| Abstract|| |
We report an interesting finding of multiple large drusen in an eviscerated eye with a longstanding retinal detachment. The origin and composition of the drusen is also speculated on the basis of special stains.
Keywords: Evisceration, large drusen, retinal detachment
|How to cite this article:|
Shenoy SK, Babu K, Prabhakaran VC. Large drusen following long-standing retinal detachment. Indian J Pathol Microbiol 2010;53:329-30
| Introduction|| |
Drusen are localized accumulations of extracellular material that build up below the retinal pigment epithelial layer (RPE) of the eye.  The presence of larger and numerous drusen in the macula is a common sign of age-related macular degeneration.  Drusen is also well documented in cases of longstanding retinal detachment.  We report a case of multiple large drusen following longstanding retinal detachment, documented histologically in an eviscerated eye.
| Case Report|| |
A 23-year-old lady presented with a history of recurrent pain and redness in the left nonseeing eye. She gave a history of injury to the left eye at the age of two years. On examination, her vision in the right eye was 20/20 with no perception of light in the left eye. The right eye examination was normal. The left eye was phthisical with a hazy, vascularized cornea. Hyphema was seen in the anterior chamber with no view of the fundus. Ultrasonography (B-scan) of the left eye revealed vitreous opacities with longstanding retinal detachment. Evisceration of the left eye was performed for relief of symptoms and cosmesis. The eviscerated specimen was sent for histopathological examination.
Hematoxylin-eosin stained sections showed a disorganized retina and choroidal tissues with extensive hemorrhage. The cornea showed sub-epithelial fibrosis with calcification of Bowman's membrane and loss of endothelial cells. Multiple-large spherical structures with a fibrillar radiating spoke-like appearance were seen situated beneath the RPE [Figure 1]a. These measured approximately 0.2 mm in diameter and stained positively with periodic acid-Schiff (PAS), Masson trichrome and Luxol fast blue stains [Figure 1] b-d. Micro-calcification was noted in some of the drusen and focal osseous metaplasia was also noted in the specimen.
| Discussion|| |
Large drusen following longstanding retinal detachment have been described in isolated case reports.  It is unclear why drusen occur following retinal detachment. It may possibly be related to retinal pigment epithelium metaplasia, since, in many cases of chronic retinal detachment, osseous metaplasia of the retinal pigment epithelium and drusen occurs concomitantly.  Studies have shown that the chemical composition of this subset of drusen (following retinal detachment) is not very different from age-related drusen. However, in our case they are composed of glycoprotein and collagen (positive staining with Masson trichrome) and this may be a reflection of the fibroblast potential of the retinal pigment epithelium.
Since these large drusen differ significantly in size from the usual drusen, they have to be distinguished from Giant drusen and collagenous spherulosis. Giant drusen are hamartomatous cellular lesions, seen in conditions like tuberous sclerosis. They are usually situated superficially in the optic nerve head and the adjacent retina and are characterized by astrocytic hyperplasia.  In contrast, the drusen are acellular.  Collagenous spherulosis is an unusual pathologic finding seen in breast lesions, salivary gland tumors and chondroid syringomas. The spherules show a fibrillar structure of concentric or radial spikes associated with micro-calcification and stain positively for PAS and Masson trichrome,  similar to drusen. However, while collagenous spherulosis is collagen rich,  drusen have a core of glycoprotein and an outer dome of crystallins, lipoproteins and vitronectin together with collagen.  They are thus positive for Luxol fast blue in addition to PAS and Masson trichrome as seen in our case.
Very large drusen can occur following chronic retinal detachment and it is important to recognize this finding, not only as a pathological curiosity but also to differentiate it from astrocytic hamartoma and collagenous spherulosis.
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