Clinical manifestations and initial management of infants with bladder exstrophy
- Joseph G Borer, MD, FAAP, FACS
Joseph G Borer, MD, FAAP, FACS
- Associate Professor in Surgery (Urology)
- Harvard Medical School
- Director of Bladder Exstrophy Program
- Boston Children's Hospital
- Director of Neurourology and Urodynamics
- Boston Children's Hospital
Bladder exstrophy is a complex congenital anomaly involving the musculoskeletal system and the urinary, reproductive, and intestinal tracts. It is one of three disorders within the exstrophy-epispadias complex.
The diagnosis, clinical features, and initial management of bladder exstrophy are reviewed here. The surgical management and postoperative care of children with bladder exstrophy are discussed separately. (See "Surgical management and postoperative outcome of children with bladder exstrophy".)
The exstrophy-epispadias complex (EEC) encompasses the following congenital anomalies, including bladder exstrophy.
●Epispadias, the least severe defect of the group, is characterized by failure of the urethra to close normally, and as a result, the inner lining of the urethra lays flat and exposed on the dorsal (top) surface of the penis. Patients with bladder exstrophy will also have epispadias. However, isolated epispadias is less common than classical bladder exstrophy and is diagnosed in 1 in 200,000 to 400,000 live births.
●Bladder exstrophy is classically characterized by an open, inside-out bladder (the inner surface exposed) on the surface of the lower abdominal wall and an open exposed dorsal urethra (picture 1 and figure 1 and picture 2 and picture 3). Bladder exstrophy usually involves several organ systems in the body, including the urinary tract, the reproductive tract, the digestive system, the muscles and the skin of the lower abdominal wall, and the muscles and bones of the pelvis.
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