Cynthia D. Downard

Abdominal wall defects.

Survival for newborns with congenital abdominal wall defects (primarily omphalocele and gastroschisis) has improved, but controversy remains regarding etiology, anatomy and embryology, the role of prenatal diagnosis and mode of delivery, and initial management. A number of recent studies have added to our knowledge and understanding of several of these topics, while several others have raised questions regarding traditional initial management of these infants. Continued improvement in the survival of these infants can be anticipated with further understanding of the in utero and antepartum diagnosis and management of infants with these common congenital abnormalities.

Treatment of necrotizing enterocolitis: an American Pediatric Surgical Association Outcomes and Clinical Trials Committee systematic review

OBJECTIVE The optimal treatment of necrotizing enterocolitis (NEC) is a common challenge for pediatric surgeons. Although many studies have evaluated prevention and medical therapy for NEC, few guidelines for surgical care exist. The aim of this systematic review is to review and evaluate the currently available evidence for the surgical care of patients with NEC. METHODS Data were compiled from a search of PubMed, OVID, the Cochrane Library database, and Web of Science from January 1985 until December 2011. Publications were screened, and their references were hand-searched to identify additional studies. Clinicaltrials.gov was also searched to identify ongoing or unpublished trials. The American Pediatric Surgical Association Outcomes and Clinical Trials Committee proposed six questions deemed pertinent to the surgical treatment of NEC. Recent Cochrane Reviews examined three of these topics; a literature review was performed to address the additional three specific questions. RESULTS The Cochrane Reviews support the use of prophylactic probiotics in preterm infants less than 2500 grams to reduce the incidence of NEC, as well as the use of human breast milk rather than formula when possible. There is no clear evidence to support delayed initiation or slow advancement of feeds. For surgical treatment of NEC with perforation, there is no clear support of peritoneal drainage versus laparotomy. Similarly, there is a lack of evidence comparing enterostomy versus primary anastomosis after resection at laparotomy. There are little data to determine the length of treatment with antibiotics to prevent recurrence of NEC. CONCLUSION Based on available evidence, probiotics are advised to decrease the incidence of NEC, and human milk should be used when possible. The other reviewed questions are clinically relevant, but there is a lack of evidence-based data to support definitive recommendations. These areas of NEC treatment would benefit from future investigation.

The diagnosis and management of empyema in children: a comprehensive review from the APSA Outcomes and Clinical Trials Committee.

The aim of this study is to review the current evidence on the diagnosis and management of empyema. The American Pediatric Surgical Association Outcomes and Clinical Trials Committee compiled 8 questions to address. A comprehensive review was performed on each topic. Topics included the distinction between parapneumonic effusion and empyema, the optimal imaging modality in evaluating pleural space disease, when and how pleural fluid should be managed, the first treatment option and optimal timing in the management of empyema, the optimal chemical debridement agent for empyema, therapeutic options if chemical debridement fails, therapy for parenchymal abscess or necrotizing pneumonia and duration of antibiotic therapy after an intervention. The evidence was graded for each topic to provide grade of recommendation where appropriate.

Strategies for the prevention of central venous catheter infections: an American Pediatric Surgical Association Outcomes and Clinical Trials Committee systematic review

PURPOSE The aim of this study is to review the current evidence-based data regarding strategies for prevention of central venous catheter (CVC) infections at the time of catheter insertion and as a part of routine care. METHODS We conducted a PubMed search from January 1990 to November 2010 using the following keywords: central venous catheter, clinical trials, pediatric, infection, prevention, antibiotic, chlorhexidine, dressing, antiseptic impregnated catheters, ethanol lock, impregnated cuff, insertion site infection, and Cochrane systematic review. Seven questions, selected by the American Pediatric Surgical Association Outcomes and Clinical Trials Committee, were addressed. RESULTS Thirty-six studies were selected for detailed review based on the strength of their study design and relevance to our 7 questions. These studies provide evidence that (1) chlorhexidine skin prep and chlorhexidine-impregnated dressing can decrease CVC colonization and bloodstream infection, (2) use of heparin and antibiotic-impregnated CVCs can decrease CVC colonization and bloodstream infection, and (3) ethanol and vancomycin lock therapy can reduce the incidence of catheter-associated bloodstream infections. CONCLUSION Grade A and B recommendations can be made based on available evidence in adult and limited pediatric studies for multiple components of proper CVC insertion practices and subsequent management. These strategies can minimize the risk of CVC infections in pediatric patients.

Current therapy of infants with congenital diaphragmatic hernia

Congenital diaphragmatic hernia (CDH) is a life-threatening anomaly with a significant mortality rate. Despite widespread prenatal diagnosis, few parameters have been well defined to aid in prediction of outcome of these infants. Antenatal maternal steroid administration and foetal surgery are not proven interventions. Postnatal treatment has changed over the last 10 years, with avoidance of hyperventilation and ventilator-induced lung injury resulting in improved survival. Therapies such as inhaled nitric oxide, exogenous surfactant administration and extracorporeal membrane oxygenation (ECMO) have undergone limited study, but show no clear benefit in this population. With improved outcome, principally due to avoidance of barotrauma, greater opportunity exists for long-term evaluation of survivors. To date, continuing problems with pulmonary function, nutrition and growth, effects of right ventricular hypertension and developmental issues have been identified. Through co-ordinated, multidisciplinary evaluation of CDH survivors, improved long-term outcome for these challenging patients can be attained.

Management of congenital diaphragmatic hernia: A systematic review from the APSA outcomes and evidence based practice committee

OBJECTIVE Variable management practices complicate the identification of optimal strategies for infants with congenital diaphragmatic hernia (CDH). This review critically appraises the available evidence to provide recommendations. METHODS Six questions regarding CDH management were generated. English language articles published between 1980 and 2014 were compiled after searching Medline, Cochrane, Embase and Web of Science. Given the paucity of literature on the subject, all studies irrespective of their rank in the levels of evidence hierarchy were included. RESULTS Gentle ventilation with permissive hypercapnia provides the best outcomes. Initial high frequency ventilation may be considered but its overall efficacy is unproven. Routine inhaled nitric oxide (iNO) or other medical adjuncts for acute, severe pulmonary hypertension demonstrate no benefit. Evidence does not support routine administration of pre- or postnatal glucocorticoids. Mode of extracorporeal membrane oxygenation (ECMO) has little bearing on outcomes. While the overall timing of repair does not impact outcomes, early repair on ECMO has benefits. Open repair leads to significantly fewer recurrences. Polytetrafluoroethylene (PTFE) is the most durable patch repair material. CONCLUSIONS Limited high-level evidence prevents the development of robust management guidelines for CDH. Prospective, multi-institutional studies are needed to identify best practices and optimize outcomes.

Asymptomatic malrotation: Diagnosis and surgical management An American Pediatric Surgical Association outcomes and evidence based practice committee systematic review

OBJECTIVE Patients with malrotation, or an intestinal rotation abnormality (IRA), can experience serious adverse events. Increasingly, asymptomatic patients are being diagnosed with malrotation incidentally. Patients with symptomatic malrotation require surgery in an urgent or semiurgent manner to address their symptoms. The treatment of asymptomatic or incidentally discovered malrotation remains controversial. METHODS Data were compiled from a broad search of Medline, Cochrane, Embase and Web of Science from January 1980 through January 2013 for five questions regarding asymptomatic malrotation. RESULTS There is minimal evidence to support screening asymptomatic patients. Consideration may be given to operate on asymptomatic patients who are younger in age, while observation may be appropriate in the older patient. If reliably diagnosed, atypical malrotation with a broad-based mesentery and malposition of the duodenum can be observed. Regarding diagnostic imaging, the standard of care for diagnosis remains the upper gastrointestinal contrast study (UGI), ultrasound may be useful for screening. A laparoscopic approach is safe for diagnosis and treatment of rotational abnormalities. Laparoscopy can aid in determining whether a patient has true malrotation with a narrow mesenteric stalk, has nonrotation and minimal risk for volvulus, or has atypical anatomy with malposition of the duodenum. It is reasonable to delay Ladd procedures until after palliation on patients with severe congenital heart disease. Observation can be considered with extensive education for family and caregivers and close clinical follow-up. CONCLUSIONS There is a lack of quality data to guide the management of patients with asymptomatic malrotation. Multicenter and prospective data should be collected to better assess the risk profile for this complex group of patients. A multidisciplinary approach involving surgery, cardiology, critical care and the patients caregivers can help guide a watchful waiting management plan in individual cases.

Perioperative management and outcomes of esophageal atresia and tracheoesophageal fistula

BACKGROUND/PURPOSE Esophageal atresia/tracheoesophageal fistula (EA/TEF) is a rare congenital anomaly lacking contemporary data detailing patient demographics, medical/surgical management and outcomes. Substantial variation in the care of infants with EA/TEF may affect both short- and long-term outcomes. The purpose of this study was to characterize the demographics, management strategies and outcomes in a contemporary multi-institutional cohort of infants diagnosed with EA/TEF to identify potential areas for standardization of care. METHODS A multi-institutional retrospective cohort study of infants with EA/TEF treated at 11 childrens hospitals between 2009 and 2014 was performed. Over the 5year period, 396 cases were identified in the 11 centers (7±5 per center per year). All infants with a diagnosis of EA/TEF made within 30days of life who had surgical repair of their defect defined as esophageal reconstruction with or without ligation of TEF within the first six months of life were included. Demographic, operative, and outcome data were collected and analyzed to detect associations between variables. RESULTS Prenatal suspicion or diagnosis of EA/TEF was present in 53 (13%). The most common anatomy was proximal EA with distal TEF (n=335; 85%) followed by pure EA (n=27; 7%). Clinically significant congenital heart disease (CHD) was present in 137 (35%). Mortality was 7.5% and significantly associated with CHD (p<0.0001). Postoperative morbidity occurred in 62% of the population, including 165 (42%) cases with anastomotic stricture requiring intervention, anastomotic leak in 89 (23%), vocal cord paresis/paralysis in 26 (7%), recurrent fistula in 19 (5%), and anastomotic dehiscence in 9 (2%). Substantial variation in practice across our institutions existed: bronchoscopy prior to repair was performed in 64% of cases (range: 0%-100%); proximal pouch contrast study in 21% (0%-69%); use of interposing material between the esophageal and tracheal suture lines in 38% (0%-69%); perioperative antibiotics ≥24h in 69% (36%-97%); and transanastomotic tubes in 73% (21%-100%). CONCLUSION Contemporary treatment of EA/TEF is characterized by substantial variation in perioperative management and considerable postoperative morbidity and mortality. Future studies are planned to establish best practices and clinical care guidelines for infants with EA/TEF. LEVEL OF EVIDENCE Type of study: Treatment study. Level IV.

Surgical management of gastroesophageal reflux disease (GERD) in children: A systematic review

OBJECTIVE The goal of this systematic review by the American Pediatric Surgical Association Outcomes and Evidence-Based Practice Committee was to derive recommendations from the medical literature regarding the surgical treatment of pediatric gastroesophageal reflux disease (GERD). METHODS Five questions were addressed by searching the MEDLINE, Cochrane, Embase, Central, and National Guideline Clearinghouse databases using relevant search terms. Consensus recommendations were derived for each question based on the best available evidence. RESULTS There was insufficient evidence to formulate recommendations for all questions. Fundoplication does not affect the rate of hospitalization for aspiration pneumonia, apnea, or reflux-related symptoms. Fundoplication is effective in reducing all parameters of esophageal acid exposure without altering esophageal motility. Laparoscopic fundoplication may be comparable to open fundoplication with regard to short-term clinical outcomes. Partial fundoplication and complete fundoplication are comparable in effectiveness for subjective control of GERD. Fundoplication may benefit GERD patients with asthma, but may not improve outcomes in patients with neurologic impairment or esophageal atresia. Overall GERD recurrence rates are likely below 20%. CONCLUSIONS High-quality evidence is lacking regarding the surgical management of GERD in the pediatric population. Definitive conclusions regarding the effectiveness of fundoplication are limited by patient heterogeneity and lack of a standardized outcomes reporting framework. TYPE OF STUDY Systematic review of level 1-4 studies. LEVEL OF EVIDENCE Level 1-4 (mainly level 3-4).

Prevention of infectious complications after elective colorectal surgery in children: an American Pediatric Surgical Association Outcomes and Clinical Trials Committee comprehensive review

OBJECTIVE This goal of this review was to examine the clinical evidence in support of commonly utilized measures intended to reduce complications following elective colorectal surgery. DATA SOURCE Literature searches were performed to identify relevant studies from Medline, PubMed, and Cochrane databases. STUDY SELECTION The American Pediatric Surgery Association Outcomes and Clinical Trials Committee selected eight questions to address this topic systematically in the context of three management areas: 1) appropriate utilization of systemic antibiotics for colorectal procedures, 2) reduction of stool burden through mechanical bowel preparation, and 3) intraluminal gut decontamination through use of enteral nonabsorbable antibiotics. Primary outcomes of interest included the occurrence of infectious and mechanical complications related to stool burden and intraluminal bacterial concentration (incisional surgical site infection, anastomotic leakage, and intraabdominal abscess). RESULTS The evidence in support of each management category was systematically reviewed, graded, and summarized in the context of the reviews primary outcomes. Practice recommendations were made as deemed appropriate by the committee. CONCLUSIONS Clinical evidence in support of interventions to reduce infectious complications following colorectal surgery is derived almost exclusively from the adult literature. High-quality evidence to guide clinical practice in children is sorely needed, as the available data may have only limited relevance to pediatric colorectal diseases.