Preservation Of Good Visual Acuity Research Articles

Preserved visual function in retinal dystrophy due to hypomorphic RPE65 mutations

Background/aimsTo present detailed phenotypic and molecular findings in four patients from four families with atypical, mild, recessive RPE65-related retinal dystrophy and discuss potential implications for gene replacement therapy.MethodsFour patients from...

Bilateral Ring-shaped Intrastromal Opacities After Corneal Cross-linking for Keratoconus

To report a case of bilateral, ring-shaped, intrastromal corneal opacities after corneal cross-linking (CXL) for progressive keratoconus. A 21-year-old man with bilateral, progressive keratoconus underwent CXL with riboflavin 0.1% and ultraviolet A (UVA) light in the right eye and, 6 months later, in the left eye. One day after CXL, the right eye showed a sharply demarcated white ring in the corneal periphery. The ring involved the superficial stroma at 2 mm from the limbus, extended from 1 to 11 o'clock, and was 1.5-mm wide. Corneal topography showed marked central flattening. Six months after treatment, a residual, faint, ring-shaped opacity remained, with a decrease in corneal ectasia. Cross-linking was then performed in the left eye. Different ultraviolet devices were used in the two eyes. On postoperative day 1, the left eye also showed a white ring of stromal opacity. Scheimpflug imaging showed corneal stromal contraction in the zone of whitening. At final examination, >1 year after surgery, corrected visual acuity was 0.00 logMAR with cylinder of 2.00 diopters in both eyes. The bilateral intrastromal corneal changes were innocuous in the long-term, with preservation of good visual acuity and successful stabilization of progressive keratoconus.

Spectral domain optical coherence tomography features of multiple subfoveal retinal pigment epithelial tears after intravitreal bevacizumab

Retinal pigment epithelial (RPE) tear has been described to occur spontaneously, after laser photocoagulation and in recent times, after intravitreal injection of anti-vascular endothelial growth factor (VEGF) agents. In the latter case, the rapid contraction of the choroidal vascular membrane underneath a serous RPE detachment is believed to be the underlying cause. Preservation of good visual acuity after the occurrence of RPE tear with continued use of intravitreal VEGF agents has been reported. In this case report, we describe the occurrence of multiple RPE tears with the use of intravitreal bevacizumab and also correlate the preservation of visual acuity with features seen on spectral domain optical coherence tomography.

Management of Acute Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Utilizing Amniotic Membrane and Topical Corticosteroids

To describe the results of a novel treatment approach to the acute ophthalmic management of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). Retrospective interventional case series. setting: Institutional. study population: Sixteen eyes of 8 patients with acute, biopsy-proven SJS or TEN and significant ophthalmic involvement. interventional procedure(s): Application of amniotic membrane to the ocular surface, either in the operating room or at the bedside, and short-term use of intensive topical corticosteroid medication. main outcome measures: Visual acuity, slit-lamp appearance of the ocular surface, and patients' subjective impression of ocular comfort. Two patients expired during the hospitalization. Mean follow-up time for the surviving patients was 7.7 months. Four surviving patients in whom the entire ocular surface (ie, the cornea, bulbar and palpebral conjunctiva, and eyelid margins) was treated with amniotic membrane retained visual acuities of 20/40 or better and an intact ocular surface. In contrast, the initial 2 patients in the study who were treated with only a Prokera device or unsutured amniotic membrane sheets, leaving the palpebral conjunctiva and eyelid margins uncovered, developed more significant ocular surface abnormalities, and 1 developed a corneal perforation. Amniotic membrane coverage of the ocular surface in its entirety coupled with the use of intensive short-term topical corticosteroids during the acute phase of SJS and TEN is associated with the preservation of good visual acuity and an intact ocular surface. Partial amniotic membrane coverage of the ocular surface may not serve to minimize the cicatrizing ocular sequelae of SJS and TEN as effectively as complete coverage.

Alternaria Keratitis: Clinical Presentation and Resolution With Topical Fluconazole or Intrastromal Voriconazole and Topical Caspofungin

The purpose of this study was to describe the clinical presentation of 3 cases of Alternaria keratitis and their response to medical therapy. All cases of Alternaria keratitis diagnosed and treated at the University of Illinois Eye and Ear Infirmary from 1999 to 2007 were reviewed for clinical presentation, antifungal therapy, and final visual acuity. Three cases of Alternaria keratitis were identified. All patients presented with an indolent steroid-treated keratitis and a history of recent cataract surgery, agricultural trauma, or contact lens wear. None of the patients responded to natamycin, and 1 also failed to respond to topical and systemic voriconazole. Patients responded rapidly to either topical fluconazole 0.02% or a combination of intrastromal voriconazole and topical caspofungin 0.5%. Final visual acuity ranged between 20/15 and 20/25. Alternaria keratitis has a varied clinical presentation and may present without macroscopic pigmentation. In vitro sensitivity testing may be helpful in directing therapy for Alternaria keratitis, but clinical response should be monitored as the primary endpoint. Response to natamycin was poor, but successful resolution with topical fluconazole or intrastromal voriconazole and topical caspofungin with preservation of good visual acuity was possible.

FULL-THICKNESS MACULAR HOLE FORMATION IN IDIOPATHIC PARAFOVEAL TELANGIECTASIS

To describe the macular holes in patients with idiopathic parafoveal telangiectasis (IPT) and to propose a pathophysiologic explanation for their formation. Four eyes of two patients with IPT were evaluated with biomicroscopy and optical coherence tomography (OCT). One patient had a nearly full-thickness hole with preservation of only the internal limiting membrane (ILM), but had a 20/60 visual acuity. The other patient had a large full-thickness macular hole, but retained 20/40 visual acuity. Each patient had a fellow eye showing prominent central inner foveal cavitation under a very thin ILM, which was devoid of associated tissue. This report describes the findings of two patients with IPT who developed pronounced central foveal structural abnormalities. The induced anatomic changes noted in our patients suggest that there is a loss of the structural aspects afforded by Muller cells, particularly the Muller cell cone, in the central macula in patients with IPT. The preservation of good visual acuity in our patients implies that the holes were the result of lateral separation of the photoreceptors within the fovea and that there could not have been profound atrophy of the photoreceptors.

Familial exudative vitreoretinopathy

To evaluate the natural history of Familial Exudative Vitreoretinopathy (FEVR) with emphasis on the effect of the age of onset on its severity and on the development of late complications such as cataract and retinal detachment. Also, to evaluate affected patients for DNA abnormalities.The records of thirty-nine patients with FEVR were studied. All were asked to come in for a final follow-up examination. The referring physician was asked to provide the latest findings for those who could not. On 10 patients, karyotypes were prepared.Only 2 of 28 patients whose onset of symptoms was prior to their third birthday had a final visual acuity of 20/200 or better. Older patients had a better prognosis, because they were more likely to have asymmetrical retinal deterioration with only one eye deteriorating. Preservation of good visual acuity into the teens and later was no guarantee that deterioration would not occur. In 3 eyes of 4 patients who were asymptomatic until 15 years of age, the final visual acuity was counting fingers or worse. In 5 patients, retinal detachment developed 6 to 17 years after apparent stabilization. The karyotype of 10 patients showed no evidence for rearrangement, altered size, translocations or deletions of chromosome 11 or any other chromosome. Of the 31 eyes in patients older than 15 years, 10 (32%) had a significant cataract. Three eyes underwent cataract surgery.The prognosis for infants with FEVR is extremely poor and the long-term prognosis for patients with a later onset of the condition is guarded. Retinal detachment, macular dragging, and cataract are common late complications which can develop even in patients whose eye findings appear to be stable. Karyotype studies ruled out involvement of chromosome 11 or others at a gross level, but did not exclude them at the location for some genetic defect related to FEVR because single base changes and small deletions or insertions may be undetectable by the methods utilized.

Autosomal Dominant Central Pigment Epithelial and Choroidal Degeneration

Eight of nine individuals of a four-generation family had autosomal dominant central pigment epithelial and choroidal degeneration, which was not entirely consistent with previously described central retinal and choroidal degenerations. The early loss of the choriocapillaris with a relative sparing of the sensory retinal and pigment epithelial function is similar to central areolar choroidal degeneration. Ophthalmoscopically, however, the disease resembled the dominant progressive foveal degenerations, except that our patients additionally had multiple peripheral yellow-white lesions. An exceptional finding in our patients was the preservation of good visual acuity despite marked atrophy of the choriocapillaris.

Successful Treatment of Candidaendophthalmitis With a Synergistic Combination of Amphotericin a and Rifampin

Candida endophthalmitis, caused by transient candidemia, developed in a 14-year-old white girl receiving intravenous hyperalimentation. Antifungal synergism was established in vitro for the combination of amphotericin B and rifampin against the C. albicans isolate. A combined ten-day course of intravenous amphotericin B and oral rifampin was followed by the elimination of the infection and the preservation of good visual acuity.