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

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±3.2weeks and mean birth weight was 2536.0±758.7g. 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±2.7weeks and mean birth weight was 2895.2±765.1g. 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.

• Authors: Timothy B Lautz, Ankur Mandelia, Jayant Radhakrishnan
• Journal: Journal of pediatric surgery (J Pediatr Surg) Vol. 50 Issue 8 Pg. 1245-50 (Aug 2015) ISSN: 1531-5037 [Electronic] United States
• PMID: 25913268 (Publication Type: Comparative Study, Journal Article)
• Copyright: Copyright © 2015 Elsevier Inc. All rights reserved.

Topics

• Abnormalities, Multiple (diagnosis, surgery)
• Anal Canal (abnormalities)
• Anorectal Malformations
• Anus, Imperforate (diagnosis, surgery)
• Databases, Factual
• Esophageal Atresia (diagnosis, surgery)
• Esophagus (abnormalities)
• Female
• Heart Defects, Congenital (diagnosis)
• Humans
• Infant
• Infant, Newborn
• Kidney (abnormalities)
• Limb Deformities, Congenital (diagnosis)
• Male
• Retrospective Studies
• Spine (abnormalities)
• Trachea (abnormalities)
• Tracheoesophageal Fistula (diagnosis, surgery)