Pediatric Pseudotumor Cerebri: Descriptive Epidemiology

OBJECTIVE: To determine the epidemiological profile for pediatric pseudotumor cerebri. METHODS: A retrospective case series was identified from a geographically limited region of 205,765 children aged 2-15 years at the only tertiary care pediatric hospital with all pediatric neurologists and ophthalmologists. Health records identified 35 children with ICD9 code 348.2 (benign intracranial hypertension) presenting between April 1, 1979 and March 31, 1994. After chart review, 29 cases were identified which satisfied modified Dandy's diagnostic criteria for pseudotumor cerebri. RESULTS: The 29 cases ranged in age from 3-15 years. The annual incidence of symptomatic disease was 0.9 per 100,000 children (95% CI 0.6, 1.4). Cases were 2.7 times more likely to be female (21/29, X2 p = .01) and twice as likely to be adolescent (age 12-15 years), (X2 p = .04). Based upon these distinctions, the following estimates of age and sex specific disease incidence were derived (based upon 100,000 child years of exposure): male age 2-11 years: 0.4; male age 12-15 years: 0.8; female age 2-11 years: 1.1; female age 12-16 years: 2.2. CONCLUSIONS: These data appear to be unique for its the estimation of age and sex specific incidence rates for pediatric pseudotumor cerebri.