Lee Dong Yun, Lim SooA, Eo SuRak, Yoon Jung Soo
Department of Plastic and Reconstructive Surgery, DongGuk University Medical Center, GoYang 10326, South Korea.
World J Clin Cases. 2023 Jun 16;11(17):4079-4083. doi: 10.12998/wjcc.v11.i17.4079.
Hemophilia, an uncommon yet consequential hereditary bleeding disorder, manifests as two clinically indistinguishable forms that hinder the normal functioning of the coagulation cascade. This impairment renders individuals more susceptible to excessive bleeding during significant surgical interventions. Moreover, individuals with severe hemophilia frequently encounter recurring hemarthrosis, resulting in progressive joint destruction and, subsequently, the need for hip and knee replacement surgeries.
The patient was a 53-year-old man with hemophilia A as the underlying disease and had self-injected factor VIII twice weekly for several decades. He had undergone ankle fusion surgery for recurrent hemarthrosis at the Department of Orthopedic Surgery 1 mo prior and was referred to our department because of skin necrosis after a hematoma at the surgical site. An anterolateral thigh perforator free flap was created after three cycles of factor VIII administration in addition to the concomitant administration of tranexamic acid (TXA) (Transamin 250 mg cap, 1 cap tid, q8h). After the operation, from postoperative days (PODs) 1-5, the factor VIII dose and interval were maintained, and q12h administration was tapered to q24h administration after POD 6. Because the patient's flap was stable 12 d after the operation, factor VIII administration was tapered to twice a week. At 6 mo follow-up, the patient recovered well without any complications.
To the best of our knowledge, there are very few reports of successful free flaps in patients with hemophilia, and none have been reported in patients with hemophilia A. Moreover, there are several reports on the efficacy of TXA in free flaps in general patients; however, there are no case reports of combining factor VIII and TXA in patients with hemophilia. Therefore, we report this case to contribute to future academic research.
血友病是一种罕见但后果严重的遗传性出血性疾病,表现为两种临床难以区分的形式,会阻碍凝血级联反应的正常功能。这种损害使个体在重大手术干预期间更容易出现过度出血。此外,重度血友病患者经常反复发生关节积血,导致关节进行性破坏,随后需要进行髋关节和膝关节置换手术。
该患者为一名53岁男性,患有甲型血友病,几十年来每周自行注射两次凝血因子VIII。他1个月前因复发性关节积血在骨科接受了踝关节融合手术,因手术部位血肿后出现皮肤坏死而转诊至我科。除了同时给予氨甲环酸(TXA)(氨甲环酸250毫克胶囊,1粒,每日三次,每8小时一次)外,在给予三个周期的凝血因子VIII后,制作了股前外侧穿支游离皮瓣。术后,术后第1至5天维持凝血因子VIII剂量和间隔,术后第6天起每12小时给药逐渐减至每24小时给药。由于患者的皮瓣在术后12天稳定,凝血因子VIII给药减至每周两次。在6个月的随访中,患者恢复良好,无任何并发症。
据我们所知,血友病患者成功进行游离皮瓣手术的报道非常少,甲型血友病患者尚无此类报道。此外,有几篇关于氨甲环酸在一般患者游离皮瓣手术中疗效的报道;然而,没有血友病患者联合使用凝血因子VIII和氨甲环酸的病例报告。因此,我们报告此病例以促进未来的学术研究。