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Published on: February 2, 2015
Obstructive hydrocephalus due to developmental venous anomalies: a pediatric imaging case series
Livja Mertiri1, Vikramjeet Singh2, Francesca Gentile3,4
1Edward B. Singleton Department of Radiology, Texas Children's Hospital, Baylor College of Medicine, 6701 Fannin Street, Suite 470, Houston, TX, 77030, USA. lxmertir@texaschildrens.org.
Insights
Developmental venous anomalies (DVAs) can rarely cause obstructive hydrocephalus in children. This study highlights imaging findings, clinical presentation, and management of this rare condition, emphasizing its importance in pediatric hydrocephalus diagnosis.
Area of Science:
- Pediatric Neuroradiology
- Neuroimaging
- Vascular Malformations
Background:
- Developmental venous anomalies (DVAs) are uncommon vascular malformations.
- DVAs are a rare etiology of obstructive hydrocephalus in pediatric patients.
- Recognizing DVA-induced hydrocephalus is critical for timely diagnosis and management.
Purpose of the Study:
- To present an extensive case series of DVA-induced obstructive hydrocephalus in children.
- To describe characteristic imaging findings of this rare entity.
- To raise awareness among pediatric neuroradiologists regarding DVA as a differential diagnosis for hydrocephalus.
Main Methods:
- Retrospective review of electronic patient records from two tertiary children's hospitals.
- Inclusion criteria: pediatric patients with confirmed DVAs causing obstructive hydrocephalus.
- Data collected: age at diagnosis, gender, MRI findings, clinical presentation, symptoms, treatment, and follow-up.
Main Results:
- Five pediatric cases of DVA-induced obstructive hydrocephalus were identified.
- Mean age at diagnosis was 2.9 years; two cases diagnosed prenatally.
- All cases showed DVA causing aqueductal stenosis; one had multiple DVAs.
Conclusions:
- DVA-induced aqueductal stenosis, though rare, must be considered in pediatric hydrocephalus differentials, including prenatal screening.
- Brain MRI, particularly post-contrast T1WI and SWI sequences, aids in visualizing the characteristic "caput medusae" of DVAs.
- Imaging is crucial for detecting DVAs and associated complications like hemorrhages.
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.
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