Mortality from gastrointestinal congenital anomalies at 264 hospitals in 74 low-income, middle-income, and high-income countries: a multicentre, international, prospective cohort study

Summary Background Congenital anomalies are the fifth leading cause of mortality in children younger than 5 years globally. Many gastrointestinal congenital anomalies are fatal without timely access to neonatal surgical care, but few studies have been done on these conditions in low-income and middle-income countries (LMICs). We compared outcomes of the seven most common gastrointestinal congenital anomalies in low-income, middle-income, and high-income countries globally, and identified factors associated with mortality. Methods We did a multicentre, international prospective cohort study of patients younger than 16 years, presenting to hospital for the first time with oesophageal atresia, congenital diaphragmatic hernia, intestinal atresia, gastroschisis, exomphalos, anorectal malformation, and Hirschsprung’s disease. Recruitment was of consecutive patients for a minimum of 1 month between October, 2018, and April, 2019. We collected data on patient demographics, clinical status, interventions, and outcomes using the REDCap platform. Patients were followed up for 30 days after primary intervention, or 30 days after admission if they did not receive an intervention. The primary outcome was all-cause, in-hospital mortality for all conditions combined and each condition individually, stratified by country income status. We did a complete case analysis. Findings We included 3849 patients with 3975 study conditions (560 with oesophageal atresia, 448 with congenital diaphragmatic hernia, 681 with intestinal atresia, 453 with gastroschisis, 325 with exomphalos, 991 with anorectal malformation, and 517 with Hirschsprung’s disease) from 264 hospitals (89 in high-income countries, 166 in middleincome countries, and nine in low-income countries) in 74 countries. Of the 3849 patients, 2231 (58·0%) were male. Median gestational age at birth was 38 weeks (IQR 36–39) and median bodyweight at presentation was 2·8 kg (2·3–3·3). Mortality among all patients was 37 (39·8%) of 93 in low-income countries, 583 (20·4%) of 2860 in middle-income countries, and 50 (5·6%) of 896 in high-income countries (p<0·0001 between all country income groups). Gastroschisis had the greatest difference in mortality between country income strata (nine [90·0%] of ten in lowincome countries, 97 [31·9%] of 304 in middle-income countries, and two [1·4%] of 139 in high-income countries; p≤0·0001 between all country income groups). Factors significantly associated with higher mortality for all patients combined included country income status (low-income vs high-income countries, risk ratio 2·78 [95% CI 1·88–4·11], p<0·0001; middle-income vs high-income countries, 2·11 [1·59–2·79], p<0·0001), sepsis at presentation (1·20 [1·04–1·40], p=0·016), higher American Society of Anesthesiologists (ASA) score at primary intervention (ASA 4–5 vs ASA 1–2, 1·82 [1·40–2·35], p<0·0001; ASA 3 vs ASA 1–2, 1·58, [1·30–1·92], p<0·0001]), surgical safety checklist not used (1·39 [1·02–1·90], p=0·035), and ventilation or parenteral nutrition unavailable when needed (ventilation 1·96, [1·41–2·71], p=0·0001; parenteral nutrition 1·35, [1·05–1·74], p=0·018). Administration of parenteral nutrition (0·61, [0·47–0·79], p=0·0002) and use of a peripherally inserted central catheter (0·65 [0·50–0·86], p=0·0024) or percutaneous central line (0·69 [0·48–1·00], p=0·049) were associated with lower mortality. Interpretation Unacceptable differences in mortality exist for gastrointestinal congenital anomalies between lowincome, middle-income, and high-income countries. Improving access to quality neonatal surgical care in LMICs will be vital to achieve Sustainable Development Goal 3.2 of ending preventable deaths in neonates and children younger than 5 years by 2030

An assessment of the accuracy of contrast enema for the diagnosis of Hirschsprung disease at a South African tertiary hospital

Objectives: To compare radiological findings with the histological diagnosis of Hirschsprung disease (HD) to establish the usefulness of contrast enema as an initial screening and diagnostic tool. To correlate accuracy of radiological diagnosis at Grey’s Hospital with international standards. Materials and methods: Systematic searches were conducted through the Picture Archiving and Communication System and the National Health Laboratory Service records for patients aged 0–12 years, with clinically suspected HD, for whom both contrast enemas and rectal biopsies were performed between 01 January 2011 and 31 August 2015 in a tertiary-level hospital. A total of 54 such patients were identified. Diagnostic accuracy levels were calculated by comparing radiological results with histology results, which is the gold standard. Results: Diagnostic accuracy of contrast enema was 78%, sensitivity was 94.4% and the negative predictive value was 95.7%. Specificity (68.8%) and positive predictive values (63%) were considerably lower. A lower false-negative rate of 5.6% was obtained at Grey’s Hospital as compared with the international reports of up to 30%. Conclusion: Contrast enema remains useful as an initial screening and diagnostic test for HD. Results of this South African tertiary referral hospital were consistent with the best international results for sensitivity of the contrast enema (approximately 80% – 88% in excluding the disease)

Isolated vascularized gastric tube biliary enteric drainage: a paediatric case series experience

Abstract Background Despite the enormous surgical advancements in the last century, access to the biliary system is lost when a Roux-en-Y (RY) biliary drainage procedure is performed. Attempts have been made to overcome this inconvenient sequel using variations in the RY anastomosis, small bowel grafts and vascular grafts. These have been predominantly unsuccessful. An isolated vascularized gastric tube (IVGT) graft has been reported in the literature, which was successfully used for adult patients with common bile duct injuries. We have adopted the technique of using an IVGT graft for bile duct reconstruction in the paediatric patients at our institution. We reviewed our experience at our institution between January 2015 and October 2019. This was a retrospective review of all paediatric patients undergoing an IVGT graft procedure for biliary tract anatomical obstruction in the past 5 years. We looked at the indications for surgery, the demographic profile of the patients and outcomes following surgery and outlined the surgical technique used. Results IVGT bile duct reconstruction was performed on eight patients. Patients ranged from 2 months to 7 years, and there was an equal number of males and females. The diagnosis was made on clinical suspicion and confirmed with ultrasound (U/S) and magnetic resonance cholangiopancreatography (MRCP). There was an 87.5% resolution of biliary obstruction, and two patients who had bile leaks postoperatively were managed conservatively. Unfortunately, one patient died in the early postoperative period from sepsis due to pneumonia. Follow-up was for a minimum of 6 months and up to 5 years. Conclusion IVGT biliary enteric drainage is a safe, reproducible procedure that allows access to the biliary tree if required in the future. Thus, this procedure serves as an alternative, especially in limited-resource areas where interventional radiology is not available for future interventions. </jats:sec

Significant haematuria caused by a pseudo-aneurysm in nephroblastoma

A 7-year-old boy presented with a nephroblastoma, frank haematuria and hypovolemic shock. The haemorrhage was controlled with embolization of the right renal artery and followed by a nephrectomy. In paediatric oncology, interventional radiology has limited application beyond specific indications. This case describes a rare complication where embolization was the key to successful treatment. Keywords: Haematuria, Pseudo-aneurysm, Nephroblastom

Implementation of Laparoscopic Nissen Fundoplications in a Developing South African Pediatric Surgical Service

Introduction: Pediatric laparoscopic Nissen fundoplication (LNF) has become the standard approach at many centers. We developed a minimal access surgery (MAS) training curriculum to enhance the delivery of MAS for pediatric patients in a resource-limited setting. We reviewed our 10-year experience in implementing and performing LNF at our institution. Methods: We described the challenges of implementing MAS training for LNF and how we addressed them. Beneficial technical considerations were described. A retrospective review was performed on all pediatric LNFs performed. Results: We performed 268 LNFs. Specialists or trainees under supervision performed all LNFs. The trainee group performed 43 LNFs (16%). The median operative time for the specialists was 94 min (interquartile range [IQR] 50), and the trainee group was 140 min (IQR 62.5). The median number of cases performed until we improved operative time amongst the trainees was nine (IQR 3). There were seven repeat LNFs, and 11 cases were converted to open. The overall complication rate was 8.9%. A reduction in complications among specialists occurred over the years. The 30-day mortality post-LNF was 0.7%. Conclusion: LNF can be successfully introduced at a tertiary training centre in South Africa with good outcomes. A comprehensive quality improvement program, including MAS training, supported this

Transitioning from thoracotomy to thoracoscopy for esophageal atresia in an LMIC setting

Introduction: Although the benefits of thoracoscopic esophageal atresia repair (TEAR) are well documented, there has been resistance to the uptake of this approach in low- and middle-income countries. This study reviews a single unit's experience introducing TEAR in a South African state sector tertiary hospital. Method: We describe how we set up MIS for esophageal atresia (EA) at our centre. All neonates with EA managed at our institution from January 2016 to January 2022 were included. Excluded patients included those who were not operated on or if data was missing. We compared the different approaches for managing type C EA in our setting. We analyzed the data using the Mann-Whitney U test. Results: Sixty-five patients were managed with EA over the study duration. There were 54 patients who had type C EA. Thirty-nine patients underwent thoracotomy to repair the defect, and eighteen underwent TEAR, of which sixteen were completed thoracoscopically. There was a statistically significant difference in weight (p-value 0.035), gestational age (p-value 0.002), and age at operation (p-value 0.004) between the groups treated by TEAR and OEAR (open esophageal atresia repair). There was a small median difference in the operative time between TEAR and OEAR of 20 min. The mortality in the OEAR group was higher (20.5 %) compared to the TEAR group (5.5 %), with a p-value of 0.094. Conclusion: A dedicated quality improvement program focused on introducing MIS for EA can produce results comparable to the open procedure in an LMIC setting

Developing a novel laparoscopic training model during the Covid-19 pandemic in a resource-limited setting

Background: This paper describes the development and implementation of a unique laparoscopic suturing course in a resource-constrained setting and reviews the initial experience with the program. Methods: This study describes the development of Grey's laparoscopic suturing course (GLSC) and reviews the questionnaires and feedback over the past year. Results: The GLSC has been run for over a year and has enrolled 47 participants. Most participants were registrars, followed by consultants and medical officers, and most participants had limited minimal access surgery (MAS) experience. Only three had previously undertaken a formal course or observership. The mean result for the pre-course test was 50%, and for the post-course test, 88%. During the skills laboratory session, every participant competently performed intra-corporeal suturing. The entire group unanimously agreed that the GLSC should be recommended for all surgical trainees in the evaluation form. All participants expressed interest in an advanced MAS course. Conclusion: We have demonstrated that developing a local MAS suturing course with limited resources and industry support during the Covid 19 pandemic is possible. It has benefited a large group of trainees thus far and hopefully will become part of the curriculum of surgical trainees in South Africa