鼻中隔切除术和鼻中隔真皮成形术治疗遗传性出血性毛细血管扩张症伴鼻中隔穿孔的严重输血依赖性鼻出血
Septectomy and septal dermoplasty for the treatment of severe transfusion-dependent epistaxis in patients with hereditary hemorrhagic telangiectasia and septal perforation.
作者信息
Lesnik Gregory T, Ross Douglas A, Henderson Katharine J, Joe John K, Leder Steven B, White Robert I
机构信息
Yale University School of Medicine, New Haven, Connecticut 06520-8041, USA.
出版信息
Am J Rhinol. 2007 May-Jun;21(3):312-5. doi: 10.2500/ajr.2007.21.3017.
BACKGROUND
Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disorder characterized by abnormal angiogenesis with resultant telangiectasia formation in mucocutaneous tissues, visceral organs, and the central nervous system. The most common manifestation of HHT is epistaxis resulting from trauma to thin-walled telangiectasias. Many patients with HHT experience worsened epistaxis due to the presence of a septal perforation. Septal perforation in HHT patients results from aggressive noncartilage sparing treatments such as monopolar cauterization. Although the mainstay of treatment for patients with severe transfusion-dependent HHT remains to be septal dermoplasty (SD), patients with a septal perforation are less likely to have a successful outcome. In this small subset of patients, septectomy (ST) combined with SD is proposed to eliminate this variable to improve skin graft uptake and therefore outcome. This study reviews the indications, procedure, and outcome of nine patients with severe transfusion-dependent HHT and septal perforation who underwent the combined procedure of SD/ST.
METHODS
Nine HHT patients with severe transfusion-dependent epistaxis and septal perforation underwent SD/ST at our institution over a 5-year period. Quality of life, including number of daily events of epistaxis, and transfusion requirements were determined before and after surgery. Technical aspects of the procedure as well as complications were reviewed.
RESULTS
The combined procedure of SD/ST resulted in a long-lasting subjective improvement in quality of life for all patients. Similarly, transfusion requirements were reduced from 22.61 to 9.57 (p < 0.05). There were no complications or increased morbidity from the procedure.
CONCLUSION
Combined SD/ST is a safe and effective treatment for HHT patients with transfusion-dependent epistaxis and septal perforation.
背景
遗传性出血性毛细血管扩张症（HHT）是一种常染色体显性疾病，其特征为血管生成异常，导致在黏膜皮肤组织、内脏器官和中枢神经系统中形成毛细血管扩张。HHT最常见的表现是薄壁毛细血管扩张受创伤引起的鼻出血。许多HHT患者因鼻中隔穿孔而鼻出血加重。HHT患者的鼻中隔穿孔是由诸如单极烧灼等激进的非保留软骨治疗导致的。尽管对于严重依赖输血的HHT患者，主要治疗方法仍是鼻中隔真皮成形术（SD），但有鼻中隔穿孔的患者成功治疗的可能性较小。对于这一小部分患者，建议采用鼻中隔切除术（ST）联合SD以消除这一变量，从而提高皮肤移植的成功率，进而改善治疗效果。本研究回顾了9例严重依赖输血的HHT且有鼻中隔穿孔患者接受SD/ST联合手术的适应证、手术过程及治疗效果。
方法
9例严重依赖输血且有鼻中隔穿孔的HHT患者在我们机构于5年期间接受了SD/ST手术。在手术前后测定生活质量，包括每日鼻出血次数和输血需求。回顾了手术的技术细节以及并发症情况。
结果
SD/ST联合手术使所有患者的生活质量在主观上得到了长期改善。同样，输血需求从22.61降至9.57（p < 0.05）。该手术无并发症发生，也未增加发病率。
结论
SD/ST联合手术对于依赖输血且有鼻中隔穿孔的HHT患者是一种安全有效的治疗方法。
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