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


CONCLUSIONS
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.
Division of Pediatric Surgery, Department of Surgery, Ann & Robert H. Lurie Children's Hospital of Chicago and Northwestern University Feinberg School of Medicine, Chicago, IL, USA. Electronic address: tlautz@luriechildrens.org.
Timothy B
TB
Lautz
Division of Pediatric Surgery, Department of Surgery, Ann & Robert H. Lurie Children's Hospital of Chicago and Northwestern University Feinberg School of Medicine, Chicago, IL, USA.
Ankur
A
Mandelia
University of Illinois College of Medicine, Chicago, IL USA.
Jayant
J
Radhakrishnan
D000015:Abnormalities, Multiple / Q000175:diagnosis / Q000601:surgery; D001003:Anal Canal / Q000002:abnormalities*; D000071056:Anorectal Malformations; D001006:Anus, Imperforate / Q000175:diagnosis / Q000601:surgery*; D016208:Databases, Factual; D004933:Esophageal Atresia / Q000175:diagnosis / Q000601:surgery*; D004947:Esophagus / Q000002:abnormalities*; D005260:Female; D006330:Heart Defects, Congenital / Q000175:diagnosis; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007668:Kidney / Q000002:abnormalities*; D017880:Limb Deformities, Congenital / Q000175:diagnosis; D008297:Male; D012189:Retrospective Studies; D013131:Spine / Q000002:abnormalities*; D014132:Trachea / Q000002:abnormalities*; D014138:Tracheoesophageal Fistula / Q000175:diagnosis / Q000601:surgery*
Anorectal malformation; Esophageal atresia; Trachea-esophageal fistula; VACTERL