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[Treatment of bladder exstrophy. Reconstruction or urinary diversion].

Bladder exstrophy is seen in 1 of 30,000-40,000 live births, and is seldom treated in many urological departments. Treatment options for children with exstrophy are upper urinary tract diversion or reconstruction of the bladder and plastic surgery of the bladder neck to gain urinary continence by the age of 4-7 years. Historical reviews report continence rates of 10-30% after a staged approach with primary reconstruction and secondary bladder neck repair. This formerly meant upper urinary tract diversion as a third stage in 70-90%. Multiple operative procedures could be avoided when primary diversion was done. The best results were reported following antirefluxive implantation of ureters into the sigmoid colon (ureterosigmoidostomy). In boys, the base of the bladder was removed, leaving a small residual bladder which together with the reconstructed epispadias served as a "seminal tract". Total removal of the bladder was performed in girls. Long-term follow up of upper urinary tract diversion showed disturbances of serum electrolytes, urinary tract infections and stone formation, and after ureterosigmoidostomies an increased rate of colon carcinomas was documented. These results led to renewed interest in reconstruction. The technique of bladder neck reconstruction was changed, resulting in a higher rate of late urinary continence: augmentation cystoplasties, clean intermittent catheterization and the artificial sphincter help to achieve a continence rate of more than 90%. This goal was reached only after multiple operations and without knowledge of the long-term sequelae of augmentation cystoplasties. The years to come will show whether new concepts of ureterosigmoidotomies, such as the sigma-rectum pouch, will be preferable, or a late urinary tract diversion after failed reconstruction. Most centers are now agreed that primary reconstruction of bladder exstrophy should be attempted in the newborn child.

Abdominal Muscles

Long-term results of ureterosigmoidostomy in children with bladder exstrophy.

The records of 87 children with bladder exstrophy seen between 1912 and 1935 are reviewed. We believe that ureterosigmoidostomy with cystectomy can provide a satisfactory solution to the problem of bladder exstrophy. Furthermore, ureterosigmoidostomy appears suitable from the standpoint of preservation of upper tracts and provides a socially acceptable solution of the question of disposal of the diverted urine.

Adolescent

Pregnancy in women with bladder exstrophy.

Little is known about pregnancies in women with bladder exstrophy. Of 28 women with bladder exstrophy who were evaluated 7 became pregnant and 5 delivered successfully. Problems seen during pregnancy, the long-term effects and the need for early, informed counseling by the urologist are discussed.

Adolescent

[Invasive adenocarcinoma with epidermoid carcinoma on the site of bladder exstrophy. Histochemical and immunocytochemical study].

To date, one hundred and ten cases of malignant neoplasms arising from untreated bladder exstrophy have been reported. We describe another case of uncorrected bladder exstrophy with invasive adenocarcinoma and in situ squamous carcinoma discovered in a 51 year old woman. Postoperative radiotherapy was performed after cystectomy with nephroureterectomy and hysterectomy. The neoplastic samples were investigated by mucin histochemistry and immunocytochemistry. The epithelium lining the tumor villi and the surrounding mucosae was colonic with mucin histochemistry and reacted to anti KL1, EMA and ACE antibodies. These features were those of colonic and vesical adenocarcinomas. Although early vesical reconstruction is the best oncologic prevention, patients with bladder exstrophy run the risk of sigmoid adenocarcinoma after cystectomy and urinary diversion including ureterosigmoidostomy.

Adenocarcinoma

Gas cystometry in cases of continent bladder exstrophy.

Cystometry in 21 cases of closed continent bladder exstrophy revealed that 14 patients had a normal reflexic bladder. A simultaneous anal plug electromyogram showed an appropriate response in all patients when correlated with a cystometrogram.

Adolescent

Ureterosigmoidostomy: an outdated approach to bladder exstrophy?

Long-term results among 46 children with ureterosigmoidostomy are presented. The indication for ureterosigmoidostomy had been bladder exstrophy in 40 patients, incontinent epispadias in 5 and neurogenic bladder dysfunction in 1. Of the 40 patients with bladder exstrophy 8 had undergone ureterosigmoidostomy after failure of other types of urinary tract reconstruction (6 had upper tract dilatation before ureterosigmoidostomy). Three patients with previously damaged upper urinary tracts required early postoperative conversion because of severely increasing kidney dilatation. Three other patients required conversion after a mean of 10 years to preserve kidney function. One patient died after 16 years of a cause not related to ureterosigmoidostomy. The remaining 39 patients were alive with a functioning ureterosigmoidostomy after a mean followup of 14.7 years. The daytime continence rate was 97.4% (38 of 39 patients) and the complete continence rate was 92.3% (36 of 39). Except for 1 tubular adenoma that was removed successfully during routine colonoscopy, no bowel neoplasia has been observed. None of the 45 living patients has renal insufficiency.

Adolescent

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

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

Inguinal pathology and its association with classical bladder exstrophy.

One hundred thirty-four cases of classical bladder exstrophy, managed at our institution, were reviewed. Fifty-six percent of the boys and 15% of the girls developed inguinal hernias over an average follow-up time-span of 10 years. Thirty-one percent of the patients with hernias underwent repair at the time of initial bladder closure. Forty-six percent of the patients who developed a hernia were diagnosed during the first year following their initial procedure. More than 50% of the individuals in the latter category presented with an incarcerated hernia, and required emergent management. Boys managed by staged reconstruction had a statistically significant risk of developing an inguinal hernia (P less than .001) compared with boys undergoing primary cystectomy and diversion. We believe the increased incidence of herniation with this congenital anomaly is secondary to a lack of obliquity of the inguinal canal, due to pubic diastasis along with an increased elevation of intraabdominal pressure following initial closure of the abdominal wall and bladder plate. To decrease the attendant morbidity of incarcerated hernias in this population, we stress the need for careful physical examination of the inguinal region and spermatic cord prior to surgery, along with repair of the patent processus vaginalis at the time of initial repair.

Bladder Exstrophy

[Genital prognosis of boys with bladder exstrophy or epispadias with incontinence. Apropos of 14 cases].

Bladder exstrophy and epispadias with incontinence are associated with urinary tract and genital anomalies. The genital and sexual aspects were studied in 14 adolescent or adult males (12 exstrophies and 2 epispadias). The appearance of the penis was satisfactory in fifty percent of cases. Erections were always preserved but normal ejaculations were present in only one half of cases. Fertility potential was reduced. The improvement of surgical technique, and especially penile lengthening, has greatly improved sexual intercourse for these patients.

Adolescent

[Surgical treatment of bladder exstrophy by functional reconstruction by stages (author's transl)].

A brief summary is given of the clinical statistical findings in 37 patients with exstrophy of the bladder, most of the whom have been reported in a previous paper. Four cases are presented for whom the method of operating by stages was applied. They have now been followed up for 17, 10, 8 and 6 years respectively and a very satisfactory functional result was obtained. The experience gained from the use of such a method clearly indicates that treatment should be carried out, whenever possible, by a one-stage operation, or by two stages within a short period of time, and at an early age. Two cases operated upon by this latter method of total primary functional reconstruction, with very encouraging morphological results, are reported.

Age Factors

[Genital prognosis of girls with bladder exstrophy or epispadias].

The authors report ten cases of women with previous exstrophy of the bladder or epispadias with incontinence followed up to child-bearing age. Six of the ten females had urinary diversion and four retained their bladder and were continent. Three key aspects are considered: physical appearance of the external genitalia which was satisfactory in all cases; preservation of sexual function, known in only four of the ten cases and which was reported by these patients to be satisfactory, and finally, the ability to bear children. Three patients became pregnant resulting in four normal births (one patient had twins). Cesarean sections were recommended in patients with pregnancies at term. Cesarean should be performed systematically in patients with bladder reconstruction.

Adolescent

Bladder exstrophy and anterior pelvic osteotomy.

Between August 1988 and December 1991, 36 children with bladder exstrophy underwent surgery for primary bladder reconstruction. Each child was either untreated or had already been treated unsuccessfully. The operative technique involved bilateral osteotomy of the superior ramus of the pubic bone. In infants the cartilaginous ischiopubic junction, acting as an articulation, allowed symphyseal approximation, while in older children this was achieved by fracture of the inferior ramus of the pubic bone. The bladder was either closed or, in most cases, the exstrophic bladder plate was inserted deep into the pelvis, allowing subsequent epithelialisation of the bladder and further formation and growth. Follow-up up for 3.5 years showed bladder capacities of 40 to 150 ml. Some patients underwent an additional augmentation enterocystoplasty. Primary bladder reconstruction remained uncompromised in 7 patients who developed moderate (and 1 complete) rediastasis of the pubic bones. All exstrophic bladders are reconstructible, particularly in older children.

Bladder Exstrophy

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