Anaesthetic management of a patient with homocystinuria

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)

[1]  M. Tsou,et al.  General anesthesia for patient with homocystinuria--a case report. , 2002, Acta anaesthesiologica Sinica.

[2]  M. Cattaneo,et al.  A pilot study to investigate the effects of an infusion of aminophylline on renal function following major abdominal surgery , 2001, Anaesthesia.

[3]  N. Badner,et al.  Nitrous Oxide-Induced Increased Homocysteine Concentrations Are Associated with Increased Postoperative Myocardial Ischemia in Patients Undergoing Carotid Endarterectomy , 2000, Anesthesia and analgesia.

[4]  P. Mannucci,et al.  High prevalence of hyperhomocyst(e)inemia in patients with juvenile venous thrombosis. , 1994, Arteriosclerosis and thrombosis : a journal of vascular biology.

[5]  J. Tobias,et al.  Anesthetic implications of the child with homocystinuria. , 1994, Journal of clinical anesthesia.

[6]  G. Gascon,et al.  Neurometabolic Diseases at a National Referral Center: Five Years Experience at the King Faisal Specialist Hospital and Research Centre , 1992, Journal of child neurology.

[7]  K. Pettigrew,et al.  The natural history of homocystinuria due to cystathionine beta-synthase deficiency. , 1985, American journal of human genetics.

[8]  R. Ross,et al.  Homocystine-induced arteriosclerosis. The role of endothelial cell injury and platelet response in its genesis. , 1976, The Journal of clinical investigation.

[9]  J. Towers,et al.  Management of patients with homocystinuria requiring surgery under general anaesthesia. A case report. , 1971, British journal of anaesthesia.