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

Reducing the environmental impact of surgery on a global scale: systematic review and co-prioritization with healthcare workers in 132 countries

Abstract Background Healthcare cannot achieve net-zero carbon without addressing operating theatres. The aim of this study was to prioritize feasible interventions to reduce the environmental impact of operating theatres. Methods This study adopted a four-phase Delphi consensus co-prioritization methodology. In phase 1, a systematic review of published interventions and global consultation of perioperative healthcare professionals were used to longlist interventions. In phase 2, iterative thematic analysis consolidated comparable interventions into a shortlist. In phase 3, the shortlist was co-prioritized based on patient and clinician views on acceptability, feasibility, and safety. In phase 4, ranked lists of interventions were presented by their relevance to high-income countries and low–middle-income countries. Results In phase 1, 43 interventions were identified, which had low uptake in practice according to 3042 professionals globally. In phase 2, a shortlist of 15 intervention domains was generated. In phase 3, interventions were deemed acceptable for more than 90 per cent of patients except for reducing general anaesthesia (84 per cent) and re-sterilization of ‘single-use’ consumables (86 per cent). In phase 4, the top three shortlisted interventions for high-income countries were: introducing recycling; reducing use of anaesthetic gases; and appropriate clinical waste processing. In phase 4, the top three shortlisted interventions for low–middle-income countries were: introducing reusable surgical devices; reducing use of consumables; and reducing the use of general anaesthesia. Conclusion This is a step toward environmentally sustainable operating environments with actionable interventions applicable to both high– and low–middle–income countries

Early experience with laparoscopic management of nonpalpable undescended testes

Background: Nonpalpable undescended testes (NPT) constitute 20%–30% of undescended testes, and its management has been a challenge both in diagnosis and treatment. Worldwide, laparoscopy is the current gold standard of management. In Nigeria, the management of NPT has largely been by open surgery with consequent high morbidity. In Nigeria, the trend is changing from a largely open management with its attendant high morbidity, to laparoscopic management which is the current worldwide gold standard of care. Aim: This study aims to classify the laparoscopic features of NPT and determine the outcome of managed cases in our center. Methodology: Prospective data were collected from consecutive patients who had laparoscopy for NPT at the Paediatric Surgical Unit of Nnamdi Azikiwe University Teaching Hospital, Nnewi, Nigeria from June 2014 to July 2016. Results: A total of 15 patients with 23 testes were treated. There were eight patients with bilateral NPT; four had left and the remaining three right NPT. The age ranged from 1.2 to 29 years with a median of 5 years. Eleven out of the 22 internal inguinal rings were open. The position of the testes was canalicular (2), peeping (2), low abdominal (6), high abdominal (6), blind-ended vas (1), absent vas and vessels (5). No further intervention was needed for the six agenetic/atrophic testes. Standard open orchiopexy was done for the two canalicular testes. Eight testes were brought down by one stage laparoscopic orchiopexy while four were brought down by staged laparoscopic Fowler-Stephens procedure. Laparoscopic orchiectomy was done in two patients (a grossly dysmorphic testes [nubbin] and a high abdominal testis in a 29-year-old). Orchiopexy was successful in 11 out of 15 fixed testes. Of the unsuccessful ones, three testes were atrophic (volume less than what it was initially) while two were high scrotal (one testes has both complications). There was no conversion to open abdominal surgery. All patients were discharged within 24 h of surgery. Conclusion: Laparoscopy provides for a better management of NPT by combining diagnosis and intervention in the same sitting with a good success rate and minimal postoperative morbidity

Parental circumcision preferences and early outcome of plastibell circumcision in a Nigerian tertiary hospital

Background: Parents are central in decisions and choices concerning circumcision of their male children and plastibell circumcision is a widely practiced technique. This study determined parental preferences for male neonatal and infant circumcisions and evaluate the early outcomes of plastibell circumcisions in a tertiary centre. Patients and Methods: This is a prospective study on consecutive male neonates and infants who were brought for circumcisions at Nnamdi Azikiwe University Teaching Hospital Nnewi, South-East Nigeria and their respective parents between January 2012 and December 2012. Data on demography, parental choices and early outcome of plastibell circumcision were obtained and analysed. Results: A total of 337 requests for circumcisions were made for boys with age range of 2-140 days. Culture and religion were the most common reasons for circumcision requests in 200 (59.3%) and 122 (36.2%), respectively, other reasons were medical, cosmesis, to reduce promiscuity and just to follow the norm. Most parents, 249 (73.9%) preferred the procedure to be performed on the 8 th day and 88.7% would like the doctors to perform the procedure while 84.6% preferred the plastibell method. Among those who had circumcision, 114 complied with follow-up schedules and there were complications in 22 (19.3%) patients. Parents assessed the early outcome as excellent, very good, good and poor in 30.7%, 45.6%, 18.4% and 5.3% of the patients, respectively. Conclusion: Parents request for male circumcision in our environment is largely for cultural and religious reasons; and prefer the procedure to be performed by a physician. Plastibell method is well known and preferred and its outcome is acceptable by most parents

Post-circumcision urethrocutaneous fistulae: presentations, repairs and outcomes in a tertiary centre

Background: Circumcision is regarded as the most common surgical procedure world over. It is also perceived to be a simple and safe procedure; however, it could be fraught with major urological complications such as urethrocutaneous fistula (UCF). Repair of these fistulae poses a great reconstructive challenge to the paediatric surgeon/urologist with varied outcomes. Aim: We seek to review the presentations, repair and outcome of post-circumcision UCFs managed in a tertiary centre. Patients and Methods: This is a retrospective review of all the consecutive cases of repaired post-circumcision UCFs in Nnamdi Azikiwe University Teaching Hospital, Nnewi, South-East Nigeria, over a period of 9 years (January 2012–December 2020). Data on demography, size of fistula, type of repair and outcome were retrieved from the Records Department and patients' case notes and were analysed using SPSS (version 22, Chicago, Illinois). Results: A total of 22 boys had post-circumcision UCF repair within the period, and they were aged between 2 weeks and 108 months with a median age of 4 months at presentation and aged between 8 months and 144 months with a median age of 24 months at the time of surgery. Circumcisions were by freehand technique in 21 (95.5%). Nurses performed most of the circumcisions in 19 (86.4%), and most of the circumcisions were performed in private hospitals 7 (31.8%), maternity homes 3 (13.6%) and general hospitals in 10 (45.5%) each, respectively. Most 17 (77.3%) fistulae were coronal, and the size of defect ranged from 1 mm to 10 mm. The most commonly employed technique of repair was simple closure in 16 (72.2%). Meatal stenosis and re-fistulation occurred in 2 (9.1%) and 4 (18.2%), respectively. Only two (12.5%) required reoperation. There was no relationship between the size of defect and re-fistulation, P = 0.377. Conclusion: Majority of the Post-circumcision urethrocutaneous fistulae were from free hand technique of circumcisions . These were performed mostly by nurses in general, private hospitals and maternity homes. Hence, there is a need to ramp up training of providers of neonatal circumcision in our environment. Most UCF in children could be repaired with simple closure technique reinforced with dartos flap

Comparison of trans-perineal ultrasound-guided pressure augmented saline colostomy distension study and conventional contrast radiographic colostography in children with anorectal malformation

Background: In children with high and intermediate anorectal malformation, distal colostography is an important investigation done to determine the relationship between the position of the rectal pouch and the probable site of the neo-anus as well as the presence or absence of a fistula. Conventionally, this is done using contrast with fluoroscopy or still X-ray imaging. This, however, has the challenges of irradiation, availability and affordability, especially in developing countries. This study compared the accuracy of trans-perineal ultrasound-guided pressure augmented saline colostomy distension study (SCDS) with conventional contrast distal colostography (CCDC) in the determination of the precise location of the distal rectal pouch and in detecting the presence and site of fistulous communication between the rectum and the urogenital tract was studied. Materials and Methods: Trans-perineal ultrasound-guided pressure augmented SCDS, CCDC and intra-operative measurements were done sequentially for qualified infants with anorectal malformation and colostomy. Pouch skin distance and presence or absence of recto urinary or genital fistula was measured prospectively in each case. Statistical significance was inferred at P-value of 0.01. On its ability to detect presence or absence of a fistula: SCDS had a sensitivity of 50.0%, specificity of 100.0%, accuracy of 69.2%, negative predictive value of fistulas of 55.6% and a positive predictive value of fistulas of 100.0%. Conclusion: Ultrasound-guided pressure augmented SCDS can safely and reliably be used to assess the distal colonic anatomy and the presence of fistula in infants with Anorectal malformation who are on colostomy

Classical bladder exstrophy in an adolescent: A case report on management, challenges and outcome

Classical bladder exstrophy is a congenital anomaly whose management and outcome has advanced over years. Management and outcome are better when management starts at the newborn period. This was the management of a neglected bladder exstrophy in a male presenting at 16 years of age. We report our challenges, management and outcome to highlight the rarity of this presentation, and the adaptation to the usual protocol of care. The patient presented at 16 years of age with classic bladder exstrophy. The bladder plate was contracted and had cystitis. The patient had a modification of complete primary repair of exstrophy (CPRE) with bilateral pelvic osteotomy stabilised with a 7-hole plate and 4 screws, then bladder neck reconstruction + bladder augmentation + cross-trigonal neocystoureterostomy in a 12-h procedure. He had surgical site infection, superficial wound breakdown and vesicocutaneous fistula that all healed with dressing and prolonged suprapubic cystostomy drainage. He achieved some degree of urinary continence and ability to void, though he still has stress incontinence and frequency at 6 months of follow-up. He has a micturition interval of 60–120 min, and is expected to improve. Presentation and repair of classic bladder exstrophy in the adolescent is very rare in the literature and therefore no known standard of care. This report adds to the body of knowledge. Again, this experience lends credence to the proponents of CPRE in reducing the number of procedures required to treat exstrophy

A case of retroperitoneal malignant triton tumor in a Nigerian boy

Malignant peripheral nerve sheath tumor is a rare tumor occurring in 5%–10% of all malignant soft tissues sarcomas and triton tumor arising from neurofibromatosis type 1 (NF-1) is even rarer with associated high rate of mortality. No case of triton tumor has been reported in Nigeria to the best of our knowledge. We seek to report a case of lately detected retroperitoneal triton tumor presenting in a 12-year-old Nigerian child who was brought with bilateral lower limb weaknesses, weight loss, and a right lumbar mass. There were multiple café au lait spots on the body. Abdominal computerized tomographic scan revealed a huge right retroperitoneal mass crossing the midline, compressing adjacent structures with multilevel intraspinal extensions. Core needle biopsy performed and both histology and immunohistochemical studies confirmed the diagnosis, but patient demised in the course of care. The aim is to heighten suspicion of this extremely very rare malignant tumor in children with NF-1

Impact of the COVID-19 pandemic on patients with paediatric cancer in low-income, middle-income and high-income countries: a multicentre, international, observational cohort study

OBJECTIVES: Paediatric cancer is a leading cause of death for children. Children in low-income and middle-income countries (LMICs) were four times more likely to die than children in high-income countries (HICs). This study aimed to test the hypothesis that the COVID-19 pandemic had affected the delivery of healthcare services worldwide, and exacerbated the disparity in paediatric cancer outcomes between LMICs and HICs. DESIGN: A multicentre, international, collaborative cohort study. SETTING: 91 hospitals and cancer centres in 39 countries providing cancer treatment to paediatric patients between March and December 2020. PARTICIPANTS: Patients were included if they were under the age of 18 years, and newly diagnosed with or undergoing active cancer treatment for Acute lymphoblastic leukaemia, non-Hodgkin's lymphoma, Hodgkin lymphoma, Wilms' tumour, sarcoma, retinoblastoma, gliomas, medulloblastomas or neuroblastomas, in keeping with the WHO Global Initiative for Childhood Cancer. MAIN OUTCOME MEASURE: All-cause mortality at 30 days and 90 days. RESULTS: 1660 patients were recruited. 219 children had changes to their treatment due to the pandemic. Patients in LMICs were primarily affected (n=182/219, 83.1%). Relative to patients with paediatric cancer in HICs, patients with paediatric cancer in LMICs had 12.1 (95% CI 2.93 to 50.3) and 7.9 (95% CI 3.2 to 19.7) times the odds of death at 30 days and 90 days, respectively, after presentation during the COVID-19 pandemic (p<0.001). After adjusting for confounders, patients with paediatric cancer in LMICs had 15.6 (95% CI 3.7 to 65.8) times the odds of death at 30 days (p<0.001). CONCLUSIONS: The COVID-19 pandemic has affected paediatric oncology service provision. It has disproportionately affected patients in LMICs, highlighting and compounding existing disparities in healthcare systems globally that need addressing urgently. However, many patients with paediatric cancer continued to receive their normal standard of care. This speaks to the adaptability and resilience of healthcare systems and healthcare workers globally

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