Sonographic detection of inferior vermian agenesis in Dandy-Walker malformations: prognostic implications

To compare the prognoses for fetuses with sonographically detected inferior vermian agenesis (IVA) or complete vermian agenesis (CVA).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.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.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.