Choroidal osteoma: evidence of progression and decalcification over 20 years.

Choroidal osteoma is a rare, benign, ossifying tumour of the choroid of unknown aetiology. In contrast to other types of intraocular ossification, choroidal osteoma is found typically in young healthy females in the second or third decades of life with no history of systemic or ocular disease. Choroidal osteoma is a deep, pale yellow lesion with distinct geographic borders at the juxtapapillary or macular region, with branching 'spider' vessels on the surface of the tumour. These features should help differentiate choroidal osteoma from other types of intraocular tumour and the diagnosis can be confirmed with ultrasonography and computerised tomography. Here we report an initially unilateral case of choroidal osteoma, which decalcified over 20 years but during the same period the fellow eye also developed a choroidal osteoma to become a bilateral case. Despite the benign nature of the tumour, vision may be compromised by gradual atrophy of the overlying retina, serous retinal detachment, accumulation of sub-retinal fluid and sub-retinal haemorrhage associated with choroidal neovascularisation. Frequent examinations are recommended for patients with choroidal osteoma, for early detection of a subretinal neovascular membrane and potential treatment with laser photocoagulation.

Spectrum of pattern dystrophy in pseudoxanthoma elasticum.

OBJECTIVE: To study the prevalence, type, and features of pattern dystrophy in patients with pseudoxanthoma elasticum (PXE). METHODS: A search of the photographic records at the Vanderbilt Eye Institute using the keywords "angioid streaks and pseudoxanthoma elasticum" yielded 28 names. Of the 23 subjects meeting the patient selection criteria, 22 were confirmed to have a positive diagnosis for PXE after reviewing the medical history information. The diagnosis was confirmed by the constellation of fundus findings in all 22 subjects, by a clinical examination of the skin in 9, and by a skin biopsy specimen in 1. RESULTS: Pattern dystrophy was present in 16 patients (27 eyes) of those with PXE. Fourteen patients (23 eyes) had fundus pulverulentus, 3 patients (5 eyes) had butterfly-shaped dystrophy, and 1 patient (2 eyes) each had fundus flavimaculatus and reticular dystrophy. One eye of one patient developed solitary vitelliform pattern dystrophy during follow-up. Two patients showed progression from one pattern into another during follow-up. Another patient, who at first showed no evidence of pattern dystrophy in either eye, developed fundus pulverulentus in both eyes 5 years later. One patient had simultaneous evidence of 2 types: butterfly and fundus flavimaculatus pattern in each eye. Angioid streaks were seen in each eye of all patients. Peau d'orange was noted in 18 patients, optic nerve drusen in 5, and retinal crystalline bodies in 9. Choroidal neovascular membrane was present in 15 patients. CONCLUSIONS: All 5 varieties of pattern dystrophy, 2 of which were not previously associated with PXE, were seen in patients with PXE. Fluorescein angiogram was useful in delineating the type and extent of pattern dystrophy.

Bilateral idiopathic multifocal retinal pigment epithelium detachments in otherwise healthy middle-aged adults: a clinicopathologic study.

PURPOSE: To present the results of a clinicopathologic study of patients with bilateral idiopathic multifocal serous retinal pigment epithelial detachments (RPEDs). METHOD: Retrospective review of the medical records of three middle-aged patients with innumerable RPEDs. The eye of one patient was available for histopathologic examination. RESULTS: The presence of multiple serous RPEDs was verified with fluorescein angiography and optical coherence tomography in two patients and by histopathologic examination of one eye of a third patient, who developed a submacular hematoma and vitreous hemorrhage caused by a focal area of choroidal neovascularization. CONCLUSIONS: No cause for the multifocal serous RPEDs was found.

Persistent outer retinal defect after successful macular hole repair.

PURPOSE: To report two cases of a persistent outer retinal defect evident by optical coherence tomography (OCT) after clinically successful macular hole surgery. DESIGN: Retrospective case series. METHODS: A retrospective case series of two patients who had a persistent outer retinal defect by OCT and excellent visual acuity after clinically successful macular hole surgery. RESULTS: Two patients with stage 2 macular holes and preoperative visual acuity of 20/70 and 20/60, respectively, underwent macular hole surgery. Although the macular holes were closed on clinical evaluation, a persistent outer retinal defect was identified by OCT in both patients. At 5 to 6 months postoperatively, the outer retinal defect became less prominent and the visual acuity remained at 20/20 in both patients. CONCLUSIONS: An outer retinal defect by OCT may occur in the early postoperative period after macular hole surgery. Despite this defect, good visual acuity is possible.

Penetrating trauma associated with findings of multiple evanescent white dot syndrome in the second eye: coincidence or an atypical case of sympathetic ophthalmia?

PURPOSE: To report a case of sympathetic ophthalmia (SO) resembling multiple evanescent white dot syndrome (MEWDS). METHODS: Retrospective chart review. RESULTS: A 17-year-old girl with a ruptured globe in the right eye underwent prompt primary repair and vitrectomy, scleral buckling, and silicone oil infusion 3 weeks later. Eight weeks after injury, she presented with visual loss in the left eye. Fundus examination in the left eye disclosed optic disk swelling and well-circumscribed, 100 to 500 microm diameter gray-white lesions at the level of the retinal pigment epithelium (RPE) posterior to the equator, sparing the fovea. On fluorescein angiography, the lesions appeared as areas of blocked choroidal fluorescence in the arterial phase and were associated with dye leakage in a wreathlike pattern during venous filling. Dye leakage occurred at the optic disk. Visual field testing showed depressed central sensitivity and an enlarged blind spot in the left eye. The patient was treated with prednisone and underwent diagnostic enucleation of the right eye. Histopathology showed rare choroidal granulomata and pigment phagocytosis. Vision improved to 20/20 in the left eye, and the window defects persisted. Visual field testing 6 months later was normal. One month after discontinuing prednisone, new areas of RPE hypopigmentation developed. Two weeks later, choroidal inflammation recurred and periphlebitis developed. CONCLUSION: This case indicates that SO can mimic MEWDS.

Anatomic and visual acuity outcomes following thermal laser photocoagulation or photodynamic therapy for symptomatic circumscribed choroidal hemangioma with associated serous retinal detachment.

BACKGROUND AND OBJECTIVE: This study reports and compares the anatomic and visual acuity outcomes of patients treated with either thermal laser photocoagulation or photodynamic therapy for circumscribed choroidal hemangioma with associated serous retinal detachment. PATIENTS AND METHODS: Retrospective, consecutive, interventional case series of patients treated for symptomatic circumscribed choroidal hemangioma. RESULTS: Thirteen of 23 patients (57%) treated with thermal laser photocoagulation exhibited complete resolution of subretinal fluid, 6 (26%) had partial resolution of subretinal fluid, and 4 (17%) had persistent subretinal fluid despite therapy. At 3 months posttreatment, visual acuity was improved in 6 (26%), stable in 13 (57%), and worsened in 4 (17%) of 23 patients. All 5 patients who underwent photodynamic therapy had complete resolution of subretinal fluid. At 3 months posttreatment, visual acuity improved in 4 (80%), remained stable in 1 (20%), and worsened in 0 (0%) patients. CONCLUSIONS: Photodynamic therapy shows similar if not better anatomic and visual acuity results than thermal laser photocoagulation when treating symptomatic circumscribed choroidal hemangioma.

Acute exudative polymorphous vitelliform maculopathy syndrome.

PURPOSE: The authors describe clinical characteristics of acute exudative polymorphous vitelliform maculopathy syndrome, also termed polymorphous maculopathy syndrome (PMS), an unusual tapetoretinal disorder first reported by Gass. METHODS: Detailed ophthalmoscopy, fluorescein angiography, electrophysiology, and optical coherence tomography (OCT) were performed on a patient with PMS. RESULTS: Numerous small, yellowish lesions arranged in a honeycombed pattern at the level of the retinal pigment epithelium (RPE) around the disk and the macula, a shallow macular detachment (documented by OCT), and a perifoveolar yellow ring appeared in both eyes. Initial fluorescein angiography revealed mild early hyperfluorescence and late staining of the perifoveolar rings and the multifocal yellow lesions. Gradually, subretinal yellowish deposits gravitated as a meniscus below the macula with subsequent further changes. Electrophysiology showed reduced amplitudes of the electroretinogram and electrooculogram and abnormal dark adaptometry. A rhegmatogenous retinal detachment in the left eye was successfully repaired, with a visual acuity of 20/30. Antecedent upper respiratory infection and headaches and positive serology for the coxsackie virus suggest the possibility of a virus-induced disorder. Genetic studies of this patient showed normal DNA sequences for the bestrophin and peripherin/RDS genes. CONCLUSION: This is the first report of PMS with the following characteristics: occurrence in a woman, development of a rhegmatogenous retinal detachment, and electroretinogram, visual-evoked response, color vision, and OCT findings. PMS has features similar to but also different from those of Harada disease and Best disease. Further studies are necessary to determine its relationship to other acquired conditions, such as virus-induced disorders, and genetic defects unrelated to abnormalities of the bestrophin and peripherin/RDS genes.

Congenital simple hamartoma of the retinal pigment epithelium: a study of five cases.

PURPOSE: To report 5 patients with presumed congenital, simple hamartoma of the retinal pigment epithelium (RPE). DESIGN: Retrospective, observational small case series. PARTICIPANTS: Five patients with a black macular tumor of the RPE. MAIN OUTCOME MEASURES: Initial tumor features, initial associated fundus features, tumor course on follow-up, and visual acuity outcome. RESULTS: In all 5 cases, the tumor involved the macula and the mean distance to the foveola was 0.4 mm (median, 0.2 mm; range, 0.2-1.2 mm). In all cases, the tumor was darkly pigmented, with full-thickness retinal and RPE involvement and minimal protrusion into the vitreous cavity. The mean ultrasonographic tumor thickness was 1.6 mm (median, 1.4 mm; range, 1.1-2.5 mm). Associated features included minimally dilated retinal feeding artery and draining vein (100%), surrounding mild retinal traction (80%), retinal exudation (20%), and vitreous pigmented cells (20%). None of the lesions had associated subretinal fluid, macular edema, or macular hole. Visual acuity was 20/20 in 3 cases and slightly decreased in the other 2 cases because of foveal traction. The findings remained stable in 3 patients who had adequate follow-up. We chose to classify this lesion as congenital simple hamartoma of the RPE to differentiate it from combined hamartoma of the retina and RPE, congenital hypertrophy of the RPE, adenoma or adenocarcinoma of the RPE, and acquired hyperplasia of the RPE, which are different clinical entities. CONCLUSIONS: Congenital hamartoma of the RPE is typically a black, full-thickness retinal mass, often adjacent to the foveola. Despite such location, the visual acuity was affected minimally.

Retinal arterial wall plaques in Susac syndrome.

PURPOSE: To demonstrate retinal arterial wall plaques (RAWPs) in patients with Susac syndrome, a disorder that consists of the triad of branch retinal artery occlusion, encephalopathy, and hearing loss. The clinician may misinterpret these RAWPs as emboli. DESIGN: Observational case series. METHODS: Four patients (one man and three women, aged 21-38 years) were examined and followed. RESULTS: Four patients with Susac syndrome had RAWPs in association with branch retinal artery occlusions. They were present in six of eight eyes. The plaques were yellow to yellow-white and located usually away from retinal bifurcations. Retinal arterial wall plaques occur at the midarteriolar segments, whereas Hollenhorst plaques occur at vascular bifurcations. Four of six eyes had resolution of the plaques. CONCLUSIONS: Retinal arterial wall plaques may occur with branch retinal artery occlusions in Susac syndrome. This finding should help the clinician who is struggling to make a diagnosis in a patient with an enigmatic encephalopathy and/or hearing loss. These plaques may resolve over time.

Acute macular neuroretinopathy: a review of the literature.

Acute macular neuroretinopathy (AMNR) is a rare condition that produces transient or permanent visual impairment. Typical cases have acute onset multifocal scotomas that correspond rather precisely with reddish, flat, or depressed circumscribed lesions in the macula. These lesions are wedge-shaped and generally point toward the fovea. The pathophysiology of AMNR is unclear, the causes are uncertain, and there is no specific treatment for this condition. This review summarizes the presentation, possible risk factors, and prognosis of the 41 cases of AMNR reported in the published, English-language literature from 1975 through April 2002. Possible areas for future research into the etiology of this rare condition are discussed.