Pseudotumor cerebri in children

Introduction: Pseudotumor cerebri is an uncommon cause of permanent visual loss in childhood. Demographic and outcome data in the era of neuro-imaging are needed to improve our understanding of this disease. Method;: We reviewed our database for the records of children < 18 years of age who were diagnosed with pseudotumor cerebri between 1985-1996. Visual function, treatment and eventual etiology were tabulated. Results; 25 patients had an initial diagnosis of pseudotumor cerebri. Evaluation revealed another cause of elevated intracraniai pressure in 9 of these patients. 4 patients had venous sinus thrombosis, 2 patients had Arnold-Chiari malformations, 2 patients had central nervous system malignancy and 1 patient had Lyme disease. These patients were excluded from further analysis. The remaining 16 patients included 5 boys (31%) and 11 girls (69%) with an age range of 3-17 years of age. Presenting symptoms included headache (81%), decreased vision (38%) and diplopia (25%). 3 patients (19%) had a visual acuity < 20/40 in the best eye at presentation. Other ocular signs at presentation included visual field deficits (25%), papilledema (75%), cranial nerve VI palsy (19%), esotropia (13%) and other cranial nerve palsies (19%). Follow-up data ranging from 2-26 months was available for 9 patients. Only 1 patient (11%) had a final visual acuity < 20/40 in the best eye. 5 patients (56%) had residual optic nerve atrophy and visual field deficits. Conclusions; Permanent visual loss continues to occur in patients with pseudotumor cerebri. In rare instances, the patient diagnosed with pseudotumor cerebri will prove after extended follow-up to have elevated intracraniai pressure secondary to an intracraniai neoplasm or another type of masquerade syndrome.