Vocal cord dysfunction following esophageal atresia and tracheoesophageal fistula (EA/TEF) repair.

Background: The purpose of this study was to determine risk factors and long-term outcomes in patients with esophageal atresia +/-tracheoesophageal fistula (EA/TEF) with vocal cord dysfunction (VCD) owing to recurrent laryngeal nerve (RLN) injury.

Method: A retrospective chart review was performed for EA/TEF patients repaired at our institution from 1999 to 2014 (REB #1000032265).

Results: Of 197 patients, 22 (11.

Neurodevelopmental outcomes of infants with esophageal atresia and tracheoesophageal fistula.

Background: Esophageal atresia with or without tracheoesophageal fistula (EA/TEF) is a complex disorder, and most outcome data are confined to mortality and feeding-related morbidities. Our objective was to examine mortality, growth and neurodevelopmental outcomes in a large recent cohort of infants with EA/TEF.

Methods: Single center study of EA/TEF infants referred from January 2000 to December 2015.

Key Residues at Third CDR3β Position Impact Structure and Antigen Recognition of Human Invariant NK TCRs.

The human invariant NK (iNK) TCR is largely composed of the invariant TCR Vα24-Jα18 chain and semivariant TCR Vβ11 chains with variable CDR3β sequences. The direct role of CDR3β in Ag recognition has been studied extensively. Although it was noted that CDR3β can interact with CDR3α, how this interaction might indirectly influence Ag recognition is not fully elucidated.

Pulmonary function and nutritional morbidity in children and adolescents with congenital diaphragmatic hernia.

Background: Malnutrition is common among congenital diaphragmatic hernia (CDH) survivors and may result from elevated respiratory effort. We evaluated body mass index (BMI), measured resting energy expenditure (mREE) and pulmonary function test (PFT) results in children and adolescents with CDH to determine if there is a correlation.

Methods: With ethics approval (REB# 1000035323), anthropometrics, indirect calorimetry (IC) results and PFTs were collected from patients 5-17years of age during CDH clinic visits between 2000 and 2016.

Long-term outcomes following H-type tracheoesophageal fistula repair in infants.

Purpose: To evaluate outcomes following repair of H-type tracheoesophageal fistula (TEF).

Methods: Retrospective chart review of infants with H-type TEF treated at our institution between 2000 and 2014. Patient demographics, surgical management, and postoperative function were evaluated.

Duodeno-duodenostomy or duodeno-jejunostomy for duodenal atresia: is one repair better than the other?

Purpose: The surgical management of neonates with duodenal atresia (DA) involves re-establishment of intestinal continuity, either by duodeno-duodenostomy (DD) or by duodeno-jejunostomy (DJ). Although the majority of pediatric surgeons perform DD repair preferentially, we aimed to analyze the outcome of DA neonates treated with either surgical technique.

Methods: Following ethical approval (REB:1000047737), we retrospectively reviewed the charts of all patients who underwent DA repair between 2004 and 2014.

Congenital diaphragmatic hernia: Observed/expected lung-to-head ratio as a predictor of long-term morbidity.

Aim: The aim of this study was to investigate the association of observed/expected (O/E) lung-to-head ratio (LHR) with long-term morbidity for isolated fetal congenital diaphragmatic hernia (CDH) patients in a single institution.

Methods: We performed a retrospective study of prenatally diagnosed CDH from 18 to 38weeks of gestation (01/2002-04/2010). Two cohorts of O/E LHR were defined (22.

Preservation of native esophagus in infants with pure esophageal atresia has good long-term outcomes despite significant postoperative morbidity.

Purpose: To evaluate long-term outcomes of pure esophageal atresia (EA) repair with preservation of native esophagus.

Methods: Infants with pure EA treated at our institution (2000-2010) and with minimum 5-year follow-up were reviewed (REB:1000046653). Data analysed included demographics, management and outcomes and are reported as mean ± SD/median (range).

Outcome of esophageal atresia/tracheoesophageal fistula in extremely low birth weight neonates (<1000 grams).

Purpose: To review the outcomes of extremely low birth weight (ELBW, <1000 g) infants with esophageal atresia/tracheoesophageal fistula (EA/TEF).

Methods: Health records of ELBW EA/TEF infants treated at our institution from 2000 to 2014 were reviewed (REB1000046653). Demographics, operative approach and postoperative complications were analyzed.

Nutritional Intake, Energy Expenditure, and Growth of Infants Following Congenital Diaphragmatic Hernia Repair.

Background/objectives: The pathophysiology of failure to thrive in congenital diaphragmatic hernia (CDH) has not been fully elucidated, and the nutritional care of these infants is hindered by paucity of data on the optimal calorie requirements for growth. The primary objective of this study was to investigate the energy intake required for infants with CDH to grow optimally at the time of first hospital discharge. The secondary objectives were to assess their measured resting energy expenditure in infancy, and their long-term growth outcomes.

Morbidity after elective resection of prenatally diagnosed asymptomatic congenital pulmonary airway malformations.

Background/aim: The optimal management of prenatally diagnosed asymptomatic congenital pulmonary airway malformations (CPAM) is controversial. Since there is a paucity of data relating to surgical risks in this specific population, we reviewed our experience to further inform this controversy.

Methods: Ethically approved 10 year (2004-2013) retrospective review.

Long-term burden of care and radiation exposure in survivors of esophageal atresia.

Background: Patients with esophageal atresia with or without tracheoesophageal fistula (EA/TEF) historically have had a high risk of neonatal mortality but the majority of patients are now expected to live into adulthood. However, the long-term burden of care among recent EA/TEF survivors has not been documented.

Methods: A single-institution retrospective review of newborns with EA/TEF treated from 2001-2005 was conducted, including initial and total hospitalization length of stay, and number of clinic visits and procedures requiring general anesthesia in the first three years of life.

Long-term nutritional morbidity for congenital diaphragmatic hernia survivors: Failure to thrive extends well into childhood and adolescence.

Background: Failure to thrive (FTT) is well documented among congenital diaphragmatic hernia (CDH) survivors ≤3years of age, but its etiology, severity, and persistence beyond this age require further elucidation.

Methods: We conducted a single-center, retrospective study assessing anthropometrics, measured energy expenditure, and feeding tube (FT) use of 5-17 year olds in our multidisciplinary CDH clinic since January 2001. We stratified clinic visits based on age A: 5.

New Insights into Congenital Diaphragmatic Hernia - A Surgeon's Introduction to CDH Animal Models.

In recent decades, new research into the developmental defects and pathophysiological basis of congenital diaphragmatic hernia (CDH) has revealed opportunities for the development of innovative therapies. Importantly, the use of animal models to represent this anomaly in the laboratory has resulted in the discovery of many important genetic, epigenetic, and other molecular contributors to this condition. In this review, the most commonly used and newly devised animal models of CDH are presented to familiarize the reader with the latest innovations in the basic sciences.

Late surgical outcomes among congenital diaphragmatic hernia (CDH) patients: why long-term follow-up with surgeons is recommended.

Background: CDH patients experience multi-system morbidity. Despite apparent health, late childhood complications do occur. We reviewed the long-term surgical morbidity of our CDH patients to determine whether protracted clinical surveillance is warranted.

Thoracoscopic repair of neonatal congenital diaphragmatic hernia (CDH): outcomes after a systematic quality improvement process.

Background: Higher recurrence rates have been reported for thoracoscopic repair (TR) of neonatal congenital diaphragmatic hernia (CDH) compared to open repair. Our centre initiated changes in surgical management following a quality review in order to improve outcome.

Methods: A retrospective review of TR patients from 2000 to 2011 at a single institution was performed.

Morbidity and long-term follow-up in CDH patients.

Overall survival of congenital diaphragmatic hernia (CDH) patients has increased since the widespread implementation of delayed operative repair with extracorporeal membrane oxygenation/gentle ventilation strategies starting in the mid-1990s. With the improvements in survival, CDH survivorship has been marked by increased morbidity with a greater need for monitoring and supportive interventions in infancy and early childhood. The multisystem morbidity and complexity of care for these patients mandate the dedicated resources and focus of medical specialties to ensure that the neurodevelopmental, cardiopulmonary, nutritional, and surgical sequelae of CDH are properly addressed.

What is the best prosthetic material for patch repair of congenital diaphragmatic hernia? Comparison and meta-analysis of porcine small intestinal submucosa and polytetrafluoroethylene.

Background: Prosthetic patches are widely used for congenital diaphragmatic hernia (CDH) repair. We compared the complication rate of absorbable (Surgisis [SIS]; Cook Biotech, Inc, West Lafayette, IN) and nonabsorbable (polytetrafluoroethylene [PTFE]) materials for this purpose.

Methods: This is a retrospective review of all neonates with CDH undergoing patch closure at our institution over 10 years.

Multi-institutional follow-up of patients with congenital diaphragmatic hernia reveals severe disability and variations in practice.

Background/purpose: Survivors of congenital diaphragmatic hernia (CDH) have a high incidence of morbidity. Variability in follow-up practices between institutions may affect perception of disability and prevent population-based outcome analysis.

Methods: A survey of follow-up practices at 16 centers within a population-based CDH network was performed.

Congenital diaphragmatic hernia: lung-to-head ratio and lung volume for prediction of outcome.

Objective: The purpose of this study was to evaluate observed/expected (O/E) lung-to-head ratio (LHR) by ultrasound (US) and total fetal lung volume (TFLV) by magnetic resonance imaging as neonatal outcome predictors in isolated fetal congenital diaphragmatic hernia (CDH).

Study Design: We conducted a retrospective study of 72 fetuses with isolated CDH, in whom O/E LHR and TFLV were evaluated as survival predictors.

Results: O/E LHR on US and O/E TFLV by magnetic resonance imaging were significantly lower in newborn infants with isolated CDH who died compared with survivors (30.

Leukemia-initiating cells in human T-lymphoblastic leukemia exhibit glucocorticoid resistance.

T-cell acute lymphoblastic leukemia (T-ALL) is associated with a significant risk of disease relapse, but the biological basis for relapse is poorly understood. Here, we identify leukemiainitiating cells (L-ICs) on the basis of functional assays and prospective isolation and report a role for L-ICs in T-ALL disease and relapse. Long-term proliferation in response to NOTCH1 activating signals in OP9-DL1 coculture system or capacity to initiate leukemia in xenografts by the CD7(+)CD1a(-) subset of primary T-ALL samples was superior to other subsets, refining the identity of T-ALL L-ICs.

Surgical conditions of the diaphragm: posterior diaphragmatic hernias in infants.

The care of the neonate with CDH has significantly evolved from an approach characterized by aggressive ventilation and emergency surgical repair, to the current use of permissive hypercapnea, physiologic stabilization, elective surgical repair, and multidisciplinary follow-up. Overall survival now approaches 80%, but this improvement has been associated with increased recognition of respiratory, cardiac, gastrointestinal, and neurodevelopmental morbidity. Topics of continuing controversy in the management of CDH include the value of fetal tracheal occlusion, the expanding role of ECMO, and the use of minimal access techniques for surgical repair.

Outcomes after muscle flap vs prosthetic patch repair for large congenital diaphragmatic hernias.

Background: Repair of large congenital diaphragmatic hernia (CDH) defects still pose a significant challenge, as the defects cannot be repaired primarily. Two techniques have been widely used: autologous anterior abdominal wall muscle flap and prosthetic patch. The latter has been used more often.

Pulmonary function after early vs late lobectomy during childhood: a preliminary study.

Background: One proposed reason for the early resection of asymptomatic congenital cystic adenomatoid malformations is the theoretical benefit of optimizing compensatory lung growth during infancy and early childhood. Our aim was to determine if early lobectomy is associated with better long-term pulmonary function than lobectomy later in childhood.

Methods: A retrospective chart review of children undergoing pulmonary lobectomy for benign disease from 1990 to 2006 was performed.