Body stalk anomaly: congenital absence of the umbilical cord.

On ultrasound examination, a 20-year-old pregnant woman was found to have a fetus with a large ventral abdominal wall defect diagnosed as a fetal omphalocele. The neonate was delivered by cesarean; the abdominal viscera including liver, stomach, spleen, pancreas, intestine, and uterus were contained in an extraembryonic sac directly attached to the placenta without an umbilical cord. Body stalk anomaly (also known as absence of the umbilical cord syndrome) is a fatal condition resulting from maldevelopment of embryonic body folding and is associated with multiple congenital defects. Prenatal ultrasonographic recognition of the absence of an umbilical cord and direct apposition of the membranous sac to the amniochorionic membrane would permit early termination of pregnancy or avoidance of operative intervention.