Abstract

 

Meckel-Gruber syndrome is characterized by the triad: occipital meningo-encephalocele, cystic displastic kidneys and postaxial polidactyly. Meckel-Gruber syndrome is a lethal and autosomal recessive condition. This syndrome seen rarely but have high risk of reccurence. In this report was presented a newborn with encephalosel and bilateral renal polycyst, absent polydactyly. In this case postnatal recognition requires genetic counseling of parents and obtaining early prenatal diagnosis in next pregnancy.

Key Words: Meckel-Gruber Syndrome; Polydactyly; Encephalocele; Polycystic Kidney.