Pediatric Idiopathic Intracranial Hypertension

Abstract

Purpose To examine anthropometric and maturational characteristics at diagnosis in pediatric idiopathic intracranial hypertension (IIH). Design Retrospective, international, multisite study. Participants Pediatric patients (2–18 years of age at diagnosis) with IIH. Main Outcome Measures Body mass index (BMI), height, and weight Z-scores; sexual maturation. Methods Cases of IIH were identified retrospectively based on diagnostic code, pediatric neuroophthalmologist databases, or both and updated diagnostic criteria (2013) were applied to confirm definite IIH. Anthropometric measurements were converted into age- and gender-specific height, weight, and BMI Z-scores CDC 2000 growth charts. When available, sexual maturation was noted. Results Two hundred thirty-three cases of definite IIH were identified across 8 sites. In boys, a moderate association between age and BMI Z-scores was noted (Pearson’s correlation coefficient, 0.50; 95% confidence interval [CI], 0.30–0.66; P < 0.001; n = 72), and in girls, a weak association was noted (Pearson’s correlation coefficient, 0.34; 95% CI, 0.20–0.47; P < 0.001; n = 161). The average patient was more likely to be overweight at diagnosis at age 6.7 years in girls and 8.7 years in boys, and obese at diagnosis at age 12.5 years in girls and 12.4 years in boys. Compared with age- and gender-matched reference values, early adolescent patients were taller for age (P = 0.002 in girls and P = 0.02 in boys). Data on Tanner staging, menarchal status, or both were available in 25% of cases (n = 57/233). Prepubertal participants (n = 12) had lower average BMI Z-scores (0.95±1.98) compared with pubertal participants (n = 45; 1.92±0.60), but this result did not reach statistical significance (P = 0.09). Conclusions With updated diagnostic criteria and pediatric-specific assessments, the present study identifies 3 subgroups of pediatric IIH: a young group that is not overweight, an early adolescent group that is either overweight or obese, and a late adolescent group that is mostly obese. Data also suggest that the early adolescent group with IIH may be taller than age- and gender-matched reference values. Understanding these features of pediatric IIH may help to illuminate the complex pathogenesis of this condition.

The incidence of obesity, venous sinus stenosis and cerebral hyperaemia in children referred for MRI to rule out idiopathic intracranial hypertension at a tertiary referral hospital: a 10 year review

“…Obesity raises the central venous pressure by up to 20 mmHg [ 9 , 23 ] (the third component of Ohm’s law) and therefore sinus pressure in IIH. In comparison, in a large series of children with IIH, in those less than 12.4 years of age, 45% were obese [ 24 ]. In the current study, both patients with definite IIH were obese, the probable IIH patient was not (Additional file 1 : Table S1).…”

What are the research priorities for idiopathic intracranial hypertension? A priority setting partnership between patients and healthcare professionals

“…It is well documented the expectantly different phenotype between adult and those prepubescent children with IIH. 14 However, responses were not limited by those who submitted and hence, those with children with IIH are likely to be included. Indeed, at the final stakeholder meeting, there was representation from carers of children with IIH.…”

An Unusual Presentation of Atypical Idiopathic Intracranial Hypertensionin a Pre-Pubertal Child

“…Secondly, this patient is a female who falls in the pre-pubertal age group of IIH which has shown to usually have a male predominance and often associated with adiposity that exceeds the threshold for obesity; this goes against the current consensus that there is no sex predilection and no association to obesity between this subgroup of early adolescent children [2,3,5,7].…”

“…In paediatrics, IIH shares some but not all features of adult IIH and presents differently in pre-pubertal and post-pubertal children [2]. There are differences in the sex distribution at onset, and absence of significant correlation with obesity as incidences in children seem to peek at between 4-6 years and the other at 13-14 years [3].…”