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Results of surgical treatment in children with bladder exstrophy.

A series of 42 children (30 boys and 12 girls) underwent surgery for bladder exstrophy between 1972 and 1989. Primary bladder closure was performed in 11 patients and was successful in 6. Four of these children are about 2 years old and so it is not yet possible to assess their continence. Ureterosigmoidostomy was performed in 35 children, one of whom was converted to cutaneous ureterostomy. Follow-up ranges from 2 to 20 years (average 9 years 3 months) and 50% of the patients are symptom-free. The most frequent problems were acidosis and urinary tract dilatation. Other complications, such as hypokalaemia and pyelonephritis, were seldom seen. Although ureterosigmoidostomy has some disadvantages, our patients have adapted well and lead a normal life.

Adolescent↗

Surgical management of incontinence in bladder exstrophy.

Between 1978 and 1990, 86 patients with previously closed classical bladder exstrophy and 10 patients seeking undiversion have presented for continence management and have undergone selective reconstruction designed for voiding and/or intermittent urethral catheterisation. The reconstruction in these 96 patients has been reviewed. Eight of the 10 patients undergoing undiversion achieved a satisfactory state of continence but 4 required Mitrofanoff procedures to enable catheterisation. Of the other 86 patients, 2 reached a satisfactory state of continence without further surgery; 79 underwent bladder neck surgery for continence either without augmentation (n = 32) or with augmentation (n = 47). Twenty of the 32 patients who were treated by bladder neck reconstruction alone were later found to require augmentation. Five patients had very early augmentation either to facilitate neonatal closure or on account of severe upper tract dilatation. Of these, 1 became continent without further surgery and 4 demonstrated the need for bladder neck reconstruction. Thus 12 children achieved successful continence (n = 6) or are evolving satisfactorily with potential success (n = 6) as a result of bladder neck reconstruction. Of the 71 patients requiring bladder neck reconstruction and augmentation, 68 have completed their surgery. The current status of these patients is: satisfactory in 57 (80%) (42 void/urethral clean intermittent catheterisation (CIC), 7 waiting to learn CIC, 5 Mitrofanoff, 3 artificial urinary sphincter (AUS]. Of the remaining 11 patients (20%), 8 are unsatisfactory to varying degrees and the status of the other 3 is unknown.

Bladder Exstrophy↗

Variations in the bladder exstrophy complex associated with large omphalocele.

Two newborns with a large omphalocele associated with the bladder exstrophy complex are presented. In 1 case the omphalocele was combined with a musculoskeletal defect and imperforate anus with an anovulvar fistula. The other patient had a musculoskeletal deformity and a duplicated bladder with exstrophy. Surgical treatment was successful in both cases.

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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↗

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.

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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↗

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↗

The 3-loop technique: a reliable technique for anterior pubic fixation in bladder exstrophy.

PURPOSE: In exstrophic anomalies the ultimate urological outcome largely depends on successful initial closure of the lower urinary tract and soft tissues. We believe that secure anterior pubic fixation is crucial for ensuring successful closure. After being dissatisfied with other methods of anterior pubic fixation we introduced the 3-loop method. The 3-loop technique and our experience with it are described. MATERIALS AND METHODS: In 2 years 7 consecutive cases of bladder exstrophy were closed using the 3-loop technique. Patient age at closure ranged from newborn to 9 years. RESULTS: In all 7 patients closure was successful and there was no cutting through of the wires, bony erosion, or erosion into the reconstructed bladder neck or urethra. The duration of postoperative traction was only 2 weeks. CONCLUSIONS: The 3-loop method is useful and reliable for secure anterior pubic fixation of the pubes in bladder exstrophy patients and it contributes positively to the ultimate urological outcome.

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The obstetric and gynaecological complications of bladder exstrophy and epispadias.

A study has been made of 16 girls (14 with bladder exstrophy and 2 with epispadias) treated in the United Birmingham Hospitals since 1946 and surviving to puberty. Eight of these are married, of whom 5 have been delivered of 8 children. Ten have required some form of gynaecological surgery, with uterine prolapse as the most troublesome lesion.

Adolescent↗

Cantwell-Ransley epispadias repair in male epispadias and bladder exstrophy.

A total of 16 boys (8 with primary epispadias and 8 with bladder exstrophy) underwent epispadias reconstruction as a 1-stage procedure. Reverse meatal advancement of MAGPI, ventral transposition of the neourethra and chordee correction by corporeal rotation or "cavernocavernostomy" were performed as primary (13 boys) or secondary/salvage (3 boys) procedures. At a mean followup of 27 months all children had a horizontal or downward angled penis while standing. Catheterization in 10 children revealed an easily negotiable neourethral channel. Minor revision surgery was necessary in the area of previous paraexstrophy flaps in 2 cases, and 1 child required neourethral tapering and proximal anastomotic revision. The Cantwell-Ransley epispadias repair produces a good functional and cosmetic result.

Adolescent↗

Bladder exstrophy. Primary reconstruction with human dura mater.

A new surgical technique for primary reconstruction of bladder exstrophy is described. Human cranial dura mater is used as an alloplastic free graft to replace the missing anterior bladder wall. The technique has been successfully applied to 8 patients and the early achievement of good bladder capacity seems to improve urinary continence.

Bladder Exstrophy↗

The staged approach to bladder exstrophy closure and the role of osteotomies.

Since the 1970's, the staged reconstruction of bladder exstrophy has yielded consistent surgical success. The Johns Hopkins Hospital approach begins with early pelvic ring approximation with abdominal wall, bladder, and posterior urethral closure. Within the first 72 hours of life, the malleable pelvis can sometimes be approximated without osteotomies. Beyond this age, the author's prefer a combined vertical iliac and horizontal innominate osteotomy. Second, we typically perform the epispadias closure at 1 year of age. A modified Cantwell-Ransley technique is performed, usually yielding an increase in bladder capacity and very satisfactory results. In the last phase, the modified Young-Dees-Leadbetter continence procedure along with transtrigonal/cephalotrigonal ureteroneocystostomies are performed when the urethra is catheterizable, the bladder capacity is 60 cc or greater, and the child will participate in a postoperative voiding program (typically 4-5 years of age). This applied approach usually results in a continent, voiding patient with pleasing external genitalia and preserved renal function.

Bladder Exstrophy↗