The Stromal Dystrophies

In Vivo Confocal Microscopy of Combined Pre-Descemet Membrane Corneal Dystrophy and Fuchs Endothelial Dystrophy

“…Biomicroscopic findings include grayish plaques, linear streaks, and vesicular lesions on the endothelial surface. 1,19 In vivo confocal microscopy revealed craters, streaks, and cracks over the corneal endothelium surface. Guttae and clusters of abnormal endothelial cells were also identified.…”

“…Characteristically, it spares the epithelium, Bowman layer, Descemet membrane, and endothelium layers, limiting the deposits to the posterior stromal layer. [1][2][3] Fuchs endothelial dystrophy is an inherited disorder of unknown etiology specific to the corneal endothelium. Pathological changes involving other corneal layers such as epithelium and stroma may occur as a consequence of the endothelial dysfunction.…”

Penetrating Keratoplasty for Corneal Amyloidosis in Familial Amyloidosis, Finnish Type

“…38 However, only 1 case report of a patient with FAF who had a previous PK has been published. 39 The patient had received 4 keratoplasties in 3 years, roughly after the age of 40, and a fifth keratoplasty 10 years later.…”

Mutation Analysis of CHST6 Gene in Chinese Patients With Macular Corneal Dystrophy

“…Histopathologically, the disease is characterized by the accumulation of glycosaminoglycans within keratocytes, the surrounding stroma, the subepithelial area, Bowman layer, Descemet membrane, and the endothelium. 2 MCD has been divided into clinically indistinguishable types (I, Ia, and II) by immunohistochemical studies and serum analysis of antigenic keratan sulfate (AgKS). 3 In 2000, the carbohydrate sulfotransferase 6 (CHST6) gene on chromosome 16q22, encoding corneal N-acetylglucosamine 6-O-sulfotransferase (C-GlcNAc6ST), was identified as a causative gene of MCD types I and II.…”