Faculty Bibliography

PURPOSE: To evaluate the outcomes of the Boston Keratoprosthesis (KPro) at our institution. METHODS: A computerized database retrospectively identified all patients who received a Boston KPro from August 2005 to October 2007. RESULTS: There were 37 patients, 24 (65%) were male. Mean age was 66.3 years (median 69 years, range: 24-93 years; SD = 18.1 years). Mean follow-up was 16 months (range: 6-28 months; SD = 6.0 months). There were 36 type 1 (97%) and 1 type 2 (3%) KPros. The primary indication was failed penetrating keratoplasty in 29 patients (78%)-22 (59%) from multiple failures. Preoperative comorbidities included glaucoma [27 patients (73%)] and limbal stem cell deficiency [13 patients (35%)]. No intraoperative complications occurred. Postoperative complications included retroprosthetic membrane [24 patients (65%)], increased intraocular pressure [14 patients (38%)], glaucoma progression [5 patients (13.5%)], and endophthalmitis [4 patients (11%)-3 who discontinued prophylactic antibiotics secondary to compliance]. Thirty-six KPros (97%) were retained-1 type 2 KPro (3%) in a patient with ocular cicatricial pemphigoid extruded and was replaced. Mean best-corrected visual acuities were counting fingers preoperatively (range: light perception to 20/100) and 20/90 at last follow-up (range: light perception to 20/25). At last follow-up, 31 patients (84%) improved 2 lines or greater-3 patients (8%) had worse vision. CONCLUSIONS: Retention rates were excellent, and best-corrected visual acuities improved in the vast majority of patients. Complications can occur and require frequent follow-up to monitor and treat glaucoma progression, endophthalmitis, and inflammation. Patient compliance is of paramount importance. Despite the potential complications, the Boston KPro provides visual improvement in patients with an otherwise poor prognosis

PURPOSE: To compare the incidence and characteristics of graft rejection in keratoconus patients with and without self-reported atopy. METHODS: From November 2007 to February 2008, 75 consecutive keratoconus patients who had undergone penetrating keratoplasty completed a questionnaire regarding personal history of atopy. The questionnaires were reviewed without knowledge of history of graft rejection. The patients were divided into three groups: nonatopic (group 1), mild atopic (group 2), and severe atopic (group 3). Charts were retrospectively reviewed with regard to the incidence and characteristics of graft rejection episodes for each patient. RESULTS: There were 13 patients in group 1, 36 patients in group 2, and 26 patients in group 3. The incidence of graft rejection (rejection episodes per 100 person-years) in groups 1, 2, and 3 was 7.3, 15.8, and 13.4, respectively. Although the mild and severe atopic groups had roughly twice the rate of rejection of the nonatopic group, there was no statistically significant difference among the groups (P = 0.25). The mean age of 37.1 +/- 8.9 years at the time of surgery was significantly lower (P = 0.004) and the rate of bilateral penetrating keratoplasty was significantly higher (40/55 eyes 73%) (P = 0.01) in group 2 than in the two other groups. The majority of first graft rejection episodes were mild or moderate in atopic patients (69% in group 2 and 94% in group 3) and severe (50%) in nonatopic patients; however, no significant difference was found among the groups (P = 0.13). CONCLUSIONS: Atopic conditions may increase the incidence of graft rejection in keratoconus patients, and the characteristics of graft rejection in atopic patients may differ from those in nonatopics because of the immune alteration, but differences in this study were not statistically significant

PURPOSE: The aim of the present study was to evaluate the frequency of steroid-induced intraocular pressure (IOP) elevation and/or glaucoma in patients with keratoconus (KCN) compared with patients with Fuchs endothelial dystrophy after penetrating keratoplasty (PK). METHODS: A retrospective review of the medical records of patients with KCN or Fuchs dystrophy, who underwent PK and were examined on the Cornea Service, Wills Eye Institute, was performed. IOP measurements were recorded preoperatively; postoperative first month and maximal IOP measurements between 1 and 3 months, 4 and 6 months, 7 and 12 months, 1 and 2 years, 2 and 3 years, and 3 and 4 years were noted. Steroid-induced IOP elevation and/or glaucoma were grouped into 5 different categories; an increase in IOP of at least 5 or 10 mm Hg over the preoperative baseline and also IOP > or =22, 30, and 40 mm Hg. Addition of glaucoma medications and/or characteristic glaucomatous optic disc and visual field changes were also assessed. RESULTS: A total of 100 patients with KCN and 58 patients with Fuchs dystrophy were included in this study. The overall frequency of steroid-induced IOP elevation after PK was 73% in the KCN group and 60.3% in the Fuchs dystrophy group. The frequency of IOP elevation of at least 5 or 10 mm Hg over the preoperative baseline were 72% and 24% in KCN group and 56.9% and 20.7% in the Fuchs dystrophy group, respectively. The frequency of IOP elevation > or =22 or > or =30 mm Hg was 22% and 6% in the KCN group and 29.3% and 1.7% in the Fuchs dystrophy group, respectively. There was one patient in the KCN group who had IOP >40 mm Hg. There was no difference between the groups in terms of frequency of IOP elevation (P > 0.05 for all). Glaucomatous visual field defect was detected in 4 patients in the KCN group and only one patient in the Fuchs dystrophy group. Despite the maximum medical therapy, 2 patients in the KCN group underwent glaucoma surgery and none in the Fuchs dystrophy group. CONCLUSION: Steroid-induced IOP elevation or glaucoma after PK is not unusual in eyes with KCN or Fuchs dystrophy. Careful and ongoing observation of IOP throughout the prolonged follow-up period is recommended for these individuals with prompt attention to IOP treatment as indicated

OBJECTIVES: To assess the impact of the different types of contact lenses (CLs) on quality of life (QoL) in patients with keratoconus based on self-reported results from The Contact Lens Impact on Quality of Life (CLIQ) Questionnaire. METHODS: Consecutive keratoconus patients who wore CLs (rigid gas permeable [RGP], hybrid or soft toric) at least in one eye were asked to complete the CLIQ questionnaire on the Cornea Service, Wills Eye Institute. RESULTS: A total of 71 patients with a mean age of 42.6 +/- 13.1 year were included in the study. One eye of each patient was included in the study. Of these, 40 eyes used rigid gas-permeable lenses, 20 eyes used hybrid lenses and 11 eyes used soft toric lenses. The mean CLIQperson measure was 45.5 +/- 8.2 in RGP group, 45.4 +/- 7.5 in hybrid group and 48.4 +/- 10.5 in soft toric group. There was no significant difference among the three groups in self-reported results from the CLIQ questionnaire (P = 0.8). CONCLUSIONS: Subjects with keratoconus who wear RGP, hybrid or soft toric CLs, reported similar contact lens impact on their QoL

OBJECTIVE: To report the concomitant presentation of keratoconus and corneal dystrophies at Wills Eye Hospital for the 10-year period from January 1, 1997, to December 31, 2006. METHODS: Patients with concomitant keratoconus and corneal dystrophies were identified using a computer database. Complete ophthalmologic examination, keratometry, pachymetry, and computerized videokeratography were performed in all patients. When present, cornea guttata were confirmed by clinical examination and specular microscopy. Histopathologic examination with special stains of excised corneal buttons was performed. RESULTS: Fifty-one patients manifested typical signs and topographic evidence of keratoconus associated with another corneal dystrophy. Fuchs dystrophy was the most common association accounting for 27 cases (52.9%), followed by anterior basement membrane dystrophy with 13 cases (25.5%) and posterior polymorphous dystrophy with 7 cases (13.8%). A bilateral combination of Fuchs dystrophy and anterior basement membrane dystrophy with keratoconus was seen in 3 cases (5.8%). Finally, there was 1 bilateral case (2%) of granular dystrophy. Histopathologic studies in cases that underwent penetrating keratoplasty confirmed the clinical diagnoses. CONCLUSION: To our knowledge, this is the largest report of such a concurrence in the English literature and could lead to further studies on the possible pathophysiologic or genetic links between these entities, although a chance association cannot be excluded

PURPOSE: To report the outcomes of contact lens-intolerant patients with keratoconus (KCN) with subepithelial nodules treated with excimer laser phototherapeutic keratectomy (PTK). METHODS: The charts of all contact lens-intolerant patients with KCN who underwent excimer laser PTK for subepithelial nodules were identified using a computer database and were retrospectively reviewed. Preoperative and postoperative visual acuity and method of correction, surgical treatment, epithelial healing, complications, and KCN nodule recurrence were all analyzed. RESULTS: Fifteen eyes of 15 patients with a minimum follow-up of 3 months were reviewed. Mean follow-up time was 23.1 months (SD +/- 42.5 months, range 3-143 months). In 9 eyes, the epithelial defect healed by postoperative day 3 and all eyes healed by postoperative day 6. There was no infection or corneal melting in any of the eyes. After PTK, 11 patients were refit successfully with rigid gas-permeable contact lenses and 4 wore glasses. Three patients had a recurrence of the KCN nodule at 3, 8, and 23 months postoperatively. One of them had a repeat PTK 23 months after the initial surgery, improving to 20/40 with contact lenses at 55 months postoperatively. Another patient decided to have a penetrating keratoplasty, and the third patient is considering repeat PTK or penetrating keratoplasty. CONCLUSION: PTK effectively removed KCN nodules without recurrences in most patients and can be a successful modality to improve contact lens tolerance and delay or avoid more invasive surgery

PURPOSE: To identify current indications and trends in indications for penetrating keratoplasty (PKP) and associated procedures. METHODS: Retrospective chart review of all patients who underwent PKP at Wills Eye Institute from January 1, 2001, to December 31, 2005. RESULTS: A total of 1,162 cases were performed in this 5-year period. Leading indications for PKP were pseudophakic corneal edema (PCE) in 330 (28.4%) cases, followed by regraft in 250 (22.0%), keratoconus in 186 (16%), and Fuchs' endothelial dystrophy in 126 (10.8%) cases. Of the 330 cases of PCE, 232 (70.3%) were associated with posterior chamber intraocular lenses (PCIOLs) and 96 (29.1%) with anterior chamber lenses. In 330 eyes with PCE, the lens was not exchanged in 246 (74.5%) cases and was exchanged in 76 (23%) cases. Seventy of the exchanged lenses were anterior chamber intraocular lenses (ACIOLs) and six lenses were PCIOLs. In cases of ACIOL exchanges, 10 were for scleral sutured IOLs, 18 for PCIOLS, and 42 for another ACIOL. CONCLUSIONS: Pseudophakic corneal edema remains the leading indication for PKP at our institution followed by regraft, continuing a trend noted in our previous studies. Although the percentage of cases of PCE associated with PCIOLS increased, fewer lenses were exchanged, perhaps reflecting increased confidence in biocompatibility of newer IOLs. The decrease in overall number of corneal transplants in these 5 years continues a trend noted in our previous study and mirrors the national decline in PKP

In this review, we analyze the trends in corneal transplantation over the past 25 years in the United States. The most dramatic change was the progressive sharp increase in the number of corneal transplants performed during the 1980s, corresponding with the rise of pseudophakic bullous keratopathy (PBK) as the leading indication for keratoplasty. More recently, there has been a steady annual decline in total keratoplasties for more than a decade, corresponding with a decline in the cases performed for PBK, which still accounts for the highest percentage of cases done. Regrafts have been an increasingly important indication for keratoplasty, as older grafts fail. Keratoconus, Fuchs dystrophy, and other inherited diseases have remained consistent indications for keratoplasty during the last quarter century