Current Management of Congenital Pulmonary Airway Malformations: A "European Pediatric Surgeons' Association" Survey.

Aim:  To define current management of congenital pulmonary airway malformation (CPAM).

Methods:  A total of 181 European Pediatric Surgeons' Association members (91% senior) from 48 countries completed an online questionnaire.

Main Results:  Prenatal: 93% respondents work in centers with prenatal diagnosis facilities, and 27% in centers offering in utero surgery.

Difficulties, guidelines and review of developing an acute rejection model after rat intestinal transplantation.

Experimental small bowel transplantation (SBT) in rats has been proven to be a useful tool for the study of ischemia-reperfusion and immunological aspects related to solid organ transplantation. However, the model is not completely refined, specialized literature is scarce and complex technical details are typically omitted or confusing. Most studies related to acute rejection (AR) use the orthotopic standard, with small sample sizes due to its high mortality, whereas those studying chronic rejection (CR) use the heterotopic standard, which allows longer term survival but does not exactly reflect the human clinical scenario.

Congenital Portosystemic Shunts: Clinic Heterogeneity Requires an Individual Management of the Patient.

Introduction: Congenital portosystemic shunt (CPSS) is a rare entity without insufficiency in treatment issues. The aim of this article is to show our experience in the heterogeneity of this condition.

Material And Methods: A retrospective study of 25 CPSS in the period 1995 to 2014 was conducted.

Portal Cavernoma in the Era of Mesoportal Shunt (Rex) and Liver Transplant in Children.

Objective: The mesoportal shunt (MPS) and liver transplantation (LT) have changed the scenario of extrahepatic portal vein obstruction (EHPVO) since the MPS, the only "curative" technique, can now be offered in asymptomatic patients and also thrombotic complications of LT have increased the incidence of EHPVO.

Material And Methods: A retrospective study of patients undergoing surgery for EHPVO was conducted between 1990 and 2015. An analysis was done for the shunt permeability and clinical evolution over time.

Cephalic pancreaticoduodenectomy for bleeding duodenal arteriovenous malformation.

Introduction Treatment of recurrent severe gastrointestinal bleeding due to arteriovenous malformations may require complex resections. In some particular locations, extensive surgery is the only way out, as shown in this report. Case Report A 2.

Tertiary surgery for complicated repair of esophageal atresia.

Aim: The ideal repair of esophageal atresia (EA) is primary anastomosis with closure of the fistula if present. Long gap or local circumstances prompt other procedures that occasionally lead to disastrous complications. The aim of this study was to analyze the management of these complications in a tertiary referral center.

Severe tracheobronchial injuries: our experience.

Introduction: Severe tracheobronchial injuries (TBI) in children are usually traumatic or iatrogenic. However, they can also be caused by mediastinal infections that lead to critical situations. We herein report our experience in the treatment of these lesions.

Early endoscopic dilation and mitomycin application in the treatment of acquired tracheal stenosis.

Introduction: Acquired airway stenosis is a common complication in children after periods of tracheal intubation. We reviewed our experience in the endoscopic treatment of these lesions.

Patients And Methods: We performed a retrospective review of patients who presented acquired tracheal-subglottic stenosis (SGS) treated at our center from 2005 to 2012.

Failed Nissen fundoplication in children: causes and management.

Introduction: Nissen fundoplication (NF) is the gold standard procedure for the treatment of gastroesophageal reflux (GER) in children. However, it fails in a significant proportion of patients. The purpose of this study was to identify preoperative predictors of failure and to examine the results of reoperations.

Abnormal development of the enteric nervous system in rat embryos and fetuses with congenital diaphragmatic hernia.

Background/aim: Esophageal dilatation, gastroesophageal reflux, and intestinal obstruction have been demonstrated in CDH survivors. Abnormal esophageal and intestinal innervations were recently found in rats and babies with this disease. Our aim was to further characterize these malformations in embryos and fetal rats exposed to nitrofen.

Graft-vs-host disease after small bowel transplantation in children.

Purpose: Graft-vs-host disease (GVHD) is a rare complication of transplantation of organs rich in immunocompetent cells. The goal of this study was to report the features of GVHD after small bowel transplantation (SBTx) in children.

Methods: The study involved a retrospective review of patients undergoing SBTx between 1999 and 2009 who had GVHD.

Compound heterozygous mutations in the SRD5A2 gene exon 4 in a male pseudohermaphrodite patient of Chinese origin.

The goal of this study was to perform 5-alpha-reductase type 2 gene (SRD5A2) analysis in a male pseudohermaphrodite (MPH) patient with normal testosterone (T) production and normal androgen receptor (AR) gene coding sequences. A patient of Chinese origin with ambiguous genitalia at 14 months, a 46,XY karyotype, and normal T secretion under human chorionic gonadotropin (hCG) stimulation underwent a gonadectomy at 20 months. Exons 1-8 of the AR gene and exons 1-5 of the SRD5A2 gene were sequenced from peripheral blood DNA.