CTICasesHereditary Hemorrhagic Telangiectasia
Imaging findings
Chest radiograph shows a curved, tubular soft-tissue nodule in the left lower lobe. Contrast-enhanced chest CT demonstrates a classic pulmonary arteriovenous malformation (AVM) with a feeding artery arising from a subsegmental branch of the left lower lobe pulmonary artery and a draining vein connecting to a pulmonary vein. Two other smaller AVMs are present elsewhere in the lungs. The patient has a history of recurrent epistaxis.
Key takeaways
Hereditary hemorrhagic telangiectasia (HHT or Osler-Weber-Rendu disease) is an autosomal dominant vascular disorder. The classic triad includes telangiectasias, recurrent epistaxis, and visceral vascular malformations, most commonly pulmonary AVMs. Pulmonary AVMs act as right-to-left shunts, predisposing patients to paradoxical emboli, stroke, and brain abscesses, and are typically managed with embolotherapy if the feeding artery is 2-3 mm or larger.
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