Spinal anesthesia in a patient with Fabry disease and multiorgan involvement: a rare case with long-term success.

backgroundFabry disease is a rare X-linked lysosomal storage disorder characterized by the progressive accumulation of globotriaosylceramide in various tissues. Disease progression can lead to complications involving multiple organ systems, most notably the heart, kidneys, nervous system, and eyes. General anesthesia can pose substantial risks in patients with Fabry disease due to cardiac hypertrophy, diastolic dysfunction, autonomic instability, and altered drug metabolism. CASE: We report the case of a 57-year-old woman who required bilateral hallux valgus correction. The patient was receiving enzyme replacement therapy for Fabry disease and exhibited significant multiorgan involvement, as indicated by the presence of proteinuria (albumin-to-creatinine ratio, 1011.2 mg/g), concentric left ventricular hypertrophy (interventricular septal thickness, 14.3 mm), reduced global longitudinal peak strain (-11.2%), polyneuropathic symptoms, and ocular features. OUTCOME: To minimize anesthetic risks, spinal anesthesia was selected and administered without sedation. The procedure was uneventful, with the patient exhibiting stable intraoperative vital signs and making a full neurological recovery within 2 h. The patient experienced no anesthetic or surgical complications over the 2-year follow-up period.

conclusionThis case highlights spinal anesthesia as a potential anesthetic strategy for patients with Fabry disease and advanced systemic involvement. It emphasizes the importance of individualized perioperative planning and contributes valuable clinical evidence to the limited literature on spinal anesthesia in this high-risk population.