Machado Francisco, Gouveia Henrique, Freitas Sara, Rodrigues Filipa
Department of Anaesthesiology, Hospital Central do Funchal - SESARAM, EPERAM, São Martinho, Funchal, Portugal.
Saudi J Anaesth. 2025 Jul-Sep;19(3):443-445. doi: 10.4103/sja.sja_839_24. Epub 2025 Jun 16.
Von Hippel-Lindau disease (VHLD) is a rare autosomal dominant genetic disorder characterized by the development of highly vascularized tumors, including central nervous system (CNS) hemangioblastomas, pheochromocytomas, and renal cell carcinomas. Pregnancy in patients with VHLD presents unique anesthetic challenges due to tumor-associated risks and physiological changes. This report describes the anesthetic management of a 31-year-old woman with VHLD and twin gestation during active labor at 35 weeks of pregnancy. Despite the advantages of neuraxial anesthesia for cesarean delivery, general anesthesia was selected due to the unavailability of recent imaging and the potential risks of neuraxial techniques in such cases. The case emphasizes the importance of individualized planning, hemodynamic stability, and multidisciplinary collaboration to optimize outcomes for both mother and neonates.
冯·希佩尔-林道病（VHLD）是一种罕见的常染色体显性遗传病，其特征是发展出高度血管化的肿瘤，包括中枢神经系统（CNS）血管母细胞瘤、嗜铬细胞瘤和肾细胞癌。由于肿瘤相关风险和生理变化，VHLD患者怀孕会带来独特的麻醉挑战。本报告描述了一名31岁患有VHLD且怀有双胎的女性在妊娠35周活跃期分娩时的麻醉管理。尽管剖宫产采用神经轴索麻醉有诸多优点，但由于近期影像学检查不可用以及此类病例中神经轴索技术存在潜在风险，故选择了全身麻醉。该病例强调了个体化规划、血流动力学稳定以及多学科协作对于优化母婴结局的重要性。
