Obstructive hydrocephalus due to developmental venous anomalies: a pediatric imaging case series

Purpose: Developmental venous anomalies are a rare cause of obstructive hydrocephalus in the pediatric population. In this study, we present the most extensive case series of DVA-induced obstructive hydrocephalus in the pediatric population. We thoroughly describe the imaging findings related to this uncommon entity and comprehensively discuss its clinical presentation and management strategies. The goal is to alert pediatric neuroradiologists to consider this rare condition in the differential diagnosis of hydrocephalus, particularly during prenatal screening or in pediatric cases.

Methods: The electronic patient record systems of 2 tertiary care children's hospitals were reviewed to identify pediatric patients with confirmed DVAs leading to obstructive hydrocephalus. Age at diagnosis, gender, MRI findings (including location of the obstruction), clinical presentation, and symptoms were recorded. Data on treatment and follow-up imaging were also collected.

Results: The search yielded 5 cases of pediatric patients with DVA-induced obstructive hydrocephalus. The mean age at diagnosis of the DVA was 2.9 years (range: 0-7 years), and in two cases, ventriculomegaly was diagnosed in utero during prenatal cranial ultrasound screenings. In all patients, the DVA caused stenosis of the aqueduct of Sylvius, and one case presented with multiple DVAs.

Conclusions: Although aqueductal stenosis caused by a DVA is rare, it is crucial to consider it in the differential diagnosis of hydrocephalus during prenatal screening or in the pediatric population. Brain MRI, especially post-contrast T1WI, and SWI sequences are particularly valuable for visualizing the typical "caput medusae" appearance of DVAs and detecting associated complications such as hemorrhages.