Brown B, Steed D L, Webster M W, Makaroun M S, Spero J A, Bontempo F A, Ragni M V, Lewis J H
Surgery. 1986 Feb;99(2):154-9.
From 1976 to 1984, 23 operations were performed on 22 patients with hemophilia (18 patients with factor VIII and four with factor IX deficiency). Elective procedures included resection of abdominal aortic aneurysm, liver transplantation, vagotomy/pyloroplasty, insertion of Mousseau-Barbin tube, colectomy, cholecystectomy, inguinal herniorrhaphy (four patients), colonoscopy/polypectomy, mediastinoscopy, arteriovenous fistula for dialysis, anal fistulectomy, and miscellaneous skin and soft-tissue procedures (five patients). Emergency operations were appendectomy (two patients), repair of bleeding liver biopsy site, and repair of an incarcerated inguinal hernia. There were two deaths (9%) within 30 days of operation, neither directly caused by the coagulopathy. Four patients had bleeding after surgery, which was treated with additional cryoprecipitate or factor concentrate. There were no nonhemorrhagic complications. Before operation, appropriate replacement therapy with factor VIII concentrate, cryoprecipitate, or fresh-frozen plasma was provided. Coagulation factor levels were measured before operation and monitored daily after operation. Generally, factor levels were raised to at least 1.0 U/ml and maintained at greater than 0.5 U/ml for 7 to 14 days after operation. However, when patients were treated with fresh-frozen plasma, plasma exchange was performed and factor levels of approximately 0.35 U/ml were achieved before surgery. We conclude that operations in patients with hemophilia can be accomplished safely with careful monitoring of coagulation factor levels and appropriate replacement therapy.
1976年至1984年期间，对22例血友病患者实施了23次手术（18例VIII因子缺乏患者和4例IX因子缺乏患者）。择期手术包括腹主动脉瘤切除术、肝移植、迷走神经切断术/幽门成形术、插入穆索 - 巴尔宾管、结肠切除术、胆囊切除术、腹股沟疝修补术（4例患者）、结肠镜检查/息肉切除术、纵隔镜检查、用于透析的动静脉瘘、肛瘘切除术以及各种皮肤和软组织手术（5例患者）。急诊手术包括阑尾切除术（2例患者）、出血性肝活检部位修复以及嵌顿性腹股沟疝修补术。术后30天内有2例死亡（9%），均非直接由凝血障碍所致。4例患者术后出血，通过补充冷沉淀或凝血因子浓缩物进行治疗。无非出血性并发症。术前给予适当的VIII因子浓缩物、冷沉淀或新鲜冰冻血浆替代治疗。术前测定凝血因子水平，术后每日监测。一般来说，术后将因子水平提高到至少1.0 U/ml，并在术后7至14天维持在大于0.5 U/ml。然而，当患者接受新鲜冰冻血浆治疗时，进行血浆置换，术前达到约0.35 U/ml的因子水平。我们得出结论，通过仔细监测凝血因子水平和适当的替代治疗，血友病患者的手术可以安全完成。
