Optical coherence tomography findings in ocular argyrosis.

A 68-year-old Caucasian man with a remote history of daily colloidal silver ingestion presented for ophthalmic examination in which he was noted to have a distinct slate gray skin discoloration. Funduscopy revealed confluent perimacular drusenoid deposits bilaterally, most of which localized at the level of or anterior to the inner segment ellipsoid band by optical coherence tomography (OCT) imaging. Enhanced depth imaging OCT demonstrated marked choroidal thinning. Fluorescein angiogram displayed a dark or silent choroid. Confirmatory serum silver levels were found to be markedly elevated. This report describes a unique geographic maculopathy with large drusenoid deposits anterior to the ellipsoid layer and severe choroidal thinning in association with ocular argyrosis.

Optical coherence tomography findings in ocular argyrosis.

A 68-year-old Caucasian man with a remote history of daily colloidal silver ingestion presented for ophthalmic examination in which he was noted to have a distinct slate gray skin discoloration. Funduscopy revealed confluent perimacular drusenoid deposits bilaterally, most of which localized at the level of or anterior to the inner segment ellipsoid band by optical coherence tomography (OCT) imaging. Enhanced depth imaging OCT demonstrated marked choroidal thinning. Fluorescein angiogram displayed a dark or silent choroid. Confirmatory serum silver levels were found to be markedly elevated. This report describes a unique geographic maculopathy with large drusenoid deposits anterior to the ellipsoid layer and severe choroidal thinning in association with ocular argyrosis.

Peripapillary subretinal neovascular membranes: a review.

Peripapillary subretinal neovascular membranes (PSRNVM) are most commonly associated with age-related macular degeneration and idiopathic causes in older patients. In younger patients, the condition has been linked to a wide variety of other conditions. As with the more commonly occurring macular form of choroidal neovascular membranes, PSRNVM can also lead to severe vision loss. Therefore, clinicians must take care to avoid overlooking this event to provide appropriate management and treatment. Current knowledge of PSRNVM suggests the importance of regular examinations of the affected eye in both treated and untreated cases to watch for progression and recurrence, which are unpredictable, and also of the fellow eye because there is a high risk of bilateral involvement.

Charles Bonnet syndrome.

BACKGROUND: Charles Bonnet syndrome (CBS) involves nonthreatening hallucinations in patients who have no neurological and no psychological abnormalities but with significant visual impairment secondary to ocular disease, such as macular degeneration and diabetic retinopathy. Because of the fear of a mental illness being diagnosed, patients are often reluctant to discuss these hallucinations. CASE REPORTS: Three cases are presented of patients who experienced CBS caused by decreased vision. Each patient had decreased vision and related visual hallucinations that were consistent with CBS. The first patient underwent magnetic resonance imaging and psychological evaluation, which confirmed our suspicion. The other 2 patents were not willing to undergo further testing, so our diagnosis is presumptive. CONCLUSION: Management for these hallucinations includes treatment of the actual ocular disease as well as optimizing vision for the patient using appropriate low vision devices. As the population continues to age, more patients will be seen with reduced vision caused by a myriad of ocular diseases, increasing the likelihood that more patients may present with CBS in the future. It is therefore prudent to become familiar with the syndrome so primary care optometrists can properly identify CBS and help their patients deal with it.

Retinal pigment epithelium tears after bevacizumab injection.

BACKGROUND: The advent of anti-vascular endothelial growth factor (VEGF) treatment for choroidal neovascularization has become the standard of care for wet age-related macular degeneration (AMD). Among the choices, intravitreal bevacizumab, for off-label use, has gained popularity. Recent attention has been on the complications of anti-VEGF treatment, one of them being a retinal pigment epithelial (RPE) tear. A tear in the RPE is a visually devastating complication, most commonly associated with fibrovascular pigment epithelial detachments (PED) in AMD, and may develop during the course of intravitreal bevacizumab treatment. CASE REPORT: We report a case of an 85-year-old white man with decreased visual acuities of 20/70 in the right eye (O.D.) and 20/150 in the left eye (O.S.) secondary to dry AMD O.D. and wet AMD with a fibrovascular PED O.S. The patient underwent treatment with an intravitreal bevacizumab injection O.S. Six weeks after the initial injection, the patient returned with further declining vision in the left eye secondary to an RPE tear. CONCLUSION: Intravitreal bevacizumab has proven to be an effective treatment for choroidal neovascularization and shows a significant improvement of vision for wet AMD patients. However, there are risks associated with the procedure. One of the most visually significant is an RPE tear, which can occur at an incidence rate as high as 17%. Optometrists should be aware of this rare, but serious, complication associated with anti-VEGF treatment for wet AMD.

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.

Idiopathic juxtafoveal retinal telangiectasia.

BACKGROUND: Idiopathic juxtafoveal retinal telangiectasia (IJRT) is a rare retinal condition that can be detected even in asymptomatic patients. The most common appearance, type 2A, typically presents as juxtafoveolar telangiectasias with minimal exudation, superficial retinal crystalline deposits, and right-angle venules. Though initially asymptomatic, late complications such as foveolar atrophy and subretinal neovascularization can develop and should be recognized and treated accordingly. CASE REPORT: Two patients with IJRT are presented. The first patient, a 60-year-old Hispanic man, had asymptomatic type 2A IJRT with subretinal neovascularization diagnosed for which he underwent photodynamic therapy on 2 separate occasions. Currently, his visual acuity remains stable at 20/30 in the treated eye. The second patient, a 78-year-old man, had bilateral type 2A IJRT with decreased vision. Fluorescein angiography found no active leakage, and optical coherence tomography found significant foveal atrophy. Therefore, no treatment was advised, and the patient remains with acuities of 20/100- in the right eye and 20/80- in the left. CONCLUSION: Initially, retinal signs can be subtle in type 2A IJRT, with no treatment being indicated. In the late stages of the disease, laser photocoagulation may be necessary to treat subretinal neovascular membranes, which can result as a complication, leading to dramatic vision loss if the fovea is affected. Careful inspection, including the use of fluorescein angiography and ocular coherence tomography, is often helpful in monitoring these patients.

Primary acquired melanosis of the conjunctiva.

BACKGROUND: Primary acquired melanosis (PAM) presents as a unilateral patchy area of conjunctival pigmentation mostly found in middle-aged or elderly white patients. Because PAM has the potential of becoming malignant, it is important to recognize PAM and to rule out other causes of pigmented lesions. The presence or absence of atypia is helpful in determining the potential for malignancy, because PAM without atypia is usually benign, whereas PAM with atypia may convert into a conjunctival melanoma. If atypia is present, the presence or absence of epithelioid cells and the pattern of intraepithelial growth are the main factors in determining the likelihood of neoplastic transformation. However, atypia can only be determined with histopathologic examination. Therefore, a biopsy is usually recommended. CASE REPORT: A 72-year-old white man presented for his annual examination with no visual complaints. His ocular history was remarkable for early cataracts and for a choroidal nevus. Slit lamp examination found a large dark area of elevated conjunctival pigmentation at the medial canthus that extended onto the superior bulbar conjunctiva and superior palpebral conjunctiva in the left eye. No conjunctival pigmentation had been noted previously. Results of a biopsy indicated primary acquired melanosis with atypia. The lesion was surgically excised, and the conjunctiva was reconstructed with an amniotic membrane graft. The patient was subsequently treated with topical 5-fluorouracil chemotherapy. There have been no signs of recurrence to date after his treatment. CONCLUSION: Because of the potential for malignancy, biopsies of all cases of primary acquired melanosis are indicated. If atypia is present, treatment options include local excision, cryotherapy, and topical chemotherapy.

Adult-onset foveomacular vitelliform dystrophy.

BACKGROUND: Adult-onset foveomacular vitelliform dystrophy (AOFVD) is a condition that presents classically as bilateral, symmetrical, grayish-yellow, round or oval-shaped lesions within the macular area. These lesions are mildly elevated and are typically one third to one half disc diameter in size. The onset of the disease is usually between 30 and 50 years of age with variable genetic inheritance, although some have suggested an autosomal dominance inheritance pattern. Patients with AOFVD typically present with symptoms of blurred vision or mild metamorphopsia. Results of diagnostic testing show a normal or mildly subnormal electro-oculogram (EOG). Fluorescein angiography results (FA) typically show hypofluorescence in the area corresponding to the vitelliform lesion and a surrounding ring of hyperfluorescence. Results of optical coherence tomography (OCT) show the vitelliform lesion as being located in the retinal pigment epithelium (RPE) layer or between the RPE and photoreceptor layer. CASE REPORTS: Two cases of AOFVD are presented with each patient having different macular appearances owing to the different stage of the disease process. In case 1, a 76-year-old white man presented with stage II AOFVD characterized by typical vitelliform lesions. His best-corrected acuities were 20/70+ in the right eye (O.D.) and 20/80- in the left eye (O.S.). In case 2, a 54-year-old white man presented with stage V AOFVD with bilateral atrophic maculae with best-corrected acuities of 10/60- O.D. and 10/160- O.S. CONCLUSION: Patients with adult-onset foveomacular vitelliform dystrophy typically have slow progressive vision loss. However, patients can develop dramatically decreased vision owing to subfoveal choroidal neovascularization (CNV). Thus, it is important to establish the correct diagnosis and monitor this condition. Furthermore, because there are reports of AOFVD having an autosomal dominance inheritance pattern with variable penetrance, it is recommended that the patient's family members have a comprehensive eye examination to rule out any early signs of this rare eye condition.

Treatment of cytomegalovirus retinitis with oral valganciclovir in an acquired immunodeficiency syndrome patient unresponsive to combination antiretroviral therapy.

BACKGROUND: Cytomegalovirus (CMV) retinitis was a much-feared visual complication of late-stage acquired immunodeficiency syndrome (AIDS) in the past. Its incidence has waned significantly owing to the benefits of potent antiretroviral combination therapy, which for many individuals has provided some degree of immune reconstitution and avoidance of opportunistic infections, particularly this blinding disease. CASE REPORT: A 45-year-old white man with long-standing, multidrug-resistant human immunodeficiency virus (HIV) infection and severe immunodeficiency despite multiple antiretroviral drug regimens, presented to the eye clinic reporting decreased vision and spider web patterns in his left eye for the past week. Best-corrected visual acuity was 20/20 in the right (O.D.) and 20/25 in the left eye (O.S.). Dilated funduscopic examination of the left eye found vasculitis of the midperipheral inferonasal arcade in the midperiphery, with surrounding intraretinal hemorrhage and granular retinal necrosis. Diagnosis of cytomegalovirus retinitis was made, and the patient began induction therapy with oral valganciclovir 900 mg twice a day for 3 weeks. Maintenance therapy after retinitis stabilization was 900 mg every day until any observed recurrence of infection. Three months after complete resolution of the active retinitis, the patient returned to the clinic reporting new floaters of recent onset. A reactivation of the CMV retinitis warranted a reinduction with valganciclovir 900 mg orally twice a day for 3 weeks. CONCLUSION: This case is illustrative of the efficacy and relative ease of administration of valganciclovir, the newest medication approved for treatment and maintenance of CMV retinitis. Despite his severe immunodeficiency, our patient tolerated the induction and maintenance therapy of oral valganciclovir well, and the CMV retinitis was stabilized and resolved with full recovery of visual acuity.

Sector retinitis pigmentosa.

BACKGROUND: Retinitis pigmentosa (RP) is one of the most common hereditary retinal dystrophies and causes of visual impairment affecting all age groups. The reported incidence varies, but is considered to be between 1 in 3,000 to 1 in 7,000. Sector retinitis pigmentosa is an atypical form of RP that is characterized by regionalized areas of bone spicule pigmentation, usually in the inferior quadrants of the retina. CASE REPORT: A 57-year-old Hispanic man with a history of previously diagnosed retinitis pigmentosa came to the clinic with a longstanding symptom of decreased vision at night. Bone spicule pigmentation was found in the nasal and inferior quadrants in each eye. He demonstrated superior and temporal visual-field loss corresponding to the areas of the affected retina. Clinical measurements of visual-field loss, best-corrected visual acuity, and ophthalmoscopic appearance have remained stable during the five years the patient has been followed. DISCUSSION: Sector retinitis pigmentosa is an atypical form of RP that is characterized by bilateral pigmentary retinopathy, usually isolated to the inferior quadrants. The remainder of the retina appears clinically normal, although studies have found functional abnormalities in these areas as well. Sector RP is generally considered a stationary to slowly progressive disease, with subnormal electro-retinogram findings and visual-field defects corresponding to the involved retinal sectors. CONCLUSION: Management of RP is very difficult because there are no proven methods of treatment. Studies have shown 15,000 IU of vitamin A palmitate per day may slow the progression, though this result is controversial. Low vision rehabilitation, long wavelength pass filters, and pedigree counseling remain the mainstay of 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.

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.