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Case of the Month

Case of the Month Listing

Edited by

Robert N. Johnson, MD

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November, 2009

65-year-old male with blurred vision for four weeks.

Figure 1: Color fundus photo of the left eye. Note the mildly dilated and tortuous veins.  Some flame-shaped hemorrhages are present inferiotemporally to the nerve, and scattered intraretinal hemorrhages are seen

Case History

A 65-year-old man presented with a four-week history of poor vision in his left eye.  His past ocular history was noteworthy for poor vision in the right eye due to complications of an ischemic central retinal vein occlusion five years ago.  His has a history of type II diabetes and hypertension.

His best-corrected visual acuity was 2/200 in the right eye and 20/32 in the left eye.  His intraocular pressures were normal and neither eye had rubeosis.  The fundus examination of the right eye revealed evidence of a previous central retinal vein occlusion (CRVO) and panretinal photocoagulation.  In the left eye, the retinal veins were dilated and tortuous.  There was cystic edema in the macula and scattered dot-blot intraretinal hemorrhages in all four quadrants (Figure 1).  Fluorescein angiography of the left eye showed delayed filling of the retinal veins, blockage from intraretinal hemorrhages, and late leakage in the macula (Figure 2).  Optical coherence tomography (OCT) of the left eye demonstrated cystic macular edema with a central retinal thickness (CRT) of 435μm (Figure 3).

Figures 2: Fluorescein angiogram of the left eye. Note slow filling of the retinal veins.  In a healthy eye, the retinal veins should completely fill within approximately 10-12 seconds of the time dye appears in the central retinal artery.  Also, in this case, dye does not enter the eye until 45 seconds.  This is prolonged as well, and could reflect problems with the timer, the injection site, or carotid artery disease.

Figure 3: The optical coherence Tomography study shows central retinal thickening due to intraretinal cystic edema, which is shown on the map on the right.

What is your Diagnosis?

Diagnosis

This patient has the typical retinal findings of a nonischemic central retinal vein occlusion with macular edema. The challenge of this case is not the diagnosis but the management.

 

Discussion

Retinal venous occlusive disease is the second most common cause of severe vision loss in the United States. Population-based studies report a prevalence of 0.1 to 0.4%.  It is usually a unilateral disease, though, the annual risk of developing any type of retinal vascular occlusion in the fellow eye is approximately 1% per year.   Within 5 years of the onset of CRVO in one eye, it is estimated that up to 7% of persons may develop a CRVO in the fellow eye.

Historically, numerous treatments have been attempted, including intravitreal and systemic fibrinolytics, systemic anticoagulation, plasma dilution, laser anastomosis, and radial optic neurotomy.  None of these proved effective.  Grid laser photocoagulation was studied in the Central Retinal Vein Occlusion study and shown to help reduce macular edema.1 However, there was no benefit for visual acuity.

More recently, interest has turned towards intravitreal triamcinolone and anti-vascular endothelial growth factor drugs (Lucentis™ and Avastin™).  Results of randomized prospective controlled clinical trials have been recently announced.

In September 2009, results from the Standard Care vs Corticosteroid for Retinal Vein Occlusion (SCORE) Study were released.  This study showed intravitreal triamcinolone is superior to observation for treating vision loss associated with macular edema secondary to CRVO2.  In this National Eye Institute sponsored study, patients receiving 1-mg or 4-mg of intravitreal triamcinolone were five times more likely to gain 15 letters or more at 12 months than those under observation.  Although these visual results are encouraging, enthusiasm is tempered by the frequency of cataract progression and glaucoma in the triamcinolone treated groups.

Last month at the Retina Congress in New York, initial results of a randomized prospective trial of intravitreal Lucentis compared with sham injection (CRUISE Study) were presented.  Monthly intravitreal Lucentis resulted in at least a 15-letter gain in vision in approximately 46% of patients as compared with 16% in the sham group at 6 months.  The safety profile of these injections was similar to what we have seen in treatment of macular degeneration patients.  These early 6-month results are encouraging, but have not yet been published and longer-term follow-up data will determine if this treatment has long-term benefit.

 In our patient, our options were to consider one of 3 treatment courses: observation, intravitreal triamcinolone or Lucentis.  Because of his monocular status and significant difficulty with his vision and his livelihood, he preferred some treatment.  The patient chose monthly intravitreal ranibizumab (Lucentis™) to avoid the risk of glaucoma and cataract associated with intravitreal triamcinolone treatment. After one intravitreal ranibizumab injection, his vision improved from 20/32- to 20/25+ with accompanied decrease in CRT from 435mm to 275mm and complete resolution of retinal cysts (Figure 4).  Four weeks later, he received a second ranibizumab injection with further improvement of CRT to 260mm and return of the foveal depression (Figure 4). During 10 months of follow-up, he has maintained this good vision, but has required a total of 6 intravitreal Lucentis injections.

Figures 4: Optical coherence tomography studies. Top: Pre-Lucentis treatment. Middle: 4 weeks after first injection. Bottom: 4 weeks after second injection

Take Home Points

  • Retinal venous occlusive disease is the second most common cause of severe vision loss in the United States.
  • The Central Vein Occlusion Study (CVOS) showed grid laser photocoagulation does not improve vision when compared to observation in CRVO-associated macular edema and did not recommend grid laser for this condition.
  • The SCORE and the CRUISE studies both intravitreal triamcinolone and ranibizumab treatment had better visual acuity outcomes than the untreated natural history of macular edema secondary to perfused CRVO.

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References

  1. Central Vein Occlusion Study Group. Evaluation of grid pattern photocoagulation for macular edema in central vein occlusion. The Central Vein Occlusion Study Group M report. Ophthalmology 1995; 102:1425-1433.
  2. SCORE Study Research Group. A randomized trial comparing the efficacy and safety of intravitreal triamcinolone with observation to treat vision loss associated with macular edema secondary to central retinal vein occlusion: the Standard Care vs Corticosteroid for Retinal Vein Occlusion (SCORE) study report 5.  Arch Ophthalmol. 2009 Sep;127(9):1101-14.
  3. Brown, D. Safety and Efficacy of Intravitreal Ranibizumab (Lucentis) in Patients with Macular Edema Secondary to Central Retinal Vein Occulusion: CRUISE Study.  Presented at the Retina Congress. October 4, 2009.