Lower pole ureteral obstruction secondary to fecal impaction in an 8-year-old girl.

An 8-year-old girl presented with frequent, nonfebrile, urinary tract infections and a lifelong history of severe constipation. Ultrasound revealed bilateral duplicated renal systems, severe right upper pole hydronephrosis, and moderate left lower pole hydronephrosis. Computerized tomographic scan revealed a large, fecal-impacted colon. Diuretic renogram showed high-grade obstruction (T1/2 >30 minutes) of the right upper pole and left lower pole moieties. After administering an aggressive bowel preparation, a repeat diuretic renogram was performed, which revealed resolution of the left lower pole obstruction. Constipation has long been established as a contributing factor to urinary tract infections in children. However, fecal impaction causing ureteral obstruction in a child has not been reported. We report the case of an 8-year-old girl who had left lower pole ureteral obstruction secondary to fecal impaction.

A large animal model of bladder exstrophy: observations of bladder smooth muscle and collagen content.

PURPOSE: The development of adequate bladder capacity is not ensured in all patients with bladder exstrophy despite successful bladder closure and reconstruction. To determine the factors leading to the development and maturation of the exstrophic bladder we created a large animal model of exstrophy. We compared biopsies obtained from a cohort of experimentally induced exstrophic neonatal sheep bladders to those of normal control bladders and related the findings to a previously reported comparison of human neonatal normal and exstrophic bladders. MATERIALS AND METHODS: Bladder specimens of 7 newborn lambs with experimentally induced exstrophy were compared to specimens of 10 newborn control lamb bladders. All specimens were stained with Masson's trichrome as well as with specific monoclonal antibodies to types I and III collagen. Stained sections were then analyzed using a morphometric image analysis system to quantify the amounts of smooth muscle and collagen present. RESULTS: A significant increase in the ratio of collagen-to-smooth muscle was noted in exstrophic versus normal control bladders (p <0.05). This difference was similar to that in the previous study of neonatal human bladders. There was no significant difference in the ratios of types I and III collagen in the 2 groups of sheep bladders. This finding is different from that reported in the previous human studies. CONCLUSIONS: Overall changes in the ratio of smooth muscle-to-collagen in the sheep exstrophy model are similar to those in humans. However, the differences in collagen types I and III do not seem to be present.

Immunohistochemical description of nitric oxide synthase isoforms in human clitoris.

PURPOSE: Our aim was to identify and localize nitric oxide synthase isoforms in the human clitoris in support of the hypothesis that nitric oxide mediates erectile function in this organ. MATERIALS AND METHODS: Nitric oxide synthase immunohistochemistry studies specific for neuronal, inducible and endothelial isoforms of the enzyme were performed on human clitoral tissue obtained from 4 patients (3 with female pseudohermaphroditism and 1 with true hermaphroditism) at feminizing genitoplasty and from 1 phenotypically normal woman at autopsy. RESULTS: Neuronal nitric oxide synthase immunoreactivity was detected in nerve bundles and fibers coursing within the glans clitoris and corpora cavernosa of the clitoris, predominating in the latter tissue. Specific inducible nitric oxide synthase immunoreactivity was not identified. Endothelial nitric oxide synthase immunoreactivity was detected in vascular and sinusoidal endothelium of these tissues with a predominance in the glans clitoris. CONCLUSIONS: The presence and anatomical localizations of nitric oxide synthase isoforms in the human clitoris indicate that nitric oxide is generated in this organ. These data suggest that nitric oxide may be involved in the erectile physiology of the clitoris as a modulator of clitoral smooth muscle activity. Functional studies are required to support this hypothesis.

Prevalence and repair of inguinal hernias in children with bladder exstrophy.

PURPOSE: We delineated the prevalence, recurrence rates and optimal treatment of inguinal hernia in the exstrophy population. MATERIALS AND METHODS: Of 181 children with exstrophy followed at our hospital inguinal hernias developed in 121 (66.8%). RESULTS: In a 12-year period inguinal hernias developed in 81.8% of the boys and 10.5% of the girls. In 18.2% of the cases the hernia was repaired via a preperitoneal approach at the same time as exstrophy closure. The remaining patients underwent an inguinal operation. Most patients had a wide defect at the internal ring in addition to a patent processus vaginalis. The overall recurrence rate was 8.3%. The incidence of synchronous or asynchronous bilaterality was 81.8%. CONCLUSIONS: Children with bladder exstrophy should be carefully examined for inguinal hernias before bladder closure. If a unilateral hernia is present, the contralateral side should be explored. Careful preperitoneal repair should emphasize repair of the internal ring.

Submucosal bladder neck injections of glutaraldehyde cross-linked bovine collagen for the treatment of urinary incontinence in patients with the exstrophy/epispadias complex.

During the last 7 years 19 patients underwent 33 transurethral injections of glutaraldehyde cross-linked bovine collagen into the bladder neck for stress incontinence. Of the 15 patients 14 have classic bladder exstrophy, 3 have complete male epispadias and 2 have cloacal exstrophy. The procedure was performed after a Young-Dees-Leadbetter bladder neck reconstruction in 15 patients and before it in 4. Injections were repeated in 10 patients after a mean of 12 months. After a mean followup of 26 months (range 9 to 84) improvement of continence was noted in 10 of 19 patients (53%) of whom 4 have significant improvement. Of the 8 patients whose condition failed to improve after collagen injections 6 underwent additional successful surgery to achieve urinary continence. Of the 10 patients who underwent repeated collagen injections 9 (90%) had additional improvement. Although there were no complications related to the injected collagen itself, postoperative complications developed in 2 patients. Submucosal injection of collagen to the bladder neck is simple and safe, and has a reasonable success rate. Thus, it may be used to improve continence in patients with the exstrophy/epispadias complex who lack full control after appropriate reconstructive surgery.

Applications of osteotomy in the cloacal exstrophy patient.

During the last 18 years we treated 22 patients with cloacal exstrophy of whom 13 were referred for further treatment after initial treatment elsewhere. One patient underwent cystectomy with ileal conduit urinary diversion soon after birth and 9 of the remaining 21 underwent initial closure without osteotomy. Of these 9 patients significant complications developed in 8 (89%) after bladder closure, including dehiscence in 6 (1 underwent 2 unsuccessful closures), a vesicocutaneous fistula and postoperative ventral hernia in 1, and bladder prolapse in 1. In contrast, complications developed in only 2 of the 12 patients (17%) who underwent osteotomy at the time of initial closure, including bladder dehiscence in 1 and significant prolapse in 1. Patients who underwent osteotomy and those who did not were similar in terms of the size of omphalocele, presence of myelomeningocele and time of primary closure. We also found that osteotomy or failed closure has no effect on the eventual continence of cloacal exstrophy patients. While osteotomy is not the only variable involved in successful cloacal exstrophy closure, our results indicate the need for osteotomy in these patients to increase the success rate at the time of initial bladder closure.

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.

Complete male epispadias: genital reconstruction and achieving continence.

We report on 15 patients who were primarily treated for complete male epispadias at our institution since 1975. Repair of epispadias was performed using a modified Young urethroplasty in 13 patients and a Cantwell-Ransley urethroplasty in 2. In addition, 2 patients underwent a Cantwell-Ransley urethroplasty with chordee repair after a previous Young urethroplasty failed. Bladder capacity increased from a mean of 50 cc before repair to 92 cc after urethroplasty. A urethrocutaneous fistula developed in 6 cases, including 5 Young repairs and 1 Cantwell-Ransley. Three fistulas resolved spontaneously and there were no urethral strictures. Bladder neck reconstruction was performed in 11 patients. Time to initial continence ranged from 21 days to 6 months (mean 3 months) postoperatively. All patients attained daytime continence in a mean of 9 months (range 21 days to 24 months) after bladder neck reconstruction, including 9 of 11 (82%) who achieved total day and night continence. Mean followup was 7 years (range 1 to 10). Modern treatment of complete male epispadias allows for an excellent genital appearance and achievement of urinary continence.

The application of continent urinary stomas to bladder augmentation or replacement in the failed exstrophy reconstruction.

OBJECTIVE: To determine the application and success of continent urinary stomas in the failed bladder exstrophy reconstruction. PATIENTS AND METHODS: A total of 35 patients underwent construction of a continent urinary stoma during bladder replacement or bladder augmentation between 1987 and 1993. The continent stoma was constructed with appendix (19 patients), tapered ileum (2), Benchekroun (12), Indiana (1) and Mainz type stoma (1). RESULTS: Three patients required revision of their continent stoma, one for incontinence and two for difficulty in catheterization. Overall, 95% experienced daytime continence on an intermittent catheterization regimen, while 90% were dry at night. The upper tract remained normal in 34 of 35 patients. CONCLUSIONS: While the Benchekroun technique is reliable in producing continence, difficulty with catheterization has been a chronic problem due to stomal stenosis. Our positive experience with using the appendix as a catheterizable conduit into either the bladder template or bowel segment has led us to prefer this method of continent stoma construction in the patient with a failed exstrophy. In the absence of an appendix, our preference is a tapered ileal segment. The failed exstrophy reconstruction can be salvaged with a continent stoma and substitution or augmentation cystoplasty in most patients, thus avoiding urinary diversion.

Percutaneous embolotherapy of adolescent varicocele: results and long-term follow-up.

PURPOSE: The authors evaluated the technical success and immediate and long-term results of percutaneous varicocele embolotherapy in the adolescent population. PATIENTS AND METHODS: Fifty-nine adolescent patients were referred for outpatient spermatic venography and possible varicocele embolotherapy. Embolization was attempted with use of detachable balloons, coils, "sandwiched" dextrose, or a combination of these techniques. Data regarding follow-up were obtained through telephone interviews or mailed questionnaires. RESULTS: The technical success rate for spermatic vein occlusion was 90%. Follow-up, obtained in 79% of the patients, ranged from 6 months to 8.75 years (mean, 4 years). Thirty-nine of 42 patients (93%) reported disappearance (n = 31) or only a slight, asymptomatic residual varicocele (n = 8). Three patients reported a recurrence of their varicocele. Complications occurred in three of 59 cases (5%), none had any long-term sequelae. In six cases, embolization was not feasible because of multiple collateral vessels or venous spasm. CONCLUSIONS: Given the convenience of performing the procedure on an outpatient basis, the rapid recovery time, and long-term success and complication rates comparable to those with surgical ligation, we believe spermatic venography and percutaneous embolization is the treatment modality of choice for adolescent varicocele.

Techniques to create continence in the failed bladder exstrophy closure patient.

We reviewed retrospectively 315 patients with bladder exstrophy treated at our hospital between July 1976 and April 1992 to assess the outcome of those who failed primary closure of the bladder. Of the patients 47 required reclosure of the bladder, including 28 who have undergone a procedure to restore urinary continence. Methods used to achieve dryness included bladder neck reconstruction in 18 patients, bladder neck reconstruction along with augmentation in 4, augmentation alone in 4, repeat bladder neck reconstruction in 1, and reclosure with creation of a continent stoma and augmentation in 1. Nine of 18 patients who underwent primary bladder neck reconstruction are dry on intermittent catheterization, while 8 of the remaining 9 are dry and voiding without catheterization. Four patients who underwent primary bladder neck reconstruction and augmentation, and 4 who underwent augmentation after bladder neck reconstruction are dry on intermittent catheterization. The patient who underwent reclosure, bladder augmentation and creation of a continent abdominal stoma is dry on intermittent catheterization. Virtually all patients who failed the initial closure and later bladder neck reconstruction for continence require augmentation and intermittent catheterization to remain dry. Of 28 patients who underwent salvage procedures only 1 had upper tract changes. With attention to detail and the use of a variety of reconstructive techniques children who have failed exstrophy closure can achieve continence and have stable renal function.

Complications of paraexstrophy skin flaps in the reconstruction of classical bladder exstrophy.

We reviewed the cases of the exstrophy/epispadias complex treated at our institution between July 1976 and April 1992. A total of 78 patients was identified who had paraexstrophy skin flaps used in the bladder closure, of whom 31 (40%) had a complication as a result of the flaps. The main complication encountered was a urethral stricture where the paraexstrophy skin flaps joined the urethral plate area. Multiple maneuvers were undertaken to correct these problems, including direct vision internal urethrotomy (12 cases), multiple urethral dilations (4), open revision (3) and full thickness skin grafts (5). Seven patients had such a complex stricture situation that they required either continent urinary diversion (5), colon conduit diversion (1) or cutaneous ureterostomy (1), the latter 2 patients having undergone vesicostomy elsewhere before referral. Of the remaining 24 patients who did not undergo a diversionary procedure 12 have undergone an epispadias repair and bladder neck reconstruction, 7 underwent an epispadias repair and 5 await further treatment. Freedom from complications in the initial closure of exstrophy significantly improves the chances of successful reconstruction. The avoidance of problems leading to obstruction, infection, hydronephrosis and reflux nephropathy will provide better kidneys regardless of bladder suitability for function or augmentation. Our use of paraexstrophy flaps has decreased but when they are required, special care in design, placement and followup is advised to avoid complicating strictures and their sequelae.

The cephalotrigonal reimplant in bladder neck reconstruction for patients with exstrophy or epispadias.

A modified technique of ureteroneocystostomy with bladder neck plasty was used in 36 of 75 patients undergoing staged repair of bladder exstrophy or epispadias between 1986 and 1992. This procedure entails mobilizing the ureter while preserving the trigonal hiatus as with the cross-trigonal technique. The distal ureteral segments are directed superiorly toward the bladder dome rather than across the mid line. Of 75 patients 36 underwent cephalotrigonal reimplantation and 39 had a conventional cross-trigonal reimplant. Continence rate was 77% in the patients who underwent cephalotrigonal reimplantation and 72% in those who had a cross-trigonal reimplant. No patient had ureteral obstruction or vesicoureteral reflux. The ureter in exstrophy patients enters the bladder from an inferior position within the true pelvis. Directing the ureter superiorly rather than across the mid line provides a more gradual course through the hiatus and submucosal tunnel. The cranial course of the distal ureter frees more of the trigone for use in the rolled segment of the bladder neck and provides more muscle area for the tube. This is especially important in the patient in whom the distance between the mid prostate and trigone is particularly short.

Complete genitourinary reconstruction in female epispadias.

While female epispadias is a rare congenital anomaly, the treatment of complete epispadias in the female patient does not significantly differ from that of their male counterparts, although the female defect can be overlooked as a cause of incontinence. Attention must be given to the creation of an adequate urethral channel so that an adequate bladder capacity can be achieved and eventual bladder neck plasty can be performed. Finally, attention must be given to the reconstruction of the external genital defect. During the last 7 years 11 female patients with complete epispadias were treated. Of these patients 4 were referred from elsewhere and 3 had failed a previous procedure (2 had recently undergone external genital and urethral reconstruction, 1 underwent urinary diversion after multiple failed bladder neck procedures). Nine patients underwent bladder neck plasty: 5 are completely continent day and night, 3 are dry for greater than 3 hours during the day, and 1 is dry for only 1 to 3 hours during the day and wet at night, for an overall continence rate of 87.5%. Our experience with these patients has taught us that the bladder in this condition is much like that found in complete male epispadias. Therefore, creating a urethral outlet with sufficient length and resistance along with simultaneous reconstruction of the external genitalia allows for bladder regrowth, thus, facilitating achievement of greater volumes and bladder neck reconstruction with an excellent chance of success.

The failed exstrophy closure: strategy for management.

In the last 7 years, 29 boys and 11 girls have been referred with failure of their exstrophy closure; 38 patients had classic bladder exstrophy and 2 had cloacal exstrophy. Reclosure was performed for complete bladder dehiscence in 28 cases and for significant bladder prolapse in 10. Two patients underwent revision of the posterior urethra after primary closure due to a severe urethral stricture secondary to the use of para-exstrophy skin flaps. Six patients underwent posterior iliac osteotomy at the time of initial bladder closure and in 34 no osteotomy was performed. Thirty-seven patients underwent either posterior iliac osteotomy (15) or anterior innominate osteotomy (22). Epispadias repair together with reclosure of bladder exstrophy was done in 20 boys. The upper tract has remained normal in all patients. Fourteen have undergone subsequent bladder neck reconstruction. Seven patients have undergone simultaneous bladder neck reconstruction and augmentation cystoplasty and 1 has undergone augmentation cystoplasty and Mitrofanoff procedure; all are dry on intermittent self-catheterisation. The failed exstrophy reconstruction represents a formidable dilemma. However, a well planned reconstruction including osteotomy (even if previously performed), reclosure with or without epispadias repair or revision of the urethra can be performed with an excellent chance of proceeding with staged reconstruction.

An evaluation of 646 stone patients treated on the HM4 extracorporeal shock wave lithotriptor.

Extracorporeal shock wave lithotripsy (ESWL) has become the preferred treatment modality for the majority of urinary tract calculi. This form of treatment boasts excellent patient acceptance and has significantly reduced the need for surgical intervention. An evaluation of our first 646 patients undergoing 722 ESWL treatments at Walter Reed Army Medical Center revealed an overall stone-free rate at 1 and 3 months of 52% and 79%, respectively. The exact location of each stone within the kidney and ureter further determined the stone-free rates at 1 and 3 months. Stone-free rates at 3 months ranged from 89% for renal pelvic stones to 64% for lower pole caliceal stones. Upper third ureteral stones treated in situ without stenting resulted in a 74% stone-free rate at 3 months. This rate increased to 85% when these patients were stented and treated in situ. Although statistically insignificant, when the upper third ureteral stones were manipulated into and treated in the renal pelvis (31% of our patient population) the 3-month stone-free rate increased to 87%. Attempts were made to stent all ureteral stones before treatment. The stone-free rate at 3 months was 86% for mid third ureteral stones and 81% for lower third ureteral stones. The overall retreatment rate was 11.8% with a complication rate of 7.6% and a 6.3% post-ESWL intervention rate (open or percutaneous surgery, stent or percutaneous nephrostomy placement).

Use of glycosylated hemoglobin to identify diabetics at high risk for penile periprosthetic infections.

We report an 18-month prospective study of 90 patients undergoing penile prosthesis implantation to evaluate a possible cause-and-effect relationship between degree of diabetic control and the risk of infection complicating the operation. Long-term diabetic control was objectively evaluated by measurement of the glycosylated hemoglobin of the patient, which is known to provide an objective value for degree of control for the preceding 60 to 90 days. Of 90 patients 5 (5.5%) had a periprosthetic infection requiring explantation and all infections occurred in the 32 diabetics (36%) in the population (p less than 0.009). Of the 32 diabetics 13 (41.1%) were poorly controlled with time as demonstrated by a glycosylated hemoglobin level of greater than 11.5% and 4 of the infections occurred in this group. Of the 19 remaining controlled diabetics (glycosylated hemoglobin level less than 11.5%) only 1 infection occurred. Therefore, infection occurred in 31% of the poorly controlled versus 5% of the adequately controlled patients (p less than 0.0003). Measurement of glycosylated hemoglobin values appears to be a useful tool to evaluate diabetic patients before implantation of a penile prosthesis. Patients with a glycosylated hemoglobin level of 11.5% or greater should be more optimally controlled before undergoing implantation in an effort to avoid infectious complications.

Nonsurgical management of primary vesicoureteral reflux in complete ureteral duplication: is it justified?

We reviewed the treatment of 56 children with vesicoureteral reflux and complete duplication of the collecting system, including 14 who had complete bilateral duplication. A total of 70 refluxing duplicated systems was analyzed. Of the patients 18% demonstrated spontaneous resolution of reflux within 42 months, 23% are currently stable on prophylactic antibiotics and 57.1% underwent surgical correction. Spontaneous resolution of reflux occurred in 58% of the children with grades I to III/V reflux. In comparing the group with reflux and duplication to a group with reflux into single systems, we conclude that the patients with duplication and lower grades of reflux can be managed nonoperatively, while infection is prevented with antibiotic prophylaxis.