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


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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    • "Hypoglycemia, the third anaesthetic problem, is considered to be the result of increased insulin release caused by high levels of methionine, which usually is seen in type Ι homocystinuria [1] [3] [10]. To decrease the incidence of hypoglycemia, blood glucose levels should be monitored frequently, exogenous glucose should be administered World J Med Surg Case Rep 2013; 2: 1‐4 Zahedi H et al. 4 "
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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.
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    ABSTRACT: Homocystinuria is an inherited metabolic disorder associated with cystathionine –beta-synthase deficiency. A 6 yr. old male child presented with the complaints of diminshed vision and was found to have ectopia lentis. He was suspected to have homocystinuria due to his clinical findings and this was confirmed by his serum homocysteine level. He was started on medication to reduce homocysteine levels and then was taken up for surgery i.e. pars plana vitrectomy with lensectomy with scleral fixation under general anaesthesia. Precautions were taken in the perioperative period to avoid the various complications and mortality associated with this syndrome. Nitrous oxide was avoided in the anaesthetic technique intraoperatively The patient was fine postoperatively.
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    ABSTRACT: Homocystinuria, a rare autosomal recessive genetic disease, is generally classified into 3 types (types I-III). Extremely rare type III is from a defect of the enzyme tetrahydrofolate reductase which converts homocysteine to methionine. The anesthetic problem of type III homocystinuria is the elevation of serum homocysteine and the deficiency of essential amino acid methionine, which result in the perioperative thromboembolic events and nitrous oxide-induced neurological impairment. We provided successful anesthetic management for a patient with type III homocystinuria, during which we were careful to take measures against perioperative thromboembolism and did not use nitrous oxide.
    Journal of Clinical Anesthesia 12/2005; 17(7):565-7. DOI:10.1016/j.jclinane.2005.01.005 · 1.19 Impact Factor