Ankush Gosain

Hirschsprung’s Associated Enterocolitis

Purpose of review Hirschsprungs disease (HSCR) is characterized by an absence of ganglion cells in the distal hindgut, extending from the rectum to a variable distance proximally, and results from a failure of cranial–caudal neural crest cell migration. Hirschsprungs-associated enterocolitis (HAEC) is a condition with classic manifestations that include abdominal distention, fever and foul-smelling stools, and is a significant and life-threatening complication of HSCR. The purpose of this review was to critically evaluate recent findings regarding the pathophysiology of HAEC. Recent findings Several recent studies have investigated the cause of HAEC in humans and mouse models. These studies suggest that alterations in the intestinal barrier, including goblet cell number and function, and Paneth cell function, impaired gastrointestinal mucosal immunity, including B-lymphocyte trafficking or function and secretory immunoglobulin A production, and dysbiosis of the intestinal microbiota may contribute to the development of HAEC. Summary Recent studies add to the body of literature, suggesting that the intestinal defects observed in HSCR are not restricted to the aganglionic segment but extend to the mucosal immune system within and beyond the gastrointestinal tract. Future studies further dissecting the mechanisms of HAEC and validating these findings in humans will allow for the development of directed therapeutic interventions.

Guidelines for the diagnosis and management of Hirschsprung-associated enterocolitis

BackgroundPatients 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.MethodsThe American Pediatric Surgical Association Board of Governors established a Hirschsprung Disease Interest Group. Group discussions, literature review, and expert consensus were then used to summarize the current state of knowledge regarding diagnosis, management, and prevention of Hirschsprung-associated enterocolitis (HAEC).ResultsGuidelines for the diagnosis of HAEC and its clinical grade, utilizing clinical history, physical examination findings, and radiographic findings, are presented. Treatment guidelines, including patient disposition, diet, antibiotics, rectal irrigations and surgery, are presented.ConclusionsClear, standardized definitions of Hirschsprung-associated enterocolitis and its treatment are lacking in the literature. This guideline serves as a first step toward standardization of diagnosis and management.Level of evidenceV.

Guidelines for the management of postoperative obstructive symptoms in children with Hirschsprung disease

AbstractAlthough most children with Hirschsprung disease ultimately do well, many experience a variety of ongoing problems after pull-through surgery. The most common include obstructive symptoms, soiling, enterocolitis and failure to thrive. The purpose of this guideline is to present a rational approach to the management of postoperative obstructive symptoms in children with Hirschsprung disease. The American Pediatric Surgical Association Board of Governors established a Hirschsprung Disease Interest Group. Group discussions, literature review and expert consensus were then used to summarize the current state of knowledge regarding causes, methods of diagnosis, and treatment approaches to children with obstructive symptoms following pull-through for Hirschsprung disease. Causes of obstructive symptoms post-pull-through include mechanical obstruction; persistent or acquired aganglionosis, hypoganglionosis, or transition zone pull-through; internal sphincter achalasia; disordered motility in the proximal intestine that contains ganglion cells; or functional megacolon caused by stool-holding behavior. An algorithm for the diagnosis and management of obstructive symptoms after a pull-through for Hirschsprung disease is presented. A stepwise, logical approach to the diagnosis and management of patients experiencing obstructive symptoms following pull-through for Hirschsprung disease can facilitate treatment. Level of evidence V.

Ovarian torsion in pediatric and adolescent patients: A systematic review

OBJECTIVE Ovarian torsion in pediatric patients is a rare event and is primarily managed by pediatric general surgeons. Torsion can be treated with detorsion of the ovary or oopherectomy. Oopherectomy is the most common procedure performed by pediatric general surgeons for ovarian torsion. The purpose of this systematic review by the American Pediatric Surgical Association Outcomes and Evidence Based Practice Committee was to examine evidence from the medical literature and provide recommendations regarding the optimal treatment of ovarian torsion. METHODS Using PRISMA guidelines, six questions were addressed by searching Medline, Cochrane, Embase Central and National clearing house databases using relevant search terms. Risks of ovarian detorsion including thromboembolism and malignancy, indications for oophoropexy, benefits of detorsion including recovery of function and subsequent fertility, and recommended surveillance after detorsion were evaluated. Consensus recommendations were derived for each question based on the best available evidence. RESULTS Ninety-six studies were included. Risks of ovarian detorsion such as thromboembolism and malignancy were reviewed, demonstrating minimal evidence for unknowingly leaving a malignancy behind in the salvaged ovary and no evidence in the literature of thromboembolic events after detorsion of a torsed ovary. There is no clear evidence supporting the benefit of oophoropexy after a single episode of ovarian torsion. The gross appearance of the ovary does not correlate with long-term ovarian viability or function. Pregnancies have occurred in patients after detorsion of an ovary both spontaneously and with harvested oocytes from previously torsed ovaries. The consensus recommendation for imaging surveillance following ovarian detorsion is an ultrasound at 3months postprocedure but sooner if there is a concern for malignancy. CONCLUSION There appears to be overwhelming evidence supporting ovarian detorsion rather than oopherectomy for the management of ovarian torsion in pediatric patients. Ovarian salvage is safe and is the preferred treatment for ovarian torsion. Most salvaged ovaries will maintain viability after detorsion. TYPE OF STUDY Systematic review of level 3-4 studies. LEVEL OF EVIDENCE 3-4.

Using bibliometrics to analyze the state of academic productivity in US pediatric surgery training programs

BACKGROUND The Accreditation Council for Graduate Medical Education (ACGME) Common Program Requirements state that faculty must establish and maintain an environment of inquiry and scholarship. Bibliometrics, the statistical analysis of written publications, assesses scientific productivity and impact. The goal of this study was to understand the state of scholarship at Pediatric Surgery training programs. METHODS Following IRB approval, Scopus was used to generate bibliometric profiles for US Pediatric Surgery training programs and faculty. Statistical analyses were performed. RESULTS Information was obtained for 430 surgeons (105 female) from 48 US training programs. The mean lifetime h-index/surgeon for programs was 14.4 +/- 4.7 (6 programs above 1 SD, 9 programs below 1 SD). The mean 5-yearh-index/surgeon for programs was 3.92 +/- 1.5 (7 programs above 1 SD, 8 programs below 1 SD). Programs accredited after 2000 had a lower lifetime h-index than those accredited before 2000 (p=0.0378). Female surgeons had a lower lifetime h-index (p<0.0001), 5-yearh-index (p=0.0049), and m-quotient (p<0.0001) compared to males. Mean lifetime h-index increased with academic rank (p<0.0001), with no gender differences beyond the assistant professor rank (p=NS). CONCLUSION Variability was identified based on institution, gender, and rank. This information can be used for benchmarking the academic productivity of faculty and programs and as an adjunct in promotion/tenure decisions. TYPE OF STUDY Original Research. LEVEL OF EVIDENCE n/a.

Ovarian masses in the child and adolescent: An American Pediatric Surgical Association Outcomes and Evidence-Based Practice Committee systematic review

BACKGROUND The treatment of ovarian masses in pediatric patients should balance appropriate surgical management with the preservation of future reproductive capability. Preoperative estimation of malignant potential is essential to planning an optimal surgical strategy. METHODS The American Pediatric Surgical Association Outcomes and Evidence-Based Practice Committee drafted three consensus-based questions regarding the evaluation and treatment of ovarian masses in pediatric patients. A search of PubMed, the Cochrane Library, and Web of Science was performed and Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were followed to identify articles for review. RESULTS Preoperative tumor markers, ultrasound malignancy indices, and the presence or absence of the ovarian crescent sign on imaging can help estimate malignant potential prior to surgical resection. Frozen section also plays a role in operative strategy. Surgical staging is useful for directing chemotherapy and for prognostication. Both unilateral oophorectomy and cystectomy have been used successfully for germ cell and borderline ovarian tumors, although cystectomy may be associated with higher rates of local recurrence. CONCLUSIONS Malignant potential of ovarian masses can be estimated preoperatively, and fertility-sparing techniques may be appropriate depending on the type of tumor. This review provides recommendations based on a critical evaluation of recent literature. TYPE OF STUDY Systematic review of level 1-4 studies. LEVEL OF EVIDENCE Level 1-4 (mainly 3-4).

Extracorporeal Membrane Oxygenation (ECMO) Risk Stratification in Newborns with Congenital Diaphragmatic Hernia (CDH)

BACKGROUND A means for early postnatal stratification of ECMO risk in CDH newborns could be used to comparatively assess the utilization and outcomes of ECMO use between centers. While multiple CDH mortality risk calculators are available, no validated tool exists specifically for prediction of ECMO use. The purpose of this study was to derive and validate an ECMO risk stratification model. METHODS The study population was obtained from CDH Study Group registry for the period between 2007 and 2016. Only centers offering ECMO were included. The cohort was restricted to ECMO candidates and then divided into derivation and validation sets. Using all relevant perinatal predictors in the registry, univariate analysis was performed for the composite outcome of ECMO use or death without ECMO use. The model was derived using the derivation cohort with multivariable logistic regression and automatic stepwise forward selection (P < 0.05 for qualifying variables), and a c-statistic was obtained. The model was then tested on the validation cohort. Sample reuse validation and bootstrap validation were performed. The validated model was then tested for accuracy on CDH subgroups. RESULTS There were 1992 patients in the derivation cohort. Four significant variables were identified in the final ECMO risk model: 1-min and 5-min Apgar scores and highest and lowest post-ductal partial pressure of CO2 during the first 24 h of life. The model c-statistic was 0.824 which was confirmed with cross-validation and bootstrap optimism correction. The validation cohort c-statistic was 0.823 (N = 993). The model had good discrimination for left and right CDH, inborn and outborn patients, patients born before and after 2011, and high and low volume centers. The model performed significantly better for postnatally diagnosed patients. CONCLUSIONS This study represents proof-of-concept that a risk model can accurately estimate the probability of ECMO use in CDH newborns. This stratification could assist centers as a metric for assessment of ECMO usage and outcomes. Refinement and prospective validation of this model should be carried out prior to clinical application. LEVEL OF EVIDENCE 3.

Association of Comorbidities With Adverse Outcomes After Enterostomy Closure in Premature Neonates

This study uses the 2012-2015 American College of Surgeons National Surgical Quality Improvement Program–Pediatric database to examine the safety of enterostomy closure in premature neonates weighing less than 2 kg and identify risk factors for adverse outcomes.

What's new in critical illness and injury science? Case reports: The first step on a path toward cure

Case reports, brief scientific manuscripts which present the salient details of an interesting or extraordinary case, its diagnosis and management, and a discussion of relevant literature, are often denigrated by the scientific community. Claims are made by their detractors that case reports are the lowest possible level of “evidence” in evidence‐based practice that they do not truly add to the scientific literature, they are the realm of those that have nothing novel to study and report, or they are merely padding for one’s curriculum vitae. However, while these claims may be true in selected instances, case reports often have distinct value and may serve as the first step on a path toward achieving cures for human disease.

Time to appendectomy for acute appendicitis: A systematic review

OBJECTIVE The goal of this systematic review by the American Pediatric Surgical Association Outcomes and Evidence-Based Practice Committee was to develop recommendations regarding time to appendectomy for acute appendicitis in children within the context of preventing adverse events, reducing cost, and optimizing patient/parent satisfaction. METHODS The committee selected three questions that were addressed by searching MEDLINE, Embase, and the Cochrane Library databases for English language articles published between January 1, 1970 and November 3, 2016. Consensus recommendations for each question were made based on the best available evidence for both children and adults. RESULTS Based on level 3-4 evidence, appendectomy performed within 24h of admission in patients with acute appendicitis does not appear to be associated with increased perforation rates or other adverse events. Based on level 4 evidence, time from admission to appendectomy within 24h does not increase hospital cost or length of stay (LOS). Data are currently limited to determine an association between the timing of appendectomy and parent/patient satisfaction. CONCLUSIONS There is a paucity of high-quality evidence in the literature regarding timing of appendectomy for patients with acute appendicitis and its association with adverse events or resource utilization. Based on available evidence, appendectomy performed within the first 24h from presentation is not associated with an increased risk of perforation or adverse outcomes. TYPE OF STUDY Systematic Review of Level 1-4 studies.