Cesare Alexandra E, Rafer Lorenzo C, Myler Conrad S, Brennan Kristin B
Department of Anesthesiology and Perioperative Medicine, Penn State Health Milton S. Hershey Medical Center, Hershey, PA, USA.
Am J Case Rep. 2019 Jan 10;20:39-42. doi: 10.12659/AJCR.912799.
BACKGROUND Ehlers-Danlos syndrome, hypermobility type is characterized by increased extensibility, permeability, and fragility of the affected cartilaginous tissues, including the trachea, larynx, and skin. Anesthetic considerations for patients with this syndrome include intubation difficulties secondary to the collapse of fibro-elastic tissues in the trachea and a reported resistance to local anesthetics. CASE REPORT Our patient was a 22-year-old G4P0030 woman with a history of morbid obesity, seizures, Barrett's esophagus, hypermobility being evaluated for Ehlers-Danlos syndrome, and anaphylaxis to an unknown local anesthetic who was scheduled for cesarean delivery. She refused allergy testing. After rapid-sequence induction of general anesthesia, video laryngoscopy facilitated endotracheal intubation. Delivery and recovery were uneventful for the mother and child. CONCLUSIONS No guidelines for neuraxial or general anesthesia exist for patients with Ehlers-Danlos syndrome, hypermobility type. Increased rates of cervical spine instability and local anesthetic resistance have been reported in this population and should be considered when developing the anesthetic plan.
关节活动过度型埃勒斯-当洛综合征的特征是包括气管、喉部和皮肤在内的受累软骨组织的伸展性、通透性和脆弱性增加。该综合征患者的麻醉注意事项包括气管内纤维弹性组织塌陷导致的插管困难以及对局部麻醉药的耐药性。病例报告：我们的患者是一名22岁、孕4产0、有肥胖症、癫痫、巴雷特食管病史、因关节活动过度接受埃勒斯-当洛综合征评估且对一种未知局部麻醉药过敏的女性，计划行剖宫产。她拒绝过敏测试。在快速顺序诱导全身麻醉后，视频喉镜辅助进行了气管插管。母婴分娩和恢复过程顺利。结论：对于关节活动过度型埃勒斯-当洛综合征患者，目前尚无关于神经轴索麻醉或全身麻醉的指南。该人群中颈椎不稳定和局部麻醉药耐药率增加，制定麻醉计划时应予以考虑。
