Abstract
The present case is a a newborn baby, who has some congenital malformations of multiple organs. She was delivered by Cesarean section with turbid amnioic fluid in full term. A huge occipital encephalomeningocystocele was noticed without any cerebrospinal fluid leakage. In addition to this anomaly, as external malformations right thumb defect, hyperflexion of right elbow and bilateral pes cavus were demonstrated. As internal malformations, esophageal atresia with esophago-tracheal fistula (Gross C type), defect of the right ulnar bone and systolic murmur at the cardiac apex existed.
Neuroradiological investigations showed that not only the occipital lobes, parietal lobes, temporal lobes but cerebellum and brainstem herniated into the encephalocele, which was compatible with the indings of Arnold-Chiari malformation type 3.
A case with this rare coupled malformations was reported and relevant literature was also reviewed.
