Polypoidal – March 2016

March 2016

Case Presentation

A 60 year-old African American diabetic patient was referred to us for central loss of vision in her right eye of ten (10) days in duration. She has been diagnosed with Type II Diabetes three (3) years ago but was doing well without medication. In addition, she had well controlled hypertension and glaucoma, for which she was taking Latanoprost drops OU.

Examination

On examination, her visual acuity was 20/60 OD, 20/20 OS. On funduscopy, she was noted to have a large subretinal hemorrhage in her right eye (Figure 1). The left eye posterior exam was unremarkable (Figure 2).

Figure1
Figure 1
Figure2
Figure 2

Differential Diagnosis

The differential diagnosis includes:

  • Age-related macular degeneration (AMD)
  • Polypoidal choroidal vasculopathy (PCV)
  • Retinal Macroaneurysm
  • Valsalva Retinopathy
  • Terson retinopathy
  • Bleeding from intraocular tumor, such as choroidal melanoma

Investigations

To help differentiate between the potential diagnoses, the following tests were done: Fuorescein angiography (FA), indocyanine green (ICG) angiography, and spectral domain optical coherence tomography (OCT).

The FA showed blockage of underlying choroidal vasculature in the area of the subretinal hemorrhage in the right eye, without any visible leaks (Figure 3). The left eye was unremarkable (Figure 4). ICG angiography revealed hyperfluorescence consistent with leakage in the maculopapular (MP) bundle of the right eye (Figure 5). This technology allows the detection of various pathologies not identified through FA because its higher wavelength of emission allows visualisation through blood, fluid and pigment, which usually block any fluorescein emission. Therefore, ICG angiography is comonly employed in cases of retinal or subretinal hemorrhage to help identify any underlying leaks not visible with FA.

Figure3
Figure 3 – Blockage on FA
Figure4
Figure 4 – Unremarkable Left eye FA
Figure5
Figure 5 – ICG with leakage in MP bundle

OCT imaging was normal in the left eye but revealed a significant quantity of subretinal and sub-retinal pigment epithelium fluid and blood in the right eye (Figure 6 & 7).

Figure6
Figure 6 – Subretinal elevation
Figure7
Figure 7

Discussion

The most probable diagnosis was polypoidal choroidal vasculopathy. This sybtype of AMD leading to choroidal neovascularization (CNV) is more common in African Americans and Asians and is characterized by multiple leaking aneurysmal dilations of choroidal vasculature. These patients often present with subretinal fluid and/or hemorrhage or vitreous hemorrhage. Typically, they lack the drusen of AMD.

ICG angiography is very helpful in distinguishing PCV from other causes of subretinal hemorrhage, by demonstrating focal areas of leakage from CNV, often missed by fluorescein angiography because of the presence of blood.

Treatment

It was determined to pursue aggressive Anti-VEGF therapy for this particular patient. However, there are many treatment options for this patients, used either alone or in combination:

    1. Anti-VEGF injections
      • Given that the underlying cause of the hemorrhage is a CNV, anti-VEGF can be employed successfully, on a similar schedule to AMD
    2. Vitrectomy with subretinal recominant tissue plasminogen activator (rtPA).
      • Vitrectomy can be helpful in clearing a breakthrough vitreous hemorrhage. Subretinal injection of rtPA with gas injection can help displace and absorb the blood.
    3. Pneumatic displacement of the subretinal blood.
      • An intravitreal injection of air or gas can be used to displace the subretinal blood inferiorly, out of the macula.
    4. Intravitreal rtPA injection
    5. Photodynamic therapy (PDT)
      • PDT can target the CNV

Since subretinal blood is believed to be toxic to the photoreceptors, expeditive referral and treatment is essential to improve the prognosis.

References

de Jong JH, van Zeeburg EJ, Cereda MG, van Velthoven ME, Faridpooya K, Vermeer KA, van Meurs JC. Intravitreal versus Subretinal Administreation of Recombinant Tissue Plasminogen Activator Combined with Gas for Acute Submacular Hemorrhages Due to Age-Related Macular Degeneration: An Exploratory Prospective Study. Retina.2016 Jan 20. [Epub ahead of print]

Lin TC, Hwang DK, Lee FL, Chen SJ. Visual Prognosis of Massive Submacular Hemorrhage in Polypoidal Choroidal Vasculopathy With or Without Combination Treatment. J Chin Med Assoc.2016 Jan 8. [Epub ahead of print]

Scuta GL, Cantor LB, Cioffi GA. Basic and Clinical Science Course. Singapore: American Academy of Ophthalmology, 2013:85-87

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