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

Our Experıence Of Ureteropelvıc Junctıon Obstructıon In Pedıatrıc Patıents

GİRİŞ VE AMAÇ Bu çalışma ile pediatrik üreteropelvik bileşke darlığındaki deneyimlerimizi retrospektif olarak değerlendirmeyi amaçladık. YÖNTEM Kliniğimizde 2006-2019 yılları arasında üreteropelvik bileşke darlığı (ÜPBD) nedeni ile (biri robotik) ameliyat edilen 64 hastanın verilerini retrospektif olarak inceledik. Vakalar yaş, cinsiyet, antenatal öykü, hidronefroz, ameliyat öncesi ve sonrası durumu, diferansiye böbrek fonksiyonu (DRF), izotopun böbrekten temizlenme yarı ömrü (T1/2), histopatolojik sonuçları, üriner sistem enfeksiyonu ve reoperasyon açısından değerlendirildi. Gruplar arasındaki farkları test etmek için Anova ve Ki-Kare testleri kullanıldı. İstatistiksel olarak p<0,05 altındaki değerler anlamlı kabul edildi. BULGULAR Hastaların ortalama operasyon yaşı 46,87 ay idi ve bunların 47’si (%73,4) erkek hastalardan oluşuyordu. ÜPBD %39,1 (n=25) sağda, %56,3 (n=36) solda ve %4,7 (n=3) bilateral olduğu görüldü. Çalışmadakilerin %68,8’inde (n=44) antenatal hidronefroz tanısı mevcuttu. Hastaların sadece %6,3’inde (n=4) vezikoüreteral reflü (VUR) olduğu gösterildi. Ameliyat öncesi ve sonrası ortalama DRF değerleri sırasıyla %49,7 (%21-78) ve %49,2 (%20-56) idi. Bütün hastaların T1/2 değerleri 20 dakikadan daha uzundu. Ortalama renal pelvis AP çapı 21.58 mm (10-62 mm) idi. Eksize edilen patolojik spesmenin ortalama uzunluğu 10,26 mm (3-40 mm) ve hipertrofinin daha baskın olduğu görüldü. Ureteri çaprazlayarak hidronefroza neden II olan aberan damarın hastaların %17,18’inde (n = 11) olduğu ortaya çıkarıldı. Kız çocuklarında, sol tarafta daha fazla görülmesi ve operasyon yaşının büyük olması istatistiksel olarak anlamlı bulundu. Hidronefrozun aberan damar sebebi ile oluşmayanlarda oluşanlara göre daha fazla olması istatistiksel olarak anlamlı bulundu. Diğer parametreler arasında anlamlı fark yoktu. Postoperatif 4 hastada nüks görüldü (%6,2). SONUÇ Antenatal tanılı ÜPBD hastalarının etiyolojisinde internal nedenler önemli bir rol oynamaktadır ve daha erken yaşlarda operasyona ihtiyaç duyulmaktadır. Bununla birlikte, daha geç yaşlarda teşhis ve ameliyat edilen hastaların etiyolojisinde aberan damar daha yaygındır.BACKGROUND Herein we report our clinical data and treatment outcomes of pediatric ureteropelvic junction obstruction (UPJO) patients. METHOD We retrospectively reviewed the data of 64 patients who underwent open dismembered pyeloplasty (except one robotic) because of UPJO between 2006 and 2019 at our clinic. Cases were evaluated regarding age, gender, prenatal history, hydronephrosis, pre- and postoperative courses, differential renal functions (DRFs), half-time tracer clearance (½TC), histopathologic results, urinary infection, and reoperations. Anova and Chi-Square tests were used to test the differences between the groups, where p < 0.05 was taken as statistically significant. RESULTS The mean operation age of patients was 46.87 months. forty-seven of these children (73.4%) were male. UPJO was found the right side in 39.1%(n=25), left side in 56.3 %(n=36), and bilaterally in 4.7% (n=3). Antenatal hydronephrosis was detected in 68.8% of patients (n=44). Vesicoureteral reflux demonstrated only 6.3% (n=4). Pre and postoperatively, mean DRFs were 49.7% (21–78%) and 49.2% (20–56%), respectively. All patient t1/2 was higher than 20 minutes. The mean diameter of AP was 21.58 mm (10–62 mm). The mean length of excised specimen was 10,26 mm (3-40 mm), muscular hypertrophy was seen dominantly. Crossing vessel (CV) was IV found in 17.18% (n=11). It was found statistically significant on the left side, higher operation age and female patients. Hydronephrosis was found statistically significant compared to the non-CV patients. There was no difference between the other parameters. Postoperative recurrence was seen in 4 patients (6.2%). CONCLUSION Internal causes play an important role in the etiology of UPJO patients with antenatal diagnosis, and the operation is needed at an earlier age. However, CVs are more common in the etiology of patients who are diagnosed and operated on later

Our experience of operated pediatric ureteropelvic junction obstruction patients

Aims: Ureteropelvic junction obstruction (UPJO) may originate from extrinsic or intrinsic causes in children. The aim of this study is to present preoperative and postoperative data of our patients operated for UPJO. Methods: A total of 64 patients who underwent open pyeloplasty were investigated retrospectively. They were evaluated in terms of demographically, clinics, hydronephrosis, differential renal functions (DRFs), half-time tracer clearance (½TC), and histopathologic results. Patients’ numerical results were stated as mean ± standard deviation (SD). Results: Male gender was more prevalent ( n = 47, 73.4%) and mean age at surgery was 46.87 months. UPJO was located at the left side in 56.3% ( n = 36), and at the right side in 39.1% ( n = 25) of patients. It was bilateral in 4.7% ( n = 3). Hydronephrosis was found antenatally in 68.8% ( n = 44) of patients. The mean preoperative DRF was 49.7% (21–78%) and mean postoperative DRF was 49.2% (20–56%). Mean renal scintigraphic t1/2 was &gt;20 min for all patients. The mean AP diameter was 21.58 mm (10–62 mm). Muscular hypertrophy was the most common pathological finding, mean length of excised segment was 10.26 mm (3–40 mm). Crossing vessel (CV) was detected in 17.18% ( n = 11). The CV was statistically associated with increased age of operation, left side, and female gender. Statistically significant hydronephrosis was found in non-CV patients. Re-operation was required in seven patients (7.8%). Conclusions: Intrinsic pathologies are more seen in the etiology of UPJO patients with antenatal diagnosis and this group needs operation at an earlier age. However, CV is found more commonly in patients who are diagnosed and operated at older ages. </jats:sec