Bojaxhi Elird, Kalava Arun, Greengrass Roy
Department of Anesthesiology, Mayo Clinic, Jacksonville, Florida, USA.
Department of Anesthesiology, Tampa General Hospital, Tampa, Florida, USA.
Rom J Anaesth Intensive Care. 2016 Apr;23(1):67-71. doi: 10.21454/rjaic.7518.231.nss.
We present a case of regional analgesia utilized in a 43-year-old woman with Bernard-Soulier syndrome (BSS) undergoing a Nuss procedure for the treatment of pectus excavatum. BSS is an extremely rare bleeding disorder (1:1,000,000) associated with prolonged bleeding times, giant platelets, and thrombocytopenia. Due to the rare incidence and heterogeneity in bleeding predisposition due to BSS, there is no clear consensus in management of such cases, and to our knowledge, utilization of regional analgesic techniques have not been described in the literature. The Nuss procedure is considered "minimally" invasive, and epidural analgesia is frequently utilized at our institution. Due to our patient's heterozygous presentation of BSS and mild history of bleeding, a modified perioperative multimodal analgesic plan was chosen which included bilateral single injection paravertebral nerve blocks (PVBs). Our report describes successful utilization of PVBs in a patient with BSS and our approach to this rare hereditary condition.
我们报告了一例43岁患有伯纳德-苏利耶综合征(BSS)的女性患者,在接受用于治疗漏斗胸的努斯手术时使用区域镇痛的病例。BSS是一种极其罕见的出血性疾病(发病率为1:1,000,000),与出血时间延长、巨大血小板和血小板减少有关。由于BSS的发病率罕见且出血易感性存在异质性,对此类病例的管理尚无明确共识,据我们所知,文献中尚未描述区域镇痛技术的应用。努斯手术被认为是“微创”手术,我们机构经常使用硬膜外镇痛。由于我们的患者为BSS杂合表现且有轻度出血史,因此选择了改良的围手术期多模式镇痛方案,其中包括双侧单次注射椎旁神经阻滞(PVB)。我们的报告描述了PVB在BSS患者中的成功应用以及我们针对这种罕见遗传病的处理方法。