Genitoplasty in newborn females with adrenogenital syndrome : focus on the reconstruction technique and its outcomes

The adrenogenital syndrome is an autosomal recessive disorder in which an enzyme defect in the steroid pathway leads to excessive prenatal exposure of androgens. In the female fetus, masculinization of the external genitalia is observed. Surgery aims for functional and aesthetical reconstruction. Many techniques have been described. A video of our modified pull-through reconstruction technique is hereby presented. A retrospective descriptive database was created with patients who underwent genitoplasty for a CAH-associated genital condition. A video demonstrating the reconstructive technique was recorded while operating on a 9-month-old girl. Prior to surgery a cystoscopy is performed to evaluate the length of the urogenital sinus. Surgery starts with creating a reversed U-flap, after which the urogenital sinus is mobilized. The corpora cavernosa are released and the neurovascular bundle is isolated. To create vaginal space the urogenital sinus is subsequently separated. The vaginal introitus is anchored to the perineal skin flap. Labia minora are created by splitting the preputial skin. Finally excessive skin tissue is resected. Twenty-two female patients underwent reconstructive surgery for the adrenogenital syndrome in a tertiary referral centre over 16 years. Median age at surgery was 3 months (0-190). Median follow-up was 36 months (0-108) after surgery. A good functional and aesthetical outcome was observed. The modified pull-through technique, illustrated by this video, provided satisfactory results with a low complication rate. Follow-up until adulthood is needed to evaluate long-term outcomes

Pediatric challenges in robot-assisted kidney transplantation

Kidney transplantation is universally recognized as the gold standard treatment in patients with End-stage Kidney Disease (ESKD, or according to the latest nomenclature, CKD stage 5). Robot-assisted kidney transplantation (RAKT) is gradually becoming preferred technique in adults, even if applied in very few centra, with potentially improved clinical outcomes compared with open kidney transplantation. To date, only very few RAKT procedures in children have been described. Kidney transplant recipient patients, being immunocompromised, might be at increased risk for perioperative surgical complications, which creates additional challenges in management. Applying techniques of minimally invasive surgery may contribute to the improvement of clinical outcomes for the pediatric transplant patients population and help mitigate the morbidity of KT. However, many challenges remain ahead. Minimally invasive surgery has been consistently shown to produce improved clinical outcomes as compared to open surgery equivalents. Robot-assisted laparoscopic surgery (RALS) has been able to overcome many restrictions of classical laparoscopy, particularly in complex and demanding surgical procedures. Despite the presence of these improvements, many challenges lie ahead in the surgical and technical-material realms, in addition to anesthetic and economic considerations. RALS in children poses additional challenges to both the surgical and anesthesiology team, due to specific characteristics such as a small abdominal cavity and a reduced circulating blood volume. Cost-effectiveness, esthetic and functional wound outcomes, minimal age and weight to undergo RALS and effect of RAKT on graft function are discussed. Although data on RAKT in children is scarce, it is a safe and feasible procedure and results in excellent graft function. It should only be performed by a RAKT team experienced in both RALS and transplantation surgery, fully supported by a pediatric nephrology and anesthesiology team. Further research is necessary to better determine the value of the robotic approach as compared to the laparoscopic and open approach. Cost-effectiveness will remain an important subject of debate and is in need of further evaluation as well

Health effects of environmental exposure to cadmium: objectives, design and organization of the cadmibel study: a cross-sectional morbidity study carried out in Belgium from 1985 to 1989

Cadmium is a cumulative environmental pollutant. For the general population mainly exposed by the oral route and through tobacco smoke inhalation, the kidney is the critical organ. Belgium is the principal producer of cadmium in Europe, and certain areas of the country are polluted by cadmium mainly because of past emissions from nonferrous industries. Preliminary studies carried out in one polluted area have suggested that environmental pollution might lead to an increased uptake of cadmium by the human body and possibly to health effects. Thus, a large-scale morbidity study has been initiated to assess the validity of this hypothesis. The present paper describes the protocol of this study. Its main objectives are to determine to what extent environmental exposure to cadmium resulting from industrial emissions may lead to accumulation of the metal in the human organism; to establish whether or not environmental exposure may induce renal changes and/or influence blood pressure; and to assess the acceptable internal dose of cadmium for the general population. The study design takes advantage of the fact that biological indicators of exposure, body burden, and early nephrotoxic effects of cadmium are available, which increase the likelihood of detecting a cause-effect relationship

Determinants of serum zinc in a random population sample of four Belgian towns with different degrees of environmental exposure to cadmium

This report investigated the distribution of serum zinc and the factors determining serum zinc concentration in a large random population sample. The 1977 participants (959 men and 1018 women), 20–80 years old, constituted a stratified random sample of the population of four Belgian districts, representing two areas with low and two with high environmental exposure to cadmium. For each exposure level, a rural and an urban area were selected. The serum concentration of zinc, frequently used as an index for zinc status in human subjects, was higher in men (13.1 μmole/L, range 6.5–23.0 μmole/L) than in women (12.6 μmole/L, range 6.3–23.2 μmole/L). In men, 20% of the variance of serum zinc was explained by age (linear and squared term, R = 0.29), diurnal variation (r = 0.29), and total cholesterol (r = 0.16). After adjustment for these covariates, a negative relationship was observed between serum zinc and both blood (r = −0.10) and urinary cadmium (r = −0.14). In women, 11% of the variance could be explained by age (linear and squared term, R = 0.15), diurnal variation in serum zinc (r = 0.27), creatinine clearance (r = −0.11), log γ-glutamyltranspeptidase (r = 0.08), cholesterol (r = 0.07), contraceptive pill intake (r = −0.07), and log serum ferritin (r = 0.06). Before and after adjustment for significant covariates, serum zinc was, on average, lowest in the two districts where the body burden of cadmium, as assessed by urinary cadmium excretion, was highest. These results were not altered when subjects exposed to heavy metals at work were excluded from analysis

The SAGASF-M Questionnaire, at home or in the office? A validation study

Introduction To date, information on self-perceived genital anatomy and sensitivity are not a part of long-term follow-up urogenital reconstructive procedures. This could be useful to specialists and patients involved in genital reconstructive surgery. This is especially the case for transgender patients in whom the invasive genital gender reaffirming surgery has a high risk of complications and is susceptible for redo procedures. Until shortly, no normative large sample of data on this subject existed in cisgender men. Nor is there a validated instrument available that can be used in a Dutch speaking population. Aims This study aimed to validate the "Self-Assessment of Genital Anatomy and Sexual Functioning" (SAGASF-M) questionnaire within a Dutch speaking cisgender male population. We evaluated its’ psychometric properties in this different population, environment and healthcare system. Methods 808 cisgender men with no prior history of genital surgery other than circumcision completed an internet-based survey of whom 24 were invited two weeks later to fill in the questionnaire again together with a urological examination to correlate the answers to clinical practice. Psychometric criteria included are content validity, internal consistency and reproducibility. Given this population has not undergone any surgical procedures other than circumcision, floor and ceiling effects and responsiveness could not be evaluated. Main outcome measures The SAGASF-M enables men to rate the sexual pleasure, discomfort, intensity of orgasm and effort required for achieving orgasm in eleven different detailed areas of the male genitalia using a 5-point Likert scale. Furthermore, the questionnaire assesses differences in genital anatomy. For the purpose of this study, biological men were asked to rate the sensory function in these same different areas when pointed out by the urologist. Results Median age of the participants was 39 and around 20% of them was circumcised. The vast majority of patients described an anatomic location of the meatus and average penile, scrotal and testicular size. When these anatomical variations were compared with the urological examination, we discovered an overall 97.2% match. Significant discrimination between different locations of the genital area was possible for each of the evaluated domains (sexual pleasure, orgasm intensity, orgasm effort, discomfort/pain). When these evaluations were compared with the urologist’s’ examination in the comparator group, nearly no significant differences could be noted. Except for the back of the scrotum and the perineum. This indicates that the patients are well capable of performing a correct self-examination without need of a urological examination. Conclusion The SAGASF-M questionnaire discriminates well between the different genital areas concerning erotic sensation. The ventral and lateral sides of the glans penis showed the most sensitive, which corresponds to the highest nerve density in this area. The Dutch version of the SAGASF-M questionnaire has adequate psychometric properties and is a valid instrument to assess genital anatomy and genital sensitivity as self-reported measures in cisgender men. This could be a first step in assessing the postoperative changes in genital sensitivity and sexual function transgender patients undergoing genital gender reaffirming surgery

Metoidioplasty without urethral lengthening in a transgender male patient

Introduction and objectives Metoidioplasty is a type of gender affirming surgery performed in female-to-male transgender men. Genital gender reaffirming surgery with urethral lengthening is associated with a high risk of urethral complications, such as urethral stricture and fistula. In order to decrease the risk of complications, some patients prefer not to have urethral lengthening performed. If the patient has no particular wish for voiding in a standing position, it could be a good solution to construct a primary perineostomy. The aim of our study was to report our approach of a modified metoidioplasty without urethral lengthening. This video demonstrates the different steps of our technique. Materials and Methods A 23-year-old female-to-male transgender patient underwent metoidioplasty and scrotoplasty without urethral lengthening. His past surgical history included trans-areolar mastectomy and hysterectomy with oophorectomy one year earlier. The patient had no wish to be able to void while standing and therefore, a urethral lengthening was not performed. Instead, the urethral meatus was repositioned by means of a perineostomy. As the patient wished metoidioplasty as a final surgery, maximal penile lengthening was pursued. The patient was placed in lithotomy position. Vaginectomy is performed in the standard fashion after hydro-dissection of the anterior vesico-vaginal wall. The urethral meatus is dissected away from the vestibular part of the clitoris. All other mucosal tissue underneath the clitoral corona, including the labia minora is excised. The neo-penoscrotal angle is constructed at the original lowest point of the clitoris. Scrotoplasty is performed by posterior release of the labia majora up to the point of the penoscrotal angle. The posterior edges are brought anteriorly and fixed to the midpoint of the penoscrotal angle according to the Ghent scrotoplasty technique. The urethral meatus is anchored underneath the scrotum and sutured to the skin after ventral spatulation, creating a perineostomy. The perineal defect is closed up to the point of the perineal urethrostomy. We plan second stage scrotoplasty with ventral release of the labia majora to further increase the size of the neo-scrotum and create a more anatomical position of the penis and the scrotum. Results We present our modified metoidioplasty technique in a step-by-step-video guide. The technique results in the construction of a perineostomy just below the perineal scrotal transition, an advanced neo-scrotum and a maximally lengthened neo-phallus. Conclusion Metoidioplasty as gender affirming surgery for female to male transgender patients remains a complex procedure with high risk of urethral complications. In patients who are not keen on being able to void while standing, the construction of a perineostomy be a good solution to avoid these urethral complications

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 (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 SAGASF-M questionnaire by a clinical evaluation. Main outcome measures: The SAGASF-M 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 compared to 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