Francisco Machado, Henrique Gouveia, Sara Freitas, Filipa Rodrigues
{"title":"Anesthetic challenges in a pregnant patient with Von Hippel-Lindau disease: A case report.","authors":"Francisco Machado, Henrique Gouveia, Sara Freitas, Filipa Rodrigues","doi":"10.4103/sja.sja_839_24","DOIUrl":null,"url":null,"abstract":"<p><p>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.</p>","PeriodicalId":21533,"journal":{"name":"Saudi Journal of Anaesthesia","volume":"19 3","pages":"443-445"},"PeriodicalIF":1.4000,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12240510/pdf/","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Saudi Journal of Anaesthesia","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.4103/sja.sja_839_24","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2025/6/16 0:00:00","PubModel":"Epub","JCR":"Q3","JCRName":"ANESTHESIOLOGY","Score":null,"Total":0}
引用次数: 0
Abstract
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.
Von Hippel-Lindau病(VHLD)是一种罕见的常染色体显性遗传病,其特征是高度血管化肿瘤的发展,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,妊娠期VHLD患者面临着独特的麻醉挑战。本报告描述了一名31岁女性VHLD和双胎妊娠在妊娠35周活产期间的麻醉管理。尽管神经轴向麻醉在剖宫产中具有优势,但由于近期影像学的缺乏以及在剖宫产中使用神经轴向技术的潜在风险,我们选择了全麻。该病例强调了个体化规划、血流动力学稳定性和多学科合作的重要性,以优化母亲和新生儿的预后。