title: Dandy-Walker syndrome: a review of fifteen cases evaluated by prenatal sonography.
abstract: Fifteen cases of the Dandy-Walker syndrome evaluated by prenatal sonography were reviewed retrospectively. A posterior fossa cyst communicating with the fourth ventricle was a feature in each case. Hydrocephalus was present in 53% of fetuses. Extracranial congenital malformations were documented in 60% of cases. Cardiac, genitourinary, gastrointestinal, and skeletal anomalies were noted. Of 12 available karyotypes, 4 (33%) were abnormal, including two cases of trisomy 18. Excluding terminated pregnancies, there was an overall mortality of 55%. Associated congenital defects contributed to 83% of the postnatal deaths. The Dandy-Walker syndrome can be accurately diagnosed in utero by sonographic demonstration of characteristic morphologic changes in the fetal posterior fossa. The prenatal examination should include an evaluation of associated supratentorial and extracranial defects. Coexisting structural and chromosomal anomalies occur frequently and adversely affect survival.
authors.affiliation: Department of Radiology, University of Colorado Health Sciences Center, Denver 80262.
authors.forename: P D
authors.initials: PD
authors.lastname: Russ
authors.forename: D H
authors.initials: DH
authors.lastname: Pretorius
authors.forename: M J
authors.initials: MJ
authors.lastname: Johnson
mesh_terms: D000015:Abnormalities, Multiple / Q000175:diagnosis; D001921:Brain / Q000473:pathology; D002869:Chromosome Aberrations / Q000175:diagnosis; D025063:Chromosome Disorders; D003616:Dandy-Walker Syndrome / Q000175:diagnosis*; D005260:Female; D005315:Fetal Diseases / Q000175:diagnosis*; D006801:Humans; D006849:Hydrocephalus / Q000175:diagnosis*; D011247:Pregnancy; D011296:Prenatal Diagnosis; D012189:Retrospective Studies; D014463:Ultrasonography