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Dandy-Walker Syndrome, Associated Anomalies and Survival through Infancy: A Population-Based Study

2008; Karger Publishers; Volume: 24; Issue: 2 Linguagem: Inglês

10.1159/000142146

1421-9964

Hamisu M. Salihu, Jennifer L. Kornosky, Charlotte M. Druschel,

Parvovirus B19 Infection Studies

<i>Objectives:</i> To assess infant mortality patterns associated with Dandy-Walker syndrome (DWS) and the impact of concomitant anomalies. <i>Methods:</i> Data for this study were obtained from the New York State Congenital Malformations Registry, an ongoing population-based validated surveillance system. <i>Results:</i> 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]. <i>Conclusion:</i> 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.