ABSTRACT
PURPOSE: Iridocorneal endothelial (ICE) syndrome is a group of rare ocular conditions that result from abnormal corneal endothelial cells, leading to secondary glaucoma, iris distortions, and corneal edema. The etiology of ICE is unknown, although it has been associated with viral infections, such as herpes simplex virus. In this study, we sought to identify an infectious etiology for ICE using advanced molecular techniques. METHODS: Metagenomic RNA sequencing (MDS) is a high-throughput sequencing approach that can identify all pathogens in any clinical sample, including RNA viruses. Descemet membrane and aqueous fluid from patients with ICE syndrome were subjected to MDS testing. RESULTS: Samples from 3 patients with ICE were analyzed. MDS was performed on the aqueous fluid of 3 patients and Descemet membrane and endothelial cell tissue from 1 patient. Viral pathogens were not identified in any of the samples. CONCLUSIONS: We were unable to identify a viral etiology in the tissues of patients with the Chandler variant of ICE syndrome, although this study was limited by sample size.
Subject(s)
Eye Infections, Viral/virology , High-Throughput Nucleotide Sequencing/methods , Iridocorneal Endothelial Syndrome/virology , Metagenomics , Aqueous Humor/virology , Cytomegalovirus/genetics , Descemet Membrane/virology , Endothelium, Corneal/virology , Female , Herpesvirus 3, Human/genetics , Humans , Male , Middle Aged , RNA, Viral/analysis , Retrospective Studies , Simplexvirus/geneticsABSTRACT
Iridocorneal (ICE) syndrome is a rare ocular disease characterized by abnormal proliferation of corneal endothelial cells, progressive obstruction of irido-corneal angle and atrophy of iris. ICE syndrome progressed slowly, but can cause serious complications such as secondary glaucoma in late stage. Because the etiology of ICE syndrome is not clear, there is still no effective treatment in clinical practice. Previous studies have detected herpes simplex virus (HSV) DNA inside patient's aqueous humor. However, no further explanation for HSV-related etiology of ICE syndrome was established. Besides, construction of animal models using HSV all failed, leaving behind a blank space about how HSV infection finally led to ICE syndrome. By summarizing findings from previous studies, we came up with a hypothesis about etiology of ICE syndrome: HSV infection initiated ICE syndrome by integration of viral genetic material into human genome. Infection of HSV changed activity and morphology of endothelial cells, making them regain the ability of mitosis. Proof of such hypothesis will provide a theoretical foundation for construction of animal models and effective intervention of the disease.
Subject(s)
Iridocorneal Endothelial Syndrome/etiology , Iridocorneal Endothelial Syndrome/virology , Keratitis, Herpetic/complications , Keratitis, Herpetic/virology , Simplexvirus/genetics , Simplexvirus/pathogenicity , Animals , Aqueous Humor/virology , Cornea/virology , Genes, Viral , Genome, Human , Humans , Iridocorneal Endothelial Syndrome/genetics , Iris/pathology , Models, Biological , Virus Integration/geneticsABSTRACT
PURPOSE: To report a rare case of concurrent acute retinal necrosis in a patient with iridocorneal endothelial syndrome (ICE). METHODS: Case report. RESULTS: A 42-year-old woman showed acute diminution of vision in the right eye. Her fundus examination revealed features of acute retinal necrosis. She had also experienced gradual diminution of vision in her left eye for 5 years. The examination of her left eye revealed corneal edema with mild corectopia and increased intraocular pressure with abnormal endothelium on specular microscopy pointing to a diagnosis of ICE. CONCLUSIONS: This is a rare case where concurrent acute retinal necrosis and ICE syndrome are present in the same patient, possibly pointing to a common viral etiology causing both entities.
