Faculty Bibliography

PURPOSE: To report optical coherence tomography findings in nicotinic acid maculopathy. DESIGN: Observational case report. METHOD: The patient was examined with ophthalmoscopy, fluorescein angiography, and optical coherence tomography. RESULTS: A 71-year-old man presented with bilateral decreased visual acuity with metamorphopsia. Optical coherence tomography demonstrated cystoid spaces in the outer plexiform layer and inner nuclear layer. Fluorescein angiography did not show leakage. Two weeks after stopping the nicotinic acid, the cystoid spaces resolved. CONCLUSIONS: Niacin maculopathy causes cystoid spaces in the inner nuclear and outer plexiform layers that resolve with discontinuation of the drug

BACKGROUND: Ocular manifestations of Wilms' tumor are rare, particularly in adults. We present the first reported case of a choroidal metastasis resulting from Wilms' tumor. DESIGN: Case report. PARTICIPANT: A 37-year-old white male with an adult-onset biopsy-proven Wilms' tumor with multiorgan metastatic disease. RESULTS: We report a unique case of uveal metastasis presumed from a Wilms' tumor. The patient's history included a primary Wilms' tumor with known disseminated metastasis, orbital pain, metamorphopsia, and decreased vision. Ophthalmoscopy revealed a unifocal choroidal tumor in the inferotemporal quadrant of the right eye. Ultrasonographic measurements of the tumor were 4.9 mm in apical height and 15 x 13 mm in basal diameter. Unusual ultrasound findings included echodense stromal opacities and retrobulbar edema. Fluorescein angiography revealed double circulation and late intratumoral fluorescence. CONCLUSIONS: This is the first reported case of a choroidal metastasis by a Wilms' tumor

Truly unilateral papilledema is rare and poses a diagnostic problem. The authors have prospectively looked for patients with truly unilateral papilledema and found 15 patients, 10 of whom had idiopathic intracranial hypertension. Neuroimaging did not indicate a reason for the lack of swelling in the other nerve. The visual deficits and outcomes were similar to those of patients with bilateral papilledema. Although monocular papilledema is uncommon, a lumbar puncture with opening pressure measurement should be considered

Intracranial aneurysms are rare in infancy. The commonest presentation is intracranial hemorrhage, but signs of mass effect are more frequent than in adults. We report 2 infants with cerebral aneurysms, one presenting with macrocephaly and another with strabismus. Both had visual loss and optic disc pallor; MRI revealed a suprasellar mass and anterior visual pathway compression. In both cases, the preoperative diagnosis was craniopharyngioma. It is essential to recognize that, although exceedingly uncommon, cerebral aneurysms do occur in infants and have features that differ from those in adults

The objective of this study was to determine if aspirin reduces the incidence of second eye involvement after nonarteritic anterior ischemic optic neuropathy (NAION) in one eye. Records were reviewed of 131 patients who sustained unilateral NAION. Of these, the 33 patients who sustained second eye NAION were compared to those followed for a minimum of 2 years without sustaining a second eye NAION (67). Thirty-one of the 131 patients were excluded because of inadequate follow-up. Except for diabetes (relative risk [RR] 1.43, p = 0.05), the incidence of second eye NAION was independent of gender, age, cup/disk, hypertension, anemia, and migraine. The degree of visual acuity or field dysfunction in the first eye correlated poorly with the acuity (r = 0.28) and field (r = 0.33) loss in the second eye. Aspirin (65-1,300 mg) taken two or more times per week decreased the incidence (17.5% vs. 53.5%) and relative risk (RR = 0.44, p = 0.0002) of second eye AION regardless of the usual risk factors. Even after eliminating those patients who had bilateral disease when first referred, ASA still reduced the incidence of second eye involvement (35% vs. 13%, RR = 0.74, p = 0.01). Aspirin may be an effective means of reducing second eye NAION

OBJECTIVE: To determine if moderate-or low-dose corticosteroid therapy can reduce the diplopia and frequency of deterioration to generalized disease in ocular myasthenia gravis. DESIGN: Retrospective record review. SETTING: Two university-based neuro-ophthalmology services. PATIENTS: All 32 patients with ocular myasthenia gravis, treated with prednisone, followed up for a minimum of 2 years were included. Patients were treated with 1 or more courses of daily prednisone (highest initial dose, 40-80 mg) gradually withdrawn over 4 to 6 weeks. Subsequently, in 6 patients, 2.5 to 20 mg of prednisone was given on alternate day. OUTCOME MEASURES: Diplopia in the primary position or downgaze diplopia and generalized myasthenia gravis after 2 years of follow-up. RESULTS: Diplopia, which was initially found in the primary position in 29 patients and in the downgaze position in 26 patients, was absent in 21 patients at 2 years. Generalized myasthenia gravis occurred in 3 patients at 2 years. Elevated serum acetylcholine receptor antibody levels and abnormal electromyography findings were not predictive of worsening. No patient experienced a major steroid complication. CONCLUSIONS: Moderate-dose daily prednisone for 4 to 6 weeks, followed by low-dose alternate-day therapy as needed, can control the diplopia in patients with ocular myasthenia gravis. The frequency of deterioration to generalized myasthenia gravis at 2 years may be reduced; 9.4% in this study compared with more than 40% previously reported frequency. Corticosteroids may be useful even when ocular motor dysfunction is not normalized

OBJECTIVE: To determine the hemodynamic abnormalities that result in visual acuity loss because of retinal or choroidal dysfunction associated with arteriovenous shunts (AVS) that involve the cavernous sinus. METHODS: Retrospective study of the records of 250 patients with either a carotid cavernous fistula or dural arteriovenous malformation revealed a total of 10 patients with vision loss secondary to retinopathy (group I) or choroidal effusion (group II). The cerebral angiograms of these 10 patients and 10 additional patients with visual dysfunction due to elevated intraocular pressure (group III) were evaluated to determine whether the three groups could be distinguished by a specific vasculopathic pattern. Particular attention was given to the extent of thrombosis in the ophthalmic venous system and cavernous sinus. RESULTS: There were angiographic signs of severe thrombosis in the ophthalmic vein in nine patients and in the cavernous sinus in seven patients ipsilateral to the retinal or choroidal lesion. In contrast, in group III, severe thrombosis in the ipsilateral ophthalmic vein and in the cavernous sinus occurred in one and two patients, respectively. Closure of the AVS improved the visual acuity in 1 of 4 patients in group I and in 4 of 5 patients in group II, and normalized the intraocular pressure in all patients. CONCLUSIONS: Ophthalmic vein thrombosis, rather than arterialization of the venous system or an arterial 'steal, ' is the principal cause of retinal or choroidal dysfunction associated with AVS to the cavernous sinus. Following AVS closure, visual recovery is more frequent with choroidal effusion or detachment rather than with retinopathy