Inter- and Intra-rater Reliability of A Grading System for Congenital Diaphragmatic Hernia Defect Size.

BACKGROUND: The Congenital Diaphragmatic Hernia Study Group (CDHSG) registry is a multi-institutional tool to track outcomes of patients with CDH. The CDHSG asks surgeons to categorize diaphragmatic defect sizes as type A-D based on published guidelines. The reported size of the defect has been correlated with patient outcomes, but the reliability of this system has never been studied. Our goal was to evaluate the inter- and intra-rater reliability of the CDHSG grading system. MATERIALS AND METHODS: Forty-six operative notes from CDH patients that underwent surgical repair at a single institution were collected and cropped to include only the information necessary to grade the hernia defect based on the CDHSG guidelines. The defects were graded by nine pediatric surgeons on two separate occasions (18 wk apart). Inter-rater reliability was calculated using a Cohen's kappa (κ). Intra-rater reliability was calculated using an intraclass correlation coefficient. RESULTS: Inter-rater reliability was minimal to weak (κ round1 = 0.395, κ round2 = 0.424). Agreement ranged from 19.57% (κ = -0.0745) to 82.61% (κ = 0.7543). Inter-rater agreement was similar despite operative findings and outcomes: survival yes/no (κ = 0.3690, κ = 0.3518), need for ECMO yes/no (κ = 0.3323, κ = 0.3362), patch repair yes/no (κ = 0.2050, κ = 0.1916), and liver up/down (κ = 0.2941, κ = 0.4404). Intra-rater reliability was good to excellent (intraclass correlation coefficient = 0.88, 95% CI [0.83-0.92]). Agreement with oneself ranged from 71.74% to 93.48%. CONCLUSIONS: The demonstrated weak inter-rater reliability of the current CDHSG grading system shows the need for improvement in how the grading system is applied by surgeons when reporting CDH defect size.

A multifactorial severity score for left congenital diaphragmatic hernia in a high-risk population using fetal magnetic resonance imaging.

BACKGROUND: Adverse outcomes for infants born with left congenital diaphragmatic hernia (CDH) have been correlated with fetal imaging findings. OBJECTIVE: We sought to corroborate these correlations in a high-risk cohort and describe a predictive mortality algorithm combining multiple imaging biomarkers for use in prenatal counseling. MATERIALS AND METHODS: We reviewed fetal MRI examinations at our institution from 2004 to 2016 demonstrating left-side CDH. MRI findings, hospital course and outcomes were recorded and analyzed using bivariate and multivariable analysis. We generated a receiver operating curve (ROC) to determine a cut-off relation for mortality. Finally, we created a predictive mortality calculator. RESULTS: Of 41 fetuses included in this high-risk cohort, 41% survived. Per bivariate analysis, observed-to-expected total fetal lung volume (P=0.007), intrathoracic position of the stomach (P=0.049), and extracorporeal membrane oxygenation (ECMO) requirement (P<0.001) were significantly associated with infant mortality. Youden J statistic optimized the ROC for mortality at 24% observed-to-expected total fetal lung volume (sensitivity 64%, specificity 82%, area under the curve 0.72). On multivariable analysis, observed-to-expected total fetal lung volume ± 24% was predictive of mortality (adjusted odds ratio, 95% confidence interval: 0.09 [0.02, 0.55]; P=0.008). We derived a novel mortality prediction calculator from this analysis. CONCLUSION: In this high-risk cohort, decreased observed-to-expected total fetal lung volume and stomach herniation were significantly associated with mortality. The novel predictive mortality calculator utilizes information from fetal MR imaging and provides prognostic information for health care providers. Creation of similar predictive tools by other institutions, using their distinct populations, might prove useful in family counseling, especially where there are discordant imaging findings.

Can Anorectal Stenosis be Managed With Dilations Alone? A PCPLC Review.

PURPOSE: Congenital anorectal stenosis is managed by dilations or operative repair. Recent studies now propose use of dilations as the primary treatment modality to potentially defer or eliminate the need for surgical repair. We aim to characterize the management and outcomes of these patients via a multi-institutional review using the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) registry. METHODS: A retrospective database review was performed using the PCPLC registry. The patients were evaluated for demographics, co-morbidities, diagnostic work-up, surgical intervention, current bowel management, and complications. RESULTS: 64 patients with anal or rectal stenosis were identified (57 anal, 7 rectal) from a total of 14 hospital centers. 59.6% (anal) and 42.9% (rectal) were male. The median age was 3.2 (anal) and 1.9 years (rectal). 11 patients with anal stenosis also had Currarino Syndrome with 10 of the 11 patients diagnosed with a presacral mass compared to only one rectal stenosis with Currarino Syndrome and a presacral mass. 13 patients (22.8%, anal) and one (14.3%, rectal) underwent surgical correction. Nine patients (8 anal, 1 rectal) underwent PSARP. Other procedures performed were cutback anoplasty and anterior anorectoplasty. The median age at repair was 8.4 months (anal) and 10 days old (rectal). One patient had a wound complication in the anal stenosis group. Bowel management at last visit showed little differences between groups or treatment approach. CONCLUSION: The PCPLC registry demonstrated that these patients can often be managed successfully with dilations alone. PSARP is the most common surgical repair chosen for those who undergo surgical repair. LEVEL OF EVIDENCE: III.

Surgical History and Outcomes in Trisomy 13 and 18: A Thirty-year Review.

BACKGROUND: Patients with Trisomy 13(T13) and 18(T18) have many comorbidities that may require surgical intervention. However, surgical care and outcomes are not well described, making patient selection and family counseling difficult. Here the surgical history and outcomes of T13/ T18 patients are explored. METHODS: A retrospective review of patients with T13 or T18 born between 1990 and 2020 and cared for at a tertiary children's hospital (Riley Hospital for Children, Indianapolis IN) was conducted, excluding those with insufficient records. Primary outcomes of interest were rates of mortality overall and after surgery. Factors that could predict mortality outcomes were also assessed. RESULTS: One-hundred-seventeen patients were included, with 65% T18 and 35% T13. More than half of patients(65%) had four or more comorbidities. Most deaths occurred by three months at median 42.0 days. Variants of classic trisomies (mosaicism, translocation, partial duplication; p = 0.001), higher birth weight(p = 0.002), and higher gestational age(p = 0.01) were associated with lower overall mortality, while cardiac(p = 0.002) disease was associated with higher mortality. Over half(n = 64) underwent surgery at median age 65 days at time of first procedure. The most common surgical procedures were general surgical. Median survival times were longer in surgical rather than nonsurgical patients(p<0.001). Variant trisomy genetics(p = 0.002) was associated with lower mortality after surgery, while general surgical comorbidities(p = 0.02), particularly tracheoesophageal fistula/esophageal atresia(p = 0.02), were associated with increased mortality after surgery. CONCLUSIONS: Trisomy 13 and 18 patients have vast surgical needs. Variant trisomy was associated with lower mortality after surgery while general surgical comorbidities were associated with increased mortality after surgery. Those who survived to undergo surgery survived longer overall. LEVEL OF EVIDENCE: III.