Long-Term Results of Ureterosigmoidostomy in Children With Bladder Exstrophy

Purpose

We evaluated long-term results of patients with bladder exstrophy who underwent ureterosigmoidostomy.

Materials and Methods

Of 4 women and 23 men monitored at our institution 16 (59 percent) underwent primary diversion by ureterosigmoidostomy, while 11 (41 percent) underwent primary bladder closure or an ileal conduit procedure before conversion to ureterosigmoidostomy. Average followup after ureterosigmoidostomy was 17 years.

Results

Significant upper urinary tract changes developed in 18 percent of the patients. Metabolic acidosis was well compensated in most patients but 2 had problems with urinary retention leading to hyperammonemia and acidosis. Of the 19 patients monitored with biennial colonoscopy benign polyps were removed in 4. Daytime continence was achieved in 92 percent of cases and nighttime continence in 58 percent.

Conclusions

Our experience with ureterosigmoidostomy in children with bladder exstrophy has been favorable through long-term followup. With proper imaging, metabolic surveillance, biennial colonoscopy and nonsteroidal anti-inflammatory drugs we offer ureterosigmoidostomy as a viable alternative for patients with small bladders.     

Ureterosigmoidostomy: Is it a Viable Procedure in the Age of Continent Urinary Diversion and Bladder Substitution?

Between January 1977 and June 1985, 63 patients requiring supravesical diversion underwent ureterosigmoidostomy with an antireflux technique. Of the patients 49 had bladder cancer and 14 had other conditions. Two patients died in the postoperative period. Postoperatively, all patients were instructed to empty the rectum frequently, and received bicarbonate and potassium supplementation.Median followup was 41 months (range 3 to 70). Renal function remained stable in 92 percent of the patients. Radiographic deterioration occurred in 23 percent of the renal units, which was severe in 7 percent. These results indicate that the short and intermediate followup results with ureterosigmoidostomy are comparable to those of an ileal conduit. The method has the added advantage of being a form of continent diversion. We believe that ureterosigmoidostomy remains a viable and convenient alternative in select patients with bladder cancer who are not suitable for other forms of continent diversion or bladder substitution.     

Ureterosigmoidostomy: an outdated approach to bladder exstrophy?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.     

Rectosigmoid pouch (Mainz Pouch II) in children

PURPOSE: Continent anal urinary diversion is a therapeutic option in bladder exstrophy. We report our long-term results with the rectosigmoid pouch (Mainz pouch II), a modification of the classic ureterosigmoidostomy. MATERIALS AND METHODS: A total of 38 children with a mean age of 5 years (range 0.5 to 17) underwent a Mainz pouch II procedure between 1991 and 2004. Most patients (33) had bladder exstrophy or incontinent epispadias. In 14 children (37%) urinary diversion was performed after failed primary reconstruction. In 6 children conversion was performed from an incontinent type of urinary diversion. Renal function, continence and metabolic changes were analyzed. A total of 35 children were followed for a mean of 112 months (range 5 to 147). RESULTS: All children were continent during the daytime but 3 (8.6%) suffered from nighttime incontinence requiring pads. With respect to the upper urinary tract, 6 children (15.8%) had development of pyelonephritis, mostly with stenosis of the ureterointestinal anastomosis. Reimplantation of the ureter was required in 10 of 69 RU (14.5%), of which 7 (10.1%) were due to ureterointestinal stenosis and 3 (4.3%) were due to reflux. Serum creatinine was within normal limits in all children. During followup acid-base balance was monitored, and early alkali supplementation was initiated in 24 of 35 children (69%) when the base excess was less than -2.5 mmol/l. One child had development of clinical acidosis requiring hospitalization. After followup of more than 10 years annual rectosigmoidoscopy was performed in 16 children/young adults without pathological findings. CONCLUSIONS: The Mainz pouch II procedure for children with genitourinary anomalies promises excellent continence rates. However, periodic followup studies are important to check the upper urinary tract and prevent metabolic acidosis. Due to the risk of malignancy at the ureterointestinal anastomosis, endoscopy should be performed annually beginning at postoperative year 10. The Mainz pouch II procedure is safe in the long term. Without stoma, appliance or catheterization this type of continent urinary diversion is specifically suitable for children.     

Long-term followup of 207 patients with bladder exstrophy: an evolution in treatment

Between 1945 and 1985 a total of 207 patients (male-to-female ratio 2:1) was treated at our institution for exstrophy of the bladder. Primary anatomical bladder closure was performed in 137 patients, of whom 42 (31 per cent) eventually required urinary diversion. Primary urinary diversion was the initial treatment in 70 patients, including ureterosigmoidostomy in 40, ileal conduit in 17, colon conduit in 11 and cutaneous ureterostomy in 2. Secondary urinary diversion was necessary in 35 patients and included continent diversion in 7. Primary anatomical bladder closure, done within 72 hours of birth and followed by staged reconstruction of the bladder neck, was the most successful surgical regimen for the treatment of bladder exstrophy. Acceptable urinary continence was achieved in 82 per cent of our patients with this approach. However, multiple procedures often were required to achieve continence.     

Complete primary repair of exstrophy.

PURPOSE: The surgical correction of bladder exstrophy to achieve continence with voiding remains a challenging problem for the urologist. Since 1989 we have performed complete primary repair for exstrophy based on the concept that the primary defect of bladder and cloacal exstrophy is anterior herniation. Thus, the bladder and urethra must be treated as a single unit to move them posteriorly into the pelvis. We present this technique. MATERIALS AND METHODS: From 1989 to 1997, 18 patients with bladder exstrophy and 6 with cloacal exstrophy underwent complete primary repair of exstrophy. This procedure was done on day 1 of life in 18 patients. Mean followup is 44 months (range 4 months to 8 years). RESULTS: At a median followup of 48 months 4 boys and 4 girls have volitional voiding after complete primary repair of bladder exstrophy, 21 patients have continent intervals and 2 boys void with continent intervals after complete primary repair of cloacal exstrophy. No patient has had a loss of renal function in this series. Postoperative complications included urethrocutaneous fistula formation in 2 cases. No patient had primary closure dehiscence. CONCLUSIONS: The rate of urinary continence achieved with complete primary repair compares favorably to that of staged repair for exstrophy. Complete primary repair also minimizes the number of surgical procedures required to achieve urinary continence and potentiates bladder neck function that permits bladder cycling in year 1 of life. The complication rates of these techniques are significantly lower than those reported in previous series of primary closure of exstrophy.     

Sixteen years' experience with the Heitz Boyer-Hovelacque procedure for exstrophy of the bladder

The various methods of managing bladder exstrophy are described. Bladder reconstruction and ureterosigmoidostomy are discussed and evaluated. The satisfactory results obtained with 21 patients treated by the Heitz Boyer-Hovelacque operation are reported and it is concluded that this is the procedure of choice when urinary diversion is needed for the exstrophic bladder.     

A 55-year followup of a patient with bilateral ureterosigmoidostomy.

We report on a patient with bilateral ureterosigmoidostomy who, despite many of the long-term complications associated with the procedure, was doing well 55 years after urinary diversion. This is one of the longest follow-ups reported in the literature. Despite the decreasing popularity of ureterosigmoidostomy, in well selected patients who desire a continent form of internal diversion it may still prove to be useful, as exemplified by our patient.     

Continent urinary reservoir in exstrophy/epispadias complex.

The outcome of 15 children and young adults who underwent reconstruction of continent urinary reservoirs (CUR) from January 1987 to 1990 is presented. Ten patients were male and 5 female with an age range of 3 to 20 years. There were 13 patients with bladder exstrophy and 2 with incontinent epispadias. In 8 cases the urinary diversion was performed for an inadequate bladder capacity following successful closure (3), failed attempted closure (3), female epispadias (1) and following successful urethroplasty in 1 case of male epispadias. A tiny fibrotic bladder plate unsuitable for attempted closure was the indication for diversion in 3 patients. Faecal and urinary incontinence following ureterosigmoidostomy (2 patients) and trigonosigmoidostomy (2) was the reason to consider re-diversion in 4 previously diverted patients. In 10 patients an Indiana pouch was performed. The Mitrofanoff procedure was used in 5 cases with caecum (2), sigmoid colon (2) or transverse colon (1) as the urinary reservoir. All patients are continent on clean intermittent catheterisation with stable renal function. Three patients developed large stones within the reservoir and needed open cystolithotomy. This series supports the efficacy of CUR as an alternative procedure to traditional forms of urinary diversion in the management of selected patients with exstrophy/epispadias complex.     

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.     

Neoplasia at the site of ureterosigmoidostomy.

An increased incidence of neoplasia at the site of ureterosigmoidostomy must be considered in patients being evaluated for or who have undergone this procedure. A case of mucinous adenocarcinoma 24 years following bilateral ureterosigmoidostomy is reported. This patient had bilateral hydroureteronephrosis, which led to left nephroureterectomy and right cutaneous ureterostomy. Mucinous adenocarcinoma was diagnosed only after the edematous, protruding and inflamed cutaneous ureterostomy stoma was biopsied. To our knowledge this is the first reported case of transfer of such a malignancy to the skin at the time of cutaneous ureterostomy. We recommend that ureterosigmoidostomy be performed only in patients with a limited life expectancy who agree to long-term followup. This followup should be frequent, complete and continue for the remainder of the patient's life. We consider the tendency for development of neoplasia at the ureterosigmoid anastomosis to be a relative contraindication to ureterosigmoidostomy. However, with proper patient selection and followup, ureterosigmoidostomy is still a remarkably satisfactory method of urinary diversion.     

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.     

Nonrefluxing colon conduit for temporary or permanent urinary diversion in children.

Colon conduit, with nonrefluxing ureterosigmoid anastomoses, was performed upon 21 children in the past 3 1/2 yr for the following indications: (1) previously unoperated exstrophy of the bladder; (2) exstrophy of the bladder with prior ileal conduit; (3) previously unoperated neurogenic bladder; (4) neurogenic bladder with prior ileal conduit; and (5) with anterior pelvic exenteration for sarcoma of the prostate. In some of these patients the conduit will be a permanent diversion; in others it will be anastomosed later to the colon, providing a staged method of ureterosigmoid urinary diversion. Nine patients have undergone the second stage of anstomosis of the conduit to the colon with satisfactory outcome. Colon conduit diversion is a more time-consuming procedure than ileal loop diversion. It can require 6-8 hr in a patients with a prior ileal diversion, particualarly if the ureters require tapering. Our experience with this procedure leads us to the following conclusions: (1) this is a better method than ileal conduit for permanent diversion; (2) it offers an alternative way for treating patients with bladder exstrophy; (3) it is useful in patients with pelvic cancer; and (4) it should be considered for those patients with ileal conduits who are not doing well, i.e., who have infection and renal deterioration.     

Current management of bladder exstrophy: a BAPS collective review from eight centres of 81 patients born between 1975 and 1985

The British Association of Paediatric Surgeons (BAPS) agreed to collect information on bladder exstrophy from its members. Data were obtained on 81 patients from eight centres. Twenty-one patients had other congenital abnormalities. Three died within the first year of life, and one was lost to follow-up in the early neonatal period--leaving 77 for further analysis. Forty patients had bladder closure without permanent urinary diversion. Ten were able to stay dry for two hours or longer, and 16 were constantly wet. Of the remaining 14, some were too young to assess, while others were dry for from 30 minutes to two hours. Thirty-seven had undergone diversions; fourteen had ureterosigmoidostomy, nine underwent ileal loop conduit, nine had colon loop conduit, and five had cutaneous ureterostomy. Seventeen of these diversions had been carried out before the patients were 2 years of age.     

Treatment of incontinence in children with bladder exstrophy after rectal urinary diversion: the anal sling procedure

PURPOSE: An original technique for reinforcement of the anal sphincter using a sling prosthesis with a flexible fixing point that prevents incontinence and rectal prolapse is presented. MATERIALS AND METHODS: Of 43 patients with exstrophy who have undergone urinary rectal diversion between 1988 and 1997 incontinence was observed in 9 boys and 5 girls. From January 1993 to December 1998 these children were treated with an original method, the anal sling procedure. RESULTS: There were no postoperative complications. Investigations revealed no urinary or fecal incontinence after a mean followup of 3.2 years (range 6 months to 61/2 years). CONCLUSIONS: A short and simple operation eliminates incontinence after rectal urinary diversion in patients with bladder exstrophy. It can be used as an alternative to the formation of an isolated urinary reservoir.     

Complications of the Heitz-Boyer urinary diversion: case report of late development of malignancy

We report on 3 patients with bladder exstrophy followed for more than 15 years after having undergone Heitz-Boyer urinary diversion. One patient had adenocarcinoma at the ureterocolonic anastomoses 16 years after diversion. To our knowledge this is the first reported case of a malignancy developing after a Heitz-Boyer procedure. The other 2 patients had significant problems with urinary incontinence and recurrent urinary tract infections. Initial success with this form of urinary diversion can be followed by significant long-term complications.     

Continent urinary diversion and the exstrophy-epispadias complex

PURPOSE: The use of continent urinary reservoirs has gained wide acceptance, particularly in urinary reconstruction in children with a small capacity or neuropathic bladder. When augmentation cystoplasty is combined with clean intermittent catheterization, patients are often able to achieve continence with low intravesical filling pressures and renal preservation. Often this approach requires fashioning a continent cutaneous stoma, which remains the most challenging aspect of continent urinary reservoirs. We analyzed our experience with continent diversion in patients with exstrophy-epispadias to determine complications and long-term results. MATERIALS AND METHODS: We performed a retrospective database review of 704 cases of exstrophy-epispadias. Medical records were then used to identify those patients who had undergone creation of a continent urinary reservoir. Charts were reviewed to determine initial diagnosis, augmentation technique, continence mechanism, age, preoperative and postoperative bladder capacity, continence status and complications. RESULTS: Of the 91 patients identified (68 male, 23 female) who had undergone continent urinary diversion classic bladder exstrophy was present in 80, cloacal exstrophy in 8, complete male epispadias in 2 and female epispadias in 1. The most common techniques for augmentation and continent diversion were ileocystoplasty (41 patients [45%]) and sigmoid cystoplasty (30 [33%]), respectively. Appendix was used in 67 patients (74%) and variants of the Mitrofanoff procedure using segments of tapered ileum or ureter were used to create a continent stoma in 10 (11%). Bladder neck transection was performed in 59 patients (65%). Mean age at augmentation and continent diversion was 8 years (range 2 to 25), with a mean preoperative bladder capacity of 77 cc (15 to 220). Mean followup was 6 years (range 6 months to 12 years). Of the 91 patients 85 (93%) were continent with clean intermittent catheterization per stoma. Of these 85 patients 13 required anticholinergics and alpha-agonists to achieve continence. Six patients (7%) were incontinent after the procedure. Analysis of bladder capacity measurements after augmentation and continent diversion revealed that mean postoperative volume and mean volume increase were 404 cc (range 250 to 640) and 524%, respectively. The most common complications were bladder stone formation (24 patients [26%]) and stomal stenosis (21 [23%]). Bladder stones recurred in 9 patients and stomal stenosis in 3. Other less common complications were vesicourethral fistula (3 patients) and a small bladder perforation (2). CONCLUSION: Augmentation and continent diversion procedures can increase the functional capacity of the small contracted noncompliant exstrophic bladder, and allow the vast majority of patients to achieve continence and preserve renal function. Bladder calculi and stomal stenosis pose the most significant long-term complications in these patients.     

Long-term followup of 103 patients with bladder exstrophy

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).     

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.     

Augmentation cystoplasty in the failed exstrophy reconstruction

Of the 148 patients with bladder exstrophy seen at this institution during the last 10 years 12 have ultimately required bladder augmentation. In 4 cases augmentation was performed for an inadequate bladder capacity, upper tract decompensation and dry interval of less than 1 hour after bladder neck reconstruction and epispadias repair. Likewise, 3 patients underwent augmentation for an inadequate bladder capacity and dry interval of less than 2 hours after bladder neck reconstruction and epispadias repair. Of these 7 patients 3 had undergone 2 previous bladder neck reconstructions, while 4 had undergone 1 prior repair. Five augmentations were performed for an inadequate bladder capacity before any type of continence procedure had been done. Nine patients underwent adjunctive procedures in addition to bladder augmentation, including a Young-Dees-Leadbetter procedure in 4, an artificial urinary sphincter in 3, transureteroureterostomy and psoas hitch in 1, and a Mitrofanoff procedure and bladder neck closure in 1. Of the 12 patients 11 are continent, although 9 require intermittent catheterization. There were no major complications. However, 1 artificial urinary sphincter was removed for erosion 2 years after placement. Augmentation cystoplasty has provided prolonged stability of the upper tracts and continence in these patients, and it has proved to be a successful alternative to urinary diversion in this select group of exstrophy failures.