Management of Osler-Weber-Rendu syndrome is mostly supportive. Nasal packing and cautery may be required for routine epistaxis. Autologous skin grafting of the nasal septum, called septal dermoplasty, can be used in severe cases of recurrent epistaxis. Blood transfusions and iron replacement may be needed in some patients. Aminocaproic acid is an antifibrinolytic used to prevent and treat mucosal bleeding. Oral contraceptives can also be helpful in treating the chronic, obscure GI bleed.
Electrocautery and neodymium:yttrium-aluminum-garnet (Nd:YAG) laser ablation have been used to treat cutaneous skin lesions as well as mucosal telangiectases. Transluminal embolization of pulmonary fistulae is an effective therapy for patients with bilateral lung involvement. Solitary lesions, as well as symptomatic lesions in the setting of diffuse disease, can be resected surgically. Neurovascular surgery is indicated in select CNS AVMs. As with any genetic disease, family counseling and education about inheritance should be included in the management of the condition.
During this patient's acute presentation related to her COPD, the incidental finding of telangiectases led to a diagnosis of Osler-Weber-Rendu syndrome. She received smoking-cessation counseling while hospitalized and was discharged to home, in stable condition, after several days of oxygen supplementation, antibiotics, and corticosteroids for her COPD exacerbation.
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Cite this: Noah Gudel, Alyn Hatter, Stanley L. Fox, et. al. A 50-Year-Old With Telangiectasia, Cough, and Epistaxis - Medscape - Aug 20, 2021.