Dandy-Walker anomaly is a rare congenital disorder. This syndrome is diagnosed by cerebellar vermis hypoplasia, cystic dilatation of the fourth ventricle and, enlargement of the posterior fossa with or without hydrocephalus. This study presents a report of a male fetus with Dandy-Walker syndrome and determination of clinical protests, risk factors, diagnosis, and treatment of this syndrome. The patient was a 38-year-old woman on second pregnancy (parity one), who was referred for fetal ultrasonography. In fetus neurogram scan and examining the skull, the lower segment of cerebellar vermis hypoplasia and suspicious relationship with 4 ventride and posterior fossa were detected which were indicative of Dandy-Walker variant. The mother was hospitalized for therapeutic abortion at 19 weeks and 2 days gestational age. To terminate the pregnancy 80 mg of misoprostol was administered along with IV infusion of oxytocin. This syndrome is a rare disorder which most of its aspects remains unknown and need more investigations. This syndrome is diagnosed by advanced imaging techniques. So, in order to prevent infant birth with this anomaly, prenatal care, particularly for imaging procedures should be done with greater sensitivity.
 

Keywords: Dandy-Walker syndrome, vermis hypoplasia, hydrocephalus

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