Faculty Bibliography

PURPOSE: To determine the etiologies and management of calcific band keratopathy (CBK), and assess the results and long-term follow-up after ethylenediamine-tetraacetic acid (EDTA) chelation. DESIGN: Retrospective interventional case series. METHODS: Two hundred thirty patients with clinically significant CBK were included from January 1996 to July 2002. Among these, 54 patients (65 eyes) underwent EDTA chelation. Outcome measures included symptomatic relief, visual improvement, and recurrences. The improvement or worsening of the number of lines of Snellen best-corrected visual acuity was determined at 1 month and at last follow-up visit. RESULTS: The most common causes of CBK were chronic corneal edema in 80 eyes (28%) and idiopathic in 74 eyes (25.9%). The mean follow-up time after EDTA chelation was 36.6 months (range, 1 month to 29.6 years). Forty-four of 45 patients (98%) reported partial or complete symptomatic relief. Seventeen eyes (33.3%) improved 2 or more lines at 1 month and 18 eyes (35.2%) at last follow-up visit (P =.0001). In patients with an initial visual acuity between 20/50 and 20/400, 15 eyes (47%) improved 2 or more lines at 1 month and 16 eyes (50%) at last follow-up visit. Ten of 56 eyes (17.8%) had a recurrence at a mean time of 17.7 years (range, 1 month to 26 years). The highest number of recurrences was in three of five eyes with uveitis (60%; P =.03). CONCLUSION: Chelation with EDTA is an effective treatment of CBK. Visual acuity improves most in eyes with acuity between 20/50 and 20/400. This treatment can be used as the initial surgical intervention after conservative measures fail

OBJECTIVE: To assess the safety of cataract extraction following penetrating keratoplasty for corneal graft survival and to evaluate visual and refractive outcomes in corneal graft patients undergoing cataract extraction. METHODS: Retrospective chart review of 29 eyes of 24 patients with corneal grafts who underwent cataract extraction from January 1, 1993 to December 31, 2002, followed on the Cornea Service at Wills Eye Hospital. RESULTS: The mean time from penetrating keratoplasty to cataract extraction was 8.4 years (range 2 months to 36 years). Following cataract extraction, the corneal grafts remained clear in all but 1 eye (3%), during an average follow-up time of 44.5 months (range 3-118 months). All of the remaining patients benefited from improved visual acuity, with 15 of 28 patients having a postoperative best-corrected visual acuity of 20/30 or better. Patients also benefited from decreased absolute spherical refractive error, with a preoperative mean value of 6.6 +/- 3.4 D compared with 2.4 +/- 1.6 D postoperatively, while cylindrical refractive error remained relatively stable at 3.2 +/- 2.9 D preoperatively and 2.8 +/- 2.4 postoperatively. The patient who developed graft failure had 3 episodes of preoperative endothelial rejection and a clear corneal graft at the time of cataract surgery. CONCLUSIONS: Cataract surgery following penetrating keratoplasty is a safe and effective procedure, with a low but definite risk of corneal graft failure. In patients with clear grafts and visually significant cataracts, cataract extraction alone is preferred over repeat penetrating keratoplasty and cataract extraction

OBJECTIVE: To report a case of possible iatrogenic Creutzfeldt-Jakob many years following penetrating keratoplasty. DESIGN: Observational case report. METHODS: We reviewed the patient's chart, available literature on Creutzfeldt-Jakob disease, and current recommendations from the Eye Bank of America Association. RESULTS: An 80-year-old woman with a history of granular dystrophy underwent penetrating keratoplasty in 1978 (OS) and 1987 (OD) for decreased visual acuity. In early July 2002 the patient developed rapidly progressive dementia. Attempts were made to procure information regarding the corneal donors. No information was available. The patient expired slightly less than 1 month after her onset of symptoms. Review of the EBAA medical standards before October 2002 reveal that donor records were to be kept for a minimum of 10 years. CONCLUSION: Creutzfeldt-Jakob disease occurred 15 to 24 years after bilateral penetrating keratoplasy, but donor-to-host transmission was uncertain because no information on the corneal donor could be retrieved. Eye-bank records should be kept indefinitely because CJD may occur decades after iatrogenic exposure

PURPOSE: To further characterize the mutations within the CHST6 gene responsible for causing macular corneal dystrophy in a cohort of affected patients from the United States. DESIGN: Experimental study. METHODS: Genomic DNA was extracted from buccal epithelium of 16 affected patients (14 families), 17 unaffected relatives, and 127 controls, followed by polymerase chain reaction amplification and direct sequencing of the CHST6 coding region. Subtyping of affected patients into type I and II macular corneal dystrophy was performed by measuring antigenic keratan sulfate (AgKS) serum levels. Haplotype analysis was performed in families that demonstrated common mutations. RESULTS: CHST6 coding region analysis in 10 patients identified as having type I macular corneal dystrophy revealed 10 sequence changes: eight missense mutations, four of which are novel (Met104Val, Tyr110Cys, Gln122Pro, and Leu276Pro) and four of which have been reported previously (Ser51Leu, Pro72Ser, Cys102Gly, and Leu200Arg); one novel homozygous nonsense mutation in two patients from a single family (c. 1683C>T, Gln331X); and one frameshift mutation in a heterozygous state in a single patient (c.1744_1751dupGTGCGCTG). Mutation analysis in the four patients identified as having type II macular corneal dystrophy (serum samples were not obtained from two affected patients) revealed three patients heterozygous for either the c.923G>C, c.969C>A, or c.1519T>C sequence changes. The fourth patient was compound heterozygous for c.969C>A and c.1291T>G. None of these changes was observed in 127 control individuals. Haplotype analysis using microsatellite markers flanking the CHST6 gene did not reveal a common founder for the Leu200Arg (1291T>G) missense mutation, present in five families, identifying this position as a mutation hot-spot. CONCLUSIONS: A variety of previously unreported mutations in the coding region of the CHST6 gene are associated with type I macular corneal dystrophy in a cohort of patients from the United States

PURPOSE: To report a series of patients with no previous history of herpes simplex virus (HSV) infection who had new onset of herpetic keratitis after penetrating keratoplasty (PK). DESIGN: Noncontrolled, retrospective case series. METHODS: We included in the study the patients who had new onset of herpetic keratitis after penetrating keratoplasty for corneal diseases unrelated to HSV infection who were seen at the Cornea Service at Wills Eye Hospital (Philadelphia, Pennsylvania) from January 1996 to December 2002. The diagnosis of HSV epithelial keratitis was based on clinical characteristics of either a classic herpetic dendrite, a geographic ulcer, or a nonhealing epithelial defect that responded only to antiviral therapy. RESULTS: Fourteen patients were included in the study. Eight of these (57%) had presented with a geographic ulcer whereas six patients (43%) had a classic dendrite. The most common primary corneal disease that led to PK was pseudophakic bullous keratopathy (36%), followed by keratoconus (29%), Fuchs dystrophy (21%), and corneal scar unrelated to HSV (14%). CONCLUSIONS: The ophthalmologist should be aware of the possibility of herpetic keratitis in eyes after PK, even in patients with no previous history of HSV infection