Hereditary hemorrhagic telangiectasia and the anesthesiologist

Abstract

Osler-Weber-Rendu disease (OWRD) or Hereditary Hemorrhagic Telangiectasia (HHT) is a rare autosomal dominant disorder that causes muco-cutanesous and visceral vascular dysplasia and results in increased tendency for bleeding. Patients with HHT presenting with continuous bleeding pose a serious problem to the Anesthesiologist .Pre-existing anemia due to recurrent bleeding is common and sudden decompensation may lead to heart failure. Uncontrolled bleeding may occur from skin lesions during patient positioning and transport. Epistaxis may lead to aspiration of blood into trachea causing pulmonary edema. Intravenous access may be difficult. Sudden change in blood pressure may cause bleeding from arteriovenous malformations (AVMs) anywhere in the body, most serious of which is from cerebral AVM. Gastric distension may occur from ingested blood and may cause reflux and aspiration during induction. Any instrumentation including laryngoscopy and intubation, nasogastric tube insertion, urinary catheterization should be carried out with utmost caution as bleeding may occur from undetected lesions. Management include blood transfusion, antifibrinolytics and surgical hemostasis. Anesthesia strategy should include rapid sequence induction and controlled hypotension.