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

Necrotizing Fasciitis of the Lower Limb Following Rat Bites

Necrotizing fasciitis is a rare life-threatening condition characterized by rapidly spreading infection in the subcutaneous tissues. Most of the time, the infection is precipitated by an event that produces a break in the epidermis. The authors report the case of a 55-year-old farmer who developed a necrotizing fasciitis of the left lower limb following a rat bite. The patient had serial debridement of his wound and broad-spectrum antibiotics and subsequent partial thickness skin grafting. His total duration of admission was 93 days. Early diagnosis, an aggressive wound debridement, and broad-spectrum antibiotics form the backbone of the successful management of this case. </jats:p

Parasitic twins: Report of two cases

Parasitic twin is a rare malformation, which involves two fetuses attached to each other, whereby one of the pair is viable and develops into a mature embryo (the host twin) called the autosite, while the second twin (parasite) is usually nonviable, but survives on the host twin; the site and extend of their attachment are varied and may be associated with other congenital malformations. Its exact cause is not completely understood. Though, it has been reported in many countries, there is dearth of information on its occurrence in Nigeria. Therefore, we report two cases of parasitic twins seen in our institution. The first case had an extra-limb, scrotal mass projecting from the anterior abdominal wall, which also had an anterior abdominal wall defect below the extra-limb, while the second case had a sacroccoygeal mass which was a duplicate gluteal mass with malformed lower limb, digit with a toe nail projecting from it and an atrophic scrotal skin attached. Both cases had chest, spinal X-rays and abdominal ultrasounds, which excluded any other malformation. They had successful surgical excision of parasites and are being followed-up in the surgical clinic both having appropriate developmental milestones

Barium stone formation in the vagina of a child with persistent cloaca following a distal colostogram

Persistent cloaca is a severe form of malformation of the anorectum. A distal colostogram is important in the planning of its treatment. We report herein a case of a girl who had barium stone formation within her vagina following a distal colostogram. Water soluble contrasts are recommended for this procedure.Key words: Barium stones, distal colostogram, persistent cloaca, water soluble contras

Major abdominal wall defects in the low- and middle-income setting: current status and priorities

Major congenital abdominal wall defects (gastroschisis and omphalocele) may account for up to 21% of emergency neonatal interventions in low- and middle-income countries. In many low- and middle-income countries, the reported mortality of these malformations is 30-100%, while in high-income countries, mortality in infants with major abdominal wall reaches less than 5%. This review highlights the challenges faced in the management of newborns with major congenital abdominal wall defects in the resource-limited setting. Current high-income country best practice is assessed and opportunities for appropriate priority setting and collaborations to improve outcomes are discussed

Major abdominal wall defects in the low- and middle-income setting: current status and priorities

AbstractMajor congenital abdominal wall defects (gastroschisis and omphalocele) may account for up to 21% of emergency neonatal interventions in low- and middle-income countries. In many low- and middle-income countries, the reported mortality of these malformations is 30–100%, while in high-income countries, mortality in infants with major abdominal wall reaches less than 5%. This review highlights the challenges faced in the management of newborns with major congenital abdominal wall defects in the resource-limited setting. Current high-income country best practice is assessed and opportunities for appropriate priority setting and collaborations to improve outcomes are discussed.</jats:p