Sg. Levy et al., DESCEMETS-MEMBRANE IN THE IRIDOCORNEAL-ENDOTHELIAL SYNDROME - MORPHOLOGY AND COMPOSITION, Experimental Eye Research, 61(3), 1995, pp. 323-333
The iridocorneal-endothelial syndrome is a disease of the ocular anter
ior segment characterized by corneal failure, glaucoma and iris destru
ction. Specular photomicroscopical and histological studies suggest th
e disorder is caused by a population of abnormal corneal endothelial c
ells. In other corneal endotheliopathies Descemet's membrane, the base
ment membrane underlying the endothelial cells, is disfigured by the p
resence of an abnormal region of extracellular matrix termed a posteri
or collagenous layer, which is laid down by the diseased endothelial c
ells. In this study we sought to establish the typical morphology and
composition of Descemet's membrane in the iridocorneal-endothelial syn
drome. Ultrastructural examination of Descemet's membrane in 27 kerato
plasty specimens identified three morphologic patterns. In the majorit
y there was a posterior collagenous layer which in all cases consisted
of an anterior layer of wide-spaced collagen and a posterior layer of
microfibrils embedded in an amorphous matrix. In four specimens which
did not possess a posterior collagenous layer the anterior banded zon
e of Descemet's membrane was absent, In five corneas Descemet's membra
ne was normal. The composition of the posterior collagenous layer was
examined by immunoelectron microscopy (five corneas) and histochemistr
y (six corneas). Collagen Types I, III, V, VI and VIII, fibronectin, t
enascin and oxytalan were microfibrillar components, collagen Type VII
I formed wide-spaced collagen whilst laminin was present in the amorph
ous matrix. The stereotyped derangements of structure and composition
identified in the endothelial basement membrane may significantly infl
uence the pathobiology of this disorder. (C) 1995 Academic Press Limit
ed.