Proliferative endotheliopathy with iris abnormalities. The iridocorneal endothelial syndrome
R. C. Eagle Jr, R. L. Font, M. Yanoff and B. S. Fine
Sixteen cases of essential iris atrophy and two of iris nevus (Cogan-Reese)
syndrome were reviewed retrospectively by light and electron microscopy.
Varying degrees of corneal endothelial proliferation onto the trabecular
meshwork and anterior iris were seen in all cases. The demonstration of an
endothelial-abnormal basement membrane complex on the anterior iridic
surface, deep to synechias in over half the cases, indicates that
endothelialization may be primary, since it precedes the formation of
anterior synechias. Pathologic and clinical evidence suggests that the iris
nevus syndrome, Chandler's syndrome, and essential iris atrophy represent a
continuum of clinical manifestations of a single disease process involving
proliferation of the corneal endothelial and characteristic iris
abnormalities; the latter may be secondary to variations in the pattern,
rate, and extent of endothelial proliferation. We suggest the term
"proliferative endotheliopathy" to emphasize the common pathogenetic role
of corneal endothelial proliferation in all three entities (iris nevus
syndrome, Chandler's syndrome, and essential iris atrophy). A more succinct
alternate term is "the iridocorneal endothelial (ICE) syndrome."
