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

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

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

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

Typhoid intestinal perforation: Analysis of the outcome of surgical treatment in Kano, Nigeria

Background: Intestinal perforation is a serious complication of typhoid fever with high case fatality rates in developing countries. This study aims to determine the factors associated with an adverse clinical outcome among patients managed for typhoid intestinal perforation (TIP) in our hospital. Materials and Methods: We retrospectively reviewed the records of all patients presenting to our general surgery unit with TIP between January 2012 and December 2015. The patients were categorized based on postoperative outcome status and the patient-related variables were compared and analyzed for determinants of outcome, using the Chi-square test. A statistical significance was assigned to a P 1000 ml was significantly associated with having a postoperative fecal fistula (P = 0.011) and postoperative mortality (P = 0.002). A number of intestinal perforations were not significantly associated with an adverse outcome (P > 0.05). Conclusion: Postoperative fecal fistula adversely affected the outcome of the patients in our series. Multiple intestinal perforations did not seem to affect the outcome in our patients

Open inguinal herniotomy: Analysis of variations

Background: Repair of congenital groin hernia/hydrocele is the most common surgical procedure performed by paediatric surgeons. There is dearth of literature comparing the outcomes of open herniotomy in children using various surgical approaches. This study was aimed at evaluating the efficacy of open herniotomy by comparing external ring incision, hernial sac twisting and whether or not double ligation has benefit over a single suture application. Materials and Methods: A multi-centre prospective randomised clinical trial was conducted with a total of 428 patients having congenital inguinal hernia and/or hydrocele. Patients were randomly assigned into four groups: RO (had external ring opened, hernial sac twisted and doubly ligated), ST (had hernial sac twisted and doubly ligated without opening the ring), DL (had double ligation of hernial sac without ring opening nor twisted) while SL (had single ligation of hernial sac with neither ring opening nor sac twisting). Results: A total of 458 repairs were done. Patients′ age ranged from 0.25 years (3 months) to 21 years in group RO with mean of 4.87 × 4.07 (median, 4), 0.069 years (24 days) to 17 years in group ST with mean of 4.23 × 4.03 (median, 3), 0.5 years (6 months) to 16 years in group DL with mean of 4.59 × 3.87 (median, 4) and 1 year to 19 years in group SL with mean of 5.00 × 4.19 (median, 4). Operation time per repair was 26.50 × 5.46 min, range 16-40 min (median, 27 min) in group RO, 22.18 × 5.34 min, range 12-39 min (median, 21 min) in group ST while 17.98 × 3.40 min with range of 12-39 min (median, 17 min) in group DL and 15.27 × 4.18 min, range 7-40 min (median, 15 min) in group SL P < 0.0001. The mean paracetamol dose/patient was 3.96 × 1.43, 2.94 × 0.81, 2.18 × 0.69, 1.87 × 0.78 in group RO, ST, DL and SL, respectively, P < 0.0001. Conclusion: Congenital inguinal hernia repair with opening of the external ring, hernia sac twisting and double ligation of the processus vaginalis confers no advantage

Adaptation of the Wound Healing Questionnaire universal-reporter outcome measure for use in global surgery trials (TALON-1 study): mixed-methods study and Rasch analysis

BackgroundThe Bluebelle Wound Healing Questionnaire (WHQ) is a universal-reporter outcome measure developed in the UK for remote detection of surgical-site infection after abdominal surgery. This study aimed to explore cross-cultural equivalence, acceptability, and content validity of the WHQ for use across low- and middle-income countries, and to make recommendations for its adaptation.MethodsThis was a mixed-methods study within a trial (SWAT) embedded in an international randomized trial, conducted according to best practice guidelines, and co-produced with community and patient partners (TALON-1). Structured interviews and focus groups were used to gather data regarding cross-cultural, cross-contextual equivalence of the individual items and scale, and conduct a translatability assessment. Translation was completed into five languages in accordance with Mapi recommendations. Next, data from a prospective cohort (SWAT) were interpreted using Rasch analysis to explore scaling and measurement properties of the WHQ. Finally, qualitative and quantitative data were triangulated using a modified, exploratory, instrumental design model.ResultsIn the qualitative phase, 10 structured interviews and six focus groups took place with a total of 47 investigators across six countries. Themes related to comprehension, response mapping, retrieval, and judgement were identified with rich cross-cultural insights. In the quantitative phase, an exploratory Rasch model was fitted to data from 537 patients (369 excluding extremes). Owing to the number of extreme (floor) values, the overall level of power was low. The single WHQ scale satisfied tests of unidimensionality, indicating validity of the ordinal total WHQ score. There was significant overall model misfit of five items (5, 9, 14, 15, 16) and local dependency in 11 item pairs. The person separation index was estimated as 0.48 suggesting weak discrimination between classes, whereas Cronbach's α was high at 0.86. Triangulation of qualitative data with the Rasch analysis supported recommendations for cross-cultural adaptation of the WHQ items 1 (redness), 3 (clear fluid), 7 (deep wound opening), 10 (pain), 11 (fever), 15 (antibiotics), 16 (debridement), 18 (drainage), and 19 (reoperation). Changes to three item response categories (1, not at all; 2, a little; 3, a lot) were adopted for symptom items 1 to 10, and two categories (0, no; 1, yes) for item 11 (fever).ConclusionThis study made recommendations for cross-cultural adaptation of the WHQ for use in global surgical research and practice, using co-produced mixed-methods data from three continents. Translations are now available for implementation into remote wound assessment pathways