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PubMed2000

Management of bladder exstrophy.

The exstrophy-epispadias complex is a severe anomaly of the lower urinary tract. While the surgical reconstruction of normal anatomy in patients with this disorder was once considered impossible, modern management has made possible a near-normal reconstruction of the bladder and lower abdominal wall. Early operation in the neonatal period along with closure of the pelvic ring has been shown to significantly improve results in terms of continence and achievement of adequate bladder capacity. With the possibility of prenatal diagnosis, it is important that the counseling obstetricians and neonatologists should understand the basis of the surgical management and the outcome that can be expected.

Bladder Exstrophy↗
PubMed1991

Failed bladder exstrophy repair. Evaluation and management.

Early successful closure, careful management of the incontinent interval, and a good technical bladder neck reconstruction can result in a patient with normal control and upper tracts. However, failure at any step of a staged functional reconstruction does not doom the child to failure in the eventual achievement of the above goals. Nonetheless, taking care of the exstrophy failure is still a formidable task for the reconstructive surgeon. Currently, surgical techniques exist that can salvage the vast majority of these children. Careful evaluation of the failure, waiting an appropriate interval before further reconstructive surgery is attempted, and dedication to the patient most often can produce a successful result.

Adolescent↗
PubMedDate not supplied

[The choice of the method of urinary diversion in bladder exstrophy in children].

Transplantation of the ureters into the sigmoid colon by Tikhov-Coffey in the authors' modification has been carried out in 10 children. The ureters were placed into the channel made by supramucosal dissection. Good response was achieved in 9 patients. One case of transplantation of the only ureter of heminephrectomized kidney was fatal.

Bladder Exstrophy↗
PubMed1999

Strategies for reconstruction after unsuccessful or unsatisfactory primary treatment of patients with bladder exstrophy or incontinent epispadias.

PURPOSE: Following unsuccessful or unsatisfactory primary treatment in patients with the epispadias/exstrophy complex, the options for a surgical solution to preserve the upper urinary tract, to achieve complete continence, and to reconstruct the external and female internal genitalia are limited. We reviewed the records of the patients treated at our institution to determine a surgical compromise between ingenious operative constructions and patient desires, both of which are secondary to stabilization of renal function. MATERIALS AND METHODS: From 1967 to December 1997, 128 patients with bladder exstrophy/epispadias complex were treated, of 80 whom had received previous unsuccessful or unsatisfactory treatment. Of these patients 72 were followed for an average of 22.4 years after the first surgical intervention. There were 40 patients referred to our institution after primary bladder closure and bladder neck reconstruction, 10 after rectal bladder, 7 after ureterosigmoidostomy and 5 after incontinent diversion. RESULTS: At the latest followup 19 patients had a rectal reservoir, 38 an ileocecal pouch, 12 a conduit diversion and 3 an augmented Young-Dees procedure. The upper urinary tract remained stable in 95% of the renal units with rectal reservoir, 95% with ileocecal pouch, 96% with a colonic conduit and 100% with an augmented Young-Dees procedure. Day and night continence was achieved in 95% of the patients with a rectal reservoir and 97% of those with an ileocecal pouch were continent, whereas only 2 of the 3 patients with an augmented Young-Dees procedure were continent. Of the women 16 were satisfied with the cosmetic results and 6 delivered 8 children by cesarean section. Only 1 man was dissatisfied with the final cosmetic result. All adults but 1 engaged in sexual intercourse. CONCLUSIONS: The first operative intervention in patients with bladder exstrophy/epispadias complex determines their fate. After failure of primary treatment, the upper urinary tract must be stabilized. In patients with severely impaired renal function the colonic conduit is our method of choice while in those with a normal or slightly dilated upper urinary tract and intact anal sphincter we performed a rectal reservoir. In the remaining patients an ileocecal pouch guarantees continence day and night. The results of genital reconstruction are satisfactory.

Adult↗
PubMed1978

Continence in cases of bladder exstrophy.

The relationship of the onset of continence, staged surgical procedures and continence length was investigated in 21 patients with closed continent exstrophy. Continence occurred within a year of operation in 13 patients and 10 of these 13 patients had a Young-Dees urethroplasty and Marshall-Marchetti-Krantz suspension. In 11 of these 13 patients the continence length was greater than 1 cm. In the remaining 8 patients continence was delayed beyong a year. Only 1 of these 8 patients had undergone both bladder neck procedures and in 5 patients the continence length was greater than 1 cm.

Adolescent↗
PubMedDate not supplied

Bladder exstrophy

Explore the source record for details and available documents.

Abdominal Cavity↗
PubMed1997

[Tramadol infusion for pain therapy following bladder exstrophy surgery in pediatric wards].

We investigated in 17 children (mean 7.1 years) the continuous administration of tramadol following augmentation cystoplasty or exstrophy reconstruction. Mean duration of the tramadol administration on the pediatric ward was 3.8 +/- 1.1 days (initial dosage 0.25 mg/kg/per hour, dose adjustment by the nursing staff). Mean tramadol consumption was 0.21 mg/kg/h on day 1 and was reduced to 0.08 mg/kg/per hour on day 4. Median pain score (assessed with ten-step scales) was 5 before treatment and between 2.5 (day 1) and 0.5 (day 5) during therapy. Lowest oxygen saturations (mean) ranged from 93.8% to 95.2%. Three patients (17.6%) suffered from nausea/vomiting on 3 of 64 treatment days (4.7%). Pruritus and extreme sedation did not occur. The continuous administration of tramadol is a simple and safe procedure following major urological surgery in children.

Adolescent↗
PubMed1991

Penile deformity in bladder exstrophy: correlation with closure of pelvic defect.

In the exstrophy-epispadias complex of anomalies, penile deformity represents one of the factors that causes long-term disability in males. In order to define the extent to which the separation of the bony elements may contribute to the final penile length and configuration, an in vitro model was devised using an adult male pelvis and an inflatable penile prosthesis. This model was used to study the relationship of interpubic distance to penile length. Closure of the "open book" deformity of the bony pelvis resulted in an increase in penile length. Closure of the pubic diastasis is best accomplished by iliac osteotomies. This study supports clinical evidence that iliac osteotomies, when performed in conjunction with closure of the exstrophic urinary bladder, may contribute to an increase in effective penile length.

Bladder Exstrophy↗
PubMed2003

Ureteral reimplantation before bladder neck plasty in the reconstruction of bladder exstrophy: indications and outcomes.

OBJECTIVES: After initial closure, all exstrophy patients have vesicoureteral reflux. This reflux is usually managed with antimicrobial prophylaxis, surveillance, and ureteral reimplantation concurrent with bladder neck plasty. Patients with recurrent urinary tract infections or worsening hydronephrosis may require earlier correction of reflux. This subset of patients was reviewed to determine the ability to correct reflux adequately, the difficulty with subsequent bladder neck plasty, and the long-term continence results. METHODS: We reviewed an exstrophy database of more than 700 patients and identified 19 who underwent ureteral reimplantation independent of bladder neck plasty. All had undergone bladder closure in infancy. Eleven had since undergone modified Young-Dees-Leadbetter bladder neck plasty. Five patients were awaiting bladder neck reconstruction, 2 girls were continent without bladder neck plasty, and one had undergone augmentation. RESULTS: Indications for early ureteral reimplant were recurrent febrile infections despite adequate prophylaxis (n = 15) or worsening hydronephrosis on follow-up (n = 4). In the 11 patients who had undergone subsequent bladder neck repair, prior reimplantation did not increase the operative difficulty or complications. The continence results among these 11 were comparable with those in patients without prior reimplantation. In 1 patient, reflux recurred and was corrected at the time of bladder neck plasty. None had further urinary tract infections, and hydronephrosis improved in three and stabilized in one. CONCLUSIONS: In patients who present with recurrent infections or worsening hydronephrosis after initial exstrophy closure, early reimplantation can be undertaken. If performed with later bladder neck plasty in mind, the surgical treatment of incontinence is not compromised and recurrent infections and upper tract changes will be abated.

Abdominal Wall↗
PubMed1987

Followup of ureterosigmoidostomy diversion for bladder exstrophy--behavioral biofeedback as an alternative treatment for fecal-urinary incontinence: a case report.

A total of 6 children underwent primary ureterosigmoidostomy diversion between 1972 and 1979. Despite preoperative testing of rectal competence all 6 children now experience rectal leakage. Behavioral biofeedback has been used in a 7-year-old boy who had learned to isolate and to contract the perineal muscles appropriately by 12 months of followup. The incidence of incontinence has been reduced from 15 to 5 per cent of the total hours recorded. The amount of waking hours in which the patient is wet has decreased from 31 to 6 per cent. Satisfaction of the child and parents is high.

Behavior Therapy↗
PubMed2005

Magnetic resonance imaging of pelvic musculoskeletal and genitourinary anatomy in patients before and after complete primary repair of bladder exstrophy.

PURPOSE: We characterize pelvic soft tissue and bony anatomy of patients before and after complete primary repair of exstrophy (CPRE). MATERIALS AND METHODS: We evaluated 15 measurements on pelvic magnetic resonance imaging (MRI) in patients who underwent CPRE without osteotomy at our institution from 1996 to 2004. MRI protocols included axial, sagittal and coronal fast spin echo proton density sequences. Measurements were compared before and after CPRE using a paired t test, and between patients after CPRE, and age and gender matched controls using linear regression adjusting for the matched case-control groups. Patients older than 3 years with continent intervals longer than 3 hours after CPRE were compared to age and gender matched controls using linear regression. RESULTS: A total of 29 MRIs in 18 patients with exstrophy were available for analysis. Median patient age at postoperative MRI was 25 months (range 4 to 36). The configuration of the post-CPRE pelvis was significantly different from that of controls in all parameters measured including wider symphyseal diastasis (34.5 mm vs less than 1 mm) and more obtuse iliac wing (121 degrees vs 98 degrees), puborectalis (94 degrees vs 49 degrees) and ileococcygeus angles (111 degrees vs 98 degrees ). The anatomy of continent patients after CPRE was not significantly different from that of controls in most parameters measured. CONCLUSIONS: Comparison of the pelvic anatomy in patients before and after CPRE suggests that after CPRE patients have parameters that more closely approximate, but are still significantly different, from those of control patients. Patients with greater than 3-hour continent intervals after CPRE have anatomic parameters most similar to those of age matched controls.

Bladder Exstrophy↗
PubMed1990

Fascia lata and early spica casting as adjuncts in closure of bladder exstrophy.

Correction of the skeletal defect seen in the exstrophy complex consists of reconstitution of the pelvic ring. Long-term success depends upon the formation of a fibrous union between the pubes. When this union does not occur diastasis of the pubis results. Long-term urological success appears to be related to adequate pubic approximation. We used fascia lata to construct an anterior pelvic ligament between the pubic bones in 7 patients. In the 6 patients for whom followup is available the fascia lata has persisted and appears to be viable histologically on biopsy specimens obtained at a subsequent operation. Because the fascia lata holds the pubis together so well we have been able to cease Bryant's traction in children by 2 weeks and place them into a cast. This has resulted in early discharge from the hospital in all patients. For these reasons we believe that fascia lata is a useful adjunct in the armamentarium of materials used to reapproximate the pubis.

Bladder Exstrophy↗
PubMed1997

Treatment of patients with bladder exstrophy or incontinent epispadias. A long-term follow-up.

OBJECTIVE: To determine the late outcome concerning urinary continence, late complications, sexuality, and fertility in patients with the exstrophy-epispadias complex. METHODS: Until July 1994, 115 patients underwent surgical treatment at our institution. The mean follow-up period in 102 patients is 16.7 years. Urinary diversion was performed in 88 patients, a modified Young-Dees procedure in 8, a sling plasty in 3, and genital reconstruction alone in 3 patients. RESULTS: The present continence rates are 96% for rectal reservoirs, 97% for Mainz pouch 1, and 67% for the modified Young-Dees procedure. The upper tracts have remained stable, and no bowel neoplasms have developed. 16 of 17 women > or = 18 years of age are satisfied with the cosmetic result after genital reconstruction. All adults engage in sexual intercourse; 5 women have delivered 7 children by cesarean section. 30 of 32 male adults are satisfied with the cosmetic result of the reconstructed external genitalia. Penile deviation was present in 11, distressing in 2 patients. After genital reconstruction 9 developed epididymitis, necessitating 2 orchiectomies and 3 vasectomies. No patient with reconstruction of the external genitalia can ejaculate normally or has fathered children, whereas the ejaculation was normal in 3 who did not undergo genital reconstruction. Furthermore, 2 of the 5 have fathered 4 children. CONCLUSIONS: Rectal reservoirs represent our urinary diversion of choice. After failed reconstruction/insufficient anal sphincter, a Mainz pouch I is constructed. The cosmetic results achieved by genital reconstruction are satisfactory. In women, antefixation of the uterus should be performed before or together with an introitus plasty to prevent uterine prolapse. In men, however, surgery is performed at the expense of fertility.

Adult↗
PubMed2005

The failed complete repair of bladder exstrophy: insights and outcomes.

PURPOSE: We describe the complications of complete repair and their management. MATERIALS AND METHODS: A total of 19 patients were referred after failed complete repair. Total dehiscence occurred in 6 males, major bladder prolapse in 3, minor prolapse in 3, pubic separation in 1, impassable stricture in 1, and total hemiglans and corporal loss in 2. Overall, partial glans loss was seen in 7 patients, urethral loss in 5 and penile skin loss in 3. One female had complete dehiscence and 1 had major prolapse, both losing the urethrovaginal septum. One female had an impassable stricture. RESULTS: Six males with dehiscence underwent re-closure with osteotomy. Urethral replacement was performed with full thickness skin graft (FTSG) in 3 and with buccal mucosa in 3. Five patients underwent a modified Cantwell-Ransley (C-R) epispadias repair after placement of skin expanders, and 1 awaits repair. The 3 patients with major prolapse underwent re-closure with osteotomy. A urethral buccal graft was used in 1 patient, FTSG was used in 2 at a later operation and all 3 underwent C-R epispadias repair. Of the 3 patients with minor prolapse 2 underwent re-closure with osteotomy using urethral buccal graft or FTSG followed later with a C-R repair. The final patient with minor prolapse underwent re-closure with osteotomy and C-R repair after testosterone stimulation. One patient with pubic separation and urethral and skin loss underwent re-closure with osteotomy, C-R repair after skin expanders and later bladder neck repair. In 1 case a ureteral graft replaced a posterior urethral stricture. Of the 2 patients with hemiglans and corporal loss 1 underwent penile torsion repair and later hypospadias repair, while the other is being observed. Two females underwent re-closure with osteotomy and urethral replacement with tubularized bladder. The case of stricture was managed endoscopically. CONCLUSIONS: Complications of complete repair are similar to those of other repairs but more serious if soft tissue loss occurs. Because of these increased risks, this procedure and its formidable complications are best managed by experienced exstrophy surgeons.

Bladder Exstrophy↗