Clinical and histopathologic studies of two families with lattice corneal dystrophy and familial systemic amyloidosis (Meretoja syndrome).

Lattice corneal dystrophy associated with familial systemic amyloidosis (Meretoja syndrome) has rarely been described other than in patients of Finnish origin. The authors report two North American patients with this disease who manifest blepharochalasis, lattice corneal dystrophy, open-angle glaucoma, and cranial neuropathy. In one patient, a corneal intraepithelial and subepithelial pseudodendrite was managed by superficial keratectomy, and this same patient benefited from surgical brow suspension for facial muscular weakness. In the second patient, penetrating keratoplasty was complicated by a neurotrophic persistent epithelial defect. Corneal tissue from both superficial keratectomy and penetrating keratoplasty exhibited ultrastructurally characteristic amyloid filaments and associated elastoid material. Transmission electron microscopy of conjunctiva and skin biopsies similarly revealed amyloid deposits associated with most basement membranes, the perineurium and endoneurium of most peripheral nerves, and the intima and media of arteries. By immunoperoxidase staining, the corneal amyloid deposits were positive for the amyloid P-component protein but negative for the nonimmunoglobulin amyloid A protein and prealbumin. Serum prealbumin and amyloid A related protein were normal.

Endophthalmitis following penetrating keratoplasty.

To determine the incidence of infectious endophthalmitis in the early postoperative period following penetrating keratoplasty, and the type and origin of the causative organisms, all cases of penetrating keratoplasty performed at the Emory University affiliated hospitals between January 1977 and March 1982 were reviewed. Four (0.2%) of the 1,876 cases developed infectious endophthalmitis. In all four, evidence of infection developed within 72 hours, and in three the donor rim culture grew the same organism as was obtained from the anterior chamber or vitreous. The causative organisms were Streptococcus pneumoniae, Staphylococcus aureus, group D Streptococcus-enterococcus, and Pseudomonas aeruginosa. Eyes with positive donor rim cultures had a 22-fold increased incidence of endophthalmitis.

Corneal endothelium under various storage conditions.

Three rabbit corneas each were stored in McCarey-Kaufman (M-K) medium, rabbit serum, and in a moist chamber at 4 degrees C refrigeration for various lengths of observation. The endothelial cells appeared normal under all conditions for the first 24 hours as compared with control corneas processed concurrently with each experimental group. After 48 hours of storage the specimens in the moist chamber showed isolated endothelial cell damage. The endothelia in M-K medium or rabbit serum appeared viable up to six days without significant differences although those stored in rabbit serum showed a better preservation of microvilli on individual endothelial cells. Under all conditions a mild shrinkage of the cells seemed to have taken place as indicated by the more pronounced cell boundaries. We incubated an equal number of control rabbit corneas at 37 degrees C with 5% CO2 and moist air in M-K medium, serum, and minimal essential medium (MEM) with 10% fetal calf serum and 100 units/ml of a penicillin and streptomycin mixture. In serum, the endothelia showed rapid destruction with swelling of the entire cornea. Those stored in M-K medium maintained a normal endothelial covering of the cornea up to six days. At nine days of storage, marked cellular changes were observed with dehiscence of the cellular layer. When stored in the MEM mixture, the endothelial cells showed a normal layer without obvious cell damage when compared with those stored in M-K medium up to four days. However, after six and nine days of storage, cellular destruction was greater in these specimens than in those stored in M-K medium. In addition, there was considerable swelling of the whole cornea under this storage condition.