45 years' experience with early childhood anatomical technique of feminising genitoplasty for 46 XX Congenital Adrenal Hyperplasia -observations of vaginal introital anatomy and its relationship to the perineal body.

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.

The place of retroperitoneoscopic hemi-nephroureterectomy for duplex kidney in children; risk of damage to the remnant moiety and strategies to reduce the risk.

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).

No stone unturned: The epidemiology and outcomes of paediatric urolithiasis in Manchester, United Kingdom.

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.

Novel approach to vaginal calculus in a girl with urogenital sinus anomaly.

Isolated urogenital sinus can cause distended bladder and/or vagina and may present with an abdominal mass and sepsis during infancy. Older children may present with recurrent urinary tract infections and hematocolpos. We describe a 3-year-old girl with recurrent urinary tract infections thought to be secondary to vesicoureteric reflux. On further investigation, an isolated urogenital sinus anomaly with a calculus inside one of the hemivaginae was noted. She was managed expectantly with a plan to intervene at puberty. At puberty, during removal of the stone, the hemivaginal introitus was found to be stenotic. Gradually increasing sizes of Amplatz type graduated renal dilators were introduced from the introitus of the urogenital sinus into the hemivaginal stone until a size 22F Amplatz sheath could be passed easily. Size 10F cystoscope was passed through this channel, and the stone was fragmented using electrohydraulic lithotripsy. At a later date, she underwent staged anterior sagittal transvulval mobilization of the urogenital sinus.

Novel use of an osmotic self-inflating tissue expander for hypospadias revision surgery.

We describe the successful use of an osmotic self-inflating tissue expander for complex pediatric hypospadias repair. A 5-year-old boy, who had undergone previous hypospadias repair and subsequent fistula repair, presented with significant rotation of the penile shaft, with extensive scar tissue but insufficient native penile shaft skin for redo surgery. A 15 × 6 mm Osmed self-inflating cylindrical expander was used to allow excision of scar tissue and cover with expanded native skin. An excellent cosmetic and functional outcome was achieved at clinic review, 18 months postoperatively. The use of self-inflating tissue expanders offers a novel technique to achieve adequate native skin in the management of complex pediatric hypospadias.

Foreskin retractility following hypospadias repair with preputioplasty--medium term outcomes.

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.

Resolution of primary non-refluxing megaureter: an observational study.

AIM: A prospective review of conservative management of primary non-refluxing megaureters (PM) was performed to determine the time taken for resolution (TTR) and complications. MATERIAL AND METHODS: Patient details were obtained from a prospectively maintained database from January 1, 2003, to December 31, 2011. The clinical features of USS and MAG3 findings were analyzed. All had annual USS and MAG 3 scans two yearly (and whenever necessary). RESULTS: Fifty ureteric units (UU) in forty-four patients (six bilateral) were studied. There were thirty-three (75%) males. In the unilateral PM, 22/38 were left-sided. Children were classified according to the lower ureteric diameter (UD) into two groups: Group A (Gp A) UD < 10 mm (n=25, 26 UU), and Group B (Gp B) UD ≥ 10 mm (n=19, 24 UU). Antenatal diagnosis was achieved in 21 (84%) UU in Gp A and 11 UU (58%) in Gp B. In Grp A, the median presenting UD was 6 (range 4-9) mm, and 76% resolved completely over a median duration of 60 (18-204)months. In Grp B, the median UD was 15 (10-27)mm, and 17% resolved completely over a median duration of 102 (42-210) months. Two developed ureteric calculi (removed ureteroscopically). Three with complications (obstructive drainage pattern in MAG 3 with decreasing function and debilitating infections) underwent ureteric tapering and reimplantation. An obstructed megaureter resolved after endoscopic dilatation. Another underwent temporary ureterostomy on developing hypertension. CONCLUSION: The exclusively conservative management of PM seems highly successful within Group A (i.e. UD <10 mm). Complications (stones, decreasing renal function) were more common with higher UD. TTR seems to take over five years in both groups.

Persistent Müllerian duct syndrome: lessons learned from managing a series of eight patients over a 10-year period and review of literature regarding malignant risk from the Müllerian remnants.

UNLABELLED: Study Type--Therapy (case series) Level of Evidence 4. What's known on the subject? and What does the study add? Approximately 200 cases of persistent Müllerian duct syndrome have been reported over the last 50 years and most authors suggest leaving the Müllerian remnant in situ because of the difficulty in dissection and the presumed absence of risk of malignancy. However, with increasing reports of Müllerian malignancies emerging, we report our 10-year experience of managing patients with persistent Müllerian duct syndrome, with removal of müllerian remnants. This case series shows that there is an increased risk of Müllerian malignancy that was previously unknown. With the laparoscopic approach, orchidopexy with simultaneous removal of Müllerian remnants could be accomplished with minimal surgical trauma and the benefit of no malignancy risk in the future. This is a new technique that has not been previously performed. Considering the current evidence of malignancy in the Müllerian remnant, surgeons would need to discuss with families about removal of remnants or long-term monitoring. OBJECTIVES: • To describe the presentation and management of eight patients with persistent Müllerian duct syndrome (PMDS) seen over a 10-year period at our tertiary centre. • To review the literature of Müllerian malignancies reported in PMDS. PATIENTS AND METHODS: • The hospital records of eight patients with PMDS were retrospectively reviewed between 2001 and 2011. • Extensive PubMed searches for PMDS and Müllerian malignancy were performed. RESULTS: • Eleven cases with PMDS and malignancy of the Müllerian remnants were identified. • From our own PMDS series: five males presented with bilateral undescended testes and three had unilateral undescended testis. • We found that the Müllerian remnants could be removed by laparoscopy and three patients had simultaneous laparoscopic removal of the Müllerian structures and laparoscopic orchidopexy. CONCLUSIONS: • The principle aim of orchidopexy with simultaneous laparoscopic removal of the Müllerian structures can be accomplished with minimal surgical trauma and the benefit of no malignancy risk in the future. • Surgeons should consider excision of the Müllerian remnants where possible.

Outcome study of upper pole heminephroureterectomy in children.

OBJECTIVE: We assessed the role and long-term outcome of upper pole heminephroureterectomy in the treatments of non-functioning upper renal moieties in children with duplex kidneys. METHODS: In a period of 10 years, forty-three patients (male: female ratio 6:37) underwent upper pole heminephroureterectomy; a total of 25 patients were diagnosed prenatally. Imaging modalities included renal and bladder ultrasound in all 43 patients, static 99 m technetium dimercaptosuccinic acid (DMSA) in 21 patients, micturating cystourethrogram in 28 patients, MAG-3 in 13, cystoscopy in 30, IVU in 31 and DTPA, retrograde pyelography, antegrade pyelography in 1 patient. The main presentation was with urinary tract infections. Operation was performed through a combination of anterolateral loin incision plus right or left inguinal incision in 34 patients and high flank incision in 9 patients, for various abnormalities. One patient underwent also initial puncture of a ureterocele. RESULTS: Five patients (12%) had immediate post-operative complications. Thirteen patients (30%) had late post-operative complications. Long-term follow-up revealed no complication in 35 (81%) patients and one (2%) patient had one episode of UTI and intermittent abdominal pain. Three patients (7%) had long-term incontinence. Four patients (9%) had a second procedure performed. CONCLUSION: Based on our experience, it seems that upper pole heminephroureterectomy is the treatment of choice in cases of obstructed upper segments of duplicated kidneys, when the affected segment contributes to less than 10% of the overall renal function.

Prone retroperitoneoscopy in treating complex renal vascular malformations.

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.

Prone retroperitoneoscopic approach for heminephrectomy: specific advantages relating to access to vascular pedicle.

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.

Outcome study of lower pole heminephrectomy in children.

OBJECTIVE: This study assessed the role and long-term outcome of lower pole heminephrectomy in the treatments of non-functioning lower renal moieties in children with duplex kidneys. MATERIAL AND METHODS: In a period of 10 years 31 lower pole heminephrectomies were performed in 30 patients with duplex systems. Eight patients were diagnosed prenatally, 24 patients (80%) presented with urinary tract infection and three (10%) with vomiting and failure to thrive, and five patients also had other symptoms. The indication for lower pole heminephroureterectomy was reflux nephropathy in non-functioning lower pole in 28 patients (93%), pelviureteric junction obstruction in one patient (3%) and cystic dysplasia in one patient (3%). The surgical technique used was a combination of anterolateral loin incision plus right or left inguinal incision in 28 patients (90%). RESULTS: The operative course was uneventful, no blood transfusion were required, and no damage to the upper pole moiety or upper pole ureter was observed. Four patients (13%) had immediate postoperative complications and three (10%) had late postoperative complications. Long-term follow-up revealed no complications in 27 patients (90%) and three patients (10%) with increased urinary frequency. CONCLUSIONS: The results indicate that lower pole heminephrectomy is the treatment of choice in cases of non-functioning dilated lower segments of duplicated kidneys. The use of two incisions in this procedure is effective in preventing the need for further surgical treatment secondary to complications of the stump.

Financial analysis of laparoscopic versus open nephrectomy in the pediatric age group.

The authors compared the cost of laparoscopic nephrectomy to open nephrectomy in the pediatric age group. One hundred seventeen consecutive laparoscopic nephrectomies performed by a surgeon with extensive experience with this approach between April 2003 and August 2006 were included. A control group of 24 consecutive open nephrectomies performed by urologists who do not use the laparoscopic approach were also included. Inclusion criteria for surgery were a poor or nonfunctioning kidney related to severe obstructive or refluxing nephropathy and a multicystic dysplastic kidney. The length of operation, length of stay, and disposable equipment used were recorded and the different approaches were compared statistically with an unpaired t test. The mean (standard deviation [SD]) duration of the procedure was 79 minutes (32) in the laparoscopic group and 85 minutes (35) in the control group (P = 0.41). The mean (SD) cost of the disposable instruments used during the operation was pounds sterling274 (160) in the laparoscopic group and pounds sterling20 (5) in the control group (P = 0.0001). The mean (SD) hospital stay was 1 night (0.43) with a mean (SD) cost of pounds sterling677 (291) in the laparoscopic group, and 3 nights (2) with a mean (SD) cost of pounds sterling2031 (1354) in the control group (P = 0.0001). The mean (SD) total cost of the procedure was pounds sterling951 (451) for the laparoscopic group and pounds sterling2051 (1359) for the open one (P = 0.0001). In our experience, the laparoscopic approach in the pediatric age group is 54% less expensive than the open approach.

Constant urinary dribbling due to an ectopic ureter and delays in diagnosis.

We present 2 patients who had a solitary kidney visualized on ultrasound imaging and experienced constant urinary dribbling. Further imaging modalities defined the anatomy and visualized the contralateral kidney and ectopic ureter causing the dribbling. Both patients went on to have curative surgical procedures. These cases reinforce the point that in a toilet-trained girl with normal voiding at normal intervals experiencing constant urinary dribbling, the diagnosis of an ectopic ureter must be considered early and referral made for appropriate imaging. Because of the abnormal anatomy, ultrasound imaging is particularly limited in such cases, but if used, one should take account of antenatal and postnatal scans. Referral is advocated, based on history alone, for appropriate imaging and investigation.

Acute renal papillary necrosis with complete bilateral ureteral obstruction in a child.

A 9-year-old girl presented with apparent meningococcal septicemia and developed acute renal failure after 48 hours of treatment with antibiotics and analgesics. Early ultrasound scanning demonstrated mild bilateral hydronephrosis and hydroureter. Intravenous urography showed slow contrast uptake with delay nephrogram and no contrast entering the bladder. Repeat ultrasonography revealed bilateral papillary irregularity and echogenic debris in the distal ureters. Bilateral double-J stents were inserted cystoscopically, resulting in prompt polyuria and a return of normal renal function. Although rare, recognition of sloughed papilla in papillary necrosis causing ureteral obstruction can lead to early management with no long-term sequelae.