A Novel VACTERL Assessment Tool to Facilitate Counseling for Expectant Families

John S Riley; John Schomberg; Aditya Mantha; Yigit S Guner; Amy Cuevas; Courtney D Stephenson; William H Peranteau; Peter T Yu

doi:10.1159/000531611

A Novel VACTERL Assessment Tool to Facilitate Counseling for Expectant Families

Fetal Diagn Ther. 2023;50(5):368-375. doi: 10.1159/000531611. Epub 2023 Jun 20.

Authors

John S Riley¹, John Schomberg², Aditya Mantha³, Yigit S Guner^{4

5}, Amy Cuevas⁶, Courtney D Stephenson^{7

8}, William H Peranteau⁹, Peter T Yu^{4

5

6}

Affiliations

¹ Center for Fetal Research, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA, john.riley@pennmedicine.upenn.edu.
² Department of Trauma, Children's Hospital of Orange County Children's Health Foundation, Orange, California, USA.
³ Department of Cardiovascular Medicine, Cleveland Clinic, Cleveland, Ohio, USA.
⁴ Department of Surgery, University of California Irvine Medical Center, Orange, California, USA.
⁵ Division of Pediatric Surgery, Children's Hospital of Orange County, Orange, California, USA.
⁶ The Fetal Care Center of Southern California, Children's Hospital of Orange County and University of California Irvine Medical Center, Orange, California, USA.
⁷ Department of Obstetrics and Gynecology, University of North Carolina, Chapel Hill, North Carolina, USA.
⁸ Fetal Care Center, University of North Carolina, Chapel Hill, North Carolina, USA.
⁹ Center for Fetal Research, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.

PMID: 37339617
DOI: 10.1159/000531611

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 (<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 <1,750 g, and 239 (12.7%) died prior to discharge. Associated with mortality were limb anomaly (1.8 [1.01-3.22], p < 0.05), prematurity (1.99 [1.14-3.47], p < 0.02), and weight <1,750 g (2.19 [1.25-3.82], p < 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 < 0.001), vertebral anomaly (1.1 [1.05-1.14], p < 0.001), TE fistula (1.73 [1.66-1.81], p < 0.001), anorectal malformation (1.12 [1.07-1.16], p < 0.001), and weight <1,750 g (1.65 [1.57-1.73], p < 0.001).

Conclusion: This novel assessment tool may help providers counsel families confronting a VACTERL diagnosis.

Keywords: Congenital anomalies; Prenatal counseling; Prenatal diagnosis; VACTERL.