BILATERAL NON-ISCHEAMIC CENTRAL RETINAL VEIN OCCLUSION IN A PATIENT WITH SOUTHEAST ASIAN OVALOCYTOSIS - A RARE CASE
Abdul Rahman A.H., Ho N.W.W., Norshamsiah M.D. & Othmaliza O.
abstract
Simultaneous bilateral central retinal vein occlusion (CRVO) is a rare condition often linked to underlying systemic causes such as hyperviscosity syndromes. We report a case of a 68-year-old diabetic male patient who presented with bilateral painless blurring of vision for two months. Fundus examination revealed non-ischemic bilateral CRVO with macular edema. Hematological investigation revealed the presence of stomato-ovalocytes, consistent with hereditary Southeast Asian Ovalocytosis (SAO). The patient was treated with serial intravitreal anti-VEGF injections. This case highlights the importance of recognizing rare hematologic conditions such as SAO in patients with bilateral CRVO, especially in populations from malaria-endemic regions.
references
1. Ehlers, Justis P, and Sharon Fekrat. “Retinal vein occlusion: beyond the acute event.” Survey of ophthalmology vol. 56,4 (2011): 281-99. doi:10.1016/j.survophthal.2010.11.006.
2. Rogers S, McIntosh RL, Cheung N, Lim L, Wang JJ, Mitchell P, Kowalski JW, Nguyen H, Wong TY; International Eye Disease Consortium. The prevalence of retinal vein occlusion: pooled data from population studies from the United States, Europe, Asia, and Australia. Ophthalmology. 2010 Feb;117(2):313-9.e1. doi: 10.1016/j.ophtha.2009.07.017. PMID: 20022117; PMCID: PMC2945292.
3. Suñer, Ivan J et al. “Direct medical costs and resource use for treating central and branch retinal vein occlusion in commercially insured working-age and Medicare populations.” Retina (Philadelphia, Pa.) vol. 34,11 (2014): 2250-8. doi:10.1097/IAE.0000000000000217.
4. Hayreh, S S et al. “Systemic diseases associated with various types of retinal vein occlusion.” American journal of ophthalmology vol. 131,1 (2001): 61-77. doi:10.1016/s0002-9394(00)00709-1.
5. Vichai Laosombat, Vip Viprakasit, Supaporn Dissaneevate, Roengsak Leetanaporn, Thirachit Chotsampancharoen, Malai Wongchanchailert, Sudarat Kodchawan, Warangkana Thongnoppakun, Sarapee Duangchu,Natural history of Southeast Asian Ovalocytosis during the first 3years of life,Blood Cells, Molecules, and Diseases,Volume 45, Issue 1,2010,Pages 29-32,ISSN 1079-9796,https://doi.org/10.1016/j.bcmd.2010.03.010.
6. Paquette AM, Harahap A, Laosombat V, Patnode JM, Satyagraha A, Sudoyo H, Thompson MK, Yusoff NM, Wilder JA. The evolutionary origins of Southeast Asian Ovalocytosis. Infect Genet Evol. 2015 Aug;34:153-9. doi: 10.1016/j.meegid.2015.06.002. Epub 2015 Jun 3. PMID: 26047685.
7. Ziaei, Mohammed et al. “Bilateral central retinal vein occlusion in multiple myeloma.” Canadian journal of ophthalmology. Journal canadien d'ophtalmologie vol. 48,2 (2013): e24-5. doi:10.1016/j.jcjo.2012.11.002 .
8. Kotepui KU, Mahittikorn A, Masangkay FR, Kotepui M. Association between ovalocytosis and Plasmodium infection: a systematic review and meta-analysis. Sci Rep. 2023 May 3;13(1):7164. doi: 10.1038/s41598-023-34170-3. PMID: 37137935; PMCID: PMC10156661.
9. Favale, F., Gardembas, M., Pajot, O., Saada, V., Fénéant-Thibault, M., Delaunay, J., & Garçon, L. (2009). Southeast Asian Ovalocytosis and A Sickle Cell Trait in a Young Patient with Sudden Retinal Stroke: A Fortuitous Association? Hemoglobin, 33(6), 475–479. https://doi.org/10.3109/03630260903344440.
10. Van De Loosdrecht, A.A., Franck, P.F.H., Renardel de Lavalette, V.W., Smit, J.W. and Daenen, S.M.G.J. (2000), Preretinal neovascularization in south-east asian ovalocytosis. British Journal of Haematology, 110: 1006-1006. https://doi.org/10.1046/j.1365-2141.2000.02270-5.x.
11. Liu SC, Zhai S, Palek J, Golan DE, Amato D, Hassan K, Nurse GT, Babona D, Coetzer T, Jarolim P, et al. Molecular defect of the band 3 protein in southeast Asian ovalocytosis. N Engl J Med. 1990 Nov 29;323(22):1530-8. doi: 10.1056/NEJM199011293232205. PMID: 2146504.
2. Rogers S, McIntosh RL, Cheung N, Lim L, Wang JJ, Mitchell P, Kowalski JW, Nguyen H, Wong TY; International Eye Disease Consortium. The prevalence of retinal vein occlusion: pooled data from population studies from the United States, Europe, Asia, and Australia. Ophthalmology. 2010 Feb;117(2):313-9.e1. doi: 10.1016/j.ophtha.2009.07.017. PMID: 20022117; PMCID: PMC2945292.
3. Suñer, Ivan J et al. “Direct medical costs and resource use for treating central and branch retinal vein occlusion in commercially insured working-age and Medicare populations.” Retina (Philadelphia, Pa.) vol. 34,11 (2014): 2250-8. doi:10.1097/IAE.0000000000000217.
4. Hayreh, S S et al. “Systemic diseases associated with various types of retinal vein occlusion.” American journal of ophthalmology vol. 131,1 (2001): 61-77. doi:10.1016/s0002-9394(00)00709-1.
5. Vichai Laosombat, Vip Viprakasit, Supaporn Dissaneevate, Roengsak Leetanaporn, Thirachit Chotsampancharoen, Malai Wongchanchailert, Sudarat Kodchawan, Warangkana Thongnoppakun, Sarapee Duangchu,Natural history of Southeast Asian Ovalocytosis during the first 3years of life,Blood Cells, Molecules, and Diseases,Volume 45, Issue 1,2010,Pages 29-32,ISSN 1079-9796,https://doi.org/10.1016/j.bcmd.2010.03.010.
6. Paquette AM, Harahap A, Laosombat V, Patnode JM, Satyagraha A, Sudoyo H, Thompson MK, Yusoff NM, Wilder JA. The evolutionary origins of Southeast Asian Ovalocytosis. Infect Genet Evol. 2015 Aug;34:153-9. doi: 10.1016/j.meegid.2015.06.002. Epub 2015 Jun 3. PMID: 26047685.
7. Ziaei, Mohammed et al. “Bilateral central retinal vein occlusion in multiple myeloma.” Canadian journal of ophthalmology. Journal canadien d'ophtalmologie vol. 48,2 (2013): e24-5. doi:10.1016/j.jcjo.2012.11.002 .
8. Kotepui KU, Mahittikorn A, Masangkay FR, Kotepui M. Association between ovalocytosis and Plasmodium infection: a systematic review and meta-analysis. Sci Rep. 2023 May 3;13(1):7164. doi: 10.1038/s41598-023-34170-3. PMID: 37137935; PMCID: PMC10156661.
9. Favale, F., Gardembas, M., Pajot, O., Saada, V., Fénéant-Thibault, M., Delaunay, J., & Garçon, L. (2009). Southeast Asian Ovalocytosis and A Sickle Cell Trait in a Young Patient with Sudden Retinal Stroke: A Fortuitous Association? Hemoglobin, 33(6), 475–479. https://doi.org/10.3109/03630260903344440.
10. Van De Loosdrecht, A.A., Franck, P.F.H., Renardel de Lavalette, V.W., Smit, J.W. and Daenen, S.M.G.J. (2000), Preretinal neovascularization in south-east asian ovalocytosis. British Journal of Haematology, 110: 1006-1006. https://doi.org/10.1046/j.1365-2141.2000.02270-5.x.
11. Liu SC, Zhai S, Palek J, Golan DE, Amato D, Hassan K, Nurse GT, Babona D, Coetzer T, Jarolim P, et al. Molecular defect of the band 3 protein in southeast Asian ovalocytosis. N Engl J Med. 1990 Nov 29;323(22):1530-8. doi: 10.1056/NEJM199011293232205. PMID: 2146504.