Further progress with reconstruction of the exstrophied bladder.
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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.
A new, staged approach to reconstruction of bladder exstrophy utilizing four surgical procedures is presented. This approach presents all the complications usually seen in functional reconstruction of this anomaly.
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.
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.
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.
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.
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.
Satisfactory results were obtained in 25 patients with bladder exstrophy treated by the Heitz-Boyer-Hovelacque operation.
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.
A case of adult exstrophy of the bladder was managed with urinary diversion, excision of the bladder, and closure of anterior abdominal defect, using a rectus abdominis myocutaneous island flap. This procedure is recommended as sound in cases in which reconstruction of the bladder is not feasible.
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 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.
We reviewed 103 patients with exstrophy of the bladder. Followup was more than 15 years in 51 patients. Initial management consisted of primary bladder closure in 32 patients and urinary diversion in 71. Urinary continence, renal function, urinary tract infections and development of malignant lesions were evaluated. Factors leading to success or failure were analyzed. Although the highest continence rate (83 per cent) was achieved in 40 patients with ureterosigmoidostomy, renal functional deterioration was highest in this group, with 70 per cent of the evaluable renal units being abnormal. Furthermore, 10 per cent of this group died of renal failure and 23 per cent lost 1 kidney each. In 26 patients with an ileal conduit 69 per cent of the renal units evaluated were abnormal. Only 1 patient died of renal failure but 27 per cent lost 1 kidney each. Of 32 patients with primary bladder closure 31 had preservation of renal function. Twelve of 18 patients (67 per cent) in whom vesical neck reconstruction had been completed had total urinary continence and 3 (17 per cent) had partial continence. The incidence of significant urinary tract infections was highest in the ureterosigmoidostomy group (63 per cent) and next to the highest in the ileal conduit group (48 per cent). Malignant lesions developed in 8 patients (8 per cent).
During the last 8 years 20 patients were seen for secondary or repeat closure for exstrophy of the bladder following dehiscence after initial closure. Various factors that may have contributed to the initial problems have been investigated. Wound infection (42 per cent) and bladder prolapse (46 per cent) were the major etiologies for initial failures. In 6 patients (30 per cent) colon conduits were required for bladder augmentation or colocolostomy, which emphasized the importance of achieving a good result at initial closure. Our successful protocol (only 5 per cent reclosure rate for 60 closures) is outlined.
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.
Of 158 patients who underwent ureterosigmoidostomy for exstrophy of the bladder from 1925 to 1970, 52 were available for followup by telephone interview. Of these patients 34 still had a functioning ureterosigmoidostomy (30 had complete continence day and night) but 18 required diversion by other methods. All of the patients were believed to be socially well adjusted and leading productive lives. Of the patients surviving 15 or more years after ureterosigmoidostomy 11 per cent had colon cancer. Most of the remaining patients were unaware of the risk of adenocarcinoma engendered by the ureterosigmoidostomy. Patients undergoing ureterosigmoidostomy must be fully informed of all health risks and adequate surveillance programs should be arranged. In selected patients ureterosigmoidostomy remains a useful form of urinary diversion, with excellent continence and good social adaptation.
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.