A number of conditions (eg, hydrocephalus, intracranial mass lesions, meningitis, idiopathic intracranial hypertension) can cause increased ICP in children and thus disc swelling (Table 26-2). A full evaluation, including neuroimaging possibly followed by lumbar puncture, is indicated. In infants, increased ICP results in firmness and distention of the open fontanelles. Significantly elevated pressure is usually accompanied by nausea, vomiting, and headaches. Older children may describe transient visual obscurations. Sixth nerve palsy can be a sign of elevated ICP.
Idiopathic Intracranial Hypertension
Idiopathic intracranial hypertension (IIH), or pseudotumor cerebri, is characterized by increased ICP with normal-sized or small ventricles on neuroimaging, and normal cerebrospinal fluid. IIH is uncommon in childhood but can occur at any age. It may be associated with viral infections, excessive vitamin A, and certain drugs (eg, tetracycline, corticosteroids, nalidixic acid, thyroid medications, and growth hormone). Magnetic resonance venography is recommended to rule out cerebral venous sinus thrombosis. In prepubescent children with IIH, the incidence of obesity is lower compared with that in adult IIH patients, and the male to female ratio is approximately equal. Postpubescent children with IIH have a clinical profile similar to that of adult IIH patients, with a higher incidence of obesity and female preponderance. Down syndrome is also associated with IIH.
Table 26-2 Conditions Associated With Pediatric Optic Disc Swelling
Common presenting symptoms are headache, vision loss, transient visual obscurations, and diplopia. Papilledema may be noted on routine examination of an asymptomatic child. Examination frequently reveals excellent visual acuity with bilateral papilledema. Unilateral or bilateral sixth nerve palsy may be present. The patient should be monitored closely for decreased visual acuity, visual field loss, and worsening headaches. Visual field tests can be difficult to interpret in children but should be performed if possible.
Treatment of IIH begins with discontinuation of any causative medications. Medical treatment includes acetazolamide and topiramate (see Fig 26-12). Video 26-1 shows the gradual resolution (over a 3-month period) of papilledema in a child being treated for IIH. Surgical treatment options include optic nerve sheath fenestration or shunting procedures (lumbar or ventriculoperitoneal), both of which can reduce the incidence of vision loss. Shunting procedures are preferred for patients with good visual function and severe headaches unresponsive to medical management. With treatment, the visual prognosis is excellent for most patients, although vision loss can occur secondary to chronic papilledema. In most cases, spontaneous resolution occurs within 12–18 months of initial treatment.
See BCSC Section 5, Neuro-Ophthalmology, for further discussion.
Gradual resolution of papilledema during treatment of idiopathic intracranial hypertension.
Courtesy of Robert W. Hered, MD.
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Excerpted from BCSC 2020-2021 series: Section 10 - Glaucoma. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.