Watanobe Ikuo, Kida Hiroko, Sekine Yuuki, Kawai Masaya, Miyano Shozo, Machida Michio, Kitabatake Toshiaki, Sugo Hiroyuki, Lee Yoshifumi, Kojima Kuniaki
Department of General Surgery, Juntendo University Nerima Hospital, 3-1-10 Takanodai, Nerima-ku, Tokyo 177-8521, Japan.
Department of Nursing, Juntendo University Nerima Hospital, 3-1-10 Takanodai, Nerima-ku, Tokyo 177-8521, Japan.
Case Rep Surg. 2018 Jun 28;2018:1786786. doi: 10.1155/2018/1786786. eCollection 2018.
Congenital epidermolysis bullosa is a rare, genetic condition in which even slight stimulation can cause blistering of the skin or mucosa. While previous reports of treatments requiring general anesthesia in these patients were focused on anesthesia-related procedures, such as endotracheal intubation, no report has described specific management required for these patients during surgery, such as preparation of the surgical site, fixation of infusion lines and other tubes, and adjustment of the operation table. This is probably the first report to address these issues. This report presents a case of recessive dystrophic congenital epidermolysis bullosa in which open hepatectomy was safely performed.
先天性大疱性表皮松解症是一种罕见的遗传性疾病,即使轻微刺激也可导致皮肤或黏膜水疱形成。虽然此前关于这些患者需要全身麻醉的治疗报道主要集中在麻醉相关操作,如气管插管,但尚无报道描述这些患者在手术期间的具体管理措施,如手术部位准备、输液管和其他管道的固定以及手术台的调整。本文可能是首篇探讨这些问题的报道。本文报告了1例隐性营养不良型先天性大疱性表皮松解症患者,成功实施了开放性肝切除术。