Exstrophic bladder duplication in the sagittal plane: Surgical management of a rare case.

INTRODUCTION: The bladder-Exstrophy-Epispadias complex (BEEC) contains a wide spectrum of congenital malformations. A treatment naïve refugee was referred to our center with what was identified as BEEC. MATERIALS AND METHODS: A 27-year-old female patient was referred for total incontinence since birth by the general practitioner from the refugee center. An exstrophic bladder with blind ending ureteral orifices and a second non-exstrophic bladder with two orthotopic ureters was identified, demonstrating the bladder duplication in the sagittal plane. Laparotomy was performed, dissecting the exstrophic bladder plate caudally and using it as a ventral onlay to augment the non-exstrophic bladder. A Mitchell-type bladder neck reconstruction was performed with an autologous fascia sling around the bladder neck to obtain continence. As the patient had never voluntarily voided, chances of spontaneous voiding after surgery were low. Therefore creation of a continent Mitrofanoff-type vesicostomy was additionally realized and genital reconstruction was achieved. RESULTS: 12 months post operatively, the patient was completely continent, had a bladder capacity of 250 ml, and performed self-catheterization 5 times a day. No post-operative complications were observed. CONCLUSION: Admission of political refugees can implicate challenging surgeries for congenital malformations in adults, such as BEEC. This demonstrates the importance of multidisciplinary transitional care.

Patient information video: How to take care of your child after hypospadias surgery?

INTRODUCTION: Patient/parent education and participation helps improve post-operative care. Dressing and catheter care after hypospadias surgery varies widely and young parents are keen to use available media when seeking for help, especially if surgery is done in an outpatient setting. An information video about post-operative care after hypospadias is made available through a tertiary referral hospital's website. MATERIALS AND METHODS: Hypospadias surgery is an outpatient surgery in our setting. A double diaper system is used to prevent contamination of the urinary catheter and penile bandage with stools. A video explaining how the dressing works was made, helping parents maintaining the dressing and thereby solving possible questions/problems about dressing or medications while at home. Warning signs and symptoms are mentioned in which case parents should contact the urologist: fever, continuous blood loss and lack of urinary output. RESULTS: An information folder summarizing the latter, including a link to the video is given to all parents pre-operatively, providing reassurance according to parents' feedback. CONCLUSION: Parent participation can help improve post-operative care, especially in outpatient clinic setting. This video helps parents through post-operative care after hypospadias surgery. Its availability through informative folders and hospital's website is destined to educate and reassure parents.

Urethral duplication as cause of incontinence in a 5-years-old female: Surgical approach.

INTRODUCTION: Duplication of the urethra is a rare congenital anomaly in females which, if left unidentified, might cause refractory urinary incontinence. This video demonstrates its identification and treatment. PATIENT & METHODS: A 5-year-old female was referred for primary refractory diurnal incontinence without any dry moment since birth. Initial clinical examination showed permanent urine leakage from an opening cranial to the clitoris. Ultrasound did not show any bladder or kidney abnormalities. A diagnostic cystoscopy under anesthesia revealed an accessory meatus located cranially to the orthotopic meatus and to the clitoris. RESULTS: Lying in dorsal decubitus under general anesthesia, catheters were inserted in respectively the orthotopic and accessory meatus. The accessory meatus and urethra were dissected away into the bladder dome from a circumferential skin incision. Defects were closed and bladder drainage per urethram was left in situ for 10 days. P ostoperative recovery was uncomplicated. Complete resolution of the incontinence was immediately achieved at catheter removal. Daytime and night-time continence are observed with 3 years follow-up. CONCLUSION: Urethral duplication is a rare finding in females but should be excluded during the workup of primary incontinence. In girls with isolated urethral duplication, resection of the accessory urethra is the best surgical option.

Androgen receptor expression in preputial dartos tissue correlates with physiological androgen exposure in congenital malformations of the penis and in controls.

INTRODUCTION: The androgen receptor (AR) plays an important role in the development of male genitalia, and impaired androgen signalling has been hypothesised to underlie congenital penile malformations (CPM) such as hypospadias. Previous studies exploring the role of AR expression in the development of CPM have yielded conflicting results. OBJECTIVES: To assess AR expression in human foreskin of boys/men born with hypospadias, buried penis versus controls. STUDY DESIGN: Foreskin samples of 428 boys and men undergoing primary penile surgery (198 controls, 197 hypospadias, and 33 buried penis) were collected between October 2013 and July 2018. AR staining was performed in all samples and semi-quantitatively scored by two researchers independently, using a modified quick score (mQuicks) that assesses the proportion and intensity of AR staining in smooth muscle fibres. RESULTS: The interobserver variability of the mQuicks had a high level of agreement for the total score, as well as for the subscores. Two phases of high AR expression were observed in all groups, the first following the postnatal gonadotropin surge (i.e., mini-puberty) and the second in (pre-) puberty. No differences in AR expression were found in hypospadias or buried penis cases as compared to controls matched for age at time of surgery. DISCUSSION: This study describes the physiological evolution in AR expression in the human foreskin of boys with CPM and explains the cause of the previously reported, conflicting results. Despite the very large cohort, the limitations of this study are the low number of cases younger than six months at the time of surgery and the lack of Tanner stages to correlate with the mQuicks in adolescents. CONCLUSIONS: The mQuicks is a straightforward and informative tool to semi-quantitatively assess AR expression in the dartos tissue. In this study, AR expression in human foreskin shows a bimodal distribution in boys with CMP and controls, following physiological androgen exposure. No statistically significant difference in AR expression could be found between both groups. Whether other local mechanisms are affected by these physiological changes is currently unclear. However, strict age-matching should be considered when exploring the mechanisms underlying disturbed penile and urethral development in CMP.

Urethroplasty for urethral injuries and trauma-related strictures in children and adolescents: a single-institution experience.

INTRODUCTION: Urethral injuries and trauma-related strictures (UITSs) in children are rare. The treatment is challenging but crucial to avoid life-long urinary complications such as recurrent stricture formation, urinary incontinence, and impotence. OBJECTIVE: The aim was to report on the surgical and functional outcome of urethroplasty for UITSs and to provide data on patient-reported outcome measures (PROMs). MATERIAL AND METHODS: Between November 2001 and October 2017, 18 male children (≤18 years; median: 13 years) underwent urethroplasty for UITSs at a single tertiary referral center. Etiology was iatrogenic in five (27.8%), perineal straddle injury in six (33.3%) and pelvic fracture urethral injury (PFUI) in seven (38.8%) patients. PFUIs and short (≤3 cm) bulbar strictures were treated by transperineal anastomotic repair (n = 15; 83.3%), whereas a long bulbar stricture and a penile stricture were treated by, respectively, a preputial skin graft and flap urethroplasty. A penetrating penile urethral injury during circumcision underwent early exploration with primary repair of the laceration. Failure was defined as need for additional urethral instrumentation. PROMs were sent to patients ≥16 years at the latest evaluation. RESULTS: Median follow-up was 57 (range: 8-198) months. No complications and grade 1, 2, and 3 were present in, respectively, 13 (72.2%), two (11.1%), one (5.6%), and two (11.1%) patients. The success rate in a tertiary referral center was 94.4%. An immediate failure was observed in a patient with a PFUI and concomitant bladder neck injury. PROMs were available in 12 patients. Four patients (33.3%) reported erectile dysfunction. Post-void dribbling (25%) and urgency (50%) were the most frequently reported complaints. All patients were satisfied after urethroplasty and stated that they would undergo the surgery again. DISCUSSION: This series corroborates the recent trend in favor of transperineal anastomotic repair for PFUI, with combined abdominoperineal approach reserved for complex situations (e.g. bladder neck injury). For anterior UITSs, adaption of the technique to the characteristics of UITSs (etiology, location, length, and quality of graft bed) yielded excellent outcomes. Future systematic use of PROMs is also needed in children to elucidate the impact of urethroplasty on the urinary and sexual function. CONCLUSION: External trauma is the most important etiology of UITSs, but iatrogenic causes should not be neglected. Urethroplasty, mainly by anastomotic repair (AR) but with the technique adapted to local stricture characteristics if necessary, has an excellent long-term success rate in experienced hands. Functional disturbances are frequent, but despite this, patient satisfaction is high after urethroplasty.

Robot-assisted resection of ectopic kidney in children: An anatomical illustration.

INTRODUCTION: Failure of kidney migration during embryonic life results in an ectopic kidney, with an incidence varying from 1 in 500 to 1 in 1200. Pelvic kidney can be a rare cause of recurrent urinary tract infection (UTI), warranting nephrectomy in some cases. MATERIAL AND METHODS: A 7-year-old girl with a history of recurrent UTIs and vaginal discharge was diagnosed with a dysplastic afunctional ectopic kidney located in the pelvis, with ureter draining into the cervix or vaginal wall. RESULTS: A robot-assisted approach was chosen, with side docking of the robot to allow concurrent vaginoscopic/hysteroscopic exploration. A uterus bicornis was found. The ectopic ureter was dissected toward its drainage in the vaginal wall, where it was sutured and resected. The dysplastic kidney and ureter were removed. Compared to pure laparoscopic approach, dissection deep into the pelvis toward the vaginal wall is aided by the robotic dexterity and facilitates complete resection of the structure, which avoids leaving a ureteric stump into the vaginal wall. CONCLUSIONS: Robot-assisted surgery is a safe and effective option for resection of ectopic kidneys with ectopic ureter in children. Dissection up until the vaginal wall in children is aided by robotic dexterity, making this kind of surgery the ideal indication for robotic-assisted surgery.

Gonadal dysgenesis in disorders of sex development: Diagnosis and surgical management.

Recent studies on gonadal histology have improved the understanding of germ cell malignancy risk in patients with disorders of sex development (DSD), and evidence-based gonadal management strategies are gradually emerging. Especially in 46,XY DSD and 45,X/46,XY DSD, which are characterized by gonadal dysgenesis, the risk of germ cell malignancy is significantly increased. This paper summarized the progress over the past 10 years in malignancy risk assessment in patients with DSD, and its implications for optimal surgical handling of the involved gonads.

Global minimally invasive pyeloplasty study in children: Results from the Pediatric Urology Expert Group of the European Association of Urology Young Academic Urologists working party.

INTRODUCTION: Minimally invasive pyeloplasty (MIP) for ureteropelvic junction (UPJ) obstruction in children has gained popularity over the past decade as an alternative to open surgery. The present study aimed to identify the factors affecting complication rates of MIP in children, and to compare the outcomes of laparoscopic (LP) and robotic-assisted laparoscopic pyeloplasty (RALP). MATERIALS AND METHODS: The perioperative data of 783 pediatric patients (<18 years old) from 15 academic centers who underwent either LP or RALP with an Anderson Hynes dismembered pyeloplasty technique were retrospectively evaluated. Redo cases and patients with anatomic renal abnormalities were excluded. Demographics and operative data, including procedural factors, were collected. Complications were classified according to the Satava and modified Clavien systems. Failure was defined as any of the following: obstructive parameters on diuretic renal scintigraphy, decline in renal function, progressive hydronephrosis, or symptom relapse. Univariate and multivariate analysis were applied to identify factors affecting the complication rates. All parameters were compared between LP and RALP. RESULTS: A total of 575 children met the inclusion criteria. Laparoscopy, increased operative time, prolonged hospital stay, ureteral stenting technique, and time required for stenting were factors influencing complication rates on univariate analysis. None of those factors remained significant on multivariate analysis. Mean follow-up was 12.8 ± 9.8 months for RALP and 45.2 ± 33.8 months for LP (P = 0.001). Hospital stay and time for stenting were shorter for robotic pyeloplasty (P < 0.05 for both). Success rates were similar between RALP and LP (99.5% vs 97.3%, P = 0.11). The intraoperative complication rate was comparable between RALP and LP (3.8% vs 7.4%, P = 0.06). However, the postoperative complication rate was significantly higher in the LP group (3.2% for RALP and 7.7% for LP, P = 0.02). All complications were of no greater severity than Satava Grade IIa and Clavien Grade IIIb. DISCUSSION: This was the largest multicenter series of LP and RALP in the pediatric population. Limitations of the study included the retrospective design and lack of surgical experience as a confounder. CONCLUSIONS: Both minimally invasive approaches that were studied were safe and highly effective in treating UPJ obstruction in children in many centers globally. However, shorter hospitalization time and lower postoperative complication rates with RALP were noted. The aims of the study were met.

Buccal Versus Lingual Mucosa Graft in Anterior Urethroplasty: A Prospective Comparison of Surgical Outcome and Donor Site Morbidity.

PURPOSE: We prospectively compared buccal mucosa graft and lingual mucosa graft urethroplasty with respect to donor site morbidity and urethroplasty outcome. MATERIALS AND METHODS: Patients treated with buccal mucosa graft (29) or lingual mucosa graft (29) urethroplasty were included in the study. Oral pain and morbidity were assessed using the numeric rating scale (scale 0 to 10) as well as an in-home questionnaire administered 3 days, 2 weeks and 6 months postoperatively. RESULTS: After a mean (± SD) followup of 30 (± 13) months successful urethroplasty was achieved in 24 (82.8%) and 26 (89.7%) patients treated with buccal mucosa graft and lingual mucosa graft, respectively (p = 0.306). Median numeric rating scale after 3 days, 2 weeks and 6 months was 4, 2 and 0 for buccal mucosa graft and 6, 3 and 0 for lingual mucosa graft, respectively, with no statistical differences between the groups. At day 3 significantly more patients in the lingual mucosa graft group had severe difficulties with eating and drinking (62.1% vs 24.1%, p = 0.004) and speaking (93.1% vs 55.2%, p = 0.001), and had dysgeusia (48.3% vs 13.8%, p = 0.01). Two weeks postoperatively speech impairment was still more frequent with lingual mucosa graft (55.2% vs 13.8%, p = 0.002), whereas oral tightness was more frequent with buccal mucosa graft (41.4% vs 6.9%, p = 0.005). After 6 months 44.8% and 31% of patients treated with buccal mucosa graft and lingual mucosa graft, respectively, still reported sensitivity disorders (p = 0.279). CONCLUSIONS: The success of urethroplasty with lingual and buccal mucosa grafts was similar. Oral pain was not different after both grafts. In the early postoperative period there were differences in oral morbidity between buccal and lingual mucosa grafts. Long-term oral morbidity was not infrequent with both grafts.

Congenital penile pathology is associated with abnormal development of the dartos muscle: a prospective study of primary penile surgery at a tertiary referral center.

PURPOSE: Pathophysiological mechanisms leading to chordee in patients with hypospadias and to the hidden state of buried penis in the prepubic fat remain unclear. Resection of dartos tissue usually makes the penis straight in patients with hypospadias and corrects it in those with buried penis, suggesting a common pathophysiology related to dartos tissue. MATERIALS AND METHODS: Tissue samples from 113 children undergoing primary penile surgery for hypospadias (94 patients), epispadias (1) or buried penis (18) were collected between November 2011 and September 2013. Tissue samples from 79 children undergoing circumcision for nonmedical reasons served as controls. All samples were stained with smooth muscle actin and analyzed by the same pathologist, who was blinded to indication for surgery. Chi-square and Fisher exact tests were applied. RESULTS: Three different dartos tissue patterns were observed. Pattern I (normal) consisted of smooth muscle fibers of dartos tissue organized in a parallel configuration in the subcutaneous tissue. Pattern II was characterized by poorly developed and hypotrophic smooth muscle fibers. Pattern III was determined by randomly distributed smooth muscle fibers in the subcutaneous tissue, without parallel configuration. Pattern I was observed in 45 circumcision specimens (64%). Of buried penis cases 78% were considered abnormal (pattern II in 4 cases and III in 10, p = 0.001). Of hypospadias cases 70% were considered abnormal (pattern II in 31 cases, III in 32, and mixed II and III in 3, p < 0.001). The only epispadias case was designated pattern II. CONCLUSIONS: Congenital penile pathology (hypospadias, buried penis) is associated with structural anomalies in dartos tissue. Further research is needed to unveil the pathophysiology of the condition.

Revision of perineal urethrostomy using a meshed split-thickness skin graft.

Perineal urethrostomy is considered to be the last option to restore voiding in complex/recurrent urethral stricture disease. It is also a necessary procedure after penectomy or urethrectomy. Stenosis of the perineal urethrostomy has been reported in up to 30% of cases. There is no consensus on how to treat a stenotic perineal urethrostomy, but, in general, a form of urinary diversion is offered to the patient. We present the case of a young male who underwent perineal urethrostomy after urethrectomy for urethral cancer. The postoperative period was complicated by wound dehiscence with subsequent complete obliteration of the perineal urethrostomy. Revision surgery was performed with reopening of the obliterated urethral stump and coverage of the skin defect between the urethra and the perineal/scrotal skin with a meshed split-thickness skin graft. To date, this patient is voiding well and satisfied with the offered solution.

Preoperative planning of a pedicled anterolateral thigh (ALT) flap for penile reconstruction with the multidetector CT scan.

INTRODUCTION: Recently, perforator flap surgery has been introduced in phalloplasty procedures. Especially the anterolateral thigh (ALT) flap has found its application as a pedicled flap for the penile reconstruction. Adequate shaping of the flap and the need of transferring the shaped flap on its pedicle to the pubic area requires precise localisation and preoperative evaluation of the perforators. Also preoperative measurement of the subcutaneous fat tissue is necessary to allow adequate patient selection and optimal shaping of the phallus. The objective of this report is to demonstrate the usefulness of a multidetector CT scan (MDCT) in the preoperative planning of patients undergoing an ALT flap phalloplasty. METHODS: Between September 2009 and July 2011, 13 patients were operated for ALT phalloplasty and had preoperative perforator mapping with the MDCT. An algorithm was set up to select the best perforator. Indocyanine green angiography was used in 5 patients to confirm the perforator selection. A mathematical formula was developed to calculate the necessary flap width from the flap thickness. RESULTS: Accurate identification of the main perforators was achieved in all patients with a very satisfactory concordance between the MDCT scan and surgical findings. Indocyanine green angiography confirmed the MDCT perforator selection in all cases. The flap size could be determined preoperatively in all patients by measuring the thickness of the subcutaneous fat layer. CONCLUSIONS: Preoperative evaluation of ALT perforators and the subcutaneous fat tissue layer is feasible with an MDCT scan and provides precise data to make an adequate patient and perforator selection and determine the exact flap size.

Urethral reconstruction using buccal mucosa or penile skin grafts: systematic review and meta-analysis.

INTRODUCTION: Different types of grafts have been described in urethral reconstruction (UR), with penile skin graft (PSG) and buccal mucosa graft (BMG) as the most frequently used ones. It still remains unclear whether one graft is superior in terms of success when compared to the other. MATERIAL AND METHODS: A systematic review of the literature was performed searching the MEDLINE database with the following search strategy: 'urethroplasty' AND 'penile skin'/ 'urethroplasty' AND 'buccal mucosa'. 266 and 144 records were retrieved for urethroplasty with PSG and BMG, respectively. These records were reviewed to identify papers where PSG and BMG were used in UR and where individualized data on success were available within the same series. RESULTS: 18 papers were found eligible for further analysis. In total, 428 and 483 patients were respectively treated with PSG or BMG. If available, follow-up duration was 64.1 versus 42.1 months (p < 0.0001) and stricture length 6.2 versus 4.6 cm (p < 0.0001) for PSG and BMG, respectively. Success of UR with PSG was 81.8 versus 85.9% with BMG (p = 0.01). CONCLUSIONS: Success of UR using BMG is significantly better compared to PSG. Results might be seriously biased by a longer follow-up duration and stricture length for PSG compared to BMG.

Pubertal androgenization and gonadal histology in two 46,XY adolescents with NR5A1 mutations and predominantly female phenotype at birth.

OBJECTIVE: Most patients with NR5A1 (SF-1) mutations and poor virilization at birth are sex-assigned female and receive early gonadectomy. Although studies in pituitary-specific Sf-1 knockout mice suggest hypogonadotropic hypogonadism, little is known about endocrine function at puberty and on germ cell tumor risk in patients with SF-1 mutations. This study reports on the natural course during puberty and on gonadal histology in two adolescents with SF-1 mutations and predominantly female phenotype at birth. DESIGN AND METHODS: Clinical and hormonal data and histopathological studies are reported in one male and one female adolescent with, respectively, a nonsense mutation (c.9T>A, p.Tyr3X) and a deletion of the first two coding exons (NCBI36/hg18 Chr9:g.(126306276-126307705)_(126303229-126302828)del) of NR5A1, both predicted to fully disrupt gene function. RESULTS: LH and testosterone concentrations were in the normal male range, virilization was disproportionate to the neonatal phenotype. In the girl, gonadectomy at 13 years revealed incomplete spermatogenesis and bilateral precursor lesions of testicular carcinoma in situ. In the boy, at the age of 12, numerous germ cells without signs of malignancy were present in bilateral testicular biopsy specimen. CONCLUSIONS: In SF-1 mutations, the neonatal phenotype poorly predicts virilization at puberty. Even in poorly virilized cases at birth, male gender assignment may allow spontaneous puberty without signs of hypogonadotropic hypogonadism, and possibly fertility. Patients with SF-1 mutations are at increased risk for malignant germ cell tumors. In case of preserved gonads, early orchidopexy and germ cell tumor screening is warranted. The finding of premalignant and/or malignant changes should prompt gonadectomy or possibly irradiation.

High dose chemotherapy and stem cell rescue for metastatic germ cell tumours: long-term results from a single institution.

PURPOSE: To evaluate the long term results and the characteristics of patients treated with high dose chemotherapy (HDCT) for an advanced germ cell tumour at Ghent University Hospital from 1996 to 2010. MATERIALS AND METHODS: A retrospective analysis of patients treated with HDCT for germ cell tumours was performed. Data about stage at diagnosis, different prognostic scoring systems, timing of HDCT, response to HDCT and relapse-free period were collected. The following endpoints were evaluated: complete or incomplete response to HDCT, relapse free survival and overall survival time. RESULTS: Of the 148 patients treated with chemotherapy for an advanced germ cell tumour from 1996 to 2010, 10 (6.8%) needed salvage treatment by means of HDCT. Six patients achieved a complete response to one cycle of HDCT and 2 additional patients achieved a complete response to a second cycle of HDCT. A retrospective analysis showed 8 long-term survivors with a maximum follow-up time of 152 months. Two patients were recently transplanted and are not evaluable for survival yet. CONCLUSIONS: Our study suggests that long-term survival can be obtained by means of HDCT for metastatic germ cell tumours, even in patients with bad prognostic features at diagnosis. The question of whether to use 1 or 2 cycles of HDCT still remains unanswered.

Penile reconstruction with the radial forearm flap: an update.

BACKGROUND: Many methods and many free or pedicled flaps have been used in phalloplasty. None of these techniques is able to completely fulfill the well described goals in penile reconstruction. Still, the radial forearm glap is currently the most frequently used flap and thus universally considered the gold standard. PATIENTS AND METHODS: Since 1992, we have performed the largest series of 316 radial forearm phalloplasties to date performed by a single surgical team. From these extensive data we critically evaluate how this current supposed gold standard can meet the requirements of an ideal penile reconstruction. RESULTS: We assessed outcome parameters such as number of procedures to achieve complete functional result, aesthetic outcome, tactile and erogenous sensation, voiding, donor site morbidity, scrotoplasty and sexual intercourse. CONCLUSION: While currently no controlled randomized prospective studies are available to prove the radial forearm flap is truly the 'gold standard' in penile reconstruction, we believe that our retrospective data support the radial forearm phalloplasty as a very reliable technique for the creation of a normal looking penis and scrotum. While full functionality is achieved through a minimum of 2 procedures, the patients are always able to void standing, and in most cases to experience sexual satisfaction. The relative disadvantages of this technique are the residual scar on the forearm donor site, the rather high number of initial urinary fistulas, the potential for long-term urological complications and the need for a stiffener or erection prosthesis. From our experience, we strongly feel that a structured multi-disciplinary cooperation between the reconstructive-plastic surgeon and the urologist is an absolute requisite to obtain the best possible technical results.

Gonadal pathology and tumor risk in relation to clinical characteristics in patients with 45,X/46,XY mosaicism.

CONTEXT: Gonadectomy is avoided whenever possible in boys with 45,X/46,XY. However, no clinical markers are currently available to guide clinicians in predicting gonadal tumor risk or hormone production. OBJECTIVE: The objective of the study was to test the hypothesis that gonadal histology and risk for development of a malignant germ cell tumor are reflected by the clinical presentation of a 45,X/46,XY individual. DESIGN: The design of the study was the correlation of clinical data [external masculinization score (EMS), pubertal outcome] with pathology data (gonadal phenotype, tumor risk). SETTING: This was a multicenter study involving two multidisciplinary disorder of sex development teams. PATIENTS: Patients included genetically proven 45,X/46,XY (and variants) cases, of whom at least one gonadal biopsy or gonadectomy specimen was available, together with clinical details. INTERVENTIONS: Patients (n = 48) were divided into three groups, based on the EMS. Gonadal histology and tumor risk were assessed on paraffin-embedded samples (n = 87) by morphology and immunohistochemistry on the basis of established criteria. MAIN OUTCOME MEASURES: Gonadal differentiation and tumor risk in the three clinical groups were measured. Clinical outcome in patients with at least one preserved gonad was also measured. RESULTS: Tumor risk in the three groups was significantly related to the gonadal differentiation pattern (P < 0.001). In boys, hormone production was sufficient and was not predicted by the EMS. CONCLUSIONS: The EMS reflects gonadal differentiation and tumor risk in patients with 45,X/46,XY. In boys, testosterone production is often sufficient, but strict follow-up is warranted because of malignancy risk, which appears inversely related to EMS. In girls, tumor risk is limited but gonads are not functional, making gonadectomy the most reasonable option.

Targeted therapy for metastatic renal cell cancer.

Renal cell cancer is a common urological malignancy for which the incidence has doubled over the last three decades. Thirty percent of patients have metastatic disease at the moment of diagnosis. Thirty percent of the remaining surgically treated patients will develop metastases. Before the introduction of targeted therapies the only therapeutic option for these patients was immunotherapy with bad tolerability and poor results. This review discusses these new 'targeted therapies'.

Reconstructive surgery for severe penile inadequacy: phalloplasty with a free radial forearm flap or a pedicled anterolateral thigh flap.

Objectives. Severe penile inadequacy in adolescents is rare. Phallic reconstruction to treat this devastating condition is a major challenge to the reconstructive surgeon. Phallic reconstruction using the free radial forearm flap (RFF) or the pedicled anterolateral thigh flap (ALTF) has been routinely used in female-to-male transsexuals. Recently we started to use these techniques in the treatment of severe penile inadequacy. Methods. Eleven males (age 15 to 42 years) were treated with a phallic reconstruction. The RFF is our method of choice; the ALTF is an alternative when a free flap is contraindicated or less desired by the patient. The RFF was used in 7 patients, the ALTF in 4 patients. Mean followup was 25 months (range: 4-49 months). Aesthetic and functional results were evaluated. Results. There were no complications related to the flap. Aesthetic results were judged as "good" in 9 patients and "moderate" in 2 patients. Sensitivity in the RFF was superior compared to the ALTF. Four patients developed urinary complications (stricture and/or fistula). Six patients underwent erectile implant surgery. In 2 patients the erectile implant had to be removed due to infection or erosion. Conclusion. In case of severe penile inadequacy due to whatever condition, a phalloplasty is the preferred treatment nowadays. The free radial forearm flap is still the method of choice. The anterolateral thigh flap can be a good alternative, especially when free flaps are contraindicated, but sensitivity is markedly inferior in these flaps.