Pulmonary arteriovenous fistula is a rare disease first described by Churton in 1897 at
autopsy, but was first recognized clinically by Smith an4 Horton in 1938. Thereafter
more than 350 cases have been reported in the literature, but unfortunately no one has
been fouled 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 pulmonary 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 also been experienced intermittently.
On physical examination, multiple telangiectatic lesions and hemorrhagic spots were
noted in the upper and lower lips, tongue and dorsum of the right hand, and beneath
the nail beds of both fingers. Clubbing of fingers and toes were also 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 wall.
Multiple pulmonary arteriovenous fistulas were identified on both sides of the lung in
the pulmonary angingraphy. Arterial oxygen saturation was 9l.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-Weber'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 Rendu- Osler-Weber's disease was also accompanied.
|