Common variants increase risk for congenital diaphragmatic hernia within the context of de novo variants.

Congenital diaphragmatic hernia (CDH) is a severe congenital anomaly often accompanied by other structural anomalies and/or neurobehavioral manifestations. Rare de novo protein-coding variants and copy-number variations contribute to CDH in the population. However, most individuals with CDH remain genetically undiagnosed.

Management of undifferentiated embryonal sarcoma of the liver: A Pediatric Surgical Oncology Research Collaborative study.

Background: Undifferentiated embryonal sarcoma of the liver (UESL) is a rare tumor for which there are few evidence-based guidelines. The aim of this study was to define current management strategies and outcomes for these patients using a multi-institutional dataset curated by the Pediatric Surgical Oncology Research Collaborative.

Methods: Data were collected retrospectively for patients with UESL treated across 17 children's hospitals in North America from 1989 to 2019.

Decision-Making in Pleural Drainage Following Lung Resection in Children: A Western Pediatric Surgery Research Consortium Survey.

Introduction: Studies of adults undergoing lung resection indicated that selective omission of pleural drains is safe and advantageous. Significant practice variation exists for pleural drainage practices for children undergoing lung resection. We surveyed pediatric surgeons in a 10-hospital research consortium to understand decision-making for placement of pleural drains following lung resection in children.

Critical elements of pediatric neuroblastoma surgery.

Neuroblastoma (NB) is the most common solid extracranial malignancy of childhood with an incidence of 1 per 100,000 in the United States compromising approximately 10 % of childhood cancer. Unfortunately, patients with high-risk NG continue to have long-term survival less than 50 %. Both Children's Oncology Group and the International Society of Paediatric Oncology have demonstrated the important role of surgery in the treatment of high-risk NB.

The varied spectrum of nephroblastomatosis, nephrogenic rests, and Wilms tumors: Review of current definitions and challenges of the field.

The diagnosis of multiple or diffuse renal lesions in a child is challenging by imaging and/or pathology. Optimal management requires distinguishing benign lesions such as nephrogenic rests from cancerous lesions such as Wilms tumor, but this is often difficult or impossible. This difficulty is compounded by the overlapping nature of our current radiologic and pathologic definitions of lesions along the spectrum of nephrogenic rests/nephroblastomatosis.

Postoperative Respiratory Complications in SARS-CoV-2 Positive Pediatric Patients Across 20 United States Hospitals: A Cohort Study.

Introduction: Data examining rates of postoperative complications among SARS-CoV-2 positive children are limited. The purpose of this study was to evaluate the impact of symptomatic and asymptomatic SARS-CoV-2 positive status on postoperative respiratory outcomes for children.

Methods: This retrospective cohort study included SARS-CoV-2 positive pediatric patients across 20 hospitals who underwent general anesthesia from March to October 2020.

Standardized perioperative care reduces colorectal surgical site infection in children: A Western Pediatric Surgery Research Consortium multicenter analysis.

Purpose: Surgical site infection (SSI) remains a significant source of patient morbidity and resource utilization in children undergoing colorectal surgery. We examined the utility of a protocolized perioperative care bundle in reducing SSI in pediatric patients undergoing colorectal surgery.

Methods: We conducted a prospective cohort study of patients ≤18 years of age undergoing colorectal surgery at ten United States children's hospitals.

Meconium Ileus, Distal Intestinal Obstruction Syndrome, and Other Gastrointestinal Pathology in the Cystic Fibrosis Patient.

Cystic fibrosis is an autosomal-recessive defect in the cystic fibrosis transmembrane conductance regulator (CFTR) gene located on chromosome 7 that affects 1 in 2500 live White births. Defects in the gene lead to abnormally thick secretions causing chronic obstruction in the respiratory and gastrointestinal tracts. Common gastrointestinal pathology in children with cystic fibrosis includes meconium ileus in infancy and distal intestinal obstruction syndrome in childhood and exocrine pancreatic insufficiency, constipation, and rectal prolapse.

Erratum: Identification and validation of candidate risk genes in endocytic vesicular trafficking associated with esophageal atresia and tracheoesophageal fistulas.

[This corrects the article DOI: 10.1016/j.xhgg.

Association between COVID-19 related elective surgery cancellations and pediatric inguinal hernia complications: A nationwide multicenter cohort study.

Background: Optimal inguinal hernia repair timing remains controversial. It remains unclear how COVID-19 related elective surgery cancellations impacted timing of inguinal hernia repair and whether any delays led to complications. This study aims to determine whether elective surgery cancellations are safe in pediatric inguinal hernia.

Identification and validation of candidate risk genes in endocytic vesicular trafficking associated with esophageal atresia and tracheoesophageal fistulas.

Esophageal atresias/tracheoesophageal fistulas (EA/TEF) are rare congenital anomalies caused by aberrant development of the foregut. Previous studies indicate that rare or genetic variants significantly contribute to EA/TEF risk, and most individuals with EA/TEF do not have pathogenic genetic variants in established risk genes. To identify the genetic contributions to EA/TEF, we performed whole genome sequencing of 185 trios (probands and parents) with EA/TEF, including 59 isolated and 126 complex cases with additional congenital anomalies and/or neurodevelopmental disorders.

Adolescent Vaping-Associated Trauma in the Western United States.

Introduction: Electronic cigarettes (e-cigarettes) are handheld, battery-powered vaporizing devices. It is estimated that more than 25% of youth have used these devices recreationally. While vaping-associated lung injury is an increasingly recognized risk, little is known about the risk of traumatic injuries associated with e-cigarette malfunction.

Delayed Repeat Contrast Enema for Treatment of Pediatric Intussusception.

Introduction: Ileocolic intussusception is a common cause of pediatric bowel obstruction. Contrast enema is successful in treating the majority of patients, and if initially unsuccessful, approximately one-third may be reduced with repeat enemas. We sought to study protocol implementation for delayed repeat enema in pediatric patients not reduced completely by an initial contrast enema.

Bifidobacteriumlongum subsp. infantis EVC001 Administration Is Associated with a Significant Reduction in the Incidence of Necrotizing Enterocolitis in Very Low Birth Weight Infants.

Objective: To assess the effects of Bifidobacteriumlongum subsp. infantis EVC001 (Binfantis EVC001) administration on the incidence of necrotizing enterocolitis (NEC) in preterm infants in a single level IV neonatal intensive care unit (NICU).

Study Design: Nonconcurrent retrospective analysis of 2 cohorts of very low birth weight (VLBW) infants not exposed and exposed to Binfantis EVC001 probiotic at Oregon Health & Science University from 2014 to 2020.

The need for early Kasai portoenterostomy: a Western Pediatric Surgery Research Consortium study.

Purpose: The purpose of this study was to investigate factors impacting transplant-free survival among infants with biliary atresia.

Methods: A multi-institutional, retrospective cohort study was performed at nine tertiary-level children's hospitals in the United States. Infants who underwent Kasai portoenterostomy (KP) from January 2009 to May 2017 were identified.

Implications of Tumor Characteristics and Treatment Modality on Local Recurrence and Functional Outcomes in Children With Chest Wall Sarcoma: A Pediatric Surgical Oncology Research Collaborative Study.

Objective: To determine the impact of tumor characteristics and treatment approach on (1) local recurrence, (2) scoliosis development, and (3) patient-reported quality of life in children with sarcoma of the chest wall.

Summary Of Background Data: Children with chest wall sarcoma require multimodal therapy including chemotherapy, surgery, and/or radiation. Despite aggressive therapy which places them at risk for functional impairment and scoliosis, these patients are also at significant risk for local recurrence.

Minimizing Variance in Gastroschisis Management Leads to Earlier Full Feeds in Delayed Closure.

Background: Limited guidance exists regarding appropriate timing for feed initiation and advancement in gastroschisis. We hypothesized that implementation of a gastroschisis management protocol would allow for standardization of antibiotic and nutritional treatment for these patients.

Methods: We conducted a retrospective comparison of patients with simple gastroschisis at two pediatric hospitals before and after initiation of our gastroschisis care protocol.

Thoracoscopy vs thoracotomy for the management of metastatic osteosarcoma: A Pediatric Surgical Oncology Research Collaborative Study.

Complete surgical resection of pulmonary metastatic disease in patients with osteosarcoma is crucial to long-term survival. Open thoracotomy allows palpation of nodules not identified on imaging but the impact on survival is unknown. The objective of this study was to compare overall survival (OS) and pulmonary disease-free survival (DFS) in children who underwent thoracotomy vs thoracoscopic surgery for pulmonary metastasectomy.

Optimization of percutaneous biopsy for diagnosis and pretreatment risk assessment of neuroblastoma.

Background: Image-guided percutaneous core needle biopsy (PCNB) is increasingly utilized to diagnose solid tumors. The objective of this study is to determine whether PCNB is adequate for modern biologic characterization of neuroblastoma.

Procedure: A multi-institutional retrospective study was performed by the Pediatric Surgical Oncology Research Collaborative on children with neuroblastoma at 12 institutions over a 3-year period.

Minimizing variance in pediatric surgical care through implementation of a perioperative colon bundle: A multi-institution retrospective cohort study.

Background: Employing an institutional initiative to minimize variance in pediatric surgical care, we implemented a set of perioperative bundled interventions for all colorectal procedures to reduce surgical site infections (SSIs).

Methods: Implementation of a standard colon bundle at two children's hospitals began in December 2014. Subjects who underwent a colorectal procedure during the study period were analyzed.

Perioperative neutropenia is not an independent risk factor for infectious complications of central venous line placement in children: A propensity score-matched analysis.

Background: The infectious risk of central venous line (CVL) placement in children with neutropenia (absolute neutrophil count [ANC] <500/mm) is not well defined. This study aims to investigate the early (≤30 days) and late (>30 days) infectious complications of CVLs placed in pediatric patients with and without neutropenia.

Methods: A retrospective review was conducted of all CVLs placed by pediatric surgeons at two institutions from 2010 to 2017.