Free-flap reconstruction for the management of life-threatening hereditary hemorrhagic telangiectasia

Abstract

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant multi-systemic disease that exhibits increasing penetrance with age. Some patients present with severe life-threatening epistaxis which is intractable to all common treatment modalities.A 63-year-old female patient with hereditary hemorrhagic telangiectasia (HHT) presented with recurrent life-threatening epistaxis that required repeated transfusions despite multiple embolizations and septodermoplasty. Previous septodermoplasty failed due to septal perforation. Resurfacing of the nasal lining with a free flap was planned. Total removal of the nasal mucosa and remaining septum was conducted to make the nasal cavity into one common cavity. Nasal passages were resurfaced with a radial forearm free flap. Following surgery, the patient experienced no further significant epistaxis.Fasciocutaneous free-flap resurfacing might represent a curative solution for cases of HHT intractable to conservative treatment and septodermoplasty, especially for patients with large septal perforation.