PDF(Pubmed)
Abstract：
Achondroplasia is an autosomal dominant inherited disorder that results in premature ossification of the epiphyseal growth plates and restriction of proximal long bone movement. Herein, we report the anesthetic management of such a patient undergoing stone retrieval in the lithotomy position. He had a restricted ability to flex and abduct the hip joint and lumbar hyperlordosis. These were anticipated to be a concern for the lithotomy position and prone to positional injuries. Hence, the decision was made to position the patient before induction of anesthesia in the presence of surgeons to ensure the optimal exposure needed for the procedure while avoiding any hyperflexion or abduction of the hip and knee joints. The position tolerated by the patient when awake and unsedated should be followed intraoperatively. Achondroplasia is also associated with difficult airway features, obesity, sleep apnea, and multisystem involvement. Careful preoperative evaluation and intraoperative vigilance are needed to manage these patients undergoing surgery.
摘要：
软骨发育不全是一种常染色体显性遗传性疾病，导致骨phy生长板过早骨化和近端长骨运动受限。在这里，我们报告了在截石位进行结石取出的患者的麻醉管理。他弯曲和绑架髋关节和腰椎过度前凸的能力有限。预计这些是截石术位置的担忧，容易发生位置伤害。因此，我们决定在麻醉诱导前在外科医生在场的情况下对患者进行定位,以确保手术所需的最佳暴露量,同时避免髋关节和膝关节过度屈曲或外展.术中应该遵循患者在清醒和未镇静时耐受的位置。软骨发育不全也与困难的气道特征有关，肥胖，睡眠呼吸暂停,多系统参与。需要仔细的术前评估和术中警惕来管理这些接受手术的患者。
