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

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 middle-income 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 low-income 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 low-income, 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

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

Sinonasal Chondrosarcoma, an Unusual Location

Introduction: Chondrosarcoma is a tumour with a cartilaginous matrix frequently encountered in long bones and the pelvis with rare sinonasal location.Patient and methods: We report the case of a 25-year-old patient who was referred to us for an extension work-up for sinonasal chondrosarcoma confirmed by anatomopathological examination.Results: Facial magnetic resonance imaging (MRI) confirmed by a CT scan showed an osteolytic tumour process of the hard palate and walls of the left maxillary sinus that was locally advanced.Conclusion: The sinonasal location of a chondrosarcoma is rare. Characterized by cross-sectional imaging, confirmation was provided by histological assessment

Une myelinolyse centropontine sans rapport avec la natrémie: role de l'hyperglycemie? A propos d'un cas et revue de la litterature

La myélinolyse centropontine (MCP) correspond à un état neurologique sévère.La physiopathologie exacte de la constitution des syndromes de démyélinisation osmotique est encore mal connue.Chez l'homme, la MCP survient généralement lorsque la correction du taux de sodium sérique dépasse 12 mEq/l/jour. L'IRM est l'imagerie de choix pour le diagnostic. Nous rapportons le cas d'un patient admis au service de neurologie pour tableau de paraplégie d'installation progressive associé à des troubles de déglutition et dysarthrie avec à l'IRM des hypersignaux T2 et FLAIR et diffusion centropontiques symétriques s'étendant au niveau des pédoncules cérébelleux supérieurs faisant évoquer une MCP , sans anomalie du bilan sodique mais plutôt un déséquilibre glycémique chronique qui aurait eu les mêmes effets qu'une perfusion rapide de sérum salé hypertonique sur hyponatrémie

Unusual Location of a Recurrent Epidermoid Cyst

Introduction: Epidermoid cysts of the temporal bone are rare, benign and slow-growing lesions. Patient and Methods: We report the case of a 69-year-old female patient followed up for a symptomatic intradiploic epidermoid cyst of the temporal scale and left mastoid region, which had been operated on but recurred.Results: MRI demonstrated a well-limited lesion seen as a hyposignal on T1-weighted images, hypersignal on T2-weighted images, on FLAIR and on diffusion-weighted images not enhanced by gadolinium. The tumour was compressive, and bone lysis was seen on CT.Conclusion: Epidermoid cysts of the temporal bone are rare, benign lesions whose diagnosis is based on fluid signals seen on MRI but absent on FLAIR sequences

Non-traumatic cauda equina syndrome in adults. What etiology? About 76 cases

Cauda equina syndrome is a rare neurological disease caused by compression of the cauda equina. The ponytail consists of the spinal nerves L2-L5, S1-S5 and the coccygeal nerve. We carried out a retrospective study over a period of two years (24 months), ranging from January 01, 2020, to December 31, 2021, from the files of patients referred for lumbosacral magnetic resonance imaging (MRI) or CT scan exploration in the context of low back pain or sciatica resistant to disabling medical treatment, a neurological deficit of the lower limbs associated or not with the bladder and/or rectal sphincter disorders, without any notion of trauma, with as judgment criterion the demonstration of an anomaly responsible for compression of the roots of the ponytail. We collected seventy-six (76) patients, with an average age of 53.5 years old and at extremes ages of 15 years and 89 years. We noted a female predominance with a sex ratio of 1.5 in favour of the female sex. Most of the patients were explored with MRI in 89 % of cases. Our patient history was dominated by cancer pathologies, with breast cancer in 24% of cases and prostatic cancer in 18% of cases. Sphincteric disorders mainly represented the symptomatology presented by the patients in 47% of cases and lumboscialitica in 28% of cases. The conflicting disc herniation dominated the etiologies in 36 % of cases, followed by secondary vertebral neoplastic lesions in 20% of cases. MRI and CT scans are important radiological modalities in characterizing lesions responsible for cauda equina syndrome in adults. The CT scan is effective in the study of bone and the MRI in the study of nerve roots, intervertebral discs and paravertebral soft tissues