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INTRODUCTION AND OBJECTIVE:The Prostate Imaging-Reporting and Data System (PI-RADS) version 2.1 has become the standard in risk categorization of prostate lesions. However, there is significant int...

Recent comparisons of the impact of the surgical approach on pediatric pyeloplasty outcomes have generally incorporated a form of internal ureteral drainage. We hypothesized that the surgical approach does not affect outcomes in children who undergo unstented pyeloplasty and stenting offers no long-term benefit in those with pediatric pyeloplasty.After receiving institutional review board approval we examined the records of all children who underwent initial pyeloplasty from December 2001 to December 2009. We compared unstented and stented pyeloplasties, and each surgical approach in the unstented group.During the study period 367 pyeloplasties were performed, including 231 unstented and 136 stented procedures. When comparing unstented to stented pyeloplasties, there was no difference in the complication or failure rate. Of unstented pyeloplasties 71, 67 and 93 were done using a transperitoneal laparoscopic approach, a flank approach and dorsal lumbotomy, respectively. There were 5 failures, of which 2 were laparoscopic, 2 used a flank approach and 1 used dorsal lumbotomy (p = 0.51). A total of 31 patients, including 10 treated with a laparoscopic approach, 3 with a flank approach and 18 with dorsal lumbotomy (p = 0.02), required second procedures, of which 24 were temporary drainage for a urine leak. Univariate analysis of factors associated with secondary procedures in the unstented pyeloplasty group showed that only surgical approach was significant (p = 0.05).In pediatric pyeloplasty there is no significant difference in outcome between stented and unstented repairs. In unstented repairs complications may vary by surgical approach. Regardless of the approach unstented pyeloplasty is safe and effective in the pediatric population.

Laparoscopic orchiopexy is a safe operation. However, the bladder can be injured during creation of the transperitoneal tunnel for the cryptorchid testis. We reviewed our experience with this complication.We searched the operative notes of patients who had undergone laparoscopic orchiopexy between August 15, 2002 and October 1, 2008, and identified bladder injuries and their treatment.A total of 93 patients underwent laparoscopic orchiopexies for 101 undescended testes during the study interval, with 3 procedures resulting in bladder injuries. The 3 operations varied with regard to whether the injury was recognized intraoperatively or postoperatively, and repaired in an open or laparoscopic fashion.Bladder injury during laparoscopic orchiopexy is a rare but serious complication that can be managed by an open or laparoscopic approach. We recommend placement of a urethral catheter and syringe assisted drainage of all urine from the bladder at the beginning of the operation, careful perivesical dissection particularly in children with prior inguinal surgery, filling and emptying of the bladder during the procedure, and maintaining a high index of suspicion especially when hematuria is observed.

Purpose: We explored the psychological adjustment of children with bladder or cloacal exstrophy.Materials and Methods: We assessed 29 subjects with a mean age plus or minus standard deviation of 7.8 ± 3.97 years using age appropriate standard psychological instruments. Psychological adjustment scores in the exstrophy group were compared to the norms of the various instruments. Subjects were divided into dichotomous groups according to several medical and demographic factors. For each factor the differences between the means of the 2 groups on the outcome variables were calculated using a t test.Results: Children with exstrophy perceived their appearance more positively than the norm. Older children performed more poorly than younger children in adaptive behavior, specifically in skills related to functioning in school. Children who achieved continence after age 4 years were more likely to have problems with acting out behavior. There were no differences in adjustment in boys versus girls, bladder ...

Purpose: The rapidly growing field of molecular biology has caused exponential growth in our knowledge of the processes of embryogenesis. Since the cloning of the androgen receptor gene in 1988, investigators have been able to clarify many of the molecular events of male sexual differentiation that are mediated through the androgen receptor. We reviewed the current state of knowledge of the androgen receptor and its role in male genital development.Materials and Methods: An intensive literature search was conducted to review reports on the androgen receptor and sexual differentiation since 1988. This review also includes ongoing research from our laboratory on the role of the androgen receptor in human genital development, as well as collaboration with other investigators.Results: We reviewed the basic molecular biology of androgenic action mediated through the androgen receptor. This information has been integrated into the current understanding of human male sexual differentiation to clarify how...

Hypospadias has no known single etiology but it has been linked to androgen insensitivity caused by mutations of the androgen receptor gene. The purpose of this study was to search for such mutations in cases of various degrees of isolated hypospadias to determine whether such an association exists and, if so, with any particular anatomical subgroup.Isolated deoxyribonucleic acid from the penile tissue of 40 patients undergoing reconstructive surgery was screened for mutations of the coding regions of the androgen receptor gene using single strand conformational polymorphism analysis. In cases with abnormal single strand conformational polymorphism findings sequence analysis of the deoxyribonucleic acid was performed to define the mutation.A missense mutation of exon 2 of the androgen receptor gene was noted in 1 patient with isolated distal penile shaft hypospadias. Sequence analysis revealed that the mutation changed amino acid residue 546 from proline to serine. No abnormalities were detected in the other 39 patients.Isolated distal shaft hypospadias is associated with mutations of the androgen receptor gene but these mutations appear to be a rare cause of hypospadias.

Purpose: We assessed the role of surgery, particularly exenteration, in the treatment of children with lower urinary tract and pelvic rhabdomyosarcoma.Materials and Methods: We treated 23 children with bladder and/or prostate[11], or pelvic retroperitoneal tumors[12]. Initial management was tumor resection in 6 cases, anterior pelvic exenteration in 5 and biopsy only in 12, combined with chemotherapy in 23 and radiotherapy in 20.Results: The bladder salvage rate for surviving patients with pelvic tumors was 92 percent versus 27 percent for those with prostate/bladder tumors. Estimated 5 and 10-year survival probability for patients with pelvic retroperitoneal tumors was 49 plus/minus 50 percent compared to 81 plus/minus 24 percent for those with bladder and/or prostate tumors (log rank test, p = 0.11).Conclusions: Exenterative surgery is frequently required to achieve a durable complete response.

While long used for primary hypospadias repair, meatal based flap urethroplasty has not been widely considered a viable alternative for a secondary procedure. We reviewed 200 patients who underwent meatal based flap urethroplasty and identified 9 (4.5%) who underwent the procedure for correction of complications of previous hypospadias repair, including urethrocutaneous fistula, severe meatal retraction, persistent chordee and hair in the urethra. The complication was corrected with a single operation in 8 of the 9 patients (89%). A single small fistula developed in 3 patients, which closed spontaneously in 2 and required another procedure in 1. Meatal based flap urethroplasty should be considered for correction of distal complications of hypospadias repair.

Lower urinary tract malignancy is an uncommon cause of hematuria in children. The most common pediatric bladder malignancies are rhabdomyosarcoma, leiomyosarcoma and transitional cell carcinoma. To our knowledge primary Ewing's sarcoma of the bladder has not been previously reported in any age group. We report a case of primary Ewing's sarcoma of the bladder associated with elevated antinuclear antibody titer. CASE REPORT A previously healthy 15-year-old white girl presented for evaluation of painless gross hematuria and clot urinary retention. Physical examination was normal and medical history was unremarkable. Urinalysis revealed numerous isomorphic erythrocytes but no pyuria or bacteriuria. Creatinine, serum electrolytes, liver function tests, alkaline phosphatase, lactate dehydrogenase, hematocrit and coagulation studies were normal. An antinuclear antibody titer that was obtained to rule out nephrological hematuria was elevated at 1:10,240 (normal up to 1:15). Excretory urogram showed normal kidneys and an unremarkable upper urinary tract. However, a filling defect was noted in the bladder. Cystoscopic examination revealed a 2 x 2 cm. bleeding, sessile globular tumor with focal areas of necrosis and no papillary features. The lesion was at the right anterolateral bladder wall. Transurethral resection was incomplete and biopsy specimens were not consistent with transitional cell carcinoma. Immunohistochemical and electron microscopy studies revealed small round blue malignant cells (fig. 1). Staging chest, abdomen and pelvis computerized tomography (CT) failed to reveal intra-abdominal or retroperitoneal pathology.

Evaluation of 48 cases of male infertility included testicular biopsy. The histologic classification included normal spermatogenesis (normal or abnormal supporting tissue, maturation arrest (spermatocyte or spermatid stage), hypospermatogenesis, Klinefelter's syndrome and Sertoli-cell-only syndrome. No diagnostic advantage was found in bilateral over unilateral testicular biopsies. Clinical presentation of the patient was correlated with histologic classification: 1) 4 of 13 azoospermic cases had normal spermatogenesis, suggesting post-testicular obstruction, 2) severe oligospermic cases (1 X 10(6) sperm per ml.) had spermatogenic abnormalities and 3) only 1 of 8 mild oligospermic cases (10 to 20 X 10(6) per ml.) had abnormalities of spermatogenesis. The histologic pattern in patients with varicocele seemed consistent with hypospermatogenesis in 9 of 13 cases studied. Testicular biopsy may be useful in the diagnosis of reversible post-testicular obstruction in some azoospermic patients. In addition, some patients with severe oligospermia may find an advantage of increased accuracy of diagnostic counseling based on histologic information. Testicular biopsy in cases of moderate oligospermia may be useful as investigational information in determining histologic patterns that may be responsive to certain modes of therapy. Histologic information obtained in cases of mild oligospermia was not sufficient to provide indication for testicular biopsy in mild oligospermic patients.

A total of 11 children with chronic meningomyeloceles underwent a clinical neurological, urodynamic and neurophysiological evaluation to define further the level and type of lesion present in the lumbosacral spinal area. The neurophysiological evaluation was correlated with detrusor activity. Detrusor hyperreflexia was present in 45 per cent of the patients and functional activity of the somatic sacral reflex arc was demonstrated in 4. Absolute interruption of the somatic sacral reflex arc was found in the remaining patients with detrusor areflexia. All patients had evidence of a lower motor neuron lesion on clinical neurological evaluation, with weak or absent myotatic and musculocutaneous reflexes. Electromyographic evaluation revealed signs of severe partial to complete denervation of the affected muscle groups. However, increased motor unit potentials were observed in 5 patients after coughing and stimulation of the perianal skin. The bulbocavernosus reflex was absent clinically and electrophysiologically in all 6 patients tested. However, this finding did not indicate absolute interruption of the reflex arc, since further temporal and spatial stimuli resulted in an increase in motor unit potentials. Somatosensory cortical evoked potentials were useful in demonstrating an intact afferent input system to the spinal cord and brain area. However, lumbosacral evoked potentials appeared to be the least useful neurophysiological method to demonstrate partial preservation of the input to the spinal cord level. Therefore, detrusor and external urethral sphincter function may be documented on urodynamic and neurophysiological evaluation when the clinical examination has revealed a complete lower motor neuron lesion. The complexity of the lesion caused by the malformation in patients with meningomyelocele is shown.

In most recent series of artificial sphincter implantations there has been a reduction in mechanical and surgical complications, with continence rates approaching 90 per cent. Despite initially excellent results in 47 children, with increasing durations of followup we noted a transient hydronephrosis related to incomplete bladder emptying in 5 and a persistent physiological alteration of detrusor dynamics consisting of a rigid, noncompliant bladder in 7. Preoperatively, all patients had had in-depth radiological studies and most had a urodynamic evaluation, and were considered to be excellent candidates. The etiology of these alterations is not understood at this time. Bladder and even upper tract deterioration can occur without the appearance of urinary leakage. Therefore, children with an artificial sphincter must be monitored indefinitely with semiannual assessment of the upper tracts and periodic urodynamic evaluation.

Nephroblastoma, or Wilms tumor, is the most common renal neoplasm in children and accounts for approximately a fifth of all malignant growths in this age group. However, the incidence of Wilms tumor in adults is much less common, with less than 200 cases having been reported in the literature. In addition, since there are at least 53 synonyms for this tumor an adequate computer search for articles relating to this subject is difficult. Because of the scarcity of this disease in adults definite treatment modalities have not been accepted thoroughly until recently. The rationale for the present treatment modalities is based largely on the National Wilms Tumor Study groups 1 to 3. We present radiologic and histologic findings of this tumor in 2 additional cases. The current treatment modalities, consisting of chemotherapy with or without adjunctive radiotherapy, are discussed.

We reviewed 52 children with ureteroceles in an effort to evaluate the various facets of this disorder that influenced our surgical management. There were 12 single system ureteroceles and 40 duplex system ureteroceles. Total reconstruction was performed in 16 duplex system and 8 single system ureteroceles, of which 88 per cent required no further surgery. Upper pole heminephrectomy or ureteropyelostomy with partial ureterectomy was performed in 22 patients with duplex system ureteroceles with the goal of obviating lower tract surgery, of whom 12 (55 per cent) required subsequent surgery. In 6 patients, 4 with single system and 2 with duplex system ureteroceles transurethral incision of the ureterocele was the initial procedure with the expectation that improvement in function and hydronephrosis would facilitate subsequent lower tract surgery. Two of these patients required subsequent reconstruction. Recommendations regarding management are based on initial pathological condition, patient age and the presence of a single or duplex system ureterocele.

A 10-year-old boy had intermittent ureteropelvic junction obstruction that proved to be owing to benign fibroepithelial polyps. The clinical features and management of this rare lesion are discussed.

Bladder injury in children is an unusual event that usually is associated with trauma or a surgical mishap. We describe 2 premature male neonates who were treated by minimal stimulation therapy and systemic paralyzation. Despite normal urological anatomy, each patient had a bladder rupture that we attributed to atony of the bladder coupled with the Crede maneuver, which produced high intravesical pressures.

Single stage repair of moderately severe degrees of hypospadias with correction of chordee and reconstruction of the neourethra presents a difficult challenge for the surgeon. Traditionally, a 2-stage approach to correct these defects has been used. However, more recently a 1-stage repair with correction of chordee and creation of a neourethra has increased in popularity. From 1978 to 1985 we treated 86 patients with moderately severe hypospadias and chordee with a 1-stage technique using either a transverse island pedicle flap, a Hodgson III hypospadias repair or a free preputial skin graft. Despite an over-all complication rate of 50 per cent, our final results based on cosmesis and function were deemed excellent in 90 per cent of the cases. Of the 44 complications 23 were strictures, most frequently at the proximal anastomosis. Two-thirds of these strictures responded to a single dilation with the patient under anesthesia. Our fistula rate was 10 per cent and flap necrosis, meatal stenosis or prolapse and residual chordee accounted for the remaining 12 complications. Our experience indicates that a 1-stage repair for moderately severe hypospadias and chordee can be performed in patients with a satisfactory success rate of more than 90 per cent, minimal morbidity and an acceptable complication rate.

Two variations for resurfacing the penile shaft skin after repair of hypospadias defects are described. These techniques have been used in an effort to prevent some of the skin tags, tension at the penoscrotal angle, and oblique suture lines that may occur with the traditional Byars' flaps.