Korean J Pediatr.  2004 Oct;47(10):1119-1123.

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

• Park JS
• Lee HY
• Lee JS
• Seo JH
• Lim JY
• Choi MB
• Park CH
• Woo HO
• Youn HS

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.

Keyword

VACTERL association; Tracheal stenosis; Tracheal bronchus; Pulmonary agenesis

MeSH Terms

Anal Canal
Anus, Imperforate
Aorta
Bronchi*
Cardia
Esophagus
Extremities
Fistula
Heart
Humans
Kidney
Lung
Male
Societies, Scientific
Spine
Trachea
Tracheal Stenosis*
Tracheoesophageal Fistula*