[Surgical indications of pulmonary arteriovenous fistulae in Osler disease].
Hereditary hemorrhagic teleangiectasia (HHT) is known to be associated in 15% with pulmonary arterio-venous fistulas (PAVF). Symptoms and complications of these fistulas are: hemoptysis, dyspnea, hemothorax, cerebral embolization and brain abscess. We studied a family with 7 members known to have HHT. 3 had PAVF. Patient 1 died of a hemothorax and pleural empyema. PAVF were diagnosed at autopsy. Patient 2 (nephew) suffered from a cerebellar abscess. The fistula-bearing part of the lung was removed by wedge resection. Patient 3 (son of the former) had no symptoms, but the arterial PO2 was 6.7 kPa. In order to prevent complications like in his father, the patient was operated. A lobar resection was necessary to remove the fistula. The postoperative course was eventless in patients 2 and 3. We conclude, that periodical thorax X-ray studies are indicated for all members of families with HHT in order to find PAVF before complications occur. The classical treatment is resection of the fistula with as little lung tissue as possible. Good results have been reported lately with embolization of PAVF, but this method is not generally available yet.
Olah
A
A
Departement Chirurgie, Universitätsspital Zürich.
Carrel
T
T
Pasic
M
M
Niederhäuser
U
U
Lüthy
A
A
Turina
M
M
D000293:Adolescent; D000328:Adult; D001165:Arteriovenous Malformations / Q000235:genetics / Q000601:surgery*; D002869:Chromosome Aberrations / Q000235:genetics; D025063:Chromosome Disorders; D005799:Genes, Dominant / Q000235:genetics; D006801:Humans; D008297:Male; D010375:Pedigree; D011651:Pulmonary Artery / Q000002:abnormalities* / Q000601:surgery; D011667:Pulmonary Veins / Q000002:abnormalities* / Q000601:surgery; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000235:genetics / Q000601:surgery*
Zur Operationsindikation der pulmonalen AV-Fisteln beim M. Osler.