Spotlight Case: The Not-So-Classic Caucasian Patient in a Retina Clinic

Natalia Vila, MD

Michael A. Kapusta, MD

David E. Lederer, MD


Case history

A 57-year-old Caucasian woman presented with decreased visual acuity (VA) in the right eye for 1 month. Ocular history was remarkable for refractive amblyopia (+5D) in the left eye. She denied any ocular history (inflammatory disorders, trauma, family history of hereditary conditions), or medical history.

On examination, her best-corrected visual acuity (BCVA) was 20/100 OD and 20/400 OS. Intraocular pressures and anterior segment examination were within normal limits OU. Fundus examination demonstrated an extrafoveal subretinal nodular elevation with subretinal fluid (SRF) and lipid exudation. Fluorescein angiography (FA) revealed early hyperfluorescence and late leakage surrounding the lesion. Optical coherence tomography (OCT) revealed the presence of a pigment epithelial detachment (PED), cystoid thickening, and SRF(Figure 1).

Figure 1: Fundus photograph of the right eye showing a parafoveal orange-red nodule–like lesion, lipid exudates and pigmentary changes.

Figure 2: OCT of the right eye after 2 intravitreal injections of bevacizumab (4-week interval). Presence of PEDs with subretinal and intraretinal fluid.

What’s your diagnosis?

A choroidal neovascular membrane was presumed and intravitreal injections of bevacizumab (1.25 mg/0.05 ml) were commenced. After the second monthly injection, VA improved to 20/70. The OCT did not show significant changes compared to baseline, with persistence of SRF and the PED (Figure 2)

An indocyanine green (ICG) angiogram demonstrated a single focal nodular area of hyperfluorescence arising from the choroidal circulation consistent with a polypoidal structure(Figure 3). Our diagnosis after the ICG was polypoidal choroidal vasculopathy (PCV) and the therapeutic plan was adjusted. Photodynamic therapy (PDT) with verteporfin (half-fluence: 25 J/cm2 for 83s, spot size 1.6 mm) was performed. 

Figure 3: ICG angiogram of the right eye showing a single polypoidal lesion (arrow) with surrounding choroidal vascular alterations.

Figure 4: A. Fundus photograph of the right eye 2 months after the first verteporfin PDT showing a decreased lipid exudation and fluid. B. Corresponding OCT of the right eye demonstrating decreased subretinal and intaretinal fluid.

VA improved to 20/50 and the SRF decreased on the OCT (Figure 4). At 4 months after the first PDT, the vision decreased to 20/70 and a reactivation with recurrent SRF on OCT warranted a second session of PDT. VA improved again to 20/50 and the OCT and VA were stabilized for 1 year.

Discussion

The prevalence of PCV is 10% to 54% in Asian patients and 8% to 12% in Caucasian patients with presumed exudative age-related macular degeneration (AMD).[1-3] The initial clinical findings in our patient revealed the presence of lipid exudates, an orange nodule-like structure, a PED, and the lack of drusen, that led us to the diagnosis of PCV. 

The gold-standard test to confirm this diagnosis is ICG angiography, demonstrating the presence of polypoidal structures. Among the available therapies for PCV, verteporfin PDT has shown regression of polyps and the capability to stabilize or improve visual acuity. 

Anti-VEGF agents are being increasingly used in cases of non-AMD choroidal neovascular membranes (CNVM) from causes like pathologic myopia, inflammatory conditions, or idiopathic causes. In PCV, the rationale to use anti-VEGF agents is based on  increased VEGF in the aqueous humor in eyes with this disease. [4] Anti-VEGF therapy has proven effective in reducing the SRF but not in polyp regression. [5] 

The EVEREST studyshowed that verteporfin PDT combined with ranibizumab 0.5 mg or alone was superior to ranibizumab monotherapy in achieving complete regression of polyps in patients with symptomatic macular PCV. Only 28.6% of patients treated with ranibizumab achieved complete polyp regression, compared with PDT, 71.4%, or PDT combined with ranibizumab, 77.8%.[5] 

Histopathologic studies correlating VEGF expression in PCV were not universally positive.[6] This  reinforces that monotherapy with anti-VEGF treatment is not the best treatment for PCV.

Take-home points

  • A PED on OCT, combined with subretinal hemorrhage, lipid exudates, and an orange-red nodule–like structure on the clinical examination are considered a hallmark of PCV. The classic orange nodule may be less prominent in Caucasian patients.
  • At initial clinical presentation, the distinction with traditional AMD may alter the management decisions and influence the eventual visual outcome. An ICG should be performed to rule out PCV when the clinical features and OCT findings suggest this disease.
  • If the proper diagnosis can be made, the therapeutic strategy should be altered and PDT considered, alone or in combination with anti-VEGF agents, to achieve closure of the polyp and a VA improvement. 

References

  1. Sho K, Takahashi K, Yamada H, et al. Polypoidal choroidal vasculopathy: incidence, demographic features, and clinical characteristics. Arch Ophthalmol. 2003;121(10):1392-1396.
  2. Kwok AK, Lai TY, Chan CW, Neoh EL, Lam DS. Polypoidal choroidal vasculopathy in Chinese patients. Br J Ophthalmol. 2002;86(8):892-897. 
  3. Lafaut BA, Leys AM, Snyers B, Rasquin F, De Laey JJ. Polypoidal choroidal vasculopathy in Caucasians. Graefes Arch Clin Exp Ophthalmol. 2000;238(9):752-759.
  4. Tong JP, Chan WM, Liu DT, et al. Aqueous humor levels of vascular endothelial growth factor and pigment epithelium-derived factor in polypoidal choroidal vasculopathy and choroidal neovascularization. Am J Ophthalmol. 2006;141(3):456-462.
  5. Koh A, Lee Wonki, Chen L, et al. EVEREST study: efficacy and safety of verteporfin photodynamic therapy in combination with ranibizumab or alone versus ranibizumab monotherapy in patients with symptomatic macular polypoidal choroidal vasculopathy. Retina. 2012;32(8):1453-1464.
  6. Nakashizuka H, Mitsumata M, Okisaka S, et al. Clinicopathologic findings in polypoidal choroidal vasculopathy. Invest Ophthalmol Vis Sci. 2008;49(11):4729-4737.

Financial disclosures

Dr. Vila – None.

Dr. Kapusta – BAYER HEALTHCARE: Consulting honoraria; NOVARTIS PHARMACEUTICALS: Consulting honoraria;  ALCON LABORATORIES, INC: Consulting Honoraria; ARCTIC Dx-Advisory panel.

Dr. Lederer – BAYER HEALTHCARE: Advisory Board, Honoraria; NOVARTIS PHARMACEUTICALS CORPORATION: Advisory Board, Speaker, Honoraria; ALCON LABORATORIES, INC: Speaker, Honoraria.  

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