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Vaginal calculus as a late complication of bladder exstrophy.

STUDY OBJECTIVE: To document an unusual finding of a vaginal calculus in a patient with bladder exstrophy. DESIGN: Case report. SETTING: Tertiary Centre, Royal Children's Hospital, Melbourne, Australia. PARTICIPANTS: Fourteen year old female. INTERVENTIONS: Cystotomy, introitoplasty. MAIN OUTCOME: Removal of vaginal calculus. MEASURES: None. RESULTS: Documentation and removal of a vaginal calculus. CONCLUSIONS: Vaginal calculi should be included in the differential diagnosis of urolithiasis in patients with bladder exstrophy.

Adolescent↗

Complete primary repair of bladder exstrophy: initial experience with 33 cases.

PURPOSE: We evaluated our initial experience with complete primary repair of bladder exstrophy in 33 children. MATERIALS AND METHODS: Between 1998 and 2001, 33 children with classic bladder exstrophy were treated with 1-stage primary repair for the first time in all except 4, who had undergone previous failed initial bladder closure. Our series included 26 boys and 7 girls with a mean age of 2 months (range 3 weeks to 14 months). The bladder was closed in continuity with the urethra and complete penile disassembly was used for epispadias repair. Anterior transverse innominate osteotomy was performed in all cases. Combined general and caudal anaesthesia were applied in all cases with an indwelling epidural caudal catheter in 7. RESULTS: Median followup was 42 months (range 24 to 62). Enterocystoplasty was needed in 3 cases during primary repair of a small bladder plate. Wound dehiscence was not recorded. Bladder neck fistula was reported in 2 children, while urethral fistula was recorded in 1 boy. Abdominal ultrasound detected no hydronephrosis in all except 3 patients. Voiding cystourethrogram showed vesicoureteral reflux in 6 patients. No loss of renal function or febrile urinary tract infection was recorded. A dry interval of 3 hours or greater was reported in 24 children (72.7%), while 9 who were incontinent of urine after failed toilet training needed other procedures to achieve continence. CONCLUSIONS: Complete primary repair with penile disassembly provides a good approach to achieve this purpose without the need for bladder neck reconstruction in some cases. Selection of the proper surgical technique together with adjunctive procedures such as osteotomy and a pain-free early postoperative period can maximize the chance of successful exstrophy repair.

Anesthesia, Caudal↗

[Comparative results of ileal conduit and colonic conduit. Analysis of 50 children with bladder exstrophy].

During the last 20 years 50 children with exstrophy of the bladder were treated in the Department of Paediatric Surgery of the Children's Hospital of Cologne employing besides other surgical methods such as primary closure, ureterosigmoideostomy, ureterocutaneostomy etc., in 15 cases an ileal conduit and in 12 cases a colonic conduit. These children could be followed up on the average 8.5 or 3 years after the operation, clinically, roentgenologically and in some cases via scintigraphy. Late complications requiring surgical correction, such as stomatostenoses, conduit elongation, stenoses of the ureterointestinal anastomosis, calculus formation in the conduit, or complications like ureteral reflux, recurring infections of the urinary passages with pyelonephritis, occurred only with ileal conduits, whereas no late complications requiring surgery were seen with the colonic conduits. Similar results in respect of late complications were found among the patients in Munich from 1955 to 1983 with 35 exstrophies of the bladder (13 ileal conduits, 1 colonic conduit) in which additionally an adeno-carcinoma was seen after ureterosigmoideostomy with fatal outcome. Hence, we are of the opinion that the method of choice is the preparation of a colonic conduit in patients with exstrophy of the bladder where primary closure is not possible because the bladder lamina is too small or already epithelialised. This approach offers the safest possible long-term protection of the primary normally positioned upper urinary tract.

Bladder Exstrophy↗

Comparison of posterior and anterior pelvic osteotomy for bladder exstrophy complex.

To compare the correction and maintenance of the pelvic ring after pelvic osteotomy in bladder exstrophy complex, we analyzed all cases performed at our institution. Posterior osteotomy was performed in six patients; anterior or combined osteotomy was performed in four patients. Patients who underwent posterior osteotomy had a mean pubic approximation of 37.3%. The mean was 62.8% in patients undergoing anterior or combined osteotomy. This difference was statistically significant (P<0.05 ). The mean recurrence of separation in pubic diastasis was 90.5% for posterior osteotomy and 41.6% for anterior or combined osteotomy. These results indicate that an anterior or combined pelvic osteotomy corrects and maintains the pelvic ring with a bladder exstrophy complex more effectively than a posterior pelvic osteotomy.

Bladder Exstrophy↗

Pregnancy and sexual function in women with bladder exstrophy.

As a result of improved surgical and medical treatment, an increasing number of women with bladder exstrophy are reaching childbearing age. Unfortunately, little data exist regarding their sexual capacity, fertility, and potential complications of pregnancy. To assess these parameters, the medical records of 40 women ranging in age from nineteen to thirty-six years who had been treated in infancy for bladder exstrophy were reviewed. The 14 pregnancies in 11 women (25%) resulted in 9 successful deliveries, 3 spontaneous abortions, and 2 elective abortions. Nine women in this group had previous urinary diversion. Twelve of the 40 women agreed to participate in a survey of sexual capacity. Eight of this group reported that they engaged in regular sexual activity. Six reported regular orgasms, 4 dyspareunia, and 5 dysmenorrhea. Five of these women had achieved 7 pregnancies, and only 1 woman in this group who desired pregnancy had been unable to conceive. Complications during pregnancy included: uterine prolapse in 7, acute pyelonephritis in 1, prolapsed ileal conduit in 1, and transient urinary incontinence in 1.

Adult↗

Anterior pelvic osteotomy. A new operative technique facilitating primary bladder exstrophy closure.

Posterior iliac osteotomy is a recognised method employed to facilitate and support anterior abdominal wall closure in patients with bladder exstrophy, but it adds considerably to the already lengthy procedure of bladder exstrophy surgery. Anterior pelvic osteotomy of the superior ramus of the pubic bone was developed to overcome this problem and to achieve a stable anterior pelvic ring with tension-free soft tissue closure. It can easily be performed after completing the bladder closure, without the need to turn the patient.

Bladder Exstrophy↗

Occurrence of adenocarcinoma in the colon sigmoideum following Maydl's operation for urinary bladder exstrophy.

Two cases of colonic adenocarcinoma appearing 44 and 25 years, respectively, following an operation for urinary bladder exstrophy done according to Maydl are reported. A need for regular controls of patients with inner urine derivations has been emphasized. In the case of a suspected tumour it is necessary to alter the inner derivation to an outer one and also a resection of the colon at the site of the urinary bladder trigone is required. The Czech surgeon Karel Maydl was the first to implant the trigone of a splitted urinary bladder into the colon sigmoideum in 1892. This type of operation is used even today by several European urologists with excellent long-term results with respect to the preservation of an intact ureterovasical passage which prevents the reflux of the intestinal contents into the ureter, and also prevents the formation of strictures in the terminal parts of the ureter. In two patients who had been living for 25 and 44 years since they were operated on for urinary bladder exstrophy according to Maydl, a colonic adenocarcinoma was diagnosed. The tumour directly affected the implanted trigone wall and also involved the surrounding parts of the colon wall.

Adenocarcinoma↗

Prosthesis implantation after radial free flap phalloplasty in patients with bladder exstrophy.

METHODS: Inflatable penile prosthesis were implanted into sensate radial free-flap phalloplasties in two adult patients with bladder exstrophy. RESULTS: Neither patient reported loss of sensation, and both were able to inflate the prosthesis and engage in sexual intercourse three months later. CONCLUSION: The construction of a functional neophallus is well described however has not previously been reported in exstrophy patients who are suited to this procedure due to the presence of crura, and the absence of a neourethra. Scarring from previous reconstructive procedures however may make implantation difficult, and long-term follow up is required to evaluate this procedure in patients with bladder exstrophy.

Adult↗

[Bladder exstrophy and pregnancy. Apropos of 2 cases].

The authors report two cases of pregnant women suffering from exstrophy of the bladder. One of the patients had been operated on in childhood for reconstruction of the bladder, and the other had undergone Coffey's operation, so that both were able to lead practically normal lives in adulthood. Three children were born by prophylactic cesarean. Exstrophy of the bladder is often associated with other urological, genital or, on occasion, orthopedic, malformations. This article analyzes the consequences of these malformations on the course of pregnancy and delivery, and the consequences of pregnancy on the urinary tract or on the different types of surgical reconstruction. The indications for the type of delivery (natural passages or cesarean section) are discussed in the light of the author's personal experience and a review of the literature.

Abortion, Spontaneous↗

Outcome analysis of the psychosexual and socioeconomical development of adult patients born with bladder exstrophy.

Since sonographic evaluation of the fetus became routine, prenatally established diagnosis of multiple or severe malformations raises the question of whether a pregnancy should be terminated. To answer this question properly, all aspects concerning the life of a child born with a serious malformation, such as exstrophy of the bladder, must be considered. Therefore, more than ever an evaluation of the quality of life of these patients is necessary to be able to make a solid based decision. We studied the psychosexual and socioeconomic development of 22 adult exstrophy patients (11 men and 11 women) by a standardized personal interview, evaluation of a standard performance test SCL-90 (symptom checklist 1990) and an adapted personality score test. The latter 2 tests are well established and provide reproducible results. Based on several large scale investigations, standard scores are available. Nine women (82%) and 10 men (91%) scored better or in accordance with the standard scores. Exstrophy of the bladder does influence the psychosexual and socioeconomic development. The quality of life is hardly ever considered so poor that termination of pregnancy is a realistic alternative.

Adult↗

An alternative treatment modality in closing bladder exstrophy: use of rectus abdominus muscle flap--preliminary results in a rat model.

The aim of this study was to find a new alternate method for bladder exstrophies with small capacity and inelasticity, and to resolve complications of other bladder augmentation techniques. In 50 Wistar albino rats, a large bladder defect was created excising at least one half of their original bladder, keeping the peritrigonal zone intact. In each rat, a 2.5 x 1-cm inferiorly based rectus abdominus muscle flap was prepared from the lower abdominal quadrant. This flap was then rotated to cover the bladder defect. The inner layer formed by the peritoneum was sutured to the edges of the bladder defect by 6-0 separate sutures. The post-operative radiologic and scintigraphic examination of the urinary system done at different intervals showed no difference from that of normal rats. The only observed disadvantage of this technique was the formation of calculi in the bladder in 8/50 rats in the late post-operative period. Post-mortem histopathologic investigations performed at different intervals showed the inner layer of the flap to be completely covered by the transitional urinary epithelium of the bladder. We think this technique is easy to perform, non-time-consuming, and has a low complication rate. It may be useful in infants with small, noncompliant, inelastic bladder exstrophies.

Abdominal Muscles↗

Clinical results with anterior diagonal iliac osteotomy in bladder exstrophy.

PURPOSE: We report our clinical experience with anterior diagonal iliac osteotomy in 10 patients who underwent surgery for bladder exstrophy. Technique and long-term results are discussed. MATERIALS AND METHODS: A total of 10 boys 1 month to 9 years old with bladder exstrophy underwent this procedure during a 2-year period. RESULTS: None of the patients had bladder closure dehiscence or prolapse after the operation. There were neither infectious complications nor injury to the vessels or nerves in any case. Blood loss was minimal for anterior diagonal iliac osteotomy. The only significant complication in our series was the polypropylene erosion of the urethra, necessitating endoscopic removal in 1 patient 1 month postoperatively. All patients had wide diastasis of the pubis preoperatively (average pubic distance 53.3 cm., average pubic ratio 0.9). At surgery suturing the symphysis after bilateral osteotomy resulted in a satisfactory symphyseal approximation and tension-free closure of the abdominal wall was easily achieved in all cases. Radiological studies at a mean followup of 34.6 months (range 14.8 to 49.5) revealed significant recurrent diastasis of the pubic bones in all but 1 patient in whom bone grafts were applied between the iliac fragments. Mean interpubic distance was 42 cm. and mean pubic ratio was 0.6 at long-term followup. CONCLUSIONS: Diagonal osteotomy may correct the principal bony deformity in exstrophy and enables initial symphyseal approximation. Pubic diastasis may recur, probably due to opening forces generated by soft tissue elements of the pelvis.

Bladder Exstrophy↗

A novel technique for approximation of the symphysis pubis in bladder exstrophy without pelvic osteotomy.

PURPOSE: We evaluated the outcome of pubic bone approximation and internal fixation using a miniature metal plate and screws without osteotomy in children with bladder exstrophy. MATERIALS AND METHODS: A total of 17 children with bladder exstrophy underwent surgery. Mean patient age was 27 months. Of the patients 15 had a history of 1 or more failed bladder closures with or without osteotomy. All children underwent bladder closure and complete posterior and anterior urethroplasty with bladder neck wrap. Both ureteral orifices were brought together by the Gil-Vernet antireflux procedure. The pubes were brought together with 3 deeply placed 1 mm polyglactin sutures through the bone and fixed by placing a miniature metal plate with 6 to 7 screws. Patients remained in a frog leg bandage for 1 to 3 weeks. The plate was removed during additional surgery. Median followup was 61 months (range 14 to 71). RESULTS: All children had an uneventful postoperative period without any serious complications, except for skin erosion caused by a screw without bone infection in 1 patient, and miniature plate subluxation following cystoscopy for reinsertion of urethral catheter 3 days after initial surgery in 1. Operating time and hospital stay were significantly lower than in children undergoing osteotomy or Bryant's traction. Overall continence rate was 53.8%. CONCLUSIONS: Although the number of patients is low, these results are promising. Pubic bone adaptation with miniature plate fixation without any type of osteotomy or leg traction is safe and less invasive than bilateral osteotomy and postoperative leg traction or plaster.

Bladder Exstrophy↗

Bladder exstrophy in Sweden--a long-term follow-up study.

A retrospective review of all new cases of bladder exstrophy in Sweden in 1970 through 1989 is presented. The aim was to evaluate management of the malformation in Sweden and possibly identify factors that might improve the outlook. The incidence was 1:33,500 births. Records were reviewed of the 61 children (37 males and 24 females) treated at Sweden's four specialist centers of pediatric surgery. There was one perinatal death. Primary management was mainly early bladder closure (within 72 hours), late closure or perinatal urinary diversion. Subsequent measures included augmentation cystoplasty, bladder neck reconstruction, provision of continent urinary reservoir and penile reconstruction. The follow-up time was 4-19 years. In 15 cases renal scarring developed, though with impairment of total function in only three. Ten patients achieved continence, defined as 3-hour dry intervals, of whom four had urinary reservoirs with continent stoma. Urinary tract infections were recorded during follow-up in 43% of the surviving patients, though the true incidence of infection presumably was higher. To improve management of bladder exstrophy in sparsely populated Sweden, a nation-wide strategy of ongoing interhospital collaboration has been adopted. Results will be continuously presented.

Adolescent↗