The Journal of the Japanese Association for Chest Surgery
Online ISSN : 1881-4158
Print ISSN : 0919-0945
ISSN-L : 0919-0945
Pulmonary arteriovenous malformations with Rendu-Osler-Weber disease: A familial case
Ryoji OnariShinkichiro YoshiokaMasayuki NishikiMasaki MukaiTeruhisa TakuwaYoshihiro MiyataToshimasa Asahara
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2007 Volume 21 Issue 1 Pages 23-28

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Abstract

Pulmonary arteriovenous malformations (PAVMs) are rare, mostly congenital, and are often associated with Rendu-Osler-Weber disease (ROW dis.=Hereditary hemorrhagic telangiectasia). PAVM is caused by an abnormal communication between the pulmonary arteries and veins. We present four cases of familial PAVM with ROW, which were successfully treated by three therapeutic options. Case 1: A 45-year-old woman with a solitary large PAVM, which was located centrally in lingular lobe, underwent bisegmentectomy with thorachotomy. Case 2: A 16-year-old man with multiple PAVMs, which were located in the rt. S9b and S10b, underwent wide wedge resection by video-assisted thorachoscopic surgery (VATS). Case 3: A 22-year-old woman with multiple small PAVMs, which are located in four lobes, undedrwent angiographic embolization. Case 4: A 21-year-old man with a solitary small PAVM, which was located in the lt. S9b,underwent wide wedge resection by VATS. For most PAVMs, angiographic embolization is the standard therapy. Limited resection of the lung, with a low mortality, low morbidity and low recurrence rate, carries the same risk as angiographic embolization. In well-selected cases, i.e., when PAVMs are closely located and/or large, with a high risk associated with embolotherapy, surgery is preferable to angiographic embolization.

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© 2007 The Japanese Association for Chest Surgery
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