Free-flap reconstruction for the management of life-threatening hereditary hemorrhagic telangiectasia.
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.
Department of Plastic and Reconstructive Surgery, Ulsan University, College of Medicine, Asan Medical Center, Seoul, Republic of Korea.
Jong Woo
JW
Choi
Department of Otolaryngology, Gyeongsang National University Changwon Hospital, Changwon, Republic of Korea.
Yeon Hee
YH
Joo
Department of Plastic and Reconstructive Surgery, Ulsan University, College of Medicine, Asan Medical Center, Seoul, Republic of Korea.
Woo Shik
WS
Jeong
Department of Otolaryngology, Ulsan University, College of Medicine, Asan Medical Center, Seoul, Republic of Korea. Electronic address: jangyj@amc.seoul.kr.
Yong Ju
YJ
Jang
D004844:Epistaxis / Q000209:etiology / Q000601:surgery*; D005260:Female; D058752:Free Tissue Flaps; D006801:Humans; D008875:Middle Aged; D009296:Nasal Cavity / Q000601:surgery*; D009297:Nasal Mucosa / Q000601:surgery*; D061270:Nasal Septal Perforation / Q000601:surgery; D009300:Nasal Septum / Q000601:surgery*; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000150:complications / Q000601:surgery*
Epistaxis; Free flap; Hereditary hemorrhagic telangiectasia; Osler–Rendu–Weber syndrome; Septodermoplasty