Spark ImageWise 24

Unusual Peripapillary Lesion

Dr. Shashank Somani, Dr. Sarang Lambat, Dr. Prabhat Nangia, Dr. Vinay Nangia 
Suraj Eye Institute, 559 New Colony, Nagpur 

Case Description
A male, 41 years of age, came with rapid loss of vision in the left eye since 3 days. He was a known case of diabetes mellitus since 1.5 years. His best corrected visual acuity was 6/6, N6 in right eye and CF 2m, N36 in left eye. Anterior segment examination showed presence of posterior polar cataract in right eye and pseudophakia in Left eye. Intraocular pressure recorded by Goldmann applanation tonometer was 24 mmHg in right eye and 20 mmHg in left eye. Gonioscopy showed open angles upto scleral spur in both the eyes.

Figure 1: Fundus photograph of right eye with healthy disc and vertical cup disc ratio of 0.4:1. Macula, blood vessels, and periphery are normal.
Figure 2: Fundus photograph of the left eye shows blurring of disc margins suggestive of edema (yellow arrow). A greyish white mass is seen near the superotemporal margin of the disc (blue arrow). There is evidence of peripapillary edema reaching upto the fovea (green arrow).
Figure 3: The optical coherence tomography line scan of right eye passing through the macula shows normal foveal contour
Figure 4: The optical coherence tomography line scan of left eye passing through macula shows significant increase in thickness of the outer nuclear layer (ONL) suggestive of edema (yellow arrow). There is hypo-reflectivity in the sub retinal space under the fovea suggestive of sub retinal fluid (green arrow) and increase in the thickness of RNFL and inner nuclear layers (blue arrows).
Figure 5: The optical coherence tomography line scan of passing through the lesion shows round, well demarcated hyper reflective lesion in the sub retinal space (green arrow). There is hypo-reflectivity in the sub retinal space temporal to the lesion suggestive of sub retinal fluid (yellow arrow). Intraretinal fluid (IRF) can be seen above the lesion (pink arrow). Shadowing of the blood vessels can be seen (red arrows).
Figure 6: Left Eye – OCT RNFL scan showing edema of the disc (blue arrows)
Figure 7: Serial FFA images of the left eye. (a) Shows a normal early arteriovenous phase. (b). Late arteriovenous phase shows staining (blue arrow) of the temporal part of the disc. (c) Late recirculation phase shows subsequent leakage from the lesion and disc hyper fluorescence suggestive of disc edema.

The patient was advised intravitreal Inj. Bevacizumab for left eye. At one month follow up, his vision showed a substantial improvement to 6/6, N6. 

Figure 8: Fundus photograph of left eye with vertical cup disc ratio of 0.4:1. Disc edema and macular edema has completely resolved. Few hard exudates are seen around the macula. (Yellow arrow)
Figure 9: Left Eye OCT line scan passing through the macula shows significant reduction of SRF with normal foveal contour. Multiple intraretinal hyper reflective foci and hard exudates are seen around the fovea in outer plexiform layer (blue arrows).
Figure 10: Follow up OCT line scan of left eye shows complete reduction of SRF and RNFL edema with resolution of the lesion. There is thinning of the photoreceptor layer. (Blue arrow).

Discussion
Our patient presented with sudden loss of vision and on OCT we found a well defined lesion adjacent to the disc associated with subretinal fluid. We kept the differential diagnosis of inflammatory lesion of unknown origin, a possible inflammatory CNVM or peripapillary CNVM and a macro-aneurysm in mind. The rapid response to an anti-VEGF injection gives an impression that it could be a CNVM or an infective lesion. 

Peripapillary CNVM are defined as a collection of new choroidal blood vessels, any portion of which lies within one disc diameter of the nerve head. In peripapillary CNVM, choroidal vessels either traverse through breaks within Bruch’s membrane or extend around the termination of the membrane adjacent to the disc.

Some inflammatory lesions are known to regress rapidly with intravitreal anti-VEGF injections. However the patient did not give a history of  recent viral infection and there were not other signs in the retina. A macro-aneurysm would not have responded  so rapidly and completely to a single anti-VEGF injection. Following resolution after a single anti-VEGF injection, we did not see any  recurrence. The prominent cause of PCNVM include age related macular degeneration (AMD), angioid streaks, myopia, post laser scars, choroidal rupture, non-infective posterior uveitis, disc malformations, colobomas, choroidal tumours, polypoidal choroidal vasculopathy (PCV) and in many cases no obvious cause is found. These CNVs respond very well to anti-VEGF treatment. These CNVMs are uncommon in occurrence and can lead significant vision loss if they progress under the retinal layers and reach the macula. Early treatment has been shown to have better outcomes in such cases. 

ReadWise

  1. Jutley G, Tah V, Lindfield D, Menon G. Treating peripapillary choroidal neovascular membranes: a review of the evidence. Eye (Lond). 2011 Jun;25(6):675-81. doi: 10.1038/eye.2011.24
  2. Ozgonul C, Moinuddin O, Munie M, Lee MS, Bhatti MT, Landau K, Van Stavern GP, Mackay DD, Lebas M, DeLott LB, Cornblath WT, Besirli CG. Management of Peripapillary Choroidal Neovascular Membrane in Patients With Idiopathic Intracranial Hypertension. J Neuroophthalmol. 2019 Dec;39(4):451-457. doi: 10.1097/WNO.0000000000000781.

Dr. Sarang Lambat
MS, FRF
Consultant
Vitreoretinal services
Suraj Eye Institute
Nagpur
Email – education@surajeye.org

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