Disclaimer: The information contained within the Grand Rounds Archive is intended for use by doctors and other health care professionals. These documents were prepared by resident physicians for presentation and discussion at a conference held at Baylor College of Medicine in Houston, Texas. No guarantees are made with respect to accuracy or timeliness of this material. This material should not be used as a basis for treatment decisions, and is not a substitute for professional consultation and/or peer-reviewed medical literature. Pediatric Headache Headache is a common complaint in pediatric practice. Up to 75% of children experience a headache before the age of 15. The list of causes is extensive, and frequently the otolaryngologist's input is requested in search for an etiology. Diagnostic approaches proceed along two general lines. The more complete is to consider the pathophysiologic characteristics of the symptom complex. However this is time consuming and often confusing. A more useful approach is based on the time course of the symptoms, and this organizational construct will be presented. The differential diagnosis of headache is expansive. A thorough history is the most effective means of elucidating the cause. The quality, location, timing, severity, frequency, precipitating event and duration of the headache are important historical features. This information along with whether the headache is progressive allows one to focus the search for a specific etiology. The past medical history should investigate prior central nervous system surgery or trauma, an allergic history, and concurrent medical illnesses such endocrine disorders and hypertension. A family history is positive in greater than 90% of cases of migraine. Most patients with intracranial pathology will present with focal neurologic symptoms and signs. A fundoscopic exam can point to increased intracranial pressure as a cause of the headache. For the otolaryngologist, endoscopic intranasal examination as well as a complete oral exam will help in the diagnosis of sinusitis or a temporomandibular disorder. Laboratory and radiographic studies are overused in the diagnosis of headache. Their use should be directed by the history and physical. As noted earlier, the differential for headache is broad. It is useful to divide headaches into four temporal groups in order to narrow this list. Acute headaches are those that develop over 24 hours with no preceding history. Chronic progressive headaches are those that worsen over days to weeks. Chronic non-progressive headaches are present for longer than 2 months without significant change in character. Chronic recurrent headaches are those that recur with intervening symptom-free intervals. The differential for an acute headache includes vascular malformations with associated intracranial hemorrhage, inflammatory, or infectious causes, and trauma. Intracranial hemorrhage in children usually results from a preexisting vascular malformation. Arteriovenous malformations present more commonly than aneurysms in kids. 20% of AVM's will present prior to 20 years of age. Aneurysms also present with hemorrhage in childhood but much less frequently. Faulty heating systems that leak carbon monoxide gas, toxic ingestions, and exposure to solvents or toxic vapors can cause headache acutely. In the child with fever, an altered sensorium, and a stiff neck, meningitis or other CNS infection should be considered. A high index of suspicion is advisable as even in meningitis the child's temperature may not point to an infectious cause and rapid neurologic damage may ensue. Cerebral venous thrombosis, vasculitis, an acute hypertensive episode, and optic neuritis are much less prevalent but should be considered. Finally, and important for the otolaryngologist, is sinusitis, which will be discussed separately later as a cause of headache. Chronic progressive headache is the headache that worsens over days to a week without relenting. This pattern is usually associated with an intracranial mass lesion that causes increased intracranial pressure, traction on basilar pain-sensitive structures, or meningeal irritation. The pain is characteristically over the forehead region in a supratentorial mass, and for an infratentorial mass is referred to the occiput. Warning signs that may indicate serious intracranial pathology include a crescendo headache, being awakened by the headache, focal neurologic findings, or nausea and vomiting associated with the headache. Besides brain tumors themselves, hydrocephalus or pseudotumor may cause a progressively worsening or crescendo headache. Subdural and epidural hematomas can occur in children, and may present with a much more indolent course than in adults. Not all will require surgical intervention. Chronic non-progressive headache as stated earlier is a headache that is steadily present for longer than 2 months and has a waxing and waning character. In children, depression and anxiety-related problems often cause this type of headache. Tricyclic antidepressants have been successful in treatment. Issues of secondary gain should also be considered, such as school avoidance. Chronic recurrent headache in children with interval asymptomatic periods is most often related to migraine. Migraine occurs in approximately 4% of children and onset is less than 10 years of age in 25% of all people with migraines. The second most frequent cause of recurrent headache appears to be the tension or "muscle contraction" headache. Cluster headaches are more prevalent in male adults but can also occur in late childhood. In 1976 Prensky set forth the guidelines for the diagnosis of childhood migraine. The major criterion of a paroxysmal recurrent headache lasting 2 to 72 hours must be established in all cases. Further, three of the six minor criteria must be satisfied. These include an aura prior to the headache, unilateral headache, throbbing quality, associated GI symptoms such as nausea, relief with deep sleep, and a family history of migraines or recurrent headaches. The spectrum of what constitutes a migraine runs from the common migraine, to the classic form, to more complex syndromes associated with migraine. The common migraine, comprising almost 70% of migraine sufferers, is the most prevalent form in adults and children. No aura is associated and the pain is usually bilateral and throbbing in nature. The location of the pain is usually frontal. This is in distinction to the classic migraine that occurs in 25% of migraine sufferers. A typical visual aura and a unilateral throbbing headache are diagnostic of this form. The aura is usually blurred vision or scotomas. Syndromes with little or no associated headache are known as migraine variants. They present as organ dysfunction in a known migraineur or in a person with a family history of migraine. Basilar artery migraine is believed to be due to dysfunction in the distribution of the vertebrobasilar artery flow. Symptoms vary from headache, to vertigo, to ataxia, to cranial nerve palsies, to tinnitus, and epileptiform activity. The vast majority of children do well with symptoms resolving within 12 hours; however, in severe forms some will have permanent neurologic sequelae. The Alice-in-Wonderland syndrome consists of alterations of time sense, body image, and visual analysis of the environment in the presence of a clear sensorium. Ophthalmoplegic migraine usually manifests first in children less than 12 years old as a unilateral headache in or near the orbit followed by occulomotor palsy. Hemiplegic migraine usually occurs in children less than three and is clinically manifested as intermittent spells of hemiplegia. Although frightening to parents, permanent sequelae are fleetingly rare. Acute confusional migraine presents with an acute change in level of consciousness, impairment of memory, and variable agitation in a child 5 to 15 years old. Symptoms last form minutes up to 24 hours. Treatment of migraine in children is based mostly on empiric data. Fortunately, the majority of children with migraine remit before entering adulthood. In fact, 50% show improvement within 6 months of diagnosis. It is important to remove triggering factors such as stressful situations. Acute pharmacological therapy remains the gold standard though, and begins with acetaminophen or non-steroidal anti-inflammatory agents. In an attempt to abort an oncoming migraine, ergots have been applied. They are given during the vasoconstriction phase that precedes the symptomatic vasodilatation phase. Their intended action is to prevent significant vasodilatation, thus aborting the ensuing headache. Side effects include nausea and vomiting as well as epigastric discomfort. Prophylactic treatment is often used for a 4 to 6 month period. Propranolol and amitryptilline are the two most commonly used medications. Calcium channel blockers, cyproheptadine, and phenytoin have also been used with some success. Tension headache or muscle contraction headache is similar in adults and children. It is usually stress-related and presents with symptoms of a bilateral band-like, dull, aching frontotemporal headache. The incidence is the same in both men and women. Treatment is focused towards reassurance that the headache will resolve with analgesic medications. Cluster headaches are unusual before the second decade. The classic presentation is a unilateral periorbital pain associated with ipsilateral lacrimation and periorbital pain. This is the only headache type that has a male predominance. Treatment with supplemental oxygen has been successful in abolishing the headache. Non-steroidal anti-inflammatory drugs and calcium channel blockers have also been successfully used. As in migraine, ergots and methysergide are additionally effective. Temporomandibular disorders cause headache and result from internal derangements of the joint such as miniscus dislocation. This most often occurs anteriorly and medially and causes pain in the distribution of the auriculotemoporal nerve. Degenerative joint disease can cause of acute point tenderness over the joint, but is less common in childhood. There is also a more vaguely described myofascial pain syndrome associated with temporomandibular dysfunction. The diagnosis of temporomandibular disorders can most often be made by pain on palpation over the joint area or with mastication. A click may be felt during jaw movement. Treatment includes non-steroidal anti-inflammatory medications, orthodontic devices to correct malocclusion, and surgery to correct internal derangements of the joint space. In 1988 Faleck and Rothner from the Cleveland Clinic division of pediatric neurology looked at 150 children who presented with the complaint of chronic headache. Of this group, 15 or 10% were found to have sinusitis as the cause of the headache. There were eight boys and seven girls Eleven responded to medical therapy with antibiotics and decongestants, and 4 required surgical drainage. All responded to treatment. Faleck pointed out in the article that tension headache and sinus headache are often similar in quality, and a CT Scan offered definitive evidence for a sinus etiology. The paranasal sinuses have classic locations for referred pain that can be a clue to the site of anatomic pathology. The maxillary sinuses refer pain in an acute infection to the cheeks. In children, chronic sinusitis is often painless. The additional complaints of nasal congestion or rhinorrhea are usually present as supporting evidence of a maxillary infection. Acute and chronic ethmoiditis present similarly with a fronto-orbital headache. Nasal polyps are evidence pointing to a more prolonged process. Frontal sinusitis is more common in adults as their development in children is often rudimentary. Pain is predominantly over the forehead. As in chronic maxillary sinusitis, chronic frontal sinus infections are often painless. Sphenoid sinusitis is also more common in adults and results in deep orbital pain, fever, and purulent nasal discharge. It is most frequently concurrent with an ethmoid sinus infection. In 1986 Greenfield presented his data supporting the contact or pressure theory of rhinologic pain. He applied noxious stimuli to specific regions of the nose and documented a topographic pattern of referred pain. His research went further to demonstrate that afferent type C nerve fibers sent their impulses along the first and second branches of the trigeminal nerve. One pattern was orthodromic ending in the CNS and was responsible for the perceived pain. The other local antidromic pattern resulted in local tissue edema, vasodilatation, and smooth muscle contraction. Stammberger and Wolff later found that the neuropeptide, substance P, was responsible for these events. They also describe 5 anatomic locations that are frequently the source of a rhinologic etiology for the headache- they are septal spurs, the agger nasi cells, the uncinate process, middle turbinate, and the ethmoid bulla. A systematic examination of these locations provides a thorough evaluation of potential sites of pathology. Endoscopic evaluation should be combined with coronal CT images to elucidate sites of pathology that may be implicated by the history. Stammberger states that in patients with one of the abnormalities described above and the primary complaint of headache, a dramatic improvement can be seen within hours of surgical correction. No study to date, to this author's knowledge, has addressed the effectiveness of such an approach used solely for the management of headache. In conclusion, the cause of headache is numerous. Migraine is the most common type in children. In order to organize ones thought process is important to classify the headache as acute, chronic progressive, chronic non-progressive, or recurrent. Once this is done a more focused evaluation can follow. The otolaryngologist must pay close attention to intraoral and intranasal pathology. Examination of the five major areas of intranasal pathology described by Stammberger may help in elucidating a cause of an otherwise difficult to explain headache. Case Presentation A 9-year-old boy with cystic fibrosis presented to his pulmonologist with the complaint of a bifrontal steady aching headache for two weeks. He denied any fever, purulent nasal drainage, cough, or auras associated with the onset of the headache, but did complain of a sense of pressure and pain on palpation between his eyes. Past medical history was significant only for his cystic fibrosis. There was no family history of migraines. His physical exam showed edematous polypoid changes of the nasal mucosa, but there were no polyps obstructing the middle meatus and no purulent exudate was noted. Mild pain was elicited on palpation of the nasal bridge over the intercanthal area. The neurologic examination was non-focal. A CT scan of the sinuses was obtained and demonstrated pansinusitis. A diagnosis of headache related to sinusitis was made and he was treated with intravenous antibiotics along with nasal steroids, decongestants, and a mucolytic agent. Within one week on the initiation of therapy he was asymptomatic. He is currently being followed by the pulmonary and otolaryngology services at the Texas Children's Hospital and is without recurrence of his symptoms. Bibliography Barabas G. Management of headaches in childhood. Pediatr Ann 1983;12:806-813. Bille B. Migraine in school children. Acta Paediatr Scand 1962;51(Suppl 136):1-151. Clerico DM, Fieldman R. Referred headache of rhinogenic origin in the absence of sinusitis. Headache 1994;34:226-229. Congdon PJ, Forsythe WI. Migraine in childhood: a study of 300 children. Dev Med Child Neurol 1979;21:209-216. Elkind AH. Headache and facial pain associated with head injury. Otolaryngol Clin North Am 1989;22:1251-1269. Faleck H, Rothner AD, Erenberg G, Cruse RP. Headache and subacute sinusitis in children and adolescents. Headache 1988;28:96-98, 1988. Friedman WH, Rosenblum BN. Paranasal sinus etiology of headaches and facial pain. Otolaryngol Clin North Am 1989;22:1217-1228. Gascon, GG. Chronic and recurrent headache in children and adolescents. Pediatric Clin North Am 1984;31:1027-1051. Greenfield H. A study of referred cephalgia secondary to noxious stimulation of specific regions of the nasal mucosa and sinus ostia. Doctors Harold Wolff and Donald Delessio revisited. Presented as a scientific exhibition at the meeting of the American Academy of Otolaryngology, San Antonio, Texas, Sept. 14-18,1986. Hockaday JM, Barlow CF. Headache in children. In: Olesen J, Tfelt-Hansen P, Welch KM, eds. The Headache. New York: Raven, 1993:795-808. Jay GW, Tomasi LG. Pediatric headaches: A one year retrospective analysis. Headache 1981;21:5-9. Labbé EE. Childhood muscle contraction headache: current issues in assessment and treatment. 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Otolaryngol Clin North Am 1989;22:1131-1144. Saper JR. Migraine, migraine variants, and related headaches. Otolaryngol Clin North Am 1989;22:1115-1130. Silberstein SD. Twenty questions about headaches in children and adolescents. Headache 1990;30:716-724. Sorge F, Barone P, Steardo L, Romano MR. Amitriptyline as a prophylactic for migraine in children. Acta Neurol 1982;5:362-367. Stammberger H, Wolf G. Headache and sinus disease: the endoscopic approach. Ann Otol Rhinol Laryngol 1983;97(Suppl 134):3-23. Truta MP, Santucci ET. Head and neck fibromyalgia and temporomandibular arthralgia. Otolaryngol Clin North Am 1989;22:1159-1171. Grand Rounds Archive | Department Home page BCM Public | BCM Intranet | Privacy Notices | Contact BCM | BCM Site Map | ©2001-2006 Baylor College of Medicine
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