Faculty Bibliography

To report a case of ocular copper deposition in both eyes at the level of Descemet's membrane associated with a monoclonal gammopathy of undetermined significance (MGUS). A 49-year-old white woman had golden-brown metallic dust-like deposits on Descemet's membrane of both eyes. A systemic examination revealed an elevated serum copper, normal serum ceruloplasmin and a normal level of total protein. Serum protein electrophoresis demonstrated a single peak (M-spike) in the gamma region (M-protein in serum = 11 g/l). Flow cytometric analysis of the marrow aspirate identified a monoclonal plasma cell population that represents approximately 2% of the total marrow cells consistent with the diagnosis of monoclonal gammopathy of undetermined significance. Copper deposits at the level of Descemet's membrane may be a finding in a patient with monoclonal gammopathy of undetermined significance

PURPOSE: To study the clinical and microbiological profile of Serratia corneal ulcers at the Cornea Service of the Wills Eye Hospital. METHODS: This was a retrospective, observational case series. The clinical records of patients with Serratia marcescens corneal ulcers seen at the Cornea Service of the Wills Eye Hospital between January 1, 1998 and December 31, 2002 were reviewed. RESULTS: Twenty-four cases of Serratia keratitis were identified in 21 patients. Two patients (9.5%) had recurrent keratitis, 1 of which recurred twice. Both had corneal graft edema and were on topical steroids and antiglaucoma drops. The Serratia infection in 15 patients (71%) was associated with an abnormal corneal surface. Twelve of these patients (57%) had the ulcer in a corneal graft, 4 (19%) of which were associated with suture infiltrates. Fifteen patients (71%) were on topical medications-15 used corticosteroids and 13 used antiglaucoma drops. Six patients (29%) were contact lens wearers-1 had a concomitant suture infiltrate associated with a corneal graft, and 5 had otherwise healthy corneas. One isolate lacked in vitro susceptibility to ciprofloxacin and ofloxacin but was susceptible to gentamicin and tobramycin. Nineteen patients had a favorable response to medical therapy. Two patients with poor outcome had large corneal ulcers with severe necrosis and thinning associated with delay in treatment. CONCLUSIONS: Serratia marcescens keratitis is associated with the presence of an abnormal corneal surface, use of topical medications, and contact lens wear. Prompt medical therapy results in a good clinical response in the majority of cases

PURPOSE: To determine the outcome of patients with decreased vision associated with anterior basement membrane dystrophy (ABMD) managed with diamond burr (DB) polishing. DESIGN: Retrospective nonrandomized case series. METHODS: A chart review of all patients with ABMD who underwent epithelial debridement and DB polishing to treat visual disturbances resulting from corneal epithelial irregularity was performed. RESULTS: Ten patients' charts were analyzed. The mean age was 57.4 years. DB treatment was performed in 13 eyes. The average preoperative BCVA was 0.40 (20/50). At the last follow-up (mean 21.8 months) the average visual acuity of these patients was 0.88 (20/23), significantly better than pre-operative readings (P < .001). There was no recurrence of dystrophic changes in the treatment zone seen with the slit lamp during the follow-up period. CONCLUSIONS: In this study, DB polishing was shown to be an effective and safe treatment for decreased vision caused by to ABMD over an average follow-up of 21.8 months

PURPOSE: This study was designed to determine how patients with pellucid marginal corneal degeneration (PMCD) are managed in a tertiary corneal practice. METHODS: A retrospective chart review of 45 patients with PMCD from a corneal referral practice was performed. Information collected from the medical records included demographic data, best-corrected visual acuity, videokeratography, contact lens use, and surgical procedures. RESULTS: Forty-five patients (85 eyes; 30 males, 15 females) with PMCD were included in this study. They were followed-up for an average of 4.7 years (standard deviation (SD), +/-8.7). Seventy-five eyes (88.2%) were managed nonsurgically with spectacles in 31 eyes (36.4%) or contact lenses in 44 eyes (51.8%). Contact lens management was initially attempted in 51 eyes (60%); however, 7 eyes failed contact lenses. Visual acuity of 0.5 (20/40) or better was noted in 34 eyes (75.5%) after contact lens fit. Ten eyes (11.8%) underwent penetrating keratoplasty with an average postoperative follow-up of 9 years (SD, +/-8.2; 1.5-20.5). Clear grafts were present in all 10 eyes at the end of the study (average, 9 years; SD, +/-82). CONCLUSIONS: Nonsurgical management of PMCD continues to play a predominant role in the management of this disorder. Poor best-corrected visual acuity at presentation of 0.2 (20/100) or worse and long follow-up (8 years or more) were significantly associated with surgery

PURPOSE: To analyze the frequency of contact lens-related corneal ulcers and its relationship to the type of contact lens and care. METHODS: Charts of 376 patients with corneal ulcers seen at the Cornea Service of Wills Eye Hospital from July 1, 1999 to December 31, 2002 were retrospectively reviewed. All patients with contact lens-related corneal ulcers were identified, and information regarding cultures, lens type, usage, and cleaning was obtained. RESULTS: Of the 376 cases, 113 (30%) were related to contact lens use. The contact lens history was recorded in 83 of 113 cases (73%). The soft daily wear frequent replacement lens was the most common lens type associated with corneal ulcers (n = 36/83, 43%). Corneal cultures were performed in 71 of 113 cases (63%) and were positive in 51 of 71 cases (72%). The most common microorganism involved was Pseudomonas aeruginosa (n = 17/51, 33%). The frequency of contact lens-related corneal ulcers from 1999-2002 (n = 113/376, 30%) was significantly greater than that from years 1996-1999 (n = 37/299, 12%) at our institution (P < 0.05). CONCLUSION: There was a significant increase in the number of contact lens-related corneal ulcers between 1999 and 2002 compared with previous years (P < 0.05). The contact lens type most frequently associated with corneal ulcers was the soft daily-wear frequent-replacement contact lens (43%). Contact lens-related corneal ulcers continue to be a serious problem despite a shift in the market to the use of frequent-replacement daily-wear contact lenses and advances in contact lens technology

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