Bladder exstrophy is a rare, severe congenital defect that affects the lower urinary tract, genitalia, abdominal wall, and pelvis. The reported incidence ranges from 1:10,000 to 1:50,000 live births, with boys being affected 5 to 6 times more frequently than girls (1). Bladder exstrophy is usually an isolated defect, and in its classic form, associated chromosomal abnormalities or defects of the central nervous system, heart, and digestive tract are extremely uncommon.

Until the middle of the 19th century, bladder exstrophy was treated primarily nonsurgically. Early forms of repair focused on abdominal wall closure using skin flaps for partial reconstruction, leaving a fistula to attach a urinal for dryness. Trendelenburg recognized that pubic reapproximation would not only prevent prolapse of the reconstructed bladder but also be crucial to achieve continence. Although his theoretical assumptions were correct, his surgical efforts were not successful (2). Early failures led to an abandonment of interest in primary closure with a shift toward urinary diversion. In 1942, Hugh Hampton Young performed the first successful primary closure of a female exstrophy patient (3). Although similar reports about continent, primary closures were published in the same era, the numbers were small and most surgeons were not able to reproduce these favorable results. Both Jeffs (4) and Cendron (5) published their descriptions of successful staged anatomic reconstruction in the mid-1970s, and their pioneering work set the standard for the modern staged repair of exstrophy (MSRE). Grady and Mitchell (6) subsequently developed the complete primary repair of exstrophy (CPRE), in which the primary closure is combined with epispadias repair by complete penile disassembly, often resulting in a hypospadias position of the urethral meatus (7).

While older techniques have evolved and alternative approaches have been developed, the primary objectives of modern exstrophy management remain consistent:

The complex embryology and genetics of exstrophy are not well understood. A number of theories have been proposed, which include premature rupture of the cloacal membrane, arrested mesenchymal ingrowth, and failure of cranial yolk sac progression during development (1). In its classic form, bladder exstrophy describes the condition in which an exteriorized bladder template develops in association with a diastasis of the pubic symphysis. This pubic separation results in characteristic genital and pelvic abnormalities. The pubic diastasis is found in association with foreshortened, externally rotated anterior pubic rami and a wide pelvic inlet. The perineum is short and broad and there is anterior displacement of the anus. The appearance of the genitalia varies by gender. In boys, the urethra lies in an epispadiac position, splayed open on the dorsal surface of the corporal bodies. The pubic diastasis results in corporal shortening and severe dorsal chordee. The testes are usually descended, but inguinal hernias are extremely common, especially in boys. In girls, the pubic diastasis results in an absence of a mons pubis, with a bifid clitoris and lateral displacement of the labia. Girls usually present with a bifid clitoris and a shortened vagina, often with a stenotic orifice. In isolated epispadias, which can be viewed as the least severe form of exstrophy, the bladder is closed and covered by a normal abdominal wall, but the pubic separation and genital abnormalities persist. Degrees of epispadias vary in a manner analogous to hypospadias from the very proximal “complete” epispadias in which the urethra is open through the bladder neck, to minor distal variants. A number of other exstrophy variants have been described (8).