SynthesisChild's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery2025
Dandy Walker malformation with occipital encephalocele - personal series and updated literature review.
Synthesis in Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Who cites it
1 citing paper in PubMed.
- Giant parieto-occipital encephaloceles: always a dismal prognosis? A case report and review of the literature.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2026Review
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Authors and funding
4 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
introductionCo-existence of Dandy Walker malformation (DWM) and occipital encephalocele (OE) is extremely unique, and only a limited number of cases have been documented in literature. Their rarity poses distinct diagnostic and therapeutic challenges across the prenatal-postnatal continuum.
objectivesTo describe the correlation of these two diverse congenital disorders, and to perform a systematic review of the existing literature. METHODOLOGY: Retrospective review of prospectively maintained database identified 5/128 DWM + OE patients (3.9%) managed between 2012 and 2024. Several parameters were noted and appropriate analysis was performed. Systematic literature review following PRISMA guidelines identified 79 cases for pooled comparison.
resultsMean age was 5.6 ± 2.41 months; male-to-female ratio 4:1. Four were first-order children, requiring NICU admission with all having delayed milestones. Two had significant microcephaly while one had macrocephaly. Giant sized OE was seen in four cases. On follow-up, two cases (40%) subsequently developed age-appropriate milestones. Systematic review confirmed significant statistical correlation between patients who underwent only repair vs patient who were shunted ± repair; the cases that were shunted fared better (p-value0.03).
conclusionVP/CP shunt is essential in surgical management leading to better prognosis as compared to repair alone. Giant size of OE/OM and associated anomalies may also dictate long-term outcomes. We affirmed that the pathophysiology resulting in hydrocephalus and OE/OM in DWM to be co-dependent. Our experience adds to this limited pool of data and our analysis of the largest studied number of cases in literature to date provides comprehensive insights into the clinical implications of this association. CLINICAL TRIAL NUMBER: Not applicable.
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