General anesthesia for patient with homocystinuria--a case report.

Department of Anesthesiology, Taipei Veterans General Hospital and National Yung-Ming University School of Medicine, Taiwan, R.O.C.
Acta anaesthesiologica Sinica 10/2002; 40(3):153-6.
Source: PubMed

ABSTRACT Homocystinuria is an autosomal recessive disease with multiple systemic disorders. Here we report a 15-year-old lad suffering from homocystinuria who required an ocular surgery including lentectomy and implant of plastic lens, OS and anterior retinal cryotherapy, OD under general anesthesia because of lens subluxation and lattice degeneration. It is the elective ocular procedure most commonly performed for homocystinuric children. Proper precautions should be taken during anesthetic management since this condition inspires some particular anesthetic complications that could be prevented by careful consideration and understanding of its pathophysiology. Providentially our patient stood the operation well and was discharged without subsequent thromboembolism or other complication as an aftermath.

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    ABSTRACT: Homocystinuria is a rare autosomal recessive disease with multiple systemic manifestations, which is classified into three types (type's Ι-III). Here we report an 8-year-old boy who required anesthetic care during an ocular surgery including lensectomy under general anesthesia. Proper precautions should be taken during anesthetic management of such a patient since some particular Anesthetic complications like preoperative thromboembolism and hypoglycemia may worsen the course of Anesthesia. We provided successful anesthetic management for our patient and he was discharged without subsequent complications.