Merlyn M. Rodrigues

Endothelial Alterations in Congenital Corneal Dystrophies

We studied the clinical and ultrastructural findings in three different types of congenital endothelial dystrophies: hereditary posterior polymorphous dystrophy, congenital hereditary corneal dystrophy, and a nonhereditary congenital endothelial dystrophy. In the first patient, with hereditary posterior polymorphous dystrophy, a layer of epithelial-like cells was observed adjacent to endothelial cells on the posterior corneal surface. Descemets membrane displayed a multilaminar pattern and consisted of an anterior, thin (3 mu), PAS-positive layer and a posterior, thicker (25 to 30 mu) zone of abnormal collagen. The second patient, with congenital hereditary endothelial dystrophy, showed a thickened multilaminar Descemets membrane and scant endothelial cells. In the third patient with nonhereditary congenital endothelial dystrophy, the thickened Descemets membrane was lined posteriorly by a retrocorneal fibrous membrane. A few degenerated endothelial cells were present. All three cases showed 100- to 110-nm banding posteriorly. In these three clinically distinct entities, electron microscopy was useful in demonstrating the unusual form of endothelial transformation to epithelial-like cells in one patient, in contrast to the more common fibroblast-like metaplasia of endothelial cells seen in the other two patients.

Clinicopathologic Correlation of Microphthalmos with Cyst

Three patients had microphthalmos with cyst in one orbit and contralateral congenital cystic eye, microphthalmos alone, or microphthalmos with cyst. Four eyes were examined histopathologically. The microphthalmic eye demonstrated a spectrum of anterior segment abnormalities, retinal disorganization and gliosis, and a choroidal and scleral colomboma. The cyst connected to the colobona consisted of an outer fibrovascular layer and inner gliotic neuroectodermal layer. The cyst probably originated from proliferation of neuroectodermal tissue at the edge of a persistently open embryonic fissure. Treatment consisted of multiple aspirations of the cyst, excision of the cyst alone, and excision of both the microphthalmic eye and cyst.

Exogenous Fungal Endophthalmitis Caused by Paecilomyces

A 17-year-old white boy developed a fulminating corneal infection and endophthalmitis in his left eye after trauma. He was treated with antibiotics and corticosteroids for one week prior to diagnosis. A saprophytic fungus, Paecilomyces viridis, was cultured from corneal scrapings and was demonstrated in the vitreous cavity by histopathologic examination.

Exogenous Corneal Ulcer Caused by Tritirachium Roseum

A 29-year-old white man developed a corneal ulcer following trauma to his left cornea by a piece of wire. Tritirachium roseum, a saprophytic fungus, was cultured from corneal scrapings and demonstrated in the corneal button by histopathologic examination.

Corneal Clouding with Increased Acid Mucopolysaccharide Accumulation in Bowman's Membrane

Two infants had bilateral congenital corneal clouding and abnormal acid mucopolysaccharide accumulation in a thickened Bowmans membrane. This unusual entity was not associated with acid mucopolysaccharide deposits in the skin and visceral tissues or with increased levels of acid mucopolysaccharide in the urine. The similarity and differences have been compared to the systemic mucopolysaccharidosis and macular corneal dystrophy.