| Case Report : A Case of Pulmonary Arteriovenous Fistula Associated with Rendu-Osler-Weber`s Disease |
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Seon Yang Park1, Jin Young Kang1, Jeong Euy Park1, You Young Kim1, Yong Chul Han1, Man Chung Han2, Yong Hyeun Kim3 |
1Department of Internal Medicine, Seoul National University, Seoul, Korea
2Department of Radiology, Seoul National University, Seoul, Korea
3Y. H. Kim X-ray Clinic |
| Rendu-Osler-Weber 씨병을 동반한 폐동정맥루공 1예 |
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박선양1, 강진영1, 박정의1, 김유영1, 한용철1, 한만청2, 김용현3 |
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| Abstract |
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Pulmonary arteriovenous fistula is a rare disease first described by Churton in 1897 at autopsy, but was first recognized clinically by Smith and Horton in 1938. Thereafter more than 350 cases have been reported in the literature, but unfortunately no one has been found in Korea as yet. It is known to be accompanied by Rendu-Osler-Weber’s disease or hereditary hemorrhagic telangiectasia in 40- 65% of cases.
Recently the authors have detected a patient with multiple pulmònary arteriovenous fistulas associated with Rendu-Osler-Weber’s disease who is presented here with a brief review of the previous literatures.
The patient was a 30-year-old male who has suffered from easy fatigability and anemia since childhood. Since about three years ago, generalized weakness, mild headache and transient visual disturbance have aIso been experienced intermittently.
On physicaI examination, multiple telangiectatic lesions and hemorrhagic spots were noted on the upper and Iower lips, tongue, and dorsum of the right hand, and beneath the nail beds of both fingers. Clubbing of fingers and toes were aIso found. Continuous murmur of grade 4 was audible with late systolic accentuation about 10 cm below the inferior angle of the right scapula on the posterior chest waII.
Multiple pulmonary arteriovenous fistulas were identified on both sides of the lung in the pulmonary angiography. ArteriaI oxygen saturation was 91. 8%, and peripheral blood findings and bone marrow examination revealed iron deficiency anemia, which was thought to be due to chronic gastrointestinal bleeding which was evidenced by occult blood in stool detected by guaiac test.
Five more members of his family were suspected to have pulmonary arteriovenous fistula or Rendu-Osler-W eber’s disease, though only one was examined by the authors at present.
He was discharged without operation in view of the fact that he had multiple pulmonary arteriovenous fistulas which, however, had not caused any severe symptom, and that RenduOsIer-Weber’s disease was aIso accompanied. |
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