Paramacular coloboma.

BACKGROUND: Paramacular coloboma (plural: colobomata) is a solitary oval football or torpedo-shaped chorioretinal lesion located temporal to the fovea in one or both eyes. Previous case reports have speculated varying etiology, but few have justified its pathognomonic shape and location. We believe it to be congenital in nature and caused by incomplete differentiation of the arcuate bundles along the horizontal raphe in development of the macular architecture. Associated ocular findings may include blepharophimosis, situs inversus, or other anomalous retinal vascular patterns. CASE REPORTS: Three cases of asymptomatic unilateral paramacular colobomata are presented. In each case, a single oval chorioretinal lesion temporal to the macula was found during routine examination. Visual acuity and Humphrey threshold visual field testing were normal with no other associated congenital, systemic, or ocular abnormalities. Because the lesion is nonprogressive, these patients can be followed on an annual basis. CONCLUSION: Due to their anatomical origin, paramacular colobomata are always located temporal to the macula and have an oval football-shaped appearance. Visual acuity and visual field testing are usually normal, although highly observant patients may be aware of a mild scotoma. Differential diagnosis is important because the clinical appearance can be similar to acquired conditions, most notably age-related macular degeneration and presumed ocular histoplasmosis syndrome.

Juxtapapillary capillary hemangioblastoma.

BACKGROUND: Juxtapapillary capillary hemangioblastomas are vascular lesions that occur on the optic nerve head or immediately adjacent to the optic disc. Although juxtapapillary capillary hemangioblastomas may appear as an isolated clinical finding, they are more likely to be a precursor to the diagnosis of von Hippel-Lindau (VHL) disease. When associated with VHL, the ocular complications from hemangioblastoma are generally greater, and subsequently the prognosis is poorer than for isolated entities. CASE REPORT: A 67-year-old white male presented to our clinic complaining of decreased vision in both eyes at distance and near. A dilated fundus examination revealed a swollen left optic nerve. Visual field testing revealed an inferior arcuate defect in the left eye. Laboratory and radiological testing were normal. Fluorescein angiography showed filling during the retinal arterial phase with late diffuse leakage. Based on these results, a diagnosis of juxtapapillary capillary hemangioblastoma was made. This was later confirmed when fundus photographs taken 7 years earlier were obtained, revealing a stable appearance to the nerve. CONCLUSION: Potential ocular complications of juxtapapillary capillary hemangioblastomas include vision loss secondary to subretinal and intraretinal fluid, as well as epi-retinal membrane formation and, rarely, exudative retinal detachment. Management ranges from observation to surgical intervention, although definitive treatment guidelines have yet to be established. In addition, it is important for the eye care practitioner to be aware of the association with VHL disease, so that patients may be appropriately referred and managed for other possible systemic complications of the disease.

Ocular and systemic findings and their correlation with hemodynamically significant carotid artery stenosis: a retrospective study.

BACKGROUND: Optometrists often encounter patients with ocular signs and/or symptoms suggestive of carotid artery disease, but criteria for eye care practitioners concerning when to order carotid studies are not well established. A retrospective study in an optometry clinic was performed to determine if certain ocular findings and associated systemic risk factors were associated with hemodynamically significant carotid artery stenosis (HSCAS). METHODS: A retrospective analysis was performed on all patients examined in the optometry clinic at the Sepulveda Ambulatory Care Center from January 1, 1998 through December 31, 1999 to identify all patients who had carotid studies ordered. Charts were then reviewed to determine the ocular finding that prompted the carotid study. Statistical analysis using an odds-ratio was performed to determine whether any ocular sign/symptom or systemic risk factor was associated with HSCAS. RESULTS: Of 3822 patients, 48 (1.26%) had carotid studies ordered. Eight (17%) had HSCAS of >50% and symptomatic patients were 1.6 times more likely to have HSCAS than asymptomatic patients. Patients with HSCAS were 1.8 times more likely to have retinal vascular occlusions, 1.9 times more likely to have normotensive glaucoma, 2.4 times more likely to have peripheral retinal hemorrhages, and 2.6 times more likely to be smokers, although none of these factors were found to be a statistically significant indicator of HSCAS. However, the number of systemic diseases tended to be greater for the HSCAS patients compared with the non-HSCAS patients (HSCAS median = 3.5 and the non-HSCAS median = 2, p = 0.049). CONCLUSION: Although no single ocular or systemic risk factor was found to be a statistically significant indicator of HSCAS, the number of systemic disease risk factors tended to be greater for the HSCAS patients compared with the non-HSCAS patients (p = 0.049). An extensive literature review was also performed to help establish guidelines for when to order carotid studies on the basis of ocular signs and/or symptoms as well as the presence of additive associated systemic risk factors.