Chest ImagingOsler rendu weber disease
an inherited vascular disorder, also called hereditary haemorrhagic telangiectasia responsible for an angiodysplastic multisystemic process. The most frequent sites affected are cutaneomucous, cerebral, hepatic and pulmonary. The thoracic involvement consists rarely of bronchial or pleural telangiectasis and more often of pulmonary arteriovenous malformations (PAVMs). PAVMs are a significant cause of mortality and morbidity in patients with Osler Rendu Weber disease and must be treated. The right to left shunt through the PAVMs has the main following symptoms: hypoxia, cyanosis, digital hippocratism, stroke and cerebral abscess from paradoxical embolism. Rupture of PAVMs can occur in the bronchi with haemoptysis and air embolism or in the pleura with massive haemothorax. Until proven otherwise, PAVMs are numerous in Osler Rendu Weber disease and must be treated conservatively (see pulmonary vascular embolotherapy). They fall into three types:
simple with a single feeding
artery (
Fig.1),
"complex" with multiple feeding arteries; and
telangiectatic composed of numerous tiny vessels looking like an area of
ground glass attenuation at
CT.
It is virtually impossible to ascertain that a PAVM is simple because a tiny feeding artery can be overlooked at the first examination and become large only after the endovascular treatment of an apparently simple PAVM. Also, see Osler Rendu Weber disease.
JR - MRJ
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Large PAVM in a patient with a family history of OslerRenduWeber disease. Spiral CT acquisition and three-dimensional shaded surface display of the right lung base aimed at defining the pretherapeutic angioarchitecture of the PAVM of the laterobasal segment of the right lower lobe. This is a simple PAVM with a single feeding artery and a single draining vein.
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Osler rendu weber disease, Fig.1 | |