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Ureterosigmoidostomy and bladder exstrophy: a long-term followup.

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.

Adolescent

[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

Medicinal leeches in the postoperative care of bladder exstrophy.

This article reports the use of medicinal leech therapy for the relief of severe postoperative vascular congestion of the penis in a male infant with exstrophy of the bladder. When more conventional methods of decongestion were unsuccessful, medicinal leech therapy markedly improved the infant's chance of remaining both phenotypically and functionally male. The uneventful and successful use of medicinal leeches in this patient demonstrates that medicinal leeches may be safely used in the newborn period, if appropriate precautions are taken to prevent both infection and anemia.

Animals

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

Functional reconstruction of exstrophied bladder. Timing and technique. Follow-up of 39 cases.

Thirty-nine cases of functional reconstruction of exstrophied bladder are reviewed. There were 20 females and 19 males. In a first group of 31 staging was planned and carried out in 26 while 3 are awaiting a second stage. In a second group of 8 a single operation was performed. Out of the 31 early cases there were 2 deaths and 10 failures, 16 show good or very good functional result. All 16 show moderate renal scarring from reflux pyelonephritis which occurred between I and II stage. Single operation consisted of innominate osteotomy, bladder and bladder neck and urethral reconstruction and anti-reflux procedure (osteotomy was omitted in a 3 days old baby). Three, operated upon when aged 8 mths., 1 year and 4 years, were breakdowns of previous closure. They are incontinent and will need further surgery at the bladder outlet. Four are dry in the morning and suffer from occasional stress incontinence and enuresis. One, now aged 4 years, still wears pads. These last 5 are awaiting final assessment of and eventually further surgery to improve continence. In all 8 cases pyelo-calyceal cavities are normal except for a moderate right dilatation in one because of kinking of the reimplanted ureter. Delay in bladder closure (mean age 5 mths.) and severe changes to the bladder wall and possibly staging seems to be responsible for most of the failures and for renal damage occurring, after closure, in bladders showing moderate compliance. Single operation allows full protection of upper tract and kidney and should be preferred in patients aged over 2 months. Better functional results may be obtained if operation is performed soon after birth.

Adolescent

Urethral duplication and complete bladder exstrophy.

Urethral duplication is a rare anomaly that has been classified into epispadiac, hypospadiac, spindle, Y-duplications and collateral varieties. We report a case of an accessory urethra associated with complete bladder exstrophy in which the accessory urethra was integrated into the epispadias repair. To our knowledge this is the first case reported of urethral duplication associated with bladder exstrophy.

Abnormalities, Multiple

[Reconstructive-plastic surgery in bladder exstrophy].

Among 234 children with exstrophy of the urinary bladder 112 were subjected to reconstructive-plastic operation after G. A. Pair. A good result was produced in 71 cases. In 27 children a satisfactory result was attained only after repeated operations. Reconstructive-plastic interventions had no positive effect in 14 children. By means of electrophysiological and morphological methods three degrees of underdevelopment of the muscular coat of an urinary bladder affected by exstrophy were distinguished. In I degree plastics of the bladder with local tissues is indicated, in II degree the indications are relative, in III degree a plastic operation is not indicated. Choice of the operative method on basis of the morphofunctional condition of the bladder with exstrophy, the use of microsurgical techniques, the formation of a closed pelvic ring, and antireflux operations on the ureters improve the immediate and late-term results of reconstructive-restorative surgical interventions.

Age Factors

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.

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.

Bladder Exstrophy

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.

Bladder Exstrophy

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

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