summary Bladder Exstrophy is a rare congenital disorder that involves the musculoskeletal and genitourinary systems and may present with several pelvic abnormalities. Diagnosis can be confirmed with pelvic radiographs revealing pubic rami diastasis, shortened pubi rami, and acetabular retroversion. Treatment is surgical with staged multidisciplinary reconstruction. Epidemiology Incidence classic exstrophy 1/40,000 infants diagnosed with this condition cloacal exstrophy 1/200,000 infants with intestinal track involved as well Etiology Pathology abnormal anterior rupture of the cloacal membrane early in the embryonic period mesenchymal ingrowth into the abdominal wall is also inhibited altered migration of sclerotomes that comprise the anterior elements (pubis) Associated conditions family history should be sought out as often associated with other conditions Presentation Symptoms a thorough history and a complete examination are essential urinary system infection(s) Physical exam genitourinary system exposed bladder musculoskeletal acetabuli are ~12 degrees retroverted without pubis to tether the anterior ring, the posterior elements retrovert waddling gait with external foot progression Imaging Radiographs recommended views obtain AP pelvic radiograph findings pubic rami diastasis shortened pubic rami acetabular retroversion Treatment Goal of treatment close abdominal wall achieve urinary continence normal renal function Operative staged multidisciplinary reconstruction indications all cases require surgical treatment multidisciplinary approach management should be multidisciplinary and involve pediatric urologist and general surgeon reconstruction sequence may vary by the preference of urologist components primary closure of bladder (newborn) usually the first stage epispadias repair in males (1-2 y/o) usually 2nd stage bladder neck reconstructions (4 y/o) usually 3rd stage pelvic osteotomies performed in order to decrease tension on the bladder and repaired abdominal wall to decrease dehiscence Technique Staged multidisciplinary reconstruction technique stage I primary closure of bladder (newborn) stage II epispadias repair in males (1-2 y/o) stage III bladder neck reconstructions (4 y/o) pelvic osteotomies in order to decrease tension on the bladder and repaired abdominal wall to decrease dehiscence timing closure of pelvic ring may be performed at any stage of the process fixation pelvic osteotomy fixation depends on the age newborns not required in newborns (skin traction and hips flexed 90 degrees) younger patients external fixation in younger patients age > 8 years augment correction of diastasis with plate fixation in > 8 y/o Complications Recurrent pubic diastasis Common whether or not osteotomy was performed Does not appear to impact activity level Complications of anterior innominate osteotomy wound dehiscence transient femoral nerve palsy