Fetal Diagnosis and Therapy
Dandy-Walker Syndrome, Associated Anomalies and Survival through Infancy: A Population-Based StudySalihu H.M.a, b · Kornosky J.L.a, c · Druschel C.M.daDepartment of Epidemiology and Biostatistics, College of Public Health, bDepartment of Obstetrics and Gynecology, College of Medicine, and cBirth Defects Surveillance Program, Department of Pediatrics, College of Medicine, University of South Florida, Tampa, Fla., and dCongenital Malformations Registry, New York State Department of Health, Troy, N.Y., USA
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Article / Publication Details
Received: November 21, 2007
Accepted: February 19, 2008
Published online: July 17, 2008
Issue release date: August 2008
Number of Print Pages: 6
Number of Figures: 2
Number of Tables: 3
ISSN: 1015-3837 (Print)
eISSN: 1421-9964 (Online)
For additional information: https://www.karger.com/FDT
Abstract
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.
© 2008 S. Karger AG, Basel
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Article / Publication Details
Received: November 21, 2007
Accepted: February 19, 2008
Published online: July 17, 2008
Issue release date: August 2008
Number of Print Pages: 6
Number of Figures: 2
Number of Tables: 3
ISSN: 1015-3837 (Print)
eISSN: 1421-9964 (Online)
For additional information: https://www.karger.com/FDT
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