Yoshida Akari, Kimoto Yoshiki, Ejiri Kanako, Mitani Yasuyuki, Kawamata Tomoyuki
Department of Anesthesiology, Wakayama Medical University, 811-1 Kimiidera, Wakayama City, 641-0012 Japan.
Second Department of Surgery, Wakayama Medical University, 811-1 Kimiidera, Wakayama City, 641-0012 Japan.
JA Clin Rep. 2016;2(1):30. doi: 10.1186/s40981-016-0059-0. Epub 2016 Oct 19.
Congenital factor VII (FVII) deficiency is a rare autosomal recessive coagulation disorder that is characterized by prolongation of prothrombin time. Recombinant activated FVII (rFVIIa) is widely used in the management of bleeding in patients with congenital FVII deficiency. We experienced anesthetic management of a patient with congenital FVII deficiency who was scheduled for laparoscopic colectomy using rFVIIa.
We report a 67-year-old man with rectal cancer who was diagnosed with congenital FVII deficiency. He was scheduled for laparoscopic colectomy. General anesthesia was performed with propofol, remifentanil, and rocuronium without epidural anesthesia. For coagulation management, 1 mg of rFVIIa was intravenously administered before starting surgery. During surgery, FVII activity and prothrombin time-international normalized ratio (PT-INR) were maintained to be above 10 % and within the normal range (0.8-1.2), respectively. The surgery was uneventfully completed.
We reported successful management of a patient with congenital FVII deficiency undergoing laparoscopic colectomy with monitoring of FVII activity and/or PT-INR.
先天性凝血因子VII（FVII）缺乏症是一种罕见的常染色体隐性凝血障碍，其特征是凝血酶原时间延长。重组活化FVII（rFVIIa）广泛用于先天性FVII缺乏症患者出血的治疗。我们遇到了一名计划行腹腔镜结肠切除术的先天性FVII缺乏症患者的麻醉管理情况，该患者使用了rFVIIa。
我们报告了一名67岁的直肠癌男性患者，其被诊断为先天性FVII缺乏症。他计划行腹腔镜结肠切除术。采用丙泊酚、瑞芬太尼和罗库溴铵进行全身麻醉，未使用硬膜外麻醉。为进行凝血管理，在手术开始前静脉注射1mg rFVIIa。手术期间，FVII活性和凝血酶原时间-国际标准化比值（PT-INR）分别维持在10%以上和正常范围内（0.8-1.2）。手术顺利完成。
我们报告了对一名先天性FVII缺乏症患者行腹腔镜结肠切除术时，通过监测FVII活性和/或PT-INR进行成功管理的病例。
