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At least 793 records · Page 44Linked to original sources
PubMed1995

The Cantwell-Ransley epispadias repair in exstrophy and epispadias: lessons learned.

OBJECTIVES: We evaluated our experience with the Cantwell-Ransley epispadias repair to determine the lessons that have been learned with the increased experience and follow-up. METHODS: A total of 75 boys (60 with bladder exstrophy and 15 with complete epispadias) underwent a Cantwell-Ransley epispadias repair at our institute in the last 6 years. Primary repair was performed in 58 boys (45 with exstrophy and 13 with epispadias), and secondary repair was performed after prior failed closure in 17 boys (12 at the secondary exstrophy closure, 3 with exstrophy, and 2 with complete epispadias). RESULTS: At a mean follow-up of 28 months, all patients had a horizontal or downward angled penis while standing. The incidence of urethrocutaneous fistulas in the immediate postoperative state was 21% and at 3 months was 15%. The incidence of urethrocutaneous fistulas was no more in those patients in whom paraexstrophy skin flaps were used at anterior closure than in those in whom the urethral plate was left intact. Two patients developed a urethral stricture at the proximal anastomotic area, and 4 patients had minor skin separation of the dorsal penile skin closure. Catheterization or cystoscopy, or both, has been performed in 60 patients and revealed an easily negotiable urethral channel in all. CONCLUSIONS: The Cantwell-Ransley epispadias repair offers a straighter urethra, better correction of chordee and cosmesis, and a lower fistula rate in the exstrophy or epispadias patient.

Adolescent↗
PubMed2007

Continent cutaneous urinary diversion in children: experience with Charleston pouch I.

PURPOSE: Complete continent urinary diversion not incorporating the bladder is not commonly used in children. We evaluated the short and long-term outcome of a form of continent cutaneous urinary diversion (Charleston pouch I) in children. MATERIALS AND METHODS: A total of 17 children underwent Charleston pouch I continent cutaneous urinary diversion between 1988 and 2005. Patient records were reviewed for age, sex, indications for diversion, preoperative and postoperative laboratory and radiological studies, continence, patient and family acceptance, complications and long-term functional status. RESULTS: Patient age ranged from 6 to 16 years. The main indication for diversion was bladder exstrophy in 8 patients (47%), neurogenic bladder in 6 (35%) and cloacal abnormalities in 3 (18%). Mean followup was 87.5 months. One patient was lost to followup. With moderate fluid intake the other patients were dry with a mean catheterizing time of 3.4 hours (range 2 to 6). Catheterization intervals were adjusted for individual patients. Generally, the patients became damp or leaked if they did not catheterize at recommended intervals. Continence was achieved at variable postoperative intervals, with some patients attaining continence soon after and others at 3 to 12 months before pouch maturation. Patients irrigated the pouch a mean of 4 times weekly (range 0 to 14). Three patients (18%) had bladder stones. Ultrasound and/or other upper tract studies revealed no deterioration of the upper urinary system. No patient experienced clinical pyelonephritis or acidosis. Family and patient acceptance was satisfactory. CONCLUSIONS: Continent cutaneous urinary diversion with Charleston pouch I was satisfactory in this group of children. It provided preservation of the upper urinary tract, and achieved acceptable continence rates while allowing leakage when catheterization was not performed at recommended intervals. In addition, patient and parent acceptance was good, and complication rates were acceptable.

Adolescent↗
PubMed1976

Primary adenovillous carcinoma of the bladder.

Two cases of primary adenovillous carcinoma of the bladder are presented: one in a normally placed bladder and the other in an exstrophied bladder. The close association of these 2 neoplasms with coexistent cystitis glandularis would seem to support the possible precancerous nature of the latter entity. The remarkable similarity of these neoplasms to villous adenocarcinomas of the rectum is also described, a finding which may reflect the close embryologic origin of the rectum and bladder neck. A brief review of the classification of vesical adenocarcinomas is also given.

Adenocarcinoma↗
PubMed2004

Expanded PTFE bladder neck slings for incontinence in children: the long-term outcome.

OBJECTIVE: To assess the long-term outcome of circumferential expanded PTFE (Gore-tex, WL Gore Associates, Scotland) bladder neck slings for achieving urethral continence in children with a neuropathic bladder. PATIENTS AND METHODS: The records were reviewed of 19 children undergoing bladder reconstruction (most with a neuropathic bladder) who had a Gore-tex sling placed circumferentially at the bladder neck, over a 5-year period. Of these, seven had spina bifida; two each spinal dysraphism, surgery for anorectal anomalies and an idiopathic neuropathic bladder; five who developed a neuropathic bladder from other causes, and one born with bladder exstrophy. All children had an uncompliant bladder with a low urethral leak-point pressure on preoperative urodynamics. In all children conventional clean intermittent catheterization and pharmacotherapy had failed. Four had had previous augmentation surgery while 15 had concomitant bladder augmentation and formation of a Mitrofanoff stoma. The main outcome measure was achieving dryness. The original intention of the procedure was also to maintain urethral catheterization. RESULTS: Full details of the follow-up were available in 17 patients. Despite initial good short-term results, at a median follow up of 7 years, in 14 patients the sling had to be removed because of erosion, often with transient urethral leakage before the bladder neck subsequently closed. A bladder calculus was associated with each case of erosion except one. CONCLUSION: Although in the short term this technique had favourable results, it was not a useful technique in the long term.

Child↗
PubMed1999

[Preliminary experiences with the use of the Holmium-YAG laser in the treatment of soft tissues and of cystine calculi in children].

INTRODUCTION: Experience of the use of the Holmium: Yttrium-Aluminium-Garnet (Ho:YAG) laser in children has been limited. However, the Ho:YAG laser has been in clinical use in urology for several years but has mainly been used for the treatment of renal stones and benign prostatic hyperplasia. Due to its unique combination of vaporization and coagulation, the Ho:YAG laser allows a precise cutting action. The depth of penetration in water and tissue is limited to < 0.5 mm and therefore provides a safety margin. The Ho:YAG laser can be used in children, as the energy can be delivered via fibers that range from 200 to 1000 mu in diameter. MATERIALS AND METHODS: We used the Ho:YAG laser in 5 children (2-15 years): one child (2 years) with bladder exstrophy had a urethral stricture after bladder neck reconstruction, two children (6 years and 14 years) had ureteropelvic junction (UPJ) stenosis and refused open surgery and two children (5 years and 15 years) suffered from cystine stones (ESWL failed). The urethral stricture was incised in a retrograde fashion. We performed an antegrade incision of the UPJ with the Ho:YAG laser in the 6-year-old child and a retrograde incision in the 14-year-old child. We removed the stones in antegrade fashion in the 5-year-old child and in retrograde fashion in the 15-year-old child. RESULTS: All children now have more than 12 month's follow-up. There were no immediate or late complications. The boy with urethral stricture remained free of recurrence, the boy with UPJ stenosis obtained improved drainage on the excretory renogram and the two children with cystine stones remained stonefree. CONCLUSION: We have shown that the safety and efficacy of the Ho:YAG laser is also reproducible in urologic pathology in children. In addition, due to its vaporizing quality, the Ho:YAG laser is more effective in the treatment of cystine stones and allows minimaly invasive treatment in children.

Adolescent↗
PubMed1995

Rectus fascial wrap: early results of a modification of the rectus fascial sling.

We describe an operation that increases bladder neck resistance in patients with urinary incontinence. It is a modification of the rectus fascial sling that was designed as an adjunct to augmentation cystoplasty and is used in association with clean intermittent catheterization. The operation is performed by circumferentially wrapping a rectus fascial defatted free graft around the bladder neck and suturing it to appose the bladder neck. The procedure was done in 17 patients, including 10 with myelodysplasia, 3 with sacral lipoma, 3 with bladder exstrophy and 1 with nonneurogenic neurogenic bladder. Leak point pressure improved in patients in whom it was measured preoperatively and postoperatively. Complications developed in 5 patients, including difficulty with catheterization in 2, ventral hernia at the graft harvest site in 1, bladder calculus in 1 and detrusor hyperreflexia in 1. Early results with the bladder fascial wrap indicate that it has the ability to improve continence in patients with a dysfunctional bladder neck who have undergone augmentation.

Adolescent↗
PubMed1988

Vesical neck reconstruction in patients with epispadias-exstrophy.

Vesical neck reconstruction was performed in 50 male and 12 female patients with the epispadias-exstrophy complex. Of these patients 45 had epispadias and 17 had classical exstrophy. Patient age ranged from 3 to 27 years, with a mean age of 12.6 years. Followup after vesical neck reconstruction averaged 11.6 years. Of the 45 patients with epispadias 35 (78 per cent) and of the 17 with bladder exstrophy 13 (76 per cent) are continent, for an over-all continence rate of 77 per cent. An adequate bladder capacity was one of the most important determinants of continence. In 11 patients with a small capacity or poorly compliant bladder augmentation cystoplasty was combined with vesical neck reconstruction to increase vesical capacity and to produce complete urinary continence.

Bladder Exstrophy↗
PubMed1976

Management of urinary incontinence with electronic stimulation: observations and results.

The evolution in the management of urinary incontinence with electronic stimulation of the pelvic floor is reviewed. The effect of stimulation is analyzed. Our methods of candidate screening and the contraindications to treatment are discussed. Forty patients were treated for 2 to 15 months. Sixteen patients were post-prostatectomy, 4 had stress incontinence, 4 had upper motor neuron lesions, 1 was incontinent after repair of a rectovesical fistula and 15 were post-bladder exstrophy closure. The success rate in the first group was 45.4 per cent, in the second group 100 per cent, in the third group 50 per cent, in the fourth group 100 per cent and in the fifth group 27.2 per cent. In view of the method having been tried on cases resistant to other forms of treatment the results were encouraging. The most satisfying results were in the post-exstrophy group in which no response was expected.

Adolescent↗
PubMed2003

Urogynaecological and obstetric issues in women with the exstrophy-epispadias complex.

OBJECTIVE: To review of the sexual and urogynaecological issues faced by a large cohort of women with the exstrophy-epispadias complex (EEC). PATIENTS AND METHODS: The study comprised 83 women and girls with EEC; a confidential survey was mailed to identify their social and sexual concerns. Fifty-six women had classical bladder exstrophy (CBE), 13 had female epispadias (FE) and 14 had cloacal exstrophy (CE). Data on the initial method of reconstruction and urogynaecological problems were obtained from a review of the hospital records. Information on continence, infection and sexual function was obtained from 34 completed surveys. RESULTS: The bladder was closed in 51 patients with CBE and 13 with CE. Urinary calculi developed in 10 patients with CBE, two with FE and three with CE. Vaginal and uterine prolapse occurred an earlier age in patients with EEC. Eight women had 13 pregnancies, eight of which resulted in normal healthy children. Overall continence was achieved in 85% of the women surveyed. Urinary tract infections remained a frequent problem for women with EEC; only 27% of respondents indicated that they were infection-free. Women aged > 18 years (24) who responded indicated that they had appropriate sexual desire; 16 were sexually active and the mean age for commencing sexual activity was 19.9 years. Six patients had dyspareunia and 10 indicated that they had orgasms. However, five additional patients indicated that they had restricted intercourse, as they were dissatisfied with the cosmesis of their external genitalia. CONCLUSIONS: Sexual and gynaecological issues become increasingly important in patients with EEC as they become adults. Understanding these issues faced by patients with EEC as they mature will permit better counselling of future patients.

Adolescent↗
PubMed2006

DeltaNp63 plays an anti-apoptotic role in ventral bladder development.

The bladder, the largest smooth-muscle organ in the human body, is responsible for urine storage and micturition. P63, a homolog of the p53 tumor-suppressor gene, is essential for the development of all stratified epithelia, including the bladder urothelium. The N-terminal truncated isoform of p63, DeltaNp63, is known to have anti-apoptotic characteristics. We have established that DeltaNp63 is not only the predominant isoform expressed throughout the bladder, but is also preferentially expressed in the ventral bladder urothelium during early development. We observed a host of ventral defects in p63-/- embryos, including the absence of the abdominal and ventral bladder walls. This number of ventral defects is identical to bladder exstrophy, a congenital anomaly exhibited in human neonates. In the absence of p63, the ventral urothelium was neither committed nor differentiated, whereas the dorsal urothelium was both committed and differentiated. Furthermore, in p63-/- bladders, apoptosis in the ventral urothelium was significantly increased. This was accompanied by the upregulation of mitochondrial apoptotic mediators Bax and Apaf1, and concurrent upregulation of p53. Overexpression of DeltaNp63gamma and DeltaNp63beta in p63-/- bladder primary cell cultures resulted in a rescue, evidenced by significantly reduced expressions of Bax and Apaf1. We conclude that DeltaNp63 plays a crucial anti-apoptotic role in normal bladder development.

Animals↗
PubMed1998

Tandem ureterocystoplasty.

BACKGROUND: Bladder augmentation may be undertaken by using various gastrointestinal segments but their use is associated with a multitude of well-recognized complications. The mega-ureter has proven to be a satisfactory alternative; in patients with bilateral mega-ureters, both ureters may be used for this purpose. METHODS: Seventeen patients had augmentation ureterocystoplasty, including three in whom both distal ureters were used in tandem. The latter included two patients with neurogenic bladder and one with bladder exstrophy. RESULTS: Satisfactory augmentation was achieved in all patients undergoing tandem ureterocystoplasty. The neurogenic bladder patients are managed by urethral clean intermittent catheterization (CIC) and the exstrophy patient is managed by CIC of an appendicovesicostomy (Mitrofanoff). All are continent. CONCLUSIONS: The mega-ureter provides an excellent source of augmentation material in patients with small non-compliant bladders. In those with bilateral mega-ureters, consideration should be given to using both ureters in tandem to achieve the maximum possible bladder capacity.

Bladder Exstrophy↗
PubMed1993

[Enterocystoplasty: the results with our first 21 patients].

The use of intestinal segments for bladder replacement or augmentation is an alternative in the management of urologic patients. Twenty-one patients (13 M, 8 F) underwent enterocystoplasty by different reason, neurogenic bladder (NB) (10 p), bladder exstrophy (7 p), cloaca exstrophy (3 p) and posterior urethral valves (PUV) (1 p) are presented. Mean age at operation was 12 years (range: 6-19) and the average followed was 3 years. Five patients undergone external urinary diversion. All patients had reflux and 3 chronic renal failure. Bowel segments employed were ileum (11 p), sigma (7 p) and ileocaecal (3 p). Average bladder capacity has been of 354 ml. (range: 200-510). The reflux disappeared in 14 patients and none of them had impaired renal function. In all patients has improved continence and 71% are dry.

Adolescent↗
PubMed2002

Endoscopic treatment of urinary incontinence in pediatric patients: 2-year experience with dextranomer/hyaluronic acid copolymer.

PURPOSE: We investigated the effectiveness of dextranomer/hyaluronic acid copolymer (dextranomer microspheres in sodium hyaluronan solution) as a treatment for urinary incontinence due to sphincter incompetence in children and adolescents. MATERIALS AND METHODS: Patients with urinary incontinence due to neurogenic and structural causes were given a transurethral injection of dextranomer/hyaluronic acid copolymer to increase bladder outlet resistance. Patients were assessed at 1, 3, 6, 12 and 24 months after injection using a validated questionnaire, 1-hour pad test, ultrasonography, cystography and cystometry. Patients who remained incontinent were offered repeat injections of dextranomer/hyaluronic acid copolymer up to a maximum of 3 injections. RESULTS: A total of 16 patients 8 to 22 years old were treated with dextranomer/hyaluronic acid copolymer, including 3 with neurogenic bladder, 8 with bladder exstrophy after 3-stage reconstruction, 4 with penopubic epispadias and 1 with urogenital sinus. Mean volume injected was 2.8 ml. (range 1.8 to 4.0) and mean number of injections received was 2.3 per patient. Dry time interval increased by 43 minutes (p <0.05) and functional bladder capacity increased by 34 ml. (p <0.05) at 6 months after treatment compared with pretreatment values. Improvement in both parameters was maintained at 12 months. At 6 and 12 months of followup 12 (75%) and 8 (50%) patients reported improvements in daytime and nighttime dryness, respectively. A slight decrease in continence parameters was observed in the 13 patients who completed the 24-month followup. No adverse events were reported and no upper urinary tract deterioration was observed as a consequence of endoscopic treatment within the 2-year followup period. CONCLUSIONS: Use of dextranomer/hyaluronic acid copolymer as a bulking agent to increase bladder outlet resistance improves symptoms in children and adolescents with incontinence of neurogenic and nonneurogenic origin.

Bladder Exstrophy↗
PubMed1978

Adenocarcinoma at the site of ureterosigmoidostomies for exstrophy of the bladder.

Adenocarcinoma of the colon is a rare complications of ureterosigmoidostomy done for exstrophy of the bladder. Only 13 cases have been previously reported, occurring from 10 to 46 yr after the procedure. In a series of 150 patients who have had ureterosigmoidostomies carried out at the Children's Hospital Medical Center, Boston, over the past 40 yr, 4 patients have been treated at our institution for this complication. The tumor was noted 20 to 32 yr after the initial procedure. Two patients are alive and well 1 and 4 yr after resection. The increasing incidence of this complication with advancing age will require careful observation and will undoubtedly influence the indications for the procedure. An additional question of great importance is how to evaluate and follow the thousands of patients who have had this procedure done at one time in their lives. It is suggested that an annual intravenous pyelogram is indicated in those patients with functioning ureterostomies and that a colonoscopy to the level of the anastomoses be carried out at 6-mo intervals in all patients. In those patients in whom the ureterosigmoidostomies have been taken down, a sleeve resection of that area of the colon should be strongly considered.

Adenocarcinoma↗
PubMed1984

Management of uncorrected exstrophy of the bladder in women.

A 28-year-old Hispanic woman presented with uncorrected exstrophy of the bladder and accompanying defects of the external genitalia. These defects were corrected in a single operation. The objectives of the operation and the rationale for choosing the specific methods used are discussed in detail.

Abnormalities, Multiple↗
PubMed1997

Long-term results of lower urinary tract reconstruction with the ceco-appendiceal unit.

PURPOSE: We report long-term results in 11 patients born with bladder exstrophy who underwent lower urinary tract reconstruction using a ceco-appendiceal unit. MATERIALS AND METHODS: Four boys and 2 girls underwent lower urinary tract reconstruction using an unaltered ceco-appendiceal unit. In 2 boys and 1 girl the bladder was replaced with a reservoir of terminal ileum, cecum and ascending colon, and the appendix was used as an orthotopic neourethra. In another 2 boys and 1 girl the bladder was augmented, while the appendix was used to create a stoma to the umbilicus. In 5 patients in whom the ceco-appendiceal junction was incompetent the cecum was plicated over the base of the appendix to reinforce the sphincteric mechanism. Four patients underwent augmentation with the appendix brought out as an umbilical stoma, and in 1 the bladder was replaced and the appendix was used as a neourethra. RESULTS: Six patients in whom the ceco-appendiceal junction was unaltered and 5 in whom it was plicated remain continent 5 to 11 and 2 to 7 years postoperatively, respectively. In the initial patient urinary incontinence developed due to high intraluminal pressure, which resolved after detubularization of the urinary reservoir. Another patient underwent revision of the abdominal stoma. CONCLUSIONS: The ceco-appendiceal unit may be used for continent lower urinary tract reconstruction. Ceco-appendiceal junction competence can be tested intraoperatively and the sphincteric mechanism may be reinforced as necessary. The appendix may be ectopically or orthotopically placed and used for intermittent catheterization.

Adolescent↗