Department of Anesthesiology, Peking Union Medical College Hospital, Beijing, 100730, China.
Front Med. 2017 Mar;11(1):48-52. doi: 10.1007/s11684-017-0500-4. Epub 2017 Mar 2.
Arthrogryposis multiplex congenita (AMC) is a rare disorder characterized by non-progressive, multiple contractures. In addition to affected extremities, patients may also present microstomia, decreased temporomandibular joint mobility. Although the etiology of AMC is unclear, any factor that decreases fetal movement is responsible for AMC. Thus, accurate diagnosis and classification are crucial to the appropriate treatment of AMC. The development of ultrasound technology has enabled prenatal diagnosis. Very early treatment is favorable, and multidisciplinary treatment is necessary to improve the function of AMC patients. Most patients require surgery to release contracture and reconstruct joints. However, perioperative care is challenging, and difficult airway is the first concern of anesthesiologists. Postoperative pulmonary complications are common and regional anesthesia is recommended for postoperative analgesia. This review on AMC is intended for anesthesiologists. Thus, we discuss the treatment and perioperative management of patients undergoing surgery, as well as the diagnosis and classification of AMC.
先天性多发性关节挛缩症（AMC）是一种罕见的疾病，其特征为非进行性、多发性挛缩。除受累的四肢外，患者还可能表现为小口畸形、颞下颌关节活动度降低。虽然 AMC 的病因尚不清楚，但任何导致胎儿运动减少的因素都可能导致 AMC。因此，准确的诊断和分类对于 AMC 的恰当治疗至关重要。超声技术的发展使得产前诊断成为可能。早期的治疗非常有利，多学科治疗对于改善 AMC 患者的功能是必要的。大多数患者需要手术来松解挛缩并重建关节。然而，围手术期的护理具有挑战性，困难气道是麻醉医生的首要关注点。术后肺部并发症很常见，建议使用区域麻醉进行术后镇痛。本篇关于 AMC 的综述旨在为麻醉医生提供参考。因此，我们讨论了接受手术治疗的患者的治疗和围手术期管理，以及 AMC 的诊断和分类。
