in detail in Chapter 111, “Minor Head Injury and Concussion in Children. ” Children with identified intracranial injury and a witnessed seizure should be treated with a loading dose of antiepileptic medication, typically fosphenytoin or levetiracetam, to prevent short-term recurrence that can worsen trau matic brain injury and increase intracranial pressure 66; benzodiazepines remain first-line treatment for active seizures in the setting of trauma. Abusive Head Trauma Infants and toddlers presenting with vague complaints and seizures may be the victims of abusive head trauma (see Chapter 150, “Child Abuse and Neglect”), which can present without external signs of injury. Most children with abusive head trauma are <2 years of age, and the majority of cases occur in the first year of life (shaken baby syndrome). 67-69 Subdural hematoma is the most often identified inflicted intracranial injury in these infants. 67,69 Maintain a high index of suspicion for trauma in the setting of afebrile seizures in infants. If intracranial injury is identified, a more complete evaluation for other abusive injuries is necessary, including skeletal survey, retinal examination by an ophthalmologist, screening for blunt abdominal trauma (liver and pancreatic enzymes), and bleeding studies. REFERENCES The complete reference list is available online at www.TintinalliEM.com. Headache in Children David Sheridan Garth D. Meckler INTRODUCTION AND EPIDEMIOLOGY Headache is pain in the scalp and cranium. Headaches in children can be mild, refractory, or life threatening, and can represent an acute, sub acute, or chronic process. Headaches are broadly classified as primary headache disorders, which are intrinsic to the nervous system, and sec ondary headaches, which are caused by another condition such as trauma, infection, or intracranial or systemic disease (see later section, “Headache Classification”). Headaches are common in children, with an increasing prevalence through childhood and a peak in adolescence. The mean prevalence of headache in childhood is 58.4%, with a range of 24% to 90%, and 17% of children in the United States reported frequent or severe headaches in the past year. 1-3 Headache prevalence is similar in girls and boys prior to adolescence, but after puberty, they are more prevalent in girls. Overall, primary headache disorders are more common than secondary headaches, and migraine and tension-type headaches are most common. 4,5 The peak incidence of migraine is earlier for boys (10 to 11 years) than girls (12 to 14 years), and migraine with aura starts 3 to 5 years earlier than migraine without aura in both sexes. Tension-type headache has a mean age of onset of 7 years and is more common in girls. 7 The estimated overall prevalence of migraine in childhood is 3.76% to 18%, and the overall prevalence of tension-type headache is 10% to 25%. 7-10 Frequent or sustained headaches have a significant impact on chil dren and their families, contributing to school absenteeism and missed parental work, and may even induce behavioral disturbances.

revalence of migraine in childhood is 3.76% to 18%, and the overall prevalence of tension-type headache is 10% to 25%. 7-10 Frequent or sustained headaches have a significant impact on chil dren and their families, contributing to school absenteeism and missed parental work, and may even induce behavioral disturbances. 11-13 Migraine headaches are among the top five causes of years lost to dis ability according to the World Health Organization,14 and children with migraines experience similar impact on quality of life as those with cancer or juvenile arthritis. 12,15 There is an association between chronic headache and psychiatric comorbidity among children.16-18 CHAPTER Headaches compose about 1% to 2% of all ED visits among children, and in the ED setting, secondary headaches are more commonly diag nosed than primary headaches. 2,19-22 Infectious etiologies such as upper respiratory tract infection are the most common cause of secondary pediatric headache in the ED, whereas migraine is the most common primary headache disorder diagnosed in this setting. 2,19-21,23-25 There appears to be some seasonal variation in ED visits for headache, with peaks in September and January and lower visit rates in the spring and summer months. 26-28 PATHOPHYSIOLOGY The pathophysiology of headaches is complex and varies according to cause. The cranium, most of the overlying meninges, brain, ependymal lining, and choroid plexus do not possess pain receptors. Extracranial pain may arise from cervical nerve roots, cranial nerves, or extracranial arteries, and intracranial pain may arise from intracranial venous, arte rial, or dural structures. Cranial nerve or root pain can radiate to the occiput, ear, retroauricular areas, or throat. HEADACHE CLASSIFICATION Headaches are classified as primary or secondary based on the under lying cause. Primary headaches are physiologic or functional and are typically self-limited, although they may become persistent (e.g., status migrainosus) or chronic. Primary headache disorders include migraine, tension-type headache, chronic daily headache, and cluster headache; cluster headaches are more commonly seen in the adult than pediatric population. The gold standard diagnostic criteria are the International Criteria for Headache Disorders 3rd ed, beta version ( Table 139-1). Although these diagnostic criteria rely on recurrent attacks, in an ED, a child may present with a first migraine headache due to intractable pain. One study investigated the utility of applying the International Headache Society criteria in the ED without the “recurrent” requirement (“Irma Criteria”) and found the criteria to be quite sensitive in diagnosing first-time migraines when followed long term using the original International Headache Society criteria as the gold standard. Secondary headaches are a symptom of another underlying disorder and include a wide range of etiologies. Table 139-2 lists common and life-threatening causes of secondary headaches. 31-33 Although primary headaches can be disabling, secondary headaches result in morbidity and mortality if not treated. CLINICAL FEATURES Clinical characteristics of pediatric headache vary greatly by classifica tion and etiology; however, the details of the headache including onset, pattern, location, quality, and associated symptoms are helpful in determining the cause and directing further investigation and treatment. Red flag clinical features associated with life-threatening secondary headaches are listed in Table 139-3. 33-37  TEMPORAL ASPECTS OF HEADACHE Age of Onset As mentioned earlier, headaches increase in incidence and prevalence throughout childhood, and initial onset varies by etiology.

rther investigation and treatment. Red flag clinical features associated with life-threatening secondary headaches are listed in Table 139-3. 33-37  TEMPORAL ASPECTS OF HEADACHE Age of Onset As mentioned earlier, headaches increase in incidence and prevalence throughout childhood, and initial onset varies by etiology. Although overall rare in children <5 years of age, primary headaches can and do occur in this age group; migraine with aura, for example, can begin as early as age 5 to 7 years for boys, and 37% to 50% of 7-year-old children report experiencing headache. 2,6,38,39 Complex migraines (hemiplegic, confusional, or basilar type) may have their onset at a young age as well; however, incapacitating headache in a young child, especially when associated with vomiting or gait changes, suggests an intracranial mass with an infratentorial location being the most common location. 40 Among a cohort of children age 2 to 5 years presenting to the ED with headache, the vast majority of headaches were associated with benign viral infections, and only 1 of 364 patients in the cohort was diagnosed with a tumor (this patient had an abnormal neurologic exam). Tintinalli_Sec12_p0669-0996.indd 896 8/2/19 7:56 PM

g a cohort of children age 2 to 5 years presenting to the ED with headache, the vast majority of headaches were associated with benign viral infections, and only 1 of 364 patients in the cohort was diagnosed with a tumor (this patient had an abnormal neurologic exam). Tintinalli_Sec12_p0669-0996.indd 896 8/2/19 7:56 PM CHAPTER 139: Headache in Children 897 TABLE 139-1 International Headache Society Diagnostic Criteria for Common Primary Headache Disorders (3rd ed, Beta Version) 29 Primary Headache Disorder Diagnostic Criteria Comments Migraine without aura 1. At least 5 attacks with features 2–3 below 2. Headache duration 2–72 hours 3. At least 2 of the following: a. unilateral b. pulsating c. moderate or severe intensity d. made worse with activity In children, pain is more often bilateral and less often pulsating. Usually frontotemporal in location. Photophonophobia may be inferred from behavior in young children. Symptoms persisting >72 h are termed status migrainosus. Migraine with aura 1. At least 2 attacks with features below 2. One or more reversible auras: a. visual b. sensory c. speech/language d. motor e. brainstem f. retinal 3. At least 3 of the following: a. gradual spread of aura (>5 min) b. 2 or more symptoms may occur in succession c. individual symptoms last 5–60 min d. at least 1 symptom is unilateral e. at least 1 symptom is positive f. accompanied or followed by headache within 60 min Visual and sensory symptoms are common in children, as are autonomic symptoms. Systemic prodromal symptoms (e.g., fatigue, elated or depressed mood) may precede aura by 24–48 h. Probable migraine 1. Attacks fulfilling all but 1 of the criteria for migraine without aura 2. Attacks fulfilling all but 1 criterion for migraine with aura Includes those not meeting required number of prior attacks for migraine with or without aura. Tension-type headache 1. At least 10 episodes of headache occurring on <1 d per month (infrequent episodic) or 1–14 d per month for at least 3 mo (frequent episodic) 2. Headache lasting 30 min to 7 d 3. At least 1 of the following: a. bilateral b. pressing or tightening quality (nonpulsating) c. mild or moderate intensity d. not worsened by routine activity 4. Both of the following: a. no nausea or vomiting b. no more than 1 of photophobia or phonophobia Probable tension-type headache is missing 1 of the required features for formal diagnosis. Chronic tension-type headache is defined as occurring ≥15 d per month for >3 mo. Cluster headache 1. At least 5 attacks with features below 2. Severe or very severe unilateral, orbital/supraorbital, and/or temporal pain lasting 15–180 min (untreated) 3. Either or both of the following: a. at least 1 ipsilateral symptom or sign: i. conjunctival injection and/or lacrimation ii. nasal congestion and/or rhinorrhea iii. eyelid edema iv. forehead and facial sweating v. miosis and/or ptosis b. sense of restlessness 4. Occurs with a frequency from 1 every other day to 8 per day Attacks can occur in series and last weeks to months with symptom-free intervals. Usual age of onset is 20 y, but occasionally seen in children. Temporal Pattern Table 139-4 summarizes the association between overall temporal patterns of headaches and potential etiologies. In gen eral, a pattern of acute recurrent or chronic nonprogressive headaches suggests a primary headache disorder. Headache coinciding with the onset of fever suggests inflammation of some sort, typically infectious (e.g., sinusitis, pharyngitis, otitis, or meningitis), or may be associated with a more general viral syndrome.

ral, a pattern of acute recurrent or chronic nonprogressive headaches suggests a primary headache disorder. Headache coinciding with the onset of fever suggests inflammation of some sort, typically infectious (e.g., sinusitis, pharyngitis, otitis, or meningitis), or may be associated with a more general viral syndrome. Abrupt occurrence of severe headache due to a serious underlying condition, such as a brain tumor or intracranial hemorrhage, is typically associated with one or more objective findings on neurologic examination (e.g., altered mental status, ataxia, nuchal rigidity, papilledema, or hemiparesis). 3,20,33,36 Cluster headaches also tend to Tintinalli_Sec12_p0669-0996.indd 897 8/2/19 7:56 PM 898 SECTION 12: Pediatrics TABLE 139-2 Common and Life-Threatening Causes of Secondary Headache 31-33 Etiology Serious or Life-Threatening Causes Common Causes Infection Meningitis Encephalitis Orbital or intracranial abscess Viral upper respiratory tract infection Sinusitis Pharyngitis Odontogenic infection Trauma Traumatic intracranial hemorrhage Mild traumatic brain injury (concussion) Vascular Intracranial hemorrhage Stroke Hypertension Neoplastic Primary or metastatic intracranial tumor Toxic Carbon monoxide Heavy metals Cocaine Caffeine Systemic disease Diabetic ketoacidosis (cerebral edema) Systemic lupus erythematosus Vasculitis Sickle cell disease

898 SECTION 12: Pediatrics TABLE 139-2 Common and Life-Threatening Causes of Secondary Headache 31-33 Etiology Serious or Life-Threatening Causes Common Causes Infection Meningitis Encephalitis Orbital or intracranial abscess Viral upper respiratory tract infection Sinusitis Pharyngitis Odontogenic infection Trauma Traumatic intracranial hemorrhage Mild traumatic brain injury (concussion) Vascular Intracranial hemorrhage Stroke Hypertension Neoplastic Primary or metastatic intracranial tumor Toxic Carbon monoxide Heavy metals Cocaine Caffeine Systemic disease Diabetic ketoacidosis (cerebral edema) Systemic lupus erythematosus Vasculitis Sickle cell disease Other Idiopathic intracranial hypertension Temporomandibular joint dysfunction Postictal headache Fasting Sleep apnea develop acutely, whereas tension headaches have a more subacute onset. Hormonal cycles can trigger migraine headaches in adolescent females. Migraine headaches in children typically start relatively abruptly, intensify over several minutes, and then reach full intensity in about an hour. 19 Y oung children often have headaches that begin in the late afternoon.  HEADACHE PRECIPITANTS Viral illnesses and fever are among the most common causes of headache in children, and the associated headaches are most frequently frontal or temporal. 21,23-25,41 A history of trauma may suggest posttrau matic headache or traumatic brain injury. Posttraumatic headaches may be chronic as well. 42-45 Migraines, more common and better studied in adults and adolescents, may be accompanied by premonitory symptoms (prodromes such as fatigue, mood changes, or GI symptoms) and have identifiable triggers. 46 Children with prodromes tend to have more characteristic triggers as a whole, which may include specific foods (e.g., chocolate or monosodium glutamate), stress, light, specific odors, and weather changes. Headaches are among the most commonly reported symptoms in toxic exposures such as carbon monoxide poisoning. Additional precipitants include medications (e.g., methylphenidate, steroids, oral contraceptives, or anticonvulsants), infection (e.g., sinusitis, pharyngitis, or meningitis), hypertension, anemia, and substance abuse (e.g., cocaine). 31-33  LOCATION OF HEADACHE In a cohort of children with headaches presenting to a pediatric ED, only 27.5% of patients could identify a precise location of the pain. Among children with intracranial diseases, most either were unable to indicate the location of the pain or had an occipital headache. 20 More recently, studies of occipital headache in children have not shown an increased association with serious secondary causes of headache. 48,49 Medications, hypertension, and basilar-type migraines can also cause headaches in the occipital region. Pain at the vertex can be seen with sphenoid sinusitis. Ethmoid, maxillary, and frontal sinus infections tend to cause retro-orbital pain, as does meningitis (along with fever and neck stiffness) and dural sinus thrombosis. Pain seemingly in, around, or in front of the ear (or entire temporal region) is often seen with temporomandibular joint (TMJ) dysfunction and can be reproducible on exam. Migraine headaches are usually unilateral and involve the frontal or temporal region in ado lescents. However, in younger children, they are usually bifrontal or generalized. 29 Only about a third of children have unilateral migraines. Tension headaches tend to have the greatest variability in location. They may be generalized, frontal, or even occipital/posterior cervical.  QUALITY OF HEADACHE Y ounger children and many developmentally normal, otherwise healthy children may have a difficult time describing the quality of their headaches. An ability to describe pain quality or a description of the headache as having a pulsating quality is more frequently associated with benign headaches. An inability to describe the pain or a description of the headache as constrictive indicates a greater likelihood of a more serious cause.

e quality of their headaches. An ability to describe pain quality or a description of the headache as having a pulsating quality is more frequently associated with benign headaches. An inability to describe the pain or a description of the headache as constrictive indicates a greater likelihood of a more serious cause. 20,32,36,50 Many different qualities of pain can be identified: stabbing or hyperesthetic pain has been associated with herpes zoster; aching pain with tension headaches, meningitis, or encephalitis; and constant pain with sinusitis in all locations. A pulsating quality is one of the diagnostic criteria for migraine headache set forth by the International Headache Society, but can also be seen with headaches caused by hypertension or intracerebral hemorrhage.  SEVERITY OF HEADACHE The severity of a headache is neither a sensitive nor a specific charac teristic in determining cause. Patients with tension headaches can complain of terrible pain, whereas a child with a brain tumor may complain of mild to moderate pain. Nonetheless, complaints of very intense pain should be taken seriously and assessed in context with other historical elements. Ask about and document presenting pain assessments in children with primary headache disorders because treatment end points will be dictated by improved pain scores in the ED.  DURATION OF HEADACHE Although the duration of a headache is not particularly useful in assessing the majority of headaches, the International Headache Society defi nition of migraines requires a duration of symptoms of 4 to 72 hours in adults, but the duration may be less (1 to 48 hours) in children. A migraine that lasts >72 hours is known as status migrainosus. Children often present to the ED with this condition and should be treated appropriately (see later section, “Treatment”).  ALLEVIATING AND EXACERBATING FACTORS Patients with a sense of restlessness or agitation are more likely to have cluster headaches (rarer in children). 29 They may pace about the room or rock back and forth in a chair. In contrast, patients with migraines typically prefer silence and darkness because the lack of stimulation provides some relief, and photophobia/phonophobia are part of the diagnostic criteria for migraine headache (Table 139-1). 29 Tension headaches can be frequent and frustrating but tend not to be aggravated by routine physical activity.29 Positional preferences (such as a head tilt) may be noted in children with space-occupying lesions in order to avoid positions that increase intracranial pressure or exacerbate diplopia caused by cranial nerve dysfunction. In addition, children with increased intracranial pressure may be unable to look up or may avoid the Valsalva maneuver (e.g., defecation or coughing). Analgesic rebound headaches worsen when the patient goes a certain period of time without taking long-term pain medication or overuses analgesics.  ASSOCIATED SYMPTOMS Eliciting associated symptoms can help narrow the differential diagnosis. Migraine headache with aura, by definition, is associated with visual, sensory, or speech disturbances (all fully reversible; Table 139-1). Specific Tintinalli_Sec12_p0669-0996.indd 898 8/2/19 7:56 PM

or overuses analgesics.  ASSOCIATED SYMPTOMS Eliciting associated symptoms can help narrow the differential diagnosis. Migraine headache with aura, by definition, is associated with visual, sensory, or speech disturbances (all fully reversible; Table 139-1). Specific Tintinalli_Sec12_p0669-0996.indd 898 8/2/19 7:56 PM CHAPTER 139: Headache in Children 899 TABLE 139-3 Red Flag Clinical Features of Serious Secondary Headaches Clinical Features Red Flags Potential Causes Comments Chronology Sudden onset New-onset persistent pain (>2 wk) Vascular etiology Intracranial pathology SAH Pattern Wakens from sleep Early morning headache Progressive frequency/severity Elevated ICP Medication overuse can exacerbate the pattern of primary headache disorders, leading to progressive frequency/severity Location/quality Inability to describe pain Secondary headache Previously occipital location thought to be associated with secondary headaches, but no longer supported by recent evidence Exacerbating factors Recumbency Valsalva Elevated ICP Idiopathic intracranial hypertension is a less serious cause Associated symptoms Fever Morning vomiting Behavior/cognitive change Seizures Motor/gait dysfunction Visual symptoms Systemic symptoms Serious infection Intracranial pathology Diabetic ketoacidosis Neurologic symptoms concerning for intracranial pathology Demographics Age <4 y CNS tumor Primary headaches possible but less common; CNS tumors common in this age group Medical history Sickle cell disease Bleeding disorders Rheumatologic disease Malignancy Cranial shunt Phakomatoses Stroke Vasculitis Elevated ICP/bleed into tumor Intracranial mass lesion Family history No primary headache history Family history of SAH Inherited genetic disorders Secondary headache Migraines have a strong genetic component and frequent family history of headaches Other Medications Environmental/toxins Pseudotumor Analgesic overuse Carbon monoxide poisoning Heavy metal poisoning

CHAPTER 139: Headache in Children 899 TABLE 139-3 Red Flag Clinical Features of Serious Secondary Headaches Clinical Features Red Flags Potential Causes Comments Chronology Sudden onset New-onset persistent pain (>2 wk) Vascular etiology Intracranial pathology SAH Pattern Wakens from sleep Early morning headache Progressive frequency/severity Elevated ICP Medication overuse can exacerbate the pattern of primary headache disorders, leading to progressive frequency/severity Location/quality Inability to describe pain Secondary headache Previously occipital location thought to be associated with secondary headaches, but no longer supported by recent evidence Exacerbating factors Recumbency Valsalva Elevated ICP Idiopathic intracranial hypertension is a less serious cause Associated symptoms Fever Morning vomiting Behavior/cognitive change Seizures Motor/gait dysfunction Visual symptoms Systemic symptoms Serious infection Intracranial pathology Diabetic ketoacidosis Neurologic symptoms concerning for intracranial pathology Demographics Age <4 y CNS tumor Primary headaches possible but less common; CNS tumors common in this age group Medical history Sickle cell disease Bleeding disorders Rheumatologic disease Malignancy Cranial shunt Phakomatoses Stroke Vasculitis Elevated ICP/bleed into tumor Intracranial mass lesion Family history No primary headache history Family history of SAH Inherited genetic disorders Secondary headache Migraines have a strong genetic component and frequent family history of headaches Other Medications Environmental/toxins Pseudotumor Analgesic overuse Carbon monoxide poisoning Heavy metal poisoning Abbreviations: ICP = intracranial pressure; SAH = subarachnoid hemorrhage. TABLE 139-4 Temporal Patterns of Headache in Children Type Temporal Pattern Causes Acute headache Single episode of head pain without history of previous events Upper respiratory tract infection, sinusitis, first migraine, medication use, trauma Acute recurrent headache Pattern of head pain separated by symptom-free intervals Migraine Chronic progressive headache Gradual increase in frequency and severity; may be worse in the morning or awaken at night Space-occupying lesion, hematoma, pseudotumor cerebri Chronic nonprogressive headache (chronic daily) Frequent or constant headache Tension headache, cluster headache Mixed headache Acute recurrent headache superimposed on chronic daily background pattern; variant of chronic daily headache Typically migraine (acute recurrent headache) superimposed on chronic daily headache symptomatology may distinguish between types of migraines such as confusional (e.g., distortions of visual size, space, or time) and hemiplegic (e.g., transient hemiparesis or aphasia). Hemiplegic migraines are uncommon, so think carefully about other causes of neurologic deficits when contemplating the differential diagnosis. Abdominal pain, nausea, or vomiting may occur along with migraine headache. Cluster headaches can be associated with multiple ipsilateral symptoms (Table 139-1). with effortless vomiting but no GI complaints is characteristic of elevated intracranial pressure. Irreversible and progressive defects in visual acuity and diplopia are more suggestive of pseudotumor cerebri. Seizures and headache may indicate traumatic brain injury, concussion, arteriovenous malformation, subarachnoid hemorrhage, or tumor. A headache with fever and focal neurologic signs (with or without seizure) may suggest an intracranial abscess, encephalitis, or meningitis (especially with neck stiffness). Children who present with headache, altered mental status, and seizures must be evaluated for meningoencephalitis from herpes simplex virus. DIAGNOSIS The diagnosis of pediatric headache is primarily clinical and largely derived from a thorough history and physical examination.  HISTORY Obtain a thorough history to elicit clinical features related to the pre senting headache and past headaches as described earlier. Include a past medical history because acute headaches must be considered both independently and within the context of the child’s medical history: secondary headaches can occur even when there is a history of primary headaches; and although it is helpful if a child has a history of similar past headaches, a first-time headache does not preclude a primary headache disorder, as described previously. Concurrent chronic illness may Tintinalli_Sec12_p0669-0996.indd 899 8/2/19 7:56 PM

hes can occur even when there is a history of primary headaches; and although it is helpful if a child has a history of similar past headaches, a first-time headache does not preclude a primary headache disorder, as described previously. Concurrent chronic illness may Tintinalli_Sec12_p0669-0996.indd 899 8/2/19 7:56 PM 900 SECTION 12: Pediatrics TABLE 139-5 Physical Examination Findings Associated With Potential Secondary Causes of Headache Examination Component Finding Potential Causes Growth parameters Abnormal height, weight, head circumference for age Failure to thrive indicative of systemic disease; enlarged head circumference suggestive of increased intracranial pressure Vital signs Abnormal heart rate, blood pressure, respiratory rate, temperature Bradycardia, hypertension, and irregular respirations (Cushing’s triad) indicative of intracranial hypertension and impending herniation; severe hypertension associated with headache in hypertensive crisis; tachypnea possibly indicative of metabolic acidosis (e.g., diabetic ketoacidosis); fever possibly indicative of CNS or systemic infection (meningitis, encephalitis, viral syndrome) Head and neck Cranial or carotid bruits Arteriovenous malformation Bulging fontanelle Splayed sutures Macrocephaly Hydrocephalus Signs of trauma Accidental or inflicted trauma Head tilt Ocular nerve palsies from CNS tumor Eyes Papilledema Increased intracranial pressure (pseudotumor, tumor, shunt failure, intracranial bleeding, hydrocephalus) Inability to look up: “sunset eyes” Hydrocephalus Unilateral conjunctival injection, tearing, eyelid edema without tenderness Cluster headache Retinal hemorrhage Nonaccidental trauma Bilateral periorbital edema, facial tenderness, nasal discharge Sinusitis Proptosis Orbital cellulitis or tumor Ear, nose, throat

phalus) Inability to look up: “sunset eyes” Hydrocephalus Unilateral conjunctival injection, tearing, eyelid edema without tenderness Cluster headache Retinal hemorrhage Nonaccidental trauma Bilateral periorbital edema, facial tenderness, nasal discharge Sinusitis Proptosis Orbital cellulitis or tumor Ear, nose, throat Signs of infection Otitis media, mastoiditis, sinusitis, pharyngitis (including streptococcal pharyngitis), dental abscess or caries Malocclusion, TMJ tenderness TMJ dysfunction Heart Murmurs Potential shunting lesion with risk of embolic event or cerebral abscess Skin Café-au-lait spots, ash-leaf spots, vascular malformations Neurofibromatosis, tuberous sclerosis, congenital vascular malformations (risk for CNS hemorrhage) Neurologic Cranial nerve deficits Motor weakness Abnormal tone or reflexes Abnormal gait Ataxia Altered level of consciousness Any focal neurologic deficit may be associated with intracranial pathology or metabolic derangement (e.g., hyper-/hypoglycemia) Abbreviation: TMJ = temporomandibular joint. predispose the patient to unique headaches (e.g., those associated with ventriculoperitoneal shunt malfunction or infection), headaches from infection (e.g., related to anatomic defects or immunosuppression pre disposing to meningitis; or acute illness with sinusitis leading to brain abscess), or headaches caused by intracranial hemorrhage (e.g., due to hemophilia or anatomic arteriovenous anomalies). Ask about family history as well. Headaches occur more often in children who have a family history of headaches in either first- or second-degree relatives. 3,32 This association is particularly true for children with migraines (family history of migraines in up to 90% of cases). Children with cluster headaches rarely have a family history of similar headaches (about 7%). Children with a parent who experienced a subarachnoid hemorrhage are at a four times greater risk of this type of intracranial bleed than the general population. Predisposing genetic disorders may provide some clue to a familial link in headaches (e.g., bleeding diathe sis, familial dyslipidemias, or atherosclerotic events).  PHYSICAL EXAMINATION The physical examination is the most important tool to distinguish benign from life-threatening headaches. Table 139-5 lists physical exam features and their relation to important secondary headache etiolo gies. While a careful neurologic examination with attention to cranial nerves (including vision), gait, strength, and mental status is essential to exclude intracranial causes of secondary headache, additional clues to serious secondary headaches include vital signs, growth parameters, and a thorough head-to-toe exam.  LABORATORY TESTING No laboratory testing is needed for primary headaches, which are a clinical diagnosis. If secondary headache is a consideration, obtain laboratory evaluation directed by the history and physical examination. Blood tests that may be useful in specific clinical situations include CBC, serum glucose level, electrolyte levels, renal function tests, liver func tion tests, blood culture, and drug screen. Perform a lumbar puncture if meningitis or encephalitis is suspected, and send cerebrospinal fluid for Gram stain, culture, appropriate viral studies, glucose, protein, and cell count (see Chapter 120, “Meningitis in Infants and Children”). If pseudotumor cerebri is suspected, perform a lumbar puncture with the patient in the lateral decubitus position and measure the opening pres sure. A lumbar puncture may also be needed to rule out a subarachnoid hemorrhage if there is a high level of suspicion but the findings of a CT scan of the brain are normal.

”). If pseudotumor cerebri is suspected, perform a lumbar puncture with the patient in the lateral decubitus position and measure the opening pres sure. A lumbar puncture may also be needed to rule out a subarachnoid hemorrhage if there is a high level of suspicion but the findings of a CT scan of the brain are normal. 52,53 Focal neurologic deficits or signs of increased intracranial pressure warrant a CT scan prior to lumbar puncture; however, clinical signs of probable impending herniation are the best indicators of when to delay a lumbar puncture, even in the setting of normal CT scan results. Tintinalli_Sec12_p0669-0996.indd 900 8/2/19 7:56 PM

rologic deficits or signs of increased intracranial pressure warrant a CT scan prior to lumbar puncture; however, clinical signs of probable impending herniation are the best indicators of when to delay a lumbar puncture, even in the setting of normal CT scan results. Tintinalli_Sec12_p0669-0996.indd 900 8/2/19 7:56 PM CHAPTER 139: Headache in Children 901 TABLE 139-7 Suggested ED Treatment Algorithm for Primary Headaches* Migraine and Cluster Headache Tension Headache Normal saline bolus (20 mL/kg IV; max of 1 L) + Ketorolac (0.5 milligram/kg IV; max 30 milligrams) † + Prochlorperazine (0.15 milligram/kg IV; max 10 milligrams) Metoclopramide (0.1 milligram/kg IV; max 10 milligrams) + Diphenhydramine (1 milligram/kg IV; max 50 milligrams) ‡ ↓ If no significant relief, consider neurology consultation prior to other therapies such as dexamethasone Ketorolac (0.5 milligram/kg IV; max 30 milligrams) † + Acetaminophen PO (15 milligrams/kg; max 1000 milligrams) *For patients who have failed first-line therapy with oral ibuprofen or acetaminophen. †Do not give ketorolac if ibuprofen taken within 6 h. ‡Diphenhydramine may be given prophylactically as part of a “cocktail” or used only to treat extrapyramidal side effects of dopamine antagonists.  IMAGING Imaging studies are not needed for primary headache disorders or recurrent headaches when the findings of the neurologic examina tion are normal. Despite the importance of history and examination, one study reported that almost 40% of all children with headache who were evaluated in the ED underwent head imaging with radiation. There is a very low incidence of positive findings on neuroimaging studies in children with headache who have normal findings on physical examination. 2,34-37,56,57 Consider imaging in children with abnormal findings on neurologic examination, altered mental status, or concurrent seizures, or if medical history indicates the recent onset of severe (worst) headache, a change in the type of headache, or associated features that suggest neurologic dysfunction. 3,32,34-37,58 Reserve CT scans for instances in which there is a highly suspicious history, neurologic findings on examination are abnormal, or an intracranial hemorrhage or a space-occupying lesion is suspected. Noncon trast head CT is sensitive for detecting skull fracture and brain injury from trauma and may identify supratentorial tumors and stroke; give IV contrast if considering intracranial abscess. A normal head CT does not exclude an intracranial mass because the posterior fossa is not well visualized. The posterior fossa is the most common location of brain tumors in children, and MRI of the brain provides better visualization of the posterior fossa. Magnetic resonance angiography and venography are useful when vascular malformations or dural sinus thrombosis is suspected. MRI may require sedation based on the patient’s age. The decision to admit a child for MRI or to obtain outpatient imaging depends on the practice location and deserves a discussion with an inpatient team as well as a primary care provider. TREATMENT Treatment of headache in the ED depends on etiology. Treat the underlying cause of secondary headache and provide analgesia for pain. For secondary headaches, simple analgesics such as NSAIDs and acetaminophen are usually sufficient, although opioid analgesics may be needed for some severe secondary headaches (see Chapter 115, “Pain Manage ment and Procedural Sedation in Infants and Children”). Abortive therapy for primary headaches also varies by type. Few prospective randomized trials for migraine therapy have been conducted on children, and even fewer in the ED setting, so most recommendations are extrapolated from adult trials, and significant practice variation has been observed.

Infants and Children”). Abortive therapy for primary headaches also varies by type. Few prospective randomized trials for migraine therapy have been conducted on children, and even fewer in the ED setting, so most recommendations are extrapolated from adult trials, and significant practice variation has been observed. 55,59-63 Table 139-6 lists various pharmacologic options for migraine and cluster headache along with recommended dosing. Overall, abortive therapy in the ED is highly successful, with >85% of children successfully discharged and only a 5.5% ED return visit rate.  FIRST-LINE THERAPY First-line therapy for all types of primary headaches includes oral NSAIDs and/or analgesics. Both ibuprofen and acetaminophen are effective first-line therapy, although ibuprofen is more effective when given as monotherapy. 65 Ketorolac is an effective parenteral NSAID, although it should not be used if the patient has taken an oral NSAID within 6 hours. Although superior to placebo, ketorolac appears to be less effective than prochlorperazine (see below). 66 Do not use narcotics for primary headaches. Long-term use of narcotics can change the painmodulatory system in the brainstem and can lead to more intense pain.67,68 The most widely studied migraine-specific abortive medications are the triptans and the dopamine antagonists. Triptans have demonstrated some efficacy in the outpatient and home setting and are considered first-line therapy in that setting; however, they are more effective when taken early in the course of headache and therefore of less utility in the ED setting. 69,70 If used, intranasal and subcutaneous routes are more effective than oral administration.63,65,69  SECOND-LINE THERAPY Many children with primary headaches presenting to the ED have already tried and failed first-line therapy, and a significant proportion of those with migraine present with status migrainosus. TABLE 139-6 Pharmacologic Abortive Therapy for Primary Headaches Class Medication Dose NSAIDs Ibuprofen Ketorolac 10 milligrams/kg: PO (max: 800 milligrams/dose or 2400 milligrams/d) 0.5 milligram/kg: IV (max: 30 milligrams/dose) Analgesics Acetaminophen 15 milligrams/kg: PO/PR (max: 1 gram/dose or 4 grams/d) Dopamine antagonist Prochlorperazine Metoclopramide 0.15 milligram/kg: IV (max: 10 milligrams/dose) 0.1 milligram/kg: IV (max: 10 milligrams/dose) Other antiemetic Diphenhydramine Promethazine 1 milligram/kg: IV (max: 50 milligrams/dose) 0.25–1 milligram/kg: IV (max: 25 milligrams/dose) Triptans (more available than listed here) Sumatriptan Rizatriptan Sumatriptan/ naproxen combination Multiple routes: 5–20 milligrams: intranasal 3–6 milligrams: SC 50–100 milligrams: PO 5–10 milligrams: PO Multiple combinations 85 milligrams/500 milligrams: PO (sumatriptan/naproxen) 30 milligrams/180 milligrams: PO 10 milligrams/60 milligrams: PO Dopamine antagonists are the most studied parenteral abortive medications for migraine in the ED and include prochlorperazine, meto clopramide, promethazine, and chlorpromazine. Small studies suggest differential effectiveness among this class, with highest treatment success for prochlorperazine, followed by metoclopramide; promethazine and chlorpromazine appear to be less effective. 63,64,71,72 One large study using administrative data suggested higher rates of ED return visits among children treated with metoclopramide compared to prochlorperazine. 64 These drugs can cause dystonic or extrapyramidal reactions, which are relieved or prevented by diphenhydramine. 73,74 Many emergency medicine providers use a “cocktail” of medications for migraine treatment that includes ketorolac, prochlorperazine or metoclopramide, and diphenhydramine. This combination is effective and safe.

ugs can cause dystonic or extrapyramidal reactions, which are relieved or prevented by diphenhydramine. 73,74 Many emergency medicine providers use a “cocktail” of medications for migraine treatment that includes ketorolac, prochlorperazine or metoclopramide, and diphenhydramine. This combination is effective and safe. 48 The authors recommend this approach for patients who have failed first-line therapy, and Table 139-7 summarizes combination Tintinalli_Sec12_p0669-0996.indd 901 8/2/19 7:56 PM