Karabiyik L, Parpucu M, Kurtipek O
Department of Anaesthesiology, Ministry of Health Ankara Training and Research Hospital, Ankara, Turkey.
Acta Anaesthesiol Scand. 2002 May;46(5):618-9. doi: 10.1034/j.1399-6576.2002.460525.x.
Osteogenesis imperfecta is a genetically determined rare disease of the connective tissue, associated with abnormalities of type 1 collagen. The primary bone lesion is the lack of normal ossification of the endochondrial bone. Patients with osteogenesis imperfecta present several problems for anaesthetists. They have a tendency to develop malignant or non-malignant hyperthermia. During laryngoscopy and tracheal intubation, the mandible, teeth and cervical spine may be fractured or injured, and mucosal bruising or bleeding may occur. Renal or ureteral stones are common. The main problems are thus with airway control and intubation, and the risk of anaesthetic agents triggering malignant hyperthermia. We describe the successful anaesthetic management of a patient with osteogenesis imperfecta, undergoing nephrolithotomy and ureterolithotomy with total intravenous anaesthesia including propofol, remifentanil and cisatracurium, using an intubating laryngeal mask.
成骨不全症是一种由基因决定的罕见结缔组织疾病，与1型胶原蛋白异常有关。主要的骨病变是软骨内骨正常骨化的缺乏。成骨不全症患者给麻醉医生带来了几个问题。他们有发生恶性或非恶性高热的倾向。在喉镜检查和气管插管期间，下颌骨、牙齿和颈椎可能骨折或受伤，并且可能发生粘膜瘀伤或出血。肾或输尿管结石很常见。因此，主要问题在于气道控制和插管，以及麻醉药物引发恶性高热的风险。我们描述了一名成骨不全症患者的成功麻醉管理，该患者接受了肾切开取石术和输尿管切开取石术，采用包括丙泊酚、瑞芬太尼和顺式阿曲库铵的全静脉麻醉，使用了插管喉罩。
