Programm                 "Degeneration und Regeneration– Grundlagen, Diagnostik und Therapie"

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Phthisis bulbi

Bechrakis N. E.
Augenklinik, Universitätsklinikum Benjamin Franklin, Freie Universität Berlin

Purpose: The retinal pigment epithelium (RPE) has an extraordinary propensity towards reactive proliferation and metaplasia, induced by a variety of thermal, mechanical and other partly unknown stimuli. On the contrary malignant dedifferentiation of the RPE is extremely unusual. Especially chronic inflammatory-degenerative diseases of the eye, like chronic uveitis, advanced disciform macular degeneration, long-standing retinal detachment, phthisis bulbi and other may lead to the formation of ectopic bone in the chorioretinal interface.
Method: Case report
Results: The presented case is a phthisical eye due to a long standing retinal detachment secondary to penetrating eye injury. Fibrous metaplasia and calcification of the lens epithelium, as well as atrophy and disintegration of the normal retinal architecture with retinal cyst formation secondary to retinal detachment is present. Multiple large and partially confluent drusen are found in the mid- and outer periphery of the fundus. A bone plate is lining the inner surface of the choroid on each side of the stalk of the detached retina. Fat is present within the interspaces of the lamellar bone.
Conclusions: Bone formation in the chorioretinal interface is based pathogenetically on an ossific metaplasia of the RPE. Fibroblasts and osteoblasts arising by cellular dedifferentiation of the RPE produce collagens and extracellular matrix proteins, which bind to calcium and phosphate-ions producing the

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