Khan Saad, Min Samuel, Willard Garrett, Lo Iris, D'Souza Rachael, Park Aaron
Triangle Implant Center, Mebane, NC, USA.
Duke Regional Hospital, Durham, NC, USA.
J Dent Anesth Pain Med. 2020 Feb;20(1):39-44. doi: 10.17245/jdapm.2020.20.1.39. Epub 2020 Feb 28.
Homozygous familial hypercholesterolemia (HoFH) is a rare inherited disorder that presents as abnormally elevated levels of low-density lipoprotein cholesterol and premature heart disease, requiring frequent intervention through lipid apheresis for management. The risk of perioperative cardiac events is higher in patients with HoFH because of its pathophysiological manifestations in the vascular system. Careful cardiac precautions and anesthetic assessments are necessary to ensure patient safety. In the following case report, we discuss the clinical course and anesthetic considerations for a 14-year-old girl with HoFH undergoing sedation for dental extractions and mandibular molar uprighting in an outpatient oral surgery clinic. Considerations included the use of heparin in the patient's weekly plasma lipid apheresis treatment. In order to reduce the risks of peri- and postoperative bleeding and perioperative cardiac events, the operation was scheduled for 4 days after apheresis. This allowed for adequate heparin clearance, while also reducing the likelihood of possible cardiac events. A literature review revealed no results for the outpatient management of patients with HoFH undergoing sedation for noncardiac procedures. Our reported case serves as a clinical example for physicians to be utilized in the future.
纯合子家族性高胆固醇血症（HoFH）是一种罕见的遗传性疾病，表现为低密度脂蛋白胆固醇水平异常升高和早发性心脏病，需要通过血脂分离术进行频繁干预来管理。由于HoFH在血管系统中的病理生理表现，其患者围手术期心脏事件的风险更高。为确保患者安全，仔细的心脏预防措施和麻醉评估是必要的。在以下病例报告中，我们讨论了一名14岁HoFH女孩在门诊口腔外科诊所接受拔牙和下颌磨牙扶正镇静治疗的临床过程和麻醉注意事项。注意事项包括患者每周血浆血脂分离治疗中肝素的使用。为了降低围手术期和术后出血以及围手术期心脏事件的风险，手术安排在血脂分离术后4天进行。这既能保证肝素充分清除，又能降低可能发生心脏事件的可能性。文献综述显示，没有关于HoFH患者在非心脏手术镇静治疗的门诊管理的研究结果。我们报告的病例可为未来医生提供临床参考。
