Cytomegalovirus retinitis with panretinal occlusive vasculopathy concealed by Posner– Schlossman syndrome: a case report

Cytomegalovirus retinitis with panretinal occlusive vasculopathy concealed by Posner– Schlossman syndrome: a case report | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Case Report Cytomegalovirus retinitis with panretinal occlusive vasculopathy concealed by Posner– Schlossman syndrome: a case report Seongyong Jeong This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-4261984/v1 This work is licensed under a CC BY 4.0 License Status: Posted Version 1 posted You are reading this latest preprint version Abstract Background Cytomegalovirus (CMV) retinitis is a rare disease, and its overlapping manifestation involving anterior segment is extremely uncommon. We report a case that initially presented Posner-Schlossman syndrome (PSS) and was later diagnosed with CMV retinitis. Case presentation A 72-year-old man visited tertiary hospital with uncontrolled intraocular pressure (IOP) in his right eye. At initial presentation, IOP was 36 mmHg and the fundus was not clear due to corneal edema. The spectral-domain optical coherence tomography revealed paracentral acute middle maculopathy (PAMM). A panretinal obstructive vasculopathy was observed on ultra-widefield fluorescein angiography. Three weeks later, trabeculectomy was performed to resolve the persistent high IOP. Once the cornea edema improved, a white patch-like peripheral lesion and silver wire-like retinal vasculature were observed. Aqueous tapping for polymerase chain reaction was positive for CMV. Oral valganciclovir and intravitreal ganciclovir were administered as anti-viral therapy. Despite treatment for four months, the final visual acuity was no light perception with bullous keratopathy and neovascularization on iris. Conclusions We described a rare case of simultaneous occurrence of PSS and CMV retinitis. The presence of PAMM could be an initial identifiable sign for CMV retinitis, even when media opacity is present. Cytomegalovirus retinitis Ganciclovir Panretinal occlusive vasculopathy Paracentral acute middle maculopathy Posner–Schlossman syndrome Figures Figure 1 Figure 2 Figure 3 Background Cytomegalovirus (CMV) belongs to the herpes virus family with double-stranded DNA [ 1 ]. It can affect both the anterior and posterior segment of eye with a wide range of clinical manifestation [ 2 – 4 ]. CMV-associated anterior uveitis (AU) usually occurs in the eyes of immunocompetent patients [ 5 ]. In contrast, typical CMV retinitis is an opportunistic infection found in immunocompromised hosts such as patients infected with human immunodeficiency virus. Therefore, the coexistence of these two diseases in a single individual is uncommon. We report a case that initially presented as Posner-Schlossman syndrome (PSS) and was later revealed to have CMV retinitis with panretinal occlusive vasculopathy. Case presentation A 72-year-old man was referred to the tertiary hospital with uncontrolled intraocular pressure (IOP) in his right eye. He had diabetes mellitus under control with medication for 20 years. There was no other remarkable medical history. Visual acuity was hand motion in the right eye. The IOP of right eye was 36 mmHg despite IOP-lowering treatment including latanoprost eye drop, and oral acetazolamide 500 mg three times daily. On slit-lamp examination, right eye showed corneal edema with Descemet membrane folding. There were anterior chamber reactions (cell 1+, flare 1+) with several fine keratic precipitates on the corneal epithelium. Both lenses showed nuclear sclerosis with anterior and posterior subcapsular cataract. There were no other remarkable findings in his left eye and the visual acuity was 0.7 (Snellen equivalent). The refractive error was − 0.50 and + 1.00 diopter sphere respectively. Although, dilated fundus exam was not clear due to the corneal haze, it seems there were few dot hemorrhages in both eyes (Fig. 1 ). The cup-to-disc ratio was 0.5 and 0.6 respectively. Optical coherence tomography (OCT) depicted hyperreflectivity around perifoveal area from inner plexiform to the outer plexiform layers in the right eye (Fig. 1 ). Small cystic spaces and hyperrelective retinal spots suggesting diabetic retinopathy were observed in his left eye. A general ophthalmologist prescribed 150 mL of 20% mannitol intravenously. After 1 hour of mannitol infusion, IOP in right eye was dropped to 19 mmHg. The patient was sent home changing his medication to the dorzolamide/timolol fixed combination (twice daily), brimonidine tartrate (twice daily), fluorometholone acetate (0.1%, four times daily), and oral acetazolamide (250 mg, twice daily). Two days later, the patient underwent a follow-up at the glaucoma clinic. The IOP of right eye was 31 mmHg, and there was a persistent corneal edema and Descemet membrane folding. Other findings were similar to those of previous visit. Because of the OCT finding suggesting ischemic damage on inner nuclear layer (INL), the patient was transferred to the retinal clinic at the same day. The retinal specialist diagnosed him with paracentral acute middle maculopathy (PAMM). Due to the persistent corneal edema, the fundus was still not visible well. The patient was prescribed maximal tolerated medical therapy (MTMT), and followed-up after 1 week. One week later, the IOP was 24 mmHg in the right eye, and the corneal edema was improved slightly. The ultrawide-field and fluorescein angiography (FA) were performed. A panretinal obstructive vasculopathy was observed on ultra-widefield FA except in the area around the optic disc (Fig. 2 ). The retinal specialist prescribed empiric acyclovir at 400 mg/day and conducted a blood sample test to evaluate potential infectious causes. Despite MTMT, the IOP was poorly controlled from 24 to 32 mmHg, and trabeculectomy was performed at 3 weeks from the first visit. After 1 week from the trabeculectomy, the IOP was dropped to 15 mmHg, and the clarity of cornea was improved. The retinal specialist noticed a white patch lesion with granular appearance at the temporal area (Fig. 3 ). Acyclovir was changed to valganciclovir, and diagnostic aqueous tap was performed. Three days later, the polymerase chain reaction (PCR) analysis was positive for CMV, and the fundus was more clearly visible through the improved cornea (Fig. 3 ). Intravitreal ganciclovir was administered. For 2 weeks, three times injection of ganciclovir were given. After 2 months, the granular retinitis was decreased. The retinal specialist recommended pars plana vitrectomy and phacoemulsification to apply panretinal photocoagulation, but the patient refused it due to economic reason. After 15 months, visual acuity of right eye was no light perception despite 4 months anti-viral therapy. The patient refused any surgical treatment except cataract surgery on the contralateral eye. The right eye showed a significant cataract, diffuse neovascularization on iris, and bullous keratopathy at final visit. Discussion and conclusions CMV retinitis typically occurs in immunocompromised patients. However, a distinct form of CMV retinitis with panretinal occlusive vasculopathy has been documented in partially immuno-dysregulated conditions (e.g., old age, diabetes mellitus) [ 6 – 8 ]. It is characterized by chronic progressive panretinal occlusive vasculopathy and referred to as chronic retinal necrosis [ 7 ]. While CMV retinitis with panretinal occlusive vasculopathy is a rare disease, it can be identified by a triad of features: granular retinitis, panretinal occlusive vasculopathy, and vitritis [ 7 ]. PSS is characterized by recurrent attack of non-granulomatous AU with significantly increased IOP [ 9 ]. Typical presentations include corneal edema with high IOP, anterior chamber inflammation, and keratic precipitates. It has been reported that 52.2% of patients diagnosed with PSS were found to be positive for CMV based on PCR [ 10 ]. Also, patients with clinically diagnosed PSS showed IOP stabilization during oral valganciclovir treatment [ 11 ]. Therefore, a significant proportion of the patients with PSS should be termed as CMV associated AU. Theoretically, CMV-associated AU and CMV retinitis are difficult to coexist, because they occur in different immunologic states. Meanwhile, the CMV retinitis with panretinal occlusive vasculopathy appears to be accompanied by mild AU [ 6 , 7 ]. However, cases with concurrent IOP elevation have not been reported previously. The current study likely the first case report presenting PSS in patients with CMV retinitis and panretinal occlusive vasculopathy. Granular appearance is one of the characteristic findings of typical CMV retinitis. It is more frequently observed in peripheral area compared to the hemorrhagic appearance [ 3 ]. While granular retinitis is also one of the characteristic features of CMV retinitis with panretinal occlusive vasculopathy, it was not initially identifiable in the current study because corneal edema reduced the contrast between the normal retina and the retinitis lesion. Thus, an alternative finding is needed to help recognize CMV retinitis. Light source of OCT emits longer wavelengths than visible ray, improving penetration through media opacities. [ 12 ] It can also achieve high axial resolution that enables to differentiate the individual retinal layers in the in vivo human eye. PAMM is an OCT-based finding described by Sarraf et al. [ 13 ] The hyper-reflective lesion around INL suggests an ischemic event affecting the intermediate and deep capillary plexus. This finding has been reported in various conditions causing arterial hypoperfusion and is often accompanied by retinal vascular disorders such as central retinal artery occlusion [ 14 – 17 ]. PAMM serves as an important clue in our case, as it represents the first abnormal finding detected in the posterior segment. Although not emphasized in previous studies, it appears that all cases undergoing OCT showed INL hyper-reflectivity as a result of occlusive vasculopathy (Table 1 ) [ 6 – 8 ]. Table 1 Summary of previous case reports describing cytomegalovirus retinitis with panretinal occlusive vasculopathy Case Age/sex Underlying disease Immunosuppressant Initial VA Final VA AC cell Vitritis/ POV/GR PAMM Schneider et al. [ 7 ] 1 74/M DM, CKD, Post KT 9 years MMF (750 mg twice daily) Tacrolimus 1 mg twice daily 20/30 HM 1+ +/+/+ NA* 2 83/F DM, HTN, CAD, anemia - HM HM 1+ +/+/+ NA* 3 48/M MM (post BM transplant) GVH, Steroid induced DM Corticosteroid tacrolimus 20/25 20/20 2–3+ +/+/+ NA* 4 78/M DM - 20/30 20/30 Trace +/+/+ NA* 5 72/M Polymyositis Prednisolone 5/200 20/40 2+ +/+/+ NA* Wang et al. [ 6 ] 1 71/M DM - 0.1 0.02 1+ +/+/+ + 2 66/M DM (poorly controlled) Subtenon triamcinolone per 2–3 months 0.02 HM Trace +/+/+ NA* 3 58/M MM, hematopoietic cell transplant Dexamethasone 0.1 0.3 1+ +/+/+ + Izzo et al. [ 8 ] 1 71/M Prostate cancer (no RT or chemotherapy) Sarcoidosis (no treatment) - FC Not described 1+ +/+/+ + *Description of optical coherence tomography was not provided. CAD, coronary artery disease; CKD, chronic kidney disease; DM, diabetes mellitus; FC, finger count; GR, granular retinitis; HM, hand movement; KT, kidney transplantation; MM, multiple myeloma; MMF, mycophenolate mofetil; NA, not applicable; PAMM, paracentral acute middle mac-ulopathy; POV, panretinal obstructive vasculopathy; RT, radiation therapy; VA, visual acuity Due to the rare clinical prevalence, delayed or inappropriate treatment is common in CMV retinitis with panretinal occlusive vasculopathy [ 7 ]. Early anti-viral therapy has been known to prevent the progression of the granular retinitis [ 6 ]. However, the occlusive vasculopathy has been reported to progress despite undergoing anti-viral treatment, leading to a fatal visual prognosis [ 6 , 7 ]. Local or systemic steroid after initiation of anti-viral therapy seems to improve occlusive vasculopathy in previous studies [ 6 , 7 ]. Therefore, early detection is important for the management of CMV retinitis with panretinal occlusive vasculopathy. As the OCT manifestation of occlusive vasculopathy was PAMM, awareness of the potential CMV infection is essential when the PAMM was observed in patients with PSS. In summary, the presence of PAMM in eyes with PSS could be a supportive clue indicating CMV retinitis in immunocompetent patients. It is more valuable when the corneal edema interferes with posterior segment visualization on fundus exam. When the PAMM was identified on OCT, empiric anti-viral treatments with or without steroid should be considered to prevent the deterioration of final visual acuity. Abbreviations AU anterior uveitis CMV cytomegalovirus FA fluorescein angiography INL inner nuclear layer IOP intraocular pressure MTMT maximal tolerated medical therapy OCT optical coherence tomography OPL outer plexiform layer PAMM paracentral acute middle maculopathy PCR polymerase chain reaction PSS Posner-Schlossman syndrome Declarations Acknowledgements Not applicable Authors' contributions S.J is a sole author responsible for the following: Design and concept of the work; drafted and revised the manuscript. S.J. has read and approved the final manuscript. Funding This study was supported by Yeungnam research grant (2022). Data availability The datasets used and/or analysed during the current study are available from the corresponding author on reasonable request. Ethics approval and consent to participate The Institutional Review Board of Yeungnam University Medical Center approved the study (No. 2024-01-019). Written informed consent was obtained from the patient for participation of this study. Consent for publication Written informed consent was obtained from the patient for participation and publication of this case report. Competing interests The authors declare that they have no competing interests. References Hirai K. [The mechanisms of human cytomegalovirus DNA replication]. Nihon Rinsho. 1998;56:36–43. Port AD, Orlin A, Kiss S, Patel S, D'Amico DJ, Gupta MP. Cytomegalovirus Retinitis: A Review. J Ocul Pharmacol Ther. 2017;33:224–34. Standardization of Uveitis Nomenclature Working G. Classification Criteria for Cytomegalovirus Retinitis. Am J Ophthalmol. 2021;228:245–54. Kempen JH, Min YI, Freeman WR, Holland GN, Friedberg DN, Dieterich DT, et al. Risk of immune recovery uveitis in patients with AIDS and cytomegalovirus retinitis. Ophthalmology. 2006;113:684–94. Ye Z, Yang Y, Ke W, Li Y, Wang K, Chen M. Overview and update on cytomegalovirus-associated anterior uveitis and glaucoma. Front Public Health. 2023;11:1117412. Wang I, Yang BC, Li KH, Wu JS, Chen SN. CMV Retinitis with Panretinal Occlusive Vasculitis. Ocul Immunol Inflamm. 2022:1–8. Schneider EW, Elner SG, van Kuijk FJ, Goldberg N, Lieberman RM, Eliott D, et al. Chronic retinal necrosis: cytomegalovirus necrotizing retinitis associated with panretinal vasculopathy in non-HIV patients. Retina. 2013;33:1791–9. Izzo MC, Mathai M, Do BK. Cytomegalovirus Retinitis Without Immunocompromise. Retina today. 2021:53 – 5. Megaw R, Agarwal PK. Posner-Schlossman syndrome. Surv Ophthalmol. 2017;62:277–85. Chee SP, Jap A. Presumed fuchs heterochromic iridocyclitis and Posner-Schlossman syndrome: comparison of cytomegalovirus-positive and negative eyes. Am J Ophthalmol. 2008;146:883–9. e1. Sobolewska B, Deuter C, Doycheva D, Zierhut M. Long-term oral therapy with valganciclovir in patients with Posner-Schlossman syndrome. Graefes Arch Clin Exp Ophthalmol. 2014;252:117–24. Aumann S, Donner S, Fischer J, Muller F. Optical Coherence Tomography (OCT): Principle and Technical Realization. In: High Resolution Imaging in Microscopy and Ophthalmology: New Frontiers in Biomedical Optics. edn. Edited by Bille JF. Cham (CH): Springer International Publishing; 2019: 59–85. Sarraf D, Rahimy E, Fawzi AA, Sohn E, Barbazetto I, Zacks DN, et al. Paracentral acute middle maculopathy: a new variant of acute macular neuroretinopathy associated with retinal capillary ischemia. JAMA Ophthalmol. 2013;131:1275–87. Maltsev DS, Kulikov AN, Burnasheva MA, Chhablani J. Prevalence of resolved paracentral acute middle maculopathy lesions in fellow eyes of patients with unilateral retinal vein occlusion. Acta Ophthalmol. 2020;98:e22–8. Liang S, Chen Q, Hu C, Chen M. Association of Paracentral Acute Middle Maculopathy with Visual Prognosis in Retinal Artery Occlusion: A Retrospective Cohort Study. J Ophthalmol. 2022;2022:9404973. Louie E, Tang A, King B. Paracentral acute middle maculopathy presenting as a sign of impending central retinal artery occlusion: a case report. BMC Ophthalmol. 2023;23:268. Ilginis T, Keane PA, Tufail A. Paracentral acute middle maculopathy in sickle cell disease. JAMA Ophthalmol. 2015;133:614–6. Additional Declarations No competing interests reported. Cite Share Download PDF Status: Posted Version 1 posted You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. As a division of Research Square Company, we’re committed to making research communication faster, fairer, and more useful. We do this by developing innovative software and high quality services for the global research community. Our growing team is made up of researchers and industry professionals working together to solve the most critical problems facing scientific publishing. Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-4261984","acceptedTermsAndConditions":true,"allowDirectSubmit":true,"archivedVersions":[],"articleType":"Case Report","associatedPublications":[],"authors":[{"id":292101606,"identity":"aac05b47-3bec-494b-946d-673e9b35ee31","order_by":0,"name":"Seongyong Jeong","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAABCklEQVRIiWNgGAWjYJCDBBAhh8whTosxSVrAILGBkBb59uZjD7+22eTJOzA8fPBxR236hhsJjB9+MKTl49JicOZYurFsW1qx4QGGZMOZZ47nArUwS/Yw5Fg24NIikWMmLdl2OHFjA0OaNG/bMZAWBmkGhgoDnA6b/wauJf03UEu6AdCW3/i0MNzgMZP8CNQyn4EhjZm3rSYBqIUNaEsOTi0GZ9LSpBnOpSVuYGZIlpzZdgDon4dtlj0Gabgd1n74mOSPMpvE+e09iR8+ttXJ8x1PPnzjR0UybocBATMvG9C6wzwJQPZhBoUDjA1ALj4NDAyMP/4ArWtgPwBk1wEZ+FWPglEwCkbByAMA5gJbCUFnR4wAAAAASUVORK5CYII=","orcid":"","institution":"Yeungnam University College of Medicine","correspondingAuthor":true,"prefix":"","firstName":"Seongyong","middleName":"","lastName":"Jeong","suffix":""}],"badges":[],"createdAt":"2024-04-13 13:46:21","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-4261984/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-4261984/v1","draftVersion":[],"editorialEvents":[],"editorialNote":"","failedWorkflow":false,"files":[{"id":55251972,"identity":"e2860bb3-8c3f-466c-b201-d6514e2bcd47","added_by":"auto","created_at":"2024-04-24 17:44:58","extension":"png","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":692815,"visible":true,"origin":"","legend":"\u003cp\u003eFundus photography (a) and OCT (\u003cstrong\u003eb\u003c/strong\u003e) at initial presentation. Corneal edema hindered imaging in the right eye. OCT showed a hyperreflective band-like lesion in the IPL, INL, and OPL. INL, inner nuclear layer; IPL, inner plexiform layer; OCT, optical coherence tomography; OPL, outer plexiform layer.\u003c/p\u003e","description":"","filename":"Fig1.png","url":"https://assets-eu.researchsquare.com/files/rs-4261984/v1/ea102895c3ecd139d89f552a.png"},{"id":55251971,"identity":"24a0c8aa-7454-4f29-9792-b68989b43965","added_by":"auto","created_at":"2024-04-24 17:44:58","extension":"png","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":555675,"visible":true,"origin":"","legend":"\u003cp\u003eUltra-wide field fluorescein angiography after 1 week. The right eye shows panretinal vascular occlusion, although it was not clearly visible due to persistent corneal edema.\u003c/p\u003e","description":"","filename":"Fig22.png","url":"https://assets-eu.researchsquare.com/files/rs-4261984/v1/ef0aa512ca48c14427238268.png"},{"id":55251973,"identity":"5e6d5099-b883-4a70-ba86-da94f99205a5","added_by":"auto","created_at":"2024-04-24 17:44:59","extension":"png","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":978620,"visible":true,"origin":"","legend":"\u003cp\u003eUltra-wide field image after trabeculectomy. White lesion with granular appearance was observed through improved corneal edema (\u003cstrong\u003ea\u003c/strong\u003e). Three days later, the fundus was more clearly visible showing the characteristic granular appearance of cytomegalovirus retinitis (\u003cstrong\u003eb\u003c/strong\u003e).\u003c/p\u003e","description":"","filename":"Fig3.png","url":"https://assets-eu.researchsquare.com/files/rs-4261984/v1/1f27c47b8a643ef04338fa27.png"},{"id":56918380,"identity":"e87d3df1-e1fb-4871-aa40-5d0406cdc628","added_by":"auto","created_at":"2024-05-22 06:56:14","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":3066276,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-4261984/v1/ff8ca942-8dbc-483a-bb82-a4a9173654fc.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"Cytomegalovirus retinitis with panretinal occlusive vasculopathy concealed by Posner– Schlossman syndrome: a case report","fulltext":[{"header":"Background","content":"\u003cp\u003eCytomegalovirus (CMV) belongs to the herpes virus family with double-stranded DNA [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e]. It can affect both the anterior and posterior segment of eye with a wide range of clinical manifestation [\u003cspan additionalcitationids=\"CR3\" citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e]. CMV-associated anterior uveitis (AU) usually occurs in the eyes of immunocompetent patients [\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e]. In contrast, typical CMV retinitis is an opportunistic infection found in immunocompromised hosts such as patients infected with human immunodeficiency virus. Therefore, the coexistence of these two diseases in a single individual is uncommon. We report a case that initially presented as Posner-Schlossman syndrome (PSS) and was later revealed to have CMV retinitis with panretinal occlusive vasculopathy.\u003c/p\u003e"},{"header":"Case presentation","content":"\u003cp\u003eA 72-year-old man was referred to the tertiary hospital with uncontrolled intraocular pressure (IOP) in his right eye. He had diabetes mellitus under control with medication for 20 years. There was no other remarkable medical history. Visual acuity was hand motion in the right eye. The IOP of right eye was 36 mmHg despite IOP-lowering treatment including latanoprost eye drop, and oral acetazolamide 500 mg three times daily. On slit-lamp examination, right eye showed corneal edema with Descemet membrane folding. There were anterior chamber reactions (cell 1+, flare 1+) with several fine keratic precipitates on the corneal epithelium. Both lenses showed nuclear sclerosis with anterior and posterior subcapsular cataract. There were no other remarkable findings in his left eye and the visual acuity was 0.7 (Snellen equivalent). The refractive error was \u0026minus;\u0026thinsp;0.50 and +\u0026thinsp;1.00 diopter sphere respectively. Although, dilated fundus exam was not clear due to the corneal haze, it seems there were few dot hemorrhages in both eyes (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003e). The cup-to-disc ratio was 0.5 and 0.6 respectively. Optical coherence tomography (OCT) depicted hyperreflectivity around perifoveal area from inner plexiform to the outer plexiform layers in the right eye (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003e). Small cystic spaces and hyperrelective retinal spots suggesting diabetic retinopathy were observed in his left eye. A general ophthalmologist prescribed 150 mL of 20% mannitol intravenously. After 1 hour of mannitol infusion, IOP in right eye was dropped to 19 mmHg. The patient was sent home changing his medication to the dorzolamide/timolol fixed combination (twice daily), brimonidine tartrate (twice daily), fluorometholone acetate (0.1%, four times daily), and oral acetazolamide (250 mg, twice daily).\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003eTwo days later, the patient underwent a follow-up at the glaucoma clinic. The IOP of right eye was 31 mmHg, and there was a persistent corneal edema and Descemet membrane folding. Other findings were similar to those of previous visit. Because of the OCT finding suggesting ischemic damage on inner nuclear layer (INL), the patient was transferred to the retinal clinic at the same day. The retinal specialist diagnosed him with paracentral acute middle maculopathy (PAMM). Due to the persistent corneal edema, the fundus was still not visible well. The patient was prescribed maximal tolerated medical therapy (MTMT), and followed-up after 1 week.\u003c/p\u003e \u003cp\u003eOne week later, the IOP was 24 mmHg in the right eye, and the corneal edema was improved slightly. The ultrawide-field and fluorescein angiography (FA) were performed. A panretinal obstructive vasculopathy was observed on ultra-widefield FA except in the area around the optic disc (Fig.\u0026nbsp;\u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e2\u003c/span\u003e). The retinal specialist prescribed empiric acyclovir at 400 mg/day and conducted a blood sample test to evaluate potential infectious causes. Despite MTMT, the IOP was poorly controlled from 24 to 32 mmHg, and trabeculectomy was performed at 3 weeks from the first visit.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003eAfter 1 week from the trabeculectomy, the IOP was dropped to 15 mmHg, and the clarity of cornea was improved. The retinal specialist noticed a white patch lesion with granular appearance at the temporal area (Fig.\u0026nbsp;\u003cspan refid=\"Fig3\" class=\"InternalRef\"\u003e3\u003c/span\u003e). Acyclovir was changed to valganciclovir, and diagnostic aqueous tap was performed. Three days later, the polymerase chain reaction (PCR) analysis was positive for CMV, and the fundus was more clearly visible through the improved cornea (Fig.\u0026nbsp;\u003cspan refid=\"Fig3\" class=\"InternalRef\"\u003e3\u003c/span\u003e). Intravitreal ganciclovir was administered. For 2 weeks, three times injection of ganciclovir were given. After 2 months, the granular retinitis was decreased. The retinal specialist recommended pars plana vitrectomy and phacoemulsification to apply panretinal photocoagulation, but the patient refused it due to economic reason. After 15 months, visual acuity of right eye was no light perception despite 4 months anti-viral therapy. The patient refused any surgical treatment except cataract surgery on the contralateral eye. The right eye showed a significant cataract, diffuse neovascularization on iris, and bullous keratopathy at final visit.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e"},{"header":"Discussion and conclusions","content":"\u003cp\u003eCMV retinitis typically occurs in immunocompromised patients. However, a distinct form of CMV retinitis with panretinal occlusive vasculopathy has been documented in partially immuno-dysregulated conditions (e.g., old age, diabetes mellitus) [\u003cspan additionalcitationids=\"CR7\" citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e]. It is characterized by chronic progressive panretinal occlusive vasculopathy and referred to as chronic retinal necrosis [\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e]. While CMV retinitis with panretinal occlusive vasculopathy is a rare disease, it can be identified by a triad of features: granular retinitis, panretinal occlusive vasculopathy, and vitritis [\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e].\u003c/p\u003e \u003cp\u003ePSS is characterized by recurrent attack of non-granulomatous AU with significantly increased IOP [\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e]. Typical presentations include corneal edema with high IOP, anterior chamber inflammation, and keratic precipitates. It has been reported that 52.2% of patients diagnosed with PSS were found to be positive for CMV based on PCR [\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e]. Also, patients with clinically diagnosed PSS showed IOP stabilization during oral valganciclovir treatment [\u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e]. Therefore, a significant proportion of the patients with PSS should be termed as CMV associated AU.\u003c/p\u003e \u003cp\u003eTheoretically, CMV-associated AU and CMV retinitis are difficult to coexist, because they occur in different immunologic states. Meanwhile, the CMV retinitis with panretinal occlusive vasculopathy appears to be accompanied by mild AU [\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e, \u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e]. However, cases with concurrent IOP elevation have not been reported previously. The current study likely the first case report presenting PSS in patients with CMV retinitis and panretinal occlusive vasculopathy.\u003c/p\u003e \u003cp\u003eGranular appearance is one of the characteristic findings of typical CMV retinitis. It is more frequently observed in peripheral area compared to the hemorrhagic appearance [\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e]. While granular retinitis is also one of the characteristic features of CMV retinitis with panretinal occlusive vasculopathy, it was not initially identifiable in the current study because corneal edema reduced the contrast between the normal retina and the retinitis lesion. Thus, an alternative finding is needed to help recognize CMV retinitis.\u003c/p\u003e \u003cp\u003eLight source of OCT emits longer wavelengths than visible ray, improving penetration through media opacities. [\u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e] It can also achieve high axial resolution that enables to differentiate the individual retinal layers in the in vivo human eye. PAMM is an OCT-based finding described by Sarraf et al. [\u003cspan citationid=\"CR13\" class=\"CitationRef\"\u003e13\u003c/span\u003e] The hyper-reflective lesion around INL suggests an ischemic event affecting the intermediate and deep capillary plexus. This finding has been reported in various conditions causing arterial hypoperfusion and is often accompanied by retinal vascular disorders such as central retinal artery occlusion [\u003cspan additionalcitationids=\"CR15 CR16\" citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR17\" class=\"CitationRef\"\u003e17\u003c/span\u003e]. PAMM serves as an important clue in our case, as it represents the first abnormal finding detected in the posterior segment. Although not emphasized in previous studies, it appears that all cases undergoing OCT showed INL hyper-reflectivity as a result of occlusive vasculopathy (Table\u0026nbsp;\u003cspan refid=\"Tab1\" class=\"InternalRef\"\u003e1\u003c/span\u003e) [\u003cspan additionalcitationids=\"CR7\" citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e].\u003c/p\u003e \u003cp\u003e \u003cdiv class=\"gridtable\"\u003e\u003ctable float=\"Yes\" id=\"Tab1\" border=\"1\"\u003e \u003ccaption language=\"En\"\u003e \u003cdiv class=\"CaptionNumber\"\u003eTable 1\u003c/div\u003e \u003cdiv class=\"CaptionContent\"\u003e \u003cp\u003eSummary of previous case reports describing cytomegalovirus retinitis with panretinal occlusive vasculopathy\u003c/p\u003e \u003c/div\u003e \u003c/caption\u003e \u003ccolgroup cols=\"10\"\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c1\" colnum=\"1\"\u003e\u003c/div\u003e \u003cdiv align=\"char\" char=\".\" class=\"colspec\" colname=\"c2\" colnum=\"2\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c3\" colnum=\"3\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c4\" colnum=\"4\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c5\" colnum=\"5\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c6\" colnum=\"6\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c7\" colnum=\"7\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c8\" colnum=\"8\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c9\" colnum=\"9\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c10\" colnum=\"10\"\u003e\u003c/div\u003e \u003cthead\u003e \u003ctr\u003e \u003cth align=\"left\" colname=\"c1\"\u003e\u0026nbsp;\u003c/th\u003e \u003cth align=\"left\" colname=\"c2\"\u003e \u003cp\u003eCase\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c3\"\u003e \u003cp\u003eAge/sex\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c4\"\u003e \u003cp\u003eUnderlying disease\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c5\"\u003e \u003cp\u003eImmunosuppressant\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c6\"\u003e \u003cp\u003eInitial VA\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c7\"\u003e \u003cp\u003eFinal VA\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c8\"\u003e \u003cp\u003eAC cell\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c9\"\u003e \u003cp\u003eVitritis/ POV/GR\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c10\"\u003e \u003cp\u003ePAMM\u003c/p\u003e \u003c/th\u003e \u003c/tr\u003e \u003c/thead\u003e \u003ctbody\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\" morerows=\"4\" rowspan=\"5\"\u003e \u003cp\u003eSchneider et al. [\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e \u003cp\u003e1\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e74/M\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eDM, CKD, Post KT 9 years\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eMMF (750 mg twice daily) Tacrolimus 1 mg twice daily\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e20/30\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003eHM\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c8\"\u003e \u003cp\u003e1+\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c9\"\u003e \u003cp\u003e+/+/+\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c10\"\u003e \u003cp\u003eNA*\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e \u003cp\u003e2\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e83/F\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eDM, HTN, CAD, anemia\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e-\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eHM\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003eHM\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c8\"\u003e \u003cp\u003e1+\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c9\"\u003e \u003cp\u003e+/+/+\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c10\"\u003e \u003cp\u003eNA*\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e \u003cp\u003e3\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e48/M\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eMM (post BM transplant)\u003c/p\u003e \u003cp\u003eGVH, Steroid induced DM\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eCorticosteroid tacrolimus\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e20/25\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e20/20\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c8\"\u003e \u003cp\u003e2\u0026ndash;3+\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c9\"\u003e \u003cp\u003e+/+/+\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c10\"\u003e \u003cp\u003eNA*\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e \u003cp\u003e4\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e78/M\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eDM\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e-\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e20/30\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e20/30\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c8\"\u003e \u003cp\u003eTrace\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c9\"\u003e \u003cp\u003e+/+/+\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c10\"\u003e \u003cp\u003eNA*\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e \u003cp\u003e5\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e72/M\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003ePolymyositis\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003ePrednisolone\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e5/200\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e20/40\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c8\"\u003e \u003cp\u003e2+\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c9\"\u003e \u003cp\u003e+/+/+\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c10\"\u003e \u003cp\u003eNA*\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\" morerows=\"2\" rowspan=\"3\"\u003e \u003cp\u003eWang et al. [\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e \u003cp\u003e1\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e71/M\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eDM\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e-\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.1\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e0.02\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c8\"\u003e \u003cp\u003e1+\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c9\"\u003e \u003cp\u003e+/+/+\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c10\"\u003e \u003cp\u003e+\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e \u003cp\u003e2\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e66/M\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eDM (poorly controlled)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eSubtenon triamcinolone per 2\u0026ndash;3 months\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.02\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003eHM\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c8\"\u003e \u003cp\u003eTrace\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c9\"\u003e \u003cp\u003e+/+/+\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c10\"\u003e \u003cp\u003eNA*\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e \u003cp\u003e3\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e58/M\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eMM, hematopoietic cell transplant\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eDexamethasone\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.1\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e0.3\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c8\"\u003e \u003cp\u003e1+\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c9\"\u003e \u003cp\u003e+/+/+\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c10\"\u003e \u003cp\u003e+\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eIzzo et al. [\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e \u003cp\u003e1\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e71/M\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eProstate cancer (no RT or chemotherapy)\u003c/p\u003e \u003cp\u003eSarcoidosis (no treatment)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e-\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eFC\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003eNot described\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c8\"\u003e \u003cp\u003e1+\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c9\"\u003e \u003cp\u003e+/+/+\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c10\"\u003e \u003cp\u003e+\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003c/tbody\u003e \u003c/colgroup\u003e \u003ctfoot\u003e \u003ctr\u003e\u003ctd colspan=\"10\"\u003e*Description of optical coherence tomography was not provided.\u003c/td\u003e\u003c/tr\u003e \u003ctr\u003e\u003ctd colspan=\"10\"\u003eCAD, coronary artery disease; CKD, chronic kidney disease; DM, diabetes mellitus; FC, finger count; GR, granular retinitis; HM, hand movement; KT, kidney transplantation; MM, multiple myeloma; MMF, mycophenolate mofetil; NA, not applicable; PAMM, paracentral acute middle mac-ulopathy; POV, panretinal obstructive vasculopathy; RT, radiation therapy; VA, visual acuity\u003c/td\u003e\u003c/tr\u003e \u003c/tfoot\u003e \u003c/table\u003e\u003c/div\u003e \u003c/p\u003e \u003cp\u003eDue to the rare clinical prevalence, delayed or inappropriate treatment is common in CMV retinitis with panretinal occlusive vasculopathy [\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e]. Early anti-viral therapy has been known to prevent the progression of the granular retinitis [\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e]. However, the occlusive vasculopathy has been reported to progress despite undergoing anti-viral treatment, leading to a fatal visual prognosis [\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e, \u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e]. Local or systemic steroid after initiation of anti-viral therapy seems to improve occlusive vasculopathy in previous studies [\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e, \u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e]. Therefore, early detection is important for the management of CMV retinitis with panretinal occlusive vasculopathy. As the OCT manifestation of occlusive vasculopathy was PAMM, awareness of the potential CMV infection is essential when the PAMM was observed in patients with PSS.\u003c/p\u003e \u003cp\u003eIn summary, the presence of PAMM in eyes with PSS could be a supportive clue indicating CMV retinitis in immunocompetent patients. It is more valuable when the corneal edema interferes with posterior segment visualization on fundus exam. When the PAMM was identified on OCT, empiric anti-viral treatments with or without steroid should be considered to prevent the deterioration of final visual acuity.\u003c/p\u003e"},{"header":"Abbreviations","content":" \u003cp\u003eAU anterior uveitis\u003c/p\u003e \u003cp\u003eCMV cytomegalovirus\u003c/p\u003e \u003cp\u003eFA fluorescein angiography\u003c/p\u003e \u003cp\u003eINL inner nuclear layer\u003c/p\u003e \u003cp\u003eIOP intraocular pressure\u003c/p\u003e \u003cp\u003eMTMT maximal tolerated medical therapy\u003c/p\u003e \u003cp\u003eOCT optical coherence tomography\u003c/p\u003e \u003cp\u003eOPL outer plexiform layer\u003c/p\u003e \u003cp\u003ePAMM paracentral acute middle maculopathy\u003c/p\u003e \u003cp\u003ePCR polymerase chain reaction\u003c/p\u003e \u003cp\u003ePSS Posner-Schlossman syndrome\u003c/p\u003e "},{"header":"Declarations","content":"\u003cp\u003e\u003cstrong\u003eAcknowledgements\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eNot applicable\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAuthors\u0026apos; contributions\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eS.J is a sole author responsible for the following: Design and concept of the work; drafted and revised the manuscript. S.J. has read and approved the final manuscript.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eFunding\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThis study was supported by Yeungnam research grant (2022).\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eData availability\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe datasets used and/or analysed during the current study are available from the corresponding author on reasonable request.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eEthics approval and consent to participate\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe Institutional Review Board of Yeungnam University\u0026nbsp;Medical Center approved the study (No. 2024-01-019). Written informed consent was obtained from the patient for participation of this study.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConsent for publication\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eWritten informed consent was obtained from the patient for participation and publication of this case report.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eCompeting\u003c/strong\u003e \u003cstrong\u003einterests\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe authors declare that they have no competing interests.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\u003cli\u003e\u003cspan\u003eHirai K. [The mechanisms of human cytomegalovirus DNA replication]. Nihon Rinsho. 1998;56:36\u0026ndash;43.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003ePort AD, Orlin A, Kiss S, Patel S, D'Amico DJ, Gupta MP. Cytomegalovirus Retinitis: A Review. J Ocul Pharmacol Ther. 2017;33:224\u0026ndash;34.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eStandardization of Uveitis Nomenclature Working G. Classification Criteria for Cytomegalovirus Retinitis. Am J Ophthalmol. 2021;228:245\u0026ndash;54.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eKempen JH, Min YI, Freeman WR, Holland GN, Friedberg DN, Dieterich DT, et al. Risk of immune recovery uveitis in patients with AIDS and cytomegalovirus retinitis. Ophthalmology. 2006;113:684\u0026ndash;94.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eYe Z, Yang Y, Ke W, Li Y, Wang K, Chen M. Overview and update on cytomegalovirus-associated anterior uveitis and glaucoma. Front Public Health. 2023;11:1117412.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eWang I, Yang BC, Li KH, Wu JS, Chen SN. CMV Retinitis with Panretinal Occlusive Vasculitis. Ocul Immunol Inflamm. 2022:1\u0026ndash;8.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eSchneider EW, Elner SG, van Kuijk FJ, Goldberg N, Lieberman RM, Eliott D, et al. Chronic retinal necrosis: cytomegalovirus necrotizing retinitis associated with panretinal vasculopathy in non-HIV patients. Retina. 2013;33:1791\u0026ndash;9.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eIzzo MC, Mathai M, Do BK. Cytomegalovirus Retinitis Without Immunocompromise. Retina today. 2021:53\u0026thinsp;\u0026ndash;\u0026thinsp;5.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eMegaw R, Agarwal PK. Posner-Schlossman syndrome. Surv Ophthalmol. 2017;62:277\u0026ndash;85.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eChee SP, Jap A. Presumed fuchs heterochromic iridocyclitis and Posner-Schlossman syndrome: comparison of cytomegalovirus-positive and negative eyes. Am J Ophthalmol. 2008;146:883\u0026ndash;9. e1.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eSobolewska B, Deuter C, Doycheva D, Zierhut M. Long-term oral therapy with valganciclovir in patients with Posner-Schlossman syndrome. Graefes Arch Clin Exp Ophthalmol. 2014;252:117\u0026ndash;24.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eAumann S, Donner S, Fischer J, Muller F. Optical Coherence Tomography (OCT): Principle and Technical Realization. In: High Resolution Imaging in Microscopy and Ophthalmology: New Frontiers in Biomedical Optics. edn. Edited by Bille JF. Cham (CH): Springer International Publishing; 2019: 59\u0026ndash;85.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eSarraf D, Rahimy E, Fawzi AA, Sohn E, Barbazetto I, Zacks DN, et al. Paracentral acute middle maculopathy: a new variant of acute macular neuroretinopathy associated with retinal capillary ischemia. JAMA Ophthalmol. 2013;131:1275\u0026ndash;87.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eMaltsev DS, Kulikov AN, Burnasheva MA, Chhablani J. Prevalence of resolved paracentral acute middle maculopathy lesions in fellow eyes of patients with unilateral retinal vein occlusion. Acta Ophthalmol. 2020;98:e22\u0026ndash;8.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eLiang S, Chen Q, Hu C, Chen M. Association of Paracentral Acute Middle Maculopathy with Visual Prognosis in Retinal Artery Occlusion: A Retrospective Cohort Study. J Ophthalmol. 2022;2022:9404973.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eLouie E, Tang A, King B. Paracentral acute middle maculopathy presenting as a sign of impending central retinal artery occlusion: a case report. BMC Ophthalmol. 2023;23:268.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eIlginis T, Keane PA, Tufail A. Paracentral acute middle maculopathy in sickle cell disease. JAMA Ophthalmol. 2015;133:614\u0026ndash;6.\u003c/span\u003e\u003c/li\u003e\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":true,"highlight":"","institution":"","isAcceptedByJournal":false,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true},"keywords":"Cytomegalovirus retinitis, Ganciclovir, Panretinal occlusive vasculopathy, Paracentral acute middle maculopathy, Posner–Schlossman syndrome","lastPublishedDoi":"10.21203/rs.3.rs-4261984/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-4261984/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003ch2\u003eBackground\u003c/h2\u003e \u003cp\u003eCytomegalovirus (CMV) retinitis is a rare disease, and its overlapping manifestation involving anterior segment is extremely uncommon. We report a case that initially presented Posner-Schlossman syndrome (PSS) and was later diagnosed with CMV retinitis.\u003c/p\u003e\u003ch2\u003eCase presentation\u003c/h2\u003e \u003cp\u003eA 72-year-old man visited tertiary hospital with uncontrolled intraocular pressure (IOP) in his right eye. At initial presentation, IOP was 36 mmHg and the fundus was not clear due to corneal edema. The spectral-domain optical coherence tomography revealed paracentral acute middle maculopathy (PAMM). A panretinal obstructive vasculopathy was observed on ultra-widefield fluorescein angiography. Three weeks later, trabeculectomy was performed to resolve the persistent high IOP. Once the cornea edema improved, a white patch-like peripheral lesion and silver wire-like retinal vasculature were observed. Aqueous tapping for polymerase chain reaction was positive for CMV. Oral valganciclovir and intravitreal ganciclovir were administered as anti-viral therapy. Despite treatment for four months, the final visual acuity was no light perception with bullous keratopathy and neovascularization on iris.\u003c/p\u003e\u003ch2\u003eConclusions\u003c/h2\u003e \u003cp\u003eWe described a rare case of simultaneous occurrence of PSS and CMV retinitis. The presence of PAMM could be an initial identifiable sign for CMV retinitis, even when media opacity is present.\u003c/p\u003e","manuscriptTitle":"Cytomegalovirus retinitis with panretinal occlusive vasculopathy concealed by Posner– Schlossman syndrome: a case report","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2024-04-24 17:44:54","doi":"10.21203/rs.3.rs-4261984/v1","editorialEvents":[{"type":"communityComments","content":0}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"534808d6-1b48-43a8-86aa-85ff06db0036","owner":[],"postedDate":"April 24th, 2024","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[],"tags":[],"updatedAt":"2024-05-22T06:48:06+00:00","versionOfRecord":[],"versionCreatedAt":"2024-04-24 17:44:54","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-4261984","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-4261984","identity":"rs-4261984","version":["v1"]},"buildId":"qtupq5eGEP_6zYnWcrvyt","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}