Case Study

Combined CRVO Accompanied by Cilioretinal Artery Occlusion

A first case report of combined occlusion in a patient with ALL.

Stephen De Souza, MD • Shil Patel, MD

A 29-year-old man with a history of acute lymphocytic leukemia presented with decreased central vision in the right eye one week in duration. He denied any ocular trauma or previous ocular history.

His best-corrected visual acuity was 20/400 in the right eye and 20/25 in the left eye. On funduscopy, the right eye showed dilated tortuous veins with intraretinal hemorrhages in all four quadrants, a swollen optic nerve, and retinal whitening and edema in the distribution of the cilioretinal artery (Figures 1-4). The left eye posterior segment examination was normal (Figure 5). His blood parameters were as follows: WBC 0.2; RBC 2.36; Hgb 7.3; plate lets 22; absolute neutrophil count 0.13.

Figure 1. Fundus photograph of the right eye showing central retinal vein occlusion and retinal whitening in the distribution of the cilioretinal artery.

Figure 2. Fluorescein angiogram of the right eye. Blockage by intraretinal hemorrhage in the peripapillary region and hy perfluoresence in the distribution of the cilioretinal artery, indicating retinal edema.

Figure 3. Fluorescein angiogram of the right eye. Late leakage in distribution of the cilioretinal artery with venous wall staining.

Figure 4. Fundus photograph montage of the right eye.

Figure 5. Fundus photograph of the left eye.

DISCUSSION

This is the first case report, to our knowledge, of a combined central RVO and a cilioretinal artery occlusion in a patient with leukemia. Ocular manifestations of leukemia include superficial flame-shaped hemorrhages, preretinal hemorrhages, vitreous hemorrhages, microaneurysms, Roth spots, cotton wool spots, perivascular sheathing and disc edema. Rarely, leukemic infiltration of the optic nerve may be seen.

Leukemic retinopathy occurs primarily due to the associated anemia, thrombocytopenia and hyperviscosity associated with the underlying leukemia. Hayreh et al. published a case series of 38 eyes in 38 patients who had a combined CRVO and a cilioretinal artery occlusion. His study population consisted of 30 eyes that had nonischemic CRVO, five eyes with ischemic CRVO and three eyes with nonischemic hemi-CRVO.¹

Brown et al. reported that cilioretinal artery obstruction may occur in isolation or in conjunction with ischemic op tic neuropathy or central RVO.² The majority of cases occur in young adults, and it is very uncommon in the elderly.

The pathogenesis of this clinical entity has not yet been established. It has been suggested that the infarction is caused by an increase in venous pressure, increase in central retinal vein resistance or a decrease in perfusion.^3,4 This clinical entity may occur more frequently than it appears because diffuse retinal hemorrhages in a CRVO can obscure cilioretinal artery obstruction.⁵ RP

REFERENCES

1. Hayreh SS, Fraterrigo L, Jonas J. Central retinal vein occlusion associated with cilioretinal artery occlusion. Retina. 2008;28:581-594.
2. Brown GC, Moffat K, Cruess A, Magargal LE, Goldberg RE. Cilioretinal artery obstruction. Retina. 1983;3:182-187.
3. McLeod D. Cilio-retinal arterial circulation in central retinal vein occlusion. Br J Ophthalmol. 1975;59:486-492.
4. Noble KG. Central retinal vein occlusion and cilioretinal artery infarction. Am J Ophthalmol. 1994;118:811-813.
5. Schatz H, Fong AC, McDonald HR, et al. Cilioretinal artery occlusion in young adults with central retinal vein occlusion. Ophthalmology. 1991;98:594-601.