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Idiopathic Intracranial Hypertension in Neonates, Infants, and Toddlers
Efstathios Beys-Kamnarokos1,2, Ioannis Mavridis1
1Pediatric Neurosurgery Unit, Department of Neurosurgery, School of Medicine, Democritus University of Thrace, University General Hospital of Alexandroupolis, Dragana Campus, 68100 Alexandroupolis, Greece.
Insights
Idiopathic intracranial hypertension in young children is understudied. This review found males are more affected, papilledema is less common, and most cases resolve with conservative treatment, suggesting new diagnostic criteria are needed.
Area of Science:
- Pediatric Neurology
- Ophthalmology
- Nephrology
Background:
- Idiopathic intracranial hypertension (IIH) is a condition of elevated cerebrospinal fluid (CSF) pressure without a clear cause, typically affecting obese women.
- IIH in neonates, infants, and toddlers remains poorly understood, necessitating further investigation into its unique characteristics.
Purpose of the Study:
- To investigate the clinical features, risk factors, treatments, and outcomes of idiopathic intracranial hypertension in young children.
- To inform clinical care and propose new diagnostic criteria for IIH in this understudied population.
Main Methods:
- A comprehensive literature review was conducted, analyzing 104 relevant articles from PubMed, Scopus, and Web of Science Core Collection.
- Data from 300 pediatric cases were extracted and analyzed, focusing on demographics, clinical signs, triggering factors, CSF pressure, and treatment interventions.
Main Results:
- Males constituted a higher proportion of affected children (48.3%) compared to females (26.0%).
- Common symptoms included bulging fontanelle (67.7%), irritability (34.3%), and vomiting (33.0%); papilledema was present in only 23.0% of cases.
- Medications (35.3%) and infections (15.0%) were the most frequent triggering factors, with a mean CSF opening pressure of 35.1 cm H2O.
Conclusions:
- Idiopathic intracranial hypertension in young children presents differently than in adults, with a male predominance and less frequent papilledema.
- Conservative treatments like lumbar punctures and medication discontinuation were effective in most cases, suggesting a potential overlap between IIH with and without papilledema.
- New age-specific diagnostic criteria are proposed based on the findings to improve the diagnosis and management of IIH in pediatric populations.
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.
