Introduction:
Central retinal vein occlusion (CRVO) is a common cause of sudden unilateral loss of vision in elderly. It is usually a unilateral phenomenon presenting in more than 90% of cases as sudden loss of vision. Only in less than 10% of cases it presents as bilateral disease and simultaneous presentation is even more rare. Clinically it presents in two forms i.e., non ischemic and ischemic variety. The non ischemic type carries good prognosis while the ischemic type carries poor prognosis.[1]
Case Report:
A fifty years old male with a medical history of diabetes mellitus presented with complaints of sudden painless bilateral vision loss since 2 weeks. Ophthalmic examination revealed visual acuity of counting fingers at 2 meters. Intra ocular pressure and anterior segment examination findings in both eyes were normal. Fundus in both eyes showed clear media, optic discs were swollen with intense hyperemia, blurred margins, dilated and tortuosity of all the retinal vein branches. The entire retina was splashed with haemorrhages [Figure 1,2]. Optical Coherence tomography scan revealed bilateral extensive macular oedema [Figure 3,4]. A diagnosis of bilateral simultaneous central retinal vein occlusion was made. Reports of haematological investigations are shown in Table 1.
Table 1: Haematological investigations |
Fasting Glucose |
243mg/dl |
HbA1c |
11.6% |
White blood cell count |
5100cells/cumm |
M.C.V* |
73.0 |
HCT/PCV** |
18,8 [High] |
Hemoglobin |
5.4 |
Hematocrit |
45.1 |
Platelets |
4lakhs/mm3 |
Prothrombin time |
17.6 [High] |
INR*** |
1.21 |
APTT# |
33.6 [Normal] |
ANA, AntidsDNA , AntiCardiolipin Antibodies |
Negative |
Rheumatoid factor |
Negative |
ESR |
140mm/hr |
Lipid profile |
Normal |
Creatinine |
4mg/dl |
*MCV Mean Corpuscular Volume. ** HCT Haematocrit, PCV Packed cell volume. *** INR International Normalized Ratio. # APTT Activated Partial Thromboplastin time |
Severe anemia, raised ESR, osteolytic lesion of skull spine radiograph, M band in serum protein electrophoresis, [Figure 5], and bone marrow cytology features of markedly elevated plasma cells confirmed diagnosis of multiple myeloma [Figure 6]. Medical oncology reference was sought and plasmapheresis was advised.
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Fig 1: Fundus Photograph OD Showing Central retinal vein occlusion.
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Fig 2: Fundus Photograph OS Showing Central retinal vein occlusion |
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Fig 3: Optical coherence tomography OD showing macular edema. |
Fig 4: Optical coherence tomography OS showing macular edema. |
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| Fig 5: Serum protein electrophoresis showing M band |
Fig 6: Bone marrow cytology showing markedly elevated plasma cells |
Discussion
The pathogenesis of retinal vein occlusion is believed to follow the principles of Virchow's triad, thrombogenesis involving vessel wall damage, stasis and hypercoagulability, with abnormal blood viscosity playing a role in its causation.[2]
This patient was thoroughly investigated for systemic diseases and was diagnosed to have type 2 diabetes mellitus and multiple myeloma.
Diabetes is a hypercoagulable state, with vascular endothelium, the primary defence against thrombosis, being abnormal. Endothelial abnormalities undoubtedly play a role in the enhanced activation of platelets and clotting factors seen in diabetes.[3]
An increased risk of cancer, in particular pancreatic cancer, liver cancer, breast cancer, multiple myeloma, and cancer of the endometrium among patients with diabetes has been observed in several studies.[4,5–7] The proposed biological mechanism relates to insulin resistance and insulin-like growth factors, both of which have been shown to stimulate tumor cell proliferation in experiments.[8–11]
This patient had a double hyper viscosity insult with severe diabetes mellitus and multiple myeloma.
A severe hyperviscosity state predisposes to a bilateral affection as were all the cases suffering from myeloma reported by Carr et al.[12] Bilateral simultaneous retinal vein occlusion due to multiple myeloma is a rare entity. Ocular signs of multiple myeloma may appear at the initial presentation or develop later in the disease process. Bilateral simultaneous CRVO presenting with bilateral blindness was the first presentation of multiple myeloma in our patient.
Plasmapheresis is offered as the immediate therapy of symptomatic hyperviscosity. Long-term management is directed at control of the underlying disease to prevent production of the monoclonal protein.[13]
Conclusion:
Since abnormal blood viscosity plays a role in the causation of bilateral CRVO, systemic investigation for underlying hyperviscosity syndromes including blood dyscrasias such as multiple myeloma is warranted, illustrating the need for a primary care ophthalmologist to be involved in the basic assessment for associated underlying diseases in retinal disorders. Serum electrophoresis along with coagulation profiles should be performed as a standard practice in patients with bilateral simultaneous CRVO.
References
- Elman MJ, Bhatt AK, Quinlan PM, Enger C. The risk for systemic vascular diseases and mortality in patients with central retinal vein occlusion. Ophthalmology. 1990;97:1543–8.
- Trope GE, Lowe GDO, McArdle BM, Douglas JT, Forbes CD. Abnormal blood viscosity and heamostasis in long standing retinal vein occlusion. Br J Ophthalmol. 1983;67:137-42.
- Carr ME. Diabetes mellitus: a hypercoagulable state. J Diabetes Complications. 2001 Jan-Feb;15(1):44-54.
- Hjalgrim H, Frisch M, Ekbom A, Kyvik KO, Melbye M, Green A. Cancer and diabetes – a follow-up study of two population-based cohorts of diabetic patients. J Intern Med. 1997; 241:471–5.
- Chow WH, Gridley G, Nyren O, Linet MS, Ekbom A, Fraumeni JF Jr., et al. Risk of pancreatic cancer following diabetes mellitus: a nationwide cohort study in Sweden. J Natl Cancer Inst 1995;87:930–1.
- Wideroff L, Gridley G, Mellemkjaer L, Chow WH, Linet M, Keehn S, et al.Cancer incidence in a population-based cohort of patients hospitalized with diabetes mellitus in Denmark. J Natl Cancer Inst 1997;89:1360–5.
- Adami HO, McLaughlin J, Ekbom A, Berne C, Silverman D, Hacker D, et al. Cancer risk in patients with diabetes mellitus. Cancer Causes Control 1991;2:307–14.
- Rousseau MC, Parent ME, Pollak MN, Siemiatycki J. Diabetes mellitus and cancer risk in a population-based case-control study among men from Montreal, Canada. Int J Cancer 2006;118:2105–9.
- LeRoith D, Baserga R, Helman L, Roberts CT Jr. Insulin-like growth factors and cancer. Ann Intern Med. 1995;122:54–9.
- Giovannucci E. Insulin and colon cancer. Cancer Causes Control. 1995;6:164–79.
- Kaaks R, Lukanova A.Energy balance and cancer: the role of insulin and insulin-like growth factor-I. Proc Nutr Soc. 2001;60:91–106.
- Carr RE, Henkind P. Retinal findings with serum hyperviscosity. Am J Ophthalmol. 1963;56:23-31.
- Mehta J, Singhal S. Hyperviscosity syndrome in plasma cell dyscrasias. Semin Thromb Hemost. 2003 Oct;29(5):467-71.
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