Inflammatory Bowel Disease Serological Immune Markers Anti-Saccharomyces cerevisiae Mannan Antibodies and Outer Membrane Porin C are Potential Biomarkers for Hirschsprung-associated Enterocolitis.

Objective: Hirschsprung-associated enterocolitis (HAEC) is the most frequent complication in Hirschsprung disease (HSCR) patients. Currently HAEC is diagnosed clinically, leaving uncertainty in the diagnosis thereby potentially leading to over- or undertreatment of patients. The aim of this study was to identify immune biomarkers to aid in the diagnosis of HAEC.

Fact or myth? The long shared common wall between the fistula and the urethra in male anorectal malformation with urethral bulbar fistula.

Aim: It has long been considered surgical dogma that the length of the shared common wall (CW) between a fistula and the urethra in males with anorectal malformation (ARM) and rectourethral bulbar fistula (RUBF) is considerably longer than in males with ARM and rectourethral prostatic fistula (RUPF). This belief has led surgeons who perform laparoscopic-assisted anorectoplasty (LAARP) for RUPF to avoid LAARP for RUBF for risk of potential injury to the urethra or incomplete removal of the fistula. In this study, we compared CW between RUBF and RUPF using distal colostography (DCG) and direct intraoperative measurements.

Bacterial Microbiome Dynamics in Post Pull-Through Hirschsprung-Associated Enterocolitis (HAEC): An Experimental Study Employing the Endothelin Receptor B-Null Mouse Model.

Purpose: Hirschsprung-associated enterocolitis (HAEC) is the most frequent potentially life-threatening complication in children with Hirschsprung disease (HSCR) even after definitive corrective surgery. Mounting evidence suggests that intestinal microbiota likely contribute to the etiology of enterocolitis, so the aim of this study was to use a mouse model of post pull-through HAEC to compare the fecal bacterial communities of animals which developed HAEC to those free of enterocolitis.

Methods: Ten and 8wild type mice underwent the microsurgical pull-through surgery, and stool was collected at the time of surgery, and then either at 2 and 4 weeks after the operation, or when the mice developed enterocolitis.

Critical evaluation of the Hirschsprung-associated enterocolitis (HAEC) score: A multicenter study of 116 children with Hirschsprung disease.

Objective: To identify the optimal clinical criteria to diagnose Hirschsprung-associated enterocolitis (HAEC) in children with Hirschsprung disease (HSCR).

Background: HAEC is the most common life-threatening complication in HSCR patients, yet the diagnostic criteria for HAEC remain unclear. The consensus-based HAEC scoring system was not validated using patient data, thereby making its diagnostic accuracy uncertain.

Development of a Unifying Target and Consensus Indicators for Global Surgical Systems Strengthening: Proposed by the Global Alliance for Surgery, Obstetric, Trauma, and Anaesthesia Care (The G4 Alliance).

After decades on the margins of primary health care, surgical and anaesthesia care is gaining increasing priority within the global development arena. The 2015 publications of the Disease Control Priorities third edition on Essential Surgery and the Lancet Commission on Global Surgery created a compelling evidenced-based argument for the fundamental role of surgery and anaesthesia within cost-effective health systems strengthening global strategy. The launch of the Global Alliance for Surgical, Obstetric, Trauma, and Anaesthesia Care in 2015 has further coordinated efforts to build priority for surgical care and anaesthesia.

Guidelines for the diagnosis and management of Hirschsprung-associated enterocolitis.

Background: Patients with Hirschsprung disease are at risk for Hirschsprung-associated enterocolitis (HAEC), an inflammatory disorder of the bowel that represents the leading cause of serious morbidity and death in these patients. The diagnosis of HAEC is made based on clinical signs and symptoms which are often non-specific, making it difficult to establish a definitive diagnosis in many patients. The purpose of this guideline is to present a rational, expert-based approach to the diagnosis and management of HAEC.

A Study of VITOM in Pediatric Surgery and Urology: Evaluation of Technology Acceptance and Usability by Operating Team and Surgeon Musculoskeletal Discomfort.

Introduction: We studied operating team acceptability of Video Telescopic Monitor (VITOM) exoscope by exploring the ease of use of the device in two centers. We also assessed factors affecting surgeon musculoskeletal discomfort.

Methods: We focused on how the operating team interacted with the VITOM system with surrogate measures of usefulness, image quality, ease of use, workload, and setup time.

Altered fecal short chain fatty acid composition in children with a history of Hirschsprung-associated enterocolitis.

Purpose: Children with Hirschsprung disease (HD) who have a history of enterocolitis (HAEC) have a shift in colonic microbiota, many of which are necessary for short chain fatty acid (SCFA) production. As SCFAs play a critical role in colonic mucosal preservation, we hypothesized that fecal SCFA composition is altered in children with HAEC.

Methods: A multicenter study enrolled 18 HD children, abstracting for history of feeding, antibiotic/probiotic use, and enterocolitis symptoms.

Human and Murine Tissue-Engineered Colon Exhibit Diverse Neuronal Subtypes and Can Be Populated by Enteric Nervous System Progenitor Cells When Donor Colon Is Aganglionic.

Purpose: Tissue-engineered colon (TEC) might potentially replace absent or injured large intestine, but the enteric nervous system (ENS), a key component, has not been investigated. In various enteric neuropathic diseases in which the TEC is derived from aganglionic donor colon, the resulting construct might also be aganglionic, limiting tissue engineering applications in conditions such as Hirschsprung disease (HD). We hypothesized that TEC might contain a diverse population of enteric neuronal subtypes, and that aganglionic TEC can be populated by neurons and glia when supplemented with ENS progenitor cells in the form of neurospheres.

Characterization of Bacterial and Fungal Microbiome in Children with Hirschsprung Disease with and without a History of Enterocolitis: A Multicenter Study.

Development of potentially life-threatening enterocolitis is the most frequent complication in children with Hirschsprung disease (HSCR), even after definitive corrective surgery. Intestinal microbiota likely contribute to the etiology of enterocolitis, so the aim of this study was to compare the fecal bacterial and fungal communities of children who developed Hirschsprung-associated enterocolitis (HAEC) with HSCR patients who had never had enterocolitis. Eighteen Hirschsprung patients who had completed definitive surgery were enrolled: 9 had a history of HAEC and 9 did not.

Enterocolitis causes profound lymphoid depletion in endothelin receptor B- and endothelin 3-null mouse models of Hirschsprung-associated enterocolitis.

Potentially life-threatening enterocolitis is the most frequent complication in children with colonic aganglionosis (Hirschsprung disease, HSCR), and little is known about the mechanisms leading to enterocolitis. Splenic lymphopenia has been reported in the Endothelin Receptor B (Ednrb)-null mouse model of HSCR that develops enterocolitis. In this study, we sought to identify molecular mechanisms underlying this immune phenotype.

Late onset of necrotizing enterocolitis in the full-term infant is associated with increased mortality: results from a two-center analysis.

Purpose: The effect of timing of onset of necrotizing enterocolitis (NEC) on outcomes has not been determined for the full-term infant. In this study we aimed to characterize the full-term NEC population and to evaluate onset of NEC.

Methods: We performed a two-center retrospective review of all full-term infants (≥ 37weeks) with a diagnosis of NEC between 1990 and 2012.

Is routine preoperative screening echocardiogram indicated in all children with congenital duodenal obstruction?

Introduction: Congenital duodenal obstruction (DO) is frequently associated with congenital heart disease (CHD). Operative repair of DO is often postponed until an echocardiogram is completed, which may result in unnecessary delays. We aimed to identify and characterize CHD in children with DO to determine if appropriately selected patients could forego preoperative echocardiogram.

Laparoscopic versus open treatment of congenital duodenal obstruction: multicenter short-term outcomes analysis.

Background: Laparoscopic repair of congenital duodenal obstruction has become popularized over the past decade. Comparative data on outcomes, however, are sparse. We hypothesized that laparoscopic repair of congenital duodenal obstruction could be performed with similar outcomes to traditional open repair.

Advances in Hirschsprung disease genetics and treatment strategies: an update for the primary care pediatrician.

Hirschsprung disease (HSCR) is a multigenic condition with variable presentation. Most commonly, it presents in the neonatal period as a functional intestinal obstruction secondary to failure of caudal migration of the enteric nervous system. Classically, this manifests as dilated proximal bowel and constricted distal bowel with absent ganglia and hypertrophic nerve trunks.

Distinct phenotypes of children with perianal perforating Crohn's disease.

Purpose: Perianal perforating disease (PF) has been reported in approximately 15% of children with Crohn's disease (CD). It is unknown whether children who present with PF at the time of diagnosis have a different course than those that develop PF while on therapy.

Methods: From a prospective, single institution observational registry of children diagnosed with CD, we identified children with perianal perforating CD, defined as perianal abscesses and/or fistulae.

Multiphoton microscopy to identify and characterize the transition zone in a mouse model of Hirschsprung disease.

Background: The distribution of ganglion cells in the transition zone of Hirschsprung Disease (HD) colons is extremely variable. Determining the resection margin based on intraoperative biopsies may be imprecise. Multiphoton microscopy (MPM) is a novel imaging technology with the ability to visualize tissues in real time.

Evaluation of a video telescopic operating microscope (VITOM) for pediatric surgery and urology: a preliminary report.

Introduction: Optical magnification is an essential tool in the practice of pediatric surgery. Magnifying loupes are the most frequently used instrument, although their use often comes at the expense of neck pain experienced by the operating surgeon. Recent advances have led to the development of a compact video microscope (VITOM(®); Karl Storz Endoscopy GmbH, Tuttlingen, Germany) that displays high-definition magnified images on a flat screen.

Laparoscopic common bile duct exploration in children is associated with decreased cost and length of stay: results of a two-center analysis.

Purpose: Our aim was to compare outcomes of children undergoing laparoscopic cholecystectomy with laparoscopic common bile duct exploration (LC+CBDE) to those undergoing laparoscopic cholecystectomy with adjunctive endoscopic retrograde cholangiopancreatography (LC+ERCP).

Methods: We performed a two-center retrospective chart review of all children (<18 years) undergoing LC+CBDE or LC+ERCP between January 2000 and July 2011. Wilcoxon test was performed on continuous variables and logistic regression modeling on categorical data.

Hirschsprung-associated enterocolitis: prevention and therapy.

Hirschsprung-associated enterocolitis remains the greatest cause of morbidity and mortality in children with Hirschsprung disease. This chapter details the various approaches used to treat and prevent this disease process. This includes prevention of complications, such as stricture formation, prophylaxis with rectal washouts, and identification of high-risk individuals.

Duodenal necrosis secondary to a closed-loop bowel obstruction with novel reconstruction.

Duodenal necrosis is rare in children and presents a significant surgical challenge. We describe a 12-year-old girl who presented with duodenal necrosis secondary to a closed-loop bowel obstruction. We describe the diagnostic images, operative findings, and a novel reconstruction technique.

Splenic lymphopenia in the endothelin receptor B-null mouse: implications for Hirschsprung associated enterocolitis.

Purpose: The aim of the study was to describe and characterize a novel small spleen phenotype with splenic lymphopenia in the Ednrb-null (Ednrb-/-) mouse with aganglionosis known to also develop enterocolitis.

Methods: We compared spleen weight as a percent of body weight from Ednrb+/+, Ednrb+/-, and Ednrb-/- mice to quantify our initial observation. Splenic microarchitecture of Ednrb+/+ and Ednrb-/- mice was assessed using both H and E staining and immunofluorescence staining for CD45R+ (B cells) and CD3+ (T cells) on tissue sections.

Murine model of Hirschsprung-associated enterocolitis. I: phenotypic characterization with development of a histopathologic grading system.

Purpose: The aim of the study was to characterize enterocolitis in the Ednrb-null (Ednrb-/-) mouse with aganglionosis of the colon and to develop and validate a semiquantitative histopathologic grading system to assess enterocolitis.

Methods: We isolated colon and ileal specimens of Ednrb-/- and control mice (Ednrb+/+) and performed histochemical staining (H&E) on tissue sections. After establishing inflammation grading criteria, 2 blinded pathologists independently assessed the severity and depth of inflammation of proximal colon segments on 2 separate occasions.