Retinal astrocytic hamartoma.

BACKGROUND: Retinal astrocytic hamartomas are glial tumors of the retinal nerve fiber layer that arise from retinal astrocytes. Classically they appear as a cream-white, well-circumscribed, elevated lesion that may present as multiple or solitary sites. A lesion is commonly seen with a multilobulated, "mulberry" appearance, but can also appear flat and semitranslucent. It is most frequently associated with tuberous sclerosis (TS) but may also be found rarely in patients with neurofibromatosis. Although the finding may point toward a systemic association, it can also be found incidentally on retinal examination as an isolated presentation. CASE REPORTS: Two cases of solitary retinal astrocytic hamartomas without systemic complications discovered at routine examination are presented. Both patients were found to have a raised, multilobulated retinal lesion consistent with the appearance of a retinal astrocytic hamartoma. In both cases, the patients had no personal or family history of seizures or any signs of unusual dermatologic lesions. B-scan ultrasonography, fluorescein angiography, and magnetic resonance imaging (MRI) were performed as necessary. CONCLUSION: A retinal astrocytic hamartoma is typically associated with TS but can also present as a spontaneous, idiopathic lesion. Prompt referral to rule out TS or other systemic associations, particularly in young children, is important. Neuroimaging, along with other diagnostic tests, may ensure the proper management and rule out need for further evaluation. Spontaneous lesions without systemic association can initially be monitored closely to rule out possible progression. Once stability has been established, annual follow-up with a primary care eye practitioner is appropriate.

Iritis presumed as secondary to disseminated coccidioidomycosis.

BACKGROUND: Coccidioidomycosis is a systemic disease caused by a fungus found in soil and transmitted through inhalation. It is prevalent in western and southwestern United States, Mexico, and South and Central America. Results of skin testing, serologic testing, and tissue cultures confirm the diagnosis. Coccidioidomycosis can manifest in various ways: the infected individual may present asymptomatically, with an acute respiratory infection, or, in more severe or chronic cases, with a multiorgan presentation. Ocular involvement may include anterior segment, posterior segment, or extraorbital involvement. CASE REPORT: A case concerning a patient with iritis presumed as secondary to disseminated coccidioidomycosis is discussed. The patient initially presented to our clinic with signs and symptoms of acute, unilateral iritis and a recent history of iritis in the contralateral eye. The active inflammation was treated topically with Pred Forte and cyclopentolate and resolved without sequelae. Because the presentation was bilateral with an asymmetric timecourse, laboratory tests were ordered to rule out systemic association. Because all tests yielded negative results, the known history of disseminated coccidioidomycosis was presumed to be the etiology of this iritis. CONCLUSION: Although eye findings are rare, disseminated coccidioidomycosis is an important differential to consider when a patient presents with uveitis. For this reason, awareness and recognition of ocular signs and symptoms of this disease is significant in proper patient care and management.

Idiopathic sclerochoroidal calcification.

BACKGROUND: Idiopathic sclerochoroidal calcification (ISC) is an uncommon condition of calcium deposition at the level of the sclera and choroid of the posterior pole. With normal acuity and visual field, it typically manifests in asymptomatic older males. It can be diagnosed on clinical grounds, appearing as multiple geographic, yellowish, placoid-like lesions in the sclera and choroid, commonly found in the superotemporal arcade of the midperipheral fundus in both eyes. Ocular ultrasound and fluorescein angiography have classic results. CASE REPORT: An 85-year-old white man came to us for routine examination. Best-corrected visual acuity was 20/40 O.D. and O.S. Dilated fundus examination revealed several midperipheral yellowish-white deposits with retinal pigment epithelium hyperpigmentation temporally in each eye. Ocular ultrasound revealed high reflectivity consistent with calcium. The lesions demonstrated mild hyperfluorescence in the late phase of fluorescein angiography. Laboratory testing was void of abnormal calcium or phosphorous metabolism, and a diagnosis of ISC was made. CONCLUSION: Idiopathic sclerochoroidal calcification is a benign lesion of calcium deposition in the posterior pole, often discovered on routine examination. Although it is idiopathic, systemic evaluation is warranted to rule out dystrophic or metastatic calcification by various pathological conditions associated with abnormal calcium-phosphorus metabolism--most notably, hyperparathyroidism. Differential diagnoses include choroidal osteoma, melanoma, and metastatic carcinoma.

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.