Challenges and Changes in Pediatric Surgical Practice during the COVID-19 Pandemic Era.

Aim: Working practices in pediatric surgery underwent enormous changes during the era of the COVID-19 pandemic. While certain surgical conditions in children can be managed temporarily with nonsurgical options, most neonates with congenital surgical malformations require emergent operations. We discuss the challenges faced; measures adopted in dealing with surgical emergencies and analyze the diagnoses and outcomes of patients with COVID-19 infection in our institute during the pandemic era. Materials and Methods: When the lockdown was imposed, it was mandated that all elective procedures should be put on hold. We formulated criteria for triaging procedures as emergent, urgent, and elective. A standard operating protocol was devised regarding admission, pre and postoperative management. Protocols for surgical procedures were established in a separate Covid-designated operation room including a specified sequence of donning and doffing personal protective equipment. Results: In the COVID era, from March 23, 2020 to mid-July 2021, 1282 surgeries have been done in our department, 344 emergencies and 461 planned procedures, which include 31 COVID-19 positive cases, with overall good outcomes. 103 emergency surgeries were done during the first wave (March-end to June 2020), and 103during the second wave (April to mid-June 2021). Moreover, not a single healthcare worker in the department has been infected. Conclusion: Pediatric Surgeons are adapting to the new guidelines to continue to provide emergency services with safe and effective care to their patients during the COVID-19 pandemic. Simultaneously, focus on personal and staff protection is ensured to keep the healthcare workers healthy and able to discharge their duties adequately.

The Bracka two-stage repair for severe proximal hypospadias: A single center experience.

BACKGROUND: Surgical correction of severe proximal hypospadias represents a significant surgical challenge and single-stage corrections are often associated with complications and reoperations. Bracka two-stage repair is an attractive alternative surgical procedure with superior, reliable, and reproducible results. PURPOSE: To study the feasibility and applicability of Bracka two-stage repair for the severe proximal hypospadias and to analyze the outcomes and complications of this surgical technique. MATERIALS AND METHODS: This prospective study was conducted from January 2011 to December 2013. Bracka two-stage repair was performed using inner preputial skin as a free graft in subjects with proximal hypospadias in whom severe degree of chordee and/or poor urethral plate was present. Only primary cases were included in this study. All subjects received three doses of intra-muscular testosterone 3 weeks apart before first stage. Second stage was performed 6 months after the first stage. Follow-up ranged from 6 months to 24 months. RESULTS: A total of 43 patients operated for Bracka repair, out of which 30 patients completed two-stage repair. Mean age of the patients was 4 years and 8 months. We achieved 100% graft uptake and no revision was required. Three patients developed fistula, while two had metal stenosis. Glans dehiscence, urethral stricture and the residual chordee were not found during follow-up and satisfactory cosmetic results with good urinary stream were achieved in all cases. CONCLUSION: The Bracka two-stage repair is a safe and reliable approach in select patients in whom it is impractical to maintain the axial integrity of the urethral plate, and, therefore, a full circumference urethral reconstruction become necessary. This gives good results both in terms of restoration of normal function with minimal complication.

Kidney function outcomes in patients after complete primary repair of bladder exstrophy and penopubic epispadias: Results from the international bladder exstrophy consortium.

INTRODUCTION: Historically, repair of bladder exstrophy (BE) is associated with compromise to the upper tracts; the single stage repair of BE was considered to exacerbate risks of kidney impairment. OBJECTIVE: We aim to evaluate the risk of upper urinary tract deterioration or chronic kidney disease after the complete primary repair of exstrophy (CPRE). STUDY DESIGN: As part of the U.S.-India Multi-institutional Bladder Exstrophy Collaboration, we prospectively performed data collection on all patients managed at the Civil Hospital, Ahmedabad from 2010 to 2020. All patients who underwent primary or redo BE or primary penopubic epispadias (PE) repair using CPRE were included. Data on annual VCUG and DMSA, serum creatinine and cystatin-C, urinary albumin, and creatinine were aggregated. RESULTS: 72/104 patients who underwent CPRE at a median age of 1.7 years (IQR: 1.1-4.6) were included: 43(60%) patients with primary BE, 17(24%) with redo BE, and 12(17%) with primary PE. At a median follow-up of 4 years (IQR: 3-6), the overall median eGFR was 105 for BE, and 128 ml/min for PE. 14(19%) patients had eGFR<90, and 22(31%) had microalbuminuria. 21(30%) patients had kidney scarring in DMSA and 31(44%) had VUR. Multivariate analysis showed that neither kidney scarring nor VUR could predict the presence of eGFR<90 or microalbuminuria. Of 72 patients, 2 (3%) patients had dry intervals >3 h, 9 (13%) patients have dry intervals of 1-3 h and 44 (61%) patients had dry intervals <1 h during follow-up. We found that kidney function outcomes (i.e., eGFR and microalbuminuria) were not associated with continence status (p = 0.3). DISCUSSION: In this series, we report a 5% incidence of CKD stage 2 or above that was not impacted by continence status. Furthermore, a 40% incidence of VUR and a 30% incidence of kidney scarring during follow-up was observed within this cohort, neither of which had a significant impact on renal function deterioration (i.e, decline in eGFR), but underscores the need for close kidney surveillance in children that have undergone bladder exstrophy repair. CONCLUSIONS: Modern CPRE technique for the repair of BE may increase the risk of kidney scarring in the intermediate-term follow-up, however, this finding does not correlate with low eGFR and presence of albuminuria inpatients. Therefore, close follow-up with serial kidney function measurements is warranted and necessary after CPRE.

Caregiver distress: A mixed methods evaluation of the mental health burden of caring for children with bladder exstrophy.

Introduction: Caring for children with bladder exstrophy-epispadias complex (BEEC) exacts a long-term emotional toll on caregivers. Previous studies leave a gap in understanding the impact that caring for a child with BEEC has on caregivers in low- and middle-income countries (LMIC). We hypothesize that families and caregivers experience psychological distress that has long gone unaddressed. Materials and methods: From 2018 to 2020, researchers conducted a multi-method evaluation of caregiver distress with participants recruited as part of the annual International Bladder Exstrophy Collaboration based in Ahmedabad, Gujarat, India. In 2018, pilot data was collected through cognitive interviews. In 2019, researchers conducted structured interviews predicated on themes from the previous year, which subsequently prompted formal mental health screenings in 2020. Caregivers who reported suicidal thoughts were immediately referred for intervention. Results: In 2018, caregivers described the primary source of stigma arose from their village (n = 9, 26.5%). Caregivers also identified long-term concerns (n = 18, 52.9%), including future fertility and marital prospects, as sources of anxiety. In 2019, caregivers substantiated preliminary findings with the primary source of anticipated (n = 9, 31%) and experienced (n = 19, 65.5%) stigma again stemming from their communities. Both cohorts identified the collaboration as a positive source of support (n = 23, 36.5%). In 2020, caregivers stated decreased emotional wellbeing as number of subsequent repairs increased (n = 54, 75%, p = 0.002). Caregivers of children who underwent initial surgery within 5 years of screening reported higher anxiety (n = 46, 63.8%) and this was exacerbated as the number of subsequent repairs increased (p = 0.043). Conclusion: Complex, long-term course of care, including additional surgeries, significantly impacts caregiver distress in the LMIC setting. Screening for caregivers of children with complex congenital anomalies, like BEEC, should be an essential element of any comprehensive effort to alleviate the global burden of disease.

Laparoscopic pyloromyotomy for congenital hypertrophic pyloric stenosis: Our experience with twenty cases.

PURPOSE: Laparoscopic pyloromyotomy for hypertrophic pyloric stenosis has become quite popular over the past decade. There have been many modifications in the technique initially described by Alain et al. in 1991. We describe our experience of the laparoscopic procedure performed in twenty cases. MATERIALS AND METHODS: This study includes twenty patients of pyloric stenosis who underwent laparoscopic pyloromyotomy from March 2017 to March 2020. All the infants had classical clinical symptoms and abdominal ultrasound confirming the diagnosis of pyloric stenosis. Two 3-mm ports and one 5-mm port were used. The duodenum was grasped to stabilise the olive; a stab knife cut to 10 mm and mounted on a needle holder was introduced through the 3-mm trocar in the left hypochondrium to perform the myotomy, and subsequently, the myotomy was spread with a 5-mm Maryland forceps. Feeding was started 6 h postoperatively. RESULTS: Twenty patients with congenital idiopathic pyloric stenosis underwent laparoscopic pyloromyotomy by this technique. The average operating time was 42 min. There were no peri- or post-operative complications. The post-operative hospital stay ranged between 36 h and 54 h. CONCLUSION: Laparoscopic pyloromyotomy using a stab knife mounted on a needle holder is a technically feasible, safe and effective surgical procedure for pyloric stenosis.

Variant of Bladder Exstrophy With an Intact Penis: Surgical Options and Approach.

Variants of bladder exstrophy are a rare but diverse spectrum of bladder exstrophy-epispadias complex. This case series describes a group of 4 unique exstrophy variant cases who had an intact phallus, but a completely open bladder plate. These patients underwent exstrophy repair and concomitant umbilicoplasty at the Civil Hospital, Ahmedabad as part of the US-India Multi-institutional Bladder Exstrophy Collaboration and were followed at the same institution. We believe that a detailed assessment of bladder neck prior to reconstructive repair and bladder closure would be beneficial in these cases as the extent of bladder neck involvement would affect reconstructive approach.

A Model for Sustained Collaboration to Address the Unmet Global Burden of Bladder Exstrophy-Epispadias Complex and Penopubic Epispadias: The International Bladder Exstrophy Consortium.

Importance: International collaboration to alleviate the massive burden of surgical disease is recognized by World Health Organization as an urgent need, yet the surgical mission model to treat reconstructive surgical challenges is often constrained in ensuring adequate patient follow-up, optimal outcomes, and sustainability. Objective: To determine whether a collaboration predicated on long-term commitment by surgeons returning to the same institution annually combined with an experienced host surgical team and infrastructure to ensure sustained patient follow-up could provide surgical care with acceptable outcomes to treat bladder exstrophy-epispadias complex (BE) and penopubic epispadias (PE). Design, Setting, and Participants: In this prospective, observational study, long-term collaboration was created and based at a public hospital in Ahmedabad, India, between January 2009 and January 2015. The entire postoperative cohort was recalled in January 2016 for comprehensive examination, measurement of continence outcomes, and assessment of surgical complications. Seventy-six percent of patients (n = 57) who underwent complete primary repair of exstrophy during the study interval returned for annual follow-up in 2016 and formed the study cohort: 23 patients with primary BE, 19 patients with redo BE, and 11 patients with PE repair. Main Outcomes and Measures: Demographics, operative techniques, and perioperative complications were recorded. A postoperative protocol outlining procedures to ensure monitoring of study participants was followed including removal of ureteral stents, urethral catheter, external fixators, imaging, and patient discharge. Results: Of the 57 patients, 4 were excluded because they underwent ureterosigmoidostomy. Median age at time of surgery was 3 years (primary BE), 7 years (redo BE), and 10 years (PE), with median follow-up of 3 years, 5 years and 3 years, respectively; boys made up more than 70% of each cohort (n = 17 for primary BE, n = 15 for redo BE, and n = 9 for PE). All BE and 3 PE repairs (27%) were completed with concurrent anterior pubic osteotomies. Seventeen of 53 patients (32%) experienced complications. Only 1 patient with BE (4%) had a bladder dehiscence and was repaired the following year. Conclusions and Relevance: A unique surgical mission model consisting of an international collaborative focused on treating the complex diagnoses of BE and PE offers outcomes comparable with those in high-income countries, demonstrating a significant patient retention rate and an opportunity to rigorously study outcomes over an accelerated interval owing to the high burden of disease in India. Postoperative care following a systematized algorithm and rigorous follow-up is mandatory to ensure safety and optimal outcomes.

Persistent Cloaca associated with Unilateral Lung Agenesis- A Rare Presentation.

We report a case of full term female child having persistent cloaca who was diagnosed to have right lung agenesis on investigations.

Extra-hepatic biliary atresia in association with polysplenia and intestinal malrotation.

The syndromic form of biliary atresia accounts for 10-25% and is associated with a poor prognosis due to associated anomalies. We report a case of extrahepatic biliary atresia and polysplenia syndrome with jaundice since 19th day of life and who had undergone surgical correction of malrotation in the neonatal period. Inspite of successful Kasai's portoenterostomy at 52nd day of life, the child succumbed to post-operative sepsis.

Single Stage Transanal Pull-Through for Hirschsprung's Disease in Neonates: Our Early Experience.

OBJECTIVE: Hirschsprung's disease is one of the common causes of intestinal obstruction in neonates. Transanal endorectal pull-through represents the latest development in the concept of the minimally invasive surgery for Hirschsprung's disease. In this study, we present our early experience with single stage transanal pull through in neonates. DESIGN: Retrospective study of neonates with single stage transanal pull-through done for Hirschsprung's disease in our institute from January 2011 to January 2013. MATERIAL AND METHOD: Five newborn boys who presented with Hirschsprung's disease were studied. The selection criteria included radiological transition zone at rectosigmoid or mid-sigmoid region, weight more than 2 kg, no evidence of enterocolitis or sepsis and no associated major anomaly. Single stage transanal endorectal pull-through was done in these patients. The follow-up period ranged from 6 months to 2 years. RESULTS: Five patients with a mean age of 26.4 days (range 15-45 days) and a mean weight of 2.6 Kg (range 2.2 to 3.7 Kg) underwent transanal endorectal pull through. The mean operating time was 68 min (range 60 to 120 min). The average intra-operative blood loss was 20 ml (range - 10 to 30 ml) and the average length of bowel resected was 12.8 cm (range - 10 to 18 cm). Post-operatively patients passed first stool between 2nd and 3rd day. Oral feeding was resumed on 5th to 6th post-operative day. The average post-operative duration of stay in hospital was 10 days. None of the patients had post-operative bleeding, urethral injury, anastomotic leak or retraction of anastomotic site. Three patients developed perianal excoriation and one patient had post-operative enterocolitis. No mortality occurred in the series. CONCLUSION: Advancement in pediatric anaesthesia, availability of pediatric surgical expertise, improvement in pre-operative and post-operative management and nursing care has made single stage transanal pull-through in neonates a feasible option. The early results are comparable to single stage or multistage surgery in older children.