Hypomelia With Mullerian Duct Anomalies

Halal (1986) described association of severe upper limb hypoplasia and mullerian duct anomalies. One or both were present in 2 males and 3 females in 3 generations. The limb anomalies varied from postaxial polydactyly to ectrodactyly to severe upper limb hypoplasia with split hand. In 1 woman, the mother of the proband, uterus didelphys (2 completely separate uterine cavities with 2 cervices) was demonstrated. Her mother also had 2 cervices. Another affected female had a vertical vaginal septum just behind an intact hymen that required hymenectomy. This appears to be distinct from other reported acrogenital syndromes.