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Objectives: To present the surgical, functional, and cosmetic results of anterior pelvic floor reconstruction in female exstrophic patients who underwent single-stage surgical repair. To verify differences in outcome from male exstrophic patients., Methods: Among the 31 exstrophy-epispadias complex (EEC) patients treated in 10 yr, 13 (42%) were females. We studied 10 of them (9 classic exstrophies and 1 prolapsing epispadia), aged 2 d to 6 yr, who received one-stage repair with pelvic floor reconstruction. The reconstructive steps were posterior pelvic osteotomy, en bloc mobilisation of bladder neck-urethra/vagina within the midline pelvic floor, symmetrical reassembly of the muscular complex that constitutes the pelvic diaphragm (using a bipolar stimulator), tubularisation and elongation of the bladder neck and urethra, and genitoplasty. At 2- to 3-yr follow-up, bladder capacity and dry intervals were evaluated by cystogram and urodynamic study, respectively. Surgical complications and cosmetic appearance were also assessed. Results were compared with a group of 18 male EEC patients treated in the same period with similar technique. Fisher exact test and chi-square test were used for statistical analysis., Results: No bladder/urethra dehiscence, exstrophy relapse, or uterine procidentia were observed. Cosmesis was fully satisfying in all. Bladder capacity ranged from 35 to 137 ml (mean: 87). Cyclic voiding with 45- to 90-min dry intervals was achieved in 7 patients (70%), but stress incontinence was present in 5 patients. Volitional micturition control was achieved in 5 of 6 (83.3%) girls aged 4-8 yr. In the male group, we observed two surgical complications (glans disruption and urethrocutaneous fistula) and one poor cosmetic outcome. Mean bladder capacity was 70 ml (range: 25-140). Dry intervals were present in 6 patients (33%). Volitional voiding was achieved in 5 of 12 (40%) male exstrophic patients older than 4 yr, with little stress incontinence. Female and male EEC patients presented significantly different outcomes (p<0.05) regarding both surgical complications and functional bladder behaviour., Conclusions: Pelvic floor reconstruction and its correct relationship with the lower genitourinary tract may facilitate the development of volitional micturition control. Female patients behaved slightly better than males concerning dry intervals and coordinated bladder activity achievement.

Objectives: To assess the role of correct anatomical reconfiguration of the anterior perineal musculature in exstrophy-epispadias (E-E) patients. To stress the use of a bipolar stimulator to detect the perineal muscular complex intraoperatively, and to increase the functional results of reconstruction in E-E patients., Methods: A total of 22 patients with E-E complex were treated in a 7-year period: 17 patients presenting classic bladder extrophy (aged 3 days to 6 years) and 5 incontinent male epispadias (aged 9 months to 16 years). An electric bipolar stimulator was used to identify and reapproximate at the midline the muscular fibers that constitute the periurethral muscular complex, as a part of the anterior perineal membrane. Outcome was evaluated at 24 months from surgery, considering bladder capacity, dry intervals, urinary infections (UTI's), upper tract deterioration and surgical complications (fistula, obstruction, dehiscence). Results were compared with a matched group of 19 E-E patients treated in the previous 5-year period, without the presented technique (control group). Student T-test was used for statistical analysis, considering p

Purpose: We review our experience during the last 16 years, adopting different surgical approaches for epispadias repair, and compare the results of complete penile disassembly technique with perineal muscular complex reassembly since 1995 with previous repairs., Materials and Methods: From 1984 to 1999, 58 epispadias repairs were performed in 53 male patients 3 days to 13 years old, including 18 with primary penopubic epispadias, 35 with bladder exstrophy and 5 treated with secondary genito-urethroplasty after previous repairs of the exstrophic complex. Cases were divided into 2 periods of the surgical procedure. Different techniques succeeded in 41 patients in the first decade (group 1) while the complete penile disassembly with perineal muscular complex reassembly technique was used in 17 patients during the last 5-year period (group 2). Results of both groups were compared, and Fisher's exact test was used for statistical analysis., Results: Of the 41 group 1 cases complications (mostly fistula and/or urethral stenosis) in 21 (51%) required 1 or multiple operations. Continence was never achieved with urethroplasty alone. Cosmetic appearance of the phallus was not satisfactory in 23 cases (29%) and urethral catheterization was difficult in 8 (19%). Of the 17 group 2 cases only 2 complications (11%) occurred (1 fistula and 1 distal urethral stenosis). Dry intervals or voluntary continence was achieved in 6 of the 10 patients with exstrophy and in all but 1 with epispadias without bladder neck surgery. The penis had a satisfactory cosmetic appearance and no dorsal chordee with an easily catheterizable urethra was noted. The complication rate was significantly different in the 2 groups (Fisher's exact test p = 0.0042)., Conclusions: The complete penile disassembly with perineal muscular complex reassembly technique, with deeper positioning of the urethra in the perineal musculature, seems to guarantee a significant step forward in functional epispadias repair.

Purpose: 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., Materials and 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.

Objective: To report our experience of the use of desmopressin to improve nocturnal dryness in patients who have undergone a staged reconstruction of the exstrophy-epispadias complex (EEC), who although continent by day, have nocturnal incontinence because their nocturnal urinary output exceeds their bladder capacity., Patients and Methods: Seven children (aged 8-12 years) who had undergone a staged reconstruction for EEC (six with classical bladder exstrophy, one with incontinent epispadias) were treated with intranasal desmopressin for persistent nocturnal incontinence despite daytime dryness. Previous additional procedures for continence had been self-augmentation in one and periurethral collagen injection in three others. The criteria for inclusion in the study were: normal renal function, no upper tract deterioration, no urinary tract infections, spontaneous voiding during the day with dry intervals between micturitions, a postvoid residual volume of <10% of bladder capacity and night-time incontinence for 7 nights/week. Desmopressin was administered at bedtime at increasing dosages from 10 to 30 microg until effective. Body weight, arterial blood pressure, and serum electrolytes were measured, and all patients assessed using renal ultrasonography, a voiding diary and a nocturnal pad-test., Results: Desmopressin at doses of 10-30 microg was successful in keeping all the patients dry. The nocturnal urinary output was decreased so that it did not exceed bladder capacity. There was only one minor side-effect (nose bleeding)., Conclusions: In selected patients with EEC, desmopressin is effective in improving nocturnal dryness, with no significant side-effects.

We injected endoscopically 0.75 to 2.5 cc of cross-linked bovine collagen at the 3, 9 and 12 o'clock positions of the urethra or bladder neck of 9 children with epispadias and 7 with exstrophy. This procedure was performed in 10 children who were still incontinent after a Young-Dees operation. In 6 girls collagen was injected submucosally before bladder neck surgery to increase outlet resistance and to gain bladder capacity. A repeat injection was given to 3 patients after 1 year. Continence improved in 9 of the 10 cases injected after surgery of the bladder neck. Of the 6 children who underwent collagen treatment before reconstruction of the lower urinary tract capacity increased an average of 47% in 2 years in 5 and the procedure failed in 1. Submucosal injection of collagen is helpful in modulating outflow resistance and increasing bladder volume after primary closure of exstrophic bladders. Patients with small bladders following closure, who would otherwise be candidates for augmentation cystoplasty, may gain adequate bladder size with collagen injection.

Continence is the main goal in the treatment of patients with bladder exstrophy and prognosis is necessarily long-term. Over an 8-year period, 44 patients presented with bladder exstrophy and/or incontinent epispadias. Twenty-five bladder closures (7 secondary), 23 male urethroplasties, 19 bladder neck (BN) reconstructions and 6 bladder augmentations were performed in these patients. In 18 children 2 additional procedures were employed in the last 3 years: female genito-urethroplasty (15) and submucosal periurethral collagen injection (11). Eight children underwent both procedures. Genito-urethroplasty and collagen injection were performed before BN reconstruction in 6 and 5 cases respectively, in order to increase bladder outlet resistance. The submucosal injection was performed at 3, 9 and 12 o'clock in the BN or sphincteric urethra, using 0.75 to 2.5 ml of cross-linked bovine collagen. Continence in the children who underwent the complete staged reconstruction was good in 58%, fair in 32% and poor in 11%. Following female genito-urethroplasty and periurethral collagen injection, bladder capacities increased by 25%. These complementary procedures are effective in increasing outlet resistance and bladder capacity in patients with exstrophy and/or epispadias; they may improve continence and lessen the need for further bladder augmentation.

The results of endoscopic treatment for urinary incontinence (ETUI) depend on the condition of the urethral mucosa and on the length of the urethra. ETUI is quite difficult to perform in female patients, and, in general, in cases of scarred urethral mucosa. A new device (Zuidex, Q-Med, Uppsala, Sweden) has recently been designed for the "non-endoscopic" treatment of urinary stress incontinence in women. Three paediatric cases are described hereunder. Three patients were treated using Zuidex: two patients (two girls aged 8 and 18) on intermittent catheterization for neurogenic bladder, and one 11-year-old girl, with epispadias, who had already undergone bladder neck reconstruction (Young-Deese). Zuidex is a special implacer for dextranomer implants. It consists of four syringes filled with dextranomer, one implacer, and four needles (25 gauges). Once the device is positioned, the four injections are performed. At the end of this procedure, four implants are symmetrically positioned at four points of the urethral wall, increasing the outlet resistance. In the follow-up phase (5-13 months), the increase in the continent period was +56, 50 and 36%; the increase in bladder capacity (leak volume point) was 40, 28 and 27% in the three patients. Although the new device for "non-endoscopic" treatment of urinary incontinence has been designed for women, the size of the implacer virtually allows its use in any paediatric patient who is above the age of six. Our early experience indicates that this new device could play an important role in the treatment of urinary incontinence in paediatric age. [ABSTRACT FROM AUTHOR]