RESUMO
Hyperhomocysteinemia is a disorder caused by a disruption of any of the enzymes or cofactors involved in the pathways of homocysteine metabolism. The resultant high plasma levels of homocysteine increase the risk for thromboembolic events. These patients are frequently anticoagulated in the perioperative setup. Interruption of anticoagulant therapy may subject the patient to an increased risk of thrombosis, infarction, and death. Neuraxial anaesthesia techniques may be relatively contraindicated in anticoagulated patients and nitrous oxide may exacerbate the condition by inhibiting the conversion of homocysteine to methionine. We describe a case of the anaesthetic management of a unique case of hyperhomocysteinemia with multiple recent thrombotic episodes proposed for total abdominal hysterectomy conducted under general anaesthesia, the intraoperative and postoperative monitoring and considerations.