Mitchell R O, Austin E H
Department of Surgery, University of Louisville School of Medicine, KY 40292.
J Pediatr Surg. 1993 Dec;28(12):1536-8. doi: 10.1016/0022-3468(93)90089-4.
Pulmonary arteriovenous malformation (AVM) is a congenital condition, but because the symptoms usually do not occur until middle age, the diagnosis is often delayed. The authors report on a neonate, diagnosed at 2 weeks of age, who was treated by lobectomy at 35 months of age. This prompted a review of the diagnosis, pathophysiology, and treatment of pulmonary AVM. In the literature there are approximately 500 cases of pulmonary AVM. Special attention is given to the reports involving children. Eight cases have been reported in which the diagnosis was made in the first year of life. In two series, a chest roentgenogram showed abnormalities in all affected patients. Because of the strong association of pulmonary AVM with hereditary telangiectasia (Rendu-Osler-Weber syndrome), it is recommended that family members of affected patients be screened by chest roentgenograms for pulmonary AVM.