Pediatric emergency medicine trisk 2624 2624
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Clinical Recognition. Headache of variable severity and duration is the most
common presenting symptom. It is typically worse in the morning. Nausea,
vomiting, dizziness, and double or blurred vision also occur. If the process is long
standing, decreased visual acuity or visual field deficits can result. Infants often
have nonspecific symptoms of lethargy or irritability. Papilledema is seen in
virtually all cases, although a syndrome of IIH without papilledema exists. Other
neurologic symptoms and signs are often absent; however, unilateral or bilateral
VI nerve paresis may be seen.
Initial Assessment. Diagnosis should be considered when a child with a
prolonged history of headache is found to have evidence of papilledema without
other neurologic findings. IIH is a diagnosis of exclusion, and other conditions,
particularly mass lesions, must be ruled out. Because posterior fossa tumors and
obstructive or nonobstructive hydrocephalus may mimic IIH early in the course
of disease, neuroimaging should be obtained in all children with this constellation
of findings. MRI is the study of choice, though contrast-enhanced CT may be
used if MRI is unavailable or contraindicated. Magnetic resonance venography
(MRV) is useful in identifying cerebral venous anomalies in atypical (i.e.,
younger, male, or nonobese) patients. In cases of IIH, the ventricles will appear
normal or small.
If no mass lesion is present, an LP should be performed with a manometer to
measure opening pressure. The patient must be in the lateral decubitus position
with legs extended to ensure an accurate reading of the opening pressure.
Children with idiopathic (e.g., not secondary to Lyme infection or other causes)
have elevated opening pressure (greater than 280 mm CSF) but normal CSF cell
count, protein, and glucose. In children with intermittent symptoms, the opening
pressure may be normal when the headache is waning, even though papilledema
may persist for several weeks.
Management. For patients with visual changes or cranial nerve involvement,
neurosurgical as well as ophthalmologic consultation is recommended. For the
large majority of patients, removal of sufficient CSF to normalize ICP usually
leads to improvement in symptoms. Treatment may then be started with
acetazolamide (Diamox) to decrease CSF production (60 mg/kg/day divided four
times daily). Although recommended by some, corticosteroids have not been
proven to be effective in the management of this condition and should only be
administered after consultation with a neurologist.
