Periurethral muscle complex reassembly for exstrophy-epispadias repair.
OBJECTIVE
Continence is a difficult goal in exstrophy-epispadias complex repair. It is presumed that all anatomical components involved in the exstrophy-epispadias abnormality are present but laterally and anteriorly displaced. The penile disassembly technique for epispadias restores the normal anatomical relationship of the male genital components. Its extension to complete primary bladder exstrophy closure enables deeper positioning of the bladder neck within the pelvic diaphragm. We identified the perineal striated muscular complex and present its appropriate periurethral reassembly as a main step in exstrophy-epispadias complex repair.


METHODS
Bladder exstrophy and epispadias repairs were performed in 10 male and 3 female consecutive patients with the exstrophy-epispadias complex, including 1-stage reconstruction in 2 male newborns and 2 females with exstrophy, and as further surgery in a female with cloacal exstrophy and previous failed 1-stage repair, 4 males with incontinent epispadias (secondary repair in 1) and 4 males with epispadias in whom exstrophy closure had been previously done. In the males after bladder plate closure and corporeal body splitting a sagittal incision was made in the intersymphyseal tissue and extended posteriorly to the perineal body midline. The bipolar electrical stimulator was used to identify pelvic muscle components in the sagittal plane and reapproximate them along the tubularized posterior urethra to form the periurethral muscle complex. In the 3 females the urethral plate and vagina were similarly mobilized posterior through the sagittal incision of the perineal body. No patient underwent bladder neck plasty.


RESULTS
At 9 months to 4 years of followup cosmesis was good in 12 patients, while 1 required secondary glanular urethroplasty. There was mild pyelectasis in 3 cases but no severe hydronephrosis and no renal function deterioration. Pyelonephritis developed in 6 patients (46%). Cystography at 1 year showed that bladder capacity was 35 to 80 and 65 to 120 cc in exstrophy and epispadias cases, respectively. There was cyclic voiding with 30 to 90-minute dry intervals in 7 patients (54%), of whom 5 had exstrophy and 2 had epispadias. Daytime voiding control with a 2 to 3-hour voiding interval was achieved in 1 female with exstrophy and 2 patients with epispadias (23%). Incontinence was present in 2 patients with previous exstrophy closure and 1 with cloacal exstrophy (23%).


CONCLUSIONS
Early restoration of a physiological vesicourethral balance of coordinated activity is feasible for the progressive achievement of continence in patients with the exstrophy-epispadias complex. Sagittal splitting of the perineal tissue with identification of the muscle components as well as midline reassembly of the periurethral striated muscular complex helps to reconfigure the pelvic anatomy in a more normal fashion and allows better restoration of coordinated vesicourethral activity.
Caione
P
P
Division of Pediatric Urology, Department of Surgery, Bambino Gesù Children's Hospital Research Institute, Rome, Italy.
Capozza
N
N
Lais
A
A
Matarazzo
E
E
D001746:Bladder Exstrophy / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D004842:Epispadias / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D018482:Muscle, Skeletal / Q000601:surgery*; D010502:Perineum / Q000601:surgery; D011183:Postoperative Complications; D014521:Urethra / Q000601:surgery*; D001743:Urinary Bladder / Q000601:surgery