Elsevier

Pediatric Neurology

Volume 61, August 2016, Pages 76-82
Pediatric Neurology

Original Article
Manifestations of Pediatric Intracranial Hypertension From the Intracranial Hypertension Registry

https://doi.org/10.1016/j.pediatrneurol.2016.04.007Get rights and content

Abstract

Objective

The purpose of this study was to examine the presenting symptoms, demographics, and interventions in pediatric patients enrolled in the Intracranial Hypertension Registry.

Methods

We analyzed confirmed intracranial hypertension patients ≤18 years at the time of initial diagnosis who were enrolled in the registry.

Results

A total of 203 patients met the criteria for inclusion; 142 (70%) were considered primary intracranial hypertension. Females made up 72.5% (103 of 142) and 75.8% (47 of 61) in the primary intracranial hypertension and secondary intracranial hypertension groups, respectively. There were no clinically significant differences in age, body mass index, or opening pressure between the primary intracranial hypertension and secondary intracranial hypertension groups. Symptoms most often reported were headache and blurred vision. Bilateral optic disc edema occurred in 89.3% of primary intracranial hypertension and 78.7% of secondary intracranial hypertension patients. When divided into pre- and postpubertal status, 32.5% of patients were classified prepubertal; 77.3% of these had primary intracranial hypertension. This resulted in a female to male ratio of 1:1.04 for prepubertal and 6:1 for postpubertal primary intracranial hypertension patients. The body mass index was significantly higher in the postpubertal primary intracranial hypertension group (P = 0.0014). There was no significant difference in opening pressure.

Conclusions

The common symptoms of intracranial hypertension, including headache, optic disc edema, and vision changes, occurred with similar frequencies in our cohort to those reported in the literature. In separate subanalyses, we found significantly higher rates of obesity in postpubertal females with primary intracranial hypertension. The female-to-male ratios in the postpubertal primary intracranial hypertension and secondary intracranial hypertension groups were higher than reported in the literature.

Introduction

Primary intracranial hypertension (PIH; idiopathic intracranial hypertension, pseudotumor cerebri) has been considered rare. Although the annual incidence in the United States is estimated at 0.9 per 100,000, there are no good estimates in the pediatric population.1 In Germany the annual pediatric incidence is 0.5 per 100,000 and, in Croatia, 1.2 per 100,000.2, 3

The Intracranial Hypertension Research Foundation (henceforth the Foundation) began in 2001 as a nonprofit organization in Vancouver, Washington to support research on this condition. The Foundation, in partnership with the Casey Eye Institute at Oregon Health and Science University, formed the Intracranial Hypertension Registry (the Registry) in 2003 as an initial step to promote research. The Registry is a physician-controlled project that invites and collaborates with outside researchers wishing to use Registry data. Periodically, the Foundation hosts informational patient conferences with physician and research speakers. The Registry is comprised of intracranial hypertension patients who enrolled by completing an entry questionnaire and agreeing to collection of their medical records and annual follow-up reporting. Patients of any age are accepted after their records are reviewed to confirm the diagnosis of intracranial hypertension.

The purpose of this study was to examine the presenting symptoms, demographics, and interventions in pediatric patients enrolled in the Registry.

Section snippets

Materials and Methods

Institutional Review Board for Human Research of Nationwide Children's Hospital approved the study. We analyzed aggregated de-identified Registry data from individuals who were 18-years-old or less at the time of initial diagnosis.

The Registry is made up of individuals who were largely self-referred to the Registry after being diagnosed with intracranial hypertension. After initial contact from the patient or patient provider, releases are signed and the patient's medical records are gathered.

Results

A total of 203 individuals aged 18 years or less met the criteria for inclusion in the analysis: 142 (70%) were classified as PIH and the remaining 61 as secondary intracranial hypertension (SIH). Females made up 72.5% (103 of 142) and 75.4% (46 of 61) in the PIH and SIH groups, respectively (Table 1). The ethnic composition between the groups was 90.9% and 91.8% Caucasian, 3.5% and 1.6% African-American, 2.8% and 1.6% Hispanic, and 2.8% and 4.9% other for PIH and SIH, respectively. Neither

Discussion

We analyzed a cohort of 203 pediatric patients diagnosed with intracranial hypertension and enrolled in a registry with systematic collection and review of the patient records. Our report has several limitations, the first of which is that most were self-referred to the Registry which may produce a bias toward more severe disease or more unusual manifestations. Anytime records are sent from another location; there is the potential for incomplete records being obtained. This is apparent in the

Conclusions

Overall, many of the common symptoms of intracranial hypertension, including headache, optic disc edema, and vision changes, occurred with similar frequencies in our large cohort to those reported in the literature. Medical management with acetazolamide was the most common treatment. In separate subanalyses, we found significantly higher rates of obesity in postpubertal PIH females. The female-to-male ratios in the postpubertal groups were higher than reported in the literature. This may be the

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