urinary bladder exstrophy
DEF: a herniation of the urinary bladder through an anterior abdominal wall defect; refers to congenital absence of a portion of the lower anterior abdominal wall and the anterior urinary bladder wall, with eversion of the posterior bladder wall through the defect, an open pubic arch, and widely separated ischia connected by a fibrous band.
SYN: ectopia vesicae
SYN: everted urinary bladder
SYN: exposed urinary bladder
See also J:119657
Bladder exstrophy (BE) is a serious congenital anomaly affecting one in 36,000 live births (Martinez-Frias et al., 2001). In cases of BE, the ventral abdominal and bladder walls are either absent, leaving the bladder cavity exposed, or covered only by an amniotic sac; the pubic bones, external genitalia and rectus abdominis muscles are separated along the midline, whereas the anus is displaced ventrally, often with an associated narrowing or atresia.
In humans, BE complex is evidenced by a cluster of ventral midline defects, including: (1) ventral abdominal- and ventral bladder-wall defects; (2) bifid external genitalia; and (3) separation of the pubic bones.