Corneal endotheliitis

Corneal endotheliitis is an intriguing clinical entity manifested by corneal edema, keratic precipitates, and mild anterior chamber reaction, and can be defined as a spectrum of the disorder in which the corneal endothelium is the primary site of the inflammation. The disease etiology consists of accumulating evidence of various viral infections including herpes simplex virus, varicella zoster virus, and cytomegalovirus. Corneal endotheliitis can be classified clinically into four forms: linear, sectorial, disciform, and diffuse. Antiviral treatment in combination with topical corticosteroids is generally effective to suppress the inflammation; however, irreversible corneal endothelial dysfunction may develop in some cases.     

Demonstration of "owl's eye" morphology by confocal microscopy in a patient with presumed cytomegalovirus corneal endotheliitis

PURPOSE: To report confocal microscopic observations of characteristic corneal endothelial lesions in a patient with presumed cytomegalovirus (CMV) corneal endotheliitis. DESIGN: Case report. METHODS: A 77-year-old, immunocompetent man was admitted with corneal edema, keratic precipitates, and coin-shaped lesions in the right eye. Confocal microscopy was performed to examine the corneal endothelium. Polymerase chain reaction (PCR) was used to identify viral DNA in an aqueous humor sample. RESULTS: CMV DNA was detected by PCR. Confocal microscopy showed large corneal endothelial cells with an area of high reflection in the nucleus surrounded by a halo of low reflection. This "owl's eye" morphology is characteristic of CMV infection. Topical and intravenous ganciclovir treatment resulted in rapid resolution of the corneal precipitates and edema, followed by disappearance of the owl's eye morphology. CONCLUSIONS: Confocal microscopy can detect the owl's eye morphology in the corneal endothelium of patients with presumed CMV corneal endotheliitis.     

Corneal Endotheliitis

Corneal endotheliitis is an intriguing clinical entity manifested by corneal edema, keratic precipitates, and mild anterior chamber reaction, and can be defined as a spectrum of the disorder in which the corneal endothelium is the primary site of the inflammation. The disease etiology consists of accumulating evidence of various viral infections including herpes simplex virus, varicella zoster virus, and cytomegalovirus. Corneal endotheliitis can be classified clinically into four forms: linear, sectorial, disciform, and diffuse. Antiviral treatment in combination with topical corticosteroids is generally effective to suppress the inflammation; however, irreversible corneal endothelial dysfunction may develop in some cases.     

Cytomegalovirus in aqueous humor from an eye with corneal endotheliitis

PURPOSE: To report cytomegalovirus (CMV) DNA in aqueous humor from a patient with unilateral corneal endotheliitis. DESIGN: Case report. METHODS: A 51-year-old man presented with unilateral corneal endotheliitis with linear keratic precipitates and coin-shaped lesions. Tear and aqueous humor samples were subjected to polymerase chain reaction to look for DNA from herpes simplex virus (HSV), varicella zoster virus (VZV), and CMV. RESULTS: Aqueous humor from the diseased eye contained DNA from CMV but not HSV or VZV. Its specificity was confirmed by Southern blot tests. Intravenous ganciclovir treatment resulted in the localization of his corneal edema and the reduction in keratic precipitates. There was severe destruction of corneal endothelial cells. CMV DNA was not detected in tears or control samples. CONCLUSIONS: In this healthy man with corneal endotheliitis, we detected CMV DNA in aqueous humor from the affected eye, but not HSV or VZV. This suggests that CMV may cause corneal endotheliitis in patients without immunodeficiency.     

Herpes simplex virus in the trabeculum of an eye with corneal endotheliitis

PURPOSE: To report an eye with corneal endotheliitis and increased intraocular pressure in which the trabeculum demonstrated immunoreactivity for herpes simplex virus. METHOD: Case report. A 62-year-old man presented with increased intraocular pressure, keratic precipitates, and corneal stromal edema in his left eye. The tissue excised during trabeculectomy was immunohistochemically examined for herpetic viruses. RESULT: Immunoreactivity for herpes simplex virus was identified in the trabeculum. CONCLUSION: Herpes simplex virus may cause trabeculitis and increased intraocular pressure in patients with corneal endotheliitis.     

The validity of clinical feature profiles for cytomegaloviral anterior segment infection

Background  

Anterior segment cytomegalovirus (CMV) infection, which can be presented as anterior uveitis and corneal endotheliitis, has recently been reported in immunocompetent patients. We would like to access the validity of two presumed characteristic clinical profiles: profile 1, non-herpes simplex virus (HSV)/varicella zoster virus (VZV) corticosteroid-recalcitrant inflammatory ocular hypertensive syndrome (IOHS), and profile 2, corneal endotheliitis with specific coin-shaped keratic precipitates (KPs), that could be helpful in identifying CMV anterior segment intraocular infection.     

Cytomegalovirus-related corneal endotheliitis: A review article

Cytomegalovirus (CMV)-related corneal endotheliitis is an inflammation of the corneal endothelium caused by CMV. It typically presents as coin-shaped keratic precipitates (KPs), with or without corneal edema, in otherwise healthy individuals. It may be associated with anterior uveitis and raised intraocular pressure (IOP). Patients with CMV-related corneal endotheliitis respond to systemic and topical ganciclovir with the use of topical steroid. Making an accurate early diagnosis is crucial in preventing loss of corneal endothelial cells and unnecessary treatment resulting from misdiagnosis in these patients.     

Acute idiopathic corneal endotheliitis

Seven patients presenting with acute corneal stromal edema without prior surgery, trauma, ocular disease or known exposure to noxious agents are reported. One patient had a severe iridocyclitis; two others subsequently developed herpetic keratouveitis. Four cases, however, were unassociated with other known ocular disease. All four of these patients exhibited keratic precipitates and displayed minimal or no flare and cells in the anterior chamber, although the latter was largely obscured by the corneal edema. In each of these four instances, the inflammation and corneal edema resolved following topical corticosteroid therapy. In two of the four cases, both children, antibody to Herpes simplex virus could not be identified. We suggest that, in patients without known prior corneal disease or trauma, acute diffuse corneal stroma edema may stem from severe iridocyclitis or a primary endotheliitis due either to herpetic infection, the recently described presumed autoimmune corneal endotheliopathy, or a condition which we herein designate acute idiopathic corneal endotheliitis.     

DNA of cytomegalovirus detected by PCR in aqueous of patient with corneal endotheliitis after penetrating keratoplasty

PURPOSE: Corneal endotheliitis often leads to severe endothelial dysfunction and can be caused by herpes simplex virus (HSV), varicella zoster virus (VZV), and other viruses (eg, the mumps virus). We report a case of corneal endotheliitis caused by cytomegalovirus (CMV) that developed after a penetrating keratoplasty. METHODS: A complete ophthalmologic examination was performed on a patient with corneal endotheliitis that developed after a penetrating keratoplasty. To determine the cause of the endotheliitis, polymerase chain reaction (PCR) was used to amplify the DNA of HSV, VZV, and CMV in samples of the aqueous humor. RESULTS: Slit-lamp biomicroscopy showed a moderate stromal edema in the upper temporal part of the transplanted cornea along with keratic precipitates (KPs) arranged in a coin-shaped pattern. Repeated treatments with steroids and acyclovir were only temporarily successful. PCR detected the DNA of CMV in an aqueous sample, and the treatment was switched to topical and systemic application of ganciclovir. This resulted in the disappearance of the KPs and resolution of the stromal edema within 2 weeks. CONCLUSIONS: From the PCR results and the favorable response to ganciclovir, the corneal endotheliitis was most likely caused by cytomegalovirus in this case.     

Autoimmune endotheliopathy and chronic herpetic conjunctivitis

A migrating endothelial line of keratic precipitates associated with overlying corneal edema suggests an immune attack on the corneal endothelium. This is seen most commonly in corneal allotransplantation rejection. The etiology of such lines in the absence of this condition is unclear. We document the presence of an intranuclear virus compatible with herpesvirus in this condition.     

Self-limiting corneal edema with multiple parallel lines on the endothelium (SCEMPLE)

The purpose of this study was to report the natural course and in vivo confocal microscopy (IVCM) findings of five cases with unilateral self-limiting corneal edema and multiple parallel lines on the endothelium (SCEMPLE). This study is an observational case series. Five patients, who experienced a blurred vision due to SCEMPLE, were studied using slit-lamp examination and white-light IVCM (Confoscan 4; Nidek Technologies, Padova, Italy). IVCM of the linear deposits revealed characteristic hyperreflective material protruding between the endothelial cells. The lines also displayed spot-like holes and polygonal precipitates, which resembled dislodged endothelial cells. Because other signs of corneal inflammation, such as stromal infiltration and ciliary injection, were lacking, the condition was left untreated. After 1 day, slit-lamp examination and IVCM revealed only a small residual of the lines. Spontaneous, complete resolution occurred in all five cases within 1 week, leaving a lower endothelial cell density of the affected eyes as only sequela. SCEMPLE requires no treatment but may result in endothelial cell loss. The self-limiting character and IVCM appearance dispute SCEMPLE being a form of endotheliitis and suggest a different etiology.     

Histopathology of herpetic corneal endotheliitis - a case report

BACKGROUND: Clinically, corneal endotheliitis is characterized by endothelial cell edema associated with immune precipitates. It is supposed to be triggered by a humoral or cellular immunologic reaction against antigens of the corneal endothelium. As yet, only few histopathologic studies of this disease have been performed. PATIENT: A 42-years-old patient developed a complete corneal nebula with neovascularizations due to relapsing herpetic keratitis. He underwent perforating keratoplasty. The inflammatory infiltrate lining Descemet's membrane and the corneal endothelium consisted mostly of CD 45 R0-positive T-lymphocytes with only few neutrophils, macrophages and CD 20-positive B-lymphocytes. We could not demonstrate any viral particles in the removed corneal tissue by either electron microscopy or using antibodies against Herpes simplex type I or II. CONCLUSIONS: Histologically, herpetic endotheliitis appears to correspond to corneal endotheliitis due to graft rejection. Complete virus particles are not necessarily required in case of long-standing inflammation and corneal neovascularizations.     

Identification of cytomegalovirus and human herpesvirus-6 DNA in a patient with corneal endotheliitis

Purpose

To report the case of a patient with unilateral corneal endotheliitis in which both cytomegalovirus (CMV) and human herpesvirus-6 (HHV6) DNA was identified in the aqueous humor.

Case

A 67-year-old man with corneal endotheliitis OD was referred to us for decreased visual acuity. Local corneal stromal edema, pigmented keratic precipitates, a coin-shaped lesion and minimal anterior chamber reaction were observed by slit-lamp biomicroscopy. Cells with owl’s eye appearance in the endothelial cell layer were observed by in vivo laser confocal microscopy. The patient had rheumatoid arthritis, which was treated by oral prednisolone and intravenous abatacept. Polymerase chain reaction analysis of aqueous humor samples detected both CMV and HHV6 DNA, but not other HHVs. Treatment with topical ganciclovir and systemic valganciclovir resulted in a clear cornea.

Conclusions

A patient with corneal endotheliitis had both CMV and HHV6 DNA identified in the aqueous humor. Although both viruses were identified in this case, clinical manifestations resembled CMV corneal endotheliitis, and it was unclear whether HHV6 could affect the clinical course. Systemic abatacept and corticosteroid therapy might play a positive role in cases with both CMV and HHV6 DNA in this corneal endotheliitis.     

Clinical Evaluation of Intravitreal Injection of Ganciclovir in Refractory Corneal Endotheliitis

ABSTRACT

Purpose: To evaluate the efficacy and safety of intravitreal ganciclovir (GCV) injection in refractory endotheliitis.

Methods: Retrospectively recruited 25 eyes with endotheliitis, proved by clinical manifestations, positive PCR for viral DNA and responded poor to topical and systemic antiviral medications. All patients received additional continued intravitreal GCV injections.

Results: Cytomegalovirus (CMV), varicella zoster virus (VZV), and herpes simplex virus (HSV) DNA were detected in 64.0%, 28.0%, and 8.0% of the eyes, respectively. Within 2 weeks after the last injection, 16/25 eyes recovered corneal clarity; active keratic precipitates (KPs) were eliminated in 21/25 eyes; intraocular pressure (IOP) was controlled in 12/15 eyes with elevated IOP on study entry. Best-corrected visual acuity increased at the last follow-up (p = 0.016). Clinical recurrence occurred in three patients. No complications were detected.

Conclusions: CMV endotheliitis was the main type of refractory endotheliitis. Despite its invasive nature, intravitreal GCV injection appears to be an effective method for refractory endotheliitis.     

Clinical Features of CMV-Associated Anterior Uveitis

Cytomegalovirus (CMV) anterior uveitis is the most common ocular manifestation of CMV disease in immunocompetent individuals. It is thought to be due to a local reactivation of latent CMV and is usually unilateral. The acute form presents as Posner-Schlossman Syndrome, a recurrent hypertensive anterior uveitis with few granulomatous keratic precipitates. There are geographic differences in the chronic form of CMV anterior uveitis. Asian patients commonly present as Fuchs Uveitis Syndrome with diffuse stellate keratic precipitates, while the European patients present with a chronic hypertensive anterior uveitis with fewer keratic precipitates that are brown in color and located inferiorly. Characteristic features of CMV anterior uveitis include mild anterior chamber inflammation, elevated intraocular pressure, stromal iris atrophy. Synechiae, macular edema and retinitis are typically absent. CMV disease may also be associated with the development of corneal endotheliitis with a reduced endothelial cell count. Long-term complications include glaucomatous optic neuropathy and cataract formation.     

病毒性角膜内皮炎的诊治探讨

病毒性角膜内皮炎并不少见,但因对其临床表现认识不足,误诊、漏诊病例屡见不鲜.角膜内皮炎常由单纯疱疹病毒、带状疱疹病毒感染所致.临床分为三种类型：盘状角膜内皮炎、弥漫性角膜内皮炎和线状角膜内皮炎.临床表现为病变部位角膜水肿,水肿部位羊脂状KP,当合并小梁网炎时伴有眼压升高,临床上常与青光眼-睫状体炎综合征相混淆.治疗关键点是抗病毒和抗炎,正确合理地使用抗病毒药和皮质类固醇激素类药物非常重要.用药的方法分为全身用药和局部用药.大部分患者眼局部用药是主要的给药途径,对于部分反复发作和病情严重的患者可考虑全身给药.皮质类固醇激素应用的浓度和频度要根据患者的病情而定,但基本原则是在足量应用全身和局部抗病毒药的基础上选择生物利用率高的皮质类固醇激素,将炎症迅速控制后逐渐减量,缓慢停药.     

Corneal endotheliitis and idiopathic sudden sensorineural hearing loss

PURPOSE: To report a case with corneal endotheliitis and idiopathic sudden sensorineural hearing loss, in which herpes simplex virus type 1 DNA was demonstrated in the trabeculum and the aqueous humor by polymerase chain reaction. DESIGN: Interventional case report. METHODS: A 60-year-old man presented with corneal stromal edema in the right eye and sudden bilateral sensorineural hearing loss. The trabeculum excised during trabeculectomy and the aqueous humor were examined for the presence of herpes simplex virus type 1 DNA by polymerase chain reaction. RESULTS: Polymerase chain reaction demonstrated herpes simplex virus type 1 DNA in the aqueous humor and the trabeculum. CONCLUSION: Herpes simplex virus type 1 may cause corneal endotheliitis and idiopathic sudden sensorineural hearing loss simultaneously.     

The Effect of Topical Ganciclovir and Corticosteroid on Cytomegalovirus Corneal Endotheliitis in Korean Patients

Purpose: To report long-term outcomes of topical ganciclovir (GCV) and corticosteroids in Korean patients with cytomegalovirus (CMV) corneal endotheliitis.

Methods: This retrospective study included 13 eyes from 13 patients with CMV corneal endotheliitis, with a follow-up period of 24.5 ± 8.2 months. The patients were consistently maintained with topical 2% GCV and 1% prednisolone acetate eyedrop.

Results: All patients demonstrated unilateral typical coin-shaped keratic precipitates (KPs) or linear KP, and positive CMV polymerase chain reaction of aqueous humor. After 2 weeks of treatment, all patients showed decrease of clinical signs. During the follow-up, four patients developed mild anterior chamber inflammation with increased intraocular pressure without typical coin-shaped KPs or edema, started to use the initial dose, and resolved the clinical signs. One patient showed recurrence of corneal edema twice, and was administered systemic valgancyclovir for 2 weeks upon second recurrence with resolution of clinical signs.

Conclusion: Long-term maintenance therapy with topical GCV and corticosteroids are effective and maintain corneal endothelial function in Korean patients with CMV endotheliitis.     

Demystifying viral anterior uveitis: A review

A viral aetiology should be suspected when anterior uveitis is accompanied by ocular hypertension, diffuse stellate keratic precipitates or the presence of iris atrophy. The most common viruses associated with anterior uveitis include herpes simplex virus, varicella‐zoster virus, cytomegalovirus and rubella virus. They may present as the following: Firstly, granulomatous cluster of small and medium‐sized keratic precipitates in Arlt's triangle, with or without corneal scars, suggestive of herpes simplex or varicella‐zoster virus infection. Secondly, Posner‐Schlossman syndrome with few medium‐sized keratic precipitates, minimal anterior chamber cells and extremely high intraocular pressure; this is mainly associated with cytomegalovirus. Thirdly, Fuchs uveitis syndrome, with fine stellate keratic precipitates diffusely distributed over the corneal endothelium, with diffuse iris stromal atrophy but without posterior synechiae, is associated mainly with rubella or cytomegalovirus infection. In rubella, the onset is in the second to third decade. It presents with posterior subcapsular cataract, may have iris heterochromia and often develops vitritis without macular oedema. Cytomegalovirus affects predominantly Asian males in the fifth to seventh decade, the keratic precipitates may be pigmented or appear in coin‐like pattern or develop nodular endothelial lesions, but rarely vitritis. Eyes with cytomegalovirus tend to have lower endothelial cell counts than the fellow eye. As their ocular manifestations are variable and may overlap considerably, viral AU can pose a diagnostic dilemma. Thus, quantitative polymerase chain reaction or Goldmann‐Witmer coefficient assay from aqueous humour samples are preferred to confirm the aetiology and determine the disease severity as this impacts the treatment.     

A Case of CMV Endotheliitis Treated with Intravitreal Ganciclovir Injection

We report a case of CMV corneal endotheliitis that was treated with intravitreal ganciclovir injection. A 56-year-old man who has suffered from uveitis was referred to our clinic due to corneal endothelial abnormality. Slit lamp examination showed a localized sectoral corneal edema and linear keratic precipitates along the boundary of edema. In spite of treatment with oral steroid and acyclovir, the disease progressed and two new coin-like lesions were developed. After topical ganciclovir and intavitreal injection of ganciclovir, the corneal lesions disappeared.