Phakic patients with cystoid macular edema, retinal periphlebitis, and vitreous inflammation.

OBJECTIVE: To characterize a group of phakic patients with idiopathic intermediate uveitis as defined by vitritis, cystoid macular edema, and retinal periphlebitis. DESIGN: Cross-sectional study. PARTICIPANTS: Nineteen phakic patients (35 eyes) with vitreous inflammation, cystoid macular edema, and/or retinal periphlebitis of unknown cause. INTERVENTION: None. MAIN OUTCOME MEASURES: Best-corrected final visual acuities, standardized clinical examinations, photographic and fluorescein angiographic evaluations, and class I and II HLA analysis on all 19 patients. RESULTS: Fifteen of the 19 patients were women. The mean age was 38 years, the mean follow-up was 104 months, and the mean duration of symptoms was 154 months. All 35 affected eyes had significant vitritis; 21 eyes (60%) had cystoid macular edema, 21 eyes (60%) had retinal periphlebitis. The median initial visual acuity was 20/30. The median final visual acuity was 20/20 with 32 (91%) of 35 eyes having 20/40 or better visual acuity at the final visit. No patient developed "snow-banks" or evidence of systemic disease, including multiple sclerosis or sarcoidosis, during the follow-up period. There were no statistically significant HLA associations in these patients compared with controls from another study from Iowa, but the Iowa phakic patients with cystoid macular edema did differ from the Iowa patients with pars-planitis at loci HLA-B8, HLA-B51, and HLA-DR2. CONCLUSIONS: We describe a disease entity of idiopathic intermediate uveitis that affects primarily young to middle-aged women and usually causes bilateral vitritis, cystoid macular edema, and retinal periphlebitis. Most patients retained good vision over a prolonged follow-up period. Multiple sequential examinations and HLA associations suggest that these conditions are distinct from other syndromes of intermediate uveitis, particularly parsplanitis.

Systemic small noncleaved cell lymphoma presenting as a posterior choroidal mass.

PURPOSE: To describe a patient with intraocular involvement by systemic, small noncleaved cell lymphoma. METHODS: Case report and review of the literature. RESULTS: A patient presented with a diffusely elevated choroidal mass. Systemic evaluation led to the diagnosis of unsuspected disseminated lymphoma. CONCLUSION: Small noncleaved cell lymphoma should be included in the differential diagnosis of a choroidal mass.

Familial internal limiting membrane dystrophy. A new sheen retinal dystrophy.

OBJECTIVE: To describe the clinicopathologic features of a previously unreported retinal dystrophy. METHODS: Fourteen members of a single family were examined. The medical records of 2 additional family members were reviewed. Pathologic examination was performed on 2 eyes of 1 affected patient. RESULTS: Five individuals were identified with a retinal dystrophy characterized by a glistening inner retinal surface throughout the posterior pole. Visual loss occurred in 3 affected patients in later life owing to superficial polycystic retinal edema and retinal folds. Electroretinographic testing revealed a selective diminution of the b wave. Pathologic examination revealed an abnormal internal limiting membrane with schisis cavities in the inner retina. Endothelial cell swelling, pericyte degeneration, and basement membrane thickening were present in retinal capillaries. CONCLUSIONS: A previously unreported sheen retinal dystrophy is described. Pedigree analysis suggests an autosomal dominant mode of inheritance. A primary defect in Müller cells is the suspected, but unproved, cause. No effective treatment for the associated visual loss is known. The term familial internal limiting membrane dystrophy is proposed to describe this condition.

Bilateral visual function after macular hole surgery.

PURPOSE: In this study, the authors apply objective measures of bilateral visual function to investigate the benefits of macular hole surgery to overall visual function. METHODS: Anatomic and visual acuity outcomes were reviewed for patients undergoing primary macular hole surgery at one institution during a 3-year period. Several measures of bilateral visual function were defined to determine the impact of surgery on bilateral visual function. Patient data were analyzed in two separate cohorts based on whether the fellow eye was normal (visual acuity > or = 20/40, fellow eye normal group) or abnormal (visual acuity <20/40, fellow eye abnormal group) at baseline to determine the effect on visual function improvement. RESULTS: The rim of subretinal fluid resolved after surgery in 85% of patients and 82% gained two or more Early Treatment Diabetic Retinopathy Study lines of visual acuity. After surgery, visual acuity in the surgical eye was better than or equal to the fellow eye in 35%, and average visual impairment according to the American Medical Association Guidelines for Disability decreased from 29% to 17%. Among the FEA group, vision in the surgical eye was better than or equal to that of the fellow eye in 70%, and visual impairment was reduced from 52% to 35%. CONCLUSION: Bilateral visual function was improved after macular hole surgery. The improvement rate was markedly better in patients with subnormal vision in the fellow eye.