Classification and Surgical Management of Anorectal Malformations: A Systematic Review and Evidence-based Guideline From the APSA Outcomes and Evidence-based Practice Committee.

OBJECTIVE: Treatment of neonates with anorectal malformations (ARMs) can be challenging due to variability in anatomic definitions, multiple approaches to surgical management, and heterogeneity of reported outcomes. The purpose of this systematic review is to summarize existing evidence, identify treatment controversies, and provide guidelines for perioperative care. METHODS: The American Pediatric Surgical Association Outcomes and Evidence Based Practice Committee (OEBP) drafted five consensus-based questions regarding management of children with ARMs. These questions were related to categorization of ARMs and optimal methods and timing of surgical management. A comprehensive search strategy was performed, and the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were used to perform the systematic review to attempt to answer five questions related to surgical care of ARM. RESULTS: A total of 10,843 publications were reviewed, of which 90 were included in final recommendations, and some publications addressed more than one question (question: 1 n = 6, 2 n = 63, n = 15, 4 n = 44). Studies contained largely heterogenous groups of ARMs, making direct comparison for each subtype challenging and therefore, no specific recommendation for optimal surgical approach based on outcomes can be made. Both loop and divided colostomy may be acceptable methods of fecal diversion for patients with a diagnosis of anorectal malformation, however, loop colostomies have higher rates of prolapse in the literature reviewed. In terms of timing of repair, there did not appear to be significant differences in outcomes between early and late repair groups. Clear and uniform definitions are needed in order to ensure similar populations of patients are compared moving forward. Recommendations are provided based primarily on A-D levels of evidence. CONCLUSIONS: Evidence-based best practices for ARMs are lacking for many aspects of care. Multi-institutional registries have made progress to address some of these gaps. Further prospective and comparative studies are needed to improve care and provide consensus guidelines for this complex patient population.

Screening and Prophylaxis for Venous Thromboembolism in Pediatric Surgery: A Systematic Review.

OBJECTIVE: The American Pediatric Surgical Association Outcomes and Evidence-Based Practice Committee conducted a systematic review to describe the epidemiology of venous thromboembolism (VTE) in pediatric surgical and trauma patients and develop recommendations for screening and prophylaxis. METHODS: The Medline (Ovid), Embase, Cochrane, and Web of Science databases were queried from January 2000 through December 2021. Search terms addressed the following topics: incidence, ultrasound screening, and mechanical and pharmacologic prophylaxis. The Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines were followed. Consensus recommendations were derived based on the best available literature. RESULTS: One hundred twenty-four studies were included. The incidence of VTE in pediatric surgical populations is 0.29% (Range = 0.1%-0.48%) and directly correlates with surgery type, transfusion, prolonged anesthesia, malignancy, congenital heart disease, inflammatory bowel disease, infection, and female sex. The incidence of VTE in pediatric trauma populations is 0.25% (Range = 0.1%-0.8%) and directly correlates with injury severity, major surgery, central line placement, body mass index, spinal cord injury, and length-of-stay. Routine ultrasound screening for VTE is not recommended. Consider sequential compression devices in at-risk nonmobile, pediatric surgical patients when an appropriate sized device is available. Consider mechanical prophylaxis alone or with pharmacologic prophylaxis in adolescents >15 y and post-pubertal children <15 y with injury severity scores >25. When utilizing pharmacologic prophylaxis, low molecular weight heparin is superior to unfractionated heparin. CONCLUSIONS: While VTE remains an infrequent complication in children, consideration of mechanical and pharmacologic prophylaxis is appropriate in certain populations. TYPE OF STUDY: Systematic Review of level 2-4 studies. LEVEL OF EVIDENCE: Level 3-4.

Management and Outcomes of Pediatric Lymphatic Malformations: A Systematic Review From the APSA Outcomes and Evidence-Based Practice Committee.

BACKGROUND: Significant variation in management strategies for lymphatic malformations (LMs) in children persists. The goal of this systematic review is to summarize outcomes for medical therapy, sclerotherapy, and surgery, and to provide evidence-based recommendations regarding the treatment. METHODS: Three questions regarding LM management were generated according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). Publicly available databases were queried to identify articles published from January 1, 1990, to December 31, 2021. A consensus statement of recommendations was generated in response to each question. RESULTS: The initial search identified 9326 abstracts, each reviewed by two authors. A total of 600 abstracts met selection criteria for full manuscript review with 202 subsequently utilized for extraction of data. Medical therapy, such as sirolimus, can be used as an adjunct with percutaneous treatments or surgery, or for extensive LM. Sclerotherapy can achieve partial or complete response in over 90% of patients and is most effective for macrocystic lesions. Depending on the size, extent, and location of the malformation, surgery can be considered. CONCLUSION: Evidence supporting best practices for the safety and effectiveness of management for LMs is currently of moderate quality. Many patients benefit from multi-modal treatment determined by the extent and type of LM. A multidisciplinary approach is recommended to determine the optimal individualized treatment for each patient.

Rib spur causing a hemothorax, pneumothorax, and diaphragmatic injury in a pediatric patient.

Pleural effusion is a relatively common condition encountered in the pediatric emergency department. Evaluation of pleural effusion in the emergency department typically includes advanced imaging such as computer tomography or ultrasound, as well as diagnostic thoracocentesis. We report a case of a 10-year-old female with a rib spur at the anterolateral left sixth rib that caused a hemothorax, pneumothorax, and diaphragmatic injury. The patient underwent video-assisted thoracoscopic surgery and resection of the rib spur. The procedure was well-tolerated without any complications.

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. The primary outcome was frequency of postoperative respiratory complications, including: high-flow nasal cannula/non invasive ventilation, reintubation, pneumonia, Extracorporeal Membrane Oxygenation (ECMO), and 30-day respiratory-related readmissions or emergency department (ED) visits. Univariate analyses were used to evaluate associations between patient and procedure characteristics and stratified analyses by symptoms were performed examining incidence of complications. RESULTS: Of 266 SARS-CoV-2 positive patients, 163 (61.7%) were male, and the median age was 10 years (interquartile range 4-14). The majority of procedures were emergent or urgent (n = 214, 80.5%). The most common procedures were appendectomies (n = 78, 29.3%) and fracture repairs (n = 40,15.0%). 13 patients (4.9%) had preoperative symptoms including cough or dyspnea. 26 patients (9.8%) had postoperative respiratory complications, including 15 requiring high-flow oxygen, 8 with pneumonia, 4 requiring non invasive ventilation, 3 respiratory ED visits, and 2 respiratory readmissions. Respiratory complications were more common among symptomatic patients than asymptomatic patients (30.8% vs. 8.7%, p = 0.01). Higher ASA class and comorbidities were also associated with postoperative respiratory complications. CONCLUSIONS: Postoperative respiratory complications are less common in asymptomatic versus symptomatic SARS-COV-2 positive children. Relaxation of COVID-19-related restrictions for time-sensitive, non urgent procedures in selected asymptomatic patients may be reasonably considered. Additionally, further research is needed to evaluate the costs and benefits of routine testing for asymptomatic patients. LEVEL OF EVIDENCE: Iii, Respiratory complications.

Ethics in the Era of COVID-19.

The COVID-19 pandemic has led to new ethical challenges and exposed or exacerbated others that were already present. Through the lens of pediatric surgery, this article aims to discuss issues that have been impacted by the pandemic including triage of care and allocation of scarce resources, equity and access to care, and a physician's competing responsibilities to their patients, families, and selves.

Effects of a Patient Activation Tool on Decision Making Between Surgery and Nonoperative Management for Pediatric Appendicitis: A Randomized Clinical Trial.

Importance: Strategies to activate and engage patients and caregivers in shared decision making in the acute care setting may result in improved outcomes. Objective: To determine whether a patient activation tool (PAT) can improve decision-making and patient-centered outcomes among pediatric patients and their caregivers who choose between surgery and nonoperative management for their child's appendicitis. Design, Setting, and Participants: This single-blind, randomized clinical trial collected data from a single tertiary children's hospital from March 1, 2014, through April 30, 2016, with 1-year follow-up completed on May 1, 2017. Two hundred of 236 eligible children and adolescents aged 7 to 17 years with uncomplicated appendicitis enrolled with their caregivers. After receiving the randomized clinical intervention, caregivers chose surgery or nonoperative management. Data were analyzed from March 1, 2014, through May 1, 2017. Interventions: Randomization to a scripted standardized surgical consultation that emphasized patient choice or a scripted standardized surgical consultation plus the PAT (a tablet-based tool that presents each treatment, encourages participation in medical decision making, and aims at alleviating decisional uncertainty). Main Outcomes and Measures: Decisional self-efficacy immediately after treatment decision, health care satisfaction at discharge, and disability days for the child at 1-year follow-up. Results: Among 200 participants (median age, 12 years [interquartile range (IQR), 9-15 years]; 120 [60.0%] male), 98 were randomized to the PAT and 102 to the standardized consultation groups. The percentages choosing nonoperative management were similar (standardized consultation group, 42 of 102 [41.2%]; PAT group, 31 of 98 [31.6%]; P = .19). Immediate decisional self-efficacy was similar in the standardized consultation and PAT groups (median score, 100 [IQR, 97.7-100] vs 100 [IQR, 95.5-100]; P = .03), which was not significant at the planned significance level of P = .02. Total scores on health care satisfaction at discharge were similar (median, 99 [IQR, 94.7-100] vs 98 [IQR, 91.7-100]; P = .27). Disability days at 1-year follow-up were also similar (median, 6 [IQR, 2-11] vs 5 [IQR, 2-15]; P = .67). No difference in the failure rate of nonoperative management at 1 year (13 of 38 [34.2%] vs 11 of 30 [36.7%]; P > .99) or in the rate of complicated appendicitis 30 days after discharge (7 of 68 [10.3%] vs 9 of 71 [12.7%]; P = .79) occurred. Conclusions and Relevance: In this study, a technology-based PAT did not improve measures of decision making for pediatric patients and caregivers needing to make an urgent treatment decision between surgery and nonoperative management for appendicitis. However, the overall high scores in both groups suggest that pediatric patients and caregivers can process information in the acute care setting and effectively participate in an informed shared decision-making process around the need for surgery. Trial Registration: ClinicalTrials.gov identifier: NCT02110485.

High failure rate of nonoperative management of acute appendicitis with an appendicolith in children.

BACKGROUND: The purpose of this study was to investigate the feasibility of nonoperative management of acute appendicitis in children with an appendicolith identified on preoperative imaging. STUDY DESIGN: We performed a prospective nonrandomized trial of nonoperative management of uncomplicated acute appendicitis with an appendicolith in children aged 7 to 17years. The primary outcome was the failure rate of nonoperative management, defined as having undergone an appendectomy. Early termination was set to occur if the lower limit of the 95% confidence interval of the failure rate was greater than 20% at 30days or 30% at 1year. RESULTS: Recruitment for this study was halted after enrollment of 14 patients (N=5 nonoperative; N=9 surgery). The failure rate of nonoperative management was 60% (3/5) at a median follow-up of 4.7months (IQR 1.0-7.6) with a 95% CI of 23%-88%. None of the three patients that failed nonoperative management had complicated appendicitis at the time of appendectomy, while six out of nine patients who chose surgery had complicated appendicitis (0/3 vs. 6/9, p=0.18). The trial was stopped for concerns over patient safety. CONCLUSIONS: Nonoperative management of acute appendicitis with an appendicolith in children resulted in an unacceptably high failure rate.

Effectiveness of Patient Choice in Nonoperative vs Surgical Management of Pediatric Uncomplicated Acute Appendicitis.

IMPORTANCE: Current evidence suggests that nonoperative management of uncomplicated appendicitis is safe, but overall effectiveness is determined by combining medical outcomes with the patient's and family's perspective, goals, and expectations. OBJECTIVE: To determine the effectiveness of patient choice in nonoperative vs surgical management of uncomplicated acute appendicitis in children. DESIGN, SETTING, AND PARTICIPANTS: Prospective patient choice cohort study in patients aged 7 to 17 years with acute uncomplicated appendicitis presenting at a single pediatric tertiary acute care hospital from October 1, 2012, through March 6, 2013. Participating patients and families gave informed consent and chose between nonoperative management and urgent appendectomy. INTERVENTIONS: Urgent appendectomy or nonoperative management entailing at least 24 hours of inpatient observation while receiving intravenous antibiotics and, on demonstrating improvement of symptoms, completion of 10 days of treatment with oral antibiotics. MAIN OUTCOMES AND MEASURES: The primary outcome was the 1-year success rate of nonoperative management. Successful nonoperative management was defined as not undergoing an appendectomy. Secondary outcomes included comparisons of the rates of complicated appendicitis, disability days, and health care costs between nonoperative management and surgery. RESULTS: A total of 102 patients were enrolled; 65 patients/families chose appendectomy (median age, 12 years; interquartile range [IQR], 9-13 years; 45 male [69.2%]) and 37 patients/families chose nonoperative management (median age, 11 years; IQR, 10-14 years; 24 male [64.9%]). Baseline characteristics were similar between the groups. The success rate of nonoperative management was 89.2% (95% CI, 74.6%-97.0%) at 30 days (33 of 37 children) and 75.7% (95% CI, 58.9%-88.2%) at 1 year (28 of 37 children). The incidence of complicated appendicitis was 2.7% in the nonoperative group (1 of 37 children) and 12.3% in the surgery group (8 of 65 children) (P = .15). After 1 year, children managed nonoperatively compared with the surgery group had fewer disability days (median [IQR], 8 [5-18] vs 21 [15-25] days, respectively; P < .001) and lower appendicitis-related health care costs (median [IQR], $4219 [$2514-$7795] vs $5029 [$4596-$5482], respectively; P = .01). CONCLUSIONS AND RELEVANCE: When chosen by the family, nonoperative management is an effective treatment strategy for children with uncomplicated acute appendicitis, incurring less morbidity and lower costs than surgery. TRIAL REGISTRATION: clinicaltrials.gov Identifier: NCT01718275.

A comparison of pediatric gastrostomy tube placement techniques.

PURPOSE: This study aimed to compare different techniques for placement of gastrostomy tubes in a pediatric population. STUDY DESIGN: A retrospective review was performed for patients less than 18 years of age who underwent gastrostomy tube placement at a single academic children's hospital between 2010 and 2012. Techniques for gastrostomy placement included Open Stamm, percutaneous endoscopic gastrostomy (PEG), fluoroscopy guided, laparoscopic, and laparoscopic assisted PEG. Pre-operative characteristics of patients and post-operative outcomes were compared between techniques. RESULTS: Most patients underwent an Open Stamm (43 %) or PEG (39 %). There were significant differences between groups with respect to primary diagnoses, prior surgeries, and ASA classification. Major complications were rare, with less than 3 % requiring reoperation within 30 days; however, minor complications and returns to the emergency department were common. Unintentional tube dislodgements occurred in 22 % of all patients, with Open Stamm technique identified as an independent predictor of unintentional dislodgement (p < 0.0001). CONCLUSIONS: Although conclusions from this retrospective analysis are limited due to heterogeneity between groups, open Stamm gastrostomy placement in children was associated with increased negative outcomes including unintentional tube dislodgements, returns to the emergency department, and need for reoperation within 30 days. Prospective analysis of the various techniques is needed to confirm that minimally invasive techniques for gastrostomy tube placement are associated with a less complicated post-operative course.

Sacral nerve stimulation: a promising therapy for fecal and urinary incontinence and constipation in children.

PURPOSE: This study describes our series of children with bowel and bladder dysfunction (BDD) treated with sacral nerve stimulation in order to begin to identify characteristics associated with better outcomes and guide future therapies. METHODS: Between May 2012 and February 2014, 29 patients were evaluated before and after sacral nerve stimulator (SNS) placement. A prospective data registry was developed that contains clinical information and patient-reported measures: Fecal Incontinence Qualify of Life Scale, Fecal Incontinence Severity Scale, PedsQL Gastrointestinal Symptom Scale, and Vancouver DES Symptom Scale. RESULTS: The median age of patients was 12.1 (interquartile range: 9.4, 14.3) years and the median follow-up period was 17.7 (12.9, 36.4) weeks. 93% had GI complaints and 65.5% had urinary symptoms while 7% had urologic symptoms only. The most common etiologies of BBD were idiopathic (66%) and imperforate anus (27%). Five patients required reoperation due to a complication with battery placement. Six of 11 patients (55%) with a pre-SNS cecostomy tube no longer require an antegrade bowel regimen as they now have voluntary bowel movements. Ten of eleven patients (91%) no longer require anticholinergic medications for bladder overactivity after receiving SNS. Significant improvements have been demonstrated in all four patient-reported instruments for the overall cohort. CONCLUSIONS: Early results have demonstrated improvements in both GI and urinary function after SNS placement in pediatric patients with bowel and bladder dysfunction.

Early versus delayed surgical correction of malrotation in children with critical congenital heart disease.

PURPOSE: The purpose of this study was to compare outcomes between early and delayed surgical correction of malrotation in children with critical congenital heart disease (CHD). METHODS: Patients with CHD who underwent cardiac surgery by 1 year of age and had malrotation diagnosed during their initial admission at 34 hospitals contributing to the Pediatric Health Information System in 2004-2009 were included. Ladd's procedures performed during the first admission were considered early correction, and those at a subsequent admission were considered delayed. Interhospital variability in the proportion of patients undergoing delayed correction was assessed, and outcomes were compared between the groups. RESULTS: Of the 324 patients identified, 85.2% underwent early correction. Significant variability existed in the proportion of patients undergoing delayed correction across hospitals (p<0.0001). Baseline characteristics, including severity of CHD, were similar between the groups. In the delayed group, 27% of patients underwent a Ladd's procedure during an urgent or emergent admission, but none had volvulus or underwent intestinal resection. Rates of mortality and readmission within 1 year of malrotation diagnosis were similar in both groups. Chart validation confirmed 100% accuracy of diagnosis and treatment group assignment. CONCLUSIONS: In patients with critical CHD, delayed operative intervention for malrotation without volvulus may be a reasonable alternative.

Does timing of neonatal inguinal hernia repair affect outcomes?

PURPOSE: The purpose of this study was to examine practice variability and compare outcomes between early and delayed neonatal inguinal hernia repair (IHR). METHODS: Patients admitted to neonatal intensive care units with a diagnosis of IH who underwent IHR by age 1 year in the Pediatric Health Information System from 1999 to 2011 were included. IHR after the index hospitalization was considered delayed. Inter-hospital variability in the proportion of delayed repairs and differences in outcomes for each group were compared. A propensity score matched analysis was performed to account for baseline differences between treatment groups. RESULTS: Of the 2030 patients identified, 32.9% underwent delayed IHR with significant variability in the proportion of patients having delayed repair across hospitals (p<0.0001). More patients in the delayed group had a congenital anomaly or received life supportive measures prior to IHR (all p<0.01), and 8.2% of patients undergoing delayed repair had a diagnosis of incarceration at repair. More patients in the early group underwent reoperation for hernia within 1 year (5.9% vs. 3.7%, p=0.02). Results were similar after performing a propensity score matched analysis. CONCLUSIONS: Significant variability in practice exists between children's hospitals in the timing of IHR, with delayed repair associated with incarceration and early repair with a higher rate of reoperation.

Variability in noncardiac surgical procedures in children with congenital heart disease.

BACKGROUND: The purpose of this study was to examine the volume and variability of noncardiac surgeries performed in children with congenital heart disease (CHD) requiring cardiac surgery in the first year of life. METHODS: Patients who underwent cardiac surgery by 1 year of age and had a minimum 5-year follow-up at 22 of the hospitals contributing to the Pediatric Health Information System database between 2004 and 2012 were included. Frequencies of noncardiac surgical procedures by age 5 years were determined and categorized by subspecialty. Patients were stratified according to their maximum RACHS-1 (Risk Adjustment in Congenital Heart Surgery) category. The proportions of patients across hospitals who had a noncardiac surgical procedure for each subspecialty were compared using logistic mixed effects models. RESULTS: 8857 patients underwent congenital heart surgery during the first year of life, 3621 (41%) of whom had 13,894 noncardiac surgical procedures by 5 years. Over half of all procedures were in general surgery (4432; 31.9%) or otolaryngology (4002; 28.8%). There was significant variation among hospitals in the proportion of CHD patients having noncardiac surgical procedures. Compared to children in the low risk group (RACHS-1 categories 1-3), children in the high-risk group (categories 4-6) were more likely to have general, dental, orthopedic, and thoracic procedures. CONCLUSIONS: Children with CHD requiring cardiac surgery frequently also undergo noncardiac surgical procedures; however, considerable variability in the frequency of these procedures exists across hospitals. This suggests a lack of uniformity in indications used for surgical intervention. Further research should aim to better standardize care for this complex patient population.

Single-stage versus multi-stage pull-through for Hirschsprung's disease: practice trends and outcomes in infants.

PURPOSE: The aim of this study was to evaluate surgical treatments and outcomes in a multi-institutional cohort of neonates with Hirschsprung's disease (HD). METHODS: Using the Pediatric Health Information System (PHIS) from 1999 to 2009, neonates diagnosed with HD were identified and classified as having a single stage pull-through (SSPT) or multi-stage pull-through (MSPT). Diagnosis and classification algorithms and clinical variables and outcomes were validated by multi-institutional chart review. Groups were compared using logistic regression modeling and propensity-score matched analysis to account for baseline differences between groups. RESULTS: 1555 neonates with HD were identified; 77.2% underwent SSPT and 22.8% underwent MSPT. Misclassification of disease or surgical treatment was <2%. Rates of SSPT increased over time (p=0.03). Compared to SSPT, patients undergoing MSPT had significantly lower birth weights and higher rates of prematurity, non-HD gastrointestinal anomalies, enterocolitis, and preoperative mechanical ventilation. Patients undergoing MSPT had significantly higher rates of readmissions (58.5 vs. 37.9%) and additional operations (38.7 vs. 26%). Results were consistent in the propensity-score matched analysis. CONCLUSION: Most neonates with HD undergo SSPT. In patients with similar observed baseline characteristics, MSPT was associated with worse outcomes suggesting that some infants currently selected to undergo MSPT may have better outcomes with SSPT. However, there remains a subgroup of MSPT patients who were too ill to be adequately compared to SSPT patients; for this subgroup of severely ill infants with HD, MSPT may be the best option.

Morbidity and mortality in patients with esophageal atresia.

BACKGROUND: This study reports national estimates of population characteristics and outcomes for patients with esophageal atresia with or without tracheoesophageal fistula (EA/TEF) and evaluates the relationships between hospital volume and outcomes. METHODS: Patients admitted within 30 days of life who had International Classification of Diseases, 9th Edition, Clinical Modification diagnosis and procedure codes relevant to EA/TEF during 1999-2012 were identified with the Pediatric Health Information System database. Baseline demographics, comorbidities, and postoperative outcomes, including predictors of in-hospital mortality, were examined up to 2 years after EA/TEF repair. RESULTS: We identified 3,479 patients with EA/TEF treated at 43 children's hospitals; 37% were premature and 83.5% had ≥1 additional congenital anomaly, with cardiac anomalies (69.6%) being the most prevalent. Within 2 years of discharge, 54.7% were readmitted, 5.2% had a repeat TEF ligation, 11.4% had a repeat operation for their esophageal reconstruction, and 11.7% underwent fundoplication. In-hospital mortality was 5.4%. Independent predictors of mortality included lower birth weight, congenital heart disease, other congenital anomalies, and preoperative mechanical ventilation. There was no relationship between hospital volume and mortality or repeat TEF ligation. CONCLUSION: This study describes population characteristics and outcomes, including predictors of in-hospital mortality, in EA/TEF patients treated at children's hospitals across the United States. Across these hospitals, rates of mortality or repeat TEF ligation were not dependent on hospital volume.

Feasibility of a nonoperative management strategy for uncomplicated acute appendicitis in children.

BACKGROUND: For decades, urgent operation has been considered the only appropriate management of acute appendicitis in children. The purpose of this study was to investigate the feasibility of nonoperative management of uncomplicated acute appendicitis in children. STUDY DESIGN: A prospective nonrandomized clinical trial of children with uncomplicated acute appendicitis comparing nonoperative management with urgent appendectomy was performed. The primary result was 30-day success rate of nonoperative management. Secondary outcomes included comparisons of disability days, missed school days, hospital length of stay, and measures of quality of life and health care satisfaction. RESULTS: Seventy-seven patients were enrolled during October 2012 to October 2013; 30 chose nonoperative management and 47 chose surgery. There were no significant differences in demographic or clinical characteristics. The immediate and 30-day success rates of nonoperative management were 93% (28 of 30) and 90% (27 of 30). There was no evidence of progression of appendicitis to rupture at the time of surgery in the 3 patients for whom nonoperative management failed. Compared with the surgery group, the nonoperative group had fewer disability days (3 vs 17 days; p < 0.0001), returned to school more quickly (3 vs 5 days; p = 0.008), and exhibited higher quality of life scores in both the child (93 vs 88; p = 0.01) and the parent (96 vs 90; p = 0.03), but incurred a longer length of stay (38 vs 20 hours; p < 0.0001). CONCLUSIONS: Nonoperative management of uncomplicated acute appendicitis in children is feasible, with a high 30-day success rate and short-term benefits that include quicker recovery and improved quality of life scores. Additional follow-up will allow for determination of longer-term success rate, safety, and cost effectiveness.

Management of short bowel syndrome.

Most intestinal failure in children is due to short bowel syndrome (SBS) where congenital or acquired lesions have led to an extensive loss of intestinal mass. The vast majority of morbidity and mortality of patients with SBS is due to complications secondary to their long term dependence on parenteral nutrition. In response to SBS, the intestine undergoes a process of remodeling termed adaptation. Principles guiding the medical management of SBS include providing adequate parenteral nutrition, fluids and electrolytes for growth and normal development, promoting small bowel adaptation, and preventing and treating complications related to the patient's underlying disease and their parenteral nutrition. Catheter associated blood stream infection (CABSI) is major source of morbidity and mortality in patients with intestinal failure from SBS. Intestinal failure associated liver disease (IFALD)is another major source of morbidity and mortality in patients with SBS. IFALD is the most consistent negative predictor of outcome including death and continued parenteral nutrition dependence. Enteral nutrition is critical for intestinal adaptation and preventing IFALD. Patients with SBS who develop dilated dysmotile segments may benefit from autologous intestinal reconstruction surgery (AIRS) with the goal of decreasing stasis and disordered motility through intestinal narrowing and lengthening. Patients with SBS should be referred for transplantation if they have failed intestinal rehabilitation including AIRS, have no reasonable chance for enteral feeding tolerance, develop irreversible IFALD, have recurrent sepsis, or have exhausted their central venous access sites. With improvements in medical and surgical care, overall survival of patients with SBS now exceeds 90%.

Using the Pediatric Health Information System to study rare congenital pediatric surgical diseases: development of a cohort of esophageal atresia patients.

BACKGROUND/PURPOSE: Administrative databases include large multi-institutional cohorts of patients with rare congenital anomalies that can potentially be used to characterize these diseases and study variations in practice and outcomes. The purpose of this study was to develop a methodology to accurately identify a cohort of patients with a rare disease (esophageal atresia and tracheoesophageal fistula, EA/TEF) in the Pediatric Health Information System (PHIS) database. METHODS: Patients with EA/TEF treated from 2001 to 2010 were identified by chart review at two institutions and then located within the PHIS database to find ICD-9-CM coding patterns unique to EA/TEF. Subsequently, a step-wise search strategy for PHIS was developed to identify patients with EA/TEF: this included searching the ICD-9-CM diagnosis code for congenital EA/TEF; adding the ICD-9-CM code for acquired TEF; limiting age to ≤ 30 days; and adding at least one of a number of specified ICD-9-CM procedure codes. The PHIS search results were subsequently validated by chart review at each institution. RESULTS: The institutional chart reviews identified 207 patients with EA/TEF. The most refined PHIS search strategy identified 221 patients. The positive predictive value of the search increased incrementally from 65% with using only the correct ICD-9 code to 96% with the full methodology. A cohort of 2977 patients with EA/TEF is identified when this search strategy is applied to the entire PHIS database. CONCLUSION: Administrative databases such as PHIS can be utilized to identify cohorts of patients with rare congenital anomalies; however, cohort development requires a systematic search strategy and validation process to ensure correct identification of patients.