Associated congenital anomalies between neonates with short-gap and long-gap esophageal atresia: a comparative study

Abstract

Background:Predicting the presence of long-gap esophageal atresia (EA) prior to the surgery is of clinical importance. No comparison between short-gap and long-gap EA for the prevalence of VACTERL and non-VACTERL-type anomalies has yet been performed.Objective:The aim of this study was to compare VACTERL and non-VACTERL-type anomalies between patients with short-gap and long-gap EA.Methods:Retrospectively, medical records of all newborns managed for EA/tracheoesophageal fistula (TEF) in Tabriz Children’s Hospital and Tehran Mofid Hospital between 2007 and 2010 were evaluated. Demographic data and associated anomalies including both the VACTERL and non-VACTERL-type defects were listed. The VACTERL spectrum defects covered vertebral/costal, anorectal, cardiovascular, TEF, and renal- or radial-type limb anomalies. The non-VACTERL-type anomalies included hydrocephalus, orofacial defects, respiratory system anomalies, gastrointestinal anomalies, genital anomalies, and non-VACTERL limb defects. Demographic data, and the VACTERL and non-VACTERL-type anomalies were compared among children with long-gap EA and those with short-gap EA.Results:Two hundred and seventy-six children were included in the study: 230 (83.3%) in the short-gap EA group and 46 (16.7%) in the long-gap EA group. Although prevalence of the VACTERL spectrum anomalies did not differ between the two groups, the non-VACTERL anomaly was more common in the long-gap EA group (P = 0.02). Among the VACTERL-type defects, TEF was detected in 30 (65.2%) and 218 (94.7%) patients in long-gap and short-gap EA groups, respectively (P = 0.0001).Conclusion:The non-VACTERL-type anomalies, but not the VACTERL spectrum defects, are more frequent in patients with long-gap EA than those with short-gap EA.

Long-Gap Esophageal Atresia Is a Unique Entity within the Esophageal Atresia Defect Spectrum

“…How can we reconcile our results with a previous study 14 that found no statistically difference in the occurrence VACTERL-associated anomalies between subjects with LGEA and non-LGEA? It is notable that the rate of detection of VACTERL anomalies was considerably higher in our study, raising the possibility that we detected these such anomalies with greater sensitivity.…”

“…For example, compared to other studies, we had a high proportion of LGEA patients that is attributable to our institution being a referral center for LGEA. 14 Our cohort included complex patients, often transferred from other facilities with complicated records and potentially inconsistent data collection, raising the possibility that despite all efforts some anatomical anomalies may have been missed. In addition, our CMA analysis covered only a subset of patients; this costly testing had been used clinically in only a subset of patients, and our study funds did not cover research-based testing for this cohort.…”

Neurologic Injury and Brain Growth in the Setting of Long-Gap Esophageal Atresia Perioperative Critical Care: A Pilot Study

“…Similar findings of increased extra-axial space (viz. idiopathic external hydrocephalus without evidence of ventricular enlargement or hydrocephalus) and ventricular enlargement were previously reported in infants with LGEA [16,17,67]. Such findings have also been described as part of a benign condition in the absence of surgery [68][69][70][71] and as part of external hydrocephalus not associated with cerebral atrophy [72].…”

Infant Corpus Callosum Size After Surgery and Critical Care for Long-Gap Esophageal Atresia: Qualitative and Quantitative MRI

“…Although some patients had VACTERL association (Table 1), none had concomitant hydrocephalus (viz. VACTERL-H) 48,49 , known to be of autosomal recessive or X-linked recessive inheritance [49][50][51][52] . Similarly, none of the subjects included in analysis had cardiac involvement (less severe cases).…”