Background and ObjectivesPatients with Hirschsprung's disease are at risk of developing Hirschsprung-associated enterocolitis, especially in the first 2 years of life. The pathophysiology of this inflammatory disease remains unclear, and intestinal dysbiosis has been proposed in the last decade. The primary objective of this study was to evaluate in a large cohort if Hirschsprung-associated enterocolitis was associated with alterations of fecal bacterial composition compared with HD without enterocolitis in different age groups.MethodsWe analyzed the fecal microbiota structure of 103 Hirschsprung patients from 3 months to 16 years of age, all of whom had completed definitive surgery for rectosigmoid Hirschsprung. 16S rRNA gene sequencing allowed us to compare the microbiota composition between Hirschsprung's disease patients with (HAEC group) or without enterocolitis (HD group) in different age groups (0–2, 2–6, 6–12, and 12–16 years).ResultsRichness and diversity increased with age group but did not differ between HD and HAEC patients, irrespective of the age group. Relative abundance of Actinobacteria was lower in HAEC than in HD patients under 2 years of age (−66%, P = 0.045). Multivariate analysis by linear models (MaAsLin) considering sex, medications, birth mode, breast-feeding, and the Bristol stool scale, as well as surgery parameters, highlighted Flavonifractor plautii and Eggerthella lenta, as well as Ruminococcus gnavus group, as positively associated with Hirschsprung-associated enterocolitis in the 0–2 years age group.ConclusionHirschsprung-associated enterocolitis was associated with features of intestinal dysbiosis in infants (0–2 years) but not in older patients. This could explain the highest rate of enterocolitis in this age group.Clinical Trial Registrationhttps://clinicaltrials.gov/ct2/show/NCT02857205, MICROPRUNG, NCT02857205, 02/08/2016.

Objectives: The present study aimed to assess long-term functional outcomes of children with anorectal malformations (ARMs) across a network of expert centers in France. Methods: Retrospective cross-sectional study of patients ages 6-30 years that had been surgically treated for ARM. Patient and ARM characteristics (eg, level, surgical approach) and functional outcomes were assessed in the different age groups. Results: Among 367 patients, there were 155 females (42.2%) and 212 males (57.8%), 188 (51.2%) cases with, and 179 (48.8%) higher forms without, perineal fistula. Univariate and multivariate statistical analyses with logistic regression showed correlation between the level of the rectal blind pouch and voluntary bowel movements (odds ratio [OR] = 1.84 [1.31-2.57], P < 0.001), or soiling (OR = 1.72 [1.31-2.25], P < 0.001), which was also associated with the inability to discriminate between stool and gas (OR = 2.45 [1.28-4.67], P = 0.007) and the presence of constipation (OR = 2.97 [1.74-5.08], P < 0.001). Risk factors for constipation were sacral abnormalities [OR = 2.26 [1.23-4.25], P = 0.01) and surgical procedures without an abdominal approach (OR = 2.98 [1.29-6.87], P = 0.01). Only the holding of voluntary bowel movements and soiling rates improved with age. Conclusion: This cross-sectional study confirms a strong association between anatomical status and functional outcomes in patients surgically treated for ARM. It specifically highlights the need for long-term follow-up of all patients to help them with supportive care.

Objective To identify the risk factors for early mortality and morbidity in a population with distal esophageal atresia (EA)-tracheoesophageal fistula. Study design Cohort study from a national register. Main outcomes and measures included early mortality, hospital length of stay (LoS), need for nutritional support at 1 year of age as a proxy measure of morbidity, and complications during the first year of life. Results In total, 1008 patients with a lower esophageal fistula were included from January 1, 2008, to December 31, 2014. The survival rate at 3 months was 94.9%. The cumulative hospital LoS was 31.0 (17.0-64.0) days. Multivariate analysis showed that intrahospital mortality at 3 months was associated with low birth weight (OR 0.52, 95% CI [0.38-0.72], P <.001), associated cardiac abnormalities (OR 6.09 [1.96-18.89], P =.002), and prenatal diagnosis (OR 2.96 [1.08-8.08], P =.034). LoS was associated with low birth weight (-0.225 +/- 0.035, P <.001), associated malformations (0.082 +/- 0.118, P <.001), surgical difficulties (0.270 +/- 0.107, P <.001), and complications (0.535 +/- 0.099, P <.001) during the first year of life. Predictive factors for dependency on nutrition support at 1 year of age were complications before 1 year (OR 3.28 [1.23-8.76], P < .02) and initial hospital LoS (OR 1.96 [1.15-3.33], P <.01). Conclusions EA has a low rate of early mortality, but morbidity is high during the first year of life. Identifying factors associated with morbidity may help to improve neonatal care of this population.

Aim: Splenectomy, except for a traumatic purpose, is now performed through a laparoscopic approach. There are mainly two ways for laparoscopic total or partial splenectomies. For the classic anterior dissection of the splenic vessels, patient is placed in supine position and five ports are required to elevate the spleen and proceed to vessel divisions. With a lateral approach of the pedicle, patient is placed in lateral decubitus position and three ports are sufficient, because gravity help to provide traction on the splenic ligaments and to present hilar vessels and pancreas tail. The aim of our study was to compare surgical complications of those two approaches of laparoscopic splenectomy in children. Methods: We reviewed 84 medical records of patient operated on for hematological disease between January 1993 and December 2009. Results: There were 47 anterior and 37 lateral approaches. Sex, disease, median age, operative time, blood lost or hospital stay, and associated laparotomy were not different between the two groups. Operative complications included hemorrhage (5), bowel injury (1), diaphragmatic wound (1), pancreas tail section (1), and parietal hematoma (1) in the anterior group (9 cases) versus 1 hemorrhage in the lateral group (P<.02). There were five laparotomies owing to surgical complications in the anterior group, and none in the lateral group. Conclusion: Splenectomy through laparoscopic approach is an effective technique. Lateral dissection of the vessels provides less operative complications in children.