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A Journal on Obstetrics and Gynecology

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Minerva Ginecologica 2003 February;55(1):63-8

language: Italian

Reliability of ultrasound examination confirmed at autopsy in foetuses suffering from Dandy-Walker Syndrome

D'Ettore A., Sole F., D'Armiento M., Zeppetella Del Sesto F. S., Caccavale C., De Simone G., Sciorio S., D'Ambrosio M., Tolino A.


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 worm may be absent or hypoplastic, the hemispheres small, the encephalic trunk and cervical spinal bone marrow flattened, complete or partial absence of cerebellar worm, 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 9th and 10th weeks, at the 21st-23rd weeks and at the 31st to 34th 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 15th, 18th and 20th 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 liquor 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.

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