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PubMed1999

Appendicovesicostomy and newer alternatives for the Mitrofanoff procedure: results in the last 100 patients at Riley Children's Hospital.

PURPOSE: We present our experience using the various Mitrofanoff techniques to create a continent catheterizable stoma as an adjunct to continent urinary tract reconstruction in children and young adults. MATERIALS AND METHODS: Between 1990 and 1998 a Mitrofanoff procedure was performed at our institution in 55 male and 45 female patients with a mean age of 10.5 years. The etiology of incontinence was diverse but more than 90% of the patients had neurogenic bladder, the epispadias-exstrophy complex or a cloacal anomaly. Surgery included appendicovesicostomy in 57 cases, a Yang-Monti ileovesicostomy in 21, continent vesicostomy in 21 and formation of a tapered ileal segment as a catheterizable channel in 1. Simultaneously bladder augmentation was performed in 52 patients, bladder neck reconstruction was done in 48 and a Malone antegrade colonic enema stoma was constructed for fecal incontinence in 17. RESULTS: The abdominal stoma is continent in 98 of our 100 patients. Mean followup is 2 years (range 2 months to 8 years) with the longer followup in the appendicovesicostomy group. One patient with stomal incontinence who underwent revision is now dry. Postoperative complications requiring an additional procedure developed in 20 patients, including stomal stenosis in 12. Continent vesicostomy was most prone to stomal problems (6 of 21 patients, 29%). CONCLUSIONS: The Mitrofanoff procedure is a reliable technique for creating a continent catheterizable urinary stoma. Appendicovesicostomy continues to be our first option for this procedure, although we have also had good results with the Yang-Monti ileovesicostomy and continent vesicostomy. These newer options have allowed preservation of the appendix for the Malone antegrade colonic enema stoma procedure in patients with urinary and fecal incontinence.

Adolescent↗
PubMed2001

Antenatally diagnosed cloacal exstrophy variant with intravesical phallus in a twin pregnancy.

We report a rare case of covered cloacal exstrophy variant with a hemiphallus trapped within partially closed bladder halves. The persistence of the cloacal membrane until at least 18 weeks' gestation, confirmed by antenatal ultrasound scanning, is discordant with existing theories of embryogenesis of cloacal exstrophy. The clinical presentation highlights the need for careful assessment, before and during surgery, to obtain a complete understanding of the anatomic defect before gender assignment and appropriate reconstructive efforts. A multispecialty approach and antenatal counseling are important, especially when only one fetus of twins has major birth defects.

Abnormalities, Multiple↗
PubMed1993

Glutaraldehyde cross-linked bovine collagen in exstrophy/epispadias complex.

We injected endoscopically 0.75 to 2.5 cc of cross-linked bovine collagen at the 3, 9 and 12 o'clock positions of the urethra or bladder neck of 9 children with epispadias and 7 with exstrophy. This procedure was performed in 10 children who were still incontinent after a Young-Dees operation. In 6 girls collagen was injected submucosally before bladder neck surgery to increase outlet resistance and to gain bladder capacity. A repeat injection was given to 3 patients after 1 year. Continence improved in 9 of the 10 cases injected after surgery of the bladder neck. Of the 6 children who underwent collagen treatment before reconstruction of the lower urinary tract capacity increased an average of 47% in 2 years in 5 and the procedure failed in 1. Submucosal injection of collagen is helpful in modulating outflow resistance and increasing bladder volume after primary closure of exstrophic bladders. Patients with small bladders following closure, who would otherwise be candidates for augmentation cystoplasty, may gain adequate bladder size with collagen injection.

Adolescent↗
PubMed1991

Use of the Benchekroun hydraulic valve as a catheterizable continence mechanism.

Problems with continence and access for catheterization with previous continent stoma techniques have led us to search for an alternate method. We report our initial experience with the Benchekroun hydraulic valve in 9 patients. Five patients underwent creation of an ileocolic bladder: for myelodysplasia in 1, after cystectomy for bladder cancer in 2 and for exstrophy in 2. Two patients underwent conversion from an uncatheterizable Indiana pouch because of exstrophy (1) and interstitial cystitis (1). Two patients underwent small bowel augmentation of a small, poorly compliant bladder with an abdominal Benchekroun stoma: 1 had exstrophy and 1 had myelodysplasia. Patient age ranged from 7 to 63 years. Two patients had de novo creation of an ileocolic bladder, whereas 7 had bladder or previous bowel conduits incorporated in the continent reservoirs. At up to 20 months of followup all patients had diurnal and nocturnal continence, and none had experienced serious long-term complications. Problems were encountered in 5 patients. One patient had a valve fistula that was surgically revised. One patient experienced transient difficulty with catheterization when the reservoir was overdistended and a false passage developed. Three patients had stomal stenosis and require periodic dilation. We believe the Benchekroun hydraulic valve to be a useful adjunct in the construction of a continent urinary reservoir. It is easy to construct, provides reliable continence and is easy to catheterize. Furthermore, it may be applied to standard ileocolic bladders either de novo or as a salvage procedure for failed efferent limbs.

Adolescent↗
PubMed1988

Use of the AS800 device in exstrophy and epispadias.

Urinary continence is one of the more difficult goals to achieve in patients with exstrophy or epispadias. The artificial genitourinary sphincter provides an alternative to reconstruction of the bladder neck for management of this problem. Although excellent continence can be obtained with bladder neck reconstruction, results of a second procedure of this type seldom are reported. We used the AS800 device in 16 patients with exstrophy and epispadias, 13 of whom had undergone a prior bladder neck operation. We defined our results as total success and continence success, and have achieved over-all rates of 69 per cent (excludes those awaiting revision) and 90 per cent (those with an active device), respectively. Although the frequency of revision and erosion was significant, the ultimate outcome in this difficult patient group was satisfactory.

Adolescent↗
PubMed1994

Prenatal diagnosis and management of the fetus with an abdominal wall defect.

Prenatal ultrasound has advanced our understanding of congenital abdominal wall defects. In addition to providing insights into the divergent embryological origins and natural history of abdominal wall defects, ultrasound has had an important impact on the management of these anomalies. For fetuses with gastroschisis, the changes in appearance of the bowel may suggest expeditious delivery. In cases of omphalocele, the presence of additional anomalies is significantly associated with the ultimate prognosis for these fetuses. Giant omphalocele may preclude vaginal delivery secondary to dystocia. Exstrophies of the cloaca and bladder are rare congenital abnormalities that often present complex management issues, including gender reassignment in cases of cloacal exstrophy, for those couples wishing to continue the pregnancy. We believe that the optimal management of a fetus diagnosed with an abdominal wall defect requires a coordinated effort among specialists from maternal fetal medicine, pediatric surgery, and pediatrics.

Abdominal Muscles↗
PubMed1997

Urethral lengthening with anterior bladder wall flap (Pippi Salle procedure): modifications and extended indications of the technique.

PURPOSE: We report a clinical case series of an innovative method of urethral reconstruction for the treatment of urinary incontinence. Modifications of our original technique are presented. MATERIALS AND METHODS: Bladder neck repair was done in 17 patients, mean age 9.3, with neurogenic incontinence (13) or exstrophy (4). Average followup is 25.6 months. Of the patients 9 with neurogenic bladder underwent the original procedure using a midline anterior bladder wall flap. In the 4 patients with exstrophy a modified procedure was done using an anterolateral bladder wall flap. In another 4 patients an extended flap of distal mucosa was used to avoid ureteral reimplantation. Augmentation was performed in 13 of the 17 cases (10 detubularized ileum and 3 detubularized colon). RESULTS: Continence (greater than 4 hours) was obtained in 12 of the 17 patients (70%), 2 are dry for 1 to 2 hours and 3 are incontinent. A urethrovesical fistula developed in 2 patients (1 closed successfully), and 3 patients have problems with catheterization. CONCLUSIONS: Urethral lengthening with anterior bladder wall flap is a versatile alternative in the surgical treatment of urinary incontinence. Variations of the original technique resulted in an improved vascular supply and decreased the formation of fistula at the base of the flap. The modified technique was successful in patients with exstrophy, including those who had failed bladder neck surgery.

Bladder Exstrophy↗
PubMed1989

Continent urostomy with hydraulic ileal valve in 136 patients: 13 years of experience.

The hydraulic ileal valve, which we developed in 1975, ensures continence by adapting to 5 different urinary reservoirs. The valve is made by isolating a 14 cm. long intestinal loop with the mesentery. The isolated ileal segment then is folded inward on itself throughout its entire length. We performed 136 continent urostomies with this hydraulic valve. An ileocecal reservoir was used in 122 patients, ileum in 8, sigmoid in 1, rectum in 1 and bladder (continent cystostomy) in 4. Indications for continent urostomy were bladder tumor in 55 patients, complex vesicovaginal fistulas in 5, neurogenic bladder in 13, vesical exstrophy in 12 and miscellaneous reasons in 5. Of the patients 103 (75 per cent) were continent immediately. Continence was obtained after repair of the valve in 24 patients (17.6 per cent). Therefore, 127 patients over-all were continent. Mean followup of our patients was 38 months (range 3 to 154 months). Continence remained excellent with self-catheterization performed easily in 88.3 per cent of the patients. Over-all, all of our continent urostomies were well tolerated biologically and radiologically.

Cecum↗
PubMed1992

[Historic evolution of the use of the intestine in urology].

Knowing the history of Urology has allowed urologists to recover techniques which had been abandoned for different reasons, yet when modified or utilized with current technological advancements, have proved to be very useful. A brief review of the history of urinary diversion clearly shows that it has evolved in parallel with the important diseases; i.e., vesical exstrophy, urinary tuberculosis and bladder cancer. Time tests all new, and thus controversial, concepts or procedures. They become either well established or are condemned to oblivion. All the experience, both successes and failures, that make up the history of Urology undoubtedly contribute towards finding new therapeutic alternatives.

History, 20th Century↗
PubMed1990

[Endoscopic treatment of vesicorenal reflux in children. Review of three years experience and prospects for the future].

Endoscopic injection of teflon paste, used since 1984, represents an alternative to the surgical treatment of vesico-renal reflux. The success rate varies from 75 to 89% after a single injection and from 85 to 95% after two injections, according to the teams. The results are less satisfactory for the high grades and for refluxes on duplicity (25 to 45% failure rate). This technique is particularly attractive in the case of a difficult surgical intervention, notably when a reimplantation has failed, in the case of reflux on neurological bladder, urethral valves or exstrophy. Various causes of failure or relapse are considered. The advantages of the technique are obvious (low morbidity, very short hospital stay) and surgery is always possible in the case of failure. After one or two years, the reflux reappears in 2 to 9% cases. Long term results cannot be assessed as the follow-up period is too brief at the present time. For this reason, and although the endoscopic and surgical findings are reassuring, tolerance to teflon both locally and at a distance requires confirmation and other products such as collagen are now being used.

Adolescent↗
PubMed2006

The OEIS complex: two case reports that illustrate the spectrum of abnormalities and a review of the literature.

We present two cases of OEIS (omphalocele, exstrophy, imperforate anus, spinal defects) complex -MIM 258040 and a review of the literature. Case 1 was a 14-year-old girl who presented at 30 weeks' gestation. An ultrasound examination showed an omphalocele and spina bifida; the bladder was not visualised. She went into spontaneous labour two weeks later and the baby died shortly after birth. A full post-mortem examination was refused, but the mother did agree to an external examination, skin biopsy for fibroblast culture, X rays and MR imaging. The MR imaging showed a pelvic kidney, a large omphalocele containing the other kidney, liver, bowel and a fluid filled structure thought to represent an exstrophy of the bladder (EB). Case 2 was a 30-year-old woman who had an ultrasound examination at 20 weeks' gestation; this showed an omphalocele, but the bladder was not visualised. The pregnancy was subsequently terminated and a post-mortem examination showed a low set umbilical cord associated with a small omphalocele; there was an imperforate anus; a blind ending rectum terminated in the omphalocele. We conclude that these two cases illustrate the variability of the OEIS complex.

Abnormalities, Multiple↗
PubMed1989

Augmentation cystoplasty in patients with exstrophy-epispadias.

In a series of over 200 patients with exstrophy-epispadias from the Mayo Clinic, a subgroup of 11 with small bladder capacities, decreased detrusor compliance, and urinary incontinence underwent augmentation cystoplasty. Preaugmentation bladder capacities were between 25 and 250 mL (mean, 73 mL). Bladder capacities after enterocystoplasty improved significantly, with a range between 180 and 500 mL (mean, 355 mL). Ten of the 11 patients have total urinary continence for greater than 3 hours. One child remains dry between 1 1/2 and 2 hours. Nine patients are in complete urinary retention and are managed by clean intermittent catheterization. Two patients void spontaneously by pelvic muscle relaxation and abdominal straining alone. All patients maintain normal or stable renal function postoperatively. Excretory urography showed decreased caliectasis in two patients with preoperative hydronephrosis and stable upper urinary tracts in nine patients. Enterocystoplasty can produce total urinary continence, preserve renal function, and avoid the long-term sequelae of urinary diversion in selected children born with exstrophy-epispadias.

Adolescent↗
PubMed1993

Covered exstrophy with sequestered colonic remnant.

We report on a woman who presented with urinary incontinence, covered exstrophy and an abdominal mass. Radiographic studies and cystoscopy confirmed that the anterior abdominal mass did not communicate with the bladder or colon and that the gastrointestinal tract was normal. Subsequent surgical resection of the mass and Young-Dees-Leadbetter bladder neck reconstruction were performed. Histological examination of the mass revealed colonic mucosa. The etiology of the covered exstrophy and sequestered colonic remnant is discussed.

Abnormalities, Multiple↗
PubMed1986

Bladder neck reconstruction with omentum, silicone and augmentation cystoplasty--a preliminary report.

A new method of bladder neck reconstruction in which the Young-Dees tube was wrapped with omentum and silicone was used in 39 patients during a 20-month period. In 43 per cent of the patients simultaneous augmentation cystoplasty also was performed. The silicone was intended to maintain the Young-Dees tube length and to allow safe artificial sphincter cuff placement later when necessary. Among exstrophy patients 85 per cent of those who underwent augmentation and 15 per cent of those who did not are dry. A dry exstrophy patient had a remarkably easy sphincter cuff placement 6 months after bladder neck reconstruction. Of the epispadias patients 25 per cent are dry and 58 per cent have a dry interval of 2 hours or less. Erosion of silicone into the Young-Dees tube occurred in the first 5 patients in whom a thick (0.01-inch) wrap was used and in 14 per cent of those in whom a thin (0.005-inch) sheet was used. The details of this technique and modifications to reduce further the complication rate are discussed.

Adolescent↗
PubMed2005

Long-term followup of augmentation enterocystoplasty and continent diversion in patients with benign disease.

PURPOSE: We evaluated long-term outcomes in patients undergoing augmentation enterocystoplasty (AC) (with or without an abdominal stoma) or continent urinary diversion in patients with benign urological disorders. MATERIALS AND METHODS: This was a retrospective study of 76 consecutive adults who underwent AC (with or without an abdominal stoma) or continent urinary diversion because of benign urological conditions. The outcomes assessed were a patient satisfaction questionnaire, continence status, catheterization status, bladder capacity, bladder compliance, detrusor instability, maximum detrusor pressure, upper tract status, significant postoperative morbidity, need for reoperation, persistent diarrhea and vitamin B12 deficiency. RESULTS: The 76 patients (18 men and 58 women) were 19 to 80 years old (mean age 49). Followup was 1 to 19 years (mean 8.9). Preoperative diagnoses were neurogenic bladder in 41 patients, refractory detrusor overactivity in 9, interstitial cystitis in 7, end stage bladder disease in 7, radiation cystitis in 3, exstrophy in 3, postoperative urethral obstruction in 3 and low bladder compliance in 3. A total of 50 patients underwent simple AC, 15 underwent AC with an abdominal stoma and 11 underwent continent supravesical diversion. Of the 71 evaluable patients 49 (69%) considered themselves cured, 14 (20%) considered themselves improved and 8 (11%) considered treatment to have failed. All 7 patients with interstitial cystitis had failed treatment. Mean bladder capacity increased from 166 to 572 ml and mean maximum detrusor pressure decreased from 53 to 14 cm H2O. Serum creatinine improved or remained normal in all patients. Five patients experienced persistent diarrhea requiring intermittent antispasmodics but none had vitamin B12 deficiency, pernicious anemia or malabsorption syndrome. Long-term complications were stomal stenosis or incontinence in 11 of 26 patients (42%) with stomas, de novo bladder and renal stones in 2 of 71 (3%) and 1 of 71 (1%), respectively, and recurrent bladder stones in 6%. Small bowel obstruction occurred in 5 of 71 patients (7%), requiring surgical exploration in 4 (6%). CONCLUSIONS: AC and urinary diversion provide a safe and effective long-term therapy in patients with refractory neurogenic bladder but stomal problems in patients with continent diversion continue to be a source of complications.

Adult↗
PubMed2006

Can a complete primary repair approach be applied to cloacal exstrophy?

PURPOSE: Surgical reconstruction for children with cloacal exstrophy remains challenging. The operative approach to cloacal exstrophy has expanded with the addition of the complete primary exstrophy repair. We assessed the safety and efficacy of complete primary exstrophy repair for this complex condition. MATERIALS AND METHODS: We performed a retrospective review of children treated from birth for cloacal exstrophy between March 1, 1994 and January 1, 2003 at our institution. We evaluated associated anomalies, method of closure, complications and urinary continence. RESULTS: Seven patients with cloacal exstrophy were initially treated at our institution. One mortality occurred before complete primary exstrophy repair was attempted. Six patients were converted to a classic exstrophy appearance and underwent closure using complete primary exstrophy repair principles within 7 to 182 days (mean 68, median 32) postoperatively. Postoperative development of moderate hydronephrosis was seen in 1 patient and severe hydronephrosis in 1. Three of six patients had vesicoureteral reflux. Six patients had dry intervals and spontaneous voids before toilet training. Two patients had stress urinary incontinence. Two patients have been treated with bladder neck injections. One has undergone bladder neck reconstruction and construction of a nonorthotopic channel for clean intermittent catheterization (Mitrofanoff). One patient reported complete dryness after toilet training. One child has undergone bladder augmentation. CONCLUSIONS: This series represents our initial efforts to use complete primary exstrophy repair for cloacal exstrophy. The application of the principles of complete primary exstrophy repair in a sequential fashion appears to be a viable and safe addition to the surgical armamentarium in this challenging patient population.

Abnormalities, Multiple↗