Abstract
Purpose
To report a case of central retinal artery obstruction (CRAO) caused by protein S deficiency.
Case summary
A 36-year-old man presented with acutely reduced visual acuity in the right eye. The patient had no other diseases such as diabetes mellitus or hypertension. The visual acuity was 20/20 in the right eye and light perception in the left eye. Afferent pupillary defect was observed in theleft eye. Fundus examination showed diffuse retinal opacification and a cherry-red spot at the macula. Thus the patient was diagnosed with CRAO in the left eye. There were no abnormal findings on routine hematologic tests including blood coagulation test. Protein C activity, antithrombin III activation, anticardiolipin antibody were within normal limits but protein S activity was decreased. Thus, the patient was diagnosed with CRAO with protein S deficiency.
References
1. Heistinger M, Rumpl E, Illiasch H. . Cerebral sinus thrombosis in a patient with hereditary protein S deficiency: case report and review of the literature. Ann Hematol. 1992; 64:105–9.
2. Draganov P, Lazarchick J, Portwood G. Mesenteric vein thrombosis secondary to protein S deficiency. Am J Gastroenterol. 1999; 94:3616–9.
3. Fields MC, Levine SR. Thrombophilias and stroke: diagnosis, treatment, and prognosis. J Thromb Thrombolysis. 2005; 20:113–26.
4. El-Hazmi MA. Hematological risk factors for coronary heart disease. Med Princ Pract. 2002; 11:56–62.
5. Loh BK, Lee SY, Goh KY. Protein S deficiency manifesting simultaneously as central retinal artery occlusion, oculomotor nerve palsy, and systemic arterial occlusive diseases. Eye. 2007; 21:684–6.
6. Brown GC, Magargal LE, Sheild JA. . Retinal arterial obstruction in children and young adults. Ophthalmology. 1981; 88:18–25.
7. Brown GC, Magargal LE. Central retinal artery obstruction and visual acuitiy. Ophthalmology. 1982; 89:14–9.
8. Sharma S, Brown GC, Pater JL, Cruess AF. Does a visible retinal emboli increase the likelihood of hemodynamically significant carotid artery stenosis in patients with acute retinal artery occlusion? Arch Ophthalmol. 1998; 116:1602–6.
9. Dykes AC, Walker ID, McMahon AD. . A study of Protein S antigen levels in 3788 healthy volunteers: influence of age, sex and hormone use, and estimate for prevalence of deficiency state. Br J Haematol. 2001; 113:636–41.
10. Simioni P, Sanson BJ, Prandoni P. . Incidence of venous thromboembolism in families with inherited thrombophilia. Thromb Haemost. 1999; 81:198–202.
11. Sakata T, Okamoto A, Mannami T. . Prevalence of protein S deficiency in the Japanese general population: The suita study. J Thromb Haemost. 2004; 2:1012–3.
12. Cumming AM, Shiach CR. The investigation and management of inherited thrombophilia. Clin Lab Haematol. 1999; 21:77–92.
13. Song KS, Won DI, Lee AN. . A case of nephrotic syndrome associated with protein S deficiency and cerebral thrombosis. J Korean Med Sci. 1994; 9:347–50.
14. D'Angelo A, Vigano-D'Angelo S, Esmon CT, Comp PC. Acquired deficiencies of protein S.Protein S activity during oral anticoagulation, in liver disease, and in disseminated intravascular coagulation. J Clin Invest. 1988; 81:1445–54.