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PubMed1990

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↗
PubMed1991

[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↗
PubMed1979

[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↗
PubMed1991

[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↗
PubMed1992

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↗
PubMed1992

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↗
PubMed1991

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↗
PubMed1977

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↗
PubMed1991

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↗
PubMed1990

[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↗
PubMed1991

Lower urinary tract reconstruction in patients with bladder exstrophy after failed primary treatment in early childhood.

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.

Adolescent↗
PubMed1991

Abnormalities of colonic mucin secretion and metabolic changes after internal urinary diversion for bladder exstrophy. A prospective study.

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.

Adolescent↗
PubMed1990

Factors predisposing to renal scarring: following staged reconstruction of classical bladder exstrophy.

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).

Anti-Bacterial Agents↗
PubMed1992

[Pyeloureteral lieberkuhn adenocarcinoma after trans-intestinal ureterostomy for bladder exstrophy].

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.

Adenocarcinoma↗
PubMed1991

Horizontal pelvic osteotomies for bladder exstrophy. A preliminary report.

We have treated 11 patients aged three days to 15 years with bladder exstrophy by horizontal osteotomies of the innominate bones. The operation was originally used for older patients with severe deformity or failed previous surgery but is now applied as a primary procedure in the first week of life. The osteotomies enable the complex malformations to be corrected in a single operation without turning the patient: the pubic bones can be brought together, the abdominal wall repaired and the bladder closed with reconstruction of the urethra and external genitalia. The early results have been very satisfactory in all cases with only minor complications; we felt that a preliminary report should be made, despite a mean follow-up of only seven months.

Adolescent↗