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ContentBackground:Fabry's disease (FD) is a rare systemic X-linked disease, even though its real incidence is unknown. It is related to a glycosphingolipid metabolism disorder in the alpha-galactosidase A enzyme, causing an accumulation of glycosphingolipids in the lysosomes. This results in anatomic abnormalities and organ dysfunction that can increase the risk of anesthesia complications. Clinical picture combines neurological (Fabry's pain crises), dermatologic, renal, cardiovascular (hypertrophic cardiomyopathy, valvular disease, conduction disorder, coronary heart disease) and cerebrovascular affectations.Epidural block is debated in patients with neurological diseases and recommendations are not unequivocal. Case Report:A 27 y.o. pregnant patient (38 weeks of gestation) with known FD asked for labour analgesia. Her clinical symptoms were fatigue, irritable bowel, fingers, hands and legs pain, hearing loss, dyslexia and migraine, with no treatment. Preoperative evaluation showed ASA II, BMI 34, proteinuria (667 mg/24 hs), important oedema in face and lower limbs, difficult airway and ventilation predictors (Mallampati III, interincisor distance
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