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A 26-year-old man with renal failure and vision loss
Digital Journal of Ophthalmology 2008
Volume 14, Number 13
July 12, 2008
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Tarek Alasil | University of Southern California
Mario Meallet | University of Southern California
Diagnosis and Discussion
The patient was diagnosed with bilateral central retinal vein occlusion (CRVO) with ischemia. The patient was noted to have a right relative afferent pupillary defect (RAPD), a high-risk characteristic of CRVO. The large retinal lesion seen in ischemic CRVO can explain this. Iridocorneal touch (a finding in the left eye) can be seen in trauma, after surgery, angle closure glaucoma, and inflammation. However, the history, review of systems, laboratory tests, fundus examination, fluorescein angiogram, and other exam findings were suggestive of CRVO rather than an inflammatory etiology.

Complete hematological and coagulation evaluations were performed (summarized in table 5). The homocysteine level was found to be elevated, and this is associated with a hypercoagulable state in nephrotic syndrome. The C-reactive protein was elevated as well. A rheumatology consult was not entertained given the clinical picture, pertinent negative review of systems, and the negative laboratory workup. The patient was followed closely by ophthalmology for six months, including gonioscopy and undilated examination of the iris. Serial exams showed no signs of neovascularization of the iris or neovascular glaucoma. The patient, however, was lost to follow up due to a prolonged admission to an outside hospital for a dialysis catheter infection. The patient was called and encouraged to follow up once his acute line infection issues resolve.

Central retinal vein occlusion typically occurs in people over 50 years of age. Major risk factors are hypertension, diabetes mellitus, and atherosclerosis. Other risk factors are glaucoma, syphilis, sarcoidosis, vasculitis, increased intraorbital or intraocular pressure, hyphema, hyperviscosity syndromes (multiple myeloma, Waldenstromʼs macroglobulinemia, and leukemia), high homocysteine levels, sickle cell, and HIV. Younger patients, especially those less than 45 years of age, who present with central retinal vein occlusion should have further hematologic and coagulation workup to rule-out an underlying thrombotic disorder.(1)

CRVO has two types:
• The Nonischemic type (incidence of 70%) is defined as <10 disk diameters (DD) of capillary nonperfusion. It is characterized by vision that is better than 20/200. Among this group, 16% progress to nonperfused CRVO and 50% resolve completely without treatment.
• The Ischemic type (incidence of 30%) is defined as more than 10 DD of nonperfusion. Patients are usually older and have worse vision. Iris neovascularization is seen in 60%. Up to 33% develop neovascular glaucoma. Ten percent are combined with branch retinal arterial occlusion (usually the cilioretinal artery is occluded due to low perfusion pressure of the choroidal system).(2)

The central vein occlusion study (CVOS) data did not support the recommendation for prophylactic panretinal photocoagulation (PRP). The CVOS found that early PRP decreased the rate of iris neovascularization (INV); however, the reduction was not statistically significant. Moreover, the study showed that early PRP reduced, but did not eliminate, the possibility of anterior-segment neovascularization. The CVOS recommended close follow-up of eyes with CRVO during the first 6 months (including gonioscopy and undilated slit-lamp examination of the iris) and prompt PRP of eyes in which iris neovascularization (INV) or angle neovascularization (ANV) develops.(3)

Risk factors for developing iris neovascularization in patients with CRVO are the amount of nonperfused retina, extent of retinal hemorrhages, male sex, and central vein occlusion of less than one month duration.(3) The baseline visual acuity of patients with CRVO is a strong predictor for the development of INV/ANV, as is the amount of nonperfusion seen by fluorescein angiogram.(4)

Although PRP was better than selective PRP or photodynamic therapy at preventing INV and anterior segment neovasculariation progression, selective PRP or PDT can also be safely used to treat anterior segment neovascularization secondary to ischemic CRVO.(5)

Every eye with CRVO is at risk for developing neovascular glaucoma. Lowering intraocular pressure helps to improve retinal circulation in an eye with CRVO(6), and there is a 10 % risk for development of BRVO or CRVO in the fellow eye.(7)

This case illustrates the potential importance of regular eye exams in young patients with end-stage renal disease on hemodialysis. Young patients with nephrotic syndrome and end-stage renal disease can develop CRVO.