Dandy walker malformation with unusual findings in fetal autopsies

The Dandy-Walker malformation is a rare group of congenital abnormalities of brain, typically involving the fourth ventricle and the cerebellum. The Dandy-Walker malformation has an estimated prevalence of about 1 in 25,000 to 35000 live births with a slight female preponderance and is responsible for 4-12% of infantile hydrocephalus. Dandy-Walker malformation is frequently associated with other intracranial anomalies such as agenesis of the corpus callosum, holoprosencephaly, occipital encephaloceles and ocular abnormalities. Extra-cranial anomalies include polycystic kidneys, cardiovascular defects, polydactyly and cleft palate. Present study was conducted on 1200 fetuses obtained from department of Obstetrics and Gynecology, Government medical college and hospital, Chandigarh. Autopsy was done in department of Anatomy, GMCH Chandigarh. In our present study the incidence of Dandy walker syndrome was 0.4% (out of 1200 fetuses DWS was noted in 5 fetuses). Associated anomalies were noted in each case. Prenatal diagnosis of DWS is important to detect and avoid further complications in life.