Fabry Disease and General Anesthesia: A Case Report and Literature Review

Tsai-Shan Wu ； Wei-Cheng Tseng ； Hou-Chuan Lai ； Yi-Hsuan Huang ； Jyh-Cherng Yu ； Zhi-Fu Wu

《Journal of Medical Sciences》 39卷6期 (2019/12) Pp. 289-292

https://doi.org/10.4103/jmedsci.jmedsci_26_19

摘要

Fabry disease (FD) is an X-linked lysosomal storage disease. FD presents with a variety of symptoms such as corneal clouding, reduced sweating, abdominal pain, neuropathic pain, angiokeratoma, sleep-disordered breathing, renal failure, cardiovascular diseases, and stroke. Severe organ damage may occur, and its gravity differs between individuals, usually being more serious in males and elders. According to previous reports, possible complications include hypertension, hypotension, bronchospasm, and intubation difficulty. Therefore, identifying FD patients carefully and performing detailed preoperative assessments, such as for cardiovascular, pulmonary, and renal functions, are both critical to increasing the chances of a positive treatment outcome. Advanced hemodynamic monitoring was recommended to prevent severe cardiovascular and respiratory impairment during surgery of advanced FD patients. To the best of our knowledge, there are only four reports on FD treatment, none being from Taiwan. Here, we report on a case of FD treatment undergoing general anesthesia in Taiwan and provide a literature review.

關鍵字

Fabry disease ； general anesthesia ； anesthetic events