Abstract

Classic bladder exstrophy is a developmental defect resulting in pubic diastasis and bladder protrusion through the abdominal wall. Within this spectrum of disease is cloacal exstrophy, defined by the development of omphalocele in addition to diastasis and bladder protrusion. This spectrum of diseases is caused by the failure of mesoderm tissue growth around the cloacal membrane during development, resulting in the described midline defects. This may include undergrowth of the pubic rami and external orientation of the anterior and posterior parts of the pelvis, contributing to a diastasis. Patients can also be born with neural tube defects and peripheral defects such as clubfoot. Long-term effects of bladder exstrophy may include incontinence, renal dysfunction, genital and body appearance dissatisfaction, impaired fertility, hip pain, and impaired gait. Treatment involves surgical closure of the bladder, repair of epispadias, and closure of the anterior abdominal wall with subsequent bladder neck reconstruction. The anterior pelvic and abdominal wall closure often benefits from bilateral pelvic osteotomies, which allow for improved pubic symphysis approximation. The anterior innominate osteotomy with posterior iliac osteotomy is the corrective osteotomy commonly performed in this procedure. It has been shown to improve initial exstrophy closure success and benefit future urogenital repairs.

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