Bower Ryan I, McArdle Ian W, Kohli Arpan, Unger Merv
Anesthesiology and Perioperative Medicine, West Virginia University School of Medicine, Morgantown, USA.
Cureus. 2025 Jul 7;17(7):e87471. doi: 10.7759/cureus.87471. eCollection 2025 Jul.
Ehlers-Danlos Syndrome (EDS) is a group of inherited, autosomal dominant connective tissue disorders caused by defects in procollagen and collagen production. Vascular EDS, also known as type 4 EDS, involves mutations in the COL3A1 gene, resulting in fragile vascular tissues and compromised organ integrity, which pose significant challenges during both labor and anesthesia. We report the case of a 19-year-old female patient with type 4 EDS, opioid-induced seizures, and a family history of malignant hyperthermia, who presented to the labor ward at 37 weeks of gestation. Due to her complex medical history, traditional methods for labor analgesia, including neuraxial techniques and remifentanil patient-controlled analgesia, were associated with significantly increased risks. A tailored pain management regimen was implemented, consisting of intravenous acetaminophen, cyclobenzaprine, and a ketamine infusion. The patient delivered a healthy neonate without complications. This case highlights the successful use of ketamine for labor analgesia in a high-risk obstetric patient with EDS.
埃勒斯-当洛综合征(EDS)是一组由前胶原和胶原蛋白生成缺陷引起的遗传性常染色体显性结缔组织疾病。血管型EDS,也称为4型EDS,涉及COL3A1基因突变,导致血管组织脆弱和器官完整性受损,这在分娩和麻醉过程中都带来了重大挑战。我们报告了一例19岁的4型EDS女性患者,她有阿片类药物诱发的癫痫发作以及恶性高热家族史,在妊娠37周时入住分娩病房。由于她复杂的病史,包括神经轴技术和瑞芬太尼患者自控镇痛在内的传统分娩镇痛方法风险显著增加。实施了一种定制的疼痛管理方案,包括静脉注射对乙酰氨基酚、环苯扎林和氯胺酮输注。患者顺利产下一名健康新生儿,无并发症。该病例突出了氯胺酮在一名患有EDS的高危产科患者分娩镇痛中的成功应用。