1/7. Ocular changes in mucopolysaccharidosis iv A (Morquio A syndrome) and long-term results of perforating keratoplasty.BACKGROUND: The mucopolysaccharidoses (MPS) are an inhomogeneous group of disorders of errors in the carbohydrate metabolism with severe ocular involvement (corneal opacification, retinal degeneration, optic atrophy). PATIENT PRESENTATION: We report on a boy aged 12 years, with Morquio A (MPS IV A) syndrome. Ocular findings: progressive pseudoexophthalmus due to shallow orbits, increasing corneal stromal clouding, intermittent dissociated manifest nystagmus of the left eye, nyctalopia. visual acuity OD cc = 0.16, OS cc = 0.05. electrophysiology: changes suggesting a symptomatic tapetoretinal degeneration and optic atrophy. TREATMENT AND COURSE OF disease: OS: perforating keratoplasty. Postoperative improvement of visual acuity to 0.25 for nearly a year, followed by progressive reopacification of the corneal graft. Both eyes: progressive signs of tapetoretinal degeneration and optic atrophy. visual acuity now reduced to OD 0.05, OS 0.1. CONCLUSIONS: Success of a keratoplasty is limited by (1) reopacification of the cornea, (2) visual impairment due to (a) retinal degeneration and (b) optic atrophy. The indication for perforating keratoplasty has to be thought about very carefully in these multimorbid patients. In our patient, beside progressive visual impairment there is a progressive deafness which dominates his social and school life. Attending school is severely complicated by the double handicap. Perforating keratoplasty enabled the boy to attend a school for physically handicapped without a special low-vision care for another year. Progressive visual loss without further treatment options now renders optical and electronic low-vision aids necessary. Although the time of improved visual acuity lasted less than a year, we think patients with a life expectancy of less than 20 years should have every possible improvement of their situation - even if it does not last permanently. We therefore propose perforating keratoplasty in spite of insufficient long-term results.- - - - - - - - - - ranking = 1keywords = error (Clic here for more details about this article) |
2/7. Haze after laser in situ keratomileusis in eyes with previous photorefractive keratectomy.In cases with previous photorefractive keratectomy (PRK), there is a risk of developing severe haze after laser in situ keratomileusis (LASIK). We report 3 patients (4 eyes) who developed severe haze after LASIK treatment for residual myopia following PRK. Both PRK and LASIK procedures were performed using the VISX 20/20 excimer laser. We report the grade of haze, amount of regression, and visual acuity after the patients were treated with topical steroids. In 2 eyes, the uncorrected visual acuity was 1.0 after 1 year with grade I haze. In the other 2 eyes, there was a residual refractive error, and the best spectacle-corrected visual acuity was 0.7 with grade II haze.- - - - - - - - - - ranking = 1106.9948282809keywords = refractive error, error (Clic here for more details about this article) |
3/7. Recessively inherited, late onset spondylar dysplasia and peripheral corneal opacity with anomalies in urinary mucopolysaccharides: a possible error of chondroitin-6-sulfate synthesis.Two male and two female sibs with an unusual form of spondyloepiphyseal dysplasia were reported. The main clinical features were low stature, moderate shortness of trunk and neck, abnormal span: height ratio, low-normal UBS: LBS ratio, and peripheral corneal punctate opacities only seen by the slitlamp. Normal mental status was present. Typical metachromatic granules were not seen either in bone-marrow cells or in peripheral blood cells. The X-ray picture showed spondylar and pelvic dysplasia. Qualitative rather than quantitative anomalies were shown in the urinary mucopolysaccharides, mostly involving chondroitin-6-sulfate. The genetic data are consistent with autosomal recessive inheritance.- - - - - - - - - - ranking = 4keywords = error (Clic here for more details about this article) |
4/7. Hyperopic photorefractive keratectomy with adjunctive topical mitomycin C for refractive error after penetrating keratoplasty for keratoconus.OBJECTIVE: To present a case of photorefractive keratectomy (PRK) with adjunctive topical mitomycin C (MMC) in an anisometropic hyperopic patient after penetrating keratoplasty (PKP) for keratoconus. methods: Interventional case report, chart review, and literature review. RESULTS: A 43-year-old man with a refraction of 7.00 -4.75 x 125 in the right eye underwent PRK 10 months after PKP for keratoconus. The patient had sutures removed for 3 months and was intolerant of contact lenses. After photoablation, 0.02% MMC was applied to the corneal stromal bed. The patient was followed up daily until the epithelium closed and at 1 week, 1 month, 3 months, and 6 months postoperatively. CONCLUSIONS: To our knowledge, this represents the first reported case of the use of MMC to prevent postoperative haze after PRK for PKP in an eye with keratoconus. MMC (0.02%) applied topically to the cornea immediately after PRK is safe and effective to treat a hyperopic refractive error after PKP and prevent postoperative corneal haze formation without the risks of performing a lamellar flap into an ectatic corneal bed.- - - - - - - - - - ranking = 5534.9741414044keywords = refractive error, error (Clic here for more details about this article) |
5/7. Polysulfone corneal inlays. Adverse reactions: a preliminary report.The use of polysulfone to manufacture a lens for insertion into the corneal stroma to correct large refractive errors has been described. Two patients are presented in whom the procedure has been associated with significant loss of vision due to development of secondary changes in the cornea.- - - - - - - - - - ranking = 1106.9948282809keywords = refractive error, error (Clic here for more details about this article) |
6/7. Posterior lenticonus, corneal opacity, glaucoma and preretinal membrane in a young woman.A case of posterior lenticonus with corneal opacity, glaucoma and preretinal membrane is presented. Gonioscopic findings and slit-lamp examination suggested that angle closure due to a large volume of the lens may have caused high intraocular pressure levels. Also, refractive error, exotropia and bilateral nystagmus were accompanied by posterior lenticonus in this case. Our case demonstrates a rare combination of abnormalities involving various ocular structures.- - - - - - - - - - ranking = 1106.9948282809keywords = refractive error, error (Clic here for more details about this article) |
7/7. Deep corneal stromal opacities associated with long term contact lens wear.BACKGROUND: One male and three female long term contact lens wearers (mean age 30.3 years; range 26-33) demonstrated unusual deep corneal stromal opacities which were predominantly just anterior to Descemet's membrane. None had any history of corneal dystrophy. These opacities were more common centrally, but were also identified in the corneal periphery. methods: All patients underwent routine ophthalmic examinations and, where appropriate, slit-lamp photography and specular microscopy. RESULTS: Mean lens wear in years and hours per day was 14.3 (range 10-17) and 14.3 (range 12-16) respectively. Specular microscopy disclosed cell densities within normal limits (mean 3041.5 cells per mm2) and coefficient of variation of mean cell area; COV = 0.31. refractive errors ranged from -12.25 D to 6.25 best vision sphere and all four subjects attained at least 6/9 Snellen visual acuity. The subjects' contact lens wearing history included low water content hydroxymethylmethacrylate (HEMA) contact lenses and high water content HEMA contact lenses. Stromal opacity density was observed to diminish over a period of months on cessation of contact lens wear in two cases. CONCLUSION: The possible causes of these rarely reported opacities are discussed.- - - - - - - - - - ranking = 1keywords = error (Clic here for more details about this article) |