Hereditary hemorrhagic telangiectasia: a rare cause of portal thrombosis
DOI:
https://doi.org/10.11606/issn.1679-9836.v103i5e-227301Keywords:
Portal Thrombosis, Epistaxis, Hereditary Hemorrhagic TelangiectasiaAbstract
Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease manifested through arteriovenous malformations in skin, lungs, brain, mucous surfaces, and gastrointestinal tract. HHT has multiple clinical signs and symptoms such as epistaxis, gastrointestinal bleeding, and cerebral and pulmonary arteriovenous malformations. HHT confers an increased risk of venous thromboembolism. We present a 57-year-old woman with HHT manifested by epistaxis, intrapulmonary arteriovenous shunt, and portal thrombosis with cavernomatous transformation. She was submitted to a nasal cauterization, pulmonary angioembolization, and anticoagulation. The patient was discharged with remission of symptoms and oral rivaroxaban anticoagulation.
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