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Year : 2021  |  Volume : 1  |  Issue : 2  |  Page : 329-331

Spontaneous closure of macular hole with conversion to type 2 choroidal neovascular membrane in a case of Vogt Koyanagi Harada disease

Sankara Nethralaya, Medical Research Foundation, Chennai, Tamil Nadu, India

Date of Submission08-Jul-2020
Date of Acceptance16-Dec-2020
Date of Web Publication01-Apr-2021

Correspondence Address:
Dr. Parveen Sen
Sankara Nethralaya, Medical Research Foundation, New no. 41, Old no. 18, College Rd, Opposite Women's Christian College, Thousand Lights West, Nungambakkam, Chennai - 600 006, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_2225_20

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Macular hole in uveitic pathologies is a rare entity. Spontaneous closure of macular hole with conversion to a type 2 choroidal neovascular membrane has been reported even more sparsely. We present an interesting case of a 51-year-old woman with convalescent stage of Vogt-Koyanagi-Harada disease of 20 years duration, who developed a macular hole along with reactivation of the disease. Immunosuppressive therapy was started to control the ocular inflammation, while the macular hole was observed. One and half years later, there was a repeat episode of inflammation when she presented with a decline in vision in the same eye with a choroidal neovascular complex and spontaneous closure of the macular hole. She received two intravitreal injections of ranibizumab biosimilar under systemic immunosuppressive cover. The CNV was stable and the macular hole remained closed over a 6-month drug free interval from the last injection. This is the first report of spontaneous closure of a macular hole with a concurrent neovascular membrane in a case of VKH.

Keywords: Convalescent, macular hole, spontaneous closure, type 2 CNVM, VKH

How to cite this article:
Vaidya H, George A, Sen P. Spontaneous closure of macular hole with conversion to type 2 choroidal neovascular membrane in a case of Vogt Koyanagi Harada disease. Indian J Ophthalmol Case Rep 2021;1:329-31

How to cite this URL:
Vaidya H, George A, Sen P. Spontaneous closure of macular hole with conversion to type 2 choroidal neovascular membrane in a case of Vogt Koyanagi Harada disease. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Aug 3];1:329-31. Available from: https://www.ijoreports.in/text.asp?2021/1/2/329/312364

Macular abnormalities confirmed on spectral-domain OCT were noted in 58.6% pathologies, in a series of 413 uveitic eyes.[1] Macular hole (MH) is noted in 1-2.6% uveitic cases and rarely observed (0.2%) in Vogt Koyanagi Harada (VKH) disease.[2] However, spontaneous closure of the MH in VKH has not been reported, to the best of our knowledge. Spontaneous closure of idiopathic macular hole with concurrent or conversion to exudative AMD and PCV has been reported.[3],[4] We report the first case of spontaneous closure of inflammatory macular hole (IMH) with conversion to inflammatory choroidal neovascular (iCNV) membrane in a case of VKH.

  Case Report Top

A 51-year-old woman, a case of VKH disease for 20 years and secondary open-angle glaucoma in the right eye for 5 years, presented to the retina clinic with a best-corrected visual acuity (BCVA) of 20/400 and 20/30 in the right eye (OD) and left eye (OS) respectively. Anterior segment evaluation of both eyes revealed pigments on endothelium, stable posterior chamber intraocular lens, and presence of anterior vitreous cells. Posterior segment evaluation of OD revealed dense vitritis which precluded a thorough macular assessment [Figure 1]a. OS had minimal vitreous haze along with a sunset glow fundus, peripapillary atrophy, and scattered depigmented areas over the fundus. Swept-source optical coherence tomography (SS-OCT, Atlantis, Topcon Corporation, Tokyo, Japan) of OD revealed a full-thickness MH (Hole forming factor: 0.83) with cystic spaces [Figure 1]c. OS showed a normal foveal contour with reduced choroidal thickness in both eyes (sub-foveal choroidal thickness OD: 90μ, OS: 170μ). Inflammation resolved in subsequent visits with immunosuppressive therapy (oral steroids and azathioprine) while the macular hole was observed.
Figure 1: (a) Fundus photograph of the right eye showing hazy media, disc pallor with peripapillary atrophy, sunset glow fundus with unclear posterior pole showing pigmentation just superior to fovea. (b) Wide field fundus photograph additionally showing pigmented scar at the Macula. (c) SSOCT at presentation of the right eye showing FTMH with cystic spaces at the edges of MH with outer retinal layer loss. (d) Follow up SSOCT showing a type 2 CNV with subretinal hyper-reflective material (SHRM), along with ONL thinning. (e-h) ORCC slab on SSOCTA with enface image of the showing network of vessels of the CNV (e) which subsequently shrinks in size with intravitreal ranibizumab biosimilar injection (f, g) and scarred CNV (h)

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She was lost to follow up and presented 18 months later with reactivation of inflammation. BCVA in this visit was 20/200 in OD and 20/20 in OS. Both eyes showed signs of inflammation. Right eye fundus examination additionally revealed a hemorrhagic spot at the macula. SSOCT of OD revealed a type 1 closure of macular hole along with subretinal hyper-reflective material and type 2 CNV with areas of scarring within it [Figure 1]b, [Figure 1]d and [Figure 1]e. OS showed a normal study. Immunosuppressive therapy was re-started. Intravitreal injection Razumab (ranibizumab biosimilar) was administered twice, at monthly intervals [Figure 1]f and [Figure 1]g. At final follow up, 6 months after the last intravitreal injection, vision was stable.no evidence of inflammation and SSOCTA (PLEX® Elite 9000 v 1.6, Carl Zeiss Meditec, Dublin, California, USA) of OD revealed a scarred CNV with thinning of ONL around the fibrous tissue, resolution of SHRM, presence of outer retinal tubulations, and no evidence of activity [Figure 1]h.

  Discussion Top

Inflammatory macular hole can form in two distinct ways: 1) inflammatory PVD induction and VMT which progresses to MH formation. 2) Necrotic tissue loss around the fovea caused by inflammatory foci just around the fovea.[5] Macular abnormalities are observed more frequently in cases with recurrent episodes of inflammation as observed in our case of MH.[2] Patient presented with reactivation of disease and a macular hole with tall edges (HFF 0.83), cystoid changes along with outer retinal layer loss and choroidal thinning, consistent with the description of IMH.[6] In the same review article, a low likelihood of spontaneous closure of inflammatory MHs was suggested with 50-90% surgical closure and moderate visual gains (20/40 – 20/80). Unlike spontaneous closure of traumatic MH (10-47%), spontaneous closure of IMH has been sparsely documented and none in case of VKH.[7] Since there was no tractional component to the IMH, the spontaneous closure in our patient could most likely have been due to glial proliferation of retinal tissue as suggested by Yamada et al.[8]

The prevalence of iCNV in patients with VKH disease is between 9-15%.[9] Chronic retention of subretinal fluid under a macular hole causes inflammatory mediators to disrupt the Bruch's membrane leading to CNV formation.[10] Sakata et al. believed that the presence of CNV and activity of VKH was suggestive of inadequate clinical control of the disease.[11]

Su et al. reported a case of a 90-year-old female who presented with a long-standing idiopathic macular hole with a pigment epithelial detachment. The macular hole spontaneously closed with conversion to exudative age-related macular degeneration forming a type 2 neovascular membrane, which stabilized with 4 intravitreal injections of bevacizumab.[3] Lee et al. described a case of a 75-year-old African American female with spontaneous closure of a chronic idiopathic macular hole 2 years after a failed macular hole surgery, along with a fibrotic scar due to a presumably regressed PCV lesion.[4] At 4 year follow up, the patient had a vision of 20/50 parts, and the authors attribute it to the preserved thickness of the outer nuclear layer as an indicator of photoreceptor integrity.

Ours is a unique case of a 51-year-old woman with a convalescent stage VKH disease, who developed spontaneous closure of a macular hole with conversion to an inflammatory (type 2) macular neovascular membrane. The neovascular membrane stabilized with two intravitreal injections of ranibizumab biosimilar under immunosuppressive cover. Although there were no tractional forces, presence of intraretinal cysts, relatively small macular hole with possible glial proliferation induced by the inflammatory mediators favored the closure of the macular hole in our case. Prolonged exposure of the RPE-Bruch's complex at the base of the macular hole to the inflammatory mediators within the vitreous may have triggered the formation of the choroidal neovascular membrane. The neovascular membrane was managed using immunosuppressive therapy and fewer intravitreal anti-VEGF injections as opposed to ARMD cases.

  Conclusion Top

This case highlights macular changes in uveitic cases and the role of OCT in diagnosis and management of these cases.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Liu T, Bi H, Wang X, Gao Y, Wang G, Ma W. Macular abnormalities in Chinese patients with uveitis. Optom Vis Sci 2015;92:858-62.  Back to cited text no. 1
Yang P, Ye Z, Xu J, Du L, Zhou Q, Qi J, et al. Macular abnormalities in vogt-koyanagi-harada disease. Ocul Immunol Inflamm 2019;27:1195-202.  Back to cited text no. 2
Su D, Klufas MA, Hubschman JP. Spontaneous closure of a full-thickness macular hole with conversion to exudative age-related macular degeneration. JAMA Ophthalmol 2016;134:604-6.  Back to cited text no. 3
Lee J, Nguyen VQ, Doss MK, Eller AW. Spontaneous closure of a chronic full thickness macular hole after failed surgery. Am J Ophthalmol Case Rep 2018;13:59-61.  Back to cited text no. 4
Shukla D. Secondary macular holes: When to jump in and when to stay out. Expert Rev Ophthalmol 2013;8:437-46.  Back to cited text no. 5
Kumawat D, Venkatesh P, Brar AS, Sahay P, Kumar V, Chandra P, et al. Atypical macular holes. Retina 2019;39:1236-64.  Back to cited text no. 6
Bonnin N, Cornut PL, Chaise F, Labeille E, Manificat HJ, Feldman A, et al. Spontaneous closure of macular holes secondary to posterior uveitis: Case series and a literature review. J Ophthalmic Inflamm Infect 2013;3:34.  Back to cited text no. 7
Yamada H, Sakai A, Yamada E, Nishimura T, Matsumura M. Spontaneous closure of traumatic macular hole. Am J Ophthalmol 2002;134:340-7.  Back to cited text no. 8
Agarwal A, Invernizzi A, Singh RB, Foulsham W, Aggarwal K, Handa S, et al. An update on inflammatory choroidal neovascularization: Epidemiology, multimodal imaging, and management. J Ophthalmic Inflamm Infect 2018;8:13.  Back to cited text no. 9
Singh RP, Kaiser PK. Treatment of Co-existent occult choroidal neovascular membrane and macular hole. Surv Ophthalmol 2007;52:547.  Back to cited text no. 10
Sakata VM, Morita C, Lavezzo MM, Rodriguez EE, Abdallah SF, Pimentel SL, et al. Outcomes of intravitreal bevacizumab in choroidal neovascularization in Vogt-Koyanagi-Harada disease-a prospective study. Ocul Immunol Inflamm 2019:1-7. doi: 10.1080/09273948.2019.1687731.  Back to cited text no. 11


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