MANAGEMENT OF PATIENTS WITH HOMOCYSTINURIA REQUIRING SURGERY UNDER GENERAL ANAESTHESIA

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Abstract
An 8-year-old boy with homocystinuria required a prolonged dental operation under general anaesthesia. The successful management, without subsequent thromboembolism, is described. The patient showed a partial response to treatment with pyridoxine over a two-year period. The response was marked by a reduction in the plasma and urinary homocystine levels and by decreased platelet adhesiveness. It is suggested that a similar regime of pre-operative and operative management should be considered in all affected patients undergoing surgery, since there was no increase in platelet adhesiveness by the third postoperative day, as would normally have occurred.

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