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. MethodsWe 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. FindingsWe 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•4...

Cloacal exstrophy is an extremely rare congenital malformation resulting in an exstrophy of the urinary, intestinal, and genital organs and associated with anomalies of other organ systems. We present a complicated case of cloacal exstrophy and the recent progress in the management of this probably most complicated anomaly in pediatric urology and surgery.

IntroductionPyloric duplication is an extremely rare gastrointestinal tract malformation in neonates. This is the first case report of pyloric duplication in our country (Lithuania).Case presentationWe report the case of a 2-day-old Lithuanian girl who suffered from pyloric duplication mimicking an alternative common bile duct cyst or other intra-abdominal organs cysts. A laparotomy was performed and the cystic formation of the pyloric area was successfully resected. The postoperative course was uneventful.ConclusionsThere are only a few reports describing abdominal masses caused by pyloric duplication mimicking common bile duct cyst or other intra-abdominal organs cysts. Therefore thorough clinical and instrumental examination is needed to determine the most accurate diagnosis that allows one to choose the right treatment.

No abstract

TikslasIšanalizuoti stemplės plastikos modifikuotu Collis-Nissen metodu efektyvumą gydant vaikus dėl stemplės atrezijos esant la bai ilgam tarpui tarp stemplės galų. Medžiaga ir metodai 1996-2016 metais buvo gydytas 151 ligonis dėl stemplės atrezijos, iš jų 23 pacientams buvo ilgas tarpas tarp stemplės galų. Trims ligoniams buvo atlikta Collis-Nissen vėlyvoji stemplės plastika. Atokieji rezultatai analizuoti iš ambulatorinių kortelių. Rezultatai Trims berniukams, gimusiems 35 sav., 35 sav. ir 37 sav., per pirmąsias gyvenimo valandas buvo diagnozuota B, C ir C tipo stemplės atrezija. Du pacientai buvo operuoti pirmą gyvenimo parą, trečiasis -antrą. Visiems ligoniams buvo likviduotos tracheoezofaginės fistulės, atliktos gastrostomijos, vienam -ir sigmostomija. Nustatyti labai ilgi tarpai (7 cm, 4 cm ir 6 cm) tarp stemplės galų. Stemplės auginimas vyko iki 7 mėn., 7 mėn. ir 4 mėn., kol buvo atlikta Collis-Nissen gastroplastika ir fundo plikacija. Visi pacientai patyrė ankstyvų ir vėlyvų komplikacijų: pasireiškė anastomozių nesandarumas, mediastinitas, sepsis, randinė stemplės stenozė, tačiau atlikta remediastinotomija, tarpuplaučio drenavimas, stemplės bužavimas buvo sėkmingi. Visi pacientai gana greitai pradėti maitinti pro burną. Ligonių ambulatorinių kortelių įrašuose matyti, jog skundų pacientai neturi, maitinasi įprastai, apetitas geras. Išvada Collis-Nissen stemplės plastika -gera alternatyva kitoms rekonstrukcinėms stemplės operacijoms esant labai ilgam tarpui tarp stemplės galų. Reikšminiai žodžiai: stemplės atrezija, labai ilgas tarpas tarp stemplės galų, Nissen fundoplikacija, Collis gastroplastika