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PubMed2002

Surgical management of urinary incontinence in children with anatomical bladder-outlet anomalies.

BACKGROUND: Pediatric urinary incontinence secondary to anatomical anomalies of the bladder outlet is relatively rare, and there is no consensus on its surgical treatment. We reviewed the clinical courses and surgical management of children with this pathology. METHODS: From 1991 to 2001, we performed bladder neck reconstruction on seven children (three boys and four girls). The mean age at the first intervention was 7 years (range 5-12 years). The underlying diagnosis was bladder exstrophy in two children, pseudoureterocele-type ectopic ureter in three, bladder neck incompetence from unknown etiology in one boy with hypospadias, and bilateral single ectopic ureter associated with cloacal malformations in one child. Lower urinary tract was evaluated through various imagings, endoscopy, and urodynamic studies. RESULTS: The methods used to reconstruct the bladder neck was the Young-Dees-Leadbetter procedure (performed on four children); the Kropp procedure (two children); and the Pippi-Salle procedure (one child). Bladder augmentation with intestinal segment was performed on four children; Mitrofanoff continent stoma was created in all patients. Urethral catheterization could not be routinely used in all patients, because of pain, stricture, or complicated urethral anatomy. All patients achieved urinary continence. CONCLUSION: The treatment of pediatric urinary incontinence from anatomical anomalies is technically challenging. Depending on the underlying disorders and anatomy, one needs to decide which method of reconstruction to perform. It would be safer to employ simultaneous augmentation and to create catheterizable continent stoma.

Child↗
PubMed1998

Renal transplantation following renal failure due to urological disorders.

BACKGROUND: Renal allograft outcome, during an 8 year period (1985-1992), has been assessed in 56 renal transplants performed in 55 patients who had end-stage renal failure as a consequence of urological abnormalities. The abnormalities were: primary vesicoureteric reflux (VUR) or renal dysplasia (26 patients); posterior urethral valves (PUV) (15); neuropathic bladders (6); vesico-ureteric tuberculosis (5); bladder exstrophy (3); and prune belly syndrome (1). Six patients had augmented bladders, and eight transplants were performed in seven patients with urinary diversions. RESULTS: Overall, 1 and 5 year actuarial graft survival was 89 and 66%, with mean creatinine of 154 micromol/l +/- 11 (SE) and 145 +/- 9 respectively. Patients with abnormal bladders or conduits (n = 28) had worse graft function than those with normal bladders (n = 28) although graft survival was not significantly different in the two groups at 1 and 5 years: 93 and 75% with normal bladders vs 86 and 57% with abnormal systems. Symptomatic urinary tract infections were common in the first 3 months after transplantation (63%); fever and systemic symptoms occurred in 39% with normal bladders and 59% with abnormal bladders. Urinary tract infection directly contributed to graft loss in six patients with abnormal bladders, but had no consequences in those with normal bladders. CONCLUSIONS: Abnormal bladders must be assessed urodynamically before transplantation, and after transplantation adequacy of urinary drainage must be re-assessed frequently. Prophylactic antibiotics are now given for the first 6 months and urinary tract infections must be treated promptly. With these measures, good results, similar to those of patients without urological problems, can be obtained.

Adolescent↗
PubMed1975

Exstrophy of the bladder. I. Long-term results in a series of 37 cases treated by ureterosigmoidostomy.

Of the 37 patients with exstrophy of the bladder in whom ureterosigmoidostomy was selected as the mainstay of treatment 4 died of causes related to the procedure. Faulty judgment and poor followup accounted for these deaths, which might well be preventable today. In 18 survivors, or approximately half of the entire series, the operation has held up well for many years, with no further surgical procedure being required for complications. In 12 patients, or approximately a third of the group, remedial operations for complications attributable to the ureterosigmoidostomy have been required but it was elected to retain this form of diversion. The long-term end result in these patients also remains acceptable. Combining these 2 groups 30 of 37 patients with ureterosigmoidostomy may be considered to have been eventually successful. However, in 7 of 37 patients the method was a frank failure, in that 4 patients died and 3 required substitution of another form of diversion. While all forms of treatment of exstrophy of the bladder leave much to be desired, results obtainable in today's setting lead us to recommend ureterosigmoidostomy as first choice in the management of this disorder. This recommendation is accompanied by the admonition that systematic followup is imperative so that if things do go badly from the clinical, laboratory or urographic viewpoint corrective measures can be done before renal deterioration occurs. The measures required may range from simple correction of electrolytes to conversion to an ileal loop.

Adolescent↗
PubMed1994

Use of bowel for vaginal reconstruction.

There is a wide range of congenital vaginal abnormalities that can be reconstructed using bowel. From 1964 to 1993 we used bowel for vaginal reconstruction in 65 patients and all but 5 were treated since 1981. Patient age ranged from 9 months to 26 years. In 29 patients the bowel was used to extend a short vagina to the perineum, and in 36 the entire vagina was absent and required a substitute vagina. Indications for surgery included cloacal malformations in 34 cases, congenital absence of vagina in 12, cloacal exstrophy in 9 genetic male subjects, postoperative exenteration in 7, intersex in 2 and bladder exstrophy in 1. Rectum was used in 12 cases, sigmoid colon in 27 and small bowel in 26. Ten patients who are now adults have reported satisfactory coitus.

Adolescent↗
PubMed1999

[Cystitis glandularis].

OBJECTIVE: To discuss the presenting features, diagnosis and treatment of cystitis glandularis. METHODS/RESULTS: 15 patients consulted for voiding syndrome, hematuria, hypogastric pain and chronic cystitis. Ten patients were evaluated by IVP which showed bilateral ectasia in 6, an abnormal renal pelvis in one and the remaining three patients were normal. Eight patients were evaluated by cystography which showed a reduced bladder capacity in three, bilateral reflux in three and an abnormal bladder wall in two patients. Urinalysis was performed in all 15 patients; only 5 patients showed a positive culture. These 5 patients had recurrent episodes and underwent cystoscopy and biopsy and/or TUR. Sixty percent was associated with a foreign body, lithiasis, bladder exstrophy, pelvic lipomatosis and BPH. Diagnosis was based on the histological findings; 40% had a bladder tumor. CONCLUSIONS: Patients with cystitis glandularis present with voiding syndrome and chronic cystitis. The IVP, US and cystographic findings are usually normal or may indicate a tumor. Diagnosis is based on the histological findings.

Cystitis↗
PubMed1990

A technique for bladder neck reconstruction in exstrophy: the cinch.

A surgical modification of the Young-Dees-Leadbetter procedure for bladder neck reconstruction is presented. The procedure uses a bladder muscle flap devoid of mucosa as a cinch to encircle and compress the reconstructed neourethra, increasing urethral resistance and helping to achieve continence in patients with bladder exstrophy. The operation combines the continence enhancing features of bladder neck narrowing, urethral elongation, cuff compression, sling suspension of the urethra and urethropexy. Of 10 patients who underwent an operation 6 are dry during the day and night, and they achieved continence relatively soon postoperatively.

Bladder Exstrophy↗
PubMed2001

Bladder preservation in adult classic exstrophy: early results of four patients.

OBJECTIVES: To report our experience with the treatment of classic exstrophy of the bladder in a small series of adult patients using ileocystoplasty, bladder neck reconstruction, and abdominal wall closure with flaps. The presentation of exstrophy of the bladder in adulthood is rare. The problems encountered include difficulty in abdominal closure, malignant potential, and upper tract dysfunction. The treatment of choice has been cystectomy with urinary diversion in all reported cases. METHODS: We treated 4 adult male patients with classic exstrophy of the bladder and complete epispadias. They had not received any previous treatment. Multiple random bladder biopsies revealed nonspecific inflammatory changes with focal areas of keratinization. Three patients were treated in two stages. The first stage included ileocystoplasty, bladder neck reconstruction, and abdominal wall closure with the use of flaps. The epispadias was repaired in the second stage. In 1 patient, the reconstruction was completed in a single stage. RESULTS: All patients were continent at the last follow-up visit, with three using self-catheterization and one voiding spontaneously. The renal parameters and ultrasound scans were normal at a follow-up of 2 to 48 months. Cystoscopy performed at 6 months postoperatively revealed normal-looking mucosa in 2 patients and mild inflammation in 1 patient. Three patients were satisfied with the cosmetic results and one complained of the small size of his penis. All patients were evaluated by psychiatrists and revealed anxiety disorders preoperatively. After surgery, all demonstrated improved social interaction. CONCLUSIONS: Vesical preservation with primary reconstruction of bladder exstrophy in adults is safe and feasible in the absence of significant histologic changes in the bladder mucosa. Abdominal closure can be achieved without difficulty with the use of transposition flaps. However, these patients require strict follow-up to detect malignant transformation at an early stage.

Abdominal Muscles↗
PubMed2005

Renal transplantation in children with severe bladder dysfunction.

PURPOSE: Renal transplantation in children with bladder dysfunction carries a risk for the renal graft. We report our experience with transplantation in 15 patients 6 to 18 years old with severe abnormalities of the lower urinary tract. MATERIALS AND METHODS: A total of 18 renal transplants were performed in 15 children with bladder dysfunction secondary to myelomeningocele (3), occult spina bifida (1), malformation/agenesis of the sacrum (5), posterior urethral valves (4), female hypospadias (1) and bladder exstrophy (1) between 1979 and 2003. Urological surgery was performed before transplantation in 14 cases-7 bladder augmentations, 5 incontinent urinary conduits/reservoirs and 2 vesicostomies. Voiding was maintained by intermittent catheterization in 9 cases and incontinent ostomies in 6. Graft implantation was performed by extraperitoneal route with ureteral anastomosis to the native bladder in cases of bladder augmentation. Immunosuppression consisted of triple therapy with polyclonal/monoclonal antibodies. RESULTS: Urological complications consisted of urethral obstruction due to mucus hypersecretion (1), urinary fistula (1), ureterovesical obstruction (1), stone formation (3), urinary tract incrustation by Corynebacterium urealyticum (1) and pyelonephritis (2). Graft survival rates at 1 and 5 years were 77% and 62%, respectively, with a median of 79 months (95% CI 51 to 107). Three graft losses were related to urological disease. CONCLUSIONS: Renal transplantation in children with severe bladder dysfunction can achieve similar results to those obtained in the general population. Meticulous selection of patients and surgical reparative techniques ensuring voiding and adequate control of urinary infections are mandatory. Augmentation cystoplasty and intermittent catheterization are appropriate techniques currently used for achieving this outcome.

Adolescent↗
PubMed2005

Gender identity outcome in female-raised 46,XY persons with penile agenesis, cloacal exstrophy of the bladder, or penile ablation.

This review addresses the long-term gender outcome of gender assignment of persons with intersexuality and related conditions. The gender assignment to female of 46,XY newborns with severe genital abnormalities despite a presumably normal-male prenatal sex-hormone milieu is highly controversial because of variations in assumptions about the role of biological factors in gender identity formation. This article presents a literature review of gender outcome in three pertinent conditions (penile agenesis, cloacal exstrophy of the bladder, and penile ablation) in infancy or early childhood. The findings clearly indicate an increased risk of later patient-initiated gender re-assignment to male after female assignment in infancy or early childhood, but are nevertheless incompatible with the notion of a full determination of core gender identity by prenatal androgens.

Adolescent↗
PubMed1979

Tumour of the colon as a late complication of ureterosigmoidostomy for exstrophy of the bladder.

Two cases of cancer of the colon have occurred recently in our series of 38 patients with exstrophy of the bladder treated by ureterosigmoidostomy. Twenty-six and 14 years elapsed between the original operation and the diagnosis of malignancy. Since 1929 the literature contains reports of 55 patients who have developed bowel neoplasms as a late complication of this form of diversion. The operation was performed for exstrophy in 35 of these, in whom the resulting tumour at the anastomotic site was malignant in 28 and benign in 7 patients. The shortest interval between ureterosigmoidostomy and recognition of the growth was 10 years with a mean latent period of 25 years. The aetiology remains uncertain but it is clear that long-term survivors after ureterosigmoidostomy should have diagnostic large bowel studies included in the follow-up.

Adult↗
PubMed1990

The state of the testicle and the epididymis associated with exstrophy of the bladder in undescended testes.

In undescended tests, the large number of testicular dysplasias/hypoplasias and of that of the fusion abnormalities of the epididymis, and the joint occurrence of the two, can achieve even 50%. The frequency of the above anomalies were studied in undescended testes associated with exstrophy of the bladder. Bilateral undescended testes were observed in 5 out of 26 boys born with vesical exstrophy. In 3 of them bilateral orchiopexy had already been performed. In 5 of the 6 operations intact testicles and intact epididymis were found. Fusion abnormality was observed only in one case, but the testicle was intact also in that case. This can be attributed to the fact that in exstrophy of the bladder, the testicles have failed to descend not due to the insufficient effect of fetal androgen, but due to anatomical causes. This is also supported by the fact that where the disorder of descension has been caused by mechanical factors, there was a much lower ratio of testicular-epididymal fusion abnormality.

Bladder Exstrophy↗
PubMed2003

The outcome of bladder neck closure in children with severe urinary incontinence.

PURPOSE: We evaluated the outcome of children who underwent bladder neck closure for the management of severe urinary incontinence. MATERIALS AND METHODS: Bladder neck closure was performed in 12 patients with bladder exstrophy, 4 with myelomeningocele and 4 with urogenital sinus anomalies for severe urinary incontinence that persisted after multiple failed bladder outlet procedures. The effectiveness of bladder neck closure and the need for subsequent surgeries were ascertained. RESULTS: Mean followup was 5.4 years (range 1 to 12). At 3 months after surgery 40% of the patients were completely dry, 20% had leakage via the stoma and 40% had a urethral fistula. After additional surgeries 85% of the patients were completely continent 2 years following bladder neck closure (15% declined further surgeries or had an ileal conduit). Of the 15 patients with followup longer than 3 years only 40% remained completely dry, and leakage via the stoma developed in 47% after being dry for 1 year. Repeat urodynamic studies did not show any adverse changes in bladder dynamics in the incontinent patients. Stomal stenosis occurred in 30% of the patients and bladder stones developed in 40%. None of the patients had hydronephrosis or bladder perforation. The early complication of fistula formation and the late development of leakage via the stoma appear to be related in part to compliance with intermittent catheterization. CONCLUSIONS: Bladder neck closure is an effective method of achieving urinary continence in children in whom other bladder outlet surgery has failed. However, its success is dependent in part upon compliance with intermittent catheterization. Finally children who have undergone bladder neck closure are at an increased risk for stomal stenosis and bladder stones.

Adolescent↗
PubMed1990

Continent urinary diversion in childhood.

We review 24 children and young adults who underwent continent urinary diversion. The indications for an operation included bladder exstrophy in 11 patients, myelomeningocele in 8, sacral agenesis in 3, cloacal anomaly in 1, and traumatic disruption of the bladder neck and urethra in 1. The operations performed included an Indiana pouch in 19 patients, including 12 whose stoma was brought to a perineal position and 7 whose stoma was placed in the anterior abdominal wall. A Kock pouch was used in 2 patients and the Mitrofanoff principle was used in 3. The particular indications for the different procedures are discussed at length. Postoperative daytime continence as defined by at least 4 hours of dryness is present in all 24 patients to date, while 4 have nocturnal incontinence. Renal function is stable in all patients to date. In 18 patients postoperative urine cultures were positive during followup. All patients are on clean intermittent catheterization and reoperation has been required in 2 relating to an inability to perform postoperative intermittent catheterization. Two patients underwent reoperation for small bowel obstruction. The series supports the use of continent urinary diversion as a viable alternative to traditional forms of conduit diversion in children and young adults.

Adolescent↗
PubMed1996

Continent gastric pouch.

Between January 1985 and June 1995 a total of 12 patients (9 female, 3 male) underwent total reconstruction of the lower urinary tract using gastric tissue. Their mean age was 10 years (range, 5-25 years). Total gastric bladder substitution was performed in seven patients, whereas five other patients had composite continent reservoirs (stomach plus bowel) created. The diagnoses were cloacal exstrophy, classic bladder exstrophy, bladder rhabdomyosarcoma, bilateral ectopic ureters, and VACTERL association. The mean follow-up period was 4.5 years (range, 16 months to 9 years). The average bladder capacity was 309 ml, and the compliance averaged 12.9 ml/cmH2O. Continence was achieved in all patients, but the continence mechanism often required revision. Renal deterioration was noted only in one patient due to an obstruction at the site of a transureteroureterostomy. The complications included hyponatremic, hypochloremic metabolic alkalosis (two patients); prolapse of the neovagina (one patient); a ureterovesical junction obstruction (three patients); and revision of the neourethra or Mitrofanoff (six patients). Revision of the gastric tube used as the catheterizable channel was performed in three instances. Lengthening of the tube, including nippling in one instance, corrected persistent incontinence. Distal stenosis of a gastric tube required several plastic procedures for correction. A ureteral Mitrofanoff developed a distal stricture necessitating revision. Persistent leaking of an appendiceal Mitrofanoff was corrected with reimplantation. A tapered ileal neourethra required a tunneling procedure followed by Teflon injection to provide urinary continence. Complications seen most often involved the continence mechanism of these gastric neobladders or composite urinary reservoirs. Persistence, patience, and perseverance led to total urinary continence and adequate reservoirs for urine storage in all patients.

Adolescent↗
PubMed2004

The use of isolated sigmoid colon segment for vaginal replacement in young adults.

PURPOSE: Vaginoplasty for congenital atresia, a component of the Mayer-Rokitansky-Kuster syndrome, or for gender confirmation, may be achieved by several techniques. This report focuses on the efficacy of rectosigmoid neocolporrhaphy (RSNC) for primary vaginal replacement. MATERIALS AND METHODS: From 1990 to 2002 we evaluated six patients 16-33 years old (average age 23) who required vaginal replacement. The diagnoses included the Mayer-Rokitansky syndrome in two cases, classic bladder exstrophy in 1 and male pseudohermaphrodites in three cases. The vagina was reconstructed using a 15-cm isolated sigmoid segment placed between the bladder and rectum and anastomosed to the introitus in four patients. The Frank procedure was used in two patients. RESULTS: A minimum of 1 year of followup is available in five of the six patients (mean of 32 month). Patients treated with sigmoid vaginoplasty had functional neovagina. with excellent cosmetic results and without excessive mucous production or the need for routine dilation. Stenosis at the mucocutaneous junction in one patient with a sigmoid vagina was treated with Y-V plasty. In the two patients managed by nonoperative perineal self-dilatation, there was little success noted and the two patients became surgical candidates. CONCLUSIONS: Experience with this group of patients leads us to believe that isolated sigmoid segments provides a cosmetic, self-lubricating neovagina with low rates of failure and revision, and without the need for routine dilation.

Adolescent↗
PubMed1998

Construction of a female urethra using the vaginal wall and a buttock flap: experience with 40 cases.

METHODS: From 1975 to 1996 a urethra was constructed using tubularized anterior vaginal wall covered with a buttock flap in 40 female patients in whom urinary incontinence was associated with a short or absent urethra. The underlying pathology included bilateral single ectopic ureters, cloacal malformation, urogenital sinus malformation, previous failed surgery of the urethra, severe trauma, myelodysplasia, female hypospadias, ectopic ureterocele with destruction of the urethra, congenital epispadias, bladder exstrophy, and previous hysterectomy and vaginectomy for clear cell carcinoma caused by intrauterine exposure to diethylstilbestrol. Other reconstructive procedures in these patients included bladder neck narrowing from above in 33 patients, ureteral reimplantation in 35, and bladder augmentation in 21. In the prone position, a tube of vaginal wall was used to create a urethra when it was absent or lengthen the urethra if it was too short. The neourethra was extended up to the base of the clitoris, using a multilayered closure of soft tissue over it distally with introital muscle and adjacent mucosa. The proximal neourethra was covered with a buttock flap. RESULTS: All patients were originally wet. Thirty-four are now dry. Four patients have minimal stress incontinence. Two patients have urinary diversions. The majority (n=25) void normally. Fourteen patients self-catheterize to empty their bladders. One patient wears an external appliance on the abdominal wall for collection of urine. Complications included: creating stress incontinence in one patient who had been previously dry by extracting a stone from the bladder, necrosis of a buttock flap from applying a tight perineal dressing, fistula requiring surgical closure in three patients, and slippage of the buttock flap in one patient. CONCLUSION: This operation is a useful method to correct diverse congenital and acquired pathology that causes incontinence in girls.

Adult↗
PubMed2005

Diphallus with ectopic bowel segment: a case report.

A 1-year-old boy presented with complete duplication of the penis, with an ectopic bowel segment as an associated anomaly. The boy had duplicated epispadiac urethras. The right one ended blindly whereas the left one was patent, leading into the bladder. There was separation of the symphysis pubis and bifid scrotum. Investigations revealed no other anomalies. A review of the literature concerning diphallus showed a preponderance of associated anomalies such as exstrophy, bladder duplication, ureteral or renal defects, and imperforate anus, but no cases with an ectopic bowel segment. We report this case because of its extreme rarity.

Choristoma↗
PubMed2000

[Artificial sphincter in severe urinary incontinence: 9 years of experience].

OBJECTIVE: To present the results achieved with the artificial urinary sphincter in the treatment of 44 patients with severe urinary incontinence. METHODS: From April 1990 to December 1998, an artificial urinary sphincter was implanted in 44 patients with severe urinary incontinence, aged 21 to 82 years (mean 56). These patients were categorized into the following groups: Group A comprised 24 males, aged 37 to 82 years (mean 66), who had undergone previous prostatic procedures. Four of them had undergone radical retropubic prostatectomy. The remaining 20 had undergone a total of 34 de-obstructive procedures (mean 1.7 procedures/patient; range 1-3); 28 were endoscopic procedures and 6 open prostatectomy. One of them had a previous ileocystoplasty due to severely low compliance. Ten were completely incontinent and were using a condom catheter (7), penile clamp (2) or permanent indwelling Foley catheter (1). The rest used a mean of 3.7 pads per day. Group B comprised 11 males with a complex urologic history: 9 had neurogenic dysfunction, 1 had bladder exstrophy and 1 had a previous cystectomy and Studer neobladder. Eight were using a condom catheter. The cuff was placed in the bulbar urethra in 8 (4-4.5 cm) and in the bladder neck in 4 (9-10 cm). Group C comprised 9 women with type III stress incontinence after failed previous interventions (range 1-4, mean 2.7). All of these patients required at least 3 pads per day (mean 4.5). The cuff was placed in the bladder neck with diameters ranging from 5 to 10 cm. RESULTS: The mean follow-up was 28.5 months (range 8-96). One patient died two years after implantation of the artificial sphincter which was never activated. In group A, 83% are socially continent and 17 do not require pads. Three of the 20 patients with the artificial sphincter have undergone a total of 4 surgical revisions. In group B, the social continence rate was 63% with a 36% revision rate. Four patients required a complete change of the system. In group C, The continence rate with revision was 66%. One patient underwent two successive successful revisions and 3 required removal of the device, accounting for a failure rate of 33%. Definitive removal of the artificial sphincter was required in 3 females and 5 males. Aside from one male who refused a new implant, the rest were considered unsuitable for a new device. Six patients underwent at least one reoperation to change one or more parts of the system. Continence was recovered in 66% after the revision. CONCLUSIONS: The artificial urinary sphincter achieves a good continence rate in patients with severe incontinence, although careful patient follow-up is required.

Adult↗