Lens changes in hereditary hyperferritinemia-cataract syndrome.

PURPOSE: To provide detailed description and illustration of the lens changes found in hereditary hyperferritinemia-cataract syndrome, a newly reported autosomal dominant condition. METHODS: Observational case reports. A 19-year-old man was referred for evaluation of possible hereditary hyperferritinemia-cataract syndrome. His serum ferritin level was increased at 1291 microg/L during a routine screening examination. Genetic analysis revealed mutation G51C on chromosome 19, predicting an altered iron response element in L-ferritin mRNA. Subsequent evaluation of his 46-year-old father revealed similar findings. RESULTS: Multiple breadcrumb-like nuclear and cortical lens opacities were seen in this father-son pair. These cases represent the first detailed description and illustration of hereditary hyperferritinemia-cataract syndrome cataracts in the ophthalmic literature. CONCLUSION: Hereditary hyperferritinemia-cataract syndrome can be associated with distinct breadcrumb-like lens opacities. Recognition of these characteristic cataracts may aid identification and study of patients with this unusual disorder and provide insight into mechanisms of cataract formation.

Functional improvement after phacoemulsification cataract surgery.

PURPOSE: To evaluate subjective functional visual improvement after phacoemulsification and intraocular lens (IOL) implantation and identify patient characteristics and treatment features associated with no functional improvement. SETTING: Academic referral practice, Cullen Eye Institute, Department of Ophthalmology, Baylor College of Medicine, Houston, Texas, USA. METHODS: A retrospective review was performed of the surgical results in 106 consecutive eyes of 102 patients who completed the American Society of Cataract and Refractive Surgery (ASCRS) Cataract Data Collection Form preoperatively and 1 month postoperatively. One surgeon performed all the surgery. The relationship of patient characteristics and treatment features and reported visual function was analyzed using an analysis of variance and the Wilcoxon rank sum and Fisher exact tests. RESULTS: One month postoperatively, all patients demonstrated improved visual acuity. However, 15 patients (14%) reported unimproved or decreased visual function. No statistically significant intergroup difference (P > .05) was detected in mean preoperative, postoperative, and fellow eye best spectacle-corrected visual acuities or refractive errors; reported severity of preoperative symptoms or satisfaction with vision; reported preoperative daily activity functioning or prevalence of hypertension and diabetes; type of anesthesia, lens material, or wound closure used; intraoperative or postoperative complication rates. However, patients reporting unimproved vision tended to be older and less satisfied with their quality of life and medical care than those reporting improved vision (P = .02, .03, .01, respectively). CONCLUSIONS: Self-reported visual function generally improved after phacoemulsification and IOL implantation; however, some older patients less satisfied with their quality of life and medical care reported no functional improvement despite improved visual acuity. These patients might benefit from an extended preoperative discussion of postoperative expectations.

Familial exudative vitreoretinopathy mimicking persistent hyperplastic primary vitreous.

PURPOSE: To report an unusual case of familial exudative vitreoretinopathy in an infant. METHODS: Case report. A 6-day-old girl had unilateral microphthalmia in the right eye, with a retrolental plaque initially diagnosed as persistent hyperplastic primary vitreous. Three months later, peripheral retinal vascular changes and a fibrovascular ridge were noted in the left eye, suggesting familial exudative vitreoretinopathy as the cause in both eyes. RESULTS: The microphthalmic right eye was unsalvageable. The left eye developed an exudative retinal detachment despite photocoagulation of the peripheral avascular retina. Additional cryotherapy resulted in resolution of the detachment and regression of the vascular changes. CONCLUSIONS: With highly asymmetric involvement, neonatal familial exudative vitreoretinopathy can mimic persistent hyperplastic primary vitreous. Fellow eye involvement can progress rapidly.

Changes in computerized videokeratography induced by artificial tears.

PURPOSE: To analyze the effect of several standard artificial tear preparations on computerized videokeratographic measurements. SETTING: Cullen Eye Insitute, Baylor College of Medicine, Department of Ophthalmology, Houston, Texas, USA. METHODS: We evaluated one eye each in 18 normal volunteers. Using the EyeSys Corneal Analysis System (EyeSys Technologies), we obtained corneal topographic measurements at baseline and 0.5, 1, 2, 3, 4, 5, 6, 8, and 10 minutes after instillation of the following preparations: balanced salt solution, Tears Naturale II, Tears Naturale Free, Cellufresh, Celluvisc, HypoTears, and HypoTears PF. We analyzed changes in curvature of the keratographic rings at radii 1 to 5 mm and changes in keratometric-equivalent astigmatic power and meridian. RESULTS: All preparations except HypoTears and Tears Naturale II induced statistically significant, time-dependent changes in mean corneal power in the central 5 mm corneal zone compared with baseline measurements (P < .05). The relationship between change in dioptric power over time varied with preparation type and was nonlinear in nature. In all cases, the mean induced change was < or = 0.5 diopter. Except for Celluvisc, tear administration produced minimal changes in the values of corneal astigmatic power or meridian. CONCLUSION: When performing serial measurements of mean corneal power, the greatest consistency was achieved with no tears or with instillation of HypoTears or Tears Naturale II.

Complete ophthalmoplegia after zoster ophthalmicus.

Complete ophthalmoplegia following herpes zoster ophthalmicus (HZO) is rare. We report three cases of HZO-associated complete ophthalmoplegia and review thirteen additional cases reported in the English language medical literature over the past 30 years. HZO-associated complete ophthalmoplegia occurs mostly in individuals over the age of 50 years and usually occurs within one to two weeks of the development of cutaneous HZO. The prognosis for recovery is good, with-significant improvement typically seen within 2 months and complete or near resolution within 18 months time.