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Purpose: A major goal of bladder exstrophy management is urinary continence, often using bladder neck reconstruction. We report our experience with bladder neck reconstruction after complete primary repair of exstrophy., Materials and Methods: Patient history, ultrasound, voiding cystourethrogram, examination using anesthesia and urodynamics were performed during a prospective evaluation. Continence was assessed using the International Children's Continence Society classification and the dry interval. Bladder capacity was measured by examination using anesthesia, voiding cystourethrogram and/or urodynamics. Urodynamics were also done to assess bladder compliance and detrusor muscle function., Results: From 1994 to 2010 we treated 31 male and 15 female patients with bladder exstrophy after complete primary repair of exstrophy. Of patients 5 years old or older bladder neck reconstruction was performed after complete primary repair in 9 of 21 males (43%) and in 3 of 11 females (27%) at a mean age of 6.3 and 8.1 years, respectively. By the International Children's Continence Society classification 6 of 12 patients (50%) were continent less than 1.5 years after bladder neck reconstruction and 2 of 9 (23%) were evaluable 1.5 years or greater after reconstruction. Median bladder capacity was 100 ml before, 50 ml less than 1.5 years after and 123 ml 1.5 years or greater after bladder neck reconstruction. Three males and 2 females emptied via an appendicovesicostomy. Two boys underwent augmentation., Conclusions: In our experience most patients with bladder exstrophy require bladder neck reconstruction after complete primary repair of exstrophy. The need for reconstruction is more common in males. Our rates of bladder neck reconstruction after complete primary repair of exstrophy and of continence after bladder neck reconstruction are similar to those in other reports., (Copyright © 2011 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

Purpose: The new technique of complete primary repair of bladder exstrophy has offered the promise of improved bladder functional outcomes and yet longitudinal followup in patients with exstrophy who have undergone this form of closure is sparse. We present our median 5-year data on patients who have undergone complete primary repair of bladder exstrophy., Materials and Methods: The records of patients who underwent complete primary repair of bladder exstrophy were analyzed retrospectively. Patients were followed prospectively using case report forms, radiological and clinical data, and semistructured parental and patient interviews. We reviewed parameters including clinical events, scarring on renal scan, vesicoureteral reflux, surgical procedures, urodynamic studies, urinary and fecal continence status, and episodes of urinary tract infection., Results: From 1994 to 2007 complete primary repair of bladder exstrophy was performed in 32 patients. Of these patients 28 underwent closure within 72 hours of life, including 20 males and 8 females, and 4 underwent it after 72 hours at ages 7 days to 12 months. One patient underwent complete primary repair of bladder exstrophy elsewhere. Median followup was 5 years (range 1 to 13). In 32 patients a total of 193 surgical procedures were done, of which 40% were minor endoscopic cases. Six of 23 males (26%) underwent bladder neck reconstruction at a median age of 4.5 years (range 4 to 10). Two boys and 1 girl received Deflux injection to the bladder neck at ages 3 to 5 years. To date no patient has undergone bladder augmentation. Nine patients (28%) had 1 to 4 episodes of pyelonephritis and 6 had cortical defects on renal scan. A total of 30 patients were voiding or incontinent via the urethra. One patient performed clean intermittent catheterization via the urethra and 1 performed it via appendicovesicostomy. All 4 children who were at least 6 months from bladder neck reconstruction after complete primary repair of bladder exstrophy had urinary continence periods of 2 to 3 hours or greater. Parents did not report any fecal incontinence or soiling in children older than 4 years. Relative to males the females had better urinary continence and a decreased need for bladder neck reconstruction., Conclusions: Complete primary repair of bladder exstrophy has been shown to be safe and efficacious. Prospective followup in this small number of evaluable patients reveals that continence periods of more than 2 hours are possible in patients after bladder neck reconstruction. Long-term followup in patients after complete primary repair of bladder exstrophy continues to be necessary to establish the long-term effects of this procedure.

Purpose: We assessed bladder growth and dynamics following complete primary repair of bladder exstrophy (CPRE) compared to the staged approach., Materials and Methods: We reviewed the records of 16 boys and 7 girls who underwent CPRE within 3 days of life from 1996 to 2004 and compared them to the records of 8 boys and 6 girls treated with a staged repair from 1979 to 1996. Screening methods included voiding cystourethrogram, radionuclide cystogram and urodynamic study. We estimated growth curves for bladder capacity following repair in each group, and compared percent predicted bladder capacity (PPBC), compliance and detrusor overactivity between the CPRE and staged repair groups following bladder neck reconstruction., Results: Bladder capacity in the staged repair group was 69.8 ml (95% CI 46.7-104.4) immediately after bladder neck reconstruction and increased by 15.0% per year thereafter (95% CI 6.2-24.5, p = 0.002). In the CPRE group bladder capacity was 29.0 ml (95% CI 21.3-39.5) initially and increased by 28.9% per year thereafter (95% CI 17.4-41.5, p <0.001). PPBC started at 45.6% (95% CI 35.7-55.5) and increased 1.2% per year (95% CI -1.1-3.5, p = 0.29) following repair for all genders and surgery groups. Compliance was 124.4% (95% CI 22.6-310.7, p = 0.01) greater in the CPRE group at all times following repair. Detrusor overactivity was present in 0 of 19 patients in the CPRE group and 6 of 13 (46%) in the staged group (exact p = 0.002)., Conclusions: Within the CPRE group bladder stability was universal, and sphincter electromyography was normal suggesting no neuromuscular compromise of the pelvic floor. At early followup, our results suggest that PPBC is equivalent irrespective of gender or management. Further objective evaluation is needed in both groups.

Purpose: To our knowledge nephrogenic adenoma, an unusual benign metaplastic lesion of the urothelium, has not been reported previously following urethral reconstruction using bladder mucosa. Histopathological features, etiological factors and clinical features are discussed., Materials and Methods: We reviewed the records of 117 patients who underwent hypospadias repair with free grafts of bladder mucosa from 1982 to 1995., Results: Five children 6 to 11 years old and a 39-year-old man had nephrogenic adenoma of the urethra. Hematuria or dysuria was the usual reason for presentation. The lesions were located at the site of anastomosis between the graft and proximal urethra in 3 cases and the distal graft in 2, and they involved the whole graft in 1. Successful treatment consisted of endoscopic resection of the polyps in 4 patients, while open surgical correction was performed in 2, requiring complete excision of the previous graft in 1 and segmental resection in 1., Conclusions: The gross appearance of the lesion may be confused with that of transitional cell carcinoma or clear cell adenocarcinoma of the urethra, necessitating biopsy to rule out malignancy. Endoscopic management is possible for small tumors, although severe nephrogenic adenoma involving most of the reconstructed urethra may require complete resection of the graft and creation of a new urethra.

Gastrocystoplasty was performed in 29 female and 14 male patients 1 to 23 years old (average age 11 years). Urodynamic studies were completed in 20 patients preoperatively and within 6 months postoperatively. When postoperative studies were compared with the preoperative evaluation, it was noted that overall capacity increased an average of 220% (range 20 to 750%). Compliance also improved dramatically postoperatively. Bladder pressures increased from a mean volume of 34% of capacity preoperatively to 73% of the post-augment capacity. The average maximum filling pressure decreased by 32%, from 57 to 39 cm. water. Uninhibited contractions resolved completely in 6 of 10 patients who had them preoperatively. No one had uninhibited contractions postoperatively. Cyclical contractions of the stomach segment not exceeding 50 cm. water developed in 6 patients. The gastric patch seems to be an adequate source for improving the dynamics of a poorly functioning detrusor muscle in most patients. When a diamond-shaped patch of stomach is harvested and added to the bladder a spherical configuration is created, enhancing the storage properties. This result is in sharp contrast to other bowel segments, which must be detubularized to produce a suitable reservoir. The long-term effects of gastric augmentation remain to be determined.

In 12 of 264 children treated with enterocystoplasty 15 spontaneous perforations occurred. Of the 12 children 9 had myelodysplasia. All segments of the gastrointestinal tract were used for the augmentation and most were detubularized. Surgery to increase bladder outlet resistance was done in 8 cases. At the time of each perforation 9 children had sterile cultures, however, 3 died of overwhelming sepsis. Presenting signs included abdominal pain in 8 cases, septic shock in 4 cases and shoulder pain in 4 older myelodysplastic children with diaphragmatic irritation from escaping urine. Cystography demonstrated a leak in 10 of 11 cases. Urodynamic studies revealed good compliance with low maximum filling pressure in 8 of 10 children. Hyperreflexia was noted in only 5 cases and outlet resistance greater than 85 cm. water was demonstrated in 5. Histological analysis showed changes in the bowel wall consistent with ischemia but suture granulomas were present in areas adjacent to the perforation site or thinned areas in biopsy or autopsy specimens. In addition to the theory that overdistention may cause enterocystoplasty perforation, current detubularization techniques may produce areas of relative ischemia, which become accentuated when the augmented bladder is distended beyond a reasonable volume.

Since 1985, 7 children 2 to 17 years old with urinary incontinence from cloacal exstrophy underwent bladder augmentation and creation of a small bowel nipple at the bladder outlet. There were 3 genetic female and 4 genetic male patients being reared in the female role. In 3 early cases augmentation was done with small bowel, while gastric augmentation was used in 3 later cases and 1 had both types. Six patients became completely dry and they empty by intermittent self-catheterization. In the youngest patient the operation failed because the nipple became infarcted and disappeared. Reoperation will be performed after at least 1 year has passed. In cloacal exstrophy if there is insufficient local tissue to create a bladder neck and urethra, a reversed, catheterizable bowel nipple may be an option to consider.

A personal experience with 105 cloacal malformations is described, showing the wide range of anatomy that can occur with the urogenital sinus, the vagina(s), and the rectum, as well as in the external features of the perineum. Follow-up is presented on 98 patients, excluding 4 who died preoperatively, 2 who are preoperative, and 1 lost to follow-up but never repaired. Fifty-seven patients had vesicoureteral reflux. Data are given on urinary control, bowel control, and sexual function for those who are now adults. Two have had babies. Operation often requires multiple positions, including the posterior sagittal approach, laparotomy, and lithotomy position. Isolated rectal pull-through should never be performed in these patients, because the urogenital aspects of the malformations are the most difficult to repair and are the most life-threatening to the patient. Six recent cases are presented to illustrate the breadth of the cloacal spectrum. In most of these patients a satisfactory functional result can be achieved for urinary and bowel control as well as sexual function.

From 1977 to 1989 bladder augmentation was performed in 56 male and 73 female patients from 1 to 35 years old (average age 12.7 years). In 59 cases augmentation was performed as part of an undiversion operation. Indications for augmentation included neurogenic bladder, severely scarred bladder from prior operations, noncompliant bladder after previous treatment of severe urethral valves, small bladder in former exstrophy patients, epispadias with a small bladder, cloacal exstrophy, and small, noncompliant bladder after therapy for cancer, trauma, cloaca and miscellaneous conditions. We used 145 bowel segments, since 16 patients had 2 bowel segments. Segments included cecum in 65 cases, sigmoid in 46, small bowel in 24, stomach in 4 and left colon in 1. The most common complication was stones. Detubularized bowel for augmentation of small noncompliant bladders allows functional reconstruction in a wide range of urological disorders that were formerly treated by diversion. Reconstruction is possible in many previously diverted patients if bladder augmentation is used.

We evaluated urodynamically 41 patients with posterior urethral valves because of signs or symptoms of incontinence (35), frequency (3), hydronephrosis (2) and infection (1). Findings included normal urodynamic evaluations in 3 patients, 2 had high voiding pressures secondary to outlet resistance and 1 had incontinence on the basis of external urethral sphincter damage. In the remainder 3 patterns of bladder dysfunction were identified. Myogenic failure with overflow incontinence occurred in 14 patients. In this group clean intermittent catheterization or Valsalva's voiding was used for emptying. Hyperreflexic bladders were seen in 10 patients. Pharmacological suppression of instability was effective in 5 of 7 patients treated; 1 required bladder augmentation. Eleven children had a small capacity bladder and poor compliance. Post-void residuals were low and these bladders were generally but not always stable. Pharmacological bladder relaxation was successful in 3 patients, 3 underwent augmentation, 1 did well with alpha-agonists and followup is unavailable on the other 4. These 3 patterns of bladder dysfunction represent an overlapping constellation of residual urodynamic abnormalities due to previous bladder outlet obstruction. Individual patients may show facets of several types of dysfunction associated with 1 predominant pattern.

Many children with severe urologic problems have been treated by urinary diversions, often after unsuccessful operations to correct the original pathology. Urinary diversion should be avoided when possible, since often it is accompanied by chronic infection and quality of life is less good than it could be. Most diversions can be prevented by use of other alternatives including 1) reimplanting 1 ureter and transureteroureterostomy of the other, 2) psoas hitch, 3) wide mobilization and downward displacement of the kidney and ureter, 4) use of bowel as ureter, 5) cecal cystoplasty and 6) early repair of complex pathology. Autotransplantation is probably seldom indicated in childhood.

A graft of bladder mucosa was used to construct the urethra in 35 males. Most were complicated hypospadias cases. In all patients there was insufficient prepucial or penile shaft skin available to use for making a substitute urethra. The bladder mucosa worked well in every case. There were no fistulas. There was no case with stenosis of the anastomosis between the graft and the patient's own urethral tissue. The one possible disadvantage encountered in using bladder mucosa was its tendency to become edematous and irritated if the graft was redundant at the meatus, a situation that is not encountered with grafts made from prepucial skin or skin taken from a remote area. Revision of the meatus was required in six patients. It is the experience of the authors that a free graft that is covered by two well vascularized layers, subcutaneous tissue, and skin, fares as well or better than a graft with a vascular pedicle.

Thirty-two children who had undergone previous urinary diversion were operated upon to refunctionalize the bladder. In 24 the diversion had been considered permanent, and in eight, temporary. Success in these procedures suggests that many young patients deserve a second look for possible "undiversion."