Idiopathic Intracranial Hypertension in Neonates, Infants, and Toddlers

TL;DR

This review examines idiopathic intracranial hypertension in young children, highlighting differences from adult cases and proposing new diagnostic criteria.

Contribution

The study identifies distinct clinical features and treatment responses in neonates, infants, and toddlers with IIH.

Findings

Males were more commonly affected than females in pediatric IIH cases.

Bulging fontanelle was the most common sign, and papilledema was less frequent compared to adults.

Most cases resolved with conservative treatments like lumbar punctures and medication adjustments.

Abstract

Idiopathic intracranial hypertension (IIH) with (IIHWP) and without papilledema (IIHWOP) is characterized by increased cerebrospinal fluid (CSF) pressure and no evident cause, mostly affecting obese women of childbearing age and possibly leading to vision loss. However, in neonates, infants, and toddlers, these conditions remain understudied entities. This review investigates clinical features, risk factors, treatments, and outcomes to inform their care. From 2278 publications found in PubMed, 2974 in Scopus, and 1684 in the Web of Science Core Collection, 104 relevant articles were analyzed. Among 300 cases, 48.3% were male and 26.0% female, with 43.0% meeting the modified Dandy criteria. Typical signs and symptoms, besides papilledema (23.0%) or its absence (49.0%), included bulging fontanelle (67.7%), irritability (34.3%), vomiting (33.0%), and fever (18.3%). The most triggering factors were medications (35.3%) and infections (15.0%). The mean CSF opening pressure was 35.1 cm H2O, ranging from 9.5 to 77 cm H2O. Main treatment options were lumbar punctures (72.7%), discontinuation of triggering medications (26.3%), acetazolamide (18.7%), and corticosteroids (7.7%); 3.0% required shunting. Unlike in adults, males were more commonly affected, and papilledema was less frequent. Most cases resolved with conservative treatment. A nosological distinction between IIHWP and IIHWOP seems unlikely. Considering our findings and age-specific CSF pressure limits, new diagnostic criteria are proposed.

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