Purpose The present study systematically investigated and quantified histopathological changes in a series of keratoconic (Kc) corneas utilizing a physiologically formulated fixative to not further distort the already distorted diseased corneas. Methods Twelve surgically removed Kc corneal buttons were immediately preserved and processed for light and transmission electron microscopy using an established corneal protocol. Measurements were taken from the central cone and peripheral regions of the host button. The sample size examined ranged in length from 390–2608um centrally and 439–2242um peripherally. Results The average corneal thickness was 437um centrally and 559um peripherally. Epithelial thickness varied centrally from 14–92um and peripherally from 30–91um. A marked thickening of the epithelial basement membrane was noted in 58% of corneas. Centrally, anterior limiting lamina (ALL) was thinned or lost over 60% of the area examined, while peripheral cornea was also affected, but to a lesser extent. Histopathologically, posterior cornea remained undisturbed by the disease. Anteriorly in the stroma, an increased number of cells and tissue debris were encountered and some of these cells were clearly not keratocytes. Conclusions It is concluded that Kc pathology, at least initially, has a distinct anterior focus involving the epithelium, ALL and anterior stroma. The epithelium had lost its cellular uniformity and was compromised by the loss or damage to the ALL. The activity of the hitherto unreported recruited stromal cells may be to break down and remove ALL and anterior stromal lamellae leading to the overall thinning that accompanies this disease.
The increasing number or corneal and intraocular surgeries performed together with longer life expectancy, elevates the risk for developing symptomatic Fuchs' endothelial dystrophy (FED). This article reviews the current understanding of FED, and, in addition, a case of early FED is presented clinically and histopathologically. Two FED corneas from one patient were examined histopathologically using an established histological protocol for light and electron microscopy. FED is an inherited autosomal dominant corneal endothelial disorder with incomplete penetrance that is up to 3 times less likely to develop in men. Treatment options are primarily palliative while surgical intervention routinely involves a penetrating keratoplasty. In this disease process the endothelium produces excessive amounts of basement membrane material of an abnormal composition resulting in the formation of a posterior collagenous layer. Extreme accumulations of this material created mushroom-like formations, guttae, projecting into the anterior chamber. The endothelial cells were extremely thinned over the guttae, to the point where the cells may provide little more than barrier function. Despite a presumably compromised endothelial pump the corneas appeared relatively free of edema clinically and histopathologically. However, some edematous pockets deep in the basal epithelial layer were present suggesting that epithelial involvement occurs at an early stage in the disease. An early diagnosis of FED will help patients and surgeons to better elect optimal surgical timing and procedures. The current trend is to intervene surgically before the patient reaches the painful end-stage. Interestingly, in the case examined, the endothelium presumably provided only a barrier function over large areas with an apparently reduced contribution from the endothelial fluid pump and yet the corneas remained relatively clear. Future research may confirm that decompensation occurs only when complete endothelial coverage is lost. If the endothelial barrier function is more important to corneal transparency than the endothelial pump function in FED, then the relative combination of these two functions in the normal cornea should also be reassessed.
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ABSTRACT.Purpose: The purpose of this study was to identify ultrastructural changes associated with ectasia and to determine the association between lamellar count and corneal thinning. Methods: Five surgically removed keratoconic corneal buttons and four, nonkeratoconic, normal eye bank control corneas were processed for transmission electron microscopy using an established protocol, ensuring minimal tissue distortion. A sequence of overlapping digital images, spanning the full apical cone corneal thickness, was assembled. A seamless digital montage was printed at 50003 magnification. Lamellae were counted in the anterior-posterior orientation, along a linear line, using established criteria for identification of individual lamellae. Results: The stromal thickness estimated as a 95% confidence interval for the mean, CI (0.95), in the keratoconic corneas was 372 AE 62 lm, while in the normal cornea, it was 446 AE 89 lm. All keratoconic corneas showed ultrastructural evidence of lamellar splitting and a loss of interweaving anterior lamellae. In the keratoconic corneas, the median total linear stromal lamellar absolute count tangential to the corneal surface was 362, (25th percentile; 75th percentile) = (355; 365) lamellae and in the normal cornea, 246, (25th percentile; 75th percentile) = (239; 251). The linear lamellar density in the keratoconic corneas was estimated as CI (0.95) 117 AE 22 and 86 AE19 lamellae per 100 lm in the anterior and posterior portion of the stroma, respectively. In normal cornea, the linear lamellar density was estimated as CI (0.95) 51 AE 8 and 80 AE 20 lamellae per 100 lm. The mean difference of linear lamellar count between the anterior and the posterior portion of the cornea was estimated as CI (0.95) 31 AE 23 for keratoconic corneas and À29 AE 28 for the normal corneas. Conclusions: The current morphometric analysis of ultrastructural changes suggests that ectasia and thinning in keratoconus is associated with lamellar splitting into multiple bundles of collagen fibrils and loss of anterior lamellae. These structural changes, possibly in addition to lateral shifting of lamellae due to the pressure gradient over the cornea, are a potential explanation to the central loss of mass.
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