Feminizing genitoplasty: a proven technique.

The female child with the adrenogenital syndrome is subject to a variable degree of masculinization. Surgery should be directed to 3 goals: (1) removing the corpora and preserving the glans with its innervation to create a clitoris with normal sensation, (2) creating a normal-appearing introitus by fashioning labia minora from phallic skin and foreskin, and (3) vaginoplasty to provide an adequate opening for the vagina onto the perineum. The entire repair may be completed before the age of 6 months unless the vagina enters the urogenital sinus at the high level, in which case vaginoplasty may be delayed until the child is older. The evolution of this operative approach is described and the details of the operative technique are presented.

Undescended testis histology correlation with adult hormone levels and semen analysis.

PURPOSE: Cryptorchidism has been associated with infertility. We hypothesize that a positive correlation exists between testicular histopathology at orchiopexy and future fertility potential in patients with cryptorchidism. MATERIALS AND METHODS: Patients with cryptorchidism who underwent orchiopexy with bilateral testis biopsies were followed into adulthood. Testis histology was stratified into groups based on total germ cells per tubule and adult dark spermatogonia per tubule. After age 18 years, patients underwent hormonal testing and semen analysis. Mean semen analysis parameters and hormone levels were compared among histopathology groups. RESULTS: A total of 91 patients with unilateral undescended testes and 19 with bilateral undescended testes had data for review. No significant differences in semen analysis parameters were seen among the germ cells per tubule groups. In unilateral undescended testis, sperm density and sperm count in the abnormal adult dark spermatogonia per tubule group remained within normal range but were significantly decreased (p = 0.005 and p = 0.028). Follicle-stimulating hormone levels were significantly higher in patients with unilateral undescended testis with abnormal adult dark spermatogonia per tubule but remained within normal range (p = 0.009). Sperm density was below normal range and was significantly decreased in the abnormal adult dark spermatogonia per tubule group in the bilateral undescended testes cohort (p = 0.0496). In bilateral undescended testes follicle-stimulating hormone level, sperm count and percent motility in the abnormal adult dark spermatogonia per tubule group were outside normal clinical range but these results were not statistically significant (p = 0.07-0.2). CONCLUSIONS: Total germ cell histopathology at the time of orchiopexy was not associated with significant changes in hormone levels or semen analysis results in adulthood. Testis biopsy at orchiopexy may be limited in predicting future fertility in unilateral undescended testis but more clinically useful in predicting fertility potential for those with bilateral undescended testes.

Histological findings in patients with cryptorchidism and testis-epididymis nonfusion.

PURPOSE: Fusion anomalies of the testis and epididymis are associated with cryptorchidism. The bilateral histology of the cryptorchid testis associated with the nonfused epididymis has not been reported previously. MATERIALS AND METHODS: We retrospectively reviewed patients who presented with unilateral undescended testes and underwent bilateral testis biopsy at orchiopexy between 1982 and 2008. Testes were stratified into groups based on degree of testis-epididymis nonfusion. Age at surgery, testicular volume, testicular position, total germ cells per tubule and adult dark spermatogonia per tubule were compared among all groups. RESULTS: A total of 2,660 testes were eligible for review, of which 2,425 had normal fusion (group 1), 55 had epididymal head nonfusion (group 2), 119 had epididymal tail nonfusion (group 3) and 61 had complete nonfusion (group 4). With increasing degrees of nonfusion trends toward younger age, smaller testicular volume and higher preoperative position were observed. However, testis-epididymis nonfusion was not a significant predictor of abnormal germ cells per tubule or adult dark spermatogonia per tubule in undescended testes and contralateral descended testes. CONCLUSIONS: Fusion anomalies are associated with smaller, higher testes with no significant abnormalities in germ cells per tubule or adult dark spermatogonia per tubule. Testis-epididymis nonfusion is not a reliable predictor of reduced histological findings, and should not be a strong consideration when counseling patients and their families about future fertility, especially in instances of complete nonfusion.

Regional differences in maturation of germ cells of cryptorchid testes: role of environment.

OBJECTIVE: To investigate differences in maturation of germ cells in cryptorchid testes in three different regions. PATIENTS AND METHODS: A total of 103 consecutive patients were operated for unilateral undescended testis in Vojvodina, from March 2006 until September 2007, and had a testicular biopsy performed. Germ cells were counted, and the presence of Ad spermatogonia was noted. Biopsies were compared to biopsies of similar patients from two different regions: Philadelphia, USA (130), and Liestal, Switzerland (55 patients). RESULTS: In Vojvodina, 84.5% of patients had Sertoli cells only, or some spermatogonia, but no Ad spermatogonia, and 15.5% had Ad spermatogonia. In Philadelphia, 59.3% of patients had poor testicular histology, and 40.7% had Ad spermatogonia. In Liestal, 61.8% of patients had no, or some, spermatogonia, but no Ad spermatogonia, and 38.2% had Ad spermatogonia. There was a difference (p = 0.000025) between the patients with normal testicular histology from Philadelphia and those from Vojvodina, as well as between the patients from Vojvodina and Liestal (p = 0.0027). CONCLUSION: The reduction in the number of germ cells in patients with cryptorchidism from Vojvodina is more pronounced than patients from either Switzerland or USA. This is a unique observation, since such a study has not been published yet.

Transient asynchronous testicular growth in adolescent males with a varicocele.

PURPOSE: We assessed the testicular growth of adolescent males followed nonsurgically for the presence of left varicocele. MATERIALS AND METHODS: We retrospectively reviewed the charts of adolescent males with a diagnosis of unilateral left varicocele and ultrasound testis volume measurements seen during a 10-year period. A total of 161 boys underwent at least 2 testicular ultrasounds as part of the evaluation for left varicocele. Patients were excluded from study for a history of inguinal/scrotal pathology or endocrinopathy that could affect testicular size. Sonographic testicular volume was calculated using the Lambert volume (length x width x height x 0.71). The resulting volumes were compared to previously published criteria for surgical repair (15%, 20% and 2 cc size differentials). RESULTS: Of the 71 boys with 3 followup ultrasounds 38 (54%) initially had a 15% or greater volume differential. After nonsurgical followup with ultrasounds for 2 years 60 boys (85%) had testicular volume differentials in the normal range (less than 15%). Of the patients 71% were spared potential surgery by size criteria and 50% were spared surgery by the same 15% volume differential criteria. CONCLUSIONS: Adolescent males with unilateral left varicocele often demonstrate asynchronous testicular growth that usually equalizes in time. Therefore, sonographic testicular size measurement at a single point during adolescence is insufficient to determine the need for varicocelectomy. When contemplating varicocelectomy we recommend at least 2, and preferably 3, testicular volume measurements 1 year apart to establish accurately decreased left testicular volume compared to a normal right testis.

Pediatric urologic oncology: bladder, prostate, testis.

Although treatment for bladder, prostate, and testis cancer comprises a large part of adult urologic practice, the tumors that affect these organs in children are rare. Rhabdomyosarcoma,which affects the bladder, prostate, vaginal, and paratesticular areas,is treated with a combination of surgery, chemotherapy, and radiation. Most transitional cell carcinomas of the bladder and prepubertal testis tumors are managed surgically owing to the low stage at presentation. Application of the technical advances learned in adults with tumors of the bladder, prostate, and testis, combined with an understanding of the difference in tumor biology, helps urologists improve the treatment of these tumors in children.

SIU/ICUD Consultation on Urethral Strictures: Urethral strictures in children.

A literature search was made through PubMed from 1990 to the present for articles on strictures in children. There were 32 articles that provided the data for this review. The studies were rated according to the level of evidence and the grade of recommendation using the International Consultations in Urologic Disease standards.

Pyocele of the scrotum in the pediatric patient.

Pyocele of the scrotum has been reported but is not well described in the pediatric population. The majority of published cases have been treated definitively with surgical drainage, and the severity of some cases has led to orchiectomy. We report a cases series of four boys with idiopathic pyocele, two of whom were managed successfully without operative intervention. Of these, one case was likely due to hematogenous spread of infection, and one case was secondary to spread of abdominal contamination via a patent processus vaginalis. To our knowledge, this is the first case series reporting non-surgical management of infant pyocele. Although rare, this clinical entity should be considered in the differential diagnosis of acute scrotum in the pediatric patient.

Genitourinary rhabdomyosarcoma: which treatment, how much, and when?

OBJECTIVE: To review the current management of pediatric genitourinary rhabdomyosarcoma (RMS). METHODS: Studies performed by the Intergroup Rhabdomyosarcoma Study Group, Children's Oncology Group (COG), International Society of Paediatric Oncology (SIOP) and others over the past 10 years were reviewed to compare the use of surgery, chemotherapy, and radiotherapy for treatment of RMS and their associated outcomes. RESULTS: Equivalent overall survival rates were reported in the last COG and SIOP trials, with worse event-free survival rates for bladder/prostate RMS in SIOP trials. The use of radiotherapy for local control was the main difference between current COG and SIOP protocols. Surgery is used to diagnose RMS, and for local control after chemotherapy. Chemotherapy is used for systemic control of RMS, but metastatic RMS will require new approaches. CONCLUSION: Risk stratification and risk-based therapy are being studied to decrease morbidity from treatment of RMS. The proper role of surgery vs radiotherapy for local control and whether additional treatment with second-line chemotherapy outweighs the avoidance of radiotherapy remain to be defined.

Cryptorchid testis histopathology in myelomeningocele patients.

PURPOSE: Cryptorchidism occurs in 25% of boys with myelomeningocele (MMC) compared to 3% of the general population. Testicular biopsy histopathology correlates with future sperm counts. We studied testicular histology in boys with cryptorchidism and MMC to investigate if the MMC influences histological findings. MATERIALS AND METHODS: The study group consisted of six patients with MMC and undescended testis (UDT) who underwent orchiopexy and bilateral testis biopsy. Twelve testicular biopsies from six patients were compared to 40 biopsies from 20 UDT-only controls. Total germ cell count per tubule (TGC/T) and the percentage of adult dark spermatogonia (%Ad) in undescended and contralateral descended testes from the patients were compared with controls. RESULTS: In the study group, two had total absence of germ cells (TGC/T=0) and three had severely reduced germ cells (TGC/T<0.2). Four had total absence of Ad spermatogonia and the remaining two had severely reduced Ad spermatogonia (%Ad=5). The mean TGC/T and %Ad in patients with UDT and MMC were conspicuously lower than controls. The differences did not reach statistical significance (P=0.09-0.29). CONCLUSION: These results suggest that patients with both MMC and UDT have a more severe reduction in total number and more severely delayed maturation of germ cells than do patients with UDT alone. With only six patients in this study, there was not the power to detect statistical significance. In addition to the reproductive problems due to erection and ejaculatory dysfunction in patients with MMC, this severe testicular histopathology may increase the risk of subfertility.

Sexual function in adult patients with spina bifida and its impact on quality of life.

PURPOSE: We evaluated sexual function in adult patients with spina bifida and its impact on quality of life. MATERIALS AND METHODS: Between March 2005 and May 2006, 76 adult patients with spina bifida, including 34 women and 42 men, with a mean age of 24.4 years (range 18 to 37) were seen for followup at our institution. Following institutional review board approval data were collected from medical records and validated questionnaires were completed, including the Watts Sexual Function Questionnaire to assess sexual function and SF-36 to assess quality of life. RESULTS: Of the 76 patients 18 (24%), including 9 women and 9 men, achieved sexual intercourse at least once in the last 2 months. There was no difference regarding gender distribution and mean age +/- SD in sexually active vs not sexually active patients (25.8 +/- 4.2 vs 24 +/- 4.5 years, p = 0.13). All levels (thoracic to sacral) of myelomeningocele were seen in the 2 groups with significant higher lesions of neurological impairment in not sexually active than in sexually active patients. No difference was seen in relation to ambulatory status and urinary incontinence. Overall total Watts Sexual Function Questionnaire scores in sexually active patients were similar in men and women. When comparing the 4 domain scores on desire, arousal, orgasm and satisfaction, women scored similar to men. Only 2 men tried to achieve paternity but failed and 1 woman gave birth. When evaluating SF-36 for quality of life, there was no significant difference in physical health (42.4 +/- 11.9 vs 38.7 +/- 7.2, p = 0.11) and mental health (54.1 +/- 11.3 vs 58.6 +/- 10.7, p = 0.12) subscores in sexually active vs not sexually active patients. CONCLUSIONS: In our cohort 24% of adult patients with spina bifida were sexually active. Sexual activity was not related to gender, level of urinary incontinence or extent of physical disability but it was more likely in patients with more caudal levels of neurological impairment. Sexual function seems not to affect health related quality of life in these patients.

Urethral dilation as treatment for neurogenic bladder.

PURPOSE: We documented the change in bladder physiology and clinical outcomes after urethral dilation for managing neurogenic bladder. MATERIALS AND METHODS: Female patients with neurogenic bladder were identified who underwent urethral dilation due to failure to empty at low pressure or upper tract changes. Urethral dilation was performed using curved sounds to 36Fr (range 18Fr to 36Fr). Clinical charts were reviewed to document urodynamics, infection rates, symptomatology, vesicoureteral reflux and upper tract dilatation before and after dilation. RESULTS: A total of 19 children underwent urethral dilation a mean of 2 times. Mean age at first dilation was 19.3 months. Mean followup was 34.9 months. The indication for dilation was high detrusor leak point pressure in 16 children. The remaining children had recurrent infections (1), poor compliance (1) and bladder spasms (1). Clinical manifestations of increased detrusor leak point pressure in 16 children were vesicoureteral reflux in 11, upper tract dilatation in 9 and recurrent infections in 3. Long-standing improvement occurred in 12 of 16 patients with increased detrusor leak point pressure, in 0 of 1 with infection, in 0 of 1 with poor compliance and in 1 of 1 with bladder spasms. Overall improvement was noted in 13 of 19 cases (68%). Vesicoureteral reflux was identified in 14 of 19 patients. Improvement was seen in 6 of 14 cases, including complete resolution in 5 and improvement in 1. Hydronephrosis was seen in 11 of 19 patients with improvement in 7, including resolution in 1 and improvement in 6. Only 6 children required further surgical intervention, that is vesicostomy in 2 and reimplantation in 4. CONCLUSIONS: Urethral dilation is effective for lowering detrusor leak point pressure in children with neurogenic bladder and for improving upper tract dilatation and vesicoureteral reflux. This procedure may prevent the need for more complicated surgical intervention. It should be investigated further in well designed, prospective studies.