Korean Journal of Pediatrics 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 Park1, Hae Young Lee1, Jong Sil Lee2, Ji Hyeon Seo1, Jae Young Lim1, Myong Bum Choi1, Chan Hoo Park1, Hyang Ok Woo1, Hee Sang Youn1
1Department of Pediatrics, College of Medicine, Gyeongsang National University, Jinju, Korea
2Department of Pathology, College of Medicine, Gyeongsang National University, Jinju, Korea
기관과 폐기형을 동반한 VACTERL(Vertebrae, Anus, Cardia, TracheoEsophageal, Renal, Limb) 연합 1례 - 기관 식도 누공 없이 기관 협착과 기관 기관지, 부분 폐 무형성을 동반 -
박지숙1, 이혜영1, 이종실2, 서지현1, 임재영1, 최명범1, 박찬후1, 우향옥1, 윤희상1
1경상대학교 의과대학 소아과학교실
2경상대학교 의과대학 병리학교실
Correspondence: 
Chan Hoo 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.
Key Words: VACTERL association, Tracheal stenosis, Tracheal bronchus, Pulmonary agenesis


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