ABSTRACT
BACKGROUND: The Dandy-Walker Syndrome (DWS) is a congenital malformation of the structures of the posterior cranial fossa with failure of the Luschka and Magendie foramina, which connect the ventricular system to the subarachnoid space, to open. In this syndrome, the cerebellar vermis may be absent or hypoplastic, the hemispheres small, the encephalic trunk and cervical spinal bone marrow flattened, complete or partial absence of cerebellar vermis, cystic dilatation of IV ventricle and frequently hydrocephalus. METHODS: We evaluated 56 cases of fetal malformations of the central nervous system from January 1998 to May 2001. Of these 56, 11 regarded the Dandy-Walker Syndrome. Ultrasound examinations were carried out at the 9(th) and 10(th) weeks, at the 21(st)-23(rd) weeks and at the 31(st) to 34(th) weeks of gestation so as to assess foetal anatomy and morphology. RESULTS: The results of the 11 pregnancies with Dandy-Walker Syndrome were as follows: 3 spontaneous miscarriages (at the 15(th), 18(th) and 20(th) weeks of gestation), 5 therapeutic abortions, 3 term pregnancies. Of the 3 newborns we were only able to follow the clinical course of two of them; both newborns underwent an intervention for ventriculo-peritoneal fluid derivation. At the moment both babies, aged respectively 30 and 25 months, are in good physical, intellectual and behavioural condition. CONCLUSIONS: Bearing in mind the notable clinical and socioeconomic importance of this malformation, we consider foetal morphological ultrasound to be a valid instrument for the correct management of pregnancy. Identification of lesions makes use of the comparison of ultrasound data with the results of histopathological examinations.
