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.

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 for Pediatric Lower Urinary Tract Dysfunction: Development of a Standardized Pathway with Objective Urodynamic Outcomes.

PURPOSE: We propose that sacral nerve stimulation is a valid adjunctive therapy for refractory pediatric lower urinary tract dysfunction, and that prospective collection of preoperative and postoperative validated questionnaires and urodynamic data in a standardized fashion is beneficial in characterizing patient response. MATERIALS AND METHODS: Patients were candidates for sacral nerve stimulation if they had refractory voiding dysfunction and standard treatments had failed. Preoperative evaluation included urodynamic studies, spinal magnetic resonance imaging, and validated bladder and bowel related questionnaires. Children were stratified into 2 groups, ie overactive bladder with or without incontinence (group 1) and detrusor underactivity/urinary retention requiring clean intermittent catheterization (group 2). A staged procedure was used with initial test lead placement, followed by permanent device insertion 2 weeks later if patients demonstrated symptom improvement with test lead. Postoperatively children were followed with questionnaires and at least 1 urodynamic study. RESULTS: A total of 26 children underwent sacral nerve stimulation. Mean patient age was 10.8 years and median followup was 1.2 years. There were 23 patients in group 1 and 4 in group 2 (1 patient was included in both groups). In group 1 voiding dysfunction scores improved significantly, and urodynamic studies revealed a significant decrease in mean number of uninhibited contractions and maximum detrusor pressure during the filling phase. In group 2 there was significant improvement in mean post-void residual. CONCLUSIONS: Sacral nerve stimulation is a treatment option that may produce significant improvement in objective and subjective measures of bladder function in children with refractory lower urinary tract dysfunction.

Prediction of symptom improvement in children with biliary dyskinesia.

BACKGROUND: Rates of cholecystectomy for biliary dyskinesia are rising. Our objective was to identify clinical determinants of symptom improvement in children undergoing cholecystectomy for biliary dyskinesia. METHODS: This retrospective cohort study included patients undergoing cholecystectomy for biliary dyskinesia from 2006-2013 who had their gallbladder ejection fraction (EF) measured by either cholecystokinin-stimulated hepatobiliary iminodiacetic acid scan and/or fatty meal ultrasound. Patients presenting from 2010-2013 were interviewed >1 y after cholecystectomy to determine symptom improvement, complete symptom resolution, and any postoperative clinical interventions related to biliary dyskinesia. Sensitivity and positive predictive values for the diagnostic tests for symptom improvement were calculated. Multivariable logistic regression models were used to identify preoperative characteristics associated with symptom improvement. RESULTS: Of the 153 included patients, 76% were female, 89% were Caucasian, and 39% were obese. At postoperative evaluation, symptom improvement was reported by 82% of the patients and complete symptom resolution in 56%. For both the hepatobiliary iminodiacetic acid and fatty meal ultrasound, the sensitivity of the test to predict symptom improvement increased with higher EF, whereas the positive predictive values remained around 80%. Of the 41 patients who participated in phone interview for long-term follow-up, 85% reported symptom improvement and 44% reported complete symptom resolution. Factors associated with symptom improvement included a shorter duration of pain, a history of vomiting, and a history of epigastric pain. CONCLUSIONS: Despite not identifying an EF level that predicted symptom improvement, over 80% of patients undergoing cholecystectomy for biliary dyskinesia reported symptom improvement. These results support continuing to offer cholecystectomy to treat biliary dyskinesia in children.

Using quality improvement methods to change surgical practice: a case example of pediatric soft-tissue abscesses.

BACKGROUND: Surgical treatments of soft-tissue abscesses (STAs) include packing and ring drain (RD) and straight drain (SD) placement. Potential benefits of SDs include a single incision, less scarring, and no need for a follow-up appointment. We used a multidisciplinary quality improvement (QI) process to promote surgeon adoption of an STA drainage technique to improve efficiency and quality of care. SUBJECTS AND METHODS: Outcome measures included the proportion of STAs drained using SDs, the number of postoperative clinic visits, the proportion of patients requiring follow-up with a pediatric surgeon and other providers, and the postoperative complication rate, defined as need for an additional drainage procedure. RESULTS: After beginning the QI initiative, the proportion of STAs drained by SDs increased from 23% to 78% (P < .00001) and the proportion of patients requiring a surgical follow-up clinic appointment decreased from 71% to 32% (P < .00001). The mean number of surgical clinic visits per patient decreased from 0.79 to 0.39 visits per patient (P < .00001). Complication rates were similar between drain types (RD: 2.4%; SD: 1.7%; P = .57). This QI initiative produced a rapid sustained shift in surgeon practice with increased use of SDs, decreased number of follow-up visits, and no increase in complications.

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.

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.

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.

Comparison of routine laboratory measures of heparin anticoagulation for neonates on extracorporeal membrane oxygenation.

Our objective was to determine the best measure of heparin anticoagulation in neonatal patients on extracorporeal membrane oxygenation. Activated clotting time (ACT), activated partial thromboplastin time (aPTT), and antifactor Xa levels, along with corresponding heparin infusion rates and heparin bolus volumes, were collected from neonates receiving ECMO at our institution from 2008 to 2013. After natural log transformation of antifactor Xa, ACT, and aPTT, overall correlations between antifactor Xa levels and either ACT or aPTT and correlations between these tests and heparin infusion rates were evaluated using linear mixed models that accounted for both within- and between-patient correlations. Twenty-six neonates with an average weight of 3.4 kg (standard deviation .7) had a total of 27 separate ECMO runs during the study period. Within each patient, ACT (r = .40, p < .0001) and aPTT (r = .48, p < .0001) were both directly correlated with antifactor Xa levels. In contrast, between patients, only aPTT maintained a direct correlation with antifactor Xa (r = .61, p = .07), whereas ACT showed a statistically significant inverse correlation with antifactor Xa (r = -.48, p = .04). Compared with ACT, aPTT is more consistently reflective of the anticoagulation status both within each patient on ECMO and between patients treated with ECMO. Future efforts to develop standardized heparin infusion algorithms for patients on ECMO should consider using aPTT levels to monitor anticoagulation.

Morbidity of peripherally inserted central catheters in pediatric complicated appendicitis.

BACKGROUND: The purpose of this study was to compare postoperative outcomes of pediatric patients with complicated appendicitis managed with or without a peripherally inserted central catheter (PICC). METHODS: Patients aged ≤18 y in the Pediatric Health Information System database with complicated appendicitis that underwent appendectomy during their index admission in 2000-2012 were grouped by whether they had a PICC placed using relevant procedure and billing codes. Rates of subsequent encounters within 30 d of discharge along with associated diagnoses and procedures were determined. A propensity score-matched (PSM) analysis was performed to account for differences in baseline exposures and severity of illness. RESULTS: We included 33,482 patients with complicated appendicitis; of whom, 6620 (19.8%) received a PICC and 26,862 (80.2%) did not. The PICC group had a longer postoperative length of stay (median 7 versus 5 d, P<0.001) and were more likely to undergo intra-abdominal abscess drainage during the index admission (14.4% versus 2.1%, P<0.001), and have a reencounter (17.5% versus 11.4%, P<0.001) within 30 d of discharge. However, in the PSM cohort (n=4428 in each group), outcomes did not differ between treatment groups, although the PICC group did have increased odds for the development of other postoperative complications (odds ratio=3.95, 95% confidence interval: 1.45, 10.71). CONCLUSIONS: After accounting for differences in severity of illness by PSM, patients managed with PICCs had a similar risk for nearly all postoperative complications, including reencounters. Postoperative management of pediatric complicated appendicitis with a PICC is not clearly associated with improved outcomes.

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%.

Hemolysis-Associated Nitric Oxide Dysregulation during Extracorporeal Membrane Oxygenation.

Acute intravascular hemolysis during extracorporeal membrane oxygenation (ECMO) leads to increased levels of cell-free hemoglobin (FHb). Our aim was to investigate whether FHb levels are associated with nitric oxide (NO) consumption and clinical outcomes. A prospective observational study was performed involving pediatric patients on ECMO. Blood samples were collected before, during, and after the ECMO run, and plasma was evaluated for FHb, oxyhemoglobin, and NO consumption. Clinical data were collected including baseline patient characteristics, indications for ECMO, circuit changes, and mortality. Correlations between laboratory measures and associations between laboratory measures and clinical observations were evaluated. Twenty-three patients (11 male, 17 neonates) were enrolled with a median weight of 3.1 kg (interquartile range, 2.8-14.0 kg) and median ECMO run of 12 days (interquartile range, 5-19 day). There was a significant increase in FHb over time on ECMO (p = .007), and significant correlations were present between NO consumption and both FHb (r = .41, p = .01) and oxyhemoglobin levels (r = .98, p < .0001). Patients on ECMO for sepsis (n = 6) had lower average levels of oxyhemoglobin (mean [standard deviation {SD}] 14.5 [4.4] versus 19.0 [5.0] µM, p = .07) and NO consumption (mean [SD] 15.8 [4.1] versus 19.8 [3.7] µM, p = .04) during ECMO than patients with other indications. In the 3 days leading up to a circuit change, there were increases in mean total cell-free hemoglobin levels (24%/day, p = .08), oxyhemoglobin (37%/day, p = .005), and NO consumption (40%/day, p = .006) (n = 5). There were no significant associations identified between peak or average plasma measures of hemolysis and type of ECMO (venovenous versus venoarterial) or mortality. For children on ECMO, we observed a strong correlation between increased levels of plasma FHb and elevations in oxyhemoglobin and NO consumption; however, these changes were not associated with increased mortality. Increased hemolysis before circuit changes may be both a marker and a contributor to circuit failure.

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.