Journal of Ultrasound in Medicine, Vol 7, Issue 2 65-71, Copyright © 1988 by American Institute of Ultrasound in Medicine

JOURNAL ARTICLE

The Dandy-Walker malformation prenatal sonographic diagnosis and its clinical significance

D. A. Nyberg, D. R. Cyr, L. A. Mack, J. Fitzsimmons, D. Hickok and B. S. Mahony
Department of Radiology, University of Washington Hospital, Seattle.

To determine the usual sonographic findings and clinical significance of the Dandy-Walker malformation (DWM) identified in utero, we reviewed seven proven cases diagnosed by prenatal ultrasound during a 6-year period. All patients demonstrated complete or partial absence of the cerebellar vermis and a posterior fossa cyst. Other central nervous system (CNS) findings included hydrocephalus (five cases) and agenesis of the corpus callosum (one case). Extra-CNS abnormalities were prospectively identified in 3 of 4 fetuses with multiple anomalies. Chromosomal analysis was performed on all four fetuses with multiple extra-CNS anomalies, yielding 2 abnormal karyotypes (trisomy 18 and mosaic trisomy 13). Fetal mortality was directly dependent on the presence of extra-CNS anomalies. Five fetuses ultimately died, including all four with multiple extra-CNS anomalies. The remaining two patients are alive at 1.5 and 6 years of age. We conclude that DWM has a distinctive sonographic appearance that can be identified by prenatal ultrasound and that identification of DWM should initiate a careful search for concurrent anomalies as well as consideration of chromosomal analysis.

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				J. J. Phillips, B. S. Mahony, J. R. Siebert, T. Lalani, C. L. Fligner, and R. P. Kapur Dandy-Walker Malformation Complex: Correlation Between Ultrasonographic Diagnosis and Postmortem Neuropathology. Obstet. Gynecol., March 1, 2006; 107(3): 685 - 693. [Abstract] [Full Text] [PDF]