%0 Journal Article
%T Modern Management of the Exstrophy-Epispadias Complex
%A Brian M. Inouye
%A Ali Tourchi
%A Heather N. Di Carlo
%A Ezekiel E. Young
%A John P. Gearhart
%J Surgery Research and Practice
%D 2014
%R 10.1155/2014/587064
%X The exstrophy-epispadias complex is a rare spectrum of malformations affecting the genitourinary system, anterior abdominal wall, and pelvis. Historically, surgical outcomes were poor in patients with classic bladder exstrophy and cloacal exstrophy, the two more severe presentations. However, modern techniques to repair epispadias, classic bladder exstrophy, and cloacal exstrophy have increased the success of achieving urinary continence, satisfactory cosmesis, and quality of life. Unfortunately, these procedures are not without their own complications. This review provides readers with an overview of the management of the exstrophy-epispadias complex and potential surgical complications. 1. Introduction The exstrophy-epispadias complex (EEC) is a rare spectrum of defects affecting the genitourinary and gastrointestinal tracts, musculoskeletal system, pelvic floor musculature, and bony pelvis. The three most common presentations of EEC are epispadias, classic bladder exstrophy (CBE), and cloacal exstrophy (CE) (Figure 1). Complete epispadias is the least severe form of EEC and presents with a dorsally open urethral meatus with mild pubic diastasis and a closed anterior abdominal wall and bladder. CBE, the most common presentation of EEC, presents with a wide pubic diastasis and an abdominal wall defect exposing an open bladder and urethra with an epispadiac opening. CE, the most severe of the three presentations, is similar, but a portion of cecum or hindgut separates the two open hemibladders. CE also presents with malformations of the gastrointestinal, musculoskeletal, and central nervous systems, also known as the OEIS (omphalocele, exstrophy, imperforate anus, and spinal abnormalities) complex. Figure 1: Four presentations of the exstrophy-epispadias complex: (a) complete male epispadias, (b) complete female epispadias, (c) classic bladder exstrophy, and (d) cloacal exstrophy. Most patients with EEC undergo multiple reconstructive surgeries beginning with closure of the bony pelvis, bladder, and anterior abdominal wall, followed later by epispadias repair. Oftentimes, children with CBE and CE must undergo pelvic osteotomy and lower extremity immobilization to ensure complete approximation and sufficient deepening of the pelvis for anatomic placement of the bladder. While current techniques achieve reasonable success in preservation of renal function, continence, and cosmesis, there are also many recognized complications associated with reconstruction. 2. Epidemiology Complete epispadias is a rare congenital malformation. It occurs in one in every
%U http://www.hindawi.com/journals/srp/2014/587064/