EXSTROPHY OF THE BLADDER.
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Bladder Exstrophy↗
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URETERAL PHYSIOLOGY AND EXSTROPHY OF THE BLADDER.
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Bladder Exstrophy↗
Advantages of converting incontinent to continent urinary diversion.
A modified Mainz pouch with catheterizable stoma was constructed in six patients who had originally undergone incontinent urinary diversion by ureteroileostomy 7 to 22 years previously for bladder exstrophy or neurogenic bladder with total urinary incontinence. The surgical technique differed from the standard as follows: after stomal excision, the preexisting ileal loop was detubularized and combined with additional ileal and colonic segments for pouch construction. In patients in whom the original ureteroileal anastomoses were patent and the contrast medium refluxed freely to the upper urinary tract during loopography, the ureters were not reimplanted but kept intact. In all patients the ileal valve was connected as stroma to the umbilicus. In addition, two patients underwent construction of a standard Mainz pouch. One had had primary ureterosigmoidostomy and the other one ureterostomies, 10 and 3 months previously, respectively. The urodynamic characteristics of the reservoir were normal in all. In six ureteropelvic units dilation improved significantly and in two patients the bilateral loop-ureter reflux diminished. Long-term follow-up (up to 45 months) showed no further impairment of the kidneys.
Adolescent↗
Pediatric urinary tract reconstruction using intestine.
OBJECTIVE: To analyse the outcome of urinary tract reconstruction in children. MATERIAL AND METHODS: Fifteen children with bladder exstrophy or neurogenic bladder, 4-18 years old, were followed in accordance with a predetermined program for bladder augmentation (13 pat) or continent urinary reservoir (2 pat). The follow-up time was 1.7-6.3 years, median 3.7 years. RESULTS: All were dry, though one case had occasional leaks. Three bladder neck reconstructions, two artificial sphincters, one sling plasty and one fistula closure with subsequent bladder neck injection were required. Bladder volumes were adequate for age at low pressures. Reflux resolved in 12/13 ureters. A boy with preoperative renal insufficiency was transplanted. Total renal function remained otherwise stable despite acidosis in one case and some glomerular impairment in all. Progressive parenchymal lesions were seen in combination with abundant mucus, infections and calculi only. Growth and bowel function was unaffected. Bone mineral density showed overall increase; some low values were not consistent between investigations. CONCLUSIONS: Urinary tract reconstruction in children results in continence and regression of reflux. Growth, bone mineralization and renal function are unimpaired during the first years, but irrigation of the bladder is essential to minimize the risk of urinary tract infection. However, glomerular function might be affected and the possible risk of metabolic complications in later life can only be determined by continuous close monitoring over an extended period of time. ABBREVIATIONS: Voiding cystourethrogram (VCUG), dimercapto-succinic acid (DMSA), Chrome51-Ethylenediaminetetraacetic acid (Cr-EDTA), single photon absorption (SPA), bone mineral content (BMC), bone mineral density (BMD), dual photon x-ray absorption (DEXA), glomerular filtration rate (GFR), urinary tract infection (UTI), immunoglobulin G (IgG), clean intermittent catheterization (CIC) and subureteral teflon injection (STING).
Adolescent↗
[A new technic of continent urinary diversion: preliminary results].
This paper presents the results of a series of 10 continent urinary valves performed in the urology department of Ibnou-Rochd hospital in Casablanca according to an original technique between 1991 and 1993. These 10 patients (6 men and 4 women) suffered from various diseases of the lower urinary tract: 4 bladder tumrous, 3 complex vesicovaginal fistulae, two of which were associated with a rectovaginal fistula, one bladder exstrophy, one neurogenic bladder and one extensive urethral stricture. The authors describe the surgical technique and the postoperative course. A detubularized ileal graft was used to create a monoblock valve-reservoir. An effective antireflux system protects the upper urinary tract and continence is ensured by a robust hydraulic valve whose flexibility and patency allow very easy self-catheterization. The physical and urodynamic characteristics of this new diversion make it a solid, perfectly continent solution providing undeniable comfort for the patient. Although based on a limited series, these results illustrate the advantages of this new continent detubularized ileal graft diversion.
Adolescent↗
[Bladder exstrophy-epispadias complex: management and preliminary results].
PURPOSE: Retrospective evaluation of operative techniques and perioperative management as related to the primary anatomical and functional situation as well as analysis of the preliminary postoperative results regarding kidney function, continence and complications. MATERIALS AND METHODS: From October 1992 to April 2003, 21 patients with bladder exstrophy-epispadias complex were treated, consisting of 12 children with untreated exstrophy and 9 who had undergone unsuccessful previous operations. Single-stage complete repair using the Erlangen concept was done during the first weeks of life in 8 infants and before school age in 3 children. One 13 year old girl had received a primary Mainz pouch II. The 9 unsuccessfully operated patients required a variety of different operations. The mean follow-up of 17 regularly controlled children was 62 months (range 3 to 129 months). RESULTS: In all children global kidney function remained stable, while reflux or obstruction was responsible for impaired unilateral split function ranging between 31 % and 45 % in 5. Four children were continent day and night after complete single-stage repair, 4 after ureterosigmoidostomy or Mainz pouch II and 5 after augmentation. CONCLUSIONS: In the newborn with previously untreated exstrophy-epispadias, we recommend single-stage complete functional reconstruction within the first weeks of life. Continence without loss of kidney function can be achieved in 50 % of these children. If adequate bladder capacity and continence cannot be achieved, an augmentation procedure or Mainz pouch II is our method of choice.
Adolescent↗
MANAGEMENT OF EXSTROPHY OF THE BLADDER.
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Bladder Exstrophy↗
Enterovesical cystoplasty for bladder closure in cloacal exstrophy.
Successful bladder closure was accomplished in 4 infants with cloacal exstrophy by enterocystoplasty. Two patients were operated upon as newborns, and 2 were 3 and 8 months old, respectively. Three patients are alive and all are incontinent awaiting secondary bladder neck reconstruction. The surgical technique and results are described.
Bladder Exstrophy↗
Female genito-urethroplasty and submucosal periurethral collagen injection as adjunctive procedures for continence in the exstrophy-epispadias complex. Preliminary results.
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.
Bladder Exstrophy↗
Complications in orthopedic management of exstrophy.
The objective of this study was to examine the complications associated with the orthopedic management of bladder exstrophy. The records of all patients seen for bladder exstrophy repair at the authors' institution between 1988 and 2002 were examined. Orthopedic complications were found to have occurred in 26 of 624 patients, yielding a complication rate of 4%. The observed complications were divided into five categories: bony complications at the osteotomy site, neurologic complications at the osteotomy site, complications of traction, deep infection, and late infections of the plate. Five of the 26 (19.2%) complications were bony and involved non-union, delayed union, joint pain, or leg length inequality stemming from the osteotomy. Thirteen of the 26 (50%) were neurologic complications. Four of the 26 (15.4%) resulted from traction. All were due to tight dressing around the legs following surgery. Two of the 26 (7.7%) were early deep infection and of the 26 (7.7%) were late infections. This study highlights many of the complications associated with the management of exstrophy and provides suggestions on how to minimize them.
Bladder Exstrophy↗
A single-centre long-term outcome analysis of artificial urinary sphincter placement in children.
OBJECTIVE: To retrospectively evaluate the outcome of artificial urinary sphincter (AUS) placement in 79 children, with a mean follow-up of 12.5 years. PATIENTS AND METHODS: A total of 89 children had an AUS placed between 1977 and 1994; complete data were obtained for 79 (63 boys and 16 girls). The indication for AUS placement was urinary incontinence caused by sphincteric deficiency with a stable bladder. Before and after surgery, all patients underwent ultrasonography, voiding cysto-urethrography and urodynamics. Logistic regression was used to assess the factors influencing the outcome. RESULTS: The mean (range) age at surgery was 11.7 (3-18) years. The cause of incontinence was a neuropathic bladder in 74 patients and bladder exstrophy in five. At a mean (range) follow-up of 12.5 (5-22) years, 63 of 79 patients (80%) had an intact AUS. The AUS was removed in 16 patients (20%) because of erosion at a mean (range) of 5.6 (1-11) years after insertion; there were 0.035 revisions per patient-year. Of 63 patients with an AUS in place, 57 (90%) are completely dry and 36 (57%) are using clean intermittent catheterization. Thirteen patients (20%) developed bladder instability during the follow-up, which was managed by anticholinergics in eight, spinal cord de-tethering in three and enterocystoplasty in two. Hydronephrosis occurred in 12 of 126 renal units (10%) that improved after enterocystoplasty or anticholinergics. The overall 10-year survival of the AUS was 79% (78% for early models and 80% for the AMS800); the survival was not affected by age, sex, model, previous bladder neck surgery, augmentation cystoplasty or intermittent catheterization. Bladder exstrophy was associated with lower AUS survival, as four of the five patients affected had the AUS removed secondary to erosion (P = 0.014). CONCLUSION: In children the AUS has a 79% 10-year survival and gives a 90% continence rate. Bladder instability after AUS placement can be managed with anticholinergics or enterocystoplasty. The AUS is not a justifiable treatment option for patients with bladder exstrophy.
Adolescent↗
Total urogenital sinus mobilization: expanded applications.
OBJECTIVE: To report further applications of total urogenital sinus mobilization, earlier described as an easier method to correct a cloaca. PATIENTS AND METHODS: Seven children (six girls and one boy, mean age 4 years, range 3 months to 10.5 years) underwent surgery and were followed for a mean of 1 year; their diagnoses included persistent cloaca and congenital adrenal hyperplasia (CAH) in two each, and a urogenital sinus (UGS), bladder exstrophy and penile agenesis in one each. The UGS is approached through a posterior sagittal incision and dissected circumferentially to the retropubic space, allowing the UGS to descend. It is then excised and separate openings of the vagina and urethra created. This technique is applicable to a UGS of </=3 cm. RESULTS: In all patients, separate openings for the urethra and vagina were created. In three patients urinary continence was preserved after surgery. The patient with bladder exstrophy remains incontinent. The remaining patients are too young to assess (not yet toilet-trained). CONCLUSION: This technique simplifies the surgical correction of UGS malformation; we confirm its usefulness in cases of persistent cloaca. It is also valuable in patients with CAH, primary UGS and in selected patients with bladder exstrophy and penile agenesis. When the UGS is not associated with a cloaca, the procedure can be performed perineally. Despite circumferential mobilization of the UGS, urinary continence is preserved.
Adrenal Hyperplasia, Congenital↗
Lower urinary tract reconstruction using stomach and the artificial sphincter.
We implanted the artificial urinary sphincter at gastrocystoplasty in 13 male and 5 female patients. The diagnoses were classical bladder exstrophy (8 patients), myelodysplasia (8), cloacal exstrophy (1) and bilateral ectopic ureters (1). Mean patient age was 14.3 years (range 7 to 32.5). Six patients (5 with bladder exstrophy and 1 with clocal exstrophy) had failed bladder neck continence procedures, 3 myelodysplastic patients had failed artificial urinary sphincter placement, and 1 exstrophy patient had failed a Young-Dees bladder neck repair and artificial urinary sphincter placement. The sphincter cuff was placed around the reconstructed bladder neck in these patients, while in the remainder the artificial urinary sphincter was placed around the intact bladder neck. Mean followup was 20.3 months (range 1 to 5). Of the patients 16 (88%) are continent day and night, while 2 are wet. A total of 11 patients (61%) use Valsalva's maneuver alone for voiding and the remainder use Valsalva's maneuver and/or clean intermittent catheterization for bladder evacuation. Complications related to the artificial urinary sphincter were recurrent pump erosion requiring conversion to a Mitrofanoff continent stoma in 1 patient, and mechanical dysfunction requiring pump cuff and reservoir replacement in 3. There were no complications due to infection. Our report demonstrates that the combination of augmentation gastrocystoplasty and an artificial urinary sphincter leads to urinary continence and can allow for spontaneous urination. The rate of infectious complications is not increased when the 2 procedures are combined simultaneously.
Adolescent↗
Newborn exstrophy closure and epispadias repair.
Over the last 150 years, bladder exstrophy has undergone a transition from a primarily nonsurgically treated disease to a disease treated by urinary diversion or staged repair and now, possibly, primary newborn reconstruction. Our enthusiasm for primary reconstruction arises because of its potential to simplify the management of this disorder and optimize the return of normal bladder function for these patients. As with most new concepts, the evolution of our primary reconstruction techniques could not have been achieved without the prior efforts of others. Other surgeons such as H.H. Young and J. Ansell have shown us the possibility of achieving urinary continence with primary newborn exstrophy closure without sacrificing renal function, but the results have been inconsistent in the past. Staged reconstruction for bladder exstrophy demonstrates the possibility to achieve consistent successful rates of continence in these patients. However, multiple surgical procedures are required to attain this success. The preliminary results of our series of primary bladder exstrophy closures has encouraged us to perform it for all neonates referred to our institution with bladder exstrophy as well as to use it as part of staged reconstructive efforts for patients who have undergone primary surgical procedures for exstrophy elsewhere. We are hopeful and optimistic that newborn primary exstrophy closure performed as described herein will produce consistent rates of urinary continence and allow normal voiding function as well.
Bladder Exstrophy↗
Stomach versus sigmoid colon in children undergoing major reconstruction of the lower urinary tract.
A review of 50 patients who underwent intestinocystoplasty (ICP) or gastrocystoplasty (GCP) replacement at our department during an 8-year period is presented. The most common diagnoses were neurogenic bladder and bladder exstrophy. A total of 48 patients underwent augmentation cystoplasty and 2 had total bladder replacement. Mean follow-up time was 42 months. The clinical and metabolic aspects of the two types of ICP are reported. Hyperchloremic acidosis requiring therapy was not encountered, although mild degrees were seen after sigmoid augmentation in 36% of patients. A dysuria-hematuria syndrome (DHS) was seen in 50% of the patients who underwent GCP. Operative mortality rate was nil. Significant surgical complications occurred in 36% of the patients. The overall success rate for ICP and GCP in this series was 79.15%. ICP gives effective results when used to increase the compliance of the lower urinary tract, but problems related to electrolyte absorption, stones, and mucus production are often encountered. In GCP electrolyte absorption is practically eliminated, so that this technique can be used in patients with renal damage. In addition, patients with a normal bladder plate (bladder exstrophy) can achieve normal voiding with time. The authors believe that patients must be made aware of the possibility of DHS and that this syndrome needs further investigation.
Adolescent↗
Complete repair of exstrophy: further experience with neonates and children after failed initial closure.
PURPOSE: The surgical repair of bladder exstrophy remains challenging for the urologist. Recently, complete primary repair has been used in neonates. We present our experience with this approach in neonates and children after failed initial closure. MATERIALS AND METHODS: Between November 1998 and November 2000, 17 boys and 2 girls with bladder exstrophy underwent complete repair. Complete primary repair was performed in the first 72 hours of life in 4 boys. Complete repair with osteotomy was at a mean age +/- SD of 23 +/- 21 months (range 1 to 74) in 15 patients including 7 with failed initial closure. The bladder and urethra were closed in continuity with epispadias repair by total penile disassembly. All patients were kept in spica cast for 3 weeks. Ureteral stents and suprapubic tube were removed 10 and 14 days after surgery, respectively. Ultrasound was performed before surgery and 3 months thereafter, and voiding cystourethrography was obtained 3 months postoperatively and then annually in all cases. RESULTS: Complete closure resulted in hypospadias in 10 boys (59%). There was no major complication. Mean followup +/- SD was 17 +/- 8 months (range 5 to 33). Temporary suprapubic urinary leakage was noted initially in 10 cases (52%) but no patient had persistent fistula. Initial postoperative renal ultrasound revealed hydronephrosis in 11 renal units (29%). However, at last followup only 1 renal unit (2%) showed pelvicaliceal dilatation. Two patients (10%) had a febrile urinary tract infection and were treated conservatively. Reflux was noted in 24 renal units (63%) but did not require surgery. The 4 boys in whom the closure was performed at birth had regular voiding with 60 to 90-minute dry intervals and mean bladder capacity +/- SD was 85 +/- 35 cc. The 15 older children had a mean bladder capacity of 74 +/- 37 cc and 5 (33%) had regular voiding with 30 to 90-minute dry intervals. CONCLUSIONS: Complete repair of exstrophy is feasible in neonates and older children including, those with failed initial closure with minimal morbidity. There is a short-term evidence of favorable outcome in newborns. Longitudinal followup is required to determine the future need of bladder neck reconstruction and augmentation in older patients.
Bladder Exstrophy↗
The long-term outcome in men with exstrophy/epispadias: sexual function and social integration.
PURPOSE: We evaluated the long-term outcome of social integration and sexual function in 29 men with bladder exstrophy and epispadias. MATERIALS AND METHODS: Four men with epispadias and 25 with bladder exstrophy were available for long-term followup. Patients were interviewed by telephone regarding sexual history and social integration. Semen analyses were obtained in 8 cases. RESULTS: Patient assessment of genital appearance was good or fair in 71%. Potency was present in all patients, and erections were straight in 66% and curved in 34% with no curvature so severe as to prevent sexual intercourse. Semen analysis showed a normal sperm count in 63% of the men and no azoospermia. Social integration was satisfactory: 100% of the men attended high school, 55% have a college education and all who are not attending school have full-time jobs. CONCLUSIONS: Our long-term review demonstrates that despite what appears to be a significant sexual handicap, patients with exstrophy/epispadias can have adequate sexual function and overall successful social integration.
Bladder Exstrophy↗
Videofetoscopically assisted fetal tissue engineering: bladder augmentation.
BACKGROUND/PURPOSE: Treatment of several congenital anomalies is frequently hindered by lack of enough tissue for surgical reconstruction in the neonatal period. Minimally invasive harvest of fetal tissue, which is then processed through tissue engineering techniques in vitro while pregnancy is allowed to continue so that at delivery a newborn with a prenatally diagnosed congenital anomaly can benefit from having autologous, expanded tissue promptly available for surgical reconstruction at birth. This concept was applied to a bladder defect. METHODS: Bladder exstrophy was surgically created in ten 90- to 95-day gestation fetal lambs, which were divided in two groups. In group I, a small fetal bladder specimen was harvested through a minimally invasive technique (videofetoscopy). Urothelial and smooth muscle cells were then separately cultivated and expanded in vitro for 55 to 60 days, resulting in a total of approximately 200 million cells. Seven to 10 days before delivery, the cells were seeded in two layers in a 16- to 20-cm2, 3-mm thick biodegradable polyglycolic acid polymer matrix. One to 4 days after delivery, autologous engineered tissue was used for surgical augmentation of the exstrophic bladder. In group II, no harvest was performed, and the bladder exstrophy was primarily closed after delivery. In both groups, a catheter was left inside the bladder for 3 weeks, at which time a cystogram was performed and the catheter then removed. In all animals, at 60 days, another cystogram was performed and urodynamic studies of the bladder were performed. The bladder was then removed for histological analysis. RESULTS: Fetal survival rate was 100%. One newborn died immediately after the implantation of the engineered bladder from an anesthetic accident. The other nine (four in group I and five in group II) survived. One of the animals from group I lost its bladder catheter prematurely and had a urinary leak detected only at the time of death. There were no other complications. The engineered bladders were more compliant (P < .05) and had greater capacity pressures greater than 20 mm Hg (P < .05) than those closed primarily. Histological analysis of the engineered tissue showed a multilayered urothelial lining on the luminal side and overlying layers of smooth muscle cells surrounded by connective tissue. CONCLUSIONS: Videofetoscopically assisted fetal bladder engineering may be a viable alternative for prompt bladder reconstruction at birth. The architecture of autologous engineered fetal bladder tissue resembles that of native bladder. This concept may prove useful for the treatment of certain human neonatal conditions such as bladder and cloacal exstrophies.
Animals↗