Keratoprosthesis in high-risk pediatric corneal transplantation: first 2 cases.
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Biomedical subjects
Publications and source records attributed to Esen Karamursel Akpek.
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PURPOSE: To describe subluxation of iris-fixated intraocular lenses (IOLs) after penetrating keratoplasty (PK) as a postoperative complication and report the long-term outcomes of a closed-chamber refixation technique used in the management. METHODS: This was a retrospective, noncomparative interventional case series in which 19 consecutive patient charts with a subluxated iris-fixated IOL after PK were retrospectively reviewed. Fourteen of the patients underwent surgery using a closed-chamber refixation technique. These patients were evaluated for visual outcome and surgical complications associated with the procedure. RESULTS: In 13 patients, postoperative follow-up was available. In all 13 cases, the fixated IOL appeared stable and remained well positioned during a follow-up period of 56 months (range, 6-122). All patients (100%) achieved improved uncorrected visual acuity. The postoperative visual acuity ranged from 20/20 to 20/100 with a mean of 20/40. Vitreous hemorrhage occurred in 1 patient, and an additional patient required a second surgery using the same technique. Both of these patients did well with a final visual acuity of 20/40 and 20/50, respectively. CONCLUSIONS: Subluxation of iris-fixated IOL after PK can occur as a postoperative complication. The technique that we herein describe enables secure refixation of subluxated IOLs and yields favorable long-term results.
PURPOSE OF REVIEW: Diseases affecting the cornea are a major cause of blindness worldwide, second only to cataract in overall importance, with an estimated 10 to 15 million affected people. Although keratoplasty is by far the most successful transplantation surgery, the outcomes in high-risk adult patients, including those with ocular surface diseases and multiple graft rejections, and in pediatric patients with congenital corneal opacities are disappointing. RECENT DEVELOPMENTS: Regrettably, no significant clinical developments have been achieved in the field of corneal transplantation since the introduction of steroids for graft rejection. Furthermore, obtaining donor corneal tissues and eye banking, particularly in the developing countries where corneal blindness is most prevalent, are problematic. Although the postoperative complications may be severe and limit the use of currently available devices, keratoprosthesis--artificial corneal implantation--has a role in the management of corneal blindness in carefully selected patients with complex ocular diseases who are at high risk for graft failure. SUMMARY: This article reviews the recent ophthalmic literature published on the current concepts and techniques of keratoprosthesis surgery.
OBJECTIVE: To evaluate the short-term efficacy and safety of topical cyclosporin A 0.05% in the treatment of patients with severe, steroid-resistant atopic keratoconjunctivitis (AKC). DESIGN: Multicenter, placebo-controlled, double-masked, randomized trial. PARTICIPANTS: Twenty-two patients with AKC refractory to topical steroid treatment. INTERVENTION: Patients were randomly assigned (1:1) to treatment with topical 0.05% cyclosporin A eyedrops or a placebo (artificial tears) for a period of 28 days, 6 times daily during the first 2 weeks and 4 times daily during the last 2 weeks. MAIN OUTCOME MEASURES: Symptoms (itching, tearing, discomfort, mucous discharge, and photophobia) and signs (bulbar conjunctival hyperemia, upper tarsal conjunctival papillae, punctate keratitis, corneal neovascularization, cicatrizing conjunctivitis, and blepharitis) of AKC recorded on the day of enrollment and at the end of the treatment period. RESULTS: A composite score computed by summing the severity grade over all 5 symptoms and 6 signs of AKC for each patient indicated a greater improvement in the cyclosporin A group relative to the placebo at the end of the 4-week treatment period (P = 0.048 and P = 0.002, for symptoms and signs, respectively). No adverse effects of the treatment with cyclosporin A 0.05% eyedrops were observed. CONCLUSIONS: Topical cyclosporin A 0.05% seems to be safe and have some effect in alleviating signs and symptoms of severe AKC refractory to topical steroid treatment.
OBJECTIVE: To evaluate the relationship between associated medical conditions and scleritis-particularly, the timing of the diagnosis of these diseases. DESIGN: Retrospective case series. PARTICIPANTS: Patients with scleritis presenting to a single center over an 18-year period. METHODS: Medical records were reviewed for the presence of an associated infectious or rheumatic disease and for the timing of the diagnosis of the systemic disease relative to the presentation for evaluation of the scleritis. MAIN OUTCOME MEASURES: Presence of an associated medical condition and timing of diagnosis relative to that of scleritis. RESULTS: In a series of 243 patients with scleritis, 44.0% had an associated medical condition: 7.0%, an infection, and 37.0%, a rheumatic disease. The most frequent infection was herpes zoster, and the most frequent rheumatic disease was rheumatoid arthritis, present in 4.5% and 15.2% of patients, respectively. Of the 107 patients with an underlying disease, 77.6% had a previously diagnosed disease, 14.0% had their conditions diagnosed as a result of the initial evaluation, and 8.4% developed a systemic disease during follow-up. Systemic vasculitis was less likely to have been previously diagnosed than other rheumatic diseases (59.1% vs. 83.8%, P = 0.015) and more likely to be diagnosed by the initial diagnostic evaluation (27.3% vs. 8.8%, P = 0.027). Ten patients (4.1%) had a positive antineutrophil cytoplasmic antibody (ANCA) test result without clinical evidence of a systemic vasculitis. Four of 5 patients with a positive cytoplasmic ANCA test result but no clinical evidence of systemic vasculitis required immunosuppressive drugs for control of the scleritis, whereas 1 of the 5 patients with a positive perinuclear ANCA test result required immunosuppressive drugs. Among patients with no evident systemic disease after the initial diagnostic evaluation, the rate of occurrence of a rheumatic disease was 4% per person-year. CONCLUSIONS: Although associated systemic diseases are frequent among patients with scleritis, the majority are previously diagnosed. Systemic vasculitis is less likely than other rheumatic diseases to have been previously diagnosed. Because vasculitis is a potentially life-threatening disorder, it should be a focus of the diagnostic evaluation.
OBJECTIVE: To investigate the role of herpes viruses in the etiology of serpiginous choroiditis. DESIGN: Interventional case report. PARTICIPANT: A 59-year-old male patient with long-term history of serpiginous choroiditis. INTERVENTION: The patient's affected eye was obtained during autopsy. Polymerase chain reaction was performed in the microdissected choroidal tissues. RESULTS: Histopathologic examination demonstrated active inflammation with lymphocytic infiltration of the choroid. No viral DNA was amplified using pairs of herpes simplex virus (HSV) P1/P2 (for HSV-1, HSV-2, Epstein-Barr virus [EBV], cytomegalovirus [CMV] and human herpes virus [HHV]-8), and varicella-zoster virus [VZV] P1/P2 (for VZV, HHV-6, HHV-7) in the infiltrating lymphocytes or choroidal tissues. CONCLUSIONS: The current observation suggests a lack of a role for herpetic viral etiology in the etiopathogenesis of serpiginous choroiditis.
PURPOSE: MRL/MpJ-fas+/fas+ (MRL/+) and MRL/MpJ-fas(lpr)/fas(lpr) (MRL/lpr) mice undergo spontaneous development of inflammation of the lacrimal and salivary glands, similar to that in the human disorder Sjögren's syndrome. Previous work has shown that these lesions appear to be largely T helper (Th)-2-driven, as evidenced by the substantially greater expression of IL-4 than interferon-gamma. The relative contributions of selected chemokines associated with Th1 and Th2 immune responses were assessed. METHODS: Lacrimal glands from MRL/+ and MRL/lpr mice, at ages 1.5 through 9 months were evaluated by immunohistochemistry for the chemokines monocyte chemoattractant protein (MCP)-1 (also known as chemokine ligand [CCL]-2), MCP-5 (CCL12), thymus activation regulated chemokine (TARC; or CCL17), and macrophage-derived chemokine (MDC; or CCL22). Additional lacrimal glands were tested by real-time RT-PCR for chemokines MCP-1 and -5, which are associated with Th2 and Th1 responses, respectively. RESULTS: By immunohistochemistry a significantly greater proportion of mononuclear inflammatory cells in the lacrimal gland lesions stained for MCP-1 (29%-48% depending on age) compared with MCP-5 (1%-3% depending on age) both in MRL/+ (mean difference 34.2%, P < 0.001) and MRL/lpr (mean difference 33.6%, P < 0.001) substrains. Real-time RT-PCR studies showed higher transcript levels of MCP-1 compared with MCP-5, in both MRL/+ (median difference, 37.3; P < 0.0001) and MRL/lpr (median difference, 77.1; P < 0.0001) mice. Relative transcripts of MCP-1 increased with age in both MRL/+ mice (P = 0.02) and MRL/lpr mice (P = 0.03). Staining for TARC was present on lacrimal gland ductular cells but not on the infiltrating lymphocytes, and staining for MDC was present on scattered individual cells throughout the lacrimal gland, but not on infiltrating lymphocytes. CONCLUSIONS: The predominant expression of a Th2-associated chemokine in the lacrimal gland lesions in this murine model of Sjögren's syndrome may contribute to the predominantly Th2-type lymphoid infiltrate in these tissues.
PURPOSE: MRL/MpJ-fas(+)/fas(+) (MRL/+) and MRL/MpJ-fas(lpr)/fas(lpr) (MRL/lpr) mice are congenic substrains of mice that have spontaneously developing lacrimal and salivary gland inflammation and are models for the human disorder Sjögren's syndrome. Nitric oxide (NO) and tumor necrosis factor (TNF)-alpha are proinflammatory and potential mediators of tissue damage. The presence of the inducible form of nitric oxide synthase (iNOS), which catalyzes the production of NO, and the presence TNF-alpha in the lacrimal glands of MRL/MpJ mice were assessed. METHODS: Lacrimal glands from MRL/+ and MRL/lpr mice, at ages 1 through 9 months, were evaluated by real-time RT-PCR for iNOS and TNF-alpha mRNA and by immunohistochemistry for the presence of iNOS and of TNF-alpha. Age-matched BALB/c lacrimal glands were used as the control. RESULTS: By quantitative real-time PCR (qPCR), mRNA for iNOS was detected in the lacrimal glands in significantly greater amounts in both MRL/+ (median, normalized to 18S rRNA, 2.90; P < 0.0003) and MRL/lpr mice (median 6.84, P < 0.001) than in BALB/c mice (median 0.34). By qPCR, mRNA for TNF-alpha in the lacrimal glands was detected in significantly greater amounts in aged MRL/+ mice than in BALB/c mice (median, normalized to actin, 221.8 vs. 77.8, P = 0.011) and in MRL/lpr mice than in BALB/c mice (median 136.7 vs. 72.5, P = 0.001). Immunohistochemistry demonstrated both iNOS and TNF-alpha in scattered mononuclear cells throughout the lacrimal glands and in mononuclear cells at the junction of the focal inflammatory infiltrates and normal acinar tissue in both MRL/+ and MRL/lpr mice. CONCLUSIONS: As demonstrated by the greater presence of iNOS and TNF-alpha in the lacrimal glands of MRL/MpJ mice than in control glands, both NO and TNF-alpha are potential mediators of lacrimal gland damage in these murine models of Sjögren's syndrome.
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PURPOSE: To evaluate the long-term outcomes of combined penetrating keratoplasty with iris-sutured posterior chamber intraocular lens implantation. PARTICIPANTS: Patients (264 eyes) with aphakic and pseudophakic bullous keratopathy. DESIGN: Retrospective case series. INTERVENTION: Penetrating keratoplasty combined with implantation of an iris-sutured posterior chamber intraocular lens performed by a single surgeon. METHODS: Data were gathered from the institutional database during a period of 10 years (1989-1999) and analyzed using customized software. MAIN OUTCOME MEASURES: Graft survival rate, visual acuity, and intraoperative and postoperative complications. RESULTS: Two-hundred fifty-two consecutive patients (264 eyes) were operated. Graft survival rates were 95% at 1 year, 89% at 2 years, and 81% at 5 years and were comparable to the results of previous corneal transplant studies. Among 180 patients (191 eyes) with a follow-up of at least 2 years (range, 2-11 years; mean, 5 years), the visual acuity at final follow-up visit, compared with preoperative visual acuity was improved in 72% of eyes (mean preoperative visual acuity, 20/250; mean postoperative visual acuity, 20/60). No intraoperative complications were encountered. CONCLUSIONS: Combined penetrating keratoplasty with iris-sutured posterior chamber intraocular lens implantation offers significant vision benefits in this patient group.
Serpiginous choroiditis is a bilateral, inflammatory condition of the inner choroid and overlying retinal pigment epithelium with a distinctive retinal distribution. Although rare, serpiginous choroiditis deserves attention because it can lead to permanent loss of vision due to foveal involvement by inflammation or choroidal neovascularization from frequent recurrences. This article reviews the recent ophthalmic literature published on the management of serpiginous choroiditis.
OBJECTIVE: To describe the management and long-term outcomes of patients with serpiginous choroiditis treated with alkylating agents. DESIGN: Retrospective, noncomparative case series. PARTICIPANTS: Nine patients with active, vision-threatening serpiginous choroiditis who had progressive inflammation while on steroids and/or immunosuppressive agents other than alkylating agents treated at three tertiary care uveitis referral centers. METHODS: Patients received systemic immunosuppression with an alkylating agent, either chlorambucil or cyclophosphamide. Prednisone also was given initially and was tapered and discontinued. MAIN OUTCOME MEASURES: Visual acuity, clinical disease activity, duration of treatment, duration of drug-free disease remission, and side effects of alkylating agent therapy. RESULTS: No patients had recurrences while on therapy. No further visual loss was encountered after starting the therapy. Six of the patients regained vision. All but two patients achieved prolonged drug-free remissions, ranging in duration between 15 and 96 months (median, 78 months). Side effects included transient bone marrow suppression, nausea, and fatigue. Secondary malignancy was encountered in one patient, whose carcinoma of the urinary bladder was treated successfully. CONCLUSIONS: Adequate immunosuppression with alkylating agents may favorably alter the long-term prognosis of patients with serpiginous choroiditis.