Lori Levin

Graves Exophthalmos Unrelated to Extraocular Muscle Enlargement: Superior Rectus Muscle Inflammation May Induce Venous Obstruction

Exophthalmos is the most commonly measured sign of Graves ophthalmopathy, whereas enlargement of the extraocular muscles is the principal pathologic abnormality. The purpose of this article is to explore possible etiologies of increased volume of orbital fat and of proptosis in patients with no substantially increased total extraocular muscle volume. Computed tomographic scans of the 13 orbits reviewed in this study had the following characteristics in common: a fine, reticular pattern within the orbital fat, a prominent superior ophthalmic vein, and an enlarged superior rectus muscle. Quantitative analysis revealed that superior rectus muscle volume showed a statistically significant correlation with proptosis, whereas medial, lateral, and inferior rectus muscle volumes did not correlate with proptosis. Based on anatomic considerations, the authors postulate that superior rectus muscle enlargement alone may produce reduced venous outflow from the orbit, thereby expanding the apparent orbital fat volume and producing proptosis.

The relation of Graves’ ophthalmopathy to circulating thyroid hormone status

Aim: The risk factors and epidemiological data for Graves’ ophthalmopathy with and without abnormal circulating thyroid levels were examined to determine the relation of thyroid dysfunction to ophthalmopathy. Methods: The authors retrospectively evaluated 482 patients seen with Graves’ ophthalmopathy. Of these, 413 were classified as having abnormal levels of circulating thyroid hormone (ALTH) and 69 as having normal levels of circulating thyroid hormone (NLTH). Results: Patients in the NLTH group, compared to the ALTH group, were older on average (56 (SD 13.5) v 52 (15.4)) and had a higher age adjusted body mass index (26.1 (0.8) v 23.4 (0.3)). In addition, a higher percentage of NLTH patients had hypercholesterolaemia. Those in the ALTH group were more likely to be female (76% v 51%), to have a family history of thyroid problems, and to have had eye surgery. Conclusion: NLTH and ALTH appear to differ from each other in terms of risk factors and epidemiological characteristics. Additionally, thyroid dysfunction seems to be associated with a more severe ophthalmopathy compared to the euthyroid state.

Visual Fields at Follow-up in the Ischemic Optic Neuropathy Decompression Trial. Evaluation of Change in Pattern Defect and Severity Over Time

PURPOSE To evaluate change from baseline to 12 months follow-up in study and nonstudy (fellow) eye visual fields from the Ischemic Optic Neuropathy Decompression Trial (IONDT). DESIGN Randomized controlled trial and observational study. PARTICIPANTS The IONDT enrolled patients >or=50 years with acute nonarteritic ischemic optic neuropathy (NAION). Randomized patients (n = 258) had visual acuity <or=20/64; nonrandomized patients (n = 160) had visual acuity >20/64 or refused randomization. INTERVENTIONS Optic nerve decompression surgery (n = 127) or careful follow-up (n = 131). MAIN OUTCOME MEASURES We measured visual fields at baseline and at 6 and 12 months follow-up. Using a computerized system, we classified visual field defects by pattern, location, and severity. We examined changes over time by treatment group, age, baseline comorbidities, and change in visual acuity. In fellow (nonstudy) eyes, we assessed change by whether NAION was present at baseline and also incidence of NAION by whether a visual field defect was present at baseline. RESULTS We analyzed 245 study eye visual field pairs (179 and 66, randomized and nonrandomized, respectively) for change from baseline to 12 months. We observed significant changes in defect distribution within the central field (P = 0.02) for randomized eyes. Superior and inferior altitudinal defects were less severe at follow-up in both randomized and nonrandomized eyes. We observed an association between change in central field severity and change in visual acuity from baseline (P<0.001 at 6 months; P = 0.01 at 12 months; Kendalls tau-b), but no association between visual field change and treatment group, age, or baseline comorbidities. Superior and inferior visual field defects present at baseline in nonstudy eyes improved at follow-up. Fellow (nonstudy) eyes with normal fields did not have an increased risk of developing NAION compared with eyes with >or=1 defects. CONCLUSIONS Visual fields of NAION patients enrolled in the IONDT were relatively stable from baseline to follow-up. A visual field defect in the nonstudy eye at baseline was not associated with development of NAION during follow-up compared with eyes with normal fields.

Occurrence of familial nonarteritic anterior ischemic optic neuropathy in a case series

OBJECTIVE To report on the occurrence of familial nonarteritic anterior ischemic optic neuropathy (NAION) in our NAION series. METHODS One hundred forty-eight consecutive retrievable cases of NAION were surveyed regarding the occurrence of NAION in other family members. Medical records of affected family members were reviewed, and clinical characteristics of documented familial NAION cases were described. RESULTS Of 79 patients who returned the survey, four reported one or more relatives with previously diagnosed NAION. There were nine cases of documented NAION in these four families. All cases occurred in siblings, with a mean age at onset of 55 years. Six patients had second eye involvement and in five, involvement became bilateral within 4 years after initial onset. None of the patients had diabetes; two had hypertension. CONCLUSION A small number of patients with NAION may belong to a familial subclass. Three previous reports of familial NAION further support this hypothesis.

One-year results of a pilot study using oral 13-cis retinoic acid as a treatment for subfoveal predominantly occult choroidal neovascularization in patients with age-related macular degeneration.

Purpose: To evaluate the safety and evidence of efficacy for oral 13-cis retinoic acid as a treatment for patients with subfoveal occult choroidal neovascularization (CNV) due to age-related macular degeneration (ARMD). Methods: Patients with active, subfoveal occult CNV with no prior treatment of the subfoveal component were eligible for inclusion. Patients received 40 mg of 13-cis retinoic acid twice daily for 5 months, stopped treatment for 2 months, and then resumed treatment for 5 months. Patients were observed monthly with Early Treatment Diabetic Retinopathy Study (ETDRS) visual acuity (VA), clinical examination, fluorescein angiography, and laboratory testing. Results: Eleven patients, aged 64 to 88 years, were enrolled and followed for 1 year. Initial VA ranged from 55 (20/40) to 5 (20/400) ETDRS letters (median 48 letters). Mild drug-related side effects (dry skin, chapped lips) occurred in all 11 patients. Three patients experienced more severe side effects (muscle aches, mood swings) and did not resume treatment after the drug holiday. Moderate VA loss occurred in 36% at both 6 and 12 months. Conclusions: Oral 13-cis retinoic acid is too toxic to be useful in patients with ARMD. Oral 13-cis retinoic acid did not improve vision although it may have slowed visual acuity loss in patients with ARMD with occult subfoveal CNV.