Health-Related Quality of Life in Pediatric Intestinal Failure from Infancy to Adolescence: An International, Multi-Center Evaluation.

Objective: To evaluate health-related quality of life (HRQOL) and associated factors in pediatric intestinal failure (PIF) using a prospective, multi-institutional structure.

Study Design: HRQOL in 336 children with PIF was assessed using the PedsQL Generic Core and Gastrointestinal (GI) Symptoms Scales at 11 North American intestinal rehabilitation programs. Scores were compared between patients, caregivers' proxy report of patient HRQOL, and reference healthy and chronic GI disease samples.

Risk Factors and Treatment Strategies for Anastomotic Ulcers in Pediatric Intestinal Failure.

Purpose: Anastomotic ulcers (AUs) are a rare cause of morbidity in intestinal failure (IF). Prior studies of AUs have been small, descriptive reports. We evaluated a large cohort of IF patients to identify risk factors and describe treatment strategies for AUs.

Comparing Healthcare Needs in Extremely Low Birth Weight Infants With NEC and Spontaneous Intestinal Perforation.

Background: Necrotizing enterocolitis (NEC) and spontaneous intestinal perforation (SIP) affect 6-8% of extremely low birth weight (ELBW) infants. SIP has lower mortality than NEC, but with similar short-term morbidity in length of stay, growth failure, and supplemental oxygen requirements. Comparative long-term neurodevelopmental outcomes have not been clarified.

Defining Practice Readiness in Pediatric Surgery Fellowship: A Qualitative Analysis.

Introduction: While competency-based training is at the forefront of educational innovation in General Surgery, Pediatric Surgery training programs should not wait for downstream changes. There is currently no consensus on what it means for a pediatric surgery fellow to be "practice-ready". In this study, we aimed to provide a framework for better defining competency and practice readiness in a way that can support the Milestones system and allow for improved assessment of pediatric surgery fellows.

Factors associated with D-lactic acidosis in pediatric intestinal failure: A case-control study.

Background: D-lactic acidosis (DLA) is a serious complication of short bowel syndrome (SBS) in children with intestinal failure (IF). Malabsorbed carbohydrates are metabolized by bacteria in the intestine to D-lactate which can lead to metabolic acidosis and neurologic symptoms.

Methods: A retrospective chart review was performed in children ≤18 years old with SBS who had one of the following criteria: unexplained metabolic acidosis, neurologic signs or symptoms, history of antibiotic therapy for small bowel bacterial overgrowth, or high clinical suspicion of DLA.

Predictors of Mortality in Very Low Birth Weight Neonates With Congenital Diaphragmatic Hernia.

Background: Limited data exists regarding the mortality of very low birth weight (VLBW) neonates with congenital diaphragmatic hernia (CDH). This study aims to quantify and determine predictors of mortality in VLBW neonates with CDH.

Methods: This analysis of 829 U.

How Many Operative Performance Ratings Does a Pediatric Surgery Fellow Need to Be Deemed Practice Ready?

Purpose: Identifying the number of cases required for a fellow to achieve competence has been challenging. Workplace-based assessment (WBA) systems make collecting performance data practical and create the opportunity to translate WBA ratings into probabilistic statements about a fellow's likelihood of performing to a given standard on a subsequent assessment opportunity.

Methods: We compared data from two pediatric surgery training programs that used the performance rating scale from the Society for Improving Medical Professional Learning (SIMPL).

Association between neurodevelopmental outcomes and concomitant presence of NEC and IVH in extremely low birth weight infants.

Objective: To quantify the association between necrotizing enterocolitis (NEC) and neurodevelopmental disability (NDI) in extremely low birth weight (ELBW) infants with intraventricular hemorrhage (IVH).

Study Design: ELBW survivors born 2011-2017 and evaluated at 16-26 months corrected age in the Vermont Oxford Network (VON) ELBW Follow-Up Project were included. Logistic regression determined the adjusted relative risk (aRR) of severe NDI in medical or surgical NEC compared to no NEC, stratified by severity of IVH.

Factors Associated With Chronic Intestinal Inflammation Resembling Inflammatory Bowel Disease in Pediatric Intestinal Failure: A Matched Case-Control Study.

Background And Aims: There is a subset of intestinal failure patients with associated chronic intestinal inflammation resembling inflammatory bowel disease. This study aimed to evaluate factors associated with chronic intestinal inflammation in pediatric intestinal failure.

Methods: This was a single-center retrospective case-control study of children <18 years old with intestinal failure.

High Rate of Venous Thromboembolism in Severe Pediatric Intestinal Failure.

Objective: To quantify the rate of venous thromboembolism (VTE) in patients with pediatric intestinal failure and identify associated risk factors.

Study Design: We performed a retrospective cohort study in pediatric patients (<21 years old) with severe pediatric intestinal failure (≥90 consecutive days of parenteral nutrition) secondary to short bowel syndrome who were treated from 2014 to 2021 at an interdisciplinary intestinal rehabilitation program. The primary outcome was the incidence of VTE.

Autoimmune Thyroiditis and Risk of Malignancy in Children with Thyroid Nodules.

It is uncertain whether the presence of autoimmune thyroiditis (AIT) increases the risk of thyroid cancer in children with thyroid nodules. This study evaluated the association between AIT and thyroid cancer in pediatric patients with thyroid nodules. A cross-sectional study was performed of pediatric patients (<19 years old) with a thyroid nodule (≥1 cm) who underwent fine-needle aspiration in an academic pediatric thyroid center.

Impact of concomitant necrotizing enterocolitis on mortality in very low birth weight infants with intraventricular hemorrhage.

Objective: To evaluate the impact of necrotizing enterocolitis (NEC) on mortality in very low birth weight (VLBW) infants with intraventricular hemorrhage (IVH).

Study Design: Data were collected on VLBW infants born 2014-2018 at Vermont Oxford Network (VON) centers. NEC and IVH were categorized by severity.

Antibiotic Therapy for Culture-Proven Bacterial Overgrowth in Children With Intestinal Failure Results in Improved Symptoms and Growth.

Objectives: To evaluate symptoms, enteral tolerance, growth, and antibiotic regimens in pediatric intestinal failure (IF) patients after treated with antibiotic therapy for small bowel bacterial overgrowth (SBBO).

Methods: Single-center retrospective review of children 0-18 years with IF with endoscopic cultures demonstrating >10 5 CFU/mL from 2010 to 2017. Symptoms, enteral tolerance, growth, and antibiotic regimens were evaluated at the time of endoscopy and 6 months later.

Current outcomes of infants with esophageal atresia and tracheoesophageal fistula: A multicenter analysis.

Objective: This study aims to quantify mortality rates and hospital lengths of stay (LOS) in neonates with esophageal atresia and tracheoesophageal fistula (EA/TEF), and to characterize the effects of birth weight (BW) and associated congenital anomalies upon these.

Methods: Data regarding patients with EA/TEF were prospectively collected (2013-2019) at 298 North American centers. The primary outcome was mortality and secondary outcome was LOS.

Morbidity associated with laparotomy-confirmed spontaneous intestinal perforation: A prospective multicenter analysis.

Background: Differences in morbidities between spontaneous intestinal perforation (SIP) and necrotizing enterocolitis (NEC) are unknown.

Methods: Prospectively collected multicenter data regarding very low birth weight (VLBW) infants 2015-2019 were analyzed. Diagnosis of SIP or NEC was laparotomy-confirmed in all patients.

Puberty and growth in patients with pediatric intestinal failure.

Background: Pediatric intestinal failure (PIF) affects nutrition, metabolism, and endocrine development, but its downstream impact on puberty is unknown.

Methods: A retrospective review was performed of patients age >8 years with PIF managed at an intestinal rehabilitation program. Outcomes of interest were peak height velocity (PHV), age at PHV, and age at pubertal onset (Tanner stage 2).

Association Between Body Mass Index and Thyroid Cancer in Children With Thyroid Nodules.

This cross-sectional study examines whether body mass index in children with thyroid nodules is associated with thyroid cancer and a greater likelihood of undergoing thyroid surgery.

Subtype of atypia on cytology and risk of malignancy in pediatric thyroid nodules.

Background: Thyroid nodules with atypia of undetermined significance (AUS) are challenging to manage because of their intermediate risk of malignancy. Subclassification of atypia can refine malignancy risk in adult AUS nodules but has not been evaluated in children.

Methods: This was a retrospective cohort study of pediatric patients (<19 years old) who underwent fine-needle aspiration (FNA) of a thyroid nodule with resulting AUS cytology.

Venous thoracic outlet syndrome and Paget-Schroetter syndrome.

Venous thoracic outlet syndrome represents a relatively rare but important diagnosis in the adolescent population with increasing recognition. Compression of the subclavian vein within the costoclavicular space can lead to episodic venous outlet obstruction in the upper extremity, with edema, rubor and functional symptoms. Over time, cumulative injury and compression can lead to thrombosis of the vein, referred to as "effort thrombosis" or the Paget-Schroetter syndrome.

Long-term assessment of bilirubin and transaminase trends in pediatric intestinal failure patients during the era of hepatoprotective parenteral nutrition.

Purpose: This study aimed to characterize the relationship between hepatoprotective parenteral nutrition (PN) dependence and long-term serum liver tests in children with intestinal failure (IF).

Methods: A retrospective review was performed of children with severe IF (> 90 consecutive days of PN) who were followed from 2012 to 2019 at a multidisciplinary intestinal rehabilitation program. Patients were stratified into three groups based on level of PN dependence at most recent follow up: EN (achieved enteral autonomy), mixed (parenteral and enteral nutrition), and PN (> 75% of caloric intake from PN).