VACTERL associations in children undergoing surgery for esophageal atresia and anorectal malformations: Implications for pediatric surgeons

Timothy B. Lautz*, Ankur Mandelia, Jayant Radhakrishnan

*Corresponding author for this work

Research output: Contribution to journalArticle

14 Scopus citations

Abstract

Purpose The aim of this study was to compare the frequency and nature of VACTERL associations between children who underwent surgery for esophageal atresia/tracheoesophageal fistula (EA/TEF) and anorectal malformation (ARM). Methods We identified all children who underwent surgery for EA/TEF and/or ARM at hospitals participating in the Pediatric Health Information System (PHIS) database between 2004 and 2012. PHIS is an administrative database of free-standing children's hospitals managed by the Child Health Corporation of America (Overland Park, KS) that contains patient-level care data from 43 hospitals. The complete records of patients in this cohort were cross-referenced for diagnoses of vertebral, cardiac, renal and limb anomalies. Results 2689 children underwent repair of esophageal atresia. Mean gestational age was 36.5 ± 3.2 weeks and mean birth weight was 2536.0 ± 758.7 g. Associated VACTERL diagnoses included vertebral anomaly in 686 (25.5%), ARM in 312 (11.6%), congenital heart disease in 1588 (59.1%), renal disease in 587 (21.8%) and limb defect in 192 (7.1%). 899 (33.4%) had 3 or more anomalies and met criteria for a VACTERL diagnosis. 4962 children underwent repair of ARM. Mean gestational age was 37.4 ± 2.7 weeks and mean birth weight was 2895.2 ± 765.1 g. Associated VACTERL diagnoses included vertebral anomaly in 1562 (31.5%), congenital heart disease in 2007 (40.4%), EA/TEF in 348 (7.0%), renal disease in 1723 (34.7%) and limb defect in 359 (7.2%). 1795 (36.2%) had 3 or more anomalies and met criteria for a VACTERL diagnosis. Conclusion VACTERL associations are relatively common in children with EA/TEF and ARM and occur in specific clusters, with cardiac disease more common in EA/TEF and spinal and renal/urinary anomalies more common in ARM.

Original languageEnglish (US)
Pages (from-to)1245-1250
Number of pages6
JournalJournal of pediatric surgery
Volume50
Issue number8
DOIs
StatePublished - Aug 6 2014

Keywords

  • Anorectal malformation
  • Esophageal atresia
  • Trachea-esophageal fistula
  • VACTERL

ASJC Scopus subject areas

  • Surgery
  • Pediatrics, Perinatology, and Child Health

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