The clinical spectrum of posterior polymorphous dystrophy
G. W. Cibis, J. A. Krachmer, C. D. Phelps and T. A. Weingeist
We examined 61 affected members of eight families with an inherited corneal
dystrophy. The corneal abnormalities varied greatly from one member of a
family to another. Some patients had only a few isolated endothelial
vesicles, while others in the same family had severe secondary stromal and
epithelial edema. In some patients edema was present at birth or in early
childhood; in others it developed later in life. The wide variation of
corneal abnormalities suggests the possibility that several conditions
previously described as separate disease entities, such as grouped
vesicles, Schnyder's posterior herpes, posterior polymorphous dystrophy,
and congenital hereditary endothelial dystrophy, are part of the clinical
spectrum of expression of a single familial corneal dystrophy. Some
affected family members also had ocular hypertension or open-angle
glaucoma. Broad iridocorneal adhesions were present in some of the patients
with glaucoma and in others with normal intraocular pressures. Other ocular
abnormalities present in a few patients include pupillary ectropion, "glass
membranes" on the anterior iris surface, and bands in Descemet's membrane.
The transmission in most of the families was autosomal dominant. In two
families it appeared to be autosomal recessive.
