J Korean Ophthalmol Soc.  2016 Jun;57(6):1012-1017. 10.3341/jkos.2016.57.6.1012.

Bilateral Simultaneous Central Retinal Vein Occlusion in a Patient with Waldenstrom's Macroglobulinemia

• Lee SE
• Shin YU
• Lim HW
• Seong MC
• Cho HY
• Kang MH

• KMID: 2290508
• DOI: http://doi.org/10.3341/jkos.2016.57.6.1012

Abstract

PURPOSE
The authors report a case of bilateral simultaneous central retinal vein occlusion caused by Waldenstrom's macroglobulinemia.
CASE SUMMARY
A 65-year-old man presented to our department complaining of decreased visual acuity for the duration of about 6 months. On his initial visit, best-corrected visual acuity was 0.02 in the right eye and 0.06 in the left eye. Based on the findings of a funduscopic examination, the patient had bilateral diffuse retinal hemorrhages, dilated tortuous veins, and macular edema. He had experienced recurrent spontaneous epistaxis 6 months previously and had undergone treatments such as intravitreal bevacizumab injection and intravitreal dexamethasone implantation at another hospital. Laboratory tests at that hospital showed anemia and hyperproteinemia, for which he was referred to our hemato-oncology department. Bone marrow biopsy was consistent with Waldenstrom's macroglobulinemia/lymphoplasmacytoid lymphoma, and he was treated with systemic chemotherapy. One year after the systemic chemotherapy, his best-corrected visual acuity was 0.15 in the right eye and 0.6 in the left eye. Funduscopy showed decreased bilateral retinal hemorrhages and macular edema.
CONCLUSIONS
When simultaneous bilateral central retinal vein occlusion occurs in a patient with no other underlying disease such as hypertension or diabetes, it might be a sign of serum hyperviscosity, and there should be a very high level of suspicion for presence or progression of systemic disease. If such a disease is properly and timely diagnosed, effective early systemic evaluation and therapy can be administered, and it is important to have initial general treatment as well as ophthalmic treatment.

Keyword

Central retinal vein occlusion; Waldenstrom's macroglobulinemia

MeSH Terms

Aged
Anemia
Bevacizumab
Biopsy
Bone Marrow
Dexamethasone
Drug Therapy
Epistaxis
Humans
Hypertension
Lymphoma
Macular Edema
Retinal Hemorrhage
Retinal Vein*
Veins
Visual Acuity
Waldenstrom Macroglobulinemia*
Bevacizumab
Dexamethasone

Figure

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  Figure 1. Fundus photographs when his first visit. Fundus photographs show dilated tortuous veins, diffuse retinal hemorrhages in all quadrants and vascular sheathing in the right eye (A) and left eye (B).

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  Figure 2. Optical coherence tomography (OCT) finding when his first visit. OCT shows macular edema in the both eyes and more severe subretinal fluid in the right eye (A) than in the left eye (B). OD = oculus dexter; OS = oculus sinister; R = right; L = left; ILM = internal limiting membrane; RPE = retinal pigment epithelium; ETDRS = Early Treatment Diabetic Retinopathy Study; S = superior; N = nasal; I = inferior; T = temporal.

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  Figure 3. Fundus photographs after the systemic chemotherapy. Fundus photographs show decreased dilated tortuous veins and retinal hemorrhages in the both eyes and decreased vascular sheathing in the right eye. There are few changes between 3 months (A, B) and 1 year (C, D).

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  Figure 4. Optical coherence tomography (OCT) finding after the systemic chemotherapy. OCT shows decreased subretinal fluid and macular edema in the right eye (A) and the left eye (B). But, subretinal fluid and macular edema still remains. Subretinal fibrin accumulation or choroid neovascularization are not detected due to serous retinal detachment. OCT shows atrophy of the retinal pigment epithelium on the macula and decrease in outer neuroretina layer thickness. OD = oculus dexter; OS = oculus sinister; R = right; L = left; T = temporal; N = nasal; S = superior.

Reference

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