Dandy-Walker syndrome, associated anomalies and survival through infancy: a population-based study.

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.