Anatomical correction of the exstrophy-epispadias complex: analysis of 34 patients

Objective To evaluate the surgical procedures required for anatomical recon struction of the bladder and penis in the exstrophy-epispadias complex. Patients and methods All primary exstrophy-epispadias repairs carried out b y one surgeon between 1987 and 1997 were reviewed, Bladder closure consiste d of full extraperitoneal mobilization, transpositional omphaloplasty, drai nage with ureteric and urethral catheters and immobilization with a 'frog-l eg' plaster-cast or 'mermaid' dressings, Osteotomies were always per formed when bladder closure was attempted after 37 h of age. Before 1990 the oste otomies were posterior vertical iliac (one patient) and subsequently anteri or oblique iliac (10 patients). Pre-peritoneal herniotomies, in the absence of a clinical hernia, were included in the primary procedure after 1992. A modified Cantwell technique was used for epispadias repair and this was un dertaken at a median of 16 months after bladder closure (range 6-30). Results Thirty-four patients (27 male) were reviewed; one patient had a chr omosomal abnormality, a deletion in the short arm of chromosome 4, The male infants required a median of four procedures (range 2-5) for bladder closu re, epispadias reconstruction and herniotomies, while the females needed a median of two (range 2-5). Complete bladder dehiscence, requiring re-closur e with osteotomies, occurred in three cases (9%, two male), There were no d ehiscences in the primary osteotomy group, Fistulae after epispadias repair occurred in four patients (17%), The bladder capacity increased to > 60 mL in 10 of 15 males by 36 months after epispadias repair. Only two of seven female infants attained a capacity of > 60 mt. Of the 15 infants who did no t undergo herniotomy at primary closure, 13 subsequently developed inguinal hernias (one uni- and 11 bilateral) with incarceration occurring in two. T welve infants underwent herniotomy at primary closure and six developed sub sequent hernias (two uni- and four bilateral; P=0.05) with documented incar ceration in two, Conclusions Anatomical correction of the exstrophy-epispadias complex remai ns challenging, but can be achieved with complication rates of <20% for eac h stage. Bladder volumes large enough to permit adequate bladder neck recon struction can be anticipated after epispadias repair in a large proportion of male infants, but remains small in female infants with low outlet resist ance. Inguinal herniotomy at the time of bladder closure significantly redu ces the incidence of subsequent herniation, which nevertheless remains high .