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Infectious scleritis can occur days to years after ocular surgery, with infection occurring after a longer interval in eyes with a history of pterygium surgery. Approximately 50% of eyes lost functional VA after infection with poor presenting VA being the strongest predictor for subsequent severe vision loss.

Purpose To describe the incidence, microbiology, associated factors and clinical outcomes of patients with infectious keratitis progressing to endophthalmitis. Design Non-randomized, retrospective, consecutive case series. Participants All patients treated for culture-proven keratitis and endophthalmitis between January 1, 1995 and December 31, 2009 at the Bascom Palmer Eye Institute. Methods Ocular microbiology and medical records were reviewed on all patients with positive corneal and intraocular cultures over the period of the study. Univariate anaylsis was performed to obtain p values described in the study. Main Outcome Measures Microbial isolates, treatment strategies, visual acuity outcomes. Results A total of 9934 corneal cultures were performed for suspected infectious keratitis. Only 49 eyes (0.5%) progressed to culture-proven endophthalmitis. Fungi (n=26) were the most common responsible organism followed by gram positive bacteria (n=13) and gram negative bacteria (n=10). Topical steroid use (37/49[76%]) was the most common associated factor identified in the current study, followed by previous surgery (30/49[61%]), corneal perforation (17/49[35%]), dry eye (15/49[31%]), relative immune compromise (10/49[20%]), organic matter trauma (9/49[18%]) and contact lens wear (3/49[6%]). There were 27 patients in which a primary infectious keratitis developed into endophthalmitis, and 22 patients in which an infectious keratitis adjacent to a previous surgical wound progressed into endophthalmitis. Patients in the primary keratitis group were more likely to be male (22/27[81%] vs. 8/22[36%], p=0.001), have history of organic matter trauma (8/27[30%] vs. 1/22[5%]), p=0.030), and have fungal etiology (21/27[78%] vs. 5/22[23%], p<0.001). Patients in the surgical-wound-associated group were more likely to use topical steroids (20/22[91%] vs. 17/27[63%], p=0.024). Visual acuity of ≥20/50 was achieved in 7/49[14%] patients, but was <5/200 in 34/49[69%] patients at last follow up. Enucleation or evisceration was performed in 15/49[31%] patients. Conclusions Progression of infectious keratitis to endophthalmitis is relatively uncommon. The current study suggests that patients at higher risk for progression to endophthalmitis include patients using topical corticosteroids, patients with fungal keratitis, patients with corneal perforation, and patients with infectious keratitis developing adjacent to a previous surgical wound. Patients with sequential keratitis and endophthalmitis have generally poor visual outcomes.

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Despite advances over the past 100 years, endophthalmitis is an important sight-threatening complication. Timely management with the appropriate use of antimicrobial agents may optimize visual outcomes.

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Mutations in the BIGH3 gene on chromosome 5q31 cause four distinct autosomal dominant diseases of the human cornea: granular (Groenouw type I), Reis-Bücklers, lattice type I, and Avellino corneal dystrophies. All four diseases are characterized by both progressive accumulation of corneal deposits and eventual loss of vision. We have identified a specific recurrent missense mutation for each type of dystrophy, in 10 independently ascertained families. Genotype analysis with microsatellite markers surrounding the BIGH3 locus was performed in these 10 families and in 5 families reported previously. The affected haplotype could be determined in 10 of the 15 families and was different in each family. These data indicate that R555W, R124C, and R124H mutations occurred independently in several ethnic groups and that these mutations do not reflect a putative founder effect. Furthermore, this study confirms the specific importance of the R124 and R555 amino acids in the pathogenesis of autosomal dominant corneal dystrophies linked to 5q.