Anaesthetic management of a patient with homocystinuria

Abstract

Homocystinuria is an autosomal recessive disease with multiple systemic involvement. Extracapsular extraction of lens is the most commonly performed elective ocular procedure for homocystinuric children. These children present with particular anaesthetic concerns during surgery. Here we report a case of a 14 year old boy with homocystinuria posted for extracapsular lens extraction under general anaesthesia. Precautions were taken perioperatively to prevent the various complications associated with this condition for a favourable perioperative outcome.

Key words: homocystinuria; thromboembolism; hypoglycemia; general anaesthesia; homocysteine metabolism.

DOI: 10.4038/slja.v18i2.1463

Sri Lankan Journal of Anaesthesiology 18(2): 84-86 (2010)