BackgroundCongenital esophageal stenosis (CES) is a rare condition frequently associated with esophageal atresia (EA). There are limited data from small series about the presentation, treatment, and outcomes of CES.MethodsMedical records of all patients with CES included in the French Network on Esophageal Malformations and Congenital Diseases were reviewed retrospectively with regard to diagnosis, treatment, and outcome.ResultsOver 18 years, 61 patients (30 boys) had CES, and 29 (47%) of these patients also had EA. The mean age at diagnosis was 24 months (1 day to 14 years) and was younger in patients with CES and EA than in those with isolated CES (7 vs. 126 months, p < 0.05). Twenty-one of the 61 patients with CES had no clinical symptoms: in three patients, the findings were incidental, and in 18 of the 29 patients with associated EA, CES was diagnosed at the time of surgical repair of EA or during a postoperative systematic esophageal barium study. In the 40 other patients, at diagnosis, 50% presented with dysphasia, 40% with vomiting, 50% with food impaction, and 42% with respiratory symptoms. Diagnosis of CES was confirmed by esophageal barium study (56/61) and/or esophageal endoscopy (50/61). Sixteen patients had tracheobronchial remnants (TBR), 40 had fibromuscular stenosis (FMS), and five had membrane stenosis (MS). Thirty-four patients (56%) were treated by dilation only (13/34 remained asymptomatic at follow-up); 15 patients were treated by dilation but required later surgery because of failure (4/15 remained asymptomatic at follow-up); and nine patients had a primary surgical intervention (4/9 were asymptomatic at follow-up). Dilation was complicated by esophageal perforation in two patients (3.4%). At follow-up, dysphagia remained in 36% (21/58) of patients, but the incidence did not differ between the EA and the isolated CS groups (10/29 vs. 7/32, p = 0.27).ConclusionsCS diagnosis can be delayed when associated with EA. Dilation may be effective for treating patients with FMS and MS, but surgical repair is often required for those with TBR. Our results show clearly that, regardless of the therapeutic option, dysphagia occurs frequently, and patients with CES should be followed over the long term.

We are unaware of any other anatomical study which deals with normal variations in the relationship between the superior mesenteric artery (SMA) and the superior mesenteric vein (SMV). In our investigation in the majority of cases, the SMV lies to the right of the SMA. It has been suggested that ultrasonographic demonstration of a left sided SMV, is characteristic of midgut malrotation so a prospective study was undertaken in order to try to correlate the position of the mesenteric vessels on Ultrasound with the radiological diagnosis of midgut malrotation on upper GI series. 427 children (95% infants) who were planned for upper GI investigation, have had color Doppler U.S. of the mesenteric vessels before the barium study. In 67 cases, the mesenteric vessels were obscured by intestinal gas. Among them, there were 2 cases of malrotation. Three different positions of the mesenteric vessels were found in the 360 remaining cases. 1) A left sided SMV was found in 4% of the cases, all had midgut malrotation on X-rays and at surgery. 2) A mesenteric vein anterior to the SMA was found in 5% of the cases, 28% had midgut malrotation. 3) A normal right sided SMV was found in 91% of the cases. However 3% of these had midgut malrotation. There are anatomical variations in the position of the mesenteric vessels and a normal relationship between SMA and SMV does not exclude the possibility of intestinal malrotation. Nevertheless, ultrasound remains a useful noninvasive screening technique for the diagnosis of midgut malrotation.

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...

Nonparasitic splenic cysts are uncommon lesions, for which partial splenectomy offers a definitive treatment while preserving splenic function. We report the use of radiofrequency energy for laparoscopic partial splenectomy to treat two children with nonparasitic splenic cysts by creating a 1-cm thick coagulated parenchyma resection plane, allowing bloodless splenic transection. This approach spares the need for blood transfusions, reduces hospital stay, and offers great promise for partial splenectomy in children.

isolated intestinal perforation may be a complication of the preventive treatment of chronic lung disease with high-dose corticosteroids in very low birth weight infants. Conversely, corticosteroids may reduce the clinical signs and the multiple organ dysfunction associated with an abdominal drama, explaining the very good surgical prognosis of these newborns. Attention must be paid to an insidious pneumoperitoneum.