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Tenley N. Bower, Bruno F. Fernandes, Devinder Cheema, Jean Deschenes, Patrick T. Logan, Miguel N. Burnier, Jr.; A Study On Recurrent Corneal Stromal Dystrophies: Fifteen Years Of Penetrating Keratoplasty. Invest. Ophthalmol. Vis. Sci. 2012;53(14):6051.
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Corneal stromal dystrophies are inherited disorders that are characterized by bilateral opacities caused by the progressive accumulation of deposits in the cornea that affect transparency. In some cases, the deposits lead to recurrent erosions, scarring, or deterioration of visual acuity necessitating penetrating keratoplasty (PKP). PKP usually restores normal visual acuity, but in some cases the deposits reappear in the donor cornea and another transplant is needed. Our goal was to study the characteristics of patients with corneal stromal dystrophies that required PKP focusing on recurrence.
A single-centered retrospective analysis was performed on PKP specimens diagnosed as corneal stromal dystrophies received by the Henry C. Witelson Ophthalmic Pathology Laboratory and Registry (Montreal, Canada) over a 15 year period (1996 - 2011). Corneal specimens obtained were subjected to hematoxylin and eosin, periodic acid-Schiff, and other special staining.
During the 15 year period of study, 1,252 corneal specimens from PKP were received. Thirty-five (2.8%) specimens from a total of 27 patients with stromal dystrophies were examined: 21 specimens with lattice (60.0%), nine with combined granular/lattice ("Avellino") dystrophy (25.7%), four with granular (11.4%), and one with macular (2.9%). The clinical and pathological diagnosis discordance rate was 31.4% (11 patients): Avellino dystrophy accounted for nine cases and granular dystrophy for two cases. Ten corneal (28.6%) buttons were from a recurrent corneal stromal dystrophy that required repeat PKP: eight specimens (six Lattice, two Avellino) came from a second PKP and two specimens came from a third PKP (Lattice, Avellino). The average age for first PKP was 58.8 years (lattice 52.3 yrs, Avellino 55.8 yrs, granular 60.5 yrs, macular 35 yrs). A second PKP occurred in lattice dystrophy at an average age of 77.8 years and in Avellino at 60 years. A third PKP occurred in lattice dystrophy at 59 years and in Avellino at 55 years.
The order of frequency of stromal dystrophy requiring PKP was lattice, followed by Avellino, granular, and macular. The frequency of patients requiring PKP according to age from youngest to oldest was macular, lattice, Avellino, and granular. The clinical and pathological discordance rate was high which reinforces the need for clinical attention to Avellino and lattice dystrophies and the need to perform special staining. The most common dystrophy to recur in a graft requiring repeat PKP was lattice followed by Avellino.
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