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 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.

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.

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.

Preoperative three-dimensional modelling and virtual reality planning aids nephron sparing surgery in a child with bilateral Wilms tumour.

Bilateral Wilms tumour (BWT) is a surgically challenging condition. Virtual reality (VR) reconstruction aids surgeons to foresee the anatomy ahead of Nephron Sparing Surgery (NSS). Three-dimensional (3D) visualisation improves the anatomical orientation of surgeons performing NSS. We herewith report a case of BWT where VR planning and 3D printing were used to aid NSS. Conventional imaging is often found to be inadequate while assessing the tumour-organ-vascular anatomy. Advances like VR and 3D printing help surgeons plan better for complex surgeries like bilateral NSS. Next-generation extended reality tools will likely aid robotic-assisted precision NSS and improve patient outcomes.

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.

Clinical, Radiological and Histological Features and Their Association with Extranodal Extension in Buccoalveolar Complex Squamous Cell Carcinoma.

Jeyashanth RijuObjectives The study was aimed to (1) evaluate the effectiveness of clinical examination, intraoperative finding, and contrast-enhanced computed tomography (CECT) to detect extranodal extension (ENE) in buccoalveolar complex squamous cell carcinoma (BAOSCC), (2) to know various factors influencing ENE, and (3) to evaluate survival outcome in patients with ENE. Materials and Methods This was a retrospective cohort study, which included 137 patients with BAOSCC who underwent curative treatment between May 2019 and April 2021. Collaborative findings suggestive of ENE were noted during preoperative clinical examination, CECT, and intraoperatively, and their efficacy was compared with postoperative histopathology. Also, the various factors associated with ENE were evaluated and compared. Statistical Analysis Univariate and multivariate analysis of parameters was done using multiple logistic regression analysis and significant correlation was determined using chi-square test between ENE positive and negative categories. Analysis of prognosis and survival was done by Kaplan-Meier curve plotting using regression analysis and its significance was compared. Results The overall prevalence of ENE was 18.98% and that of lymph node involvement was 40.88%. CECT (73.1%) was found to be more sensitive in detecting ENE compared to intraoperative examination (46.2%) and clinical examination (34.6%).In comparison with clinical examination (91.9%) or CECT (78.38%), intraoperative examination (93.7%) showed the highest specificity in detecting ENE. Clinical nodal size ≥ 3 cm ( p ≤ 0.001), fixity ( p ≤ 0.001), and clinical number of nodes ( p ≤ 0.001) had significant association with ENE. The presence of thick nodal walls on CECT increased the probability of predicting ENE 15 times ( p = 0.180, confidence interval: 0.3-765.4). After a mean follow-up of 18 months, subjects without nodal positivity had a survival advantage over patients with positive lymph nodes (86.4% vs. 53.3%) and those with ENE (86.4% vs. 23.2%), respectively. Conclusion The results demonstrated that clinical examination can be used as an adjuvant to radiological imaging for prediction of ENE preoperatively. Clinical finding suggesting size of node ≥ 3 cm and ≥ 2 nodes are strong predictor of ENE, in addition to other known predictors. Patients with ENE had an unfavorable prognosis when compared with subjects with metastatic nodes without ENE. Presence of ENE remains one of the strongest factors predicting recurrence and thus poor prognosis.

Aspergilosis del sistema nervioso central: presentación de dos casos / Central nervous system aspergyllosis: presentation of two cases

Objective: to describe and analyze two cases of CNS aspergillosis.Description: case 1: a 76 years old female, with a medical story of DM type 2 and high blood pressure, complained of headaches and left side hearing loss since 6 months before. The MRI showed a left temporal bone lesion that enhanced with contrast, including the petrous bone cerebellar surface. Case 2: a 23 years old female with an acute hepatic failure of unknown origin, ten days after she was transplanted went into coma, requiring endotraqueal intubation. A CT scan showed a bihemispheric subcortical hipodensity and a lumbar puncture showed hiperproteinoraquia with pleocitosis. Intervention. In case 1, a biopsy performed through a retrosigmoid approach was informed as aspergillosis. She received voriconazole. In case 2, at the 12° postoperative day a brain biopsy showed aspergillosis. The patient died on day 13° after surgery of multiorganic failure and cardio-respiratory arrest. The necropsy showed a disseminated aspergillosis infection that affected lungs, liver, heart, skin, brain, colon and kidneys. Conclusion: the results obtained with the treatment in both patients were in accordance with the outcome severity of aspergillosis as was described in the reports reviewed.

Esthesioneuroblastomas with intracranial extension: proliferative potential and management

O índice proliferativo celular tumoral de 8 casos cirúrgicos de estesioneuroblastomas com extensäo intracraniana (9 com extensäo intracraniana em um total de 15, coletados de 1978 a 1992 no Hospital Nordstadt de Hannover, Alemanha) foi avaliado através do anticorpo monoclonal MIB-1, notando-se uma correlaçäo prognóstica. A maioria dos tumores revelou índice proliferativo alto (3 por cento a 42 por cento, média de 16 por cento) e a classificaçäo histopatológica de Hyam foi realizada. A ressecçäo foi radical em 100 por cento dos casos e o acesso cirúrgico combinado (paranasal e subfrontal) o preconizado. Os autores apresentam revisäo da literatura discutindo a sua casuística