胃A-V malformationに基づく病変の証明されたRendu-Osler-Weber病の1例

Rendu-Osler-Weber Disease with a Gastric Lesion Consisting of A-V Malformation, Report of a Case

　Rendu-Oster-Weber病は，その別名hereditary hemorrhagic telangiectasiaが示すとおり，皮膚および粘膜の血管拡張性病変（telangiectasia）からの反復する出血が特徴であるが，この病変は消化管粘膜にも発生し，消化管出血の原因となることは既に成書に明らかに記されている．われわれは内視鏡的に，Rendu-Osler-Weber病（以下ROW病）に類似した胃病変を認め，それがangiographyにて，A-V malformationの所見を示した症例を経験したので，若干の文献的考察を加えて報告する．

　In this report, we demonstrated by celiac angiography and pathologic findings that a lesion of Rendu-Osler-Weber disease was composed of A-V malformation of the stomach.

　A 61 year-old man visited our clinic in January 1975 for precise examination because of recurrent hematemesis. By x-ray examination of upper G-Ⅰ tract, no abnormality was found, but a small red lesion was found on the greater curvature of the stomach by endoscopy, which could not be considered at that time as the cause of bleeding.

　One year later, he vomitted blood again, and the emergency endoscopy was performed. At this time, bleeding from the above gastric lesion was detected, and he was admitted immediately. By the endoscopical examination performed on the next day, the gastric lesion was reddish, slightly elevated from the mucosa, and a close observation by GIF type D-3 showed that the lesion consisted of telangiectasia. Furthermore, two lesions consisting of telangiectasia were found on the oral mucosa, and he was diagnosed as having Rendu-Osler-Weber disease although the family history was negative. Further examination with celiac angiography demonstrated that the lesion was in fact an A-V malformation.

　Because of the recurrent bleeding from the lesion, gastrectomy was performed. The gross specimen showed a small erosion in upper greater curvature, and microscopically, the lesion consisted of abundant arteries in contact with dilated veins.

　From these findings, we concluded that the gastric lesion of this patient with Rendu-Osler-Weber disease was composed of A-V malformation.