A Novel VACTERL Assessment Tool to Facilitate Counseling for Expectant Families

Abstract

Introduction: VACTERL is defined as 3 or more of the following congenital defects: vertebral, anorectal, cardiac, tracheoesophageal (TE), renal, and limb. The purpose of this study was to create an easy-to-use assessment tool to help providers counsel expecting families regarding the likelihood of additional anomalies and postnatal outcomes. Methods: Employing the Kids’ Inpatient Database from 2003–2016, neonates (&lt;29 days old) with VACTERL were identified using ICD-9-CM and ICD-10-CM codes. For each unique combination of VACTERL, multivariable logistic regression was used to estimate inpatient mortality, and Poisson regression was used to estimate length-of-stay during the initial hospitalization. Results: The assessment tool used in this study is available at <ext-link ext-link-type="uri" xlink:href="https://choc-trauma.shinyapps.io/VACTERL" xmlns:xlink="http://www.w3.org/1999/xlink">https://choc-trauma.shinyapps.io/VACTERL</ext-link>. 1,886 of 11,813,782 (0.016%) neonates presented with VACTERL. 32% weighed &lt;1,750 g, and 239 (12.7%) died prior to discharge. Associated with mortality were limb anomaly (1.8 [1.01–3.22], p &lt; 0.05), prematurity (1.99 [1.14–3.47], p &lt; 0.02), and weight &lt;1,750 g (2.19 [1.25–3.82], p &lt; 0.01). Median length-of-stay was 14 days (IQR: 7-32). Associated with increased length-of-stay were cardiac defect (1.47 [1.37–1.56], p &lt; 0.001), vertebral anomaly (1.1 [1.05–1.14], p &lt; 0.001), TE fistula (1.73 [1.66–1.81], p &lt; 0.001), anorectal malformation (1.12 [1.07–1.16], p &lt; 0.001), and weight &lt;1,750 g (1.65 [1.57–1.73], p &lt; 0.001). Conclusion: This novel assessment tool may help providers counsel families confronting a VACTERL diagnosis.