Long-term outcome of gender assignment in individuals with 46, XY DSD assigned female sex in multicultural society.

BACKGROUND: The decision regarding sex rearing in patients with Disorders of Sex Development (DSD) is heavily connected to the shared decision-making model within multidisciplinary team. Some of these patients might develop gender dysphoria, when they become adults. We have aimed to evaluate the long-term outcomes of patients with XY DSD who underwent female gender assignment at our center. METHODS: We have conducted a retrospective study of all 46, XY DSD patients who underwent female assignment in our institution over the last 30 years. RESULTS: we have found 25 46, XY patients who were raised as a female after birth. After excluding the Androgen insensitivity syndrome (AIS) patients we have identified 15 patients who have matched study criteria. The decision on gender rearing was made by the parents in 11(74%) and by the surgical team 2(13%) during hernia repair/inguinal exploration. In 2(13%) cases, the patients opted to continue identifying as women after learning about the pathology during adolescence. Nine (60%) out of 15 patients (age17.9 ± 4.7 years (mean ± SD)) agreed to answer questionnaires regarding sexual function and satisfaction from gender assignment. Mean follow up was 11.1 ± 8.2 years (mean ± SD). only one participant consented to respond to a questionnaire regarding sexual intercourse (homosexual). The overall FSFI score was 24 which included the scores 4, 4, 3, 4, 3, 2 in the categories desire, arousal, lubrication, orgasm, satisfaction, and pain respectively. Two patients regretted the decision of female gender assignment. The first with 5α-reductase deficiency, he made the decision for assignment himself as an adult and the other (3ß-hydroxysteroid dehydrogenase) who underwent gonadectomy during inguinal exploration as a child. The rest of the patients were satisfied with the choice of gender, 2 need psychological support on the daily basis. In the study group, relationship and cohabitation were significantly later in life compared to the general population. CONCLUSIONS: Despite the sensitivity of the subject and cultural differences, most patients (78%) were satisfied with the decision to undergo female gender assignment. Over the years, patients require meticulous follow-up in order to consider additional interventions, and mental support if it is necessary. The two cases of later regret highlight the importance of proper education of patients, their families and medical providers upon decision on gender assignment.

Our experience with management of congenital urological pathologies in adulthood: What pediatric urologists should know and adult urologists adopt in pediatric practice experience.

Purpose: To summarize our experience in the management of congenital anomalies in the kidney and urinary tract (CAKUT) in adults. Materials and methods: We conducted a retrospective chart review of all adult patients who underwent primary surgical intervention for CAKUT between 1998 and 2021. Results: The study included 102 patients with a median age of 25 (interquartile range, 23-36.5). Of these, 85 (83.3%) patients reported normal prenatal ultrasound, and the remaining 17 (16.7%) patients were diagnosed with antenatal hydronephrosis. These patients were followed-up conservatively postnatally and were discharged from follow-up because of the absence of indications for surgical intervention or because they decided to leave medical care. All studied adult patients presented with the following pathologies: 67 ureteropelvic junction obstructions, 14 ectopic ureters, 9 ureteroceles, and 6 primary obstructive megaureters, and the remaining 6 patients were diagnosed with vesicoureteral reflux. Forty-three percent of the patients had poorly functioning moieties associated with ectopic ureters or ureteroceles. Notably, 67% of patients underwent pyeloplasty, 9% underwent endoscopic puncture of ureterocele, 3% underwent ureteral reimplantation, 6% underwent endoscopic correction of reflux, 7% underwent partial nephrectomy of non-functioning moiety, and the remaining 9% underwent robotic-assisted laparoscopic ureteroureterostomy. The median follow-up period after surgery was 33 months (interquartile range, 12-54). Post-operative complications occurred in 5 patients (Clavien-Dindo 1-2). Conclusions: Patients with CAKUT present clinical symptoms later in life. Parents of patients diagnosed during fetal screening and treated conservatively should be aware of this possibility, and children should be appropriately counseled when they enter adolescence. Similar surgical skills and operative techniques used in the pediatric population may be applied to adults.

Visual guidelines and tutoring in pediatric urological surgery.

Background: The aim of this prospective randomized study was to evaluate the impact of visual guidelines (picture book) and parents tutoring on pediatric urological surgery on parent's stress and anxiety, the number of postoperative contacts, and complications. Materials and methods: Following institutional ethical committee approval, a special picture book reflecting different stages of the convalescent period following multiple types of pediatric urological surgery was developed. Parents were randomly divided into 2 groups in which 33 parents in Group 1 received the picture book in addition to routine instructions prior to the surgery and 31 in Group 2 received only routine postoperative instructions. The parents were asked to answer a questionnaire (Amsterdam Preoperative Anxiety and Information Scale) regarding the level of anxiety before surgery and immediately after surgery in the recovery room. The number of postoperative parent's calls, nonplaned emergency room visits, and complications were recorded. Results: No statistically significant difference in perioperative parental anxiety was found (p = 0.88). The visual tutoring group had a significantly lower rate of emergency room admissions (6.6% vs. 18.6%, p = 0.0433), however parents from this group made a higher number of postoperative calls (9.9% vs. 3.1%, p = 0.38). Two (6.6%) from the tutoring group expressed their desire to omit visual counseling in future surgical preparation and 4 (13.2%) did not have an opinion. Overall satisfaction with regards to the preoperative counseling and information and the number of postoperative complications was similar in both groups. Conclusions: Visual tutoring does not add any value to parental anxiety but seems helpful in reducing postoperative emergency room visits. Some parents preferred to exclude visual information from future preoperative counseling.

Long term results of non-refluxing ureteral reimplantation in the pediatric population.

PURPOSE: To evaluate the long-term results of UR and to determine the difference between patients with VUR and OMU in terms of re-obstruction rate, complications associated with pregnancy, and de novo reflux. METHODS: Two-site retrospective study with 69 patients (36 females and 33 males) with a mean age of 5 ± 3.4 years. Fifty-nine (85.5%) underwent UR due to VUR and 10 (14.5%) due to OMU. Mean length of surgery and hospitalization was 90 ± 29.2 min and 9 ± 2.4 days, respectively. RESULTS: Eight (13.5%) patients with VUR suffered from febrile UTI with a mean of 2.1 ± 1.3 events. In the OMU group, 1 (10%) patient suffered from febrile UTI. None of the patients showed recurrence, obstruction or de novo VUR. Two patients (20%) with OMU suffered from CKD. In the VUR group, 3 (5.1%) patients suffered from CKD. Three women suffered from UTIs during pregnancy. Mean follow-up was 17.5 ± 4.6 years. CONCLUSIONS: Successful UR is associated with a decreased rate of febrile UTI in patients with VUR. Patients with OMU maintained and improved renal function in the long term. None demonstrated technical failures in the long term. Patients who presented with bilateral VUR are more prone to developing major complications.

Medical clowns versus sedation for paediatric urinary catheter insertion-A randomised pilot study.

AIM: To investigate the potential benefits of medical clowns (MCs) in reducing anxiety and pain during paediatric urinary catheter insertion. METHODS: In this prospective pilot study, 51 children who required urinary catheter insertion were randomised into two groups; the first group (n = 29) underwent the procedure in the presence of a MCs, and the second control group (n = 22) underwent the procedure using sedation. Pain and anxiety levels as well as procedural duration were recorded. All procedures were filmed and evaluated for anxiety level by a paediatric psychiatrist. General satisfaction questionnaires were filled by the medical team and parents in the clown group. RESULTS: There were no differences in pain scores or anxiety levels between the two groups. Procedure duration and total hospital stay were significantly shorter in the MC group (p < 0.001). One hundred percent of the parents and the medical team were in favour of incorporating MCs during urinary catheter insertion. CONCLUSION: MCs are as good as sedation in lowering anxiety and pain levels in children undergoing urinary catheter insertion. In addition, MCs reduce the duration of the procedure and elicit a high degree of overall satisfaction from the parents and medical team.

Surgical Treatment of Meatal Stenosis: Lessons Learned from the Pediatric Urology Practice.

OBJECTIVE: To evaluate the indications for surgery and outcome of meatotomy and meatoplasty in a big cohort of children. METHODS: We retrospectively evaluated 2211 children who underwent either meatotomy or meatoplasty between 2009 and 2020 utilizing the same operative setup. For continuous data, we performed t-test and Mann-Whitney U test. For categorical data, chi-squared test or Fisher's exact test was used. A binary logistic regression model was used to compare the indications for surgery between the groups. RESULTS: Of 2211 children, 1906 underwent meatotomy and 305 meatoplasty. The mean age was 5 years (range: 1m-15y) and there was no age difference between the groups. There was no difference between duration of surgery, recovery time, or complication rates between the groups. Deviated stream was the most frequent indication for treatment 1477 (67%). On Univariate logistic regression children with the following indications: dysuria, meatitis, and urinary retention had increased odds toward meatoplasty, while the indication of deviated stream had increased odds toward meatotomy. On Multivariate Logistic regression only deviated stream (Odd-ratios 1.47, P: .005) and urinary retention (Odds-ratio 4.5, P: .027) remained significant for meatotomy and meatoplasty respectively. Eleven (0.58%) children who underwent meatotomy developed recurrent Urethral Meatal Stenosis and underwent another surgery. Sixty-nine children (3.6%) after meatotomy required manual opening of the meatus on the first post-operative visit. None of the children, who underwent meatoplasty needed reoperation. CONCLUSION: Meatotomy had a higher reoperation rate and frequently required manual meatal spreading in early postoperative period. We believe meatoplasty is a preferable surgery for pediatric UMS.

Is Ultrasound-Guided Single-Shot Quadratus Lumborum Block a Viable Alternative to a Caudal Block in Pediatric Urological Surgery?

OBJECTIVE: To review our experience with quadratus lumborum block (QLB) in pediatric urology. MATERIALS AND METHODS: This mixed prospective-retrospective study included 41 patients who received QLB following induction of general anesthesia. Data collected included: the duration of block induction, surgery, hospitalization, postoperative pain score, and the use of rescue analgesia. The results were compared with a matched cohort of patients who received caudal block (CB) during similar surgeries from our retrospectively acquired data registry. RESULTS: There was no difference between the type and length of surgery, weight, sex, and age of the patients between the two groups. The duration of block induction was significantly shorter in the CB group compared with the QLB group (35.6 ± 14.6 vs. 239 ± 33.4 seconds [p < 0.0001]). There was no difference between the groups in pain scores at 1, 4, and 24 hours postoperatively, in the time to first rescue analgesia, or in the postoperative opioid requirements. However, the QLB group required more rescue analgesia compared with CB group (p = 0.016). Finally, no differences were found in the use of rescue analgesics at home, pain record behavior, and overall satisfaction. CONCLUSION: Our data show that QLB might serve as a viable alternative to CB in pediatric urological surgery.

Endoscopic Correction of Reflux Utilizing Polyacrylate Polyalcohol Bulking Copolymer (Vantris) as a Tissue Augmenting Substance: Lessons Learned Over the 10 Years of Experience.

Objectives: To prove the hypothesis that modifying the endoscopic correction of vesicoureteral reflux (VUR) technique (STING procedure) and sharpening its contraindications, lead to increased success rate and decline in the complication rate. Materials and Methods: All patients that underwent endoscopic correction of VUR utilizing Vantris were divided into two groups according to procedure date; before 2015 and 2015-2019. Indication for treatment included persistent high-grade VUR or breakthrough infections. Contraindications included voiding dysfunction, active infection and since 2015 suspicion for obstructive/refluxing ureterovesical junction (UVJ) presented by "beak" sign on voiding cystourethrography (VCUG). Follow-up regiment included ultrasound and VCUG at predetermined intervals. Results: The first group included 215 (158 girls and 57 boys) children with mean age of 4.8 ± 2.8 years who underwent endoscopic correction between 2009 and 2015 and the second group included 42 children (28 girls and 14 boys) with mean age of 3.9 ± 2.1 years who underwent surgery between 2015 and 2019. In the first group, VUR was unilateral in 74 patients and bilateral in 132 comprising 338 renal refluxing units. In the second group, VUR was unilateral in 14 patients and bilateral in 30 comprising 74 RRUs. In the first group reflux was corrected in 317 (94.9%) RRUs after a single injection, after the second in 7 (2.1%) RRUs. In seven (2.1%) RRUs, reflux downgraded to Grade I-II. Three RRUs (0.9%) failed endoscopic correction and required ureteral reimplantation. Nine (2.7%) RRUs developed UVJ obstruction. In the second group reflux was corrected in 61 (82.4%) RRUs after a single injection, after the second in 12 (16.2%) RRUs. In one (1.4%) RRU, endoscopic correction failed and required ureteral reimplantation. None of the patients developed UVJ obstruction. Reflux correction has led to the significant decrease of febrile urinary tract infection (UTI) in both groups. Conclusions: Our data indicate that endoscopic injection utilizing Vantris is safe and long durable procedure. Although utilizing the proper technique and contraindication criteria, the rates of post procedural VUJ obstruction is null.

Does Renal Function Remain Stable after Puberty in Children who underwent Ureteral Reimplantation due to Ureterovesical Junction Obstruction?

INTRODUCTION: Ureteric reimplantation due to ureterovesical junction (UVJ) obstruction enjoys high success in the short term. However, renal function after reimplantation must accommodate the numerous changes in the pediatric urinary tract that occur along with child development that may theoretically cause an occult loss of renal function. The purpose of this study was to evaluate whether improved renal function after ureter reimplantation for antenatal diagnosed UVJ obstruction remains stable after puberty. MATERIALS AND METHODS: Twenty-one children who underwent open reimplantation using Politano-Leadbetter technique were followed until they completed puberty. Mean age at surgery was 14.3 months (range: 3-60 months). Five (23.8%) of 21 children had right hydronephrosis, 13 (61.9%) had left hydronephrosis, and 3 (14.3%) had bilateral hydronephrosis. The Society for Fetal Urology (SFU) level of the hydronephrosis was 3 (47.6%) in 10 children and 4 (52.4%) in remaining 11. Fourteen (66.6%) patients had poor renal function upon surgery and the remaining seven (33.4%) patients had moderate renal function. The mean renal function upon operation was 28 ± 4.3 (mean ± standard deviation [SD]). RESULTS: Reimplantation led to the increase in the RRF in the short-term period from 28 ± 4.3% prior to the surgery to 36.4 ± 5% (p < 0.001) in all patients and remains stable 35 ± 5% after puberty in all the reviewed patients. CONCLUSION: Our data demonstrate for the first time that successful ureteral reimplantation following antenatal diagnosis of UVJ obstruction is associated with an improvement in renal function, not only during short- and midterm follow-up but also allows preserving the renal function throughout the puberty period.

Learning curve of robotic-assisted laparoscopic pyeloplasty (RALP) in children: how to reach a level of excellence?

It has been suggested that up to 40 cases of RALP are required to reach the operative results equal to open surgery. We have hypothesized that previous experience in open and laparoscopic surgeries might shorten the learning curve of robotic surgery. We have retrospectively evaluated the data of all children who underwent pyeloplasty in our institute by a single surgeon since 2003. The children were divided into three groups: open pyeloplasty (OP) of 72 children, laparoscopic pyeloplasty (LAP) of 22, and RALP of 33 patients subsequently. The data included: demographics, duration of surgery, length of stay, success of surgery, and complication rate according to the Clavien-Dindo score. The groups were ordered chronologically by the operation date and each group was divided into two different phases: early and late. A comparison was made between the data of the early and the late phase. There was no difference in the demographic data between the groups; however, the patients who underwent laparoscopic surgery were significantly older compared with the patients from the other groups. The median duration of surgery in the RALP group was significantly shorter than the OP group (65 min vs 72.5 min P < 0.01), while the first RALP case was already shorter than the median duration of surgery in OP group. There was no significant decrease in the duration of surgery of the RALP group over the study period, though there was a significant trend of decreasing operative time in the OP and LAP groups. There was no difference in the length of stay in the early vs late phases in the RALP group. There was no difference in the complication and success rate between the RALP and OP group, as well as the early and late phases of the RALP group. Our data show that previous experience in OP and LAP surgery may contribute to a shorter learning curve of robotic surgery required for the surgeon to achieve a similar outcome to that of OP.

Glans injury during ritual circumcision.

BACKGROUND: Circumcision is one of the most performed surgical procedure in the world, often performed for nontherapeutic reasons. Depending on cultural and social backgrounds, the procedure may be performed by various sources; from trained health care practitioners to laypeople, giving rise to different frequencies and types of complications. Glans injury during ritual circumcision is a rare yet serious complication due to its associated morbidity and long-term consequences. OBJECTIVE: In this study we describe the experience of two medical centers with the management and follow up of glans injury after ritual circumcision. METHODS: We have retrospectively reviewed the medical files of all pediatric patients who presented to the ER with glans injury, following ritual circumcision, over a 17-year period from two medical centers. RESULTS: A total of 8 patients were identified, who underwent ritual circumcision between 7 and 9 days of age. On presentation, 1 infant had complete glans amputation, the others presented with partial amputation of the glans. 6 of the 8 patients had a simultaneous urethral injury. All patients underwent surgical correction for their injury; Patients with isolated glans injury, underwent primary glans anastomosis. Of the 6 cases with simultaneous urethral injury: 3 underwent end-to-end urethral anastomosis following anastomosis of the amputated glans, one patient with complete glans amputation underwent a similar procedure, with initial end-to-end urethral anastomosis followed by glans anastomosis to the corpora cavernosa and 2 were managed by urethrostomy together with anastomosis of the amputated glans tissue to the remaining glans. Both patients with urethral injury, in whom end to end urethral anastomosis has not been performed, were found to have hypospadias on follow up. The rest had good functional and cosmetic results. DISCUSSION: There is no single method for managing glans injury following circumcision. Some authors describe healing by secondary intention with delayed formal repair, while others advocate for primary anastomosis of the amputated glans, together with distal urethra-urethrostomy, in cases with simultaneous urethral injury. In this study, both patients without urethral anastomosis, developed secondary hypospadias due to meatal regression and required additional surgery, while the others showed good results. The limitations of our study are its retrospective nature and the small number of cases, preventing us to come to a definitive conclusion regarding the best way to treat such a rare injury. CONCLUSION: Prompt surgical correction by glanular replantation with urethro-urethrostomy, in case of concomitant urethral injury, give good cosmetic and functional results.

Sexual function and voiding status following one stage feminizing genitoplasty.

BACKGROUND: The data on long-term outcome of feminizing genitoplasty (FG) with regard to the sexual function and voiding status is limited and nearly always retrospective, reviewing only small numbers of patients. Furthermore, most articles only looked at the success as an adequately open vagina with a feminized appearance. Success should include normal erotic sensation, lubrication, sexual satisfaction, orgasm, and intercourse without discomfort. These latter factors are just beginning to be studied OBJECTIVE: To investigate sexual function and voiding status in women who underwent vaginal reconstruction in childhood. MATERIAL AND METHODS: We retrospectively reviewed medical files of patients who underwent FG at our center from 1988 to 2000. In addition, patients were asked to complete the following standardized questionnaires: Female Genital Self-Image Scale (FGSIS), dysfunctional voiding and incontinence symptoms score (DVISS), and Female Sexual Function Questionnaire (FSFI). RESULTS: patients underwent one-stage FG; of those, 36 with median age of 21 years (range 18-30 years) who completed the puberty period were included into this study. Of the participants, 15 (41.6%) lived in a committed partnership, and 12 (80%) had sexual intercourse on a regular basis. Total FSFI was 22.2 + 3.1 (mean + SE). Domain scores were 3.9, 4.3, 4.5, 4.3, 4.9, and 4.7 for desire, arousal, lubrication, orgasm, satisfaction, and pain, respectively (Summary Figure). None of the patients reported on daytime incontinence or enuresis. Female genital self-image was found to be positive in all patients and related positively to women's sexual function. DISCUSSION: The general rate of the sexual activity of our studied population was similar to that published in the literature 80% in our study vs 60-87% published in the literature. The overall FSFI was 22, showing some evidence of the female sexual dysfunction. However, only one patient regarding pain during vaginal penetration demonstrated mild dyspareunia. At the same time, we have not found any desire, arousal, lubrication, orgasm, satisfaction problems. Furthermore none of our patients complained on lower urinary tract symptoms during questioning, while three girls with congenital adrenal hyperplasia presented during follow-up with dribbling incontinence, which resolved with introitoplasty and creation of the wide vaginal opening. CONCLUSIONS: Our data demonstrate that one -tage FG in childhood is a long-term effective surgical procedure that does not cause voiding dysfunction. If a secondary intervention is required prior to the sexual life, simple introitoplasty usually solves the problem. The FSFI score showed overall acceptable sexual function, whereas mild dyspareunia was a major complain.

Pediatric robotic-assisted laparoscopic pyeloplasty (RALP): does weight matter?

PURPOSE: RALP is rapidly becoming the new gold standard treatment for UPJO in children, who suffer from uretero-pelvic obstruction (UPJO). However, presently there is a lack of data regarding the outcomes of RALP in young infants and smaller children. This study aims to compare the outcomes of RALP in children weighing less than 10 kg and matched with an analogous cohort who underwent open pyeloplasty (OP). METHODS: We prospectively compared patients who underwent RALP to a matched cohort of patients who underwent OP from our retrospectively acquired data registry. Comparative outcomes included: Demographics, success rate, complications, and length of hospital stay, postoperative pain score and failure rate. Failure was defined as the need for a secondary intervention for UPJO, or worsening hydronephrosis during follow-up. RESULTS: A total of 15 patients with a median age of 8 months (range 5-11 months) and median weight 7 kg (range 5.6-9.8 kg) underwent RALP between 2016 and 2018, a matched cohort of 15 children who underwent OP similar in terms of age, weight, gender and affected side between 2014 and 2016. All children had prenatal diagnosis of hydronephrosis and underwent surgery utilizing combined general and regional (Caudal MO) anesthesia. Intrinsic obstruction was present in 13 of RALP group (86.7%) and in 14 in OP group (93.3%). Mean operative time was 67.8 + 13.4 min in RALP group, while 66.5 + 9.5 min in OP group. (p = 0.76) All but two patients in RALP group had stent inserted and required subsequent anesthesia for stent removal, while all OP children had a Salle Pyeloplasty stent inserted during the procedure and underwent removal in an ambulatory setting without the need for anesthesia. There were no failures recorded in the RALP group, while one patient in OP required a secondary intervention. Mean hospital stay was 1 day (1-2 days) for RALP and 2 days (2-3 days) for OP. There was no difference in FLACC Pain Scale in both groups. Clavien-Dindo grade I-II complications occurred in one patient from each group. Two patients from RALP underwent subsequent ureteral reimplantation due to accompanying uretero-vescical junction obstruction. CONCLUSIONS: Our data suggest that RALP can be performed safely in pediatric patients weighing less than 10 kg. with similar outcomes when compared to patients undergoing an open procedure for the same pathology.

Voiding patterns of adult patients who underwent hypospadias repair in childhood.

OBJECTIVE: This study aimed at evaluating the voiding patterns of adult patients who underwent hypospadias repair in childhood. METHOD: Following IRB approval 103 (22.7%) of 449 adult patients who underwent hypospadias repair between 1978 and 1993 responded to the following questionnaires: International Prostate Symptom Score (I-PSS) and Short Form 12 questionnaire (SF-12). Uroflowmetry (UF) was performed for all patients. The patients were divided into three groups according to the primary meatus localization. Group I had 63 patients (61.5%) treated for glanular hypospadias, group II had 19 patients (18.4%) treated for distal hypospadias, and group III comprised the remaining 21 patients (20.4%) treated for proximal hypospadias. RESULTS: The mean ± SD I-PSS score for all patients who responded to the questionnaire was 2.3 ± 2.4, and UF was 21.1 ± 4.3 mL/s. The patients from groups I and III had fewer urinary symptoms compared with those of the group II: 1.3 ± 1.5, 5.5 ± 2.4, and 1.6 ± 1.4, respectively (p < 0.0001). With regards to UF, the patients from the groups I and III did better compared with those from the group II: 22.1 ± 4.1 mL/s, 18.91 ± 4.2 mL/s, and 20.11 ± 3.42 mL/s, respectively (p = 0.021) (Figure). The UF was better in patients with normal vs. abnormal IPSS (p = 0.0064). The physical component summary was 49.8 ± 10.3, 51.1 ± 3.6, and 46.4 ± 0.3 in groups I, II, and III, respectively. The mental summary component was 42.64 ± 4.1, 42.2 ± 2.4, and 39.89 ± 2.9 in groups I, II, and III, respectively. CONCLUSIONS: Most of the adult patients who underwent hypospadias repair in childhood had normal or mild voiding disturbance, with no effects on their physical or mental status.