All issues > Volume 47(10); 2004

Case Report

Korean J Pediatr. 2004;47(10):1119-1123. Published online October 15, 2004.

The VACTERL Association : Tracheal Stenosis, Tracheal Bronchus and Partial Pulmonary Agenesis, Instead of Tracheoesophageal Fistula

Ji Sook JS Park¹, Hae Young HY Lee¹, Jong Sil JS Lee², Ji Hyeon JH Seo¹, Jae Young JY Lim¹, Myong Bum MB Choi¹, Chan Hoo CH Park¹, Hyang Ok HO Woo¹, Hee Sang HS Youn¹

¹Department of Pediatrics, College of Medicine, Gyeongsang National University, Jinju, Korea
²Department of Pathology, College of Medicine, Gyeongsang National University, Jinju, Korea

Correspondence Chan Hoo CH Park ,Email: aroma@nongae.gsnu.ac.kr

Abstract

VACTERL association is a disease with multiple congenital anomalies of the vertebrae, anus, cardia, tracheoesophageal(TE) fistula, renal and limb. This disease is derived from VATER anomaly, accompanied by cardiac and limb anomalies. We experienced a case of a 1-day-old boy with anal atresia, who represented multiple anomalies during hospital course. The multiple anomalies were hemivertebra, anal atresia, complex heart disease(coarctation of aorta, secundum aterial septal defect, patent ductus arteriosus), horseshoe kidney, tracheal stenosis, tracheal bronchus and left upper lung agenesis. Because both trachea and esophagus arise from fetal foregut, tracheal or pulmonary anomalies may be developed in VACTERL association instead of TE fistula. VACTERL association with tracheal anomalies, such as tracheal stenosis and tracheal bronchus or pulmonary agenesis, have been reported in foreign scientific society reports. But a case of VACTERL association with both tracheal bronchus and pulmonary agenesis has not reported yet. So we report this case with a brief review of related literature and suggest the consideration of possibility of tracheal or pulmonary anomaly in VACTERL association without TE fistula.

Keywords :VACTERL association, Tracheal stenosis, Tracheal bronchus, Pulmonary agenesis