Journal
FETAL DIAGNOSIS AND THERAPY
Volume 24, Issue 2, Pages 155-160Publisher
KARGER
DOI: 10.1159/000142146
Keywords
Dandy-Walker syndrome; concomitant anomalies; infant mortality
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Funding
- Flight Attendant Medical Research Institute (FAMRI)
- Centers for Disease Control and Prevention [U50/CCU223184]
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Objectives: To assess infant mortality patterns associated with Dandy-Walker syndrome (DWS) and the impact of concomitant anomalies. Methods: Data for this study were obtained from the New York State Congenital Malformations Registry, an ongoing population-based validated surveillance system. Results: The 196 cases of DWS had a high infant mortality rate (250/1,000), and the elevated risk correlated positively with additional anomalies in a dose-effect pattern (p for trend <0.01). Infants with DWS and two or more affected organ systems were about 6 times as likely to die postneonatally than their counterparts with isolated DWS [adjusted hazards ratio (AHR) = 6.01; 95% CI = 1.52-24.21]. Conclusion: This study confirms the widely held notion that DWS is a heterogeneous rather than a homogeneous entity as shown by the dissimilar infant survival patterns found. Copyright (C) 2008 S. Karger AG, Basel.
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