Chua Criselle C, Cruz Mark Andrew B
Department of Anesthesiology, Philippine General Hospital, University of the Philippines Manila.
Acta Med Philipp. 2024 May 23;58(9):48-53. doi: 10.47895/amp.v58i9.8680. eCollection 2024.
Klippel-Feil Syndrome (KFS) continues to pose significant challenges for anesthesiologists. Beyond the expected complexities of managing difficult airways in these patients, they often present with systemic anomalies that can elevate the risk of morbidity during surgeries conducted under anesthesia. Furthermore, laparoscopic procedures bring about additional physiologic changes that must be taken into consideration when planning the anesthetic care for these individuals. This report details the anesthetic management of a 29-year-old female diagnosed with Klippel-Feil Syndrome (KFS) and concomitant Müllerian duct aplasia-Renal agenesis-Cervicothoracic Somite dysplasia (MURCS) as well as Chiari Type 1 Malformation, who underwent a successful pelvic laparoscopic surgery. The airway was secured through awake fiberoptic-guided intubation while general anesthesia was maintained with a combination of sevoflurane inhalation and remifentanil infusion. Intraoperatively, the team prioritized neuroprotection, lung-protective ventilation strategies, and renal preservation measures. The anesthetic management of patients with KFS necessitates a comprehensive assessment of their anomalies. Incorporating these considerations into the anesthetic management will help mitigate the procedure's adverse effects and lead to favorable patient outcomes.
克-费综合征(KFS)持续给麻醉医生带来重大挑战。除了这类患者气道管理的预期复杂性外,他们常伴有全身性异常,这会增加麻醉下手术时发病的风险。此外,腹腔镜手术会带来额外的生理变化,在为这些患者制定麻醉护理计划时必须予以考虑。本报告详细介绍了一名29岁女性的麻醉管理情况,该女性被诊断为克-费综合征(KFS),并伴有苗勒管发育不全-肾缺如-颈胸体节发育异常(MURCS)以及 Chiari 1型畸形,她成功接受了盆腔腹腔镜手术。通过清醒纤维支气管镜引导插管确保气道安全,同时采用七氟醚吸入和瑞芬太尼输注联合维持全身麻醉。术中,团队将神经保护、肺保护性通气策略和肾脏保护措施作为优先事项。对克-费综合征患者的麻醉管理需要对其异常情况进行全面评估。将这些考虑因素纳入麻醉管理将有助于减轻手术的不良影响,并带来良好的患者预后。