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


CONCLUSIONS
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.
Salihu
Hamisu M
HM
Department of Epidemiology and Biostatistics, College of Public Health, University of South Florida, Tampa, FL, USA. hamisu.salihu@gmail.com
Kornosky
Jennifer L
JL
Druschel
Charlotte M
CM
D000015:Abnormalities, Multiple / Q000401:mortality* / Q000473:pathology; D000328:Adult; D003616:Dandy-Walker Syndrome / Q000401:mortality* / Q000473:pathology; D005260:Female; D006801:Humans; D007223:Infant; D007226:Infant Mortality; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D008297:Male; D009518:New York / Q000453:epidemiology; D011159:Population Surveillance; D016016:Proportional Hazards Models; D012042:Registries; D018570:Risk Assessment; D013997:Time Factors