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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.
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.
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.
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.
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.
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.
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.
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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.
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.
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.
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.
A report is given on three women with ureterointestinal anastomosis after having a bladder exstrophy operation. Two of them carried out their pregnancies and had spontaneous deliveries. The problems of management during pregnancy are discussed and the literature is reviewed.
The results of neonatal surgery for bladder exstrophy are not very satisfactory. A significant percentage of patients present in later childhood or adolescence for correction of their residual deformities. We have reconstructed 26 patients, correcting their entire urogenital and cosmetic deformity in a one-stage procedure. The results show that one-stage total reconstruction is possible and is preferable to serial correction of the various individual abnormalities. The principles of surgical reoperation in adolescence are the same as those now established for the primary correction in neonatal life.
Ten patients with different types of internal urinary diversion for bladder exstrophy were studied prospectively in order to assess metabolic abnormalities and morphological, histochemical and lectin binding changes in the colorectal mucosa. The histochemical and/or lectin binding changes which were found in the majority of patients were identical to those observed in premalignant and malignant conditions of the colon. In some cases they were detectable 3 years after the initial examination but were completely absent from the colorectal mucosa of normal subjects. Metabolic disturbances (metabolic acidosis, increased anion gap, hyperchloraemia) were observed in a substantial number of asymptomatic patients. These findings stress the need for regular endoscopic, histological and metabolic follow-up in these patients and for life-long treatment with bicarbonate or citrate.
Preservation of renal function is one of the major goals of staged reconstruction of bladder exstrophy. In 68 exstrophy patients who have completed staged reconstruction, 10 (14.7%) have developed renal scarring. A multiple factorial analysis of these patients showed the following factors to be statistically related to the development of renal scarring: (1) one or more febrile urinary tract infections prior to bladder neck reconstruction (P less than .001); (2) failure to utilize antibiotic prophylaxis following initial bladder closure (P less than .005); (3) elevated urinary residuals greater than 50 mL (P less than .02); (4) a diagnosis of elevated urinary residuals greater than 6 months following the onset of continence (P less than .001); and (5) one or more febrile urinary tract infections following bladder neck reconstruction (P less than .005).
The authors report a case of pyeloureteric adenocarcinoma in a urinary diversion inserted for bladder exstrophy and in a context of chronic pyelonephritis secondary to renal stones. The various aetiopathogenic hypotheses are discussed. Renal stones and chronic inflammation very probably played an important role in the development of this type of adenocarcinoma in the urothelium.
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