Protein S Deficiency Related Retinal Artery Occlusion in a Pregnant Chinese Woman

Syed Shoeb Ahmad^1, , Shuaibah Abdul Ghani¹ and Lindfay Laura Lau¹

¹Ophthalmology Department, Queen Elizabeth Hospital, Kota Kinabalu, Malaysia

Cite this paper

Syed Shoeb Ahmad, Shuaibah Abdul Ghani and Lindfay Laura Lau. Protein S Deficiency Related Retinal Artery Occlusion in a Pregnant Chinese Woman. American Journal of Medical Case Reports. 2014; 2(9):175-180. doi: 10.12691/AJMCR-2-9-4

Abstract

Purpose: To report a case of branch retinal artery occlusion (BRAO) in a pregnant patient of Chinese descent found to have Protein S deficiency. Case report: A 35 year-old apparently healthy, pregnant female of Chinese origin reported in the eye clinic of our hospital with a complaint of sudden appearance of a “shadow” in the periphery of her left eye for the last three days. On examination, she was found to have a BRAO in that eye. A number of laboratory, radiological and systemic examinations were done to find out the cause of the occlusion. However, a deficiency of Protein S was the only abnormality found. So far there have been only a few cases reported concerning this hematological deficiency causing a BRAO. This case report describes the events leading to the diagnosis of this rare condition. Conclusion: In cases of BRAO, especially in young patients it is important to rule out Protein S deficiency which may have life-threatening consequences.

Keywords

Protein S deficiency, retinal artery occlusion, pregnancy

Copyright

This work is licensed under a Creative Commons Attribution 4.0 International License. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/

References

[1]	Ratra D, Dhupper M. Retinal arterial occlusions in the young: Systemic associations in Indian population. Indian J Ophthalmol. 2012; 60: 95-100.
[2]	Beatty S, Eong K. Acute occlusion of the retinal arteries: current concepts and recent advances in diagnosis and management. J Accid Emerg Med. 2000; 17: 324-329.
[3]	Lee WB. Pearson PA. Moreman K. Central retinal artery occlusion and disc edema in a child. J AAPOS. 2002; 6: 264-5.
[4]	Ambati J, Hanuch O, Bresnick G. Protein C and protein S deficiency associated with retinal, optic nerve, and cerebral ischaemia. Br J Ophthalmol. 1999; 83: 753.
[5]	Hayreh SS, Podhajsky PA, Zimmerman MB. Retinal Artery Occlusion: Associated Systemic and Ophthalmic Abnormalities. Ophthalmology. 2009; 116: 1928-36.
[6]	Wong DM, Ilsen PF, Bright DC, Anderson SF, Townsend JC. Case presentations of retinal artery occlusions. Optometry. 2000; 71: 703-14.
[7]	Chung YR, Kim JB, Lee K, Lew HM. Retinal artery occlusion in a healthy pregnant patient. Korean J Ophthalmol. 2008; 22: 70-1.
[8]	Greven CM, Slusher MM, Weaver RG. Retinal arterial occlusions in young adults. Am J Ophthalmol. 1995; 120: 776-83.
[9]	Brown GC, Magargal LE, Shields JA, Goldberg RE, Walsh PN. Retinal arterial obstruction in children and young adults. Ophthalmology. 1981; 88: 18-25.
[10]	Kadayifcilar S, Ozatli D, Ozcebe O, Sener E. Is activated factor VII associated with retinal vein occlusion? Br J Ophthalmol. 2001; 85: 1174-78.
[11]	Chang YS, Jan RL, Weng SF, Wang JJ, Chio CC, Wei FT, Chu CC. Retinal artery occlusion and the 3-year risk of stroke in Taiwan: a nationwide population-based study. Am J Ophthalmol. 2012; 154: 645-652.
[12]	Rishi P, Rishi E, Sharma T, Mahajan S. Hemi-central retinal artery occlusion in young adults. Indian J Ophthalmol. 2010; 58: 425-432.
[13]	Schmidt D, Hetzel A, Geibel-Zehender A, Schulte-Mönting J. Systemic diseases in non-inflammatory branch and central retinal artery occlusion--an overview of 416 patients. Eur J Med Res. 2007; 12: 595-603.
[14]	Berg W vd, Verbraak FD, Bos PJ. Homocystinuria presenting as central retinal artery occlusion and longstanding thromboembolic disease. Br J Ophthalmol. 1990; 74: 696-697.
[15]	Prince HM, Thurlow PJ, Buchanan RC, Ibrahim KM, Neeson PJ. Acquired protein S deficiency in a patient with systemic lupus erythematosus causing central retinal vein thrombosis. J Clin Pathol. 1995; 48: 387-389.
[16]	Tekeli O, Gürsel E, Buyurgan H. Protein C, protein S and antithrombin III deficiencies in retinal vein occlusion. Acta Ophthalmol Scand. 1999; 77: 628-30.
[17]	Bertram B, Remky A, Arend O, Wolf S, Reim M. Protein C, protein S, and antithrombin III in acute ocular occlusive diseases. Ger J Ophthalmol. 1995; 4: 332-5.
[18]	High KA. Antithrombin III, protein C, and protein S. Naturally occurring anticoagulant proteins. Arch Pathol Lab Med. 1988; 112: 28-36.
[19]	Greven CM, Weaver RG, Owen J, Slusher MM. Protein S deficiency and bilateral branch retinal artery occlusion. Ophthalmology 1991; 98: 33-4.
[20]	Schmidt D. Comorbidities in combined retinal artery and vein occlusions. Eur J Med Res. 2013; 18: 27.
[21]	Deokule S. 29 year old woman with loss of visual field in her right eye. Digital Journal Ophthalmol 1999, Vol 5, No: 8.
[22]	Vela JI et al. Protein S deficiency and retinal arteriolar occlusion in pregnancy. Eur J Ophthalmol 2007; 17: 1004-6.
[23]	Lee SB, Yun YJ, Kim JY. Central Retinal Artery Obstruction in Protein S Deficiency. J Korean Ophthalmol Soc. 2008; 49: 2017-2020.
[24]	Golub BM, Sibony PA, Coller BS. Protein S deficiency associated with central retinal artery occlusion. Arch Ophthalmol 1990; 108: 918-19.
[25]	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.