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MD Hirsch, BV Worgul PhD, RE Braunstein MD, DS Casper MD/PhD; Lens Epithelial Changes in Anterior Lenticonus . Invest. Ophthalmol. Vis. Sci. 2002;43(13):479.
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Purpose: To analyze the changes in lens capsule and lens epithelial cells in Alport's syndrome. This information can assist in understanding the etiology of the anterior lenticonus changes that occur in these patients. Method: A 33 year old man with a previous diagnosis of Alport's syndrome presented with a complaint of progressive decrease in vision. On slit-lamp biomicroscopy both eyes were noted to have a central nippling on the anterior lens. In addition, the right eye had an anterior subcapsular cataract. The patient was sent for Scheimpflug imaging of both lens. These images confirmed a diagnosis of anterior lenticonus in both eyes, with cataractous changes in the right eye. The patient underwent a continuous curvilinear capsulorhexis during a phacoemulsification procedure to remove the cataract in the right eye. The capsule was placed in fixative and analyzed. Results: The specimen was noted to possess lens capsule and lens epithelium. The lens epithelial cells were noted to be without abnormalities on electron microscopy. The nuclei of the epithelial cells were similar to the epithelial cells in a normal lens. The DNA content of the epithelial cells were then analyzed. Results of the DNA showed that the lens epithelial cells with unaffected by the anterior lenticonus. This suggests that the changes in capsular structure that develop in Alport's syndrome does not affect the underlying epithelium. These findings contrast previous studies which suggest abnormal lens epithelium on electron microscopy specimens. Conclusion: The findings of anterior lenticonus suggested by slit-lamp biomicroscopy can be confirmed with Scheimpflug imaging. Once anterior lenticonus is diagnosed, the patients should undergo a workup for Alport's Syndrome. The currently accepted diagnostic method for Alport's syndrome is a renal biopsy. With advances in understanding in the lens changes in Alport's syndrome, the diagnosis of Alport's may be confirmed with non-invasive imaging of the lens. Our evaluation of the capsule and lens suggest that the lens epithelial cells are not altered by the changes in the lens shape. This information can aid to the further understanding of the etiology of the anterior bowing of the anterior capsule in Alport's syndrome.
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