Snodgrass (tubularized incised plate) versus Mathieu repair of distal hypospadias: A systematic review and meta-analysis of randomized controlled trials.

INTRODUCTION: Distal hypospadias accounts for the majority of hypospadias. Mathieu and Snodgrass techniques are widely used for repair of distal hypospadias but their comparative efficacy and reported outcomes are still debated. We conducted a systematic review and meta-analysis to compare the outcomes of these two techniques. METHODS: Electronic databases and trial registries were searched for randomized controlled trials (RCTs) comparing Mathieu and Snodgrass techniques for primary distal hypospadias repair. Outcome measures included complication rates and cosmetic outcomes. Risk of bias assessment was performed using the ROB2. Data was analyzed using Review Manager 5.4; Trial Sequential Analysis (TSA) also was conducted for important outcomes. RESULTS: Twelve RCTs were eligible for inclusion. The Snodgrass technique showed a significantly lower incidence of urethrocutaneous fistula (UCF) (RR 0.48, 95% CI [0.30-0.77]). However, there was no significant difference in the incidence of meatal stenosis (MS) between the two (RR 1.64, 95% CI [0.85-3.15]). TSA supports the findings for outcome UCF but not for MS. Operative time was shorter with the Snodgrass technique. CONCLUSION: This SRMA indicates a lower incidence of UCF with Snodgrass technique compared to the Mathieu technique in distal hypospadias repair. However, there was no significant difference in the risk of MS.

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.

Impact of Urinary Tract Infection on Progression of Renal Scars and Formation of New Scars in Patients with Primary Vesicoureteric Reflux.

INTRODUCTION: Primary vesicoureteric reflux (VUR) is a prevalent cause of end-stage renal failure in children. Scars on radionuclide imaging indicate irreparable damage to the growing kidneys. This study aims to determine whether a urinary tract infection (UTI) associated with primary VUR promotes the development of new renal scars or progression in the pre-existing ones. MATERIALS AND METHODS: Children with primary VUR on continuous antibiotic prophylaxis at a tertiary teaching hospital's paediatric nephrourology clinic were observed prospectively. At recruitment, a renal cortical (dimercaptosuccinic acid [DMSA]) scan was done, and the children were followed up every 3 months. Breakthrough UTIs were documented, and follow-up DMSA scans were performed to document new scarring/grade advancement of existing scars in the renoureteric units (RUUs). RESULTS: Seventy-two RUUs in 36 patients were monitored. Fifty-four (75%) RUUs were exposed to VUR of different grades, and 46 (85.1%) had a UTI. On the DMSA scan, these showed new scar development in 10/18 (55%) RUUs and scar progression in 13/28 (46.4%) RUUs. Of the 8 RUUs with VUR and without UTI, new scars manifest in 4/5 (80%) and progressed in 1/3 (33%). On univariate analysis, there was no significant difference in the formation of new scars or scar advancement on the DMSA scan between RUU with VUR and UTI and those without UTI. There was a significant positive correlation between the scarring on DMSA scan and the grade of reflux (P < 0.001). CONCLUSION: Primary VUR patients had fresh or progressed scarring regardless of urinary infection. A higher VUR grade at presentation was a significant risk factor for scarring.

Currarino syndrome with immature teratoma: A case report with review of literature.

ABSTRACT: Currarino syndrome (CS) is a rare congenital syndrome characterized by a triad of anorectal malformation, sacral deformity, and presacral mass. In about 50% of cases, it is caused by HLXB9 gene mutation in chromosome 7q36. A 13-month-male child presented with presacral discharging sinus with a history of surgery for anorectal malformation and perineal fistula at the time of birth. On detailed investigation, the child revealed to have anal atresia, hemisacrum, and presacral mass. Histopathology of presacral mass showed features of immature teratoma. The presacral mass in CS is mostly an anterior myelomeningocele or presacral teratoma. The development of immature teratoma in presacral mass is very rare. The histopathological identification of immature component of teratoma in the presacral mass of CS is important for risk stratification and further management. Suspicion of CS should be raised in any child presenting with partial phenotype of the triad.

Urethral Caruncle with Associated Renoureteric Anomalies.

A urethral caruncle is a rare differential diagnosis for a prolapsed mass at the urethral meatus. The aetiopathogenesis of this entity is unclear. We report a three-year-old female patient who presented to a tertiary care teaching hospital in India in 2019 with intermittent bleeding from the introitus for a month. Investigations revealed a urethral caruncle and associated renal anomalies which has not been described in the previous literature. The patient was discharged with a prescription of a Sitz bath twice daily and advised to apply betamethasone (0.1%) cream locally once daily. There was a significant improvement after six weeks of therapy; at two-years follow-up, the lesion had disappeared completely.

Left Gastric Artery Pseudoaneurysm Complicating Chronic Calcifying Pancreatitis in a Child.

A left gastric artery pseudoaneurysm is a rare complication of pancreatitis and is associated with significant morbidity and mortality. We report a 14-year-old male with severe abdominal pain and a palpable upper abdominal mass, earlier diagnosed as chronic idiopathic calcifying pancreatitis, and awaiting surgical intervention. Computed tomography showed a pseudocyst and a pseudoaneurysm in the lesser sac near the left gastric artery. The patient underwent successful angiographic coiling of the left gastric artery and definitive pancreatic surgery weeks thereafter. The early detection and interventional radiologic management of the vascular complication averted a life-threatening hemorrhage without emergency surgery in a pediatric patient.

Pediatric primary extra-ovarian Sertoli Leydig cell tumor of the retroperitoneum.

Ovarian Sertoli Leydig cell tumors (SLCT) accounts for less than 0.5% of all ovarian malignancies. The incidence of primary extra-ovarian SLCT is extremely rare with reported cases occurring in young adult women till now. We report case of primary retroperitoneal extra-ovarian SLCT in a seven-year girl child without any hormonal manifestation. She presented with complaint of left side abdominal swelling associated with intermittent pain for a duration of six months. CT scan revealed a huge retroperitoneal space-occupying lesion abutting the dorsal vertebrae and present posterior to pancreas, spleen and left kidney. The tumor was diagnosed as extraovarian Sertoli Leydig cell tumor with intermediate differentiation on histopathology and immunohistochemistry.

Telemedicine - a Boon to Parents of Children with Health Care Needs During COVID-19 Pandemic: A Qualitative Study from India.

OBJECTIVE: The coronavirus disease 2019 pandemic had greatly impacted health worldwide. The nationwide lockdown was imposed to contain the virus transmission, which indirectly affected health care utilization. Pediatric patients', as they are considered as a vulnerable group, parents faced a significant challenge to manage their children's surgical and medical care needs during the coronavirus disease 2019 pandemic. The study aims to explore the parental approach to health care facilities to meet children's surgical care needs during the coronavirus disease 2019 pandemic. MATERIALS AND METHODS: A qualitative approach was adopted to fulfill the objective by conducting an in-depth interview using a semi-structured interview schedule among 26 parents of children with perioperative surgical care needs at a tertiary care hospital, eastern India. The digitally recorded interviews were transcribed verbatim. Thematic analysis was employed to understand the parent's experience toward meeting children's surgical care needs during the coronavirus disease 2019 pandemic lockdown. QSR NVIVO software version 12 was used for data management. RESULTS: The study found 3 themes related to parent's experience which include state of desperation (sub-themes: lockdown effect, ignorant to the health facility, phobic to coronavirus disease infection, and testing), state of assurance (sub-themes: telemedicine: accessibility, approachability, and applicability), and state of serenity (sub-themes: refrained from somatic symptoms and shouldering the responsibility). CONCLUSION: Despite various hurdles parents faced during the pandemic, telemedicine helped parents meet their children's surgical care needs. Framing guidance, protocols to deal with emergency and primary care delivery, and disseminating information on telemedicine facilities to grassroot level to the community can protect this vulnerable population in the upcoming surge of coronavirus disease 2019 waves.

46 XY disorder of sex development (DSD) due to 5 alpha (SRD5A2) deficiency - Experience from a multidisciplinary Pediatric Gender Clinic.

BACKGROUND: SRD5A2 deficiency leads to incomplete masculinization of individuals with a 46 XY karyotype. A definitive diagnosis in early infancy facilitates decisions concerning choice of sex of rearing and management. AIM: To review the clinical presentation, diagnosis, treatment and outcome of children with 46 XY DSD due to SRD5A2 deficiency at a Paediatric Gender Clinic. STUDY DESIGN AND METHODS: Retrospective review of cases of SRD5A2 deficiency (2000-15) managed with a standard protocol at a multidisciplinary clinic. Demographic data, clinical presentation, physical findings, investigations (hormonal profile, imaging, genitoscopy), psychological evaluation (child, family), medical and surgical management, outcome and follow up were collated and analyzed. RESULTS: There were 12 cases aged 3 days-14 years at presentation, 3 had parental consanguinity. Eight were reared as males and 4 as females. Specialist referral was sought for hypospadias (5), atypical genitalia (5) or incongruent pubertal masculinization (2). All had chordee, symmetrical inguinoscrotal gonads, rugose labioscrotum and proximal hypospadias (perineoscrotal -9, perineal -3). Both pubertal cases had significant masculinization and no gynecomastia. The median testosterone/dihydrotestosterone ratio was 22.1(IQR-8.6-55.7). Despite a classical phenotype, four (2 prepubertal, 2 pubertal) had a ratio <10. Genitoscopy showed urogenital sinus remnant (4) and hypoplastic verumontanum (5). Sex reassignment was done in 4. Surgical management was staged and completed by 4 years in those with infantile presentation. Besides correction of chordee and urethroplasty in 11, other procedures included orchidopexy (5), excision of a urogenital sinus remnant (4) and correction of penoscrotal transposition (4). The urethroplasty was single staged in 3. All operated cases were followed up (mean age at last follow up - 10.63 years, mean follow up period - 7.25 years). The overall cosmetic result was satisfactory, but the phallic structure remained relatively small across prepubertal period. Uroflowmetry curves were normal in 9. All showed penile tumescence/erection and two peripubertal cases had typical secondary sexual characters. All cases, including those with sex reassignment, have a well-adjusted male psyche. DISCUSSION AND CONCLUSION: The diagnosis, management and longitudinal follow up of cases of SRD5A2 deficiency at a multidisciplinary gender clinic is presented. Diagnostic dilemmas with low T/DHT ratios remained in a third of cases. Most were diagnosed in infancy and assigned a male sex of rearing, all underwent staged masculinizing genitoplasty. Those with sex reassignment also fared well with comprehensive management after family counseling.

Pelviureteric Junction Obstruction Due to Vascular Anomalies in Children - Simple Surgical Options.

Aims: Pelviureteric junction obstruction (PUJO) due to aberrant lower polar artery is conventionally managed with pyeloplasty. We present our experience of managing PUJO due to "vascular" anomalies-aberrant lower polar artery and vascular adhesions with simpler surgical options. Subjects and Methods: This is a protocol based, retrospective study of PUJO. Preoperative investigations included ultrasonography (USG) and diuretic renogram. An intraoperative methylene blue test (MBT) assessed transit across the Pelviureteric junction (PUJ) after release of vascular compression. Surgical management included adhesiolysis for vascular adhesions and pyelopyelostomy anterior to the aberrant polar artery. Postoperative studies were repeated after 3 and 6 months. Results: Fourteen of 144 PUJO (9.7%) were "vascular" obstructions. Those with vascular adhesions (six) were largely infants with antenatal hydronephrosis. Children with aberrant lower polar artery (eight) were older, had fleeting symptoms, minimally increased pelvic diameter and subtle impairment on diuretic renogram. Majority were term males with urinary tract infection. The MBT showed normal transit across the PUJ in all. Postoperatively, there was progressive improvement on USG and diuretic renogram after 3 and 6 months. None had any complication or redosurgeries. At a mean follow-up of 41.2 months, all are asymptomatic. Conclusions: PUJO due to extrinsic vascular anomalies is rare. Intraoperative evaluation with the MBT ruled out associated intrinsic pathology. We describe two simple surgical alternatives preserving the normal PUJ - adhesiolysis for vascular adhesions and pyelopyelostomy for aberrant lower polar artery. The preliminary outcomes are comparable to conventional pyeloplasty.

Risk factors for renal scarring and clinical morbidity in children with high-grade and low-grade primary vesicoureteral reflux.

INTRODUCTION: Primary vesicoureteral reflux (VUR) is associated with urinary tract infections (UTIs) and renal damage. However, the importance of early diagnosis of VUR has been questioned. Moreover, most studies have few patients with high-grade VUR. Hence, we retrospectively analyzed a large cohort of patients with primary high-grade and low-grade VUR and assessed risk factors for renal damage and clinical morbidity. MATERIAL AND METHODS: We included patients (<18 years) at diagnosis with low-grade (1-3) or high-grade (4-5) primary VUR and noted their clinical history and presence of hypertension, low eGFR (<60ml/in/1.73 m2), renal scarring (focal or generalised) and reduced differential renal function (DRF; <45%). Risk factors were assessed (in patients and renal units) by logistic regression and generalised estimating equation. RESULTS: Of 399 primary VUR patients, 255 (64%) had high-grade VUR. Indications for voiding cystourethrogram were recurrent UTI (38%), first UTI (28%) and antenatal hydronephrosis (17%). At diagnosis, 252 (65%) had renal scars (focal in 170 [44%], generalised in 82 [21%]), and 188 (47%) had reduced DRF. High-grade VUR patients were more likely than low-grade VUR patients to have renal scarring (75% vs. 49%, p < 0.01), low eGFR (23% vs. 13%, p = 0.04) and significant hypertension (26% vs. 13%, p = 0.02). High-grade VUR was associated with generalised scars (odds ratio [OR] 11, p < 0.001), focal scars (OR 3.1, p < 0.001) and reduced DRF (OR 2.3, p < 0.001) shown in the table. Male sex was a risk factor for generalised scars (OR 2.3, p = 0.005). Focal scars were associated with recurrent UTIs (OR = 1.8, p = 0.004) and reduced DRF (OR 1.4, p = 0.027). Patients with multiple focal scars were diagnosed at an older age (2 years [1,4] than those with single scars (1.5 years [1,4] or no scars (1 year [0, 3]), p = 0.04). DISCUSSION: The prevalence of renal damage and clinical morbidity at VUR diagnosis was higher than other studies. High-grade VUR patients had a greater prevalence of renal damage, low eGFR and hypertension than low-grade VUR patients and was a risk factor for focal scars, generalised scars and reduced DRF. Focal scars were independently associated with recurrent UTI. Those with multiple scars were diagnosed later than those with single scars or no scars. CONCLUSIONS: High-grade VUR was associated with renal damage and clinical morbidity. Our study highlights the importance of diagnosing VUR early to identify patients who may warrant long-term follow-up and intervention to minimize morbidity.

'Hybrid' Bronchopulmonary Malformation - Lobar Emphysema and Extra Lobar Sequestration.

Background: Idiopathic lobar emphysema (ILE) and bronchopulmonary sequestration (BPS) are two of the well-characterized pulmonary malformations. Case report: An antenatally detected case of a left bronchopulmonary malformation (BPM) was clinicoradiologically diagnosed to be a left upper lobar emphysema with isolated dextrocardia in the neonatal period. Besides the emphysematous left upper lobe, an accessory lobe akin to an extra lobar BPS was an operative surprise. Histopathological examination of both excised lobes led to a revised diagnosis of a 'hybrid' malformation comprising lobar emphysema and extra lobar BPS. The postoperative recovery was uneventful. Discussion/conclusion: The observations suggest that BPS and ILE may be interim entities in a continuum of abnormal embryogenesis. Such a hybrid malformation has not been hitherto reported.

Loss of interest for training in paediatric surgery in India.

Background Despite the sizeable Indian paediatric population, few students have opted for postdoctoral (Magister Chirurgiae [MCh]/Diplomate of the National Board of Examinations [DNB]) courses in the past decade. We analyse the apparent loss of interest for training in paediatric surgery in India and suggest remedies. Methods We did a combination of an online questionnaire-based survey and several interviews among students, teachers and practitioners of paediatric surgery. The results were collated and analysed. Results Information from 238 questionnaires, 35 interviews and 75 feedbacks were distilled. About 83% of respondents agreed to a definite loss of interest among students in paediatric surgery, largely because of poor exposure during undergraduate/postgraduate courses and absence of a department in medical colleges. The blanket increase in seats has led to vacancies. The low saleability of paediatric surgery is linked to high personnel and infrastructure investment, modest remunerative potential and poor insurance cover for patients. Besides increasing public awareness, strategic governmental patronage in postdoctoral training (e.g. establishing a department in medical colleges, moderating the number of seats, encouraging performing training centres and rationalizing the bond after the training course) and facilitation of patient care (e.g. insurance cover for congenital conditions and paediatric surgeon in neonatal care units in district hospitals) is suggested. Conclusions The loss of interest in paediatric surgery among medical trainees is real and urgently requires a multipronged strategy by the medical fraternity, professional organizations and regulatory bodies across government and non-governmental sectors to facilitate a revival and cater to the sick surgical child in the future.

Awareness of Common Pediatric Surgical Entities among Practicing Indian Pediatricians.

AIM: Common pediatric surgical entities often get a delayed referral to the pediatric surgeon in the average Indian scenario. This study was conducted to assess the awareness about management of select common pediatric surgical entities among practicing pediatricians. MATERIALS AND METHODS: An online multiple-choice questionnaire consisting of twenty questions related to the diagnosis and management of common pediatric surgical entities encountered by pediatricians in their routine office practice was prepared, and the electronic link was circulated among the practicing pediatricians of India. Each question had one correct response. RESULTS: One hundred and seventy-five responses were obtained, collated, grouped, and analyzed. Overall, 35% of all responses were correct. Individually, 56% of respondents marked 51%-75% of answers correctly; only 7% scored above 75%. Some clinical observations were disparate: 65% were unfamiliar with preputial adhesions and 51% would discharge a preterm neonate with inguinal hernia without a surgical consult. There is a tendency toward unnecessary imaging (60% - localization of undescended testis, 91% - wet umbilicus, and 51% - postanal dimple) and overzealous medical management (propranolol for the involuting hemangioma). However, 82%-88% concurred on the standard conservative or surgical management in index conditions such as hypospadias and umbilical problems. CONCLUSION: There was a concurrence in the principles of management of common pediatric surgical entities between pediatricians and pediatric surgeons in 35%. Misdiagnosis, unwarranted investigations, overzealous medical management, and delayed surgical consults figured in 65%. A systematic professional interaction between the physicians and surgeons would facilitate a coordinated management. A larger study would yield more meaningful data.

Xanthogranulomatous cystitis masquerading as bladder tumor in a child.

Xanthogranulomatous cystitis affecting the urinary bladder is extremely rare, and only around thirty adult cases and two pediatric cases have been reported in the literature. The treatment is predominantly surgical as the lesion is mostly infiltrative and mimics malignancy. We report probably the third pediatric case, who presented with symptoms of urinary tract infection and urinary retention and was initially suspected as bladder tumor on imaging. The diagnosis was confirmed on histopathology, and the child responded well to aggressive antibiotic therapy alone.

Heteropagus Twins: A Tale of Two.

Parasitic twin is a rare occurrence, with rachispagus being the rarest. The surgical approach and timing are not standardized. We describe the management of two cases of rachispagus and review the sparse literature on this entity. Both cases were operated early because of the social burden on the family as well as difficulty in nursing care. The first patient had associated major systemic malformations in the autosite (cerebral, spinal and cardiac), and succumbed in the postoperative period. The entity was isolated in the second who is doing well.

Intracranial, Extradural, Hemangiopericytoma in a Neonate.

Intracranial infantile hemangiopericytoma (HPC) is a rare, sparsely documented neoplasm with a relatively favorable prognosis than its adult counterpart. We describe a neonatal extradural, intracranial, infantile HPC managed with near-total excision.