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Staged closure of the pelvis in cloacal exstrophy: first description of a new approach.

PURPOSE: We describe a staged approach for pelvic closure in patients with cloacal exstrophy, and evaluate the technique for functional and cosmetic results. MATERIALS AND METHODS: Staged closure of the pelvis was performed in 5 patients 12 months to 14 years old with cloacal exstrophy at our institution, including some referred with a history of failed closure. The technique involves initial pelvic osteotomy followed by soft tissue and pelvic ring closure 2 to 3 weeks later. Clinical information is reviewed to assess the effectiveness of the technique. RESULTS: At a mean followup of 3.2 years (range 1 to 9) closure was successful in all 5 patients without technical problems or surgical complications, and with excellent functional and cosmetic results. CONCLUSIONS: This technique of staged pelvic closure may provide reliable initial or secondary repair in patients with cloacal exstrophy in whom 1-stage pelvic closure is unfeasible even with pelvic osteotomy.

Adolescent↗

Current status of tumor of the bowel following ureterosigmoidostomy: a review.

In the final analysis the major question that arises is whether urinary diversion to the intact colon should be performed at all for benign conditions in which a relatively long life expectancy may be anticipated. In answer to this question we believe that if exstrophy is the problem early primary closure with the staged reconstruction should be attempted first. If such efforts are marred by persistent incontinence management with an artificial urinary sphincter should be considered. In individuals with multiple bladder dehiscences after attempts at primary bladder closure, or in an individual with persistent incontinence despite multiple procedures surgical alternates should include diversion by a bowel conduit, continent urinary diversion, a variant of the ureterosigmoidostomy or standard ureterosigmoidostomy. Indeed, despite the appropriate concern regarding the development of tumor in ureterosigmoidostomy, this diversion may still have a major role in the educational process of urology throughout the next decade. Specifically, we must apply the knowledge gained from our clinical and laboratory investigations of ureterosigmoid diversion to the current more popular means of diversion. Of particular concern are the clinical findings of adenocarcinoma in enteric augmentations. This discovery must serve as a warning for the possibility of urocolonic tumors developing within alternative continent urinary diversions within the next 20 to 30 years. Certainly, at least annual evaluations of any diversions are mandatory until we can define accurately the morbidity and mortality arising from our interventional management.

Adenocarcinoma↗

Intestinocystoplasty and total bladder replacement in children and young adults: followup in 129 cases.

A review of 129 consecutive young patients (average age 13.4 years) who underwent intestinocystoplasty or total bladder replacement during a 6 1/2-year period at our institution is presented. The most common diagnosis was myelodysplasia and the average followup was 44 months. The clinical and urodynamic aspects of 4 types of intestinocystoplasty (ileocecal, tubular sigmoid, sigmoid patch and ileal patch) are presented. All 4 operations resulted in a significant increase in bladder volume, with a decrease in filling pressure and, thus, improved vesical compliance. In combination with clean intermittent catheterization renal function was maintained or improved in 91 per cent and urinary continence was achieved in 82 per cent of the patients. Hyperchloremic acidosis requiring therapy was noted only in patients with pre-existing renal insufficiency, although mild hyperchloremia after cystoplasty was seen with all 4 types of cystoplasty. There were no operative mortalities. Significant surgical complications occurred in 36 per cent of the patients, the most common of which was vesicoureteral reflux in the ileocecal cystoplasty. Mass unit peristaltic contractions occurred in 34 per cent of the tubular-shaped bowel segments compared to only 10 per cent of the patch segments. These peristaltic contractions contributed to the greater postoperative morbidity noted in the tubular large bowel cystoplasties. The over-all success rate for intestinocystoplasty in this series was 84 per cent. Intestinocystoplasty is an effective procedure when used to increase the compliance of the lower urinary tract. In combination with clean intermittent catheterization it can be applied successfully to patients with neurogenic bladder dysfunction. Large and small bowel seem to have similar clinical and urodynamic properties. The type of intestinal segment used for intestinocystoplasty seems to be of less importance than the size and configuration. Large bowel in its native tubular configuration should be avoided.

Adult↗

Total reconstruction of the lower urinary tract using bowel and the artificial urinary sphincter.

We report 3 cases in which the lower urinary tract was reconstructed using bowel. In each instance the cuff was placed around the bowel to provide a continence mechanism. The 2 patients in whom the sigmoid colon was used have sporadic diurnal incontinence with nocturnal enuresis. Urodynamic evaluation has shown that this is secondary to high pressures generated within the closed sigmoid loop. The ileocecocolonic segment is preferable for reconstruction of the lower urinary tract, since the pressure developed within the bowel is significantly lower. The use of the artificial urinary sphincter around the bowel extended the versatility of the device.

Adolescent↗

[Neo-bladder made from the rectum in surgical cure of uro-genital malformations (author's transl)].

The authors give a critical account of the results of artificial bladder construction using the rectum in complex genito-urinary malformations, such as, extrophic bladder, failure of correction of epispadias, and discuss 12 personal cases of which 6 out of 10 had been followed up for between 10 and 15 years. One important recommendation: in adults where the shincter system is normal, according to our experience, there is little trouble with urinary or fecal incontinence. In children, it is still necessary to develop a procedure which might permit one to express, numerically, the conclusions of the electrical examination and the volume above which resistance begins to break down. In small boys, after formation of the new bladder, one should attempt to restore micturition by the natural route, by reconstruction of the neck of the bladder, ileoplasty and reimplantation of the ureters in situ.

Adolescent↗

[Long-term observations following urinary drainage into the intact large intestine].

A report is given on 21 patients who have survived for at least 5 years after drainage of urine into the non-eliminated large intestine (Coffey-Mayo). Uretero-intestinal anastomosis was performed in 19 children, because of extrophy of the bladder, and in 2 women, following a malignant primary disease. In around half the patients there have been no complications during the post-operative observation period (on average 16.5 years), while new drainages had to be performed in the other half because of structures of the anastomoses. The patients have to adapt their daily lives to the "Coffey" and know the symptoms of an imminent electrolyte disturbance. Post-operative care must be arranged with the patient. Coffey's operation may be indicated when life expectancy is short; otherwise an antirefluxive ureterocolic anastomosis is recommended.

Adolescent↗

The patient with exstrophy in adult life.

One hundred and one cases of classical exstrophy with at least 18 years of follow-up are reported. There were 29 girls and 72 boys. Nine have died but only 3 of urological causes; 19 have been lost to follow-up at various times. In patients born before 1953 and in later patients with small bladders, primary diversion was done. In other patients primary closure was attempted with an 18% continence rate. Successful bladder closure produced a 25% incidence of any kind of renal damage. With diversion the incidence was 30% (colonic loop) and 75% (ileal loop). Sexual function is good. Six men have initiated pregnancies; 8 girls had 11 pregnancies, producing 9 normal children. Education, employment and family life have not been substantially affected.

Adolescent↗

Management of cloacal exstrophy.

This case study demonstrates our experience in the very complex management of cloacal exstrophy. It exemplifies the aggressive therapy by a dedicated multidisciplinary health team and the excellent postoperative results.

Abdominal Muscles↗

Anxiety disorders in children with epispadias-exstrophy.

OBJECTIVES: To evaluate the hypothesis that anxiety disorders are common comorbid conditions in children with the epispadias-exstrophy complex. METHODS: Twenty consecutive outpatient exstrophy subjects ranging in age from 5 to 22 years were assessed using a formalized semistructured psychiatric evaluation and were categorized according to the Diagnostic and Statistical Manual of Mental Disorders, version IV, criteria. Disorders were identified if subjects endured moderate to severe impairment in their home, academic, and social environments. RESULTS: All 20 subjects met the criteria for at least one anxiety disorder; 19 met criteria for more than one anxiety disorder. The adolescent subjects described a gradual waning of some specific symptoms some time after surgical correction of the physical conditions (eg, incontinence) but intensifying sexual anxiety with age. CONCLUSIONS: The epispadias-exstrophy complex appears to be associated with clinically significant vulnerabilities for anxiety disorders in children.

Adolescent↗

Covered exstrophy and visceral sequestration with complete double bladder.

A 13-year-old boy had a complete double bladder and urethra associated with an inferior abdominal lesion, which on histological examination proved to be a colonic remnant with no communication to the underlying bowel. This entity is one of the exstrophy/epispadias complex variants known as covered exstrophy with sequestered bowel. Although 4 cases of this anomaly have been reported in the literature, to my knowledge this is the first such case with a double bladder. Excision of the lesion and the incontinent bladder was performed, and the ureter was reimplanted in the contralateral continent bladder.

Abdominal Muscles↗

Transverse island flap and double flap procedure in the treatment of congenital epispadias in 32 patients.

According to major reports in the literature satisfactory cosmetic and genital results after correction of male epispadias have been achieved in only 50 per cent of the patients. Since 1976 we have been using the Duckett transverse island flap principle adapted to epispadiac anatomical conditions. The original technique was used in our initial 18 patients and the Asopa double flap technique was performed in the subsequent 14. Excellent results were obtained in both series. However, there was a high percentage of fistulas that required secondary repair. The techniques and results are described.

Abnormalities, Multiple↗

Ileal conduits in children at the Massachusetts General Hospital from 1955 to 1970.

The 45 ileal conduits performed on children at the Massachusetts General Hospital from 1955 to 1963 are reviewed and compared to the 45 ileal conduits performed from 1964 to 1970. Late complications involving the conduits occurred in 60 per cent of the early group and in 51 per cent of the late group. Of the renal units judged normal pyelographically preoperatively in the early group 77 per cent went on to at least some deterioration, while 62 per cent of the late group judged normal later deteriorated. Combining all renal units, 34 per cent remained unchanged, 26 per cent improved and 41 per cent showed some degree of deterioration after ileal conduit urinary diversion. The late complication and renal deterioration rates seem to increase progressively with time. There was no apparent urinary obstruction in 13 per cent of the renal units that deteriorated. Theoretical and experimental considerations indicate the reflux of infected urine as the etiology of the renal deterioration. Because of the late complications and the unacceptably high rate of renal deterioration we no longer perform ileal conduits in children. Instead every effort is made to reconstruct the urinary tract or if urinary diversion is necessary, a colon conduit with non-refluxing ureterocolonic anastomoses is performed.

Adolescent↗