The anatomical assessment of disorders of sex development (DSD).

The assessment of abnormal anatomy in cases of DSD is important to aid diagnosis, understand the aetiology and severity of the condition, guide management and assess the outcomes of treatment. In this paper we present a systematic approach to this assessment which will provide a means by which the multiple disciplines who manage patients with these rare and complex conditions can communicate and thus improve overall care.

Long-term results of the endoscopic correction of vesicoureteral reflux in children using autologous chondrocytes.

PURPOSE: Endoscopic correction of vesicoureteral reflux continues to attract research of many autologous and nonautologous substances. We previously reported on the initial results of a clinical trial using endoscopic injection of autologous chondrocytes to correct vesicoureteral reflux in children and now present our greater than 1-year followup results. MATERIALS AND METHODS: A total of 29 children (47 ureters) with grades II to IV vesicoureteral reflux were treated at 2 centers. Each child underwent cystoscopy and posterior auricular cartilage harvesting at the initial setting. Chondrocytes were grown in culture during a 6-week period. Patients returned for transurethral injection of autologous chondrocytes into the ureterovesical junction of the refluxing ureters. Ultrasound was performed at 1 month, 1 year, and 2 and 3 years after implantation, and a voiding cystourethrogram or radionuclide cystogram at 3 months and 1 year after injection. If reflux persisted re-treatment with stored chondrocytes was offered. RESULTS: At 3-month followup initial chondrocyte injection corrected reflux in 55% of ureters (27 of 47) while a second or third injection was successful in additional 15 of 29 patients, resulting in an overall success rate of 86% (42 of 49) ureters and 25 of 29 patients. At 1-year followup reflux correction was maintained in 70% of ureters (32 of 46) and 65% of patients (19 of 29). The 1-year followup results after re-treatment of 3 ureters were not available. In those patients in whom implantation failed cystoscopy revealed evidence of volume loss and shifting of subureteral mounds to account for loss of the antireflux effect. Three patients underwent successful open ureteroneocystostomy for failed autologous chondrocyte implantation. There were no significant complications. CONCLUSIONS: Transurethral injection of autologous chondrocytes to correct vesicoureteral reflux in children is safe and reasonably effective. There is a relapse rate which must be considered. Changes in the formulation of the material have been made to enhance implant reliability and increase long-term success.

Vesicoureteral reflux after ureteroneocystostomy: indications for postoperative voiding cystography.

PURPOSE: The aim of this study was to determine the risk factors for vesicoureteral reflux following ureteral reimplantation to identify a population that can be safely excluded from postoperative voiding cystography. MATERIALS AND METHODS: We retrospectively reviewed the medical records of 273 patients who underwent ureteroneocystostomy for vesicoureteral reflux between 1990 and 1998 and recorded the postoperative renal ultrasonography and voiding cystography results. RESULTS: There were 273 patients (534 ureters) who underwent ureteral reimplantation. We recorded the grade of preoperative hydronephrosis and vesicoureteral reflux and noted several preoperative and intraoperative variables, such as dysfunctional voiding, breakthrough infections, renal scarring, bladder trabeculations, type of reimplant, and postoperative urinary tract infections. With a mean follow-up of 20.6 months, persistent postoperative vesicoureteral reflux was noted in 11 patients (4%). Persistent postoperative reflux was noted in 11 patients (4%) or 12 renal units (2.2%). Reflux resolution rates for 534 renal units and 273 patients after routine follow-up voiding cystourethrogram (VCUG) was 97.8% (renal units) and 96% (patients), respectively. Contralateral vesicoureteral reflux developed in 4 (5.1%) of the 78 patients who underwent unilateral reimplantation. Two patients (0.7%) had postoperative ureteral obstruction. The risk factors for persistent postoperative reflux were identified as preoperative and postoperative hydronephrosis, renal scarring, and tapered reimplantations. The type of reimplant did not correlate with outcome. CONCLUSIONS: Vesicoureteral reflux after ureteral reimplantation is uncommon (4%). Because of the high success rate of ureteral reimplants and the benign course of those patients with persistent low-grade postoperative reflux, it is safe and efficient to eliminate postoperative VCUG in most patients who had a simple ureteral reimplantation for reflux. However, in some higher-risk patients, such as those with preoperative hydronephrosis, renal scarring, and ureteral tapering, postoperative voiding cystography may be indicated to assure resolution of vesicoureteral reflux.

Laparoscopy in pediatric urology.

Laparoscopy in pediatric urology is a rapidly evolving field that is becoming part of the operative repertoire of an increasing number of pediatric urologists. This article summarizes the latest ideas and issues in the expanding field of laparoscopy in pediatric urology. New methods of obtaining laparoscopic access and retraction are discussed. Laparoscopic experiences in urologic reconstruction, diagnosis and treatment of the nonpalpable testis, renal surgery, ureteral reimplantation, varicocelectomy, hydrocelectomy, and herniorrhaphy are reviewed. Articles assessing the safety of a pneumoperitoneum in patients with a ventriculoperitoneal shunt are also discussed.

The use of free grafts for correcting penile chordee.

PURPOSE: Patients with extensive penile chordee may require free graft placement to create a straight phallus. We review our experience with those who underwent primary (no previous surgery) and secondary repair using dermal and tunica vaginalis grafts to correct penile chordee. MATERIALS AND METHODS: Between 1992 and 1998, 28 patients required free graft placement to correct penile chordee. In 8 cases (29%) primary repair was done using tunica vaginalis in 2 and a dermal graft in 6, while in 20 (71%) secondary repair was performed due to recurrent chordee with or without hypospadias. Tunica vaginalis was used in 3 patients and dermal grafts in 17. Grafts were used when straightening did not result from penile shaft degloving, dorsal plication and urethral plate division. Mean followup was 2 years. RESULTS: Residual chordee developed in 1 of 8 patients after primary and in 2 of 20 after secondary repair. Tunica vaginalis grafts had been placed at repair in all patients with residual chordee but there was no recurrent chordee after dermal graft placement. Chordee recurred in 60% of the patients who received a tunica vaginalis graft. CONCLUSIONS: Conventional techniques, such as penile degloving and dorsal plication, may be used to correct penile chordee in most cases, while free grafts are more likely to be required for secondary repair. We believe that dermal grafts result in more successful repair than tunica vaginalis grafts, which seem to be associated with a higher incidence of residual chordee.

Congenital anterior urethrocutaneous fistula.

PURPOSE: Congenital anterior urethrocutaneous fistula is a rare anomaly that may present in an isolated fashion or in association with other penile abnormalities, such as chordee or hypospadias. There have been 18 cases of congenital anterior urethrocutaneous fistula reported in the literature. We present 14 additional cases of congenital anterior urethrocutaneous fistula. MATERIALS AND METHODS: We treated 14 patients with congenital anterior urethrocutaneous fistula, of whom 9 were uncircumcised at presentation. Two patients had evidence of chordee and 4 had distal hypospadias. RESULTS: The type of repair was determined by the anatomical variations of this anomaly. All cases were corrected electively by various techniques based on the degree of the defect, including primary closure via a Thiersch-Duplay urethroplasty, pedicle flap urethroplasty, hinged flap urethroplasty and interpositioned island pedicle tube or onlay urethroplasty. CONCLUSIONS: To our knowledge the embryological events that cause anterior urethrocutaneous fistula are unclear but they likely result from a defective urethral plate or an abnormality of the infolding of the urethral groove. Surgical technique must be individualized to fit the defect. While there has been considerable skepticism regarding the existence of congenital urethrocutaneous fistula, the fact that 9 of our 14 patients were uncircumcised confirms the congenital nature of this lesion.

Endoscopic correction of vesicoureteral reflux in children using autologous chondrocytes: preliminary results.

PURPOSE: Previous approaches to the endoscopic correction of vesicoureteral reflux have used foreign bulking substances, raising concern regarding safety and long-term efficacy. We describe the results of a clinical trial using transurethral injection of autologous chondrocytes to correct vesicoureteral reflux in children. MATERIALS AND METHODS: A total of 29 children (46 ureters) with grades II to IV vesicoureteral reflux were treated at 2 sites. Each child underwent cystoscopy and ear cartilage biopsy at the initial setting. Chondrocytes were grown in culture for 6 weeks. Patients then returned for transurethral injection of chondrocytes into the bladder trigone to correct reflux. Ultrasound was performed 1 month and radionuclide cystography was done 3 months postoperatively to confirm reflux resolution. When reflux persisted, repeat treatment with stored chondrocytes was offered. RESULTS: Initial chondrocyte injection corrected reflux in 26 of the 46 ureters (57%), while secondary injection was successful in 12 of 19 (63%). Overall reflux was corrected in 38 of the 46 ureters (83%) and in 24 of the 29 patients (83%). There were no significant complications. CONCLUSIONS: Transurethral injection of autologous chondrocytes to correct vesicoureteral reflux in children appears to be an effective and safe technique that holds promise for treating this congenital abnormality in a minimally invasive fashion.

Incidence of contralateral inguinal hernia: a prospective analysis.

BACKGROUND/PURPOSE: Contralateral groin exploration in children with unilateral inguinal hernia is still controversial, particularly in infants. The authors have attempted to determine the age- and gender-stratified incidence of contralateral hernia and the necessity of routine bilateral procedures. METHODS: This is a prospective study of 656 patients during a 34-month period at a single institution. Patients with unilateral hernia underwent an ipsilateral procedure only, regardless of age, gestational age, or gender. Follow-up was 6 to 40 months (mean, 25.5 months). Chi-square analysis was used for intergroup comparison (P < .05 significant). RESULTS: Of 656 children, 108 (16.5%) presented with synchronous bilateral hernias. Bilateral inguinal hernia was significantly more common in premature infants (28.0%) and young children (33.8% if <6 months, 27.4% if <2 years). Of the remaining 548, a metachronous contralateral hernia developed in 48 (8.8%) at a median interval of 6 months (range, 4 days to 7 years). This incidence was 13 of 105 (12.4%) in infants less than 6 months of age, 20 of 189 (10.6%) in children less than 2 years of age, 8 of 54 (14.8%) in premature infants, 6 of 81 (7.4%) in girls, and 8 of 29 (27.6%) in children with an incarcerated hernia. In the latter group, P < .05, chi2 analysis. CONCLUSION: Routine contralateral inguinal exploration, without clinical evidence of a hernia, may be advisable in children with incarceration and possibly in premature infants. The low incidence of contralateral hernias in all other patients, regardless of gender or age, does not justify routine contralateral exploration.

Ureteral valves in children.

OBJECTIVES: Congenital ureteral valves are a rare cause of ureteral obstruction in children, with only 42 cases having been reported in peer-reviewed literature. Eight additional cases of ureteral valves are herein reported. METHODS: We report on the diagnosis and management of eight children with ureteral obstruction secondary to a ureteral valve. RESULTS: Eight children with congenital ureteral valves were managed by ipsilateral ureteroureterostomy, ureteropyelostomy, or longitudinal ureterotomy with excision of valve leaflets. The obstruction was relieved in all. CONCLUSIONS: Ureteral valves should be included in the differential diagnosis of ureteral obstruction in children. Reconstruction is curative.

Buccal mucosal grafts for urethral reconstruction.

OBJECTIVES: Patients requiring urethral reconstruction but who have a paucity of usable genital tissue present a considerable technical challenge. Herein we report the experience of three centers in the use of buccal mucosa for urethral replacement. METHODS: From 1991 to 1996, 22 urethral reconstructions were completed using a graft of buccal mucosa. Eighteen of 22 patients had previously failed hypospadias repairs, while three had bulbar urethral stricture and one had penile carcinoma. Grafts were taken from either the inner cheek or the lower lip, and seven were used as a combined graft. Onlay grafts were used in 6 cases and tubularized grafts in 16 cases. RESULTS: Two patients developed complications at the donor site. Nine of 22 patients had complications of the urethroplasty-two had meatal stenosis, four developed a urethral fistula, and three developed urethral stricture. All complications have been managed successfully to date. CONCLUSIONS: Buccal mucosa is an excellent source of graft material for urethral replacement in complex urethroplasties. It is readily available, in abundant supply, and has physical properties beneficial to free graft survival.

Primary nocturnal enuresis: a review.

Bed-wetting--primary nocturnal enuresis (PNE)--is a common yet misunderstood and under-treated childhood condition. PNE is both psychologically and physically distressing. Since bed-wetting children will often avoid peer activities for fear of overnight invitations, any child who avoids social situations and withdraws from most activities should be screened for PNE. Early screening and treatment can afford bed-wetters improved quality of life. School nurses are in an ideal position to identify potential enuretics. Given the range of available treatments, it is incumbent upon nurses to screen and advocate early treatment for these children.

Cystic dysplasia of the rete testis: a benign congenital lesion associated with ipsilateral urological anomalies.

PURPOSE: Cystic dysplasia of the rete testis is a benign congenital lesion that can mimic testicular cancer. We report 6 cases, review the literature, discuss the embryological etiology and make management recommendations. MATERIALS AND METHODS: The records and pathology reports of 6 boys presenting with cystic dysplasia of the rete testis at 5 institutions were reviewed, as was the relevant literature. RESULTS: Of the 6 cases 5 presented as scrotal masses in previously healthy boys and 1 as an abdominal mass in a newborn with multiple congenital anomalies. One patient had been followed from birth for a multicystic dysplastic kidney and 4 were found to have an ipsilateral absent kidney during evaluation. Development of the contralateral side was normal in most cases. CONCLUSIONS: Cystic dysplasia of the rete testis is an unusual, benign congenital lesion that can mimic testicular cancer in presentation. The presence of ipsilateral renal anomalies, particularly renal agenesis, can suggest cystic dysplasia of the rete testis in the differential diagnosis preoperatively. Even if cystic dysplasia of the rete testis is suspected, we recommend inguinal exploration and early control of the spermatic cord in the event that neoplasia is identified. If possible, the goal of preserving as much normal testicular parenchyma as possible is desirable. Long-term followup for possible recurrence is recommended, particularly after local excision.

The mechanism of new onset contralateral reflux following unilateral ureteroneocystostomy.

PURPOSE: We studied a population of patients undergoing unilateral antireflux surgery to determine the mechanism of new onset contralateral reflux postoperatively. MATERIALS AND METHODS: A total of 141 patients underwent unilateral antireflux surgery via the Cohen, Glenn-Anderson or extravesical technique. The 18% of patients who had new onset contralateral vesicoureteral reflux were analyzed according to grade of initial reflux, presence of a Hutch diverticulum or duplex system and surgical technique. RESULTS: Surgical technique did not influence the development of contralateral reflux. As grade of corrected reflux increased, a significant trend toward development of contralateral reflux was noted. A Hutch diverticulum was not a risk factor for contralateral reflux but reflux into a duplicated system was a distinct risk factor (26 versus 12% in single system reflux). CONCLUSIONS: Our study supports the concept that new onset contralateral reflux may result from elimination of a pop-off mechanism. Surgical distortion of the contralateral hemi-trigone appears not to be responsible. Correction of severe (grade V) reflux and reflux into duplex systems put patients at particular risk for development of contralateral reflux postoperatively.

Splenogonadal fusion.

PURPOSE: We report on 7 patients with splenogonadal fusion and review the literature on this unusual condition. MATERIALS AND METHODS: The medical records of patients diagnosed with splenogonadal fusion between 1989 and 1994 at 4 institutions were retrospectively reviewed. RESULTS: All 7 patients were properly diagnosed at surgery and the testes were salvaged in 5. In 1 intersex patient gonadectomy was appropriate. CONCLUSIONS: Splenogonadal fusion is a rare condition. Familiarity with this lesion allows for intraoperative diagnosis and testicular salvage.

Contralateral reflux after unilateral ureteral reimplantation.

PURPOSE: We analyzed the incidence and outcome of postoperative contralateral reflux after unilateral ureteral reimplantation by the Cohen and Glenn-Anderson techniques. MATERIALS AND METHODS: We retrospectively reviewed the records of 120 patients 3 months to 21 years old in whom unilateral vesicoureteral reflux was treated by unilateral reimplantation. The incidence of postoperative contralateral reflux was documented by followup voiding cystourethrography. RESULTS: Overall 19% of patients who underwent unilateral reimplantation had contralateral vesicoureteral reflux postoperatively, including 21% after the Cohen and 17% after the Glenn-Anderson procedure. Of the cases 61% spontaneously resolved, 13% were surgically corrected and 26% continue to be followed. CONCLUSIONS: The rates of postoperative contralateral vesicoureteral reflux are not significantly different after Cohen and Glenn-Anderson repair. A majority of cases will resolve spontaneously within 2 years. The likelihood of trigonal distortion as the etiology of contralateral reflux is low given the similar incidence in cross-trigonal and ureteral advancement reimplantation.

Transitional cell carcinoma of the bladder in the pediatric patient.

PURPOSE: We report on 5 boys with transitional cell carcinoma of the bladder, describe the identifying characteristics, review the literature, and define the issues of diagnosis, treatment and followup in this rare disease in pediatric patients. MATERIALS AND METHODS: Five boys 11 to 18 years old were identified with transitional cell carcinoma of the bladder. Preoperative imaging and urinary cytology were correlated with cystoscopic and biopsy findings. RESULTS: In all patients evaluation was prompted by gross hematuria. Low grade lesions, definitive cystoscopic management and a low recurrence rate were uniform findings. Preoperative imaging identified the tumor in all cases and bladder ultrasound was the most sensitive scan with 4 of 4 cases identified. CONCLUSIONS: While rare, transitional cell carcinoma of the bladder in children presents a challenge in diagnosis and followup since cystoscopy typically requires general anesthesia in this age group. Bladder ultrasound was found to be extremely sensitive in identifying lesions, and it may be a valuable and minimally invasive surveillance tool.