|Year : 2022 | Volume
| Issue : 4 | Page : 932-934
Neovascular glaucoma in atypical central serous chorioretinopathy and pachychoroid disease
Aparna Rao, Srikanta Kumar Padhy
LV Prasad Eye Institute, MTC Campus, Bhubaneswar, Odisha, India
|Date of Submission||01-May-2022|
|Date of Acceptance||19-Jul-2022|
|Date of Web Publication||11-Oct-2022|
Dr. Aparna Rao
Glaucoma Services, LV Prasad Eye Institute, MTC campus, Patia, Bhubaneswar - 751 024, Odisha
Source of Support: None, Conflict of Interest: None
We report an atypical central serous chorioretinopathy (CSCR) with pachychoroid disease and consequent recalcitrant neovascular glaucoma (NVG). A 45-year-old man with persistent subretinal fluid, pachychoroid and bilateral large areas of capillary nonperfusion exudative detachment, and CSCR presented with raised intraocular pressure (IOP) and neovascular glaucoma. Failure after trabeculectomy with mitomycin-C mandated the implantation of Ahmed glaucoma valve followed by two epiconjunctival MMC applications over the AGV plate/bleb in the hypertensive phase. He was also started on 25 mg oral eplerenone twice a day for the first month, followed by 25 mg once a day over the next two months, followed by intravitreal bevacizumab injection. The retinal neovascularization regressed along with normalization of IOP at over eight months follow-up.
Keywords: Central serous chorioretinopathy, glaucoma surgery, ischemia, neovascular glaucoma, pachychoroid
|How to cite this article:|
Rao A, Padhy SK. Neovascular glaucoma in atypical central serous chorioretinopathy and pachychoroid disease. Indian J Ophthalmol Case Rep 2022;2:932-4
|How to cite this URL:|
Rao A, Padhy SK. Neovascular glaucoma in atypical central serous chorioretinopathy and pachychoroid disease. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2022 Dec 3];2:932-4. Available from: https://www.ijoreports.in/text.asp?2022/2/4/932/358202
Central serous chorioretinopathy (CSCR) represents a benign, self-limiting, ocular disease characterized by accumulation of fluid underneath the retinal pigment epithelium or rarely causing neurosensory detachment.[1–3] Usually, they resolve spontaneously and rarely require laser treatment for repeat or persisting leakage caused by hyperpermeable choroidal vessels. While the exact etiology is unknown, the precipitating factors include smoking, stress, and/or use of tobacco. Pachychoroid disease represents a distinct entity with presence of dilated outer choroidal vessels (called pachyvessels) with or without increased choroidal thickness in a focal or diffuse area and may be associated with systemic diseases.[2–5] Treatment is only required for CSCR cases with persisting subretinal fluid or macular exudation. Angiogenic factors release may be triggered in these cases by chronic inflammation of the choroid coupled with RPE-Bruch's layer disruption, thereby causing choroidal neovascularization. We present an atypical case of CSCR with pachychoroid accompanied by persisting exudative detachment, presenting with neovascular glaucoma (NVG) and describe the challenges in the treatment of these eyes.
| Case Report|| |
A 45-year-old man, a known hypertensive and non-diabetic, was referred to us with neovascular glaucoma in the right eye, diagnosed recently outside. On examination, his best-corrected visual acuity (BCVA) and intraocular pressure (IOP) was 20/125, 22 mmHg and 20/100, 14 mmHg, respectively, with neovascularization of the iris (NVI) seen at 360° [[Figure 1], inset] and closed angles on gonioscopy in the right eye. Dilated fundus examination of both eyes showed presence of retinal pigment epithelial changes with gravitational tracts at the posterior pole, inferior exudative retinal detachment, and bony spicule-like pigmentary changes. Additionally, presence of epiretinal membrane and surface neovascularization inferiorly could be noted in the right and left eye, respectively.
|Figure 1: Color fundus photograph of the right eye (a) shows presence of retinal pigment epithelial changes with gravitational tracts at the posterior pole, inferior exudative retinal detachment, bony, spicule-like pigmentary changes, and inferior peripheral retinal neovascularization (arrow); additionally, left eye fundus (b) shows presence of epiretinal membrane inferior to disc; fundus fluorescein angiography of the right eye, inset showing neovascularization of iris temporally after trabeculectomy, (c and d) demonstrates focal leaks temporally, mottled hyperfluorescence, large areas of capillary nonperfusion inferiorly (asterisk) along with the intense hyperfluorescence corresponding to the surface retinal neovascularization; swept-source optical coherence tomography scan passing through the fovea of the right eye (e) and left eye (f) reveals presence of subretinal hyperreflective material, disrupted photoreceptor layer along with evident pachychoroid; furthermore, left eye shows epiretinal hyperreflective membrane|
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Swept-source optical coherence tomography (SS-OCT) scan passing through the fovea demonstrated the presence of subretinal hyperreflective material and disrupted photoreceptor layer along with evident pachychoroid. Fundus fluorescein angiography (FFA) in both eyes showed focal leaks, window defects, bilateral large areas of capillary nonperfusion inferiorly along with the intense hyperfluorescence from the surface retinal neovascularization in the right eye [Figure 1]a,[Figure 1]b,[Figure 1]c,[Figure 1]d,[Figure 1]e,[Figure 1]f. Absence of drusen on OCT and FFA ruled out age-related macular degeneration as the cause of neovascularization elsewhere (NVE). Based on clinical and imaging features, a diagnosis of CSCR in both eyes with secondary neovascular glaucoma in the right eye was made.
Subsequently, the patient underwent fundus fluorescence–guided conventional focal laser (532 nm) in both the eyes followed by trabeculectomy with mitomycin C in the right eye for uncontrolled IOP. Early bleb failure at one week, with persisting florid NVI warranted anti-glaucoma medications for two months after which he was advised Ahmed glaucoma valve (AGV, FP7 model) surgery for uncontrolled IOP (48 mmHg) despite maximal tolerated medical therapy. At this point of time, the patient was started on 25 mg oral eplerenone twice a day for the first month, followed by 25 mg once a day over the next two months after confirming a normal potassium level.
Postoperatively, hypertensive phase with a tense bleb required two epiconjunctival MMC applications over the AGV plate/bleb spaced over two weeks along with anti-glaucoma medication. Though the IOP was controlled two months after glaucoma implant, there was not much decline in the exudative component of the detachment along with persistent foci of surface neovascularization in the right eye. Afterwards, intravitreal bevacizumab 1.25 mg/0.05 ml was injected in the right eye. At final follow-up of 8 months, the NVE/NVI were noted to have regressed [[Figure 2], inset] with an IOP of 20 mmHg in the right eye. At the final visit, a resolution of exudative retinal detachment was noted (though not to a complete extent) with disappearance of surface neovascularization [Figure 2]a and [Figure 2]b. The patient was also being counselled for targeted retinal laser photocoagulation of inferior ischemic retina in later visits.
|Figure 2: Color fundus photograph of the right eye (a) shows presence of retinal pigment epithelial changes with gravitational tracts at the posterior pole, bony, spicule-like pigmentary changes with a reduction in height of the inferior exudative retinal detachment, and complete disappearance of inferior peripheral retinal neovascularization; swept-source optical coherence tomography scan passing through the fovea of the right eye, inset showing complete disappearance of neovascularization of the iris; (b) shows an inner retinal dimpling, shallow subretinal fluid, flat pigment epithelial detachment temporally and evident pachychoroid|
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| Discussion|| |
Neovascular glaucoma represents a devastating sequela of several serious ocular and systemic etiologies, with vascular occlusions, diabetic retinopathy, and ocular ischemic syndrome being the most common ocular causes. While it can be caused by a wide variety of causes in the anterior and posterior segment, the common factor responsible for the disease pathogenesis is retinal ischemia.
Ischemia is the common trigger for the development of new vessels, owing to the increase in angiogenic factors. While other causes of NVG with an ischemic cause are common, CSCR with long-standing exudative detachment as a cause of NVG, as seen in our case, is not known so far.[1–5] While laser in our case prevented further leakage, the source of ischemia was not very clear.
Bullous exudative retinal detachment is an extremely rare and atypical presentation of CSCR that results in permanent visual dysfunction, if left untreated. Peripheral retinal neovascularization in the setting of CSCR is very rare and may deceptively mimic inflammatory ocular diseases. The proposed pathogenesis of surface retinal neovascularization has been attributed to the longstanding retinal detachment–related ischemia of the inner retinal layers and degeneration of neurons due to thickening of basal lamina of retinal vessels, thereby leading to occlusion. This, in turn, upregulates the release of vascular endothelial growth factor (VEGF) and inflammatory mediators.,,, Persistent subretinal fluid (SRF) can be treated with prompt focal laser photocoagulation of extrafoveal leaking sites or oral eplerenone.,,, In our case, chronic choroidal ischemia, choroidal hyperpermeability, and RPE dysfunction may have led to the longstanding exudative retinal detachment and retinal ischemia, thereby leading to recalcitrant NVG.
Our earlier study showed good results after epi-MMC application in trabeculectomy in NVG eyes with none of them requiring needling or repeat surgery. Epiconjunctival MMC application helps prevent early failures after trabeculectomy, though its use after AGV is unreported. In our case, epiconjunctival MMC helped control the IOP in the hypertensive phase along with parallel treatment of hypertension.
| Conclusion|| |
In summary, persisting SRF in cases of CSCR with pachychoroid must be closely monitored for development of NVG, which can be refractory to routine surgical procedures unless the trigger for chronic ischemia is also treated parallelly. Tailored approaches and parallel treatment of IOP and ischemia is required for optimal outcomes in such 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|| |
Pang CE, Freund KB. Pachychoroid neovasculopathy. Retina 2015;35:1-9.
Wang M, Munch IC, Hasler PW, Prunte C, Larsen M. Central serous chorioretinopathy. Acta Ophthalmol 2008;86:126-45.
Warrow DJ, Hoang QV, Freund KB. Pachychoroid pigment epitheliopathy. Retina 2013;33:1659-72.
Dansingani KK, Balaratnasingam C, Klufas MA, Sarraf D, Freund KB. Optical coherence tomography angiography of shallow irregular pigment epithelial detachments in pachychoroid spectrum disease Am J Ophthalmol 2015;160:1243-54.
Lehmann M, Bousquet E, Beydoun T, Behar-Cohen F. Pachychoroid: An inherited condition? Retina 2015;35:10-6.
Sahu DK, Namperumalsamy P, Hilton GF, Sousa NF., de Bullous variant of idiopathic central serous chorioretinopathy. Br J Ophthalmol 2000;84:485-92.
Madanagopalan VG, Shah K, Nagesha CK, Baskaran P. Peripheral retinal avascularity and capillary leakage in central serous chorioretinopathy. J Curr Ophthalmol 2019;31:220-4.
Bousquet E, Beydoun T, Zhao M, Hassan L, Offret O, Behar-Cohen F. Mineralocorticoid receptor antagonism in the treatment of chronic central serous chorioretinopathy: A pilot study. Retina (Philadelphia, Pa) 2013;33:2096-102.
John VJ, Mandelcorn ED, Albini TA. Internal drainage for chronic macula-involving serous retinal detachment in idiopathic central serous chorioretinopathy. Int Ophthalmol 2014;34:91-5.
Rao A, Chatterjee S. Epiconjunctival mitomycin-C application for early failing filtering blebs. Semin Ophthalmol 2014;29:48-51.
[Figure 1], [Figure 2]