A fetopathological and clinical study of the Dandy-Walker malformation and a literature review

Abstract

Aim: The Dandy-Walker malformation is a rare abnormality of the central nervous system pertaining to the group of cystic malformations. The frequency of occurrence of the malformation ranges from 1:800 to 35,000 live births. The aim of this study is to investigate the Dandy-Walker malformation in two cases – one by autopsy after abortion due to medical indications during the 25th gestational week at The Clinic of Embryo and Fetopathology, Center for Maternity and Neonatology, Tunis, Tunisia, and another case of a 1-year-old infant diagnosed with an acute internal hydrocephalus at The Clinic of Neurosurgery, Medical University Plovdiv, Bulgaria, and to compare the results with those in the literature. Results: Both cases are of an isolated Dandy-Walker malformation without any associated abnormalities of the central nervous system and other organs and systems. After the placement of a ventriculoperitoneal shunt in the second case, the child’s condition was greatly improved. Conclusion: The question arises whether the prenatal diagnosis of an isolated Dandy-Walker malformation is an indication for pregnancy termination, and who and after what examinations has the right to make this decision, given the favorable outcome after surgical treatment.