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.     

Herpetic endotheliitis and trabeculitis with delayed corneal involvement

CASE REPORT: A 62-year-old man with previous renal transplant and immunosuppressive treatment presented with decreased visual acuity (20/100) in his left eye, corneal oedema and intraocular pressure of 46 mmHg. One month later an inferior marginal dendritic keratitis appeared. Corneal scraping and real-time polymerase chain reaction showed herpes simplex virus (HSV). DISCUSSION: The autoimmune corneal endotheliopathy or acute idiopathic corneal endotheliitis is characterised by corneal stromal oedema and keratic precipitates. HSV might be secreted from the trabeculum, innervated by the trigeminal nerve. This hypothesis is supported by the clinical observation that the corneal stromal oedema usually starts from the periphery.     

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.     

Progressive herpetic linear endotheliitis

PURPOSE: To report the clinical course of a rare case of bilateral herpetic linear endotheliitis. METHODS: A 70-year-old man presented with bilateral circumferential bullous edema with stromal edema progressing centrally in the left cornea and bilateral sensorineural hearing impairment simultaneously. Serum immunoglobulin G (IgG) and IgM antibodies against herpes simplex virus type 1 (HSV1) were tested for, and aqueous humor from both eyes was examined separately using polymerase chain reaction for the presence of HSV1 DNA. RESULTS: Serum antibody titers against HSV1 were positive. In the polymerase chain reaction, the aqueous humor showed HSV1 DNA in both eyes. Forty milligrams of prednisolone was given per day and 200 mg of oral acyclovir was given 4 times daily, but corneal edema progressed. After penetrating keratoplasty surgery in the left eye, recurrent herpetic endotheliitis also seemed to occur. CONCLUSIONS: HSV-1 may cause bilateral corneal linear endotheliitis and hearing impairment simultaneously. Linear endotheliitis should be regarded as a manifestation of HSV1 corneal infection. There is a poor prognosis, and severe corneal edema can result if aggressive treatment is not used.     

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.     

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.     

Oral acyclovir therapy of virologically proven intraocular herpes simplex virus infections

Three patients with severe intraocular herpetic disease (focal serous iritis, endotheliitis, trabeculitis) were treated with oral acyclovir. Inflammatory signs subsided promptly although only low-dose steroid eye drops were administered in addition to antiviral therapy. Normally, the viral etiology of suspected intraocular herpetic disease remains equivocal because virologic proof is lacking. In these three cases, however, herpes simplex virus was isolated from the aqueous humor before initiation of therapy, and the viral nature of the diseases treated could thus be proven.     

Herpes simplex virus isolations from the aqueous humor of patients suffering from focal iritis, endotheliitis, and prolonged disciform keratitis with glaucoma (author's transl)

Aqueous humor from 33 herpes patients, 4 zoster patients, and 14 patients with etiologically unclear anterior uveitis was cultured for the presence of herpes viruses. Nine taps from 8 herpes patients with corneal endothelial disease and/or anterior uveitis yielded herpes simplex virus. In the case of one patient two taps were positive at 14 days' interval. Control cultures from the surface of conjunctiva and cornea were consistently virus-negative. Analysis of the virus-positive cases displayed three remarkable features: 1. Secondary glaucoma was uniformly present. This in itself is an indication for culturable herpes simplex virus in the aqueous. 2. Three clinical pictures could be differentiated biomicroscopically: focal iritis, peripheral endotheliitis, and prolonged disciform keratitis. 3. In a proportion of cases, tissue damage resulting from associated immune reactions seems to be more important for the functional outcome than tissue damage by viral cytolysis itself. After having tried several antiviral substances (trifluorothymidine topically, adeninearabinoside-monophosphate intravenously, human leukocyte interferon intramuscularly and intracamerally) we presently favour a topical combination therapy consisting of trifluorothymidine and steroids. This must be complemented by cycloplegics and--in cases of high intraocular pressure--by acetazolamide.     

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.     

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.     

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.     

Clinical and histopathologic findings of iris nevus (Cogan-Reese) syndrome.

PURPOSE: To report a case of Cogan-Reese syndrome. METHOD: Case report. A 37-year-old man presented with Cogan-Reese syndrome. RESULTS: Visual acuity was 0.5 in the right eye and 1.0 in the left eye. There were corneal edema and pigmented nodules on the anterior surface of the iris, iris atrophy and ectropion uvea in the right eye. The intraocular pressure was 42 mmHg in the right eye and there was glaucomatous optic atrophy of the optic disk. Trabeculectomy with mitomycin C has been performed as the intraocular pressure did not decrease with the maximum medical treatment. Electron microscopic examination of the trabeculum and the iris tissue revealed a lot of melanocytic cells in the stroma. CONCLUSION: Trabeculectomy with mitomycin C might be effective in Cogan-Reese cases with glaucoma resistant to medical treatment.     

聚合酶链反应和间接免疫荧光法对角膜内皮炎病原诊断的比较研究

目的 探讨聚合酶链反应(PCR)和间接免疫荧光法(IIF)对角膜内皮炎的病原学诊断价值.方法 分别应用PCR和IIF对临床诊断为角膜内皮炎患者的房水进行单纯疱疹病毒检测,同时以老年白内障患者的房水作为对照,并做统计学分析.结果 16例角膜内皮炎患者的房水中,用PCR法检测阳性11例,阳性检出率为68.75%,20例对照组房水中无1例阳性,二者有显著性差异(P﹤0.05);13例角膜内皮炎患者的房水中,用IIF法检测阳性4例,阳性检出率为30.77%,20例对照组房水中无1例阳性,二者有显著性差异(P﹤0.05);角膜内皮炎患者的房水中PCR阳性检出率(68.75%)与IIF阳性检出率(30.77%)差异有统计学意义(P﹤0.05).结论 PCR法和IIF法均可作为角膜内皮炎的病原学快速诊断,但PCR法比IIF法敏感,可首选.     

Isolation of a vesicular virus belonging to the family rhabdoviridae from the aqueous humor of a patient with bilateral corneal endotheliitis

PURPOSE: To report bilateral corneal endotheliitis caused by a vesicular virus (family Rhabdoviridae). METHODS: Case report of a 49-year-old man with a complaint of sudden onset of decreased vision in both eyes had diffuse corneal stromal edema with extensive folds in Descemet's membrane and was diagnosed as having bilateral viral endotheliitis. Virologic investigations were performed using aqueous humor from the right eye. RESULTS: An ether- and chloroform-sensitive cytopathic agent was isolated in Vero and BHK-21 cell lines from the aqueous humor. It was identified as a vesicular virus belonging to the family Rhabdoviridae by electron microscopy. Neutralizing antibody was demonstrated at a titer greater than 1 in 4,096 dilutions in the convalescent serum. Neurologic complications included loss of hearing and postinfectious polyradiculopathy affecting both lower limbs. Best-corrected visual acuity was 20/120 OD and 20/20 OS. Six months later, he developed glaucoma in the right eye. Trabeculectomy with intraoperative application of 5-fluorouracil was performed. CONCLUSION: This is the first report of bilateral endotheliitis caused by a vesicular virus and confirmed by virus isolation from the aqueous humor of the affected eye.     

Herpes simplex virus DNA identification from aqueous fluid in Fuchs heterochromic iridocyclitis

PURPOSE: To report the presence of herpes simplex virus DNA in the aqueous humor of an eye with Fuchs heterochromic iridocyclitis. METHODS: In an eye with a clinical diagnosis of Fuchs heterochromic iridocyclitis, samples of aqueous humor and anterior capsule of the lens were obtained during cataract surgery. Polymerase chain reaction was performed on the samples to detect the presence of viral DNA including herpes simplex virus, varicella-zoster virus, and cytomegalovirus. Serologic analysis was also performed for antiviral immunoglobulins. RESULTS: Herpes simplex virus DNA was identified in the aqueous humor but not in the anterior capsule. Serum immunoglobulin G was positive for herpes simplex virus, varicella-zoster virus, and cytomegalovirus. CONCLUSIONS: The presence of herpes simplex virus DNA in the aqueous humor of an eye with Fuchs heterochromic iridocyclitis suggests that herpes simplex virus infection may play a role in the pathogenesis of Fuchs heterochromic iridocyclitis.     

Clinical results of triple Descemet's stripping and automated endothelial keratoplasty (DSAEK)

PurposeTo evaluate visual acuity (VA), refractive outcome, endothelial cell loss rate and complications of Descemet's stripping and automated endothelial keratoplasty (DSAEK) combined with phacoemulsification and intraocular lens (IOL) implantation in patients with coexisting corneal endothelial dysfunction and cataracts.MethodsSeventeen patients underwent phacoemulsification and posterior chamber IOL implantationthrough temporal corneal incision, followed by DSAEK. The selection of IOL power was predicted by preoperative lens power calculations of fellow eye plus 0.5 to 1.0 diopters (D).ResultsThere were five cases of laser iridotomy induced corneal dysfunction, four cases of Fuch's dystrophy, three cases of cytomegalovirus (CMV) endotheliitis, three cases of iridocorneal endothelial (ICE) syndrome, one case of herpes simplex virus (HSV) endotheliitis, and one case with an unknown cause. The BSCVAs were all under 0.2 preoperatively, and the average BSCVA was 0.3 postoperatively. The postoperative spherical equivalent (SE) refractive error was ?0.11 D on an average. The endothelial cell loss rate was ?36.86% at 6 months and ?38.60% at 12 months. There was one case of graft rejection at 6 months, and one case of primary graft failure. Complications such as donor detachment, pupillary block, donor graft folds, epithelial ingrowth, or interface scar did not occur.ConclusionThis case series of DSAEK combined with phacoemulsification and IOL implantation suggests that the procedure provides rapid visual rehabilitation and allows the selection of an appropriate IOL.     

Experimental corneal endotheliitis in rabbit

PURPOSE: Corneal endotheliitis may cause permanent visual loss due to endothelial decompensation. The pathogenesis underlying this distinct clinical entity is not known. In the current study, a rabbit herpetic corneal endotheliitis model was made of induced anterior chamber-associated immune deviation (ACAID). METHODs: One group of rabbits received left-eye intracameral inoculation of UV-inactivated herpes simplex virus (HSV)-1 (strain McKrae). The second group received cell medium in the same manner as the first group. The third group subcutaneously received the same inoculum as the first group. Seven days later, all right eyes were intracamerally infected with 2.5 x 10(4) plaque-forming units of infectious HSV-1. Eyes were evaluated by slit lamp examination. Two weeks after infection, rabbits were killed, and right eyes were examined by immunohistochemical staining and electron microscopy. Aqueous humor was detected for HSV-1 DNA and antibody. RESULTS: Nonspecific inflammation occurred in the anterior segments of the eyes from the second and third groups. In contrast, at 14 days after infection, the first group of rabbits showed a specific pattern of inflammation that greatly resembled clinical features of corneal endotheliitis. Viral antigen was detected only in the endothelial layer. Electron microscopy revealed enlarged intercellular gaps and infiltration of inflammatory cells that are characteristic of endothelial defects. HSV-1 DNA was detected at a significantly higher number in the aqueous humor aspirates from endotheliitis rabbits. In addition, ACAID was shown to be induced in the rabbits with corneal endotheliitis. CONCLusIONS: HSV-1 infection can induce corneal endotheliitis and ACAID may play the pivotal role in this entity.     

Clinical aspects of herpetic eye diseases

Diagnosis and treatment of herpes simplex virus eye diseases has benefitted from a vast amount of clinical and experimental studies. In this short presentation reference can only be made to some fields, which may illustrate--pars pro toto--the progress achieved during the last years. Clinico-virological correlation studies have led to a better understanding and classification of different typical clinical pictures, especially those affecting the structures of the anterior chamber: endotheliitis trabeculitis, and focal serous iritis. Antiherpetic drugs have become available which do not only penetrate the cornea in sufficient amounts but can also be used systemically. This will lead to a new role of steroids in the treatment of intraocular herpetic diseases. The combination of high-titer human interferon eye drops with a basic therapy of topical antiviral agents has considerably improved therapeutic results in dendritic keratitis.     

A case of hypertensive keratouveitis with endotheliitis associated with cytomegalovirus

AIM: While cytomegalovirus is well known as a pathogenic organism of retinitis, especially associated with human immunodeficiency virus infection, there are few reports of anterior uveitis associated with cytomegalovirus. METHODS: The authors present a case of keratouveitis associated with cytomegalovirus. RESULTS: A 70-year-old Japanese man was referred to the authors because of poorly controlled hypertensive keratouveitis in the left eye. The patient had a history of recurrent hypertensive anterior uveitis. At presentation, the corneal stroma was edematous, with Descemet's folds and pigmented keratic precipitates. The anterior chamber angle was depigmented compared to the fellow eye. Even though pupil dilation and posterior synechiae were absent, iris atrophy was not evident. His right eye appeared normal except for moderate cataract. Funduscopy of the left eye was hazy, with the optic disc showing a normal color but poorly defined details, and no apparent exdative retinitis. The best-corrected decimal visual acuity of the right and left eyes was 0.4 and 0.02, respectively. Intraocular pressure was 11 mmHg in the right eye and 35 mmHg in the left, despite maximum medical therapy. Systemic acyclovir and prednisolone for a month did not improve the hypertensive keratouveitis. The aqueous humor was investigated for herpes simplex virus, varicella-zoster virus and cytomegalovirus. Cytomegalovius genome was detected by polymerase chain reaction analysis. Oral valganciclovir rapidly reduced ocular hypertension within a week. CMV DNA disappeared 3 months after the initiation of valganciclovir. CONCLUSION: The authors reported a case of hypertensive keratouveitis with endotheliitis associated with cytomegalovirus.