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.

Adopting ultrasound guided PCNL in nephrolithiasis management.

INTRODUCTION: This study aimed to evaluate the learning curve associated with the adoption of US guided PCNL and demonstrate that it can be carried out safely with results comparable to those obtained using standard PCNL. METHODS: Prospective study with 65 patients who underwent PCNL between 2019 and 2020. all procedures were performed in supine position and an initial attempt to gain access to the kidney using US was made. RESULTS: Mean procedure duration was 69.5 ± 27.8 min. Fluoroscopy was used with a mean dose of 276.68 ± 560.71 (cGycm3) and mean fluoroscopy time 40.25 ± 77.69 (s). Throughout the study there was a steady decrease in the use of fluoroscopy and amount of radiation to gain access to the kidney to only 25% at the study end. 76.5% of the patients were stone free at follow-up. Complication rate was 9.2%. CONCLUSIONS: Fluoroless US guided PCNL is safe, feasible and reproducible procedure.

Ultrasound-guided percutaneous nephrolithotomy (PCNL) success rates in patients with elevated body mass index: a comparative study.

Percutaneous nephrolithotomy (PCNL) is considered gold standard treatment of renal stones larger than 20 mm. Several studies have shown that ultrasound guidance during this procedure is more effective and safer than fluoroscopy. A higher body mass index (BMI) can make ultrasound-guided renal access more difficult and unsuccessful. We present a prospective analysis and comparison of ultrasound-guided PCNL in patients with normal and increased body mass index. We performed a prospective comparison of patients who underwent ultrasound-guided PCNL to remove renal stones by a single surgeon between 2020 and 2022. Patients with BMIs greater than 30 (mean 33.87-obese) were compared to those with BMIs less than 30 (mean 25.69-non-obese). Demographic, perioperative, and follow-up data were collected, analyzed, and included in this study. Total of 98 consecutive patients, with 49 patients in each group were analyzed. No statistically significant differences were observed in terms of stone volume (P = 0.085), stone density (P = 0.5590), location of renal access (P = 0.108), surgery duration (P = 0.38), blood loss (P = 0.54), or laboratory changes after surgery (P = 0.60). 87.76% of obese patients were stone free per CT scan at follow-up, compared to 73.47% of normal-weight patients (P = 0.1238). According to Clavien-Dindo classification, six patients in the non-obese group experienced grade II (10%) and grade III (2%) complications, as opposed to six patients in the obese group with grade I (2%), grade II (6%), and grade III (2%) complications. There was no significant correlation between body mass index and the success or safety of ultrasound-guided PCNL. Although more challenging, a higher BMI should not be an impediment to performing this approach. This method is safe, with no increased incidence of postoperative complications or compromise in stone-free status postoperatively and can diminish or avoid both patient's and medical team's exposure to ionizing radiation.

The effect of ureteropelvic junction obstruction and pyeloplasty on somatic growth during infancy.

Background: Evidence regarding the impact of perinatal ureteropelvic junction obstruction (UPJO) and surgical correction during infancy, on somatic growth are scarce. Understanding these impacts could help advise parents and aid in treatment decision making. Objectives: To assess the impact of unilateral UPJO and surgical correction on somatic growth in infants diagnosed antenatally and treated during infancy. Design: A retrospective bi-institutional analysis of somatic growth in patients under 2 years who underwent dismembered pyeloplasty for the treatment of UPJO was conducted. Methods: We evaluated patients who were diagnosed with unilateral hydronephrosis during pre-natal ultrasound screening for detection of fetal anomalies between May 2015 and October 2020. The height and weight of patients who were diagnosed with UPJO were recorded at the age of 1 month, time of surgery, and 6 months after surgery. Standard deviation scores (SDSs) for height and weight were calculated and compared. Results: Forty-eight patients under the age of 2 years were included in the analysis. Median age and weight at pyeloplasty were 6.9 months and 7.5 kg. At 1 month, the median SDS for weight in the entire cohort was -0.30 [interquartile range (IQR): -1.0 to 0.63] and the median SDS for height was -0.26 (IQR: -1.08 to 0.52). In 22.9% of patients (11/48), weight and height were below -1 age-appropriate standard deviations, and 6.3% (3/48) were below -2 standard deviations, suggesting growth restriction. When comparing SDS for the entire cohort, there was no significant difference corelated to measurement time or effect of surgery. In the growth restricted cohort, we found a significant improvement in linear growth for height, which was demonstrated between birth and surgery as well as after surgery. Conclusion: Infants with unilateral UPJO diagnosed antenatally as a single anomaly may be at an increased risk of somatic growth restriction in comparison with the general population. In children with growth restriction at time of birth, height seems to improve regardless of surgical treatment. Pyeloplasty during infancy does not seem to negatively affect somatic growth. These findings can be used to counsel parents regarding the potential effects of UPJO and pyeloplasty.

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.

Sprematic vein abscess after varicocele embolization - A case presentation.

Testicular varicocele is a dilatation of the pampiniform plexus. It is a common clinical problem affecting 15% of adolescents and adults that may be associated with pain and discomfort, and can affect fertility. Varicocele treatment is associated with a significant improvement in sperm parameters, and pregnancy rate. For endovascular treatment Fibered embolization coils are commonly utilized. Complications associated with coil insertion are rare, but can have serious consequences. Here, we presenting the first reported case of a retroperitoneal abscess following testicular vein coil embolization. The patient underwent successful laparoscopic removal the infected coil followed by full recovery.

Robotic versus laparoscopic ipsilateral uretero-ureterostomy for upper urinary tract duplications in the pediatric population: A multi-institutional review of outcomes and complications.

OBJECTIVE: To review and compare robotic ipsilateral uretero-ureterostomy (RIUU) and laparoscopic ipsilateral uretero-ureterostomy (LIUU) in terms of safety, efficacy, and outcomes. MATERIALS AND METHODS: A retrospective chart review, including all pediatric patients who underwent RIUU and LIUU at 5 different medical centers, between 2015 and 2019, was performed. Patient's demographics, perioperative data, surgical techniques, complications, and results were compared. RESULTS: The study included 66 pediatric patients, 22 RIUU and 44 LIUU. Median age at surgery was 12 month (IQR 7-52) and median weight was 12 kg (IQR 9-16). Upper to lower IUU was performed in 55 cases and lower to upper IUU in 11 cases. Median operative times for RIUU and LIUU were 90 min (IQR 75-97.5) and 112.5 min (IQR 81.5-121.25), respectively (p = 0.034). Clavien-Dindo grade 3 postoperative complications occurred in two LIUU patients. One patient underwent an ancillary procedure with laparoscopic distal ureteral stump removal. All patients had resolution of symptoms and improvement in hydronephrosis or a non-obstructed curve on MAG3 diuretic renal scan. CONCLUSION: RIUU and LIUU are both safe and effective minimally invasive approaches for duplex upper urinary tract anomalies in the pediatric population. RIUU demonstrated shorter operating times .

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.

Outcomes of Patients Turned Down for Percutaneous Mitral Valve Repair.

BACKGROUND: Percutaneous mitral valve repair is an alternative treatment for high-risk patients with symptomatic mitral regurgitation (MR). Proper patient selection is crucial to ensure that patients will benefit from the procedure while avoiding futile and potentially harmful medical interventions. OBJECTIVES: To assess the reasons for and outcomes of patients who were declined MitraClip (Abbott Vascular) implantation and compare them with patients who underwent the procedure at our medical center. METHODS: We screened 182 patients for percutaneous mitral valve repair with the MitraClip device. Of these, 84 were referred for MitraClip implantation and 75 underwent the procedure. RESULTS: Procedural success was achieved in 64 patients (85%) and was associated with superior survival at 30 months (73%) compared with implanted patients who did not achieve procedural success (41%; P=.02). Ninety-eight patients were turned down for the procedure due to anatomical incompatibility (72%), lack of indication ("too well") (16%), and clinical incompatibility ("too sick") (12%). Among turned down patients, those who were deemed too well had the highest survival rate (85%) at 30 months, patients with anatomical incompatibility had intermediate survival rates (63%), and patients deemed "too sick" had a dismal survival rate of only 25% (P<.01). In fact, the patients who were too well had outcomes that were equivalent to patients who underwent successful MitraClip implantation. CONCLUSIONS: We identified a number of reasons for not performing MitraClip implantation that impact patient survival. The best outcomes were seen in patients who underwent successful MitraClip implantation and in patients who were deemed too well.