Thromboembolic hazard in hereditary hemorrhagic telangiectasia.

A 23-year-old woman with hereditary hemorrhagic telangiectasia was found in her bedroom presenting right-side hemiparesis and apha-sia. Brain computed tomography angiography (CTA) showed a complete occlusion of left-medium cerebral artery ( Panel A ) that required mechanical thrombectomy. CTA revealed the typical arteriovenous malformations in the lungs ( Panel B ) but not in the brain or liver. A bubble contrasted echocar-diography ruled out intracardiacal shunts. Furthermore, repletion defects were found in subsegmentarial pulmonary arteries ( Panel C, amplified in the inset ). No thrombus was detected in lower extrem-ity veins with Doppler-ecography, and thrombophilia screening was negative. Urgent thrombectomy, rehabilitation, and anticoagulation reduced neurological sequels to hand dizziness and mild concentration com-plaints. Arteriovenous malformations were embolized. Hereditary hemorrhagic telangiectasia, also known as Rendu– Osler–Weber disease, is an autosomical-dominant condition char-acterized by angiodysplastic lesions and arteriovenous malformations. Traditionally,