Total vascular resistance increases during volume-unloading in asymptomatic single ventricle patients.

OBJECTIVE: While the surgical stages of single ventricle (SV) palliation serve to separate pulmonary venous and systemic venous return, and to volume-unload the SV, staged palliation also results in transition from parallel to series circulation, increasing total vascular resistance. How this transition affects pressure loading of the SV is as yet unreported. METHODS: We performed a retrospective chart review of Stage I, II, and III cardiac catheterization (CC) and echocardiographic data from 2001-2017 in all SV pts, with focus on systemic, pulmonary, and total vascular resistance (SVR, PVR, TVR respectively). Longitudinal analyses were performed with log-transformed variables. Effects of SVR-lowering medications were analyzed using Wilcoxon rank-sum testing. RESULTS: There were 372 total patients who underwent CC at a Stage I (median age of 4.4 months, n=310), Stage II (median age 2.7 years, n = 244), and Stage III (median age 7.3 years, n = 113). Total volume loading decreases with progression to Stage III (P< 0.001). While PVR gradually increases from Stage II to Stage III, and SVR increases from Stage I to Stage III, TVR dramatically increases with progress towards series circulation. TVR was not affected by use of systemic vasodilator therapy. TVR, PVR, SVR, and CI did not correlate with indices of SV function at Stage III. CONCLUSIONS: TVR steadily increases with an increasing contribution from SVR over progressive stages. TVR was not affected by systemic vasodilator agents. TVR did not correlate with echo-based indices of SV function. Further studies are needed to see if modulating TVR can improve exercise tolerance and outcomes.

Genetic testing and cascade screening in pediatric long QT syndrome and hypertrophic cardiomyopathy.

BACKGROUND: The efficacy of cascade screening for the inherited heart conditions long QT syndrome (LQTS) and hypertrophic cardiomyopathy (HCM) is incompletely characterized. OBJECTIVE: The purpose of this study was to examine the use of genetic testing and yield of cascade screening across diverse regions in the United States and to evaluate obstacles to screening in multipayer systems. METHODS: An institutional review board-approved 6 United States pediatric center retrospective chart review of LQTS and HCM patients from 2008-2014 was conducted for (1) genetic test completion and results and (2) family cascade screening acceptance, methods, results, and barriers. RESULTS: The families of 315 index patients (mean age 9.0 ± 5.8 years) demonstrated a 75% (254) acceptance of cascade screening. The yield of relative screening was 39% (232/601), an average of 0.91 detected per family. Genetic testing was less utilized in HCM index patients and relatives. Screening participation was greater in families of gene-positive index patients (88%) (P <.001) compared to gene-negative patients (53%). Cascade method utilization: Cardiology-only 45%, combined genetic and cardiology 39%, and genetic only 16%. Screening yield by method: combined 57%, genetic-only 29%, and cardiology-only 20%. Family decisions were the leading barriers to cascade screening (26% lack of followthrough and 26% declined), whereas insurance (6%) was the least cited barrier. CONCLUSION: Family participation in cascade screening is high, but the greatest barriers are family mediated (declined, lack of followthrough). Positive proband genetic testing led to greater participation. Cardiology-only screening was the most utilized method, but combined cardiology and genetic screening had the highest detection.

Primary versus secondary anastomosis in intestinal atresia.

PURPOSE: Neonates with intestinal atresia (IA) undergo either primary anastomosis (PA) or ostomy creation with secondary anastomosis (SA). Our purpose was to compare outcomes for PA and SA and to assess factors influencing procedure selection. METHODS: We conducted a retrospective cohort study of neonates with IA between 2009 and 2015. Patient characteristics, operative details, and outcomes were collected. Surgeon-level preferences (defined as performing >50% PA or SA) were assessed using logistic regression. RESULTS: Of 92 IA patients, 70 (76.1%) underwent PA and 22 (23.9%) underwent SA. Neonates with PA had shorter hospitalizations (27 days vs. 95 days, p < 0.001), shorter total parenteral nutrition duration (19 days vs. 74.5 days, p < 0.001), and fewer readmissions (33.3% vs. 63.2%, p = 0.024). On multivariable regression analysis, higher Apgar scores (Odds Ratio (OR) 4.16, 95% Confidence Interval (CI) 1.20-14.29) and uncomplicated atresia (OR 3.97, 95% CI 1.37-11.48) were associated with PA. At the surgeon-level, utilization of PA varied from 43.5% to 100%. Surgeon preference is not influenced by the demographic, presentation, or surgical findings of this patient population. CONCLUSIONS: PA has better outcomes than SA. Though procedural selection is influenced by the clinical status of the neonate, however surgeon preference plays a significant role in this clinical decision. LEVEL OF EVIDENCE: Level III Treatment Study.

Temporomandibular joint involvement in children with juvenile idiopathic arthritis: a preliminary report.

OBJECTIVE: Children with juvenile idiopathic arthritis (JIA) are at risk for temporomandibular joint (TMJ) arthritis. This can lead to pain, limited mouth opening, facial asymmetry, and malocclusion. Our objective was to characterize patients with JIA and TMJ involvement in a single center. STUDY DESIGN: This was a retrospective study of children with JIA evaluated at Children's Healthcare of Atlanta. Inclusion criteria were confirmed JIA and jaw complaints. Medical records were reviewed to document demographics, JIA information, age at first TMJ complaint, and involvement of other joints. Descriptive statistics were computed. RESULTS: Majority of patients were white (mean age 13 years; range 5-18 years) with polyarticular rheumatoid factor (RF) negative or oligoarticular persistent JIA. Some were antinuclear antibody (ANA) positive, RF positive, or human leukocyte antigen (HLA)-B27 positive. Patients had involvement of other joints (e.g., fingers, knees, wrists). Of those with TMJ symptoms, 6 (10%) had TMJ arthritis. CONCLUSIONS: In our cohort, 60 (10%) of patients were diagnosed with TMJ arthritis. In this population, patients who are female, white, RF negative, HLA-B27 negative, ANA negative, and polyarticular RF-negative subtype and have involvement of other joints have a higher likelihood of having TMJ symptoms. If a patient meets these criteria, careful evaluation of TMJs should take place.

Importance of Compliance Audits for a Pediatric Complicated Appendicitis Clinical Practice Guideline.

Clinical practice guidelines (CPG) have been shown to decrease practice variation, reduce resource use, and improve patient outcomes. The purpose of this study was to audit compliance of a pediatric complicated appendicitis CPG to identify areas for continued improvement. A comprehensive complicated appendicitis CPG was implemented in a children's hospital system. Outcomes were compared for 48 months pre- (01/2012 to 12/2015) and 28 months post-implementation (01/2016 to 04/2018). A detailed compliance audit was nested within the post-implementation period in 60 consecutive patients from 11/2017 to 03/2018. Feedback was provided to care providers throughout the audit. Overall, 2370 children with complicated appendicitis were identified (1366 pre-CPG and 1004 post-CPG). The CPG resulted in decrease in mean length of stay from 5.3 days to 4.5 days (p = 0.751), postoperative returns to the system (13.0% to 10.1%, p = 0.030), and readmissions (5.3% to 4.3%, p = 0.237). Central line use decreased from 11.2% to 5.5% (p < 0.001) and antibiotic selection improved from 47.0% to 84.1% (p < 0.001). On audit, only 15% (9/60) had full CPG compliance and 49% (29/60) received recommended antibiotic durations. Compliance increased from 7% to 23% with audit-derived feedback. After stratifying by appendicitis severity, audits resulted in improved antibiotic duration compliance for patients with severe appendicitis (38.1% to 66.7%, p = 0.07) and postoperative ambulation for patients with lower grade disease (37.5% to 83.3%, p = 0.06). Audit cycles on a complicated appendicitis CPG and feedback to providers improved CPG compliance and more granular outcomes of interest.

Implementing a surgeon-reported categorization of pediatric appendicitis severity.

PURPOSE: The purpose of this study was to implement a novel surgeon-reported categorization (SRC) for pediatric appendicitis severity and determine if SRC was associated with outcomes. METHODS: We conducted a retrospective review of appendectomies by 15 surgeons within a single center from January to December 2016. The SRC was defined as: simple (category 1), gangrenous or adherent (category 2A), perforation with localized abscess (category 2B), and perforation with gross contamination (category 2C). Logistic regression modeled the surgical site infections (SSI) and returns to the system. Cox proportional hazards analyses modeled the length of stay (LOS). RESULTS: The cohort included 697 patients (mean age 10.7 years). Compliance with SRC documentation increased from 33.5 to 85.9%. Review of operative findings revealed 100% concordance with SRC. The combined morbidity (SSI and revisits) rate was 9.8%. Category 2C patients had the highest odds of SSI (odds ratio 3.37 95% confidence interval 1.07-10.59). Median LOS increased with each category (category 1 = 1d, category 2A = 2d, category 2B = 4d, category 2C = 6d). When modeling intra-abdominal abscess, SRC displayed an improved model calibration and discrimination compared to wound class. CONCLUSION: SRC implementation is feasible and provides a granular assessment of appendicitis severity and outcomes. SRC may guide future quality improvement through development of grade-specific care pathways.

Ramifications of the Children's Surgery Verification Program for Patients and Hospitals.

BACKGROUND: The American College of Surgeons in 2015 instituted the Children's Surgery Verification program delineating requirements for hospitals providing pediatric surgical care. Our purpose was to examine possible effects of the Children's Surgery Verification program by evaluating neonates undergoing high-risk operations. STUDY DESIGN: Using the Kid's Inpatient Database 2009, we identified infants undergoing operations for 5 high-risk neonatal conditions. We considered all children's hospitals and children's units Level I centers and considered all others Level II/III. We estimated the number of neonates requiring relocation and the additional distance traveled. We used propensity score adjusted logistic regression to model mortality at Level I vs Level II/III hospitals. RESULTS: Overall, 7,938 neonates were identified across 21 states at 91 Level I and 459 Level II/III hospitals. Based on our classifications, 2,744 (34.6%) patients would need to relocate to Level I centers. The median additional distance traveled was 6.6 miles. The maximum distance traveled varied by state, from <55 miles (New Jersey and Rhode Island) to >200 miles (Montana, Oregon, Colorado, and California). The adjusted odds of mortality at Level II/III vs Level I centers was 1.67 (95% CI 1.44 to 1.93). We estimate 1 life would be saved for every 32 neonates moved. CONCLUSIONS: Although this conservative estimate demonstrates that more than one-third of complex surgical neonates in 2009 would have needed to relocate under the Children's Surgery Verification program, the additional distance traveled is relatively short for most but not all, and this program might improve mortality. Local level ramifications of this novel national program require additional investigation.

Hepatic and splenic blush on computed tomography in children following blunt abdominal trauma: Is intervention necessary?

BACKGROUND: There are no widely accepted guidelines for management of pediatric patients who have evidence of solid organ contrast extravasation ("blush") on computed tomography (CT) scans following blunt abdominal trauma. We report our experience as a Level 1 pediatric trauma center in managing cases with hepatic and splenic blush. METHODS: All pediatric blunt abdominal trauma cases resulting in liver or splenic injury were queried from 2008 to 2014. Patients were excluded if a CT was unavailable in the medical record. The presence of contrast blush was based on final reports from attending pediatric radiologists. Correlations between incidence of contrast blush and major outcomes of interest were determined using χ and Wilcoxon rank-sum tests for categorical and continuous variables, respectively, evaluating statistical significance at p < 0.05. RESULTS: Of 318 patients with splenic or liver injury after blunt abdominal trauma, we report on 30 patients (9%) with solid organ blush, resulting in 18 cases of hepatic blush and 16 cases of splenic blush (four patients had extravasation from both organs). Blush was not found to correlate significantly with age, gender, or type of injury (liver vs. splenic) but was found to associate with higher grades of solid organ injury (p = 0.002) and higher ISS overall (p < 0.001). Patients with contrast blush on imaging were more likely to be admitted to the intensive care unit (90% vs. 41%, p < 0.001), receive blood products, (50% vs. 12%, p < 0.001), and be considered for an intervention (p < 0.001). Eighty percent of patients with an isolated contrast blush of the spleen or liver did not require an operation. Only 17% of patients with blush required definitive treatment, such as embolization (n = 1), packing (n = 1), or splenectomy (n = 3). Blush had no significant correlation with overall survival (p = 0.13). CONCLUSIONS: The finding of a blush on CT from a splenic or liver injury is associated with higher grade of injury. These patients receive intensive medical management but do not uniformly require invasive intervention. From our data, we suggest that a blush can safely be managed nonoperatively and that treatment should be dictated by change in physiology. LEVEL OF EVIDENCE: Therapeutic study, level IV.