A Case of Meckel-Gruber Syndrome (Dysencephalia Splanchnocystica)

Abstract

An autopsy case of Meckel's syndrome in a fetus is described. The brain anomaly was detected on ultrasonography at 15 weeks of gestation and the pregnancy was terminated at the gestation age of 21 weeks. No hereditary pattern was elicited in this case. Multiple congenital anomalies consisted of posterior encephalocele with dysplastic brain, cleft palate and lip without philtrum, postaxial polydactyly and syndactyly, polycystic kidneys of Potter type III, fibrosis and bile duct proliferation of the liver, and interstitial fibrosis of the pancreas and the spleen.