Pseudotumor cerebri in pediatric patients

Abstract

Introduction: pseudotumor cerebri syndrome in children is a condition characterized by increased intracranial pressure without evidence of brain lesions, ventriculomegaly, malignancy, or infection. It is classified as primary (idiopathic intracranial hypertension, IIH) when no identifiable cause is found, or secondary when associated with factors such as medications (minocycline, corticosteroid withdrawal) or underlying medical conditions.

Objective: to systematize the clinical and epidemiological aspects of pseudotumor cerebri syndrome in children.

Method: a narrative literature search was performed from 2018 to 2025 in databases including SciELO, ScienceDirect, and PubMed Central.

Results: the annual incidence was found to be 0.9 per 100,000, with a higher prevalence in obese adolescent girls. Pathophysiology involves alterations in cerebrospinal fluid dynamics, influenced by obesity, puberty, and hormones. Symptoms vary according to age: headache (57–87%), nausea, visual obscurations, and sixth cranial nerve palsy are common, although up to 29% of young children remain asymptomatic. Diagnosis requires neuroimaging, lumbar puncture (ICP >28 cm H₂O with normal cerebrospinal fluid), and papilledema. Treatment includes acetazolamide (15–25 mg/kg/day), weight loss (6–10%), and surgery in severe cases; serial lumbar punctures have lost relevance.

Conclusions: pseudotumor cerebri syndrome in pediatrics presents diverse clinical manifestations and a complex diagnosis, particularly due to the difficulty of detecting papilledema in children. The morbidity associated with visual loss, headache, and impaired quality of life underscores the need for timely diagnosis and treatment.