Polypoidal choroidal vasculopathy in Caucasians.

PURPOSE: To study the prevalence of polypoidal choroidal vasculopathy (PCV) in Caucasian patients with occult choroidal neovascularization (CNV); to study the clinical spectrum of PCV in Caucasians and the outcome after laser photocoagulation of such lesions. METHODS: (1) A consecutive series of 374 eyes of Caucasian patients at least 58 years old, presenting occult CNV, presumed to have age-related macular degeneration (AMD) on fluorescein angiography (FA) were further characterized by indocyanine green angiography (ICGA) to determine the frequency of PCV. (2) The funduscopic, FA and ICGA findings in a cohort of 36 Caucasian patients with PCV were analyzed. (3) The outcome after laser photocoagulation was studied in 14 PCV eyes with a minimum follow-up of 6 months. RESULTS: (1) Fourteen of 374 eyes (4%) presenting occult CNV in patients at least 58 years old were diagnosed as PCV by means of ICG-A. (2) A polypoidal lesion was found in the macula in 22 of 45 PCV eyes, in the peripapillary area in 16 of 45, under the temporal vascular arcade in 6 of 45 and in the midperiphery in 6 of 45. Large or soft drusen were observed in 15 of 45 eyes with PCV. (3) Regression of fundus signs without persisting polyps 6 months after laser photocoagulation was obtained in 5 of 5 treated peripapillary lesions but in only 5 of 9 treated macular or arcade lesions. CONCLUSION: Polypoidal choroidal vasculopathy is not rare in Caucasian patients presenting with occult choroidal neovascularization. The fundus abnormalities seen in such eyes overlap with the typical manifestations of AMD. Whereas the prognosis after photocoagulation of peripapillary polypoidal lesions appears to be relatively good, it is more guarded for macular or arcade lesions.

Vasoproliferative retinal tumors associated with peripheral chorioretinal scars in presumed congenital toxoplasmosis.

BACKGROUND: The purpose of this retrospective clinical study was to demonstrate vascularization of peripheral hyperplastic chorioretinal scars in presumed congenital toxoplasmosis by choroidal vessels and secondly to report vasoproliferative tumors of the sensory retina seen in association with such lesions. METHODS: Retrospective study of a cohort of 13 patients with peripheral hypertrophic chorioretinal scars, including fluorescein angiography (eight eyes) and indocyanine green angiography (seven eyes). Congenital toxoplasmosis was serologically confirmed in seven cases and suspected on clinical grounds in six cases. RESULTS: Sixteen eyes exhibited a peripheral complex scar with a posterior atrophic and an anterior hypertrophic part. These scars were vascularized from the choroid. In eight of these eyes an additional vasoproliferative tumor was seen within or adjacent to the scar and in one fellow eye a vasoproliferative retinal tumor was found as well. These eyes experience visual loss, resulting from vitritis and exudative/traction retinal detachment. Regression of exudation was seen in five of seven cryocoagulated or endophotocoagulated tumors. CONCLUSION: Peripheral hypertrophic scars in presumed congenital toxoplasmosis can be vascularized from the choroid. A vasoproliferative tumor of the sensory retina, secondary to presumed congenital toxoplasmosis scars, can cause vitritis and exudative/traction retinal detachment. Early coagulation of this lesion may prevent a poor outcome.

Comparison of fluorescein and indocyanine green angiography in angioid streaks.

BACKGROUND: The main cause of vision loss in patients with angioid streaks is choroidal neovascularization and subsequent macular degeneration. Indocyanine green angiography allows visualization of the choroidal circulation and may be superior to fluorescein angiography in the evaluation of patients with angioid streaks. METHODS: The ophthalmoscopic, fluorescein and indocyanine green angiographic characteristics of angioid streaks were studied in 34 patients with such streaks. Nineteen patients had pseudoxanthoma elasticum and 15 patients had isolated angioid streaks. The fluorescence characteristics of the 'peau d'orange' and of choroidal neovascularization, when present, were also analyzed. RESULTS: Angioid streaks may be hyperfluorescent, hypofluorescent or invisible on indocyanine green angiography. Hyperfluorescent streaks were found in 88% of eyes, hypofluorescent streaks in 11%; in 18% of eyes some streaks were not visualized by indocyanine green angiography. The peau d'orange stained as a speckled pattern in the midperiphery; the flecks were concentrated temporal to the macula. Eighteen eyes presented classic and 6 occult choroidal neovascularization. In several eyes a plaque-like lesion was seen on indocyanine angiography that did not correspond to occult choroidal neovascularization on fluorescein angiography. CONCLUSION: Indocyanine angiography outlines angioid streaks as well as the peau d'orange appearance better than fluorescein angiography in the majority of cases. In some cases, however, funduscopically visible streaks can not be visualized. Sometimes classic choroidal neovascular membranes are not visualized by conventional indocyanine green angiography. Occult choroidal neovascularization is better defined by indocyanine green angiography. The fluorescence of angioid streaks and of plaque-like lesions makes the interpretation of indocyanine green angiography difficult.

Macular pattern dystrophy in patients with deafness and diabetes.

PURPOSE: To report the characteristic findings of a macular pattern dystrophy in patients with diabetes and deafness resulting from the mitochondrial point mutation at position 3243 and to expand the clinical spectrum of this condition by describing functional testing results. METHODS: Four diabetic patients who were referred to the eye department for diabetic fundus examination were found to harbor a macular pattern dystrophy. Further examination of visual fields; color contrast sensitivity; and the ear, nose, and throat; and molecular analysis of the mitochondrial genome were performed. Two of our patients were sisters. Their relatives also were examined. RESULTS: All four patients were found to harbor the mitochondrial point mutation at position 3243 and presented clinically with the phenotype of diabetes and deafness. The macular pattern dystrophy described in these patients seems to be typical for this condition. Results of a 9-year follow-up study of one of the patients showed mild progression of atrophic changes. The overall prognosis of the retinopathy is likely to be good. CONCLUSION: These cases demonstrate the need for further molecular investigations when a macular pattern dystrophy is found in a patient with diabetes and deafness.

Myelinated nerve fibers and retinal vascular abnormalities.

BACKGROUND/PURPOSE: Abnormal retinal vessels may develop in a region of myelinated nerve fibers, and these vessels may cause vitreous hemorrhages. METHODS: The clinical histories of seven patients with retinovascular abnormalities in a patch of myelinated nerve fibers are presented. None of the reported patients had other evidence of systemic disease. The cases were traced by a multicentric retrospective study. RESULTS: Retinal vascular abnormalities ranged from mild telangiectasis to frank neovascularization, with or without obstruction of the capillary network and signs of branch artery and vein occlusion. Age at diagnosis ranged from 15 to 68 years. Vitreous hemorrhages occurred in the four youngest patients and occurred at 15, 27, 27, and 43 years of age. Laser photocoagulation was applied in three patients and vitrectomy was performed in one. CONCLUSION: The authors' findings suggest that the abnormal structure of the myelinated nerve fibers and the thickened nerve fiber layer of the affected portions of retina may play a role in the onset of retinal vascular abnormalities and eventually cause telangiectasis, branch artery and vein occlusion, neovascularization, and vitreous hemorrhages. This suggestion is based on the absence of other causes of neovascularization or vitreous hemorrhage in all seven patients, and on the relatively young age of four of the patients with this association.

Retinal vasculitis in Lyme borreliosis.

We observed retinal vasculitis in seven patients with clinical and serologic evidence of Borrelia burgdorferi infection. Three patients presented with abrupt loss of vision due to acute retinal vasculitis. Funduscopy demonstrated engorged veins, hemorrhages, perivenous infiltrates and retinal white spots. Fluorescein angiography showed leakage from the veins, from the white spots and from the optic disc. Moreover arterial occlusions were observed in two patients. Four patients had signs of chronic uveitis with vitritis, cystoid macular oedema and retinal vasculitis, which was associated with neovascularization and vitreous hemorrhage in one patient, and with optic neuritis in another patient. Six patients received antibiotic treatment and three patients received systemic corticosteroids. Marked improvement in the three acute retinal vasculitis cases occurred within several weeks, the fundus changes disappeared in another few months, and no recurrences were observed. The final visual acuity was excellent in these patients, although optic disc pallor and visual field loss persisted in one case. In the four patients with chronic uveitis visual blurring improved following antibiotic treatment and the retinal vasculitis and vitritis slowly regressed. The proliferative retinopathy of one patient required panretinal laser treatment.

[Eye fundus of the diabetic patient with nephropathy and hypertensive retinopathy. Macroangiopathic complications]. / Le fond d'oeil du diabétique avec néphropathie et rétinopathie hypertensive. Complications macroangiopathiques.

In patients with diabetic nephropathy retinopathy is always present and proliferative retinopathy is common. Retinopathy tends to deteriorate as renal failure develops particularly in patients with poorly controlled blood pressure and in patients in whom no retinal treatment has been given before development of renal failure. Treatment of hypertension and of end stage renal failure will improve macular edema and stabilize vision. As the progression of diabetic retinopathy is independent of diabetic nephropathy and not reversed by treatment of nephropathy, further follow-up and treatment of diabetic retinopathy are imperative. In recent years medical treatment of arterial hypertension and facilities for dialysis and kidney transplantation have become available, and patients are now treated at a much earlier stage of their renal disease. Consequently, were are seeing fewer patients with renal failure and severe hypertensive fundus changes. Nevertheless, arterial hypertension is still a very important problem in diabetic patients with and without nephropathy and complications of atherosclerosis are common as a result of chronic hypertension and hyperlipidemia.

Case report: associated retinal neovascularization and choroidal hemangioma.

Choroidal hemangiomas are vascular hamartomas that may be associated with secondary changes. Although subretinal fluid and associated neurosensory retina and retinal pigment epithelial changes are well documented, two cases in which retinal neovascularization was felt to be secondary to choroidal hemangiomas are presented. Long term follow-up reveals mild changes of the retinal neovascularization in both cases. The neovascularization did not affect the visual prognosis of the involved eyes.

Early lesions of bilateral diffuse melanocytic proliferation.

The early lesions of bilateral diffuse melanocytic proliferation were studied with fluorescein angiography in a 63-year-old woman. We first observed a small choroidal melanoma in the left eye and several flat melanocytic lesions in both eyes. Extension and growth of new melanocytic lesions and leakage at the level of the pigment epithelium were then noted. Subsequently, pink patches, with an eye-catching early hyperfluorescence, appeared in the posterior fundus in both eyes. The patient experienced progressive visual loss during the 8-month follow-up period before her death. Histopathologically, the uveal tracts were diffusely thickened by melanocytic proliferation. The primary systemic neoplasm was an ovarian carcinoma, found in five of 18 patients with this unusual paraneoplastic syndrome.

Metastatic carcinoma to the retina. Clinicopathologic findings in two cases.

Two cases of metastatic carcinoma to the neuroretina are reported. One patient had an oat cell carcinoma of the lung that was metastatic to the brain and retina; this was confirmed postmortem. The other patient had metastatic breast carcinoma with seeding of tumor cells into the vitreous from a focus of retinal embolism. The diagnosis was confirmed from a vitrectomy specimen.