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

A simple scoring system to train surgeons in basic laparoscopic skills

AIM To develop and validate a scoring system for a training assessment tool using a box trainer which can objectively demonstrate progression in laparoscopic skills. METHOD 170 assessments were performed over a 5-year period by doctors working in a busy paediatric surgical department. Each participant was scored based on experience and then undertook six laparoscopic tasks in a box trainer in a dry skills lab. The quicker and more accurate the performance, the lower the score. Validity and reliability tests were applied. RESULTS Pearson correlation coefficient demonstrated that more experienced surgeons performed better than novices with an r of -0.63 (p 40) p < 0.001]. Improvement in score was seen at all experience levels with greatest improvement seen in the less experienced (2315, 1820, 1571 p < 0.001). Cronbach's alpha was 0.70 and the intraclass correlation coefficient for test-retest reliability was 0.81. CONCLUSION Construct validity with adequate reliability has been demonstrated for this simple training tool and scoring system. All experience levels demonstrated improvement in their laparoscopic skills by simulation training in a laparoscopic box trainer

Uneventful survival of a rural child after penetrating cardiac injury by a thorn: a case report

Abstract Background Paediatric penetrating cardiac injury is extremely rare, precluding published management guidelines, therefore warranting a case-by-case approach with learning points gleaned from each case. Case summary A 7-year-old boy presented to a rural hospital with a stab wound to the chest by a Withaak (Vachellia tortilis) thorn. The patient was haemodynamically stable on presentation, but a 2 cm subcutaneous, pulsatile mass was present at the cardiac apex. Echocardiography revealed a foreign body penetrating from the apex into the heart, with evidence for a fistula between a cardiac chamber and the pulsatile mass. Angiography confirmed the existence of the fistula between the right ventricle (RV) and the pulsatile mass. A controlled extraction under general anaesthaesia via median sternotomy was performed in-theatre, with blood products and cardiac bypass on standby. The patient recovered without complications and was discharged after 4 days. Discussion Our case illustrates the limitations of echocardiography in identifying the precise anatomical definition of penetrating cardiac injuries. Angiography is therefore indicated in such cases. The injury to the RV and the haemostatic effects of the in situ thorn were favourable prognostic factors. We believe that the mortality risk reduction of extraction under full control warrants the minor morbidity of a median sternotomy. </jats:sec

A decade of managing pediatric major traumatic vascular injuries: insights from a referral center

Purpose Incidence, management, and outcomes of pediatric vascular injuries secondary to non-iatrogenic trauma were reviewed over a decade in our institution. Methods a retrospective review of medical records (2013–2022) of major traumatic vascular injuries, focusing on injury profiles, treatment modalities, and clinical outcomes. Results thirty patients with 48 vessel injuries were included. Firearms were the leading mechanism, accounting for 43.3% (n = 13) of cases. We identified 29 arterial injuries and 19 venous injuries, with 30 (62.5%) of the overall injuries occurred in the lower extremities. Shock (17; 56.7%) and associated injuries (25; 83.3%) were common. Surgery was the most common management strategy. Autologous bypass graft was the most frequently performed procedure for arterial injuries (8; 42.1%), while ligation dominated in venous injuries (9; 64.3%). Blood transfusion requirements (24; 82.7%) and post-operative pre-scription of anticoagulant and antiplatelet agents (12; 41.4%) were similar for arterial and venous injuries (p > 0.05). Three patients demised, resulting in a 90% survival rate. Neither the mechanism of injury, anatomical location, and presence of shock on arrival nor the baseline hemoglobin level served as predictors of mortality. Conclusion intensive resuscitation with blood transfusion and prompt surgical intervention achieve favorable survival rates for pediatric traumatic vascular injuries. Optimal post-operative anticoagulant and antiplatelet regimens remain unclear

A 10-year analysis of paediatric extremity vascular injuries in a Tertiary Referral Centre

Background And Aims: Non-iatrogenic vascular injuries affecting the extremities of children pose a challenge to paediatric surgeons due to their rarity and non-specific presentations. Our aims were to assess the injury profiles, management strategies and short-term outcomes of this condition following paediatric trauma. Methods: A retrospective analysis of the medical records of patients who presented with vascular injuries, focusing on extremity injuries, over 10 years from 2013 to 2022 in our centre in Cape Town, South Africa was conducted. Results: 21 children sustained vascular injuries involving the extremities, affecting 32 vessels (n=22, 68.8% arteries and n=10, 31.2% veins). Males predominated at 76.2% (n=16) with a median age of 11 years and three months and a weight of 30 kg. Road traffic accidents (RTA) were predominant at 47.6% (n=10), followed by firearms at 38.1% (n=8). The femoral artery and popliteal artery were the most frequently injured vessels, accounting for 28.1% (n=9) each. Lower extremity injuries predominated over upper extremity injuries for both arteries (n=20, 90.1%) and veins (n=10, 100%) from our series, and majority (n=31, 96.5%) had surgical interventions. Autologous bypass graft was the most performed procedure at 52.9% (n=9) for the arterial injuries, while for venous injuries, ligation dominated at 85.7% (n=6). Four patients (9.5%) with seven vessels injured underwent primary amputations. All these patients sustained multiple injuries following RTAs, and all four subsequently underwent interventions i.e. amputations within four hours of presentation for haemostasis. Overall, three patients (14.3%), including one who had undergone primary amputation, succumbed to their injuries, all of whom sustained injuries involving femoral arteries. Conclusion: Early major bleeding remains the primary cause of mortality for vascular injuries involving the extremities. Hence, the more proximal the vessel is, the greater the mortality risk. Early diagnosis, resuscitation and prompt intervention, including consideration of damage control measures and/or primary limb amputation when necessary are crucial for reducing mortality rates, prioritizing the principle of saving lives over limbs

First insights into species and genotypes of Echinococcus in South Africa

Cystic echinococcosis is a serious and neglected parasitic zoonosis that is regarded as an emerging disease world-wide. Effective control of the disease is based on understanding the variability of Echinococcus granulosus (sensu lato), as genotypic characteristics may influence lifecycle patterns, development rate, and transmission. No molecular epidemiological research has previously been conducted to shed light on genotypes responsible for the disease in South Africa. To identify strains circulating in the country, parasite material was collected from patients between August 2010 and September 2012 and analyzed by PCR/RFLP methods. A total of 32 samples was characterized as E. granulosus sensu stricto (G1-G3) (81%), E. canadensis (G6/7) (16%) and E. ortleppi (G5) (3%). Furthermore, two co-amplifying G6/7 genotypes were confirmed as G7 by sequencing. This is the first report on genotyping of Echinococcus species in South Africa, and, to the best of our knowledge, the first report of the G5 and G7 genotypes from humans in Africa. (c) 2013 Elsevier B.V. All rights reserved

Human ISPD Is a Cytidyltransferase Required for Dystroglycan O-Mannosylation.

A unique, unsolved O-mannosyl glycan on α-dystroglycan is essential for its interaction with protein ligands in the extracellular matrix. Defective O-mannosylation leads to a group of muscular dystrophies, called dystroglycanopathies. Mutations in isoprenoid synthase domain containing (ISPD) represent the second most common cause of these disorders, however, its molecular function remains uncharacterized. The human ISPD (hISPD) crystal structure showed a canonical N-terminal cytidyltransferase domain linked to a C-terminal domain that is absent in cytidyltransferase homologs. Functional studies demonstrated cytosolic localization of hISPD, and cytidyltransferase activity toward pentose phosphates, including ribulose 5-phosphate, ribose 5-phosphate, and ribitol 5-phosphate. Identity of the CDP sugars was confirmed by liquid chromatography quadrupole time-of-flight mass spectrometry and two-dimensional nuclear magnetic resonance spectroscopy. Our combined results indicate that hISPD is a cytidyltransferase, suggesting the presence of a novel human nucleotide sugar essential for functional α-dystroglycan O-mannosylation in muscle and brain. Thereby, ISPD deficiency can be added to the growing list of tertiary dystroglycanopathies