Multiple Pulmonary AVM's in Osler-Weber-Rendu

S/P Embolization Multiple Pulmonary AVM's

• Multiple metallic coils are present in the right lower lung field. These are coils placed by interventional radiology in an attempt to embolize some of the multiple arterio-venous malformations present in this patient with the Osler-Weber-Rendu syndrome (ORW) also known as multiple hereditary telangectasias.
• The opacity surrounding some of the coils could be due to AVM's although gell or other embolization material could conceivably account for some of the increased density.
• Did you also notice the bilateral hilar enlargement. This HIV+ patient had a history of treated tuberculosis, and hilar adenopathy is possible. It is also possible for some of the hilar enlargement to have a vascular component, either due to increased blood flow secondary to the AVM's or due to pulmonary artery hypertension. CT scan as well as pulmonary angiography could be useful here.
• The lateral film adds little to this case, apart from confirming the hilar enlargement.

• Autosomal dominant transmission.
• AVM's common on skin, mucous membranes (e.g., tongue)
• Frequently cause GI bleeding, epistaxis, hemoptysis.
• Appears as (multiple) pulmonary nodules.
  • Presence of feeding or draining vessels can be diagnostic.
• Commonly found in lower lobes (? pressure phenomenon).
  • Interesting is hypoxia which develops on standing due to increased right to left shunting.
• CNS complications secondary to intracerebral aneurysms, as well as infarcts and abscesses from pulmonary shunting, are well known.
• Embolotherapy used but may lead to opening of previously silent AVM's.
• Iron replacement therapy needed because of recurrent episodes of blood loss.

Ref: Fraser, R.S., Pare, J.A.P., Fraser, R.G., Pare, P.D. Synopsis of Diseases of the Chest, 2nd ed., W.B. Saunders, Philadelphia, PA, 1994, pp. 277-278.
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