Radiology, Vol 193, 765-770, Copyright © 1994 by Radiological Society of North America
Sonographic detection of inferior vermian agenesis in Dandy-Walker malformations: prognostic implications
MC Chang, SA Russell, PW Callen, RA Filly and RB Goldstein
Department of Radiology, University of California, San Francisco 94143- 0628.
PURPOSE: To compare the prognoses for fetuses with sonographically detected
inferior vermian agenesis (IVA) or complete vermian agenesis (CVA).
MATERIALS AND METHODS: The sonograms of 65 fetuses with Dandy- Walker
malformations were retrospectively reviewed. Fetuses were divided into two
groups--those with IVA and those with CVA. RESULTS: Of the 65 fetuses, 37
had IVA; 28, CVA. Thirteen (20%) of the 65-nine (24%) of the 37 with IVA
and four (14%) of the 28 with CVA--lived for longer than 1 year.
Chromosomal abnormalities were found in 23 (45%) of the 51 fetuses tested
for karyotypic abnormalities or clinically thought to have Down syndrome.
Seventeen (53%) of the 32 with IVA and six (32%) of the 19 with CVA that
were tested for karyotypic abnormalities or clinically thought to have Down
syndrome had chromosomal abnormalities (P = .07). Associated morphologic
abnormalities were prenatally detected in 49 (75%) of the 65-in 28 (76%) of
the 37 with IVA and in 21 (75%) of the 28 with CVA. CONCLUSION: The overall
guarded prognosis is similar for fetuses with Dandy-Walker malformations
for whom IVA or CVA has been sonographically diagnosed. Fetuses with IVA
have a higher prevalence of karyotypic abnormalities.
