Adams-Oliver syndrome (AOS) is a rare congenital disorder characterized by defects of the limb, scalp and skull. Characteristically, affected individuals have terminal transverse limb defects, such as oligodactyly, syndactyly or brachydactyly; and aplasia cutis congenita. AOS has also been reported to be associated with congenital heart defects and a variety of abnormalities that affect the skin and blood vessels.
Heterozygous mutation in the ARHGAP31 gene is known to cause Adams-Oliver syndrome 1.
To establish the profile of major congenital malformations, Al Talabani et al. (1998) studied 24,233 consecutive births at Corniche hospital, Abu Dhabi, between January 1992 and January 1995. A total of 401 infants, including 289 of Arab origin, were detected with major malformations. In their study, Al Talabani et al. (1998) observed only one case of Adams-Oliver syndrome - in a infant from the United Arab Emirates. Recurrence was reported in the family.