Differences in Outcomes Based on Sex for Pediatric Patients Undergoing Pyloromyotomy.

BACKGROUND: Males and females are known to have varied responses to medical interventions. Our study aimed to determine the effect of sex on surgical outcomes after pyloromyotomy. MATERIALS AND METHODS: Using the Kids' Inpatient Database for the years 2003-2012, we performed a serial, cross-sectional analysis of a nationally representative sample of all patients aged <1 y who underwent pyloromyotomy for hypertrophic pyloric stenosis. The primary predictor of interest was sex. Outcomes included mortality, in-hospital complications, cost, and length of stay. Regression models were adjusted by race, age group, comorbidity, complications, and whether operation was performed on the day of admission with region and year fixed effects. RESULTS: Of 48,834 weighted operations, 81.8% were in males and 18.2% were in females. The most common reported race was white (47.3%) and most of the patients were ≥29 days old (72.5%). There was no difference in the odds of postoperative complications, but females had a significantly longer length of stay (incidence rate ratio, 1.28; 95% confidence interval [95% CI], 1.18-1.39; P ≤ 0.01), higher cost (5%, 95% CI, 1.02-1.08; P ≤ 0.01), and higher odds of mortality (odds ratio, 3.26; 95% CI, 1.52-6.98; P ≤ 0.01). CONCLUSIONS: Our study demonstrated that females had worse outcomes after pyloromyotomy compared with males. These findings are striking and are important to consider when treating either sex to help set physician and family expectations perioperatively. Further studies are needed to determine why such differences exist and to develop targeted treatment strategies for both females and males with pyloric stenosis.

Effects of Hospital Volume on Patient Outcomes and Costs in Infants With Pyloric Stenosis.

BACKGROUND: There is a well-established relationship between surgical volume and outcomes after complex pediatric operations. However, this relationship remains unclear for common pediatric procedures. The aim of our study was to investigate the effect of hospital volume on outcomes after hypertrophic pyloric stenosis (HPS). METHODS: The Kid's Inpatient Database (2003-2012) was queried for patients with congenital HPS, who underwent pyloromyotomy. Hospitals were stratified based on case volume. Low-volume hospitals performed the lowest quartile of pyloromyotomies per year and high-volume hospitals managed the highest quartile. Outcomes included complications, mortality, length of stay (LOS), and cost. RESULTS: Overall, 2137 hospitals performed 51,792 pyloromyotomies. The majority were low-volume hospitals (n = 1806). High-volume hospitals comprised mostly children's hospitals (68%) and teaching hospitals (96.1%). The overall mortality rate was 0.1% and median LOS was 2 d. High-volume hospitals had lower overall complications (1.8% versus 2.5%, P < 0.01) and fewer patients with prolonged LOS (17.0% versus 23.5%, P < 0.01) but had similar rates of individual complications, similar mortality, and equivalent median LOS as low-volume hospitals. High-volume hospitals also had higher costs by $1132 per patient ($5494 versus $4362, P < 0.01). Regional variations in outcomes and costs exist with higher complication rates in the West and lower costs in the South. There was no association between mortality or LOS with hospital volume or region. CONCLUSIONS: Patients with pyloric stenosis treated at high-volume hospitals had no clinically significant difference in outcomes despite having higher costs. Although high-volume hospitals offer improved outcomes after complex pediatric surgeries, they may not provide a significant advantage over low-volume hospitals in managing common pediatric procedures, such as pyloromyotomy for congenital HPS.

Outcomes and Costs of Common Surgical Procedures at Children's and Nonchildren's Hospitals.

BACKGROUND: Variations in the management of pediatric patients at children's hospitals (CHs) and non-CHs (NCHs) have been well described, especially within the trauma literature. However, little is known about the outcomes and costs of common general surgical procedures at NCHs. The purpose of this study was to evaluate the effect of CH designation on the outcomes and costs of appendectomy and cholecystectomy. METHODS: The Kids' Inpatient Database (2003-2012) was queried for patients aged under 18 y who underwent appendectomy or cholecystectomy at CHs and NCHs. Outcomes analyzed included disease severity, complications, laparoscopy, length of stay (LOS), and cost. RESULTS: Most of appendectomies and cholecystectomies were performed at NCHs. Overall, CHs cared for younger children were more likely to be teaching hospitals, had higher costs, and longer LOS. On multivariate analysis for appendectomies, CHs were associated with higher rates of perforated appendicitis (OR = 1.53, 95% CI = 1.42-1.66, P < 0.001), less complications (OR = 0.68, 95% CI = 0.61-0.75, P < 0.001), increased laparoscopy (OR = 2.93, 95% CI = 2.36-3.64, P < 0.001), longer LOS (RR = 1.13, 95% CI = 1.09-1.17, P < 0.001), and higher costs (exponentiated log $ = 1.19, 95% CI = 1.13-1.24, P < 0.001). Multivariate analysis for cholecystectomies revealed that CHs were associated with less laparoscopy (OR = 0.58, 95% CI = 0.50-0.67, P < 0.001), longer LOS (RR = 1.26, 95% CI = 1.19-1.34, P < 0.001), and higher costs (exponentiated log $ = 1.29, 95% CI = 1.22-1.37, P < 0.001) with similar complications. Independent predictors of LOS and cost included CH designation, negative appendectomy, perforated appendicitis, complications, younger age, black patients, and public insurance. CONCLUSIONS: Variations in surgical management, outcomes, and costs after appendectomy and cholecystectomy exist between CHs and NCHs. CHs excelled in treating complicated appendicitis. NCHs effectively performed cholecystectomies. These differences in outcomes require further investigation to identify modifiable factors to optimize care across all hospitals for these common surgical diseases.

Regional variations in outcomes and cost of appendectomy in the United States.

BACKGROUND: The study of regional variations in surgical outcomes and cost has been used to identify areas for improvement and savings. This study investigates potential regional differences in the outcomes and cost of adult appendicitis. We hypothesized that there would be no difference in rates of laparoscopy, perforation, morbidity, length of stay (LOS), and cost among different regions of the United States. MATERIALS AND METHODS: Data were obtained from the California (CA), New York (NY), and Florida (FL) State Inpatient Databases from 2005-2011. Patients between the ages of 18-69 who underwent nonincidental appendectomy in the three different states were evaluated with hierarchical and multivariate negative binomial regression analyses. Primary outcomes included laparoscopy, perforation, negative appendectomy, morbidity, LOS, and cost. RESULTS: There were 371,354 appendectomies performed. Multivariate analysis revealed multiple regional differences. Patients in FL were most likely to get laparoscopy (P < 0.01). CA had higher rates of perforation than NY (P < 0.01) and FL (P < 0.05). CA also had higher rates of negative appendectomy compared to both NY and FL (P < 0.01). Morbidity was lower in NY compared to CA and FL (P < 0.01). The LOS was shortest in CA (P < 0.01), despite CA having the highest median per patient cost (P < 0.01). CONCLUSIONS: Significant regional variations do exist with CA having the highest rate of perforation and negative appendectomy. Patients in CA also incurred the highest overall costs. A better understanding of the factors that drive these variations will help improve outcomes and lower cost across all states.

Clinical and socioeconomic factors associated with negative pediatric appendicitis.

BACKGROUND: Misdiagnosing appendicitis may lead to unnecessary surgery. The study evaluates the risk factors for negative appendectomies, as well as the clinical and socioeconomic consequences of negative appendectomy across three states. MATERIALS AND METHODS: Data were obtained from the California, New York, and Florida State Inpatient Databases 2005-2011. Patients (<18 years) who underwent nonincidental appendectomies (n = 156,660) were evaluated with hierarchical and multivariate negative binomial regression analyses on outcomes including hospital cost, length of stay (LOS), and associated morbidity. RESULTS: From 2005 to 2011, there was a decrease in the rate of negative appendicitis and perforated appendicitis, whereas the rate of true acute nonperforated appendicitis increased. Whites, females, and privately insured patients were associated with higher negative appendicitis rates, whereas those at an increased risk for perforated appendicitis were African-Americans, males, and those with public or no insurance. Compared to patients with acute nonperforated appendicitis, those with negative appendicitis have significantly higher morbidity (2.5% versus 1.3%), longer LOS (3.4 versus 1.8 d), and greater hospital costs averaged over time ($6926 versus $6492 per patient). CONCLUSIONS: Despite a low incidence, negative appendicitis is associated with greater morbidity, longer LOS, and higher cost than acute nonperforated appendicitis. Certain subpopulations are at higher risk for undergoing surgery for negative appendicitis, whereas others are at greater risk for presenting with perforated appendicitis. Further research is needed to understand what drives such disparities and to inform efforts to improve quality of hospital care across all groups of patients.

Cost modeling for management strategies of uncomplicated gastroschisis.

INTRODUCTION: Compared to operative fascial closure, nonoperative flap and/or skin-closure repair for gastroschisis has several potential advantages: avoidance of anesthesia, decreased pain, and improved cosmesis. Disadvantages include a higher risk of hernia. We hypothesized that routine nonoperative closure results in cost savings versus conventional management in uncomplicated gastroschisis. METHODS: A decision tree was constructed to compare three different strategies for the management of uncomplicated gastroschisis: nonoperative closure, primary closure, and routine silo. Model variables were abstracted from a literature review and the Medicare Physician Fee schedule. Uncertainty surrounding model parameters was assessed via one-way and probabilistic sensitivity analyses. RESULTS: According to our model, the nonoperative strategy for uncomplicated gastroschisis was the least costly, with an expected cost of $198,085 per patient. Primary closure cost $208,763 per patient. Routine silo placement was the most costly, $239,038 per patient. One-way sensitivity analysis suggested the cost of primary closure would be less costly than nonoperative management if the initial success rate of nonoperative management was less than 35.4% or if the initial success rate of primary operative closure was greater than 87.8%. Probabilistic sensitivity analysis found that nonoperative management was the least costly strategy among 97.4% of 10,000 Monte Carlo simulations. CONCLUSIONS: A nonoperative strategy for uncomplicated gastroschisis with routine attempted flap and/or skin closure repair is less costly than strategies using routine primary closure and routine silo placement. Given the expected cost savings and other potential advantages of the nonoperative strategy (including avoidance of general anesthesia), more studies examining outcomes of the flap and/or skin closure are indicated.

Factors predictive of complicated appendicitis in children.

BACKGROUND: The ability to predict whether a child has complicated appendicitis at initial presentation may influence clinical management. However, whether complicated appendicitis is associated with prehospital or inhospital factors is not clear. We also investigate whether hyponatremia may be a novel prehospital factor associated with complicated appendicitis. MATERIALS AND METHODS: A retrospective review of all pediatric patients (≤12 y) with appendicitis treated with appendectomy from 2000 to 2013 was performed. The main outcome measure was intraoperative confirmation of gangrenous or perforated appendicitis. A multivariable analysis was performed, and the main predictors of interest were age <5 y, symptom duration >24 h, leukocytosis (white blood cell count >12 × 103/mL), hyponatremia (sodium ≤135 mEq/L), and time from admission to appendectomy. RESULTS: Of 392 patients, 179 (46%) had complicated appendicitis at the time of operation. Univariate analysis demonstrated that patients with complicated appendicitis were younger, had a longer duration of symptoms, higher white blood cell count, and lower sodium levels than patients with noncomplicated appendicitis. Multivariable analysis confirmed that symptom duration >24 h (odds ratio [OR] = 5.5, 95% confidence interval [CI] = 3.5-8.9, P < 0.01), hyponatremia (OR = 3.1, 95% CI = 2.0-4.9, P < 0.01), age <5 y (OR = 2.3, 95% CI = 1.3-4.0, P < 0.01), and leukocytosis (OR = 1.9, 95% CI = 1.0-3.5, P = 0.04) were independent predictors of complicated appendicitis. Increased time from admission to appendectomy was not a predictor of complicated appendicitis (OR = 0.8, 95% CI = 0.5-1.2, P = 0.2). CONCLUSIONS: Prehospital factors can predict complicated appendicitis in children with suspected appendicitis. Hyponatremia is a novel marker associated with complicated appendicitis. Delaying appendectomy does not increase the risk of complicated appendicitis once intravenous antibiotics are administered. This information may help guide resource/personnel allocation, timing of appendectomy, and decision for nonoperative management of appendicitis in children.

Changes in Self-Reported Adult Health and Household Food Security With the 2021 Expanded Child Tax Credit Monthly Payments.

Importance: The 2021 Expanded Child Tax Credit (ECTC) provided families with children monthly payments from July 2021 to December 2021. The association of this policy with adult health is understudied. Objective: To examine changes in adult self-reported health and household food security before and during ECTC monthly payments. Design, Setting, and Participants: This repeated cross-sectional study used multivariable regression with a difference-in-differences estimator to assess adult health and food security for 39 479 respondents to the National Health Interview Survey (January 2019 to December 2021) before vs during monthly payments. Analyses were stratified by income to focus on low-income vs middle-income and upper-income households. Exposure: Eligibility for ECTC monthly payments from July 2021 to December 2021. Main Outcomes and Measures: Overall self-reported adult health and household food security as binary outcomes (excellent or very good health vs good, fair, or poor health; food secure vs food insecure). Results: In this nationally representative cross-sectional study of 39 479 US adults (mean [SD] age, 41.0 [13.0] years; 7234 [21.7%] Hispanic, 321 [0.9%] non-Hispanic American Indian/Alaska Native, 2205 [5.7%] non-Hispanic Asian, 5113 [13.7%] non-Hispanic Black, and 23 704 [55.8%] White individuals), respondents were predominantly female (21 511 [52.4%]), employed (33 035 [86.7%]), and married (19 838 [55.7%]). Before disbursement of ECTC monthly payments, 7633 ECTC-eligible adults (60.1%) reported excellent or very good health, and 10 950 (87.8%) reported having food security. Among ECTC-ineligible adults, 10 778 (54.9%) reported excellent or very good health and 17 839 (89.1%) reported food security. Following disbursement of monthly payments, ECTC-eligible adults experienced a 3.0 percentage point (pp) greater adjusted increase (95% CI, 0.2-5.7) in the probability of reporting excellent or very good health compared with ECTC-ineligible adults. Additionally, ECTC-eligible adults experienced a 1.9 pp greater adjusted increase (95% CI, 0.1-3.7) in the probability of food security than ECTC-ineligible adults. In income-stratified analyses, the association between ECTC eligibility and overall health was concentrated among middle-income and upper-income households (3.7-pp increase in excellent or very good health; 95% CI, 0.5-6.9). Conversely, the association between ECTC eligibility and food security was concentrated among low-income adults (3.9-pp increase in food security; 95% CI, 0-7.9). Conclusions and Relevance: The results of this cross-sectional study suggest that monthly ECTC payments were associated with improved adult overall health and food security. Cash transfer programs may be effective tools in improving adult health and household nutrition.

The surgical management of a stage III Wilms tumor presenting with perforated appendicitis.

The majority of the cases of nephroblastoma do not present with abdominal pain. We present a patient with an acute surgical abdomen due to perforated appendicitis and an incidental abdominal mass. He underwent an urgent appendectomy after which tumor specific therapy was successfully initiated.

County versus private hospitals: access of care, management and outcomes for patients with appendicitis.

BACKGROUND AND OBJECTIVES: Race/ethnicity and socioeconomic status may affect healthcare access (higher appendiceal perforation [AP] rates), management (lower laparoscopic appendectomy [LA] rates), and outcomes in patients with appendicitis. This study determines if disparities exist between county and private hospitals. METHODS: A review of patients > or = 18 years treated for appendicitis from 1998 through 2007 was performed. Data from a county hospital were compared to data from 12 private hospitals. Study outcomes included length of hospitalization (LOH), and rates of AP, LA, and abscess drainage. Predictor variables collected included age, sex, race/ ethnicity, per-capita income, and hospital type. RESULTS: For this study, 16,512 patients were identified (county = 1,293, private = 15,219). On univariate analysis, patients at the county hospital had lower mean per-capita incomes ($13,412 vs. $17,584, P<.0001), similar AP rates at presentation (26% vs. 24%, P = .10), and lower abscess drainage (0.2% vs. 2.1%, P < .0001). However, multivariate analysis demonstrated a higher AP (OR 1.4, CI 1.2-1.6) and LA rate (OR 1.9, CI 1.7-2.2), a lower abscess drainage rate (0.07, 95% CI 0.02-0.27), and longer LOH (parameter estimate = 0.4, P<.0001) at the county hospital. Within the county hospital cohort, LOH and rates of AP, LA, and. abscess drainage were similar across all races/ethnicities and income levels. CONCLUSIONS: When compared to private hospital patients, adults with appendicitis treated at a county hospital were of lower socioeconomic background, had higher AP rates and longer LOH, but were more likely to undergo LA and less likely to require abscess drainage. Since racial and socioeconomic disparities were no longer apparent once within the county hospital cohort, these differences may be due to differences in access to healthcare.

Equal access to healthcare does not eliminate disparities in the management of adults with appendicitis.

BACKGROUND: Race and socioeconomic status have led to inequality in access to surgical care, leading to differences in appendiceal perforation rates. However, in a setting of equal health care access, these disparities were not evident. Currently, disparities exist with use of laparoscopic appendectomy (LA) in the treatment of appendicitis. This study determines whether equal health care access eliminates differences in the management and outcomes of appendicitis. METHODS: A retrospective review from 1998 to 2007 was performed. All members in this single-provider system have equal healthcare access. Socioeconomic data were extracted from the US Census database. Study outcome was use of LA and postoperative morbidity. Independent variables included age (18-44 y, 45-65 y, and >65 y), gender, race, annual mean household income (<40 K, 40-65 K, and >65 K), and education level. Univariate and multivariable (controlling for age, gender, perforation, race/ethnicity, income, and education level) analyses were performed. RESULTS: A total of 16,196 patients were identified (mean age = 41 y, 54% male). Sixty percent of patients underwent LA. On multivariable analysis, male gender was associated with a decreased use of LA (OR 0.84, CI 0.79-0.89, P < 0.0001). There was a lower use of LA in older patients (age > 65 y) and patients age 45-65 y compared with younger patients (18-44 y). There was a lower use of LA in Blacks and a higher rate in Hispanics compared with Whites. Use of LA was higher in high income and middle income patients compared with low income patients. The use of LA was similar across all education levels. Postoperative morbidity was higher in males, older patients (age > 65 y), and patients age 45-65 y. However, postoperative morbidity was similar across all race/ethnic groups and all income levels and education levels. CONCLUSION: In a setting of equal health care access, there is evidence of gender, racial, age, and socioeconomic disparities for use of LA and worse outcomes for males and older patients. These data suggest that despite equal healthcare access, disparities continue to exist with respect to race and income level for the management of adults with appendicitis.

Optimizing pediatric histrelin implantation to improve success rates in clinic without sedation.

OBJECTIVES: The purpose of this study was to review our success rate performing the histrelin implant procedure in clinic without sedation. METHODS: A retrospective study was performed for histrelin implant procedures done at our institution from 2008 to 2020. Wilcoxon rank-sum test or Fisher's exact test was utilized to identify significant differences (p<0.05). RESULTS: A total of 73 patients underwent 184 histrelin implant procedures from 2008 to 2020. In the past few years, there has been a decrease in procedures for precocious puberty and an increase for gender dysphoria. The majority of procedures were performed in clinic without sedation (82%). The only risk factor associated with requiring sedation was younger age (median 9 vs. 10 years; p<0.003). Complications (i.e. implant fracture or need for counter-incision) were noted in 10 of the procedures (5%). The only risk factor identified for a procedural complication during implant removal/replacement was interval time from insertion (21 vs. 13 months; p<0.01). The only documented wound problem reported was dermatitis in 1 patient (no suture granuloma, dehiscence, or implant extravasation). CONCLUSIONS: Procedural refinements and distraction therapy have enabled us to perform the majority of procedures in clinic without sedation. In our experience, procedural difficulty and complications appear to increase with prolonged implant duration. Histrelin implantation is increasingly being performed for gender dysphoria.

Underrepresentation of pediatric operations in the relative value unit updating process.

BACKGROUND: The Relative Value Unit (RVU) system was designed and implemented by Medicare to standardize physician payments for a given service. Because Medicare primarily cares for older adults, RVU assignments and updates may not consider pediatric-specific procedures, despite the fact that private insurers and Medicaid often base their payments on these RVU valuations. METHODS: The CPT codes of index pediatric operations were retrieved from the ACGME. We categorized these procedures into "Peds-specific" (eg, Ladd Procedure) versus those that could be performed in both children and adults, or "Non-specific" (eg, fundoplication). We merged these codes with RVU information from publicly available CMS files and the Resource-based Relative Value Scale Data Manager. Variables included were the date of last RVU update and the vignette used by survey respondents when asked to update the RVU valuation. RESULTS: Among 85 procedures, nearly three-quarters were Peds-specific (74%), with the remainder Non-specific. Approximately half of the 85 procedures (52%) had never been updated. Compared to Non-specific CPT codes, Peds-specific CPT codes were less likely to have been updated (38% vs. 91%, p < 0.001) and, among those that were updated, were updated more remotely (median year 2000 vs. 2005, p = 0.02). Among updated Non-specific CPT codes, the vignette written to justify the valuation was based on an adult patient in 85% of cases. CONCLUSIONS: Peds-specific surgical CPT codes have either never been updated or have not been updated in decades. Procedures performed in both children and adults have been updated more often and more recently, but the vignette on which this valuation is based on is typically an adult patient. In order to remain relevant and reimburse pediatric surgeons accurately, the RVUs for pediatric procedures need to also be prioritized for revision and updating.

The impact of removing global periods on pediatric surgeon reimbursement.

PURPOSE: CMS has proposed removing postoperative care from the global periods for surgical procedures and instead requiring surgeons to bill for postoperative visits using evaluation & management (E&M) codes. This policy may alter reimbursement to pediatric surgeons. METHODS: To assess the impact of this policy, NSQIP-pediatric data were used to calculate median LOS for high-volume procedures with 10 or 90 day global periods. We then merged these data with CMS physician work time and RVU files. A CMS LOS variable was created by counting the number of hospital-based E&M codes built into the global period based on the fact that if global periods are removed, surgeons may only bill one E&M code per postoperative day. We then compared the CMS and NSQIP LOS values. RESULTS: The dataset included 201 CPT codes with NSQIP LOS estimates derived from a median of 137 operations. Twenty-nine procedures (14.4%) had higher, 24 (16.9%) had the same, and 138 (68.7%) had lower NSQIP median LOS than current CMS values. On average, NSQIP values were 40.0% (95% confidence interval [95CI] -50.0, -29.9%) lower than CMS values. Based on a daily average work RVU per postoperative E&M code of 1.09 (95% CI 1.05, 1.12), and $35.78 per RVU (2017 rate), surgeons in this sample would experience a cumulative annual reduction in reimbursement of approximately $3.4 M following the policy change. CONCLUSIONS: Most pediatric surgical procedures have RVU valuations that include more hospital-based E&M codes than the current median number of postoperative days. Holding all else equal, the removal of global periods would therefore reduce reimbursement for pediatric surgeons. The downstream effects of this policy change, such as the impact on the quality of clinical care, are uncertain and warrant further investigation. TYPE OF STUDY: Clinical research paper. LEVEL OF EVIDENCE: Level II.

Recessive ciliopathy mutations in primary endocardial fibroelastosis: a rare neonatal cardiomyopathy in a case of Alstrom syndrome.

Among neonatal cardiomyopathies, primary endocardial fibroelastosis (pEFE) remains a mysterious disease of the endomyocardium that is poorly genetically characterized, affecting 1/5000 live births and accounting for 25% of the entire pediatric dilated cardiomyopathy (DCM) with a devastating course and grave prognosis. To investigate the potential genetic contribution to pEFE, we performed integrative genomic analysis, using whole exome sequencing (WES) and RNA-seq in a female infant with confirmed pathological diagnosis of pEFE. Within regions of homozygosity in the proband genome, WES analysis revealed novel parent-transmitted homozygous mutations affecting three genes with known roles in cilia assembly or function. Among them, a novel homozygous variant [c.1943delA] of uncertain significance in ALMS1 was prioritized for functional genomic and mechanistic analysis. Loss of function mutations of ALMS1 have been implicated in Alstrom syndrome (AS) [OMIM 203800], a rare recessive ciliopathy that has been associated with cardiomyopathy. The variant of interest results in a frameshift introducing a premature stop codon. RNA-seq of the proband's dermal fibroblasts confirmed the impact of the novel ALMS1 variant on RNA-seq reads and revealed dysregulated cellular signaling and function, including the induction of epithelial mesenchymal transition (EMT) and activation of TGFß signaling. ALMS1 loss enhanced cellular migration in patient fibroblasts as well as neonatal cardiac fibroblasts, while ALMS1-depleted cardiomyocytes exhibited enhanced proliferation activity. Herein, we present the unique pathological features of pEFE compared to DCM and utilize integrated genomic analysis to elucidate the molecular impact of a novel mutation in ALMS1 gene in an AS case. Our report provides insights into pEFE etiology and suggests, for the first time to our knowledge, ciliopathy as a potential underlying mechanism for this poorly understood and incurable form of neonatal cardiomyopathy. KEY MESSAGE: Primary endocardial fibroelastosis (pEFE) is a rare form of neonatal cardiomyopathy that occurs in 1/5000 live births with significant consequences but unknown etiology. Integrated genomics analysis (whole exome sequencing and RNA sequencing) elucidates novel genetic contribution to pEFE etiology. In this case, the cardiac manifestation in Alstrom syndrome is pEFE. To our knowledge, this report provides the first evidence linking ciliopathy to pEFE etiology. Infants with pEFE should be examined for syndromic features of Alstrom syndrome. Our findings lead to a better understanding of the molecular mechanisms of pEFE, paving the way to potential diagnostic and therapeutic applications.

Air insufflation of the stomach following laparoscopic pyloromyotomy may not detect perforation.

BACKGROUND: Undetected perforation during laparoscopic pyloromyotomy can be fatal. Detecting a perforation at the time of laparoscopic pyloromyotomy is difficult. The purpose of this study was to determine whether air insufflation of the stomach reliably detects perforation during laparoscopic pyloromyotomy. CASE REPORTS: Between 2007 and 2008, 71 patients underwent laparoscopic pyloromyotomy and 2 patients (3.3%) had perforation. Insufflating the stomach with air did not demonstrate the perforation in either case. Both perforations were detected by careful inspection of the myotomy; a small amount of mucus was seen at the perforation site. Both patients underwent open suture repair with an omental patch and had unremarkable postoperative courses. CONCLUSIONS: Air insufflation of the stomach during laparoscopic pyloromyotomy does not reliably rule out perforation. As with all procedures with potential complications, a high index of suspicion and careful inspection of the entire myotomy may help detect perforation.

Complications as a result of the Heimlich maneuver.

The Heimlich maneuver is a well-described emergency procedure for management of foreign body airway obstructions. Although rare, complications of the Heimlich maneuver do exist. The purpose of this report is to review the known complications of this procedure. All reported complications published in English on Medline and PubMed were reviewed. Additionally, we present a rare case of acute pancreatitis with associated pseudocyst formation after the administration of the Heimlich maneuver on a healthy 3-year-old boy. Although life saving, the Heimlich maneuver may be associated with significant complications; thus, symptomatic patients after this maneuver should be thoroughly evaluated with appropriate laboratory and radiographic studies.

Assessing outcomes and costs of appendectomies performed at rural hospitals.

BACKGROUND: The purpose of our study was to assess the outcomes and costs of appendectomies performed at rural and urban hospitals. METHODS: The National Inpatient Sample (2001-2012) was queried for appendectomies at urban and rural hospitals. Outcomes (disease severity, laparoscopy, complications, length of stay (LOS), and cost) were analyzed. RESULTS: Rural patients were more likely to be older, male, white, and have Medicaid or no insurance. Rural hospitals were associated with higher negative appendectomy rates (OR = 1.26,95%CI = 1.18-1.34,p < 0.01), less laparoscopy use (OR = 0.65,95%CI = 0.58-0.72,p < 0.01), and slightly shorter LOS (OR = 0.98,95%CI = 0.97-0.99,p < 0.01). There was no consistent association with perforated appendicitis and no difference in complications or costs after adjusting for hospital volume. Yearly trends showed a significant increase in the cases utilizing laparoscopy each year at rural hospitals. CONCLUSIONS: Rural appendectomies are associated with increased negative appendectomy rates and less laparoscopy use with no difference in complications or costs compared to urban hospitals.

Outcomes and costs of pediatric appendectomies at rural hospitals.

BACKGROUND/PURPOSE: Despite policy efforts to support rural hospitals, little is known about the quality and safety of pediatric surgical care in geographically remote areas. Our aim was to determine the outcomes and costs of appendectomies at rural hospitals. METHODS: The Kids' Inpatient Database (2003-2012) was queried for appendectomies in children <18 years at urban and rural hospitals. Outcomes (disease severity, laparoscopy, complications, length of stay (LOS), cost) were analyzed with bivariate and multivariable regression analysis. RESULTS: Rural hospitals performed 13.6% of appendectomies. On multivariable analysis, rural hospitals were associated with higher negative appendectomy rates (OR 1.49, 95% CI 1.39-1.60, p < 0.001), decreased appendiceal perforation rates (OR 0.86, 95% CI 0.83-0.89, p < 0.001), less laparoscopy use (OR 0.48, 95% CI 0.47-0.50, p < 0.001), higher complication rates (OR 1.29, 95% CI 1.19-1.39, p < 0.001), shorter LOS (IRR 0.90, 95% CI 0.89-0.91, p < 0.001), and slightly increased costs (exponentiated log$ 1.02, 95% CI 1.01-1.02, p < 0.001) CONCLUSIONS: Rural hospitals care for fewer patients with advanced appendicitis but are associated with higher negative appendectomy rates, lower laparoscopy use, and higher complication rates. Additional studies are needed to identify factors that drive this disparity to improve the quality of pediatric surgical care in rural settings. TYPE OF STUDY: Treatment/Cost Study (Outcomes). LEVEL OF EVIDENCE: Level III.