Examining trends in the treatment of ureterocele yields no definitive solution.

INTRODUCTION: The surgical management of ureteroceles is extremely variable. Some have hypothesized that if these patients were treated with 'definitive' staged surgical intervention, the need for further revision surgery would be eliminated. OBJECTIVE: The present study sought to determine if the rate of revision surgery differed among patients who have undergone different surgical approaches for the ureterocele complex. STUDY DESIGN: A large retrospective chart review was conducted, identifying all patients having undergone ureterocele surgery at a single institution over the past 41 years. The cohort was divided into four groups based on surgical approach: upper tract approach (UTA), lower tract reconstruction (LTR), simultaneous upper and lower tract approach (ULTA), and staged lower tract reconstruction (SLTR). Demographics, the presence of preoperative/postoperative VUR, postoperative morbidity and the need for revision surgery were compared using the Chi- squared test, Fisher's exact test, Kruskal-Wallis test, Mann-Whitney U test (Bonferroni correction), logistic regression modeling and survival analyses (Kaplan-Meier and Cox proportional Hazards regression with unplanned revision operation as the outcome event). RESULTS: Between 1969 and 2010, 180 patients were identified as having undergone surgical management of ureteroceles, of which 120 had complete demographic data available for analysis. The median age at the time of initial surgical intervention was 5.8 months and the majority of patients (83.3%) were female. The median follow-up was 33.1 months. Surgical management was as follows: 18 (15.0%) patients underwent UTA, 47 (39.2%) underwent LTR, 23 (19.2%) underwent ULTA, and 32 (26.6%) underwent SLTR. Among these groups, the only difference in median age was between the LTR and SLTR groups (6.3 months vs 3.7 months, P=0.012). Additional revision surgery was required in: nine (50.0%) of UTA, ten (21.3%) of LTR, four (17.4%) of ULTA, and three (9.4%) of SLTR. The only statistically significant difference in unplanned revision surgery was noted in the UTA group versus each of the other groups with VUR as the predominant indication (88.9%). The likelihood of requiring revision surgery in comparison to the SLTR group was significantly increased in the UTA group (OR 9.67, CI 2.15-43.56), but not in the LTR (OR 2.61, CI 0.66-10.37) or the ULTA group (OR 2.04, CI 0.41-10.13). Obstruction, recurring UTIs and VUR were the main indications for revision surgery overall. DISCUSSION: There is a large body of literature examining the surgical management of ureteroceles. It most recently primarily focuses on an endoscopic approach to the lower tract. The present retrospective review examined the need for re-operative intervention by comparing four different surgical approaches, and found that there is no panacea. Although heminephrectomy (UTA) was a definitive procedure in some patients without reflux at presentation, many who underwent heminephrectomy, went on to require later bladder surgery for either recurrent UTI or persistent reflux. The present study has multiple limitations. Although VUR was an indication for revision surgery in the early part of the series, the current treatment of VUR is not necessarily as stringent. In addition, no distinction was made between an orthotopic or ectopic ureterocele, although some authors have reported differing outcomes in these two groups. However, it is felt that given the large data set of a relatively uncommon condition, the lack of superiority of one approach is apparent. CONCLUSION: There is no definitive surgical repair for the ureterocele complex. All groups except UTA had statistically similar rates of revision surgery. The widespread variability in current management echoes the lack of one superior approach found in this comprehensive series.

Is routine karyotyping necessary in the evaluation of hypospadias and cryptorchidism?

PURPOSE: The incidence of intersex states has been reported to be 27% to 100% in patients with hypospadias and cryptorchidism, and routinely determining karyotypes has been recommended. This incidence seems much higher than in our experience. We reviewed the records of patients with hypospadias and/or chordee plus cryptorchidism as well as those referred with ambiguous genitalia to determine whether these findings were associated with a high incidence of chromosomal abnormalities and whether they warrant routine karyotype screening. MATERIALS AND METHODS: We reviewed the records of patients with undescended testis plus hypospadias and/or chordee, and those with ambiguous genitalia who presented between 1986 and 1999. Patients without karyotype determination, and those with iatrogenic cryptorchidism, retractile testes, congenital adrenal hyperplasia or female-appearing external genitalia were excluded from study. Meatus and testis locations at surgery, and associated Müllerian structures and medical conditions were documented. Fisher's exact test was done to determine statistical significance. RESULTS: Of the 113 patients whose records matched study inclusion criteria only 48 had complete anatomical, karyotypic, pathological and radiographic information available. Eight patients (16.7%) had chromosomal abnormalities, including 2 (4.2%) with karyotypic intersex disorder and 6 (12.5%) with autosomal chromosomal abnormalities. There were persistent Müllerian structures in 2 patients (4.2%) with a normal 46 XY karyotype. As described by a staff pediatric urologist, 20 patients (41.7%) had ambiguous genitalia and 8 of the 48 (16.7%) had nonpalpable cryptorchidism. Ambiguous genitalia were associated with chromosomal abnormalities, in 4 of the 20 cases, including 2 karyotypic intersex cases. Only 3 patients with ambiguous genitalia had nonpalpable gonads and 1 with karyotypic intersex disorder had a nonpalpable gonad that involved the testicular elements only. The incidence of autosomal chromosomal abnormalities was not significant (2-tailed Fisher's exact test p <==0.05) and the incidence of karyotypic (autosomal or sex) abnormalities was not significant compared with hypospadias (p = 0.3), genital ambiguity (p = 0.7) or cryptorchidism (p = 0.69), including nonpalpable testis (p = 1). When patients had karyotypic abnormalities, they were more likely to have proximal hypospadias (57.2%), palpable cryptorchidism (62.5%) and ambiguous genitalia (50%). CONCLUSIONS: Most patients who present for the evaluation of hypospadias, chordee and undescended testis have a normal karyotype. Routine karyotype investigation of all patients with hypospadias, chordee and cryptorchidism does not seem warranted. If karyotypic intersex abnormalities are identified, those patients are more likely to have ambiguous genitalia, especially those with perineal hypospadias and cryptorchidism.

Ipsilateral ureteroureterostomy for the treatment of vesicoureteral reflux or obstruction associated with complete ureteral duplication.

PURPOSE: We reviewed our experience with ureteroureterostomy as definitive treatment for vesicoureteral reflux or obstruction associated with ureteral duplication to determine the efficacy and morbidity of this procedure and identify factors that affect outcome. MATERIALS AND METHODS: We performed a total of 100 ureteroureterostomies in 94 children with an average age of 28 months during the 23-year period ending May 1999. Four patients (4 kidneys) failed to return for postoperative evaluation. Followup was 2.5 to 206 months (mean 33). Indications for surgery were vesicoureteral reflux in 53 cases, obstructing ureterocele in 19, ureterocele with upper pole reflux in 4, an ectopic obstructed upper pole ureter in 18 and other in 2. In 23 kidneys ureteroureterostomy was combined with reimplantation of the recipient ureter. Surgery was considered successful when postoperative imaging revealed no reflux or obstruction and a patent ureteroureterostomy anastomosis. RESULTS: Ureteroureterostomy with or without ureteroneocystostomy was successful for treating 94% of kidneys, including 51 of 53 with reflux, 21 of 23 (91%) with ureterocele and 17 of 18 (94%) with an ectopic obstructed upper pole ureter. Significant ureteral size disparity, defined as a donor ureter diameter greater than 2-fold that of the recipient ureter, was noted in 69 kidneys. The most common complication (13 patients) was prolonged output from the Penrose drain. However, this complication did not result in a failed procedure in any case. CONCLUSIONS: Ipsilateral ureteroureterostomy is safe and efficacious for treating abnormalities associated with ureteral duplication. A significant discrepancy in ureteral size does not preclude ureteroureterostomy.

Megacystis microcolon intestinal hypoperistalsis syndrome: bladder distension and pyelectasis in the fetus without anatomic outflow obstruction.

We report a case of a 46,XY male infant with a history of normal amniotic fluid levels who was delivered by elective cesarean section at 38.5 weeks' gestation because of progressive bladder distension, hydroureteronephrosis, and what was thought to be a dilated posterior urethra. The child died at 19 days of age of cardiovascular complications. The autopsy revealed megacystis, bilateral megaureters and pyelocaliectasis, congenital absence of ganglion cells in the bladder wall, renal dysplasia, and a microcolon. No dilation or anatomic obstruction of the posterior urethra was found. These findings strongly suggest the diagnosis of megacystis microcolon intestinal hypoperistalsis syndrome. We discuss the ultrasound findings of in utero bladder distension with hydronephrosis and one of its rare etiologies.

Comparison of flaps versus grafts in proximal hypospadias surgery.

PURPOSE: We analyzed the complication rate in patients who underwent proximal hypospadias repair using preputial skin as a flap or free graft. MATERIALS AND METHODS: We retrospectively reviewed the records of 142 patients who underwent proximal hypospadias repair. Repairs were subdivided into tubed and onlay repairs within the flap and free graft groups. We analyzed postoperative complications, including fistulas, proximal strictures, diverticula and meatal stenosis. RESULTS: Median patient age at followup was 11.3 months and median followup was 9.4 months. Two-thirds of the repairs were performed with free grafts. A proximal stricture developed in 8 and 0 patients who underwent free tubed graft and free onlay repair, respectively (p = 0.047). Otherwise there was no significant difference in the complication rate of the various types of repair. Of the 43 patients who had stricture, fistula or meatal stenosis 29 (67%) presented more than 1 year after surgery. CONCLUSIONS: In repairs performed with free grafts there is a significantly higher proximal stricture rate when a tube rather than an onlay is used. Otherwise we noted no significant difference in the complication rates of flaps and grafts used to repair proximal hypospadias. A significant number of complications presented more than 1 year postoperatively and they may even present as late as 4 years. This finding suggests that longer followup may be necessary to assess completely the outcome of proximal hypospadias surgery.

The ultrasonic description of postpubertal testicles in men who have undergone prepubertal orchiopexy for cryptorchidism.

PURPOSE: We provide a sonographic description of postpubertal testicles in patients who have undergone prepubertal orchiopexy with placement of a suture through the tunica albuginea. MATERIALS AND METHODS: Testicular ultrasound was performed in men who had undergone prepubertal testicular fixation for cryptorchidism using a suture passed through the tunica albuginea. Comparison was made to the sonographic appearance of cryptorchid testicles not secured with a fixating suture through the tunica albuginea. The operative report for each patient was reviewed with specific attention to use and type of suture material, and age at orchiopexy. RESULTS: Ultrasonography was performed on 22 men in whom a tunica albuginea fixating suture was placed during orchiopexy for cryptorchidism. Average patient age at orchiopexy was 5.6 years and median time from orchiopexy to this examination was 14.8 years. Right cryptorchidism existed in 64% of the men. No significant difference was noted in size between the 2 testicles in any patient. Ultrasonography did not identify any parenchymal abnormalities. A tunica albuginea calcification with posterior shadowing on the side of fixation was noted in 32% of patients, of whom 3 had calcified areas evident on physical examination. An area of subtunical hypoechogenicity was noted in 14% of patients. All tunical abnormalities were associated with the use of chromic suture. The remaining 12 patients (54%) had normal sonographic and physical examinations. The control group comprised 6 men who underwent orchiopexy without a fixating suture through the tunica albuginea. These men were examined identically and no sonographic abnormalities were noted in the tunica albuginea or testicular parenchyma. At a median of 16 months no patient had been diagnosed with testicular cancer or had a change in testicular self-examination. CONCLUSIONS: Tunica albuginea calcifications and hypoechogenic cysts are common ultrasonic findings in men who have undergone orchiopexy using a suture passed through the tunica albuginea. Parenchymal lesions should not be considered secondary to this procedure and must be treated as de novo abnormalities. These changes seem to be induced by the use of a fixating suture through the tunica albuginea.

Renal function before and after pyeloplasty: does it improve?

PURPOSE: Controversy exists concerning the timing of surgical correction of presumed ureteropelvic junction obstruction. Different opinions on the timing of intervention focus on renal function deterioration with time, poor initial relative function, or obstructive drainage curves and/or drainage time on diuretic renography. We retrospectively determined whether there is any improvement in renal function after pyeloplasty for presumed renal obstruction. MATERIALS AND METHODS: We retrospectively reviewed the charts of patients who underwent pyeloplasty between 1990 and 1997 in whom preoperative and postoperative diuretic renography data were available. Patients were excluded from review when they had bilateral ureteropelvic junction obstruction, a solitary kidney, associated vesicoureteral reflux, or other bladder or ureteral abnormalities, and when preoperative and postoperative renography studies were not available. RESULTS: Complete data were available for review in 79 patients 2 weeks to 18 years old (median age 6 months). Of the patients 73% were male and 73% of the affected kidneys were on the left side. Prenatal hydronephrosis had been diagnosed in 58 patients (73%), of whom 19 (33%) were observed for a variable period before pyeloplasty was performed. In all cases diuretic renography performed at the same institution using a standard protocol revealed a drainage time of 20 minutes or greater preoperatively, while in 58 cases a measurable drainage time was never achieved. As a rule, drainage improved postoperatively (mean and median 25 and 16 minutes, respectively). Open renal biopsy done at pyeloplasty in 54 patients was normal in 29. Preoperatively renal function ranged from 5 to 67% (mean and median 41 and 45, respectively). In all patients the paired t test showed no statistical difference in preoperative and postoperative renal function (p = 0.078, 95% confidence interval -3.451 to 0.185). There was no statistical change in renal function in patients with an abnormal renal biopsy regardless of the severity of renal scarring (p = 0.38) or when renal function was 40% or less (mean preoperative versus postoperative 29.7 versus 28.4%, p = 0.46). The group with greater than 40% function preoperatively had no relevant difference in function before or after surgery (mean 49.7 versus 47.8%, p = 0.065). Prenatally screened patients who were initially observed had a statistically significant difference in renal function before and after pyeloplasty (mean 45.6 versus 43%, p = 0.002). CONCLUSIONS: Renal function did not improve after pyeloplasty regardless of the initial level of relative function. Renal scan revealed that differential function decreased after pyeloplasty in some patients in whom hydronephrosis was detected prenatally and who were initially followed with observation. In our opinion waiting for renal function to decrease before considering pyeloplasty is not warranted, since function does not improve even when obstruction is corrected and drainage time improves.

Refined microscopic urinalysis for red blood cell morphology in the evaluation of asymptomatic microscopic hematuria in a pediatric population.

PURPOSE: The use of refined microscopic urinalysis for the presence of dysmorphic red blood cells (RBCs) has been evaluated in children and adults with a known source of hematuria. We examined the clinical usefulness of this study in a pediatric population with an unknown source of hematuria. MATERIALS AND METHODS: Children 12 years old or younger referred for evaluation of asymptomatic microscopic hematuria exhibiting 4 or more RBCs per high power field were enrolled in this study. Patients provided a first morning urine sample subjected to refined urinalysis for RBC morphology. Standard evaluation of patients was performed until a final diagnosis of the hematuria source was identified. RESULTS: A total of 44 patients completed the study. Refined urinalysis revealed pure dysmorphic RBCs in 22 patients, pure isomorphic RBCs in 8 and mixed isomorphic/dysmorphic RBCs in 14. The presence of dysmorphic RBCs correctly predicted a glomerulotubular source of hematuria in 29 of 36 patients (sensitivity 83%, specificity 81%), while the presence of isomorphic RBCs predicted a uroepithelial source of hematuria in 2 of 8 patients (sensitivity 25%, specificity 22%). Hematuria and 2+ proteinuria (100 mg./dl.) were more sensitive (100%) and specific (83%) than the presence of dysmorphic RBCs in predicting glomerulotubular hematuria. CONCLUSIONS: We believe that this is a costly test offering little additional information to the evaluation of microscopic hematuria in children. A thoughtful history and physical examination with microscopic urinalysis and dipstick for proteinuria provide an equal amount of diagnostic information. We do not recommend its routine use in the evaluation of microscopic hematuria in children.

Malpractice risks for urologists.

OBJECTIVES: To obtain data regarding the frequency of malpractice suits against urologists. METHODS: Those urologists listed in the Best Doctors in America were sent an anonymous survey that requested their personal malpractice history (group A). Additionally, the professional responsibility history of the candidates for recertification by the American Board of Urology in 1996 (group B) was reviewed. RESULTS: One hundred ten urologists in the United States in group A were surveyed. Ninety-one (83%) responded. Seventy (77%) had been sued (average 2.36 claims per physician who had been sued). Forty-four percent of the claims resulted in payment to the plaintiff. Claims frequency of group A was 0.09 claims per physician per year. Urologists in the Northeast, North Central, and Mid-Atlantic Sections of the American Urological Association were less likely to be sued than urologists in the other five sections. There were 246 urologists in group B. One hundred twenty-two (49%) reported a claim against them (average 1.9 claims per physician who had been sued). Twenty-nine percent of the closed claims resulted in payment to the plaintiff. Claims frequency of group B was 0.09 claims per physician per year. CONCLUSIONS: Most urologists can expect to be sued at least twice in their professional careers. The longer one is in practice, the greater the chance of a suit being filed. Where one practices may be a factor in the likelihood of being sued. There does not seem to be a direct or inverse correlation between professional reputation and the incidence of being sued.

The wet child.

Urinary incontinence is one of the most frequent problems seen in pediatric urology. Although there can be serious structural problems that produce wetting, most of the children seen will have nonstructural causes for their incontinence. A systematic approach and persistence with therapy should result in resolution of the problem in most instances.

An unusual case of urinary incontinence, ataxia-telangiectasia, and metastatic dysgerminoma: case report and review of the literature.

Ataxia-telangiectasia (A-T) is a rare autosomal recessive disease notable for neurodegeneration, chromosomal instability, and a predisposition to cancer. It presents in childhood with a variable phenotype. We report the first case of an A-T related tumor presenting as urinary incontinence, and the first case of 2-year survival in an A-T patient with metastatic dysgerminoma.