Graft dilatation and Barrett's esophagus in adults after gastric pull-up and jejunal interposition for long-gap esophageal atresia.

Background: Esophageal replacement (ER) with gastric pull-up (GPU) or jejunal interposition (JI) used to be the standard treatment for long-gap esophageal atresia (LGEA). Changes of the ER grafts on a macro- and microscopic level however, are unknown.

Aim: To evaluate long-term clinical symptoms and anatomical and mucosal changes in adolescents and adults after ER for LGEA.

Airway Epithelial Cultures of Children with Esophageal Atresia as a Model to Study Respiratory Tract Disorders.

Esophageal atresia (EA) is a rare birth defect in which respiratory tract disorders are a major cause of morbidity. It remains unclear whether respiratory tract disorders are in part caused by alterations in airway epithelial cell functions such as the activity of motile cilia. This can be studied using airway epithelial cell culture models of patients with EA.

Bacterial Colonization of the Lower Airways in Children With Esophageal Atresia.

Background: Esophageal atresia (EA) is most often accompanied by some degree of tracheomalacia (TM), which negatively influences the airway by ineffective clearance of secretions. This can lead to lower airway bacterial colonization (LABC), which may cause recurrent respiratory tract infections (RTIs). This study aims to evaluate the prevalence and specific pathogens of LABC in EA patients.

Primary Posterior Tracheopexy in Esophageal Atresia Decreases Respiratory Tract Infections.

Esophageal atresia (EA) is often accompanied by tracheomalacia (TM). TM can lead to severe respiratory complaints requiring invasive treatment. This study aims to evaluate if thoracoscopic primary posterior tracheopexy (PPT) can prevent the potential sequelae of TM in patients with EA.

Thoracoscopic Repair of Esophageal Atresia.

Esophageal atresia (EA) is a rare congenital malformation of the esophagus. Surgical treatment is required to restore the continuity of the esophagus. This can be performed through thoracotomy.

Childhood outcome after correction of long-gap esophageal atresia by thoracoscopic external traction technique.

Background: Thoracoscopic external traction technique (TTT) is a relatively new surgical intervention for patients with long-gap esophageal atresia (LGEA) that preserves the native esophagus. The major accomplishment with TTT is that esophageal repair can be achieved within days after birth. This study evaluates the childhood outcome in LGEA patients treated with TTT, including gastrointestinal outcome, nutritional status and Health-Related Quality of Life (HRQoL).

Five-Year Outcome of Laparoscopic Fundoplication in Pediatric GERD Patients: a Multicenter, Prospective Cohort Study.

Background: Gastroesophageal reflux disease (GERD) is a common disease in children. When drug treatment fails, laparoscopic anti-reflux surgery (LARS) is considered. Short-term follow-up studies report high success rates; however, few studies report long-term results.

ERNICA Consensus Conference on the Management of Patients with Long-Gap Esophageal Atresia: Perioperative, Surgical, and Long-Term Management.

Introduction:  Evidence supporting best practice for long-gap esophageal atresia is limited. The European Reference Network for Rare Inherited Congenital Anomalies (ERNICA) organized a consensus conference on the management of patients with long-gap esophageal atresia based on expert opinion referring to the latest literature aiming to provide clear and uniform statements in this respect.

Materials And Methods:  Twenty-four ERNICA representatives from nine European countries participated.

ERNICA Consensus Conference on the Management of Patients with Esophageal Atresia and Tracheoesophageal Fistula: Follow-up and Framework.

Introduction:  Improvements in care of patients with esophageal atresia (EA) and tracheoesophageal fistula (TEF) have shifted the focus from mortality to morbidity and quality-of-life. Long-term follow-up is essential, but evidence is limited and standardized protocols are scarce. Nineteen representatives of the European Reference Network for Rare Inherited Congenital Anomalies (ERNICA) from nine European countries conducted a consensus conference on the surgical management of EA/TEF.

Health-related quality of life in children after laparoscopic gastrostomy placement.

Introduction: A gastrostomy placement (GP) is an established treatment to provide enteral feeding in pediatric patients with feeding difficulties aiming to improve nutritional status and health-related quality of life (HRQoL). The aim of this study was to evaluate HRQoL in children with severe feeding difficulties who have undergone GP.

Materials And Methods: A cross-sectional study was performed including 128 patients who had undergone laparoscopic GP (2004-2011).

The Effect of Gastrostomy Placement on Health-Related Quality of Life in Children.

Background And Purpose: A gastrostomy placement (GP) aims to improve nutritional status and health-related quality of life (HRQoL) in children who require long-term enteral tube feeding. We evaluated the effect of GP on HRQoL.

Methods: A prospective, longitudinal cohort study was performed including patients referred for laparoscopic GP.

ERNICA Consensus Conference on the Management of Patients with Esophageal Atresia and Tracheoesophageal Fistula: Diagnostics, Preoperative, Operative, and Postoperative Management.

Introduction:  Many aspects of the management of esophageal atresia (EA) and tracheoesophageal fistula (TEF) are controversial and the evidence for decision making is limited. Members of the European Reference Network for Rare Inherited Congenital Anomalies (ERNICA) conducted a consensus conference on the surgical management of EA/TEF based on expert opinions referring to the latest literature.

Materials And Methods:  Nineteen ERNICA representatives from nine European countries participated in the conference.

Error traps and culture of safety in esophageal atresia repair.

Esophageal atresia (EA) repair has always been a source of immense professional gratification for the pediatric surgeon. In many ways, this anomaly defines the entire profession. Due to its rarity, there is an increased risk of inadvertent events occurring during correction.

Thoracoscopic posterior tracheopexy during primary esophageal atresia repair: a new approach to prevent tracheomalacia complications.

Background: Esophageal atresia (EA) is usually accompanied by some form of tracheomalacia (TM). During the early phases in life, excessive dynamic collapse of the trachea can cause a wide spectrum of symptoms ranging from mild complaints to apparent life-threatening events (ALTE's) or brief resolved unexplained events (BRUE's). Therapeutic strategies for severe TM include aortopexy to lift the anterior weakened cartilaginous rings or posterior tracheopexy of the floppy membranous tracheal intrusion.

The incidence of associated abnormalities in patients with sacrococcygeal teratoma.

Background: Gross genetic causes for SCT are unknown; however, it might be associated with other abnormalities. We assessed the incidence of associated abnormalities in a large national cohort of neonates with SCT and aimed to identify predictive risk factors.

Procedure: The medical records were reviewed of 235 consecutive neonates with SCT treated at the six pediatric surgical centers in the Netherlands from 1970 to 2010.

Esophageal Atresia and Upper Airway Pathology.

Esophageal atresia is an anomaly with frequently occurring sequelae requiring lifelong management and follow-up. Because of the complex issues that can be encountered, patients with esophageal atresia preferably should be managed in centers of expertise that have the ability to deal with all types of anomalies and sequelae and can perform rigorous lifelong follow-up. Tracheomalacia is an often-occurring concurrent anomaly that may cause acute life-threatening events and may warrant immediate management.

Effect of general anesthesia on neonatal aEEG-A cohort study of patients with non-cardiac congenital anomalies.

Introduction: The aim of the current study was to determine the effect of general anesthesia on neonatal brain activity using amplitude-integrated EEG (aEEG).

Methods: A prospective cohort study of neonates (January 2013-December 2015), who underwent major neonatal surgery for non-cardiac congenital anomalies. Anesthesia was administered at the discretion of the anesthetist.

Choledochal Malformation in Children: Lessons Learned from a Dutch National Study.

Introduction: A choledochal malformation (CM) is a rare entity, especially in the Western world. We aimed to determine the incidence of CM in the Netherlands and the outcome of surgery for CM in childhood.

Methods: All pediatric patients who underwent a surgical procedure for type I-IV CM between 1989 and 2014 were entered into the Netherlands Study group on choledochal cyst/malformation.

Esophageal atresia and tracheo-esophageal fistula.

Management of esophageal atresia has merged from correction of the anomaly to the complete spectrum of management of esophageal atresia and all its sequelae. It is the purpose of this article to give an overview of all aspects involved in taking care of patients with esophageal atresia between January 2011 and June 2016, as well as the patients who were referred from other centers. Esophageal atresia is a complex anomaly that has many aspects that have to be dealt with and complications to be solved.

The Effect of Gastrostomy Placement on Gastric Function in Children: a Prospective Cohort Study.

Background: A gastrostomy placement is frequently performed in pediatric patients who require long-term enteral tube feeding. Unfortunately, postoperative complications such as leakage, feeding intolerance, and gastroesophageal reflux frequently occur. These complications may be due to postoperative gastric dysmotility.

Position Paper of INoEA Working Group on Long-Gap Esophageal Atresia: For Better Care.

INoEA is the International Network of Esophageal Atresia and consists of a broad spectrum of pediatric specialties and patient societies. The working group on long-gap esophageal atresia (LGEA) set out to develop guidelines regarding the definition of LGEA, the best diagnostic and treatment strategies, and highlight the necessity of experience and communication in the management of these challenging patients. Review of the literature and expert discussion concluded that LGEA should be defined as any esophageal atresia (EA) that has no intra-abdominal air, realizing that this defines EA with no distal tracheoesophageal fistula (TEF).

Respiratory function after esophageal replacement in children.

Background: Children born with esophageal atresia require an anastomosis between the proximal and distal esophagus. When this distance is too wide (long gap esophageal atresia, LGEA) esophageal replacement strategies have to be deployed. The aim of this study was to assess long-term respiratory morbidity and lung function after esophageal replacement with either stomach (gastric pull-up, GPU) or jejunum (jejunal interposition, JI) for LGEA.

The timing of surgery of antenatally diagnosed choledochal malformations: A descriptive analysis of a 26-year nationwide cohort.

Introduction: Choledochal malformations (CMs) are increasingly diagnosed antenatally. There is a dilemma between early surgery to prevent CM-related symptoms and postponing surgery to reduce complications. We aimed to identify the optimal timing of surgery in asymptomatic neonates with antenatally diagnosed CM and to identify predictors for development of symptoms.