Individualized care for patients with intersex (differences of sex development): Diagnosis and treatment of aphallia.

This review discusses issues and concerns in the management of aphallia, updating status of a post-pubertal individual who required further surgery after having initial surgery for aphallia as an infant. Through this case, which discusses an 18-year-old young adult who had penile agenesis, who desired further phalloplasty involving glanuloplasty and implantation of an erectile device, we highlight the importance of periodic evaluation and close follow up. Surgery during infancy or early childhood to create a penis is important for gender development in a boy, especially if there were functional testes during fetal life, even if this surgery would only be the first stage. There is a strong probability of subsequent surgery after initial phalloplasty before puberty, even with the use of currently refined techniques. Here we discuss the changing techniques that document the ongoing, continued refinement of these procedures, highlighting that further outcome data are needed to identify ways to further optimize current techniques.

Working towards convergence of the clinical management of differences of sex development/intersex conditions and the human rights framework: A case study.

OBJECTIVE: Medical treatments that aim to modify the appearance of the genitals in children who are born with a difference of sex development/intersex (DSD/I*) condition are highly controversial. Human Rights bodies worldwide have argued that such treatments are conflicting with the child's right of personal autonomy and should be legally restricted to the unique situation where the child's physical health is in danger. DESIGN: We here review the current status of legal initiatives in Europe that have addressed the issue of medical treatments in minors who have a DSD for which they have not been able to give personal informed consent due to their young age. PATIENTS: The management of a 3 years old child who has congenital adrenal hyperplasia (CAH) and grows up with atypical-looking genitals is discussed. RESULTS: In spite of extensive psychosocial support to the child and family from birth onwards, and good medical control of CAH, the child develops signs of emotional distress, suspected to be attributable to the genital difference. Our discussions include perspectives from the multidisciplinary DSD team caring for the child, a human rights specialist, and an intersex activist. From our discussions, we conclude that with evolving medical care, new ethical and human rights challenges are raised. A truly holistic human rights approach should not only consider physical but also mental health and psychosocial and psychosexual adaptation of the child to the medical condition, when reflecting on the acceptability of medical treatments in minors for which no personal informed consent can be obtained due to their young age. In addition it is paramount to include the meaningful participation of the child in the clinical management at the earliest possible stage. CONCLUSIONS: Continued convergence of clinical management and the human rights framework can be realised based on constructive discussions involving all stakeholders, and with the best interest of the child - and adult that they will become - as a common goal.

Secondary phallic prosthesis placement in transgender patients postmetoidioplasty: a case report on technique and outcome.

Introduction: Gender affirming surgery is common among transgender and gender-nonconforming individuals. Genital gender-affirming surgery is a form of surgery that involves transformation and reconstruction of the genitalia while maintaining urologic and sexual function. Masculinizing genital gender-affirming surgery can involve the removal of the female genital and reproductive organs and the creation of a more masculine appearance by using phalloplasty or metoidioplasty techniques. While metoidioplasty has advantages such as limited scarring and preserved genital sensation and clitoral erection, it may not always guarantee the ability to void while standing or even penetrate a sexual partner. Aim: To describe our method of secondary phallic enhancement after metoidioplasty with a phallic prosthesis. Methods: Our case is based on a 39-year-old transgender male who underwent metoidioplasty with vaginectomy, scrotoplasty, and urethral lengthening. However, the original surgery was complicated by extensive hematoma formation in the suprapubic area and neoscrotum, leading to a buried position of the neophallus. Subsequent surgery was performed to reduce the prepubic fat pad and penoscrotal interposition, but it too was complicated by hematoma formation and wound dehiscence. To maximize phallic elongation, the tethering urethra was removed, and perineal urethrostomy was performed but could not sufficiently alleviate the buried phallus. As a further step to improve the outcome, we proposed the implantation of a specific phallic prosthesis designed for metoidioplasty with testicular prosthesis placement. Surgery was performed as a single procedure through a penoscrotal incision and greatly enhanced genital appearance. Results: Three months after surgery, the patient had good sensation of the phallus and was able to have satisfactory sexual activity with his partner. However, he remained dissatisfied with the amount of prepubic tissue and opted to undergo further liposuction. Conclusion: Phallic prosthesis implantation can be a procedure to improve the condition of multioperated cases of metoidioplasty with resistant phallic burying. Reconstruction with a phallic prosthesis was performed successfully in this case. However, long-term complications and functional outcomes, such as the ability to void while standing or penetrate a sexual partner, remain uncertain. Further experience is needed to determine the efficacy and safety of using a phallic prosthesis as a secondary procedure in patients undergoing metoidioplasty.

Robot-assisted radical nephrectomy for Wilms' tumor in children.

INTRODUCTION: Surgical removal of the tumor is a key step in the management of nephroblastoma. Less invasive surgical approaches such as robot-assisted radical nephrectomy (RARN) has gained momentum over the past few years. This video presents a comprehensive step-by-step video for two cases: one uncomplicated left RARN and one more challenging right RARN. MATERIALS & METHODS: Following the UMBRELLA/SIOP protocol, both patients received neoadjuvant chemotherapy. Under general anesthesia, in a lateral decubitus position, four robotic and one assistant port are placed. After mobilization of the colon, the ureter and gonadal vessels are subsequently identified. The renal hilum is dissected, and the renal artery and vein are divided. The kidney is dissected with sparing of the adrenal gland. The ureter and gonadal vessels are divided, and the specimen is removed through a Pfannenstiel incision. Lymph node sampling is performed. RESULTS: Patients were 4 and 5 years old. The total surgical time was 95 and 200 min, with an estimated blood loss of 5 and 10 cc. The hospital stay was limited to 3 and 4 days. Both pathological reports confirmed the diagnosis of nephroblastoma, with tumour-free resection margins. No complications were observed 2 months postoperatively. CONCLUSION: RARN is feasible in children.

Robot-assisted pyeloplasty: How to deal with anatomic variants? A step-by-step video presentation.

INTRODUCTION: Pyeloplasty (open or Robot-assisted) is the gold standard of a symptomatic UPJ stenosis. Sometimes anatomic variants make the procedure challenging. This video describes a step-by-step approach in three settings: a crossing blood vessel and two different presentations of incomplete duplicated system. MATERIALS AND METHODS: Under general anesthesia, patient positioned in lateral decubitus, three trocars are placed. After mobilization of the colon, the Gerota's fascia is opened, and the renal pelvis is dissected off the surrounding structures. Ureter and obstructed pyelum were subsequently identified, mobilized, and hinged on a traction stitch. The pyelum and ureter are divided and spatulated according to the Anderson-Hynes technique; anastomosis is achieved. In variants, the drainage is one of the challenging steps, needing custom-made drainage of both moieties. Correct positioning of the drainage is confirmed with reflux of methylene blue from the bladder. RESULTS: JJ stent was removed 6 weeks postoperatively in surgical day-clinic, additional drainage was removed 1 week after surgery in the outpatient clinic. All three children remain asymptomatic with over a year of follow-up. CONCLUSION: A step-by-step plan for pyeloplasty in case of anatomic variants is presented with a video demonstrating a robot-assisted approach in duplicated systems. Moiety drainage can be challenging.

Principles and outcomes of gender-affirming vaginoplasty.

Vaginoplasty is the most frequently performed gender-affirming genital surgery for gender-diverse people with genital gender incongruence. The procedure is performed to create an aesthetic and functional vulva and vaginal canal that enables receptive intercourse, erogenous clitoral sensation and a downward-directed urine stream. Penile inversion vaginoplasty (PIV) is a single surgical procedure involving anatomical component rearrangement of the penis and scrotum that enables many patients to meet these anatomical goals. Other options include minimal-depth, peritoneal and intestinal vaginoplasty. Patient quality of life has been shown to improve drastically after vaginoplasty, but complication rates have been documented to be as high as 70%. Fortunately, most complications do not alter long-term postoperative clinical outcomes and can be managed without surgical intervention in the acute perioperative phase. However, major complications, such as rectal injury, rectovaginal fistula, and urethral or introital stenosis can substantially affect the patient experience. Innovations in surgical approaches and techniques have demonstrated promising early results for reducing complications and augmenting vaginal depth, but long-term data are scarce.

The self-assessment of genital anatomy, sexual function, and genital sensation (SAGASF-M) questionnaire in a Belgian Dutch-speaking male population: A validating study.

INTRODUCTION: Penile and genital surgery for congenital or acquired conditions is daily practice in reconstructive urology. These procedures, which carry the risk of disrupting nerves and blood vessels, may impair the genital sensation, and affect the capacity for sexual pleasure. Self-reported tools are needed to systematically assess the male genitalia before and after reconstructive surgeries in terms of genital sensation and sexual experience. AIM: This study validated the Dutch translation of the "self-assessment of genital anatomy and sexual functioning in male" (SAGASF-M) questionnaire and investigated the perceptions of healthy men regarding their genital anatomy and sensory function. METHODS: Eight hundred and eight sexually active men with a median age of 39 years (18-79 years) and no history of genital procedures other than circumcision filled out an online version of the questionnaire. Twenty-four participants were randomly recruited to confirm the responses of the "self-assessment of genital anatomy and sexual functioning in male" questionnaire by a clinical evaluation. MAIN OUTCOME MEASURES: The "self-assessment of genital anatomy and sexual functioning in male" questionnaire comprises of multiple-choice questions and clarifying illustrations asking men to rate their genital appearance, overall sexual sensitivity, and pain perception as well as the intensity and the effort to reach orgasm. Prespecified regions of the glans, penile shaft, scrotum, perineum, and anus are evaluated through this questionnaire. RESULTS: Only slight variability in anatomical ratings was observed. Overall discrimination between different genital areas in terms of genital sensation was significant. The bottom of the glans or frenular area was rated the highest contributor to "sexual pleasure," followed by the other regions of the glans and shaft. The same distribution was found for "orgasm intensity" and "orgasm effort." The anal region was generally rated the lowest. "Discomfort/pain" was rated lower than any of the other sensory function indicators and the top of the glans and anal region were rated most likely to perceive this unpleasant sensation. Participants reported significantly more sexual pleasure and intense orgasms when stimulated by a sexual partner than self-stimulation. Homosexual and bisexual men reported a higher contribution of the perineal and anal regions in sexual pleasure and orgasm. No significant difference between circumcised and uncircumcised individuals regarding overall genital sensation could be found. CONCLUSION: The Dutch translation of the SAGASF-M questionnaire is a valuable and reliable tool for self-assessment of genital anatomy and sensation, providing a site-specific attribution of a patient's perceived sexual function. Further prospective research with this questionnaire could aid in the patient-centered improvement of genital surgery.

Validation of the modified Bosniak classification system to risk stratify pediatric cystic renal masses: An international, multi-site study from the pediatric urologic oncology working group of the societies for pediatric urology.

BACKGROUND: Pediatric cystic renal lesions are challenging to manage as little is known about their natural course. A modified Bosniak (mBosniak) classification system has been proposed for risk stratification in pediatric patients that takes ultrasound (US) and/or computed tomogram (CT) characteristics into account. However, literature validating this system remains limited. OBJECTIVE: To determine if the mBosniak classification system correlates with pathologic diagnoses. The hypothesis is that mBosniak classification can stratify the risk of malignancy in children with renal cysts. STUDY DESIGN: Patients treated for cystic renal masses with available imaging and pathology between 2000 and 2019 from five institutions were identified. Clinical characteristics and pathology were obtained retrospectively. Characteristics from the most recent US, CT, and/or magnetic resonance imaging (MRI) were recorded. Reviewers assigned a mBosniak classification to each scan. mBosniak scores 1/2 were considered low-risk and 3/4 high-risk. These groups were compared with pathology (classified as benign, intermediate, malignant). Sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), positive likelihood ratio (+LR), and negative likelihood ratio (-LR) were calculated to assess this categorization as a screening tool to guide surgical intervention. Agreement between imaging modalities was also explored. RESULTS: 99 patients were identified. High-risk imaging findings were correlated with malignant or intermediate pathology with a sensitivity of 88.3%, specificity of 84.6%, PPV of 89.8%, NPV of 82.5%, +LR of 5.7, and -LR of 0.14. The sensitivity for detecting malignant lesions only was 100%. There was substantial agreement between US/CT (n = 55; κ = 0.66) and moderate agreement between US/MRI (n = 20; κ = 0.52) and CT/MRI (n = 13; κ = 0.47). DISCUSSION: The mBos classification system is a useful tool in predicting the likelihood of benign vs. intermediate or malignant pathology. The relatively high sensitivity and specificity of the system for prediction of high-risk lesions makes this classification applicable to clinical decision making. In addition, all malignant lesions were accurately identified as mBosniak 4 on imaging. This study adds substantial data to the relatively small body of literature validating the mBosniak system for risk stratifying pediatric cystic renal lesions. CONCLUSIONS: Pediatric cystic renal lesions assigned mBosniak class 1/2 are mostly benign, whereas class 3/4 lesions are likely intermediate or malignant pathology. We observed that the mBosniak system correctly identified pathology appropriate for surgical management in 88% of cases and did not miss malignant pathologies. There is substantial agreement between CT and US scans concerning mBos classification.

Treatment of Urethral Strictures in Transmasculine Patients.

BACKGROUND: Urethral strictures are a common complication after genital gender-affirming surgery (GGAS) in transmasculine patients. Studies that specifically focus on the management of urethral strictures are scarce. The aim of this systematic review is to collect all available evidence on the management of urethral strictures in transmasculine patients who underwent urethral lengthening. METHODS: We performed a systematic review of the management of urethral strictures in transmasculine patients after phalloplasty or metoidioplasty (PROSPERO, CRD42021215811) with literature from PubMed, Embase, Web of Science and Cochrane. Preferred Reporting Items for Systematic reviews and Meta-Analysis-(PRISMA) guidelines were followed, and risk of bias was assessed for every individual study using the 5-criterion quality appraisal checklist. RESULTS: Eight case series were included with a total of 179 transmasculine patients. Only one study discussed the management of urethral strictures after metoidioplasty. Urethral strictures were most often seen at the anastomosis between the fixed and pendulous urethra. For each stricture location, different techniques have been reported. All studies were at a high risk of bias. The current evidence is insufficient to favor one technique over another. CONCLUSIONS: Different techniques have been described for the different clinical scenarios of urethral stricture disease after GGAS. In the absence of comparative studies, however, it is impossible to advocate for one technique over another. This calls for additional research, ideally well-designed prospective randomized controlled trials (RCTs), focusing on both surgical and functional outcome parameters.

Long-term outcome of urethral and genital reconstruction in hypospadias and exstrophy-epispadias complex.

PURPOSE OF REVIEW: Although immediate surgical outcomes of genitourinary reconstruction used to be the main focus in hypospadias and exstrophy-epispadias complex (EEC), recent research demonstrates rising concerns about long-term functional and psychosexual outcomes. Recent results about long-term outcomes of complex genitourinary reconstruction in those children transitioning into adulthood are summarized and discussed in this comprehensive review. RECENT FINDINGS: Long-term outcomes in hypospadias focus on psychosexual satisfaction and decisional regret. Interestingly, uncomplicated hypospadias repair results in equal satisfaction rates to those found in controls. Most adult patients are happy with their parents' decision to have them undergo surgery during childhood. No decisional regret was found in parents. Reinterventions were associated with a decline in satisfaction, as was a decrease in perceived penile length. Long-term, males with EEC are more dissatisfied with penile length than with continence problems. In cases of severe penile insufficiency, phalloplasty shows promising psychosexual outcomes at the price of a high complication rate. Female EEC patients show higher rates of pregnancy complications, portending a higher risk of miscarriage. SUMMARY: Psychosexual satisfaction of patients with repaired hypospadias transitioning into adulthood demonstrates equal satisfaction rates to those found in controls, without decisional regret associated with surgery during childhood. In EEC, more complications are seen when transitioning into a sexually active adult life.

Urethral Complications After Metoidioplasty for Genital Gender Affirming Surgery.

BACKGROUND: Possible options of genital gender affirming surgery in transmasculine are metoidioplasty or phalloplasty. As opposed to phalloplasty, no flapbased neophallic reconstruction is needed in metoidioplasty. Urethral lengthening is needed in metoidioplasty if the patient desires voiding at the tip of the neophallus. This urethral lengthening poses the patient at risk for urethral complications. AIM: Our primary goal was to describe the morbidity and specific the urethral complications related to metoidioplasty. Second, we sought for predictors of these urethral complications. METHODS: Our institutional database was retrospectively analyzed to identify transmasculine who underwent metoidioplasty between 2006 and 2020. This cohort was further evaluated for surgical morbidity, urethral complications and potential predictors for urethral complications. OUTCOMES: The rate of surgical morbidity and urethral complications (temporary/permanent fistula, stricture or fistula and stricture) was calculated. Potential predictors evaluated herein were BMI, concomitant vaginectomy, active smoking and additional urethral lengthening (AUL). They were tested with logistic regression analysis with calculation of Odds Ratio (OR). RESULTS: Seventy-four patients underwent metoidioplasty with a median follow-up of 44 months. Median age was 26 years. AUL was done in 36 (48.6%) patients and established by a transverse preputial skin island and labium minus flap in respectively 34 and 2 patients. Within 30 days after metoidioplasty, 3 (4.1%) high-grade complications were noted. Urethral complications of any kind were noted in 42 (56.8%) patients. All fistulas, permanent fistulas and strictures were seen in resp. 34 (45.9%), 27 (36.5%) and 14 (18.9%) patients. AUL is a significant predictor for all urethral complications (OR 15.5), strictures (OR 24.5), all fistula's (OR 6.07) and permanent fistulas (OR 3.83). In contrast, smoking is only a predictor for all fistulas (OR 6.54) and permanent fistulas (OR 3.76). CLINICAL IMPLICATIONS: Obtaining information about the risk of complications is important in preoperative patient counselling. Patient who desires AUL are at higher risk to develop urethral complications and patients who continue to smoke at the period of metoidioplasty have a higher risk of fistula formation. STRENGTH & LIMITATIONS: Sufficient events to calculate predictors for urethral complications. However, this is a retrospective study with still a small number of patients with a relative short follow-up. CONCLUSION: Urethral complications are frequent after metoidioplasty and approximately 50% needs corrective surgery. AUL is an independent risk factor for fistula and stricture formation, whereas smoking is a risk factor for fistula formation. Waterschoot M, Hoebeke P, Verla W, et al. Urethral Complications After Metoidioplasty for Genital Gender Affirming Surgery. J Sex Med 2021;18:1271-1279.

Long-Term fate of the upper urinary tract and ITS association with continence in exstrophy patients.

INTRODUCTION: Scarce data is available in literature about the upper urinary tract outcomes of patients with Exstrophy-Epispadias Complex (EEC). After bladder closure during childhood, EEC bladders can become hostile to the upper tracts after bladder by exposing them to high pressures, leading to hydronephrosis (HN) and kidney damage. Similarly, vesicoureteral reflux (VUR) may be present and increase the likelihood for pyelonephritis. OBJECTIVE: We sought to assess long-term upper urinary tract outcomes by evaluating renal function, HN and VUR; and to assess if upper urinary tract outcomes are associated with continence status. STUDY DESIGN: A retrospective review of EEC patients having ≥1 surger(y) (ies) at our institution from 1990 until 2019 was performed. Renal function was assessed by evaluating last available estimated glomerular filtration rate (eGFR) and creatinine values. HN was assessed on ultrasound and classified according to the SFU-classification. Patients with recurrent febrile urinary tract infections (UTI) or pyelonephritis underwent a voiding-cystourethrogram (VCUG) assessing VUR, graded following the 'International system of radiographic grading of VUR'. Descriptive and comparative statistical analysis were performed to assess if upper tract outcomes are associated with continence status. RESULTS: Forty-eight patients (75% male) had a median (IQR) follow-up of 18 (10-21) years. The table shows upper tract outcomes for the entire group and stratified by continence status. The median creatinine was 0.6 (0.2-0.9) mg/dL and median eGFR was 108 (72-160) mL/min/1.73 m [2]. In two patients (4.2%), HN (SFU-grade 2) was detected. Thirty-six patients (75%) underwent VCUG, revealing high-grade VUR (stage IV-V) in 8 patients (17%) and low-grade VUR (stage I-III) in 7 patients (15%). Continence was associated with a higher need for VCUG (p = 0.02) and a higher presence of VUR (p = 0.03). DISCUSSION: Renal function in EEC patients and non-EEC patients is comparable when age matched. Only 6% had low-grade HN which was asymptomatic. 17% had high-grade VUR, which is little compared to literature (40-70%). However, results in literature are described in patients with a 'one-stage' bladder closure, whereas some of our patients had a 'two-stage' procedure. A one-stage procedure creates higher bladder pressures resulting in higher VUR-rates. Statistical analysis has showed that continence is associated with a higher prevalence of recurrent febrile UTI's or pyelonephritis and of VUR. CONCLUSIONS: No statistically differences were found between continent and incontinent patients concerning creatinine and eGFR value (p = 0.52 and p = 0.29), nor in the prevalence of hydronephrosis (p = 0.36). However, results of this study suggest that continent patients may portend a higher risk of upper tract deterioration with recurrent febrile UTI's and pyelonephritis due to VUR. Close monitoring of the upper tract status is therefore as important as focus on continence. Large-scale prospective studies defining renal function as well as pyelonephritis rates are needed to optimize the management of the upper tracts in EEC patients.

Adolescent and Young Adult Urogenital Outcome following Childhood Hypospadias Repair: Perfection Revisited.

PURPOSE: We assessed the long-term surgical, functional urinary and sexual outcomes of adolescent and young adult men who underwent childhood hypospadias repair. MATERIALS AND METHODS: Men born with nonsyndromic hypospadias and healthy male controls aged 16-21 years old were recruited, and their surgical, urinary, sexual functional and aesthetic outcomes assessed. Good outcome was defined as a patent and orthotopic meatus without fistulas, and straight erections (<30 degree curvature) without erectile or ejaculatory problems. Statistics included regression analyses, chi-square/Fisher exact tests and Student's t/Mann-Whitney U and Kruskal-Wallis tests. RESULTS: A total of 193 patients and 50 controls participated 16.4 years (range 8.2-21.2) after initial repair. At least 1 reintervention was performed in 39.2%. The highest reintervention rate was found in those younger than 12 months at initial repair, even when excluding proximal hypospadias cases. A disturbed urinary and/or suboptimal sexual functional outcome was seen in 52.9% of cases. Suboptimal voiding was found in 22.1%, although few had relevant residual urine. More reinterventions and proximal hypospadias cases were associated with suboptimal urinary outcome, and the latter also with impaired sexual function. Poor inter-observer agreements were found between physician and patient genital appraisal. CONCLUSIONS: In 52.9% of cases, at least 1 concern was identified that required long-term followup. Hypospadias repair below 12 months was associated with more reinterventions. Adopting a restrictive attitude toward aesthetic refinement, unless on the patient's own request, could improve urinary outcomes.

How I do it: Buttonholing the preputial hood in hypospadias repair-related ventral skin shortage.

Gaining an esthetical skin coverage can be challenging in some hypospadias repair cases, especially in those with significant ventral skin paucity. A local institutional technique used to correct ventral skin paucity in hypospadias is described. It consists of a redistribution of the abundant dorsal skin and its hooded prepuce to the ventral side of the penis where skin shortage is observed. Reallocation of the skin is performed by buttonholing the dorsal skin to the ventral side. This versatile technique can be adapted to most penile surgeries where some ventral skin paucity is observed.

Individualized care for patients with intersex (differences of sex development): part 4/5.Considering the Ifs, Whens, and Whats regarding sexual-reproductive system surgery.

Issues and concerns regarding surgery of the sexual-reproductive anatomy during infancy and early childhood are discussed using four actual examples. A case of a 46, XX infant with 21 hydroxylase deficiency congenital adrenal hyperplasia (CAH) with atypical (ambiguous) genitalia is discussed regarding timing and potential harms and benefits of surgery. We present the perspective of balancing the child's rights to bodily autonomy and right to an open future versus parents' decision making authority regarding what they perceive as their child's future best interests. The second case is a newborn with complete androgen insensitivity syndrome and we discuss the harms, benefits and timing of gonadectomy. The third case examines the physical and psychological impact of penile shaft hypospadias, raising the question of whether surgery is justified to prevent what may or may not be considered a permanent disability. The fourth case involves an adult woman with classic CAH, born with a urogenital sinus and clitoromegaly, who never had genital surgery and is now requesting vaginoplasty, but not clitoral reduction. The primary message of this article, as the previous articles in this series, is to encourage patient-family centered care that individualizes treatment guided by shared decision making.

Long-term sexual outcomes in patients with exstrophy-epispadias complex.

Exstrophy-epispadias complex (EEC) is a spectrum of genitourinary malformations that ranges in severity and affects external genitalia and the lower urinary tract. The aim of this study was to determine the long-term sexual outcomes of patients with EEC. Sexual outcomes were hypothesized to be related to those of urinary ones. A retrospective database including all patients with EEC who had surgery at a tertiary referral institution from 1990 to 2019 was created. Data based on patient's charts were collected: demographics, surgeries, sexual outcomes, urinary outcomes. Fifty-eight patients with EEC had surgery at tertiary referral institution and entered our database. For this analysis of sexual outcomes, a sub-set of the whole population was selected: patients being 14 years old and older, having at least one surgery at our institution and having at least 12 months of follow-up. Applying this selection criteria to our database resulted in a series of 29 patients. High rates of sexual activity were observed in pubertal and post-pubertal men (96%) and women (75%). Seventy-nine percent of men and 67% of women reported sexual satisfaction; 63% of men reported normal ejaculation. To achieve these rates, 96% of men required surgery (84% penoplasty, 52% phalloplasty), and 25% of women required introitoplasty. Fertility was achieved in 67% of men and 100% of women. Assisted reproductive technology was needed in one man. Continence rates were high (diurnal continence in 83% and nocturnal continence in 93%). However, 76% required multiple continence procedures. Men and women with EEC can have good long-term sexual and urinary outcomes, but this may require multiple surgeries. Good sexual outcomes seem to be related to good urinary and continence outcome.

OnabotulinumtoxinA for the treatment of neurogenic detrusor overactivity in children.

AIMS: This study evaluated whether one (or more) of three doses of onabotulinumtoxinA were safe and effective to treat neurogenic detrusor overactivity (NDO) in children. METHODS: This was a 48-week prospective, multicenter, randomized, double-blind study in children (aged 5-17 years) with NDO and urinary incontinence (UI) receiving one onabotulinumtoxinA treatment (50, 100, or 200 U; not to exceed 6 U/kg). Primary endpoint: change from baseline in daytime UI episodes. Secondary endpoints: change from baseline in urine volume at first morning catheterization, urodynamic measures, and positive response on the treatment benefit scale. Safety was also assessed. RESULTS: There was a similar reduction in urinary incontinence from baseline to Week 6 for all doses (-1.3 episodes/day). Most patients reported positive responses on the treatment benefit scale (75.0%-80.5%). From baseline to Week 6, increases were observed in urine volume at first morning clean intermittent catheterization (50 U, 21.9 ml; 100 U, 34.9 ml; 200 U, 87.5 ml; p = 0.0055, 200 U vs. 50 U) and in maximum cystometric capacity (range 48.6-63.6 ml) and decreases in maximum detrusor pressure during the storage phase (50 U, -12.9; 100 U, -20.1; 200 U, -27.3 cmH2 O; p = 0.0157, 200 U vs. 50 U). The proportion of patients experiencing involuntary detrusor contractions dropped from baseline (50 U, 94.4%; 100 U, 88.1%; 200 U, 92.6%) to Week 6 (50 U, 61.8%; 100 U, 44.7%; 200 U, 46.4%). Safety was similar across doses; urinary tract infection was most frequent. CONCLUSIONS: OnabotulinumtoxinA was well tolerated and effective for the treatment of NDO in children; 200 U showed greater efficacy in reducing bladder pressure and increasing bladder capacity.

Surgical repair of urethral complications after metoidioplasty for genital gender affirming surgery.

Metoidioplasty as form of genital gender affirming surgery (GGAS) is frequently complicated by urethral fistulas and/or strictures. Knowledge on how to treat these complications is scarce. This series aims to describe the techniques used to treat urethral fistulas and strictures and will report the outcomes of these techniques. For this purpose, a retrospective analysis was done of a database containing 74 transmen who underwent metoidioplasty and retrieved 26 patients who underwent surgical treatment for a urethral complication. Four patients suffered both a stricture and fistula after metoidioplasty. Fistula repair was done by 3-layer closure in 18 patients and failed in 7 (39%) cases after a median follow-up of 27 months. Urethroplasty was done by different techniques according to the type of stricture and failed in 4 (33%) out of 12 cases after a median follow-up of 15 months. No high-grade complications (Dindo-Clavien ≥grade 3) were reported after fistula repair or urethroplasty. This series highlights that fistulas and strictures are frequent after metoidioplasty, but can be successfully treated by respectively 3-layer closure and urethroplasty in about 2 out of 3 cases.

Urethroplasty for Failed Hypospadias Repair Related Strictures in Adults: A Retrospective Analysis With Long-term Follow-up.

OBJECTIVE: To report long-term surgical outcomes after urethroplasty for failed hypospadias repair (FHR) related strictures in adults. MATERIALS AND METHODS: A database of all adult (≥18 years) men who underwent urethroplasty since 2000 at Ghent University Hospital was created (prospective data since 2008). Patients with a follow-up <1 year or non-FHR related strictures were excluded. Postoperative complications were categorized according to Clavien-Dindo (<90 days), high-grade being ≥ grade 3. Failure was defined as stricture recurrence at the site of reconstruction requiring additional urethral manipulation. For penile strictures, outcomes were stratified per surgical technique. RESULTS: In total, 85 patients were included with a median (IQR) follow-up of 89 (57-165) months. Respectively 76, 6 and 3 patients had a penile, bulbar and penobulbar stricture. Postoperative complication rate was 27%. High-grade complications were seen in 7 of 85 (8.2%) patients and in 4 of 85 (4.7%) patients this involved fistula formation. Failure occurred in 29 of 85 (34%) patients, corresponding with a failure-free survival estimate (SD) of 82% (4.1), 73% (5.0) and 57% (6.7) after respectively 1, 5 and 10 years. For penile strictures, anastomotic repair had the highest failure rate (5/7, 71%) and a first stage Johanson procedure only had the lowest failure rate (1/10, 10%), followed by definitive perineal urethrostomy (1/4, 25%), pedicled flap urethroplasty (2/8, 25%) and free graft urethroplasty (7/23, 30%). CONCLUSION: FHR related strictures are predominantly seen in the penile urethra. Failure rate after urethroplasty for FHR related strictures increases steadily over time suggesting prolonged follow-up in these patients. For penile FHR related strictures, anastomotic repair should be discouraged.

Psychosexual Outcome, Sexual Function, and Long-Term Satisfaction of Adolescent and Young Adult Men After Childhood Hypospadias Repair.

BACKGROUND: The psychosexual outcome in adolescents and young adults (AYA) men born with hypospadias is precarious. However, the factors responsible for impaired outcome in some AYA men have been understudied. AIM: To explore the outcome after hypospadias repair in childhood of AYA men aged 16-21 years and examine their opinion and their parents' opinion about this type of surgery. METHODS: Cross-sectional assessment of 193 AYA men born with hypospadias and 50 male controls was performed. Questionnaires such as the Decision Regret Scale, Pediatric Penile Perception Score, Sexual Quality of Life-Male, International Index of Erectile Function, and a custom-made questionnaire were used. The Decision Regret Scale and a custom-made questionnaire were also completed by the participants' parents. Physical examination including Hypospadias Objective Penile Evaluation and measuring stretched penile length was performed. OUTCOMES: This study reports the psychosexual functioning (ie, social, relational, and sexual), erectile and sexual function after childhood hypospadias repair, using ad hoc measures. In addition, the opinion about hypospadias repair of patients and their parents is represented. RESULTS: The number of surgeries and satisfaction regarding penile appearance were the most important factors associated with the opinion on hypospadias repair and the psychosexual outcome. Most AYA men were more satisfied with their penile appearance than the physician. 80% of men were satisfied with having had a childhood hypospadias repair, even though they had not been able to consent to surgery themselves. Erectile and ejaculation problems were mild and seen in approximately 10% of the population. CLINICAL IMPLICATIONS: Based on our data, deferring hypospadias repair until the patient can decide himself is not warranted. However, physicians who accept a suboptimal esthetic outcome and withdraw from repeated surgery may contribute importantly to the patient's well-being, especially in proximal forms of hypospadias. STRENGTHS & LIMITATIONS: This is one of the rare studies addressing the AYA's psychosexual outcome after childhood hypospadias repair. Strengths include the combination of clinical and psychosexual data from a very large cohort of men and their parents to provide a more holistic view. By entering this study, participants might have a different comfort level regarding their sexuality or have a different body image than the overall population of young men. CONCLUSION: Uncomplicated hypospadias surgery results in equal psychosexual outcome as controls and in high satisfaction rates; multiple surgeries are a risk factor for poorer outcomes. 80% of men are satisfied with childhood hypospadias repair. Tack LJW, Springer A, Riedl S, et al. Psychosexual Outcome, Sexual Function, and Long-Term Satisfaction of Adolescent and Young Adult Men After Childhood Hypospadias Repair. J Sex Med 2020;17:1665-1675.