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Vesical neck reconstruction in patients with the exstrophy-epispadias complex.

PURPOSE: We evaluated the factors critical in achieving urinary medicine continence in patients with the exstrophy-epispadias complex. MATERIALS AND METHODS: A total of 51 patients with epispadias and 33 with classic bladder exstrophy underwent vesical neck reconstruction. Patient records were reviewed to identify factors associated with achievement of continence, including timing of bladder closure and urethroplasty, effect of bladder capacity before and after vesical reconstruction, and effect of enterocystoplasty. RESULTS: Complete urinary continence was achieved in 42 of the 51 patients with epispadias (82%) and in 23 of the 33 with exstrophy (70%). Delayed bladder closure did not affect the ability to gain continence but increased the likelihood of subsequent enterocystoplasty (12 of 19 patients, 63%) compared to early bladder closure (5 of 14 patients, 36%). CONCLUSIONS: Preliminary urethroplasty did not enhance urinary control or reduce the need for enterocystoplasty. Bladder capacity before vesical neck reconstruction did not predict the need for enterocystoplasty or ultimate continence status in individuals. Adequate bladder capacity after vesical neck reconstruction was an important determinant of urinary continence. Approximately half of the patients bladder exstrophy (17 of 33) required augmentation cystoplasty to achieve urinary continence.

Bladder Exstrophy↗

[Vesico-intestinal fissure: a multidisciplinary problem].

Vesico-intestinal fissure or exstrophy of the cloaca is a rare but serious birth defect of the urogenital tract and distal part of the digestive tract. The most important hallmarks are: bladder exstrophy, fusion between bladder and the exstrophied iliocoecal region, short blind-ending colon and imperforate anus. In addition the lower part of the body shows defects of the abdominal wall, symphysis, upper urinary tract, the internal and external genitals, lumbosacral spine, spinal cord and the lower extremities. It is just after 1960 that corrective surgery started to become successful. Because of the complexity of the anomaly it seems that only management by a team of specialists in a centre for pediatric surgery guarantees optimal treatment. The quality of live of these children can still be improved by application of the newest surgical techniques and excellent cooperation between the different involved specialists. The defects, surgical treatment and results in eight patients with vesico-intestinal fissure, seen at the Universitair Centrum voor Chirurgie bij Kinderen Nijmegen (UCCKN) from 1974 till 1989 inclusive, are presented.

Abnormalities, Multiple↗

Continent gastric tube: new techniques and long-term followup.

PURPOSE: The successful use of stomach for bladder augmentation and substitution is well documented. Gastric tissue has been used more recently to create continent catheterizable tubes. We describe 2 new techniques of gastric tube construction, and report our long-term followup of catheterizable gastric tubes in children and adults undergoing complex urinary tract reconstruction. MATERIALS AND METHODS: A retrospective chart review of 6 male and 4 female patients 5 to 43 years old was done. Primary diagnoses included bladder exstrophy, cloacal exstrophy, rhabdomyosarcoma and neurogenic bladder. Five patients underwent gastrocystoplasty with simultaneous creation of a continent gastric tube from the anterior gastric flap. In 2 patients who had undergone previous gastrocystoplasty a continent gastric tube was created from an anterior flap raised from the existing gastric bladder. Isolated gastric tubes were constructed in 3 patients. RESULTS: Followup ranged from 2 to 9 years (median 3.5). All patients demonstrated easy reliable catheterization. One patient required revision of the proximal end of the tube for incontinence. At followup all tubes were continent. Complications occurred only in flush or protuberant stomas, and resolved after stomal revision with recessed skin flaps. CONCLUSIONS: Several techniques can be used to create a continent gastric tube. Long-term followup reveals reliable catheterization and good continence rates. Recession of the gastric tube stoma with a skin flap prevents peristomal complications.

Adolescent↗

Laparoscopic assisted reconstructive surgery.

PURPOSE: We describe the use of laparoscopy to assist in performing complex pediatric reconstructive cases with the goals of improved cosmesis, and limited postoperative morbidity and adhesion formation. MATERIALS AND METHODS: Eight patients a mean age of 13.4 years underwent 8 laparoscopic assisted reconstructive procedures at our institution from June 1995 to February 1998. The group consisted of 5 patients with spina bifida, 1 with sacral agenesis, 1 with classic bladder exstrophy and 1 with bladder dysfunction secondary to posterior urethral valves. Information was obtained via personal communication and review of the hospital records. RESULTS: Eight successful laparoscopic assisted procedures were performed, including bladder augmentation and an appendiceal Mitrofanoff procedure in 4 cases as well as tapered ileal Mitrofanoff and Malone antegrade continence enema procedures in 1, and bladder augmentation, appendiceal Mitrofanoff and antegrade continence enema procedure, gastrocystoplasty removal, ileal augmentation and an appendiceal Mitrofanoff procedure, and an antegrade continence enema procedure in 1 each. The laparoscopic component of these operations included extensive mobilization of the right colon in all patients and complete appendiceal harvesting in 2. Reconstruction was then completed through a Pfannenstiel incision in 4 patients, previous low midline scar in 2 and a small midline incision in 2. Drains were placed via existing trocar sites and open incisions were carried through other sites whenever possible. Continent stomas were matured through a trocar site in all 8 cases. Final cosmesis was excellent. Operative time was comparable to that of similar open procedures and intraoperative blood loss was minimal. CONCLUSIONS: Laparoscopy may be used as a successful adjunct in complex pediatric reconstructive procedures to minimize disfiguring and morbid upper abdominal incisions, and decrease the risk of future adhesions.

Adolescent↗

Urinary continence after staged bladder reconstruction for cloacal exstrophy: the effect of coexisting neurological abnormalities on urinary continence.

PURPOSE: We determined whether there is a difference in the incidence of urinary continence in cloacal and classic bladder exstrophy after staged bladder neck reconstruction using the Young-Dees-Leadbetter technique. MATERIALS AND METHODS: We reviewed the records of patients with cloacal and classic bladder exstrophy who underwent staged bladder neck reconstruction from 1971 to 1997. RESULTS: The Young-Dees-Leadbetter bladder neck reconstruction was completed in 23 patients with cloacal exstrophy, of whom 5 (22%) became continent and 18 (78%) have persistent incontinence. A clinically apparent neurological abnormality significantly hindered the achievement of continence. Specifically 1 of the 13 children (7%) with versus 4 of the 10 (40%) without a neurological abnormality became continent (p <0.05). In contrast, staged reconstruction of classic bladder exstrophy resulted in urinary continence in 67 of the 82 patients (82%). Of the 67 continent patients 23 (34%) cannot void and require intermittent catheterization. None of the patients with classic exstrophy had a neurological deficit. CONCLUSIONS: The ability of the Young-Dees-Leadbetter bladder neck reconstruction to result in urinary continence significantly differs in the cloacal and classic bladder exstrophy populations (22 versus 82%, p <0.001). Our findings also suggest that a coexisting neurological abnormality significantly hinders the ability to reconstruct a functional bladder in patients with cloacal exstrophy.

Bladder Exstrophy↗

Radical single stage reconstruction in failed exstrophy.

Staged repair of exstrophy-epispadias complex cases with initial bladder closure followed by epispadias and bladder neck repair at a later date is well documented. However, the role of single stage repair as a primary and salvage procedure after failed closure is not well established. A total of 12 male patients with bladder exstrophy has undergone single stage repair at our facility (10 secondary and 2 primary procedures). Mean age of the 10 patients referred for secondary repair was 5.5 years (8 months to 13 years). In this group there had been 16 previously unsuccessful attempts at bladder closure and 15 operations for epispadias repair. The 2 primary repair patients were 15 months and 13 years old. Continence was achieved by bladder neck reconstruction combined with epispadias repair and all cases were augmented by enterocystoplasty in conjunction with a Mitrofanoff channel. Pelvic osteotomy was performed in 3 cases. After bladder neck revision in 2 children all are dry day and night on clean intermittent catheterization and have a satisfactory cosmetic appearance. Single stage reconstruction of the exstrophy complex is a satisfactory method with a low morbidity for treating children in whom previous repair has failed. Its more extended role in the primary case requires further evaluation.

Adolescent↗

Periurethral muscle complex reassembly for exstrophy-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.

Bladder Exstrophy↗

Patterns of renal damage in the management of vesical exstrophy.

We reviewed the patterns of pyelonephritic damage that occurred during surgical management of bladder exstrophy. Renal injury patterns indicate that papillary configuration and obstruction coupled with urinary infection were the primary factors in producing damage. Diversion by the ileal conduit resulted in frequent injury, whereas the colon conduit was associated rarely with renal damage. The incidence of renal injury from bladder closure and continence attempts suggested closure as a reasonable course, especially in view of present continence results.

Bladder Exstrophy↗

Application of the Mitrofanoff principle in children with severe impairment of bladder function.

Treatment of children with severe impairment of bladder function requires a large-volume, low-pressure reservoir combined with a continent, easily catheterizable valve. The Mitrofanoff principle (MP) appears to meet these requirements. Between 1986 and 1993, the MP was applied in 15 children (4 girls) aged 4 to 14 years. The primary diagnosis was bladder exstrophy in 8 (2 girls), neuropathic bladder in 3 (2 girls), urethral valves in 2, and rhabdomyosarcoma (RMS) in 2. In 10 patients bladder augmentation with an intestinal patch was performed in addition to a Mitrofanoff procedure; in 5 a neobladder and continent appendicostomy were made. One boy with RMS died of distant metastases with a well-functioning appendicostomy and adequate renal function. At 2 to 9 years follow-up of the other 14 patients, 12 have a good result defined as: (1) adequate reservoir capacity; (2) continence; (3) normal renal function; and (4) no hydronephrosis. In 1 exstrophy patient with pre-existing impairment of renal function, further deterioration necessitated frequent catheterization and additional medical treatment. In 1 boy with fulgurated urethral valves, spontaneous micturition became subsequently possible, allowing closure of his appendicovesicostomy. Complications occurred in 10 patients, necessitating reintervention in 7. The MP in combination with the creation of an adequate reservoir gives good results in children with severe impairment of bladder function. Careful attention should be given to patient education regarding emptying of the reservoir. Long-term follow-up of renal function is mandatory.

Adolescent↗

[Studies following bladder reconstructions in cystoschisis].

The question how to perform the best surgical repair of bladder exstrophy is still being discussed. By various urodynamic investigations following bladder reconstruction we came to the conclusion that the new built bladder and its outlet tract can function normally.

Bladder Exstrophy↗

Nonrefluxing colon conduit for temporary or permanent urinary diversion in children.

Colon conduit, with nonrefluxing ureterosigmoid anastomoses, was performed upon 21 children in the past 3 1/2 yr for the following indications: (1) previously unoperated exstrophy of the bladder; (2) exstrophy of the bladder with prior ileal conduit; (3) previously unoperated neurogenic bladder; (4) neurogenic bladder with prior ileal conduit; and (5) with anterior pelvic exenteration for sarcoma of the prostate. In some of these patients the conduit will be a permanent diversion; in others it will be anastomosed later to the colon, providing a staged method of ureterosigmoid urinary diversion. Nine patients have undergone the second stage of anstomosis of the conduit to the colon with satisfactory outcome. Colon conduit diversion is a more time-consuming procedure than ileal loop diversion. It can require 6-8 hr in a patients with a prior ileal diversion, particualarly if the ureters require tapering. Our experience with this procedure leads us to the following conclusions: (1) this is a better method than ileal conduit for permanent diversion; (2) it offers an alternative way for treating patients with bladder exstrophy; (3) it is useful in patients with pelvic cancer; and (4) it should be considered for those patients with ileal conduits who are not doing well, i.e., who have infection and renal deterioration.

Bladder Exstrophy↗

Primary squamous cell carcinoma in unreconstructed exstrophic bladder.

Bladder exstrophy is associated with an increased incidence of primary adenocarcinoma of the bladder. We report a rare case of squamous cell carcinoma occurring in the unreconstructed, exstrophic bladder of a 53-year-old woman treated with radical cystectomy and radiochemotherapy. This case represents the oldest patient to present with squamous cell carcinoma of an unreconstructed exstrophic bladder. We discuss the potential mechanisms of carcinogenesis in this patient, noting how they may potentially differ from those in a patient with a reconstructed bladder.

Bladder Exstrophy↗

Neural innervation of the newborn exstrophic bladder: an immunohistochemical study.

PURPOSE: Continence in bladder exstrophy is not always easy to attain. Some patients have a small noncontractile bladder while in others an adequate capacity bladder may not contract normally. Innervation of the detrusor determines its ability to contract. The exstrophic bladder during organogenesis does not store urine. Therefore, its requirements for contraction are limited and its innervation would potentially reflect this difference in function. The availability of specific immunostains allows better differentiation of the neural elements in tissue specimens. Our study focuses on myelinated nerves innervating the newborn exstrophic detrusor. MATERIALS AND METHODS: Biopsies were obtained from the anterior wall of 10 newborn exstrophic bladders at the time of initial closure and compared to 10 newborn controls. Patient age at bladder closure ranged from 1 to 90 days (mean 22). Specimens were formalin fixed and paraffin embedded. Then 4 micro. thick sections were stained with S100, an immunostain that stains neural crest elements including Schwann's elements (myelinated nerve fibers). The entire tissue section was examined. Microscopic fields were sequentially examined with a morphometric system comprising a microscope, video camera and personal computer with a video frame grabber. The output image was displayed on a second monitor and the nerves in each field and numbers of fields in each section were counted. To be considered, fields had to have greater than 75% tissue coverage. The average number of nerves per field was compared between the exstrophic bladders and normal controls. RESULTS: The average number of myelinated nerves per field in the newborn exstrophic bladders (0.13 per field) was significantly reduced compared to normal controls (1.25 per field) and statistically significant (p<0.001). This reduction in nerve fibers appeared to be due to lack of smaller fibers with preservation of larger fibers. There was no difference in innervation in cases closed at birth compared to those closed after month 1 of life. CONCLUSIONS: The newborn exstrophic bladder has fewer myelinated nerve fibers than normal controls primarily due to reduction in the smaller fibers which may represent a maturational delay in the development of the exstrophic bladder. Another possible explanation may be degeneration of the fibers due to lack of bladder contraction but no indication of nerve degeneration was evident in our study. Followup studies of patients at various stages of reconstruction will determine the evolution of neural innervation in the exstrophic bladder.

Bladder Exstrophy↗

Urethral replacement with ureter.

Proximal or total urethral replacement was performed in 8 patients using distal ureter. This was accomplished by basing the ureteral segment solely on a vascular pedicle arising from the internal iliac artery. Diagnoses included classical bladder exstrophy in 4 patients, cloacal exstrophy in 3 and an imperforate anus with hypoplasia of the bladder neck and urethra in 1. Continence was achieved by a tunneled submucosal reimplantation into either the bladder (4 cases), gastric reservoir (2) or colonic reservoir (2). Although an isolated distal segment of ureter was used in each case, all segments have remained viable. With limited followup (5 to 44 months) continence with either normal voiding or intermittent catheterization has been achieved in 7 of the 8 patients. A distal ureteral segment should be considered potentially useful in the construction or reconstruction of the proximal (male) or total (female) urethra in patients with congenital urethral malformations.

Anus, Imperforate↗

Submucosal bladder neck injections of glutaraldehyde cross-linked bovine collagen for the treatment of urinary incontinence in patients with the exstrophy/epispadias complex.

During the last 7 years 19 patients underwent 33 transurethral injections of glutaraldehyde cross-linked bovine collagen into the bladder neck for stress incontinence. Of the 15 patients 14 have classic bladder exstrophy, 3 have complete male epispadias and 2 have cloacal exstrophy. The procedure was performed after a Young-Dees-Leadbetter bladder neck reconstruction in 15 patients and before it in 4. Injections were repeated in 10 patients after a mean of 12 months. After a mean followup of 26 months (range 9 to 84) improvement of continence was noted in 10 of 19 patients (53%) of whom 4 have significant improvement. Of the 8 patients whose condition failed to improve after collagen injections 6 underwent additional successful surgery to achieve urinary continence. Of the 10 patients who underwent repeated collagen injections 9 (90%) had additional improvement. Although there were no complications related to the injected collagen itself, postoperative complications developed in 2 patients. Submucosal injection of collagen to the bladder neck is simple and safe, and has a reasonable success rate. Thus, it may be used to improve continence in patients with the exstrophy/epispadias complex who lack full control after appropriate reconstructive surgery.

Adolescent↗

Submucosal bladder neck injection of bovine dermal collagen for stress urinary incontinence in the pediatric population.

PURPOSE: The surgical management of stress urinary incontinence in the pediatric population includes various surgical techniques of which the simplest is outpatient transurethral or periurethral injection of a bulking agent. Currently purified bovine dermal collagen cross-linked with glutaraldehyde (Contigen) is the only Food and Drug Administration approved bulking agent available. Patients with stable detrusor function are ideal candidates. We review our initial experience with children. MATERIALS AND METHODS: Between January 1994 and June 1995, 23 boys and 9 girls 4 to 17 years old (mean age 9) consecutively underwent submucosal bladder neck injection with cross-linked bovine dermal collagen. Incontinence was secondary to spinal dysraphism in 24 patients, complete epispadias in 4, classic bladder exstrophy in 3 and neurogenic bladder secondary to a sacral teratoma in 1. Preoperative video urodynamics documented intrinsic sphincteric deficiency in all patients with leak point pressure of 15 to 60 cm. water (mean 37). Injection volumes ranged from 2.5 to 17 cc (mean 10). Continence results were defined as dry-requiring no protection while on a 4-hour clean intermittent catheterization program, good-improved but requiring 1 to 5 pads daily and failure-no postoperative improvement or still requiring diapers. RESULTS: Of the children with neurogenic bladder 20% became dry following the first injection and an additional 28% had a good result. Furthermore, of the 6 children with exstrophy or epispadias who underwent a Young-Dees-Leadbetter procedure 50% were dry and 17% had a good result after the first injection. Complications were limited to a febrile urinary tract infection associated with urinary retention in 1 patient and transiently worse continence in 2. CONCLUSIONS: Despite the limited success rate, we believe that transurethral collagen injection therapy has a viable role in the treatment of intrinsic sphincteric deficiency in select pediatric patients, particularly since the procedure has low morbidity and can be performed on an outpatient basis. Preoperative counseling should be given with realistic expectations.

Administration, Intravesical↗