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Use of cadaveric fascia lata for closure of bladder exstrophy with diplomyelia.

We describe a technique for the repair of bladder exstrophy using cadaveric fascia lata. The graft is folded in a four-layer configuration, passed through the obturator foramina, and then wrapped and anchored in front of the pubis. The resultant ring-shaped graft is useful when complex deformities preclude proper apposition of the pelvis anteriorly.

Abnormalities, Multiple↗

Bladder exstrophy: psychological impact during childhood.

PURPOSE: We describe the impact of bladder exstrophy on the behavior, self-esteem and quality of life of children as well as on the parents, and analyze the need for psychological intervention. MATERIALS AND METHODS: All 7 boys and 8 girls 3 to 18 years old (median age 11) under treatment at a tertiary pediatric surgery clinic were included in our followup study. Medical and psychological evaluations were performed. Behavior was assessed using the semistructured Höök-Cederblad Child Behavior Interview and the Child Behavior Checklist questionnaire. Self-esteem was assessed by the self-rating I Think I Am questionnaire. Children and parents were interviewed separately. Quality of life was estimated using the Multiattribute Health Status Mark II classification system. RESULTS: After repeat operations and hospitalization 10 children were dry, although 9 required catheterization. Four children had some behavioral problems, which were manifest in 2. All but 1 male adolescent had good or very good self-esteem. Quality of life was decreased in most cases due to limited self-care, although emotional problems were few. All mothers had experienced the birth as a traumatic event and 5 parents had had psychiatric symptoms. CONCLUSIONS: Self-esteem may be maintained despite multiple operations, urinary leakage and deviant genitalia but the abnormality had a great impact on children and on the lives of the families. Parents and children required individual intervention from a multidisciplinary team during different stages of childhood.

Adolescent↗

Factors responsible for successful primary closure in bladder exstrophy.

To assess the important factors for successful primary closure in staged reconstruction of bladder exstrophy, 25 patients (18 males, 7 females) underwent primary bladder closure during the years 1993-1997. Twenty-one were more than 72 h old; all of these underwent bilateral posterior iliac osteotomies followed by primary bladder closure during the same anesthetic. Bladder closure was done in a double layer. The ureteric catheters were removed after 2 weeks and the bladder catheter after 3.5-4 weeks. Only 1 patient had a bladder dehiscence on the 10th postoperative day due to infection; 3 had partial wound dehiscences but no bladder dehiscence. One had a partial bladder prolapse. The osteotomies needed no drainage, and no complications occurred. One patient needed a urinary diversion 3 years after surgery as the bladder capacity did not increase. Eleven important factors play a pivotal role for successful primary bladder closure: (1) Proper patient selection; (2) A staged approach; (3) Anterior approximation of the pubic bones with placement of the bladder and urethra in the true pelvis; (4) Posterior bilateral iliac osteotomies in all indicated cases; (5) Double-layered closure of the bladder; (6) Two weeks' proper ureteric catheter drainage; (7) Prevention of infection; (8) Prolonged and proper postoperative immobilization; (9) Prompt treatment of bladder prolapse; (10) Prevention of abdominal distension postoperatively; and (11) Ruling out bladder-outlet obstruction before removing the bladder catheter.

Bladder Exstrophy↗

Bladder growth and development after complete primary repair of bladder exstrophy in the newborn with comparison to staged approach.

PURPOSE: We assessed bladder growth and dynamics following complete primary repair of bladder exstrophy (CPRE) compared to the staged approach. MATERIALS AND METHODS: We reviewed the records of 16 boys and 7 girls who underwent CPRE within 3 days of life from 1996 to 2004 and compared them to the records of 8 boys and 6 girls treated with a staged repair from 1979 to 1996. Screening methods included voiding cystourethrogram, radionuclide cystogram and urodynamic study. We estimated growth curves for bladder capacity following repair in each group, and compared percent predicted bladder capacity (PPBC), compliance and detrusor overactivity between the CPRE and staged repair groups following bladder neck reconstruction. RESULTS: Bladder capacity in the staged repair group was 69.8 ml (95% CI 46.7-104.4) immediately after bladder neck reconstruction and increased by 15.0% per year thereafter (95% CI 6.2-24.5, p = 0.002). In the CPRE group bladder capacity was 29.0 ml (95% CI 21.3-39.5) initially and increased by 28.9% per year thereafter (95% CI 17.4-41.5, p <0.001). PPBC started at 45.6% (95% CI 35.7-55.5) and increased 1.2% per year (95% CI -1.1-3.5, p = 0.29) following repair for all genders and surgery groups. Compliance was 124.4% (95% CI 22.6-310.7, p = 0.01) greater in the CPRE group at all times following repair. Detrusor overactivity was present in 0 of 19 patients in the CPRE group and 6 of 13 (46%) in the staged group (exact p = 0.002). CONCLUSIONS: Within the CPRE group bladder stability was universal, and sphincter electromyography was normal suggesting no neuromuscular compromise of the pelvic floor. At early followup, our results suggest that PPBC is equivalent irrespective of gender or management. Further objective evaluation is needed in both groups.

Bladder Exstrophy↗

Interim report in humans of a previously described technique in an animal model: closure of bladder exstrophy with rectus abdominis muscle flap-II.

The results of a new bladder closure and augmentation technique in children born with bladder exstrophy are reported. The technique was performed in 6 children, including 4 who had, in addition to a small, inelastic, noncompliant bladder, squamous epithelial metaplasia and polypoid transformation. In the remaining 2 patients this technique was used after failed primary closures. A full thickness left rectus abdominis island flap containing skin, fascia, muscle and peritoneal layers is prepared with an intact neurovascular pedicle from the inferior epigastric artery. This flap is rotated to cover the bladder defect and aid in augmentation. The inner layer formed by peritoneum is sutured to the edges of the bladder defect. Postoperative endoscopic and histopathological investigations revealed the inner peritoneal layer of the flap to be completely covered by transitional bladder epithelium. No major surgical complications occurred in these 6 cases. Considering the advantages of the technique from this limited experience, evidence suggests that there is no need for a major gastrointestinal operation for bladder augmentation, since an acceptable bladder capacity was available. There was no mucus production from the inner layer of the flap, and metabolic and electrolyte disturbances were reduced.

Bladder Exstrophy↗

Reconstruction of umbilicus in bladder exstrophy.

A surgical method for reconstruction of the umbilicus in patients with bladder exstrophy is described. The technique is based on the surgical principle of eventual tubularization of a skin strip buried in the subcutaneous space.

Adolescent↗

[Continent enterocystoplasty in urinary bladder exstrophy].

During the last two years from 1992 to February 1994 5 patients (2 girls and 3 boys with median age 7 years) underwent construction of continent large-capacity low pressure reservoir for failed bladder exstrophy reconstruction. Small bladder with inadequate capacity, decreased detrusor compliance and urinary incontinence were the indication for enterocystoplasty. The pouches were reconstructed from detubularized, reconfigured bowel using incorporated bladder remnant. Bowel segments included ileocecum in 4 patients and ascendig colon in one. Reconstruction of the vesical neck was accomplished by the Young-Dees-Leadbetter procedure in 4 patients. Two children underwent reimplantation of ureters into the bladder and three ureters of two patients were reimplanted by the Goodwin technique into the colon. The Mitrofanoff continence mechanism using appendix was applied in 3 patients. Preoperation bladder capacities were 12 up to 100 mL and after enterocystoplasties improved to mean 300 mL and maximum 550 mL. Four patients are in complete urinary retention and are managed by clean intermittent catheterisation every 3 to 4 hours. One patient voids spontaneously with little residual urine by abdominal straining and pelvic muscle relaxation alone. Short-term follow-up (3 months to 2 years) showed stable renal function in all patients. In 2 patients with preoperative hydronephrosis caliectasis decreased. This series supports the efficacy of continent enterocystoplasty as an alternative procedure to previous forms of urinary diversion in the management of failed exstrophy reconstruction.

Bladder Exstrophy↗

Management of bladder exstrophy and incontinent epispadias: 25 years of experience with urinary diversion.

OBJECTIVES: Achieving complete urinary continence with preservation of the upper urinary tract in the exstrophy-epispadias complex must be the primary aim. To determine the optimal surgical approach, we reviewed the records of patients treated at our institution. METHODS: During the last 26 years, 95 patients with bladder exstrophy and 20 with incontinent epispadias were operated upon at our department. For this retrospective study a total of 102 patients could be interviewed. Mean followup after the first operation was 16.7 years. Of the 102 patients, in 43 primary treatment was performed at our institution (urinary diversion n = 39, modified Young Dees n = l, sling plasty n = 3). A further 59 patients were referred to our institution for secondary treatment, 34 of whom after primary bladder closure and/or bladder neck reconstruction (urinary diversion n = 27, modified Young Dees n = 7). RESULTS AND CONCLUSION: Of the 8 patients with modified Young Dees, 5 required conversion to a Mainz Pouch I due to obstruction of the reconstructed bladder neck or incontinence. Continence rates are 96% for the rectal reservoirs, 97% for the Mainz Pouch I and 67% for the modified Young Dees procedure. Presently, none of the 102 patients has deterioration of the upper urinary tract or has renal insufficiency; none has developed severe metabolic complications or bowel neoplasms. The physical, social and psychological development of the patients treated at our institution appears to be comparable to that of the general population. All children over 6 years of age attend elementary school, most of the adults are, well-educated, only three are unemployed and one lives in a therapeutic center as a result of multiple physical problems. Rectal reservoirs are the urinary diversion of choice at our institution in patients with bladder exstrophy or incontinent epispadias. When the upper urinary tract has deteriorated, a colon conduit is created with the option of conversion to a continent form of diversion as soon as renal and ureteral functions have recovered. In patients with failed urinary tract reconstruction/insufficient anal sphincter function, we prefer the Mainz Pouch 1.

Adolescent↗

Gastrocystoplasty in the treatment of bladder exstrophy.

A wedge-shaped segment of stomach based on the right gastroepiploic artery was used for bladder augmentation in 11 patients. Initial diagnosis in these 11 patients was bladder exstrophy. Indications for the use of stomach in bladder reconstruction were important bilateral upper tract deterioration in 10 patients, dederivation in 1. A continent appendicostomy (Mitrofanoff) has been performed in all patients. In post-operative follow-up (average 24 months), all patients have stable upper tract X-rays and stable or improved renal function. All patients require intermittent clean catheterization, 8 are totally continent, 2 are partially continent, 1 patient is still incontinent. No serious digestive problem was encountered. The increase in bladder capacity was 300 to 500% after 6 months. Mucus production is reduced relative to other intestinal segments and the patients require no bladder irrigation. The authors recommend the use of stomach for urinary tract reconstruction in compromised patients.

Adolescent↗

The use of human chorionic gonadotropin (HCG) for penile reconstruction in bladder exstrophy and total epispadias patients.

OBJECTIVES: The effect of intramuscular human chorionic gonadotropin (HCG) administration on penile enlargement before genital surgery, its influence on penile skin histology and testicular descent were investigated. METHODS: We examined 45 male patients (median age, 8 months; range 3-28) with total epispadias and classic bladder exstrophy, combined with cryptorchidism. 30 patients were administered 250-500 IU HCG intramuscularly 2 times per week for 3 weeks before reconstructive surgery. Skin biopsies were obtained for human epidermal growth factor (EGF) and human epidermal growth factor receptor (Her2/neu) determination. Skin specimens of the prepuce of 18 circumcised patients were used as controls. Post treatment testicle position was evaluated. RESULTS: HCG caused a mean increase in penile length of 1.8 cm (p < 0.01) and in circumference of 1.2 cm (p < 0.05) as well as improved local vascularity in all patients. Compared to the controls, the penile skin of exstrophy/epispadias patients showed a significant decrease in the average amount per field of EGF and Her2/neu positive material (controls 81% [mean 79, SE 2.3] vs. 31% [mean 28, SE 3.6; p < 0.001]). Treatment with HCG led to an increase in average EGF and Her2/neu positive material by 10% (mean 8, SE 2; p < 0.05). The potential side effects of HCG treatment were monitored 3 to 6 months postoperatively. Basal testosterone and LH levels were obtained in patients before and during therapy and postoperatively. Testicular descent was achieved in 21 patients (70%). No significant side effects or complications were encountered in any of our patients. CONCLUSION: Mean EGF and Her2/neu values are decreased in the penile skin of exstrophy/epispadias patients. The use of preoperative HCG administration leads to an increase in EGF and Her2/neu values and significantly contributes to successful reconstruction in these patients, especially in cases with a paucity of penile skin and in patients who have undergone previous repairs. Temporary penile stimulation by HCG in patients with bladder exstrophy combined with cryptorchidism allows the penile operation to be carried out earlier and contributes to testicular descent while demonstrating negligible side effects.

Bladder Exstrophy↗

Continence in bladder exstrophy: determinants of success.

We evaluated 19 female and 18 male patients with bladder exstrophy, who had completed staged reconstruction, had required no further surgery and underwent urodynamic studies. Of the male population 61% were continent based on a significantly higher urethral continence length (25.8 +/- 6.4 mm., mean plus or minus standard error) and a higher urethral closing pressure (69.4 +/- 5.8 cm. water) compared to the incontinent male population (11.4 +/- 3.1 mm. and 43.4 +/- 4.6 cm. water). No significant difference was noted in the bladder capacity of these 2 groups. Of the female population 57.9% were continent. They also demonstrated a higher urethral continence length (21.1 +/- 4.4 mm.) and a higher urethral closing pressure (62.7 +/- 10.2 cm. water) compared to the incontinent female subjects (8.4 +/- 2.5 mm. and 32.7 +/- 6.9 cm. water). Moreover, those who were continent had a significantly higher bladder capacity (201.2 +/- 39.5 ml.) compared to those who were incontinent (84.3 +/- 23.6 ml.). These findings support a multifactorial mechanism in achieving continence but they suggest that of all the factors urethral length may be the most important.

Bladder Exstrophy↗

[Bladder exstrophy. Treatment results over 27 years].

Between 1960 and 1988 47 patients with bladder exstrophy were attended. 7 various operative procedures were applied. 3 patients died postoperatively, 37 of the 42 patients would be examined after 1 11/12 to 27 years. All patients with ureterosigmoidostomy exhibited electrolyte disturbance and metabolic acidosis. Patients with sigmoid conduit, rectal bladder and bladder reconstruction had unremarkable findings. The uretero-enterostomy anastomotic region was endoscopically essentially unremarkable. The control of continence was from satisfactory to good, and there was not absolute incontinence. X-ray investigation revealed both distinct and indistinct changes. The psychic and social stress was more serious in childhood than in adulthood.

Bladder Exstrophy↗