Bladder Exstrophy, Epispadias, and Cloacal Exstrophy

Abstract

This group of anomalies comprises a spectrum of lower midline disorders affecting the pelvic ring, the lower abdominal wall, the bladder, and the genitalia. Predominantly occurring in males, the commonest is classic bladder exstrophy which presents antenatally or at birth with an open bladder plate continuous with a urethral plate which is open on the dorsum of the penis. Various operations or combinations of operations are described to address closing the bladder and reconstructing the penis/genitalia and these have evolved over the past 20 years with spontaneous voiding continence as a primary goal. Cloacal Exstrophy is much rarer and combines bladder exstrophy, exstrophy of the hindgut with a more severe abdominal wall and genital defect. This most challenging group of patients may also have a myriad of other non-urogenital abnormalities which affect their outcomes and necessitate a coordinated multi-disciplinary approach, although most will survive into adult life. The surgical goals are to establish a sound abdominal wall closure and obtain control of bowel and bladder function with a permanent stoma and a catheterizable reservoir, respectively, optimizing function and quality of life. Primary epispadias is perhaps wrongly considered to be a minor variant of the spectrum, whose continence outcome is additionally affected by a degree of dysplasia/hypoplasia of the bladder outlet. Voiding continence may be hard to achieve in these patients, and satisfactory genital outcomes are a challenge, especially in boys. The rare nature of these conditions (with a combined incidence of around 1 in 20,000 births) suggests that they are better treated in specialized centers where focusing the experience and skills of fewer surgeons will provide the starting point for further developments.