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 Table of Contents  
Year : 2022  |  Volume : 2  |  Issue : 1  |  Page : 46-48

An atypical case of viral endotheliitis

Department of Ophthalmology, Sarojini Devi Eye Hospital, Osmania Medical College, Hyderabad, Telangana, India

Date of Submission09-Jun-2021
Date of Acceptance26-Aug-2021
Date of Web Publication07-Jan-2022

Correspondence Address:
Dr. K Srinivas Prasad
Department of Ophthalmology, Sarojini Devi Eye Hospital, Osmania Medical College, Hyderabad, Telangana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_1601_21

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A 48-eight-year-old male presented with redness and blurred vision in his right eye for the past 10 days, sudden in onset, with circumciliary congestion and corneal edema. On examination localized disciform corneal edema, with multiple keratic precipitates, was seen and diagnosed as corneal endotheliitis. Specular microscopy showed endothelial morphological changes with pseudoguttae. Anterior chamber paracentesis subjected to polymerase chain reaction detected varicella zoster virus. He was treated with topical 3% acyclovir gel, homatropine eye drops, 1% prednisolone acetate, and 0.5% timolol maleate. Prednisolone acetate was tapered over 14 weeks. His visual acuity at presentation was 6/36, which improved to 6/9 after 4 weeks of treatment and the final visual acuity was 6/6.

Keywords: Disciform keratitis, endotheliitis, polymerase chain reaction, specular microscopy, varicella zoster virus, viral keratitis

How to cite this article:
Prasad K S, Hansraj S. An atypical case of viral endotheliitis. Indian J Ophthalmol Case Rep 2022;2:46-8

How to cite this URL:
Prasad K S, Hansraj S. An atypical case of viral endotheliitis. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2023 Feb 7];2:46-8. Available from: https://www.ijoreports.in/text.asp?2022/2/1/46/334891

Endotheliitis, defined as inflammation of the corneal endothelium, classically consists of corneal edema accompanied by keratic precipitates (KP). The most common symptoms include eye pain, photophobia, and visual disturbances. Occasionally, there may be circumciliary congestion and neovascularization. It is often complicated by coexisting or superimposed uveitis. It may be considered a form of anterior uveitis (AU) localized to the endothelium.

Endotheliitis is most commonly caused by cytomegalovirus (CMV) and herpes simplex virus (HSV). Less common causes include varicella zoster virus (VZV), Epstein-Barr virus, and coxsackie virus. Nonviral causes are uncommon that include postcollagen cross linking, endothelial graft rejection, retinoblastoma, or it may be idiopathic.

Polymerase chain reaction (PCR) analysis of anterior chamber aqueous humor is the gold standard method to identify the viral etiology of endotheliitis.[1]

Antiviral treatment in combination with topical corticosteroids is generally effective to suppress the inflammation; however, irreversible corneal endothelial dysfunction may develop in some cases.[2]

  Case Report Top

A 48-year-old male presented with redness, photophobia, and blurred vision in his right eye (OD) for the past 10 days, sudden in onset and taking oral acetazolamide prescribed elsewhere. He had been referred to us to evaluate for acute angle closure glaucoma. He had no history of any prior ocular complaints. There was no history of ocular trauma. He was a known diabetic for the past 5 years and on tablet metformin.

On the day of presentation, OD best-corrected visual acuity (BCVA) was 6/36. He had circumciliary congestion and corneal edema. The edema was paracentral; full thickness, with descemet membrane folds and micro epithelial bullae was seen. His intraocular pressure (IOP) on Goldmann applanation tonometer was 16 mm Hg. He was prescribed topical corticosteroids and cycloplegic on suspicion of acute AU. On follow-up after 4 days, the corneal edema had reduced, was localized, disciform in shape, inferior paracentral, 3 mm × 3 mm, with multiple small- and medium-sized, nonpigmented KPs on the endothelium. IOP was 24 mm Hg. Retinal examination revealed moderate nonproliferative diabetic retinopathy in both the eyes. Differential diagnosis of viral endotheliitis or acute relapsing hypertensive AU (Posner–Schlossman syndrome) was considered [Figure 1].
Figure 1: (a) Circumciliary congestion and paracentral, full thickness corneal edema with descemet membrane folds and micro epithelial bullae seen on the first day of presentation. (b) After 4 days of treatment with topical steroids, inferior paracentral disciform corneal edema seen, 3 mm × 3 mm, with multiple small- and medium-sized, nonpigmented KPs on the endothelium

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Due to the uncertain nature of the causative organism, we decided to do an anterior chamber paracentesis, subjected to PCR, which was positive for VZV. Specular microscopy (OD) showed endothelial cell morphological changes and pseudoguttae [Figure 2]. Despite repeated questioning at this point, the patient denied any vesicular eruptions of the skin. He did not have any history of chickenpox or prior similar ocular episodes.
Figure 2: Specular microscopy of the central cornea: (a) Prior to treatment, there are pseudoguttae, endothelial morphological changes. The coefficient of variation was 0.44, cell density was 1555 cells/mm2, and an average cell size was 643 μm2. (b) One month after treatment pseudoguttae were much reduced, and endothelial recovery had started. This was corroborated by the increase in coefficient of variation to 0.53 and increase in average cell size to 737 μm2, indicating polymegathism and pleomorphism. However, decrease in cell density to 1357 cells/mm2 was also seen due to cell loss

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Our patient had an HbA1c of 10%. His hematological, renal, and hepatic parameters were normal. He was negative for SARS-CoV2.

Due to the localized nature of the disease, first episode, chronic diabetes, risk of renal dysfunction with oral acyclovir, inability to pay, as well as poor compliance with his diabetic treatment; we decided to treat him with topical 3% acyclovir ointment five times per day, topical 1% prednisolone acetate six times per day, and 0.5% timolol maleate twice a day. Corticosteroids were gradually tapered under the cover of acyclovir over 14 weeks. Throughout the follow-up process, IOP was maintained within normal limits. Serial gonioscopy revealed open angles.

Our patient showed improvement in VA. After 4 weeks, BCVA improved to 6/9, with markedly reduced edema. Pigmented and non-pigmented KPs were present. At 14 weeks, his BCVA was 6/6. There was slight corneal haze, no edema, and few old pigmented KPs. At this point, we stopped all medications and asked him to return for monthly follow-ups [Figure 3].
Figure 3: (a) Two weeks after antiviral treatment. Edema has reduced. Both pigmented and nonpigmented KPs were present. BCVA 6/18. (b) Four weeks posttreatment edema had further reduced. KPs seen. BCVA 6/9. (c) At the end of treatment at 14 weeks, corneal edema is minimal, with slight corneal haze. Old KPs still seen. BCVA 6/6

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  Discussion Top

VZV is one of the less common causative organisms of endotheliitis.[1] In the study conducted by Miyazaki et al.[3] of all cases of endotheliitis, VZV was responsible for just 2.9% of affected eyes. CMV and HSV affected 34.8 and 20.3% eyes, respectively. Few case reports of VZV endotheliitis are available.[4] Ocular involvement occurs secondary to involvement of the ophthalmic division of the trigeminal nerve by VZV reactivation, which causes vesicular eruptions of the skin and affects the eye in approximately 50–72% of cases.[5] However, in our patient, there was no cutaneous involvement. Indeed, the phenomenon of Zoster sine herpete, or neuralgia in the absence of any visible skin lesions, was also not present.

In the study conducted by Qian Z, et al.[6] only 34.4% patients with VZV-AU had any form of skin involvement. Thus, if we assume endotheliitis to be a form of AU, where the major site of inflammation is the corneal endothelium, it is important to note that VZV-endotheliitis need not have skin lesions. Most authors agree that VZV causes a more severe AU than HSV.[7] In the study by Qian Z, et al. all patients of VZV-AU had either iris atrophy, raised IOP, synechiae, or vitritis.[6] However, besides raised IOP most likely due to concurrent trabeculitis, our patient had no other ocular feature.

PCR is the single most efficient test to diagnose the causative organism from intraocular fluids. Due to the rarity of VZV endotheliitis, studies are lacking. However, the study by Miyazaki et al.[3] to assess diagnostic efficacy of PCR in CMV endotheliitis showed a sensitivity of 91.7%, specificity of 88.9%, and negative predictive value of 95.2%. Their analysis proved that CMV endotheliitis is commonly associated with recurrences and raised IOP.

Anterior chamber paracentesis and aqueous humor PCR is not always preferred due to risk of false positives, endophthalmitis, wound leak, or sympathetic ophthalmia.[2] In our patient, we were suspecting CMV endotheliitis due to the localized corneal edema, ocular hypertension, and endothelial loss.[8] The drug of choice for treating CMV is ganciclovir. There are reports of poor response of CMV endotheliitis to acyclovir.[9] We felt it imperative to identify the correct causative organism. To the patient's benefit, VZV was detected, saving him from Ganciclovir, a more nephrotoxic drug. Study by Hung et al.[10] shows that topical acyclovir reaches twice the concentration of oral acyclovir in the anterior chamber of the eye.

We used topical therapy due to localized nature and the first episode of the disease. Our patient was a diabetic, with poorly maintained blood sugar. We did not want to run the risk of renal dysfunction in such a person. His poor paying capacity and poor compliance also influenced our decision. The results were satisfactory.

  Conclusion Top

We hereby report a case of varicella endotheliitis, with confusing clinical features. PCR proved extremely useful in diagnosis. Our report corroborates other studies that VZV uveitis need not have skin involvement, a point which goes against classic understanding of HZO and VZV. This phenomenon is similar to cases with viral retinitis which also do not have skin involvement. Topical therapy can be very useful to treat VZV endotheliitis but is only recommended after careful patient selection.

Our case is atypical in its uncommon etiology of VZV, which required PCR for diagnosis, localized area of involvement, and use of topical antivirals and topical corticosteroids for treatment.

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.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Moshirfar M, Murri MS, Shah TJ, Skanchy DF, Tuckfield JQ, Ronquillo YC, et al. A review of corneal endotheliitis and endotheliopathy: Differential diagnosis, evaluation, and treatment. Ophthalmol Ther 2019;8:195-213.  Back to cited text no. 1
Suzuki T, Ohashi Y. Corneal endotheliitis. Semin Ophthalmol 2008;23:235-40.  Back to cited text no. 2
Miyazaki D, Shimizu D, Shimizu Y, Inoue Y, Inoue T, Higaki S, et al. Real-time PCR for ocular cytomegalovirus infection study group. Diagnostic efficacy of real-time PCR for ocular cytomegalovirus infections. Graefes Arch Clin Exp Ophthalmol 2018;256:2413-20.  Back to cited text no. 3
Khodabande A. Varicella endotheliitis: A case report. Eur J Ophthalmol 2009;19:1076-8.  Back to cited text no. 4
Starr CE, Pavan-Langston D. Varicella-zoster virus: Mechanisms of pathogenicity and corneal disease. Ophthalmol Clin North Am 2002;15:7-15, v.  Back to cited text no. 5
Qian Z, Fan H, Tao Y, Li W, Gu W. Herpetic anterior uveitis in a Chinese referral center: Clinical manifestations and laboratory test results. Ocul Immunol Inflamm 2020;28:758-63.  Back to cited text no. 6
Babu K, Konana VK, Ganesh SK, Patnaik G, Chan NSW, Chee SP, et al. Viral anterior uveitis. Indian J Ophthalmol 2020;68:1764-73.  Back to cited text no. 7
[PUBMED]  [Full text]  
Koizumi N, Inatomi T, Suzuki T, Shiraishi A, Ohashi Y, Kandori M, et al. Japan Corneal Endotheliitis Study Group. Clinical features and management of cytomegalovirus corneal endotheliitis: Analysis of 106 cases from the Japan corneal endotheliitis study. Br J Ophthalmol 2015;99:54-8.  Back to cited text no. 8
Suzuki T, Hara Y, Uno T, Ohashi Y. DNA of cytomegalovirus detected by PCR in aqueous of patient with corneal endotheliitis after penetrating keratoplasty. Cornea 2007;26:370-2.  Back to cited text no. 9
Hung SO, Patterson A, Rees PJ. Pharmacokinetics of oral acyclovir (Zovirax) in the eye. Br J Ophthalmol 1984;68:192-5.  Back to cited text no. 10


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