Antunes Beatriz, Araújo Marta, Noversa Carlos, Pedreira Joana, Marques Miguel
Anesthesiology, Unidade Local de Saúde de Braga, Braga, PRT.
Cureus. 2024 Oct 1;16(10):e70667. doi: 10.7759/cureus.70667. eCollection 2024 Oct.
Patients with hypermobile Ehlers-Danlos syndrome (hEDS) usually present with generalized joint hypermobility and pain, soft and hyperextensible skin with atrophic scars and easy bruising, periodontitis, mitral valve prolapse, and aortic root dilation. It may also lead to cervical spine instability or collapse of larynx cartilage/trachea, which can result in intubation difficulties and possible mucosal damage. Lung protective ventilation must be performed to prevent pneumothorax. Bruising, scoliosis, spondylosis, meningeal Tarlov cysts, increased risk for postdural puncture headache (PDPH), and resistance to local anesthetics may affect neuraxial anesthesia. This article aims to report an anesthetic approach of a 24-year-old woman with hEDS proposed for cesarean delivery. Additional past medical history included cutaneous psoriasis, polycystic ovarian syndrome, and bilateral hip dysplasia. No previous allergies were reported. She has been previously submitted to an uneventful general anesthesia. After rapid-sequence induction of general anesthesia, endotracheal intubation was performed with videolaryngoscopy. Balanced anesthesia with volatile anesthetics was used. The birth and recovery were safely managed without adverse events. hEDS can present challenges for both neuraxial anesthesia and orotracheal intubation. In this case, the initial anesthetic plan included general anesthesia and an airway approach with a videolaryngoscope. Patient positioning and padding were carefully executed to prevent bruising and joint dislocations.
活动度过高型埃勒斯-当洛综合征（hEDS）患者通常表现为全身关节活动过度及疼痛、皮肤柔软且过度伸展伴有萎缩性瘢痕和易瘀伤、牙周炎、二尖瓣脱垂以及主动脉根部扩张。它还可能导致颈椎不稳定或喉软骨/气管塌陷，进而造成插管困难及可能的黏膜损伤。必须进行肺保护性通气以预防气胸。瘀伤、脊柱侧弯、脊椎病、硬脊膜外Tarlov囊肿、腰穿后头痛（PDPH）风险增加以及对局部麻醉药耐药可能会影响神经轴索麻醉。本文旨在报告一名拟行剖宫产的24岁hEDS女性患者的麻醉方法。既往病史还包括皮肤银屑病、多囊卵巢综合征和双侧髋关节发育不良。未报告有既往过敏史。她既往接受过一次顺利的全身麻醉。在快速顺序诱导全身麻醉后，使用视频喉镜进行气管插管。采用挥发性麻醉药进行平衡麻醉。分娩和恢复过程均安全管理，未出现不良事件。hEDS对神经轴索麻醉和经口气管插管均可能带来挑战。在此病例中，最初的麻醉计划包括全身麻醉和使用视频喉镜的气道处理方法。仔细进行患者体位摆放和衬垫以防止瘀伤和关节脱位。
