Choi Jong Woo, Joo Yeon Hee, Jeong Woo Shik, Jang Yong Ju
Department of Plastic and Reconstructive Surgery, Ulsan University, College of Medicine, Asan Medical Center, Seoul, Republic of Korea.
Department of Otolaryngology, Gyeongsang National University Changwon Hospital, Changwon, Republic of Korea.
Auris Nasus Larynx. 2017 Oct;44(5):607-611. doi: 10.1016/j.anl.2016.08.005. Epub 2016 Sep 8.
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.
遗传性出血性毛细血管扩张症（HHT）是一种常染色体显性多系统疾病，其发病率随年龄增长而增加。一些患者会出现严重的、危及生命的鼻出血，所有常见治疗方法对此都难以奏效。一名63岁的遗传性出血性毛细血管扩张症（HHT）女性患者，反复出现危及生命的鼻出血，尽管进行了多次栓塞和鼻中隔成形术，仍需反复输血。之前的鼻中隔成形术因鼻中隔穿孔而失败。计划采用游离皮瓣对鼻黏膜进行修复。彻底切除鼻黏膜和剩余的鼻中隔，使鼻腔成为一个共同的腔隙。用桡侧前臂游离皮瓣对鼻道进行修复。术后，患者未再出现严重鼻出血。对于保守治疗和鼻中隔成形术难以治疗的HHT病例，尤其是存在大的鼻中隔穿孔的患者，筋膜皮瓣游离皮瓣修复可能是一种治愈性解决方案。
