A Novel Large Deletion in the EVER1 Gene in a Family With Epidermodysplasia Verruciformis From India.

ABSTRACT: Epidermodysplasia verruciformis (EV) is a rare autosomal recessive genodermatosis due to mutations in EVER1 and EVER2 genes. The genetic profile of Indian patients with EV has not been previously studied. This report describes the clinical presentation and molecular analysis of a family with EV. Using genomic DNA from two affected probands and healthy controls (two other siblings), conventional polymerase chain reaction (PCR) was conducted with novel primer sets designed to amplify the coding and splice-site regions in the genes EVER1 and EVER 2 . This revealed no amplification with a primer set for exons 16 to 18 in the EVER1 gene of both the probands. Subsequently, long-range PCR spanning the length of exon 15-20 and next-generation sequencing demonstrated a homozygous deletion of 2078 bp in the EVER1 gene ( EVER1 :c.2072_2278del). Screening the family revealed the same homozygous deletion (similar to index cases) in two other affected siblings. The parents and two asymptomatic siblings were heterozygous carriers for the deletion while one healthy sibling was negative. These results were validated with Sanger sequencing. This deletion in exons 17 and 18 of the EVER1 gene results in a frameshift, followed by a premature termination resulting in a severe phenotype. The identification and validation of this large deletion was detected using stepwise amplicon-based target enrichment and long-range PCR, respectively. In this family, this simple strategy greatly enhanced genetic counseling as well as early genetic diagnosis and screening. However, functional assays and larger studies are required to characterize and validate the genetic diversity among Indians with EV.

Isolated laparoscopic extra-vesical ureteric reimplantation (I-LEVUR) for upper moiety ectopic ureter without lower moiety vesicoureteric reflux.

PURPOSE: To evaluate the feasibility and outcomes of isolated laparoscopic extra-vesical ureteric reimplantation (I-LEVUR) for upper moiety ectopic ureter in patients with non-refluxing lower moiety ureter and avoid reimplanting normal lower moiety orifice. METHODS: Between 2013 and 2024, 12 patients (8 females, 4 males) with ectopic ureter associated with a duplex system underwent I-LEVUR. Preoperative assessment included ultrasonography, voiding cystourethrography (VCUG), and magnetic resonance urography (MRU)/retrograde pyelogram (RGP). The procedure involved laparoscopic extravesical approach dissecting only the ectopic ureter in lower most part, preserving vascularity, and performing an isolated reimplantation without manipulating lower moiety ureter. RESULTS: Twelve patients, with a median age of 2.8 years, underwent I-LEVUR. The mean operative time was 127.5 min. No intraoperative complications occurred. Postoperative follow-up (median: 78.5 months) showed no cases of ureteral obstruction or significant complications. Urinary continence improved in all patients. Two patients developed mild vesicoureteral reflux, managed conservatively, and one patient had minor urine leak which resolved spontaneously. CONCLUSION: I-LEVUR is a viable and effective alternative to traditional en bloc reimplantation for upper moiety ectopic ureter. It preserves the normal ureteric orifice, reduces surgical trauma, and offers excellent outcomes in terms of renal function and urinary continence. Further studies with larger cohorts, control group of common sheath reimplantation, randomization, robust statistical validation and longer follow-up are recommended.

How effective is nephrectomy in curing hypertension in children with unilateral poorly functioning kidney? A systematic review.

PURPOSE: Some children with hypertension (HTN) have unilateral poorly functional kidney (PFK). This provides an opportunity for the clinician to cure the HTN by removal of the PFK, thereby avoiding the problems of long-term medication. However, there is sparse data in children regarding the effect of PFK nephrectomy on curing HTN. In this review, we analysed the etiology of PFK causing HTN and the effectiveness of nephrectomy in curing HTN in children. METHODS: We searched the databases to identify papers between January 2000 to December 2020 pertaining to children with PFK and HTN who underwent nephrectomy. Outcome analyzed was the resolution of HTN following nephrectomy. Duplicate publications, review articles and incomplete articles were excluded. Meta-analysis of heterogeneity was reported with I2statistics. Forest plot was constructed to compare the pooled prevalence of HTN resolution. RESULTS: Five articles with 88 patients were included. Majority (43%) of PFK were due to the unilateral atrophic kidney with or without vesicoureteral reflux (VUR); ureteropelvic junction obstruction and multicystic dysplastic kidney together accounted for 35% of cases and renovascular pathology for 22% of cases. With a follow-up of 1.5 to 3.3 years, nephrectomy was effective to cure HTN in 65.9% (95% CI 55-75%) children. CONCLUSIONS: In children with HTN and a unilateral PFK, nephrectomy cured the HTN in two-thirds of children. Unilateral atrophic kidney due to VUR was the most common cause of PFK. An increase in the utilisation of laparoscopy was observed in recent publications, hence laparoscopic nephrectomy may be considered a first choice of treatment in these children.

Utility of ultrasound-based scoring system in post-pyeloplasty recovery.

BACKGROUND/OBJECTIVE: Differentiation of uretero-pelvic junction obstruction (UPJO) from non-obstructive dilatation (NOD) is a major challenge. The aim of this retrospective study is to determine whether pyeloplasty prediction score (PPS) could predict the need for surgery and resolution after surgery. METHODS: Among patients with antenatally diagnosed hydronephrosis, those who were stable during post-natal follow-up were considered NOD. The UPJO group were the ones who worsened and underwent pyeloplasty based on conventional indications. All patients with UPJO underwent laparoscopic dismembered pyeloplasty. PPS was determined based on three ultrasound parameters obtained retrospectively: Society of Fetal Urology (SFU) grade of hydronephrosis, transverse anteroposterior (APD), and the absolute percentage difference of ipsilateral and contralateral renal lengths. RESULTS: Among 137 patients included (R:L = 59:73; M:F 102:35), 96 were conservatively managed (NOD), while 41 patients (29%) needed pyeloplasty (UPJO). Mean PPS was 4.2 (1.2) in the NOD group and it was significantly higher at 10.8 (1.63) in the UPJO group (p = 0.001). All patients with PPS > 8 needed a pyeloplasty, while two patients with PPS of 7 needed pyeloplasty due to drop in renal function. PPS cutoff value of >8 had a sensitivity 95%, specificity 100% and a likelihood ratio of 20. Post-pyeloplasty PPS resolution was proportional to the duration of follow-up. CONCLUSIONS: A PPS cutoff value of 8 or above is associated with the presence of significant UPJO. PPS is also useful in the assessment of hydronephrosis recovery post-pyeloplasty. The limitation of PPS: it can only be applied in the presence of contralateral normal kidney.

Preoperative topical estrogen application in the management of hypospadias: a systematic review.

Local estrogen therapy has been explored as an alternative to conventional testosterone therapy in children requiring urethroplasty for hypospadias. Our objective is to evaluate if preoperative estrogen stimulation reduces post-urethroplasty complications and enhances penile dimensions. A systematic search was conducted on various databases, selecting only randomized controlled trials (RCTs) that tested estrogen on hypospadias patients under 18 years. Articles underwent sorting following PRISMA guidelines and bias risk was assessed using the JBI clinical appraisal tool for RCTs. Out of 607 screened records, 10 underwent full-text review, and 4 randomized controlled trials (RCTs) were selected for analysis. The total patient cohort across studies was 387 with 174 in the estrogen group. All studies utilized topical estrogen, but in different formulations and timings. Prudence is necessary for interpreting results due to variations in formulation, timing, and hypospadias type across studies. Limited by a small number of studies and outcome presentation non-uniformity, the review suggests no change in penile dimensions or postoperative complications with topical estrogen. Further research is needed to explore wound-healing properties of estrogen in hypospadias through animal and human studies.Registration and protocol: Registered in Prospero CRD42024502183.

Does Age Influence the Functional Recovery after Infant Pyeloplasty?

Purpose: The purpose was to study the correlation between age at surgery and functional recovery after infant pyeloplasty. Materials and Methods: All infants who underwent pyeloplasty were analyzed retrospectively in this multicenter study. Anteroposterior diameter (APD) >2 cm, split renal function (SRF) <40%, and Society of Fetal Urology (SFU) grade 3-4 were surgical indications. Based on the age at pyeloplasty, they were divided into Group 1 (1-3 months) and Group 2 (4-12 months). APD and SRF were compared before and after surgery in both groups. The fractional recoverable function (post-SRF-pre-SRF)/(50-pre-SRF) ×100 was correlated with age. Results: Fifty-one infants underwent pyeloplasty (mean age: 1.6 months - Group 1 and 7.2 months - Group 2). The mean APD decreased from 3 cm to 1.2 cm in Group 1 while 2.8 cm to 2 cm in Group 2 (P = 0.001). The mean SRF increased from 32.28% to 42.81% in Group 1 while 31%-34.18% in Group 2. SRF recovery was significantly higher in Group 1 compared to Group 2 (P = 0.001). Regression analysis showed a weak negative correlation (r = -0.2792) between age at surgery and renal function improvement. Conclusion: Functional recovery after pyeloplasty is better when done earlier (1-3 months), as this gives the growing kidney the best opportunity to recover.

Unaided Visual Inspection for Assessment of Penile Curvature in the Clinical Setting of Hypospadias Surgery: Survey of Members of Society of Pediatric Urology (India).

Purpose: To compare the accuracy of unaided visual inspection (UVI) to Software App measurement (SAM) of penile curvature (PC) during hypospadias surgery. Methods: Seven clinical pictures of PC (15°-60°) taken during hypospadias repair were shared with 300 members of the Society of Pediatric Urology (India). The respondents were asked to assess the angles by UVI and indicate their preferred correction method of that PC. For each picture, the angles of curvature estimated by UVI were compared with the objective angle measured using an app (SAM), which was considered an accurate estimation. Statistical analysis was done using software; P<0.05 was considered as statistically significant. Results: Ninety-one of 101 (90%) respondents preferred UVI to measure PC during hypospadias surgery. For 6/7 pictures, <40% of participants estimated the angle correctly by UVI (P < 0.001), with the difference in estimation being 3.6°-14.9°. For pictures with PC >30°, the error in UVI estimation was >10°, with no correlation between the accuracy of UVI estimate and surgeon experience. A significant proportion of surgeons chose the incorrect option for PC correction, which was the lowest (69%) for PC 35.8°. Conclusions: Most surgeons preferred UVI to assess PC; UVI is an erroneous technique to measure PC angle, especially in the PC range 30°-60°, where the error was >10°. Most errors were an underestimation of the PC, irrespective of surgeon experience. There was a significant error in the choice of technique for PC correction for a PC of 35°. These results strongly support the objective assessment of PC using SAM during hypospadias repair.

Microbial Colonization Pattern of Indwelling Double J Stents in Children.

Background: Indwelling double-J-stent (DJ stent) kept post-urological procedures may cause urinary tract infections (UTIs) due to polymicrobial biofilm formation and colonisation. Aims: To determine the incidence and microbiological characteristics of DJ stent related UTIs in a paediatric population. Methods and Material: Patients under the age of 18 admitted for DJ stent removal following pediatric urological procedures were enrolled into the study. Prior to surgical removal of the DJ stent, a urine sample was collected and the stent was cystoscopically removed under anesthesia. The ends of the stent were inoculated into culture media and incubated up to 48 hours. When growth was observed, an antibiogram was obtained using a panel of anti-microbial agents. Results: The study group consisted of 27 patients (M: F = 23:4) with a mean age of 4.3 years (1 month - 13 years). The commonest indication for stent placement was Pyeloplasty, 19 (70%). Stent colonisation was found in 8 out of 27 patients (29.6%; CI 12-51%) and E.Coli (33%) was the commonest organism. Polymicrobial growth was noted in 4 patients. Organisms were sensitive to Cephaerazone/ Sulbactam and Amikacin, and resistant to other Cephalosporins. All 8 culture positive patients were asymptomatic and a repeat urine culture revealed no growth. Colonisation did not lead to active UTI and post stent removal the urine became sterile. Conclusions: Microbial colonisation was noted in 30% of patients with indwelling DJ stents. Prior knowledge of culture & sensitivity pattern helps to cover the patients with appropriate antibiotic on the day of stent removal.

Comparison of Urinary Biomarkers in Diagnosis of Ureteropelvic Junction Obstruction and Differentiation from Nonobstructive Dilatation.

Background and Aims: Differentiation of nonobstructive dilatation (NOD) from ureteropelvic junction obstruction (UPJO) is a challenge in children with antenatally detected hydronephrosis. The aim of this study is to compare the utility of urinary biomarkers: carbohydrate antigen (CA 19-9), neutrophil gelatinase-associated lipocalin (NGAL), and kidney injury molecule (KIM) in diagnosing UPJO. Methods: A prospective study was conducted after obtaining ethical clearance between 2021 and 2022. Group 1 - control group (n = 30): children with normal antenatal ultrasound with no urinary symptoms. Group 2 - study group (n = 48): children with unilateral hydronephrosis: Group 2a - NOD (n = 24): children stable on ultrasound and diuretic renogram and Group 2b - UPJO (n = 24): children who worsened to Grade 4 hydronephrosis on ultrasound/worsening of differential renal function (10% drop) on renogram who underwent pyeloplasty. Urinary biomarkers NGAL, KIM-1, and CA 19-9 were measured using the enzyme-linked immune absorbent assay method. Results: The urine CA 19-9 level was 128.05 ± 4.08 U/mL in the UPJO group, and this was significantly higher (P = 0.001) than NOD, 70.29 ± 4.41, and controls, 1.91 ± 1.57. The urine NGAL level was 21.41 ± 4.44 pg/mL in UPJO, and this was significantly higher than controls, 2.669 ± 0.513, but not NOD, 24.55 ± 2.67. The urine KIM level was 817 ± 15.84 pg/mL in the UPJO group, and this was significantly higher than controls, 285 ± 8.10, but not NOD, 768.23 ± 15.12. Receiver operating characteristic analysis of CA 19-9 revealed a urine biomarker cutoff of 95 U/mL for diagnosing UPJO (sensitivity 95%; specificity 96%; and area under the curve 0.99). Conclusions: CA 19-9 is a superior marker compared to NGAL and KIM in differentiating UPJO from NOD. Further studies with larger numbers are warranted.

A Pilot Survey of Indian Stakeholders: Parents, Doctors, and Grown-Up Patients of Disorders of Sexual Differentiation on Management Decisions and Associated Gender Dysphoria.

Background and Aims: Of late, there are many legal representations from select quarters to halt all medical interventions in children with differences of sex development (DSD). In this survey on management decisions in DSD, we distil the views of Indian stakeholders: parents, physicians, and grown-up patients with DSD on their management decisions to identify decisional satisfaction or gender dysphoria. Methods: The survey domains included the patient demographics, final diagnosis, decision on the sex of rearing, surgical interventions, opinion of the stakeholders on the preferred age of sex assignment, final sex of rearing, and agreement/disagreement about sex assignment (gender dysphoria). Results: A total of 106 responses were recorded (66% parents, 34% grown-up patients aged 12-50 years). Among parents, 65/70 (95%) preferred the sex to be assigned soon after birth. All grown-up patients preferred sex to be assigned soon after birth. Regarding decisions on surgery, 74% of physicians and 75% of the grown-up patients felt parents should be allowed to decide interventions. Among Indian parents, 90% felt they should have the right to decide surgery in the best interest of their child for a safe social upbringing. Overall, gender dysphoria among Indian DSD patients was <1% (1/103, 0.97%). Conclusions: The predominant preference and opinion of major Indian stakeholders (physicians, parents, and grown-up DSD patients) support the existing approach toward DSD management, including early sex assignment and necessary medical intervention.

Unaided visual assessment of ventral curvature during hypospadias repair is inferior to objective measurement using app goniometry.

OBJECTIVE: Failure to perform artificial erection or objectively assess ventral curvature (VC) during primary hypospadias repair is an important reason for residual/ recurrent chordee. The present study compares the accuracy of unaided visual inspection (UVI) with objective VC assessment using smartphone application (app) goniometry. METHODS: All patients who underwent primary hypospadias repair between January 2021 and September 2022 were included. Assistant surgeons were asked to grade the degree of VC on UVI (after degloving and an artificial erection test) into: none, mild (<30 degree), severe(>30 degree). Lateral profile photograph was taken and angle measurement was performed on an android mobile application (Angulus). Correlation was performed with both methods of assessment. RESULTS: During this period a total of 210 patients were analyzed; VC was noted in 40/138 (29%) cases of distal and in 62/72 (86%) cases of proximal hypospadias. Erroneous visual inspection was noted in 41/210 (20%; 95% CI 14-25%) on UVI (15 erroneously marked none while 26 marked mild). Among those found to have chordee, UVI assessed 39/82 (47%) as severe while app goniometry assessed 65/97 (67%) as severe. There was significant relative risk of labelling severe chordee as a mild one by UVI: 1.4 (95%CI 1-1.8; p=0.01). CONCLUSIONS: UVI was erroneous in 20% of cases. UVI was less accurate in differentiating severe chordee from mild one. In 60% patients UVI alone could have led to erroneous VC assessment and thus wrong selection of technique. Further studies are required to validate our findings and standardize VC measurement using an app goniometry.

A Cost-effectiveness Model Comparing Urinary Biomarkers with Diuretic Renogram in Diagnosing Ureteropelvic Junction Obstruction in Children.

Background and Aims: In this cost-effectiveness model, we compared the cost-effectiveness of commonly used urinary biomarkers with conventional diuretic renogram (DR) in diagnosing ureteropelvic junction obstruction (UPJO). We hypothesized that urinary biomarkers are as effective as DR. Methods: We used incremental cost-effectiveness ratio (ICER) as a tool for our cost-effectiveness analysis model. The cost of biomarker assay and renogram were sourced from the same center while the accuracy data of DR and urinary biomarkers from the relevant pubications. Results: As the accuracy of individual biomarker increased, the ICER also got better. As a panel of biomarker was introduced, the ICER went to the negative range suggesting cost saving as well. ICER of most urine biomarkers is currently less expensive and less effective. When a biomarker panel was applied ICER became more expensive and effective. With higher samples, test running cost is likely to go down in future and thus biomarkers are likely become less expensive and more effective. Conclusions: Individual urine biomarkers are currently less expensive and less effective compared to DR in predicting UPJO. In future, biomarker panel is likely to be more cost-effective and reduce the need for invasive renogram thus reducing the radiation exposure.

Reasons Behind Patients Defaulting from Elective Pediatric Urology Procedures at a Tertiary Private Teaching Hospital in South India.

Aim: The aims of the study are to audit the default rate of the provisional date of elective surgery in pediatric urology private practice and to study the factors involved in the postponement of elective operation dates by patients. Patients and Methods: The audit involved analysis of reasons behind patients defaulting from elective pediatric urology procedures between January 2019 and December 2019 at a tertiary private teaching hospital in South India. The details were obtained from the outpatient register kept for elective booking. The details of actual procedures performed were obtained from OT records. From the defaulters, the reasons for postponement were obtained through personal/telephonic interview. Results: A total of 289 patients were given dates for elective procedures. Among this, 72 (24.9%) defaulted, leaving 217 patients who underwent elective surgery. Among those who underwent surgery, 90 (41%) were elective day case (DC) procedures, whereas 127 (59%) were inpatient (IP) procedures. The default rate was 26/116 (22.4%) for DC procedures, whereas 46/173 (26.6%) for IP procedures, with no significant difference between them (P = 0.64). Among the 72 defaulters, the reasons for cancellation were as follows: financial factors (FFs) 22 (30.6%), lack of family support 19 (26.4%), function/grievance in house 10 (13.9%), respiratory illness 14 (19.4%), and treatment at a different center 7 (9.7%). FF (insurance denial) was significantly higher (P = 0.001) in crucial IP procedures at 19/46 (41%) compared to DC procedures at 3/26 (12%). The various diagnoses denied insurance were UPJO (7), VUR (6), hypospadias (4), UDT (3), and PUV (2). Conclusions: FFs were mainly responsible for parents postponing their children's elective pediatric urology procedures in India. Universal insurance coverage for congenital anomalies might help overcome this most important cause of cancellations.

Comparing accuracy of urinary biomarkers in differentiation of ureteropelvic junction obstruction from nonobstructive dilatation in children.

Multiple urinary biomarkers have been reported in differentiation of nonobstructive dilatation (NOD) from ureteropelvic junction obstruction (UPJO). In this meta-analysis, we compared the accuracy of common urinary biomarkers applicable to UPJO. A systematic literature review of electronic databases was conducted for: (UPJO) OR (NOD) AND (urinary biomarkers) AND (children) for articles published in the last decade. PRISMA guidelines were used to exclude duplicate and erroneous articles. Meta-analysis involved risk of bias analysis, heterogeneity assessment, and comparison of sensitivity/specificity by forest plot analysis using MetaXL 5.3. Among the 264 articles analyzed, 19 articles met the inclusion criteria and reported the following: neutrophil gelatinase-associated lipocalin (NGAL), monocyte chemotactic protein-1 (MCP1), carbohydrate antigen 19-9 (CA 19-9), kidney injury molecule (KIM1), epidermal growth factor (EGF), and interferon gamma induced protein-10 (IP10). There was substantial heterogeneity among articles. There was wide variation in applied cut-offs among studies. Overall sensitivity was highest at 87% for CA 19-9 while overall specificity was highest at 76% for NGAL. Overall accuracy was highest at 78% for CA 19-9 followed by 77% for NGAL and 75% for KIM1. In this meta-analysis, the overall accuracy was highest for CA 19-9 followed by NGAL and KIM1. The small number of studies for CA 19-9 and considerable heterogeneity for all should be considered while interpreting these findings. Based on the current meta-analysis, we support a panel of biomarkers combining NGAL, KIM, and CA 19-9 for the best diagnostic accuracy of UPJO in children.

Bladder height width ratio on voiding cystourethrogram as a predictor of future valve bladder in children with posterior urethral valve.

BACKGROUND/AIMS: Prompt and early treatment of valve bladder is known to improve renal outcome in patients with PUV. We hypothesized that bladder height width ratio (HWR) measurement on voiding cystourethrogram (VCUG) could predict development of valve bladder. METHODS: All patients who underwent primary PUV ablation and evaluated later with urodynamics were included. Valve bladder was suspected when there were daytime wetting, persistent hydroureteronephrosis, and elevated detrusor pressures on urodynamics. VCUGs were analysed for HWR: maximum height/width of bladder. ROC curve analysis was performed to identify HWR that could predict development of urodynamic abnormality. RESULTS: Between 2012 and 2017, 102 patients were studied: median age at valve ablation 25 days (3-125 days); follow-up 5 years (3-7 years). The ROC curve comparing post-ablation HWR with occurrence of valve bladder showed a cut-off of 1.45 (AUC 0.93). HWR cut-off of 1.45 had a sensitivity of 89% and specificity of 84% in predicting future valve bladder. Detrusor overactivity was noticed on urodynamics in 41/55 (74%) of those with HWR > 1.45 compared 5/47 (11%) in those with HWR < 1.45 (p = 0.001). CONCLUSION: Bladder height width ratio on VCUG is a useful parameter to predict development of future valve bladder in children with PUV.

Single-stage salvage urethroplasty for failed distal hypospadias: comparison of Mathieu and grafted tubularised incised plate repairs.

AIM: Distal hypospadias repair is a common operation but some children require a complete salvage operation due to major complications of initial repair. The aim of this paper is to analyze the results of single-stage salvage distal hypospadias operations with salvage Mathieu (SM) and salvage grafted tubularised incised plate (SGTIP) techniques. PATIENTS AND METHODS: A retrospective review of 115 children from 2 centers who underwent SM or SGTIP operations for failed distal hypospadias from January 2012 to July 2021 was conducted for demographic data, success of salvage operation and complications. Children with scarred urethral plate but with healthy, mobile ventral skin underwent SM operation, while children with a supple urethral plate of width 6 mm or greater underwent SGTIP operation. Twenty-four (21%) children with pre-operative glans width < 12 mm received pre-operative intramuscular testosterone injections (2 doses, 2 mg/kg/dose). In the SM group, 22 cases with shallow, scarred glans underwent distal glans augmentation using the terminal portion of the Mathieu skin flap. Pre-operative and intra-operative parameters and post-operative results were compared between SM and SGTIP techniques. Statistical analysis was done using software; student t test and Fishers exact test were used, and p value of < 0.05 was considered significant. RESULTS: During the study period, SM and SGTIP operations were performed in 84 and 31 children, respectively. Overall, at a median follow-up of 24 (3-72) months, the salvage operation was successful in 101/115 (87.8%) patients; complications were observed in 14/115 (12.2%). The SM and SGTIP groups were comparable with respect to the number of prior failed operations, mean age, mean length of the distal urethral defect, pre-operative testosterone use and use of dorsal plication for chordee correction. SM had significantly better success (77/84, 91.6%) than SGTIP (24/31, 77.4%, p = 0.044). In the SM group, urethrocutaneous fistula (UCF) was noted in 2/84 (2.3%) while this was significantly higher (p = 0.01) at 5/31 (16.1%) in SGTIP. No patient in SM group developed meatal/urethral stenosis while this was significantly higher (p = 0.0003) at 6/31 (19.3%) in SGTIP; of these, 4 children had both UCF and meatal/urethral stenosis. A higher incidence of repair breakdown/dehiscence was seen in SM group, but the difference was not statistically significant (SM 5/84, 5.9% versus SGTIP 1/31, 3.2%, p = 0.484). In the SM group, two fistulae were successfully repaired after 6 months, while the three cases of complete dehiscence underwent two-stage oral mucosal graft (OMG) repair. In the SGTIP group, two children underwent successful repair of UCF (one along with meatoplasty), one child with stenosis improved with urethral dilatations, while four children (three with UCF and stenosis and one with complete dehiscence) were managed with two-stage OMG repair. CONCLUSIONS: Single-stage salvage distal hypospadias repair was successful in 87.8% of patients. Both SM and SGTIP demonstrated acceptable results for salvage distal hypospadias repair. SM had better success and lower complications than SGTIP. SGTIP was associated with significantly higher post-operative UCF and meatal/neourethral stenosis.

A meta-analysis comparing dorsal plication and ventral lengthening for chordee correction during primary proximal hypospadias repair.

PURPOSE: Recurrent chordee (RC) is an important complication of proximal hypospadias repair. In this meta-analysis we compared RC incidence following dorsal plication (DP) versus ventral lengthening (VL). METHODS: We searched the databases to identify all papers between 2001 and 2021 pertaining to proximal hypospadias and recurrent chordee. Duplicate publications, review articles and incomplete articles were excluded. Meta-analysis of heterogeneity was reported with I2 statistics. The pooled outcomes were compared to Chi square/Fishers exact test. RESULTS: A total of 17 articles were included covering 582 patients. The I2 statistics for prevalence of RC among different publications showed no heterogeneity for DP (I2 = 0%) and low heterogeneity for VL (I2 = 26%). RC was noticed in 31/122 (25.4%; 95% CI 18%-33%) among patients who had DP alone while it was significantly lower, 24/460 (5.3%; 95% CI 4%-8%) when VL was used (p = 0.0001). When compared to DP, all VL techniques had significantly lower incidence of RC. Among the VL techniques lowest incidence of RC was found for ventral corporotomies (4%) followed by small-intestinal- submucosa (SIS 4.2%) and tunica vaginalis flap (TVF)/free graft-TVFG (5%). Among the VL subtypes: the proportion of RC with use of TVF (4/70, 5.7%) and TVFG (3/69, 4.3%) for corporoplasty was comparable (p = 1); single-layer SIS was associated with significantly less RC (1/90, 1.1%) than 4-layer SIS (5/51, 9.8%; p = 0.02). CONCLUSION: For correction of severe ventral chordee during primary proximal hypospadias repair, dorsal plication carries a higher risk of recurrence compared to ventral lengthening procedures.

Safety and short-term efficacy of mirabegron in children with valve bladder: a pilot study.

AIMS: This pilot study aims to assess the short-term efficacy and safety of mirabegron in valve bladder, an important cause of persistent hydronephrosis after successful treatment of posterior urethral valves (PUV). METHODS: Twenty-two patients with early valve bladder (no residual PUV; persistent hydronephrosis, wetting and urodynamic evidence of detrusor overactivity) were included. Three subjective parameters: frequency, wetting episodes; patient perception of bladder condition score (PPBC) and four objective parameters: uroflow index (UI = Qave/Qmax), voided volume (VV = voided volume/ expected bladder capacity), maximum filling pressure (P det-max) and society of fetal urology (SFU) hydronephrosis grading were analysed pre- and post-3-month treatment with mirabegron (0.5-1 mg/kg/day). All patients were observed for heart rate, BP, ECG changes during therapy. RESULTS: There was significant reduction (p = 0.001) in mean frequency (pre 15; post 10), wetting episodes (pre 5; post 2) and PPBC (pre 4; post 3). There was significant improvement (p = 0.01) in mean UI (pre 0.3; post 0.5), VV (pre 0.54; post 0.72), Pdet-max (pre 42; post 25) and hydronephrosis grade (pre 3.5; post 2.2). There were no significant side effects. CONCLUSION: This pilot study establishes short-term efficacy and safety of mirabegron in valve bladder with overactivity. Further larger long-term studies are warranted.

Is Routine Excision of Dysplastic Testicular Remnants/Nubbins Associated with Nonpalpable Testis Necessary? Is Routine Fixation of Contralateral Solitary Testis Indicated? A Survey on the Prevalent Practice among Indian Pediatric Surgeons.

Background and Aims: Dysplastic nubbin also referred to as testicular regression syndrome (TRS) is found in 5% of cases of the Non palpable testis (NPT). There is no consensus on the excision of the above and fixation of the contralateral solitary testis. We aimed to survey the prevalent practice of the same among members of the Indian Association of Pediatric Surgeons (IAPS). Methods: A structured questionnaire was sent through group e-mail and social media platforms to IAPS members to identify their practices in management. Results: A total of 132 surgeons responded to the questionnaire. Excision of intra-abdominal and inguinoscrotal TRS remnants was practiced by 84% (95% confidence interval [CI] 77%-89%) and 82% (95% CI 74%-87%). Fixation of contralateral solitary testis was practiced by 62% (95% CI 53%-70%) in the above scenario. Among the respondents, 30% reported encountering torsion of solitary testis during their career and this experience was a significant factor (P = 0.01) in deciding contralateral orchidopexy. Scrotal infection/necrosis was not encountered by a majority (72%) and it was not a deterrent factor in preventing contralateral orchidopexy (P = 0.68). Conclusions: The majority of pediatric surgeons favored the removal of intra-abdominal/inguinoscrotal TRS remnants identified during laparoscopy for NPT. A majority favored sutureless fixation of the contralateral solitary testis.

Renal Primitive Neuroectodermal Tumor Mimicking Wilms' Tumor in a Young Boy: A Case Report of a Rare Entity with Review of the Literature.

Primary renal primitive neuroectodermal tumors (PNET) are an extremely rare entity. The tumor is very aggressive presenting with metastasis and carries a dismal prognosis. We describe the case of renal PNET in an 11-year-old boy with a solid cystic lesion in the right kidney with a thrombus in the inferior vena cava and lung nodules, mimicking Wilms' tumor.