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In Manchester, feminising genitoplasty is offered to children with 46XX Congenital Adrenal Hyperplasia (CAH) when there is a single perineal opening and/or enlarged clitoris. Our aims are to describe the anatomical reconstructive technique and present long-term outcomes. Our hypothesis is that 'the common channel (CC) length and distance to the vagina from perineal skin is mostly due to virilisation and hypertrophy of perineal tissue over the almost normally positioned vaginal introitus (V-I) in relation to the perineal body (PB)'. METHOD AND RESULTS: This is a retrospective notes review of all consecutive 46XX CAH operations from 1976 to December 2021. 99 patients, who had feminising genitoplasty and being followed-up, were included. 15 patients who were lost to follow up were excluded. Median age at surgery was 15 months. In 91, midline division of the labia majora, spongiosum, bulbo-spongiosus muscle (BSM) and CC down to PB was performed. This was sufficient to expose the V-I at the same level or within 5 mm depth of PB in 88. In 78 V-I was adequate taking 10/12fr dilator (Type 1). In 10, CC resembled a male urethra and V-I was narrow (Type 2), requiring widening by 5-10 mm incision at 6 o'clock position. Dartos of labia majora was attached to BSM to reduce the distance to V-I from perineal skin and the gap was lined with inner foreskin to create a vestibule. Out of 70 who were post-pubertal, 75% (53/70) had adequate calibre vaginal openings. 5 had introitoplasty and 2 had dilatation under anaesthesia. 10 needed self dilators only. 29 patients, of one of the three surgeons, had measurements of clitoris, CC, urethra and vagina. A hymen was found in 86% (25/29). There was significant strong, inverse correlation between the CC length and the urethral length (r = -0.708, p < 0.001, n = 27) but not between CC and vaginal lengths. After adjusting for age, the urethral length of Type 2 patients was 3.825 mm shorter than those of Type 1 (p = 0.017). CONCLUSION: Our data show that 'high' confluence is mostly due to virilisation of genitalia; and the anatomical technique of reversing the fusion of the urethral folds, spongiosum and bulbo-spongiosus muscle could be performed with all degrees of virilisation with success in early childhood with no need of local flaps or mobilisation of the urethro-vaginal complex. About 10% require surgery to treat narrowing of vaginal opening post puberty., Competing Interests: Conflicts of interest There is no conflict of interest., (Copyright © 2022 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Objective: To analyse the outcomes of retroperitoneoscopic upper and lower moiety hemi-nephroureterectomy (HNU) and to assess the different variables that may have an impact on outcome; remnant moiety damage, morbidity and the need for secondary surgery., Methods: Prospectively recorded data of retroperitoneoscopic HNU's performed by a single surgeon from 2005 to 2018 were analysed. Patients were split into 2 groups according to moiety affected (UMHNU and LMHNU). Clinical presentation, underlying pathology, remnant moiety DRF on renal scintigraphy, and need for further surgery were recorded. Detailed operation notes were studied regards to renal vasculature, degree of dilatation, inflammatory changes and operative difficulties encountered. Renal loss was defined as remnant moiety DRF <10% post-operatively. Change in DRF was assessed regards to the moiety, pathology and age at surgery (<1 year, 1-2 years and ≥2 years). UMHNU group was further sub-divided into 3 subgroups: ureteroceles, ectopic ureters and 'other' pathology. Statistical analysis was performed using Fishers Exact test; findings were considered statistically significant at p < 0.05., Results: 78 operations met the inclusion criteria on 75 patients (3 bilateral). There were no conversions to open, and 67% were performed as day-case procedures (53/78 patients). In 91.2% (71/78) patients the procedure was definitive in resolving pathology and symptoms. 7 patients needed further procedures after HNU, 5 for ureterocele/ureteric stump. Overall, there was remnant moiety renal loss in 5.1% (4/78) patients, all with UM surgery (3 ectopic ureters and 1 ureterocele). All 4 operations were recorded prospectively as 'difficult operations' due to grossly dilated UM ureter/pelvis measuring >2 cm in diameter. 2 patients had a thinned out lower moiety (LM) sitting on top of the UM renal pelvis like a pancake with all vessels stretched over this dilated pelvis/ureter causing difficulty in accurate identification. There was intra-operative concern about some damage to LM vessels in 3 patients. Age <1year was also related to increased renal loss (2/8 patients <1 year, 1/25 patients 1-2 years, 1/45 patients ≥2 years of age P = 0.005)., Conclusion: Retroperitoneoscopic LMHNU is a safe and definitive procedure with rapid recovery and minimal scarring. UMHNU has higher rates of remnant moiety loss due to more complex renal pathology, but remains a safe, successful operation on the majority of patients. Renal damage was also related to age <1year (p = 0.005) and re-operation risk after UMHNU correlated to the presence of ureterocele (p = 0.003)., (Copyright © 2021 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Background: The epidemiology and risk factors for paediatric urolithiasis (UL) in developed countries are evolving, with increasing rates of metabolic stone-formers. In the United Kingdom (UK), only a single London cohort has been studied in the past three decades. Regional disease patterns across the UK remain unknown. UL has been associated with an increased risk of chronic kidney disease in adults, but long-term paediatric outcomes remain poorly understood., Methods: We assessed the epidemiology of paediatric UL by defining the demographics, incidence, aetiology, recurrence rates and outcomes at tertiary nephro-urology centres in Greater Manchester. Health records of 177 consecutive paediatric urology referrals (0-18 years) for UL between 2002 and 2015 were retrospectively included for observational analysis. Metabolic screening was performed in 105 children., Results: The incidence of paediatric urology referrals for UL was 1.77/100,000 person-years, increasing annually by 13.6%. Mean patient age was 8.2 years and the male:female ratio was 1.3:1. The upper urinary tract was involved in more than 90% of cases and 45% of children presented with multiple calculi. Metabolic abnormalities were identified in 52% of children screened: hypercalciuria (64%), hyperoxaluria (18%) and cystinuria (11%) were the most common. Significant family history was predictive of metabolic abnormalities (OR 4.9:1, 95% CI 1.4-17.0). 30% of children had infective stones, which were more common in children less than 6 years. Ultrasound was found to be 90% sensitive for UL detection. Dimercaptosuccinic acid (DMSA) imaging identified renal scarring in 33 of the 55 children tested (60%). Recurrence was observed in 18% of children over mean 6.4 year follow-up and was more common in metabolic stone-formers. Spontaneous passage was observed in 83% of stones ≤ 5 mm, 69% between 5 and 10 mm and only one calculi > 10 mm., Conclusions: The incidence of paediatric urology referrals for UL is rising in North West England. Metabolic abnormalities were detected in half of the children tested, justifying comprehensive screening. Recurrence is common following paediatric UL, reinforcing the need for stone prevention strategies. The majority of calculi less than 10 mm were found to pass spontaneously with supportive measures, which favours an initial observation period over surgical intervention for small calculi. UL was associated with renal scarring in the analysed population and therefore, children with UL require long-term assessment of blood pressure and proteinuria., (Copyright © 2020 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Aim: To assess the medium term outcome of hypospadias repair and preputioplasty with regard to foreskin retractility, and whether this could be predicted by assessing retractility at the end of the operation., Materials and Methods: The data were obtained from a prospectively maintained database regarding patients who had hypospadias repair with preputioplasty between January 2003 and June 2010. The foreskin retractility at the end of the operation was documented in the operation notes, and the latest foreskin status was obtained from clinical notes or by contacting parents., Results: The total number was 207, and 170 (82.1%) had distal hypospadias. At the latest follow up at a median of 2 years and 3 months after the date of operation (range 13 months to 4 years 7 months), 159 boys (76.8%) had a retractile foreskin, 27 (13%) were under observation for a tight foreskin, and 8 (3.9%) had undergone circumcision due to tightness. The foreskin was retractile at the end of the operation in 154 (73.3%) patients. Of these, 130 (84%) had a retractile foreskin at the later follow-up clinic. Of the 49 patients who had a non-retractile foreskin at the end of the operation, 29 went on to have a retractile foreskin, while 20 had a tight foreskin. Fisher's exact test showed that foreskin retractility at the end of the operation has a significant relation to foreskin retractility in the medium term, and that patients with distal hypospadias have a greater probability of achieving a retractile foreskin., Conclusion: A primarily retractile foreskin can be achieved in over 75% with less than 4% requiring a circumcision due to tightness. Distal location of hypospadias and retractility at the end of operation predicted success., (Crown Copyright © 2013. Published by Elsevier Ltd. All rights reserved.)

Introduction: The lateral approach is more widely used in retroperitoneoscopic (RP) heminephroureterectomy (HNU) due to familiar orientation and ease of conversion. The prone approach however gives early and easy access to the vascular pedicle. The main reason for not adopting a prone approach more widely is the perceived difficulty in lower ureteric access. We present a series of 30 HNUs where the prone approach was utilized extremely effectively with no conversions and few complications., Methods: Thirty consecutive HNUs performed over a 4-year period (2004-2007) using a prone RP approach were included in the study. Prospectively recorded data and notes were reviewed., Results: Upper HNU was done in 17 and lower HNU in 13 patients. Mean age was 3.2 years (range 0.9-13.3). There were no transfusions or conversions. Follow-up ultrasound showed healthy remnant moieties in all. Residual ureteric stump was seen in six patients but only one was symptomatic requiring a further procedure., Conclusion: With the prone approach the anatomy can be demonstrated quickly and effectively. Dissection can be done easily without the need for kidney retraction as gravity aids demonstration of the renal vascular pedicle. There is a low risk of complications arising from the residual ureteric stump. We recommend that this approach be considered for RP HNU in all patients, irrespective of age., (Copyright © 2009 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Introduction: Two children presented with malignant hypertension due to complex reno-vascular malformations. The 7-min video shows the use of prone retroperitoneoscopy in both. PATIENT 1: A 6-year-old girl presented with convulsions and malignant hypertension. Captopril DMSA was suggestive of right renal artery stenosis. On formal angiography, the kidney was perfused by what appeared to be an arterio-venous malformation. The renal artery was hypoplastic and there were pulsatile vessels along the ureter due to the gonadal periureteric and gonadal inferior capsular collateral supply to the kidney. The kidney could be devascularized easily by controlling these. PATIENT 2: A 14-month-old boy presented with 6-month history of poor appetite, weight loss and irritability. Formal angiography showed acute obstruction of the main upper pole branch. The vessels to the upper pole could be clearly seen and controlled at surgery for partial right nephrectomy. He recovered with no urine leak or bleeding., Conclusion: Gravity kept the renal vessels under stretch allowing excellent two-hand dissection and the space was adequate for suture closure of a calyx in Case 2. The prone approach provides excellent exposure of the renal blood vessels, aorta and vena cava, and seems to be the most suitable for complex renal vascular malformations., (Copyright © 2009 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Objectives: Laparoscopic renal surgery has only recently become popular in the UK and, still, only a handful of pediatric urologists are offering the laparoscopic approach to their patients; therefore, there are only limited places available for training in this discipline. In this institution, which is a major tertiary referral center for pediatric urology having four consultants, one urologist specializes in laparoscopic renal surgery and about 50 laparoscopic ablative renal surgical procedures are performed annually. The aim of this study was to determine the effectiveness of the training provided, for three trainees, in light of the complexity of the ablative procedures performed., Patients and Methods: Patients who required laparoscopic ablative renal surgery from June 2003 to May 2004 were identified from the prospectively maintained database and data were analyzed. During this period of 12 months, 49 operations have been performed laparoscopically. There were three trainees, A, B and C, who joined the mentor, at different times, for a continuous period of 6, 12 and 6 weeks, respectively. The training was one to one and, at an appropriate level of expertise, the trainees were allowed to perform operations independently; they continued to do so after returning to their units., Results: There were 22 males and 27 females, age ranging from 8 months to 16 years (mean 3.6 years). There were 25 nephrectomies, 23 via the prone retroperitoneal (PRP) approach and two via the transperitoneal (TP) approach; the mean time taken was 70 min. There were 12 nephroureterectomies, six via the PRP, three via the TP, and the other three via the lateral retroperitoneal (LRP) approach; the mean time taken was 100 min. There were 10 heminephroureterectomies (six upper pole and four lower pole), six via the PRP, three via the TP and one via the LRP approach; the mean time taken was 160 min. Of two bilateral nephrectomies one was via the PRP and one the TP approach, and the mean time taken was 170 min. The mean time to discharge for nephrectomy was 7 h, and for nephroureterectomy, heminephroureterectomy and bilateral nephrectomy 21, 23 and 43 h, respectively. Trainee A had been in pediatric surgery for 4 years and during a period of 6 weeks he assisted in five operations, but did not get the opportunity to perform independently. Trainee B had been in pediatric surgery for 13 years and during a period of 12 weeks he assisted in nine operations and performed three. Trainee C had been in pediatric surgery for 20 years and during a period of 6 weeks he assisted in five operations and performed one. Since returning to their overseas units, trainee B and trainee C have successfully performed two nephrectomies each., Conclusion: Retroperitoneoscopic renal surgery is within the grasp of any experienced urologist or urology trainee. With intensive exposure and one-to-one mentoring, 6-12 weeks would be sufficient to achieve adequate competence and confidence to perform a prone retroperitoneoscopic nephrectomy.