PEDIATRIC UROLOGY (M CASTELLAN AND R GOSALBEZ, SECTION EDITORS) Modern Management of Bladder Exstrophy Repair Brian M. Inouye & Eric Z. Massanyi & Heather Di Carlo & Bhavik B. Shah & John P. Gearhart # Springer Science+Business Media New York 2013 Abstract The exstrophy–epispadias complex is a rare con- genital malformation of the genitourinary system, abdominal wall musculature, and pelvic bones. Historically, surgical out- comes in patients with classic bladder exstrophy, the most common presentation of the exstrophy–epispadias complex, were poor. However, modern techniques have increased the success of achieving urinary continence, satisfactory cosmesis, and improved quality of life. Still, recent studies recognize complications that may occur during management of these patients. This review provides readers with an over- view of the exstrophy–epispadias complex, the modern man- agement of bladder exstrophy, and potential surgical complications. Keywords Bladder exstrophy repair . Management . Cosmesis . Exstrophy–epispadias complex . Surgical complications Abbreviations EEC Exstrophy–epispadias complex CBE Classic bladder exstrophy OEIS Omphalocele, exstrophy, imperforate anus, spinal abnormalities VUR Vesicoureteral reflux MRI Magnetic resonance imaging CIC Clean intermittent catheterization MSRE Modern staged repair of exstrophy CPRE Complete primary repair of exstrophy BNR Bladder neck reconstruction CUD Continent urinary diversion Introduction The exstrophy–epispadias complex (EEC) is a rare spectrum of multisystem birth defects involving the genitourinary and gastrointestinal tracts, musculoskeletal system, pelvic floor, and bony pelvis. In classic bladder exstrophy (CBE), there is a lower abdominal wall defect exposing an open bladder and urethra, a wide diastasis of the pubic symphysis, and an epispadic urethral opening. Cloacal exstrophy presents sim- ilarly but with a bilobed bladder separated by a portion of cecum between two hemibladders. Being a more severe birth defect, cloacal exstrophy also presents with various other anomalies, such as malformations of the gastrointesti- nal, musculoskeletal, and central nervous systems, also known as the OEIS (omphalocele, exstrophy, imperforate anus, spinal abnormalities) complex. Complete epispadias is the least severe form of EEC and presents with a closed bladder, dorsally open urethral meatus, and mild pubic diastasis. Patients with EEC will undergo multiple reconstructive surgical procedures beginning with closure of the urethra, bladder and anterior abdominal wall, along with approxima- tion of the pubic rami. Oftentimes, children with CBE must undergo pelvic osteotomy and lower extremity immobiliza- tion. While current techniques achieve reasonable success in preservation of renal function, continence, and cosmesis, there are also many recognized complications associated with reconstruction. Overview and Epidemiology of the Exstrophy–epispadias Complex CBE is the most common presentation of EEC, occurring in approximately one per 10,000 to 50,000 births [1] and affecting males approximately twice as often as females [2]. Additionally, Caucasian infants are more likely to pres- ent with the complex compared to non-Caucasian infants [3]. Other risk factors include young maternal age and B. M. Inouye : E. Z. Massanyi : H. Di Carlo : B. B. Shah : J. P. Gearhart (*) James Buchanan Brady Urological Institute, Division of Pediatric Urology, Charlotte Bloomberg Children’ s Hospital, The Johns Hopkins University School of Medicine, 1800 Orleans St. Suite 7304, Baltimore, MD 21287, USA e-mail: jgearha2@jhmi.edu Curr Urol Rep DOI 10.1007/s11934-013-0332-y