One year of intravitreal injections of steroids.

PURPOSE: Chronic macular oedema sometimes does not respond to classic treatment such as laserphotocoagulation, periocular and systemic steroids or carbonic anhydrase inhibitors. High dose intravitreal injection of steroids can be a valuable alternative in these patients. METHODS: Sixty-four eyes of 51 patients with chronic macular oedema, refractory to conventional treatments, received an intravitreal injection of 4 mg Kenacort (triamcinolone acetonide). At postoperative controls (1 day, 1 month, 3 months, 6 months, 1 year), the visual acuity, intraocular pressure, central retinal thickness on OCT II were analyzed retrospectively. RESULTS: In the diabetic group, but also in cases of chronic uveitis, retinal vein thrombosis, birdshot chorioretinopathy, Irvine Gass syndrome, cellophane maculopathy and age-related macular degeneration with classic subfoveolar neovascular membrane, dramatic decrease of the oedema was observed on funduscopy and OCT. A statistically significant reduction of mean central foveal thickness of 61% at 1 week and 49% at 3 months post-injection was demonstrated on OCT. Considerable gain in visual acuity was noted. The highest benefit in post-operative visual acuity was achieved after 3 months and averaged a gain of +3.55 Snellen lines (n=39). In 17% of eyes a rise in intraocular pressure was noted. Unfortunately we had one case of endophthalmitis in a poorly regulated diabetic woman. Six eyes were retreated because of recurrence of macular oedema. CONCLUSION: Intravitreal injection of steroids can dramatically help in some cases of chronic macular oedema, not reacting to classic treatment. The injection should be performed under sterile conditions since endophthalmitis is a potential risk. The most frequent complication seems to be a rise in intraocular pressure in cortisone responders.

Diabetes mellitus and the eye.

In Belgium as in other industrialized countries, diabetic retinopathy is still the most important cause of blindness in the active population group, but can be prevented by an early and adequate treatment. This highly socio-economic problem should therefore have priority with the autorities of the Belgian Health Department. The costs of a well-organized screening programme of the diabetic population are far less than the costs that arise from the economic and social consequences caused by irreversable visual loss, the costs of long and expensive treatments and long periods of inability to work, when advanced diabetic eye disease has developed. Guidelines for management and timely treatment of patients with diabetic retinopathy are available, but should be implemented more vigorously.

Indocyanine green angiography is of value for the diagnosis of chronic central serous chorioretinopathy in elderly patients.

BACKGROUND: Central serous chorioretinopathy is characterized on indocyanine green angiography by areas of transient choroidal hyperfluorescence. These findings are thought to be the consequence of altered permeability of the choroidal vessels. METHODS: The indocyanine green angiograms of 41 patients between 40 and 60 years of age, with central serous chorioretinopathy and of 120 patients above 64 years of age with occult choroidal neovascularization due to age-related macular degeneration were reviewed for the presence of transient indocyanine green leakage. Twelve eyes of 9 patients above 64 years of age with (1) fluorescein leakage of undetermined source corresponding with well-delineated zone(s) of retinal pigmentary changes and (2) transient indocyanine green hyperfluorescence are reported in detail. RESULTS: Transient indocyanine green hyperfluorescence was seen in all eyes with central serous chorioretinopathy, either typical or chronic, and was seldom seen in occult choroidal neovascularization due to age-related macular degeneration. In the series of chronic serous chorioretinopathy in patients above 64 years of age, four classic choroidal neovascular membranes were found in 12 eyes. Most patients presented multizonal transient choroidal hyperfluorescence in both eyes on indocyanine green angiography. CONCLUSION: Transient choroidal hyperfluorescence is suggestive for chronic central serous chorioretinopathy in older patients presenting retinal pigmentary disease with fluorescein leakage of undetermined source. Chronic central serous chorioretinopathy is not uncommonly associated with classic choroidal neovascularization in the elderly.

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.

Ocular non-Hodgkin's lymphoma: a clinical study of nine cases.

BACKGROUND: Primary oculocerebral large cell malignant non-Hodgkin's lymphoma, formerly called ocular reticulum cell sarcoma, runs a uniformly fatal course. Once the central nervous system (CNS) is involved, survival without treatment is very limited. Although treatment does not substantially improve the long term survival, it provides short term improvement in these patients. METHODS: The charts of all patients with ocular involvement of non-Hodgkin's lymphoma followed during the period 1984-93 were reviewed. The diagnosis of non-Hodgkin's lymphoma was made by different diagnostic approaches: CNS biopsy, anterior chamber tap, vitrectomy, haematology, and necropsy. RESULTS: Eight patients had oculocerebral large cell and one had small cell non-Hodgkin's lymphoma. Five patients with pure ocular localisation had initially received steroid treatment for intermediate uveitis. First diagnosis was made on CNS biopsy in three, anterior chamber tap in one, vitreous aspirate in three, haematology in one, and necropsy in one case. CONCLUSION: Ocular non-Hodgkin's lymphoma is a difficult diagnosis. Vitrectomy allows cytological diagnosis in most but not all cases. When no treatment is given, patients survive for only a few weeks once the CNS is involved. Although the disease is eventually fatal, treatment by means of radiotherapy, steroid administration, and vitrectomy can allow these patients to lead a normal professional and social life during the years between recurrences.

Indocyanine green angiography in angioid streaks.

PURPOSE: To present the indocyanine green angiographic features of angioid streaks. METHODS: The angiographic findings of five typical cases, three cases of idiopathic angioid streaks and two pseudoxanthoma elasticum patients, are reported. RESULTS: In most cases angioid streaks are hyperfluorescent in the late venous and late phase of the indocyanine green angiogram. Within areas of chorioretinal atrophy they are not visualized. The 'peau d'orange' appearance is much better visualized by indocyanine green angiography than by fluorescein angiography. Indocyanine green angiography further characterizes choroidal neovascularization, which is the major complication of angioid streaks. CONCLUSION: Indocyanine green angiography contributes to the visualization of alterations in Bruch's membrane such as angioid streaks and the 'peau d'orange' appearance.

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.

Premacular fibrosis in juvenile Coats' disease with spontaneous peeling after photocoagulation of the congenital vascular anomalies.

We studied two cases of juvenile coats' disease with visual loss at presentation due to premacular fibrosis. The retinal area with altered vascularization was photocoagulated with the Argon green laser. The membrane retracted in a matter of a few months resulting in improved visual acuity. Afterwards the membrane completely peeled and was seen as a floater attached to the posterior hyaloid. Peeling of premacular fibrosis in juvenile's Coats disease may be observed after photocoagulation treatment. It is suggested that induced posterior vitreal detachment was associated with peeling of the premacular fibrosis.

Diagnostic problems in chronic vitreous inflammation.

We reviewed 188 patients with chronic vitreous inflammation initially diagnosed as intermediate uveitis, who were seen at the Ghent Eye Clinic during the period 1978 to 1988. We were able to establish a more specific diagnosis in 55 patients. The different approaches that contributed to the diagnosis were analyzed, (comprehensive history-taking and clinical examination, laboratory tests and special investigations, vitrectomy, and following the evolution of the disease). No etiology was found in 133 patients (70.7%), subsequently diagnosed as idiopathic intermediate uveitis. The major characteristics of the disease were analyzed.

[Birdshot chorioretinopathy]. / Chorioretinopatia typu birdshot.

The clinical picture of birdshot chorioretinopathy has been presented, basing on the studies of 102 cases, treated in 16 university clinics and ophthalmological departments in Europe. The records of the patients were sent to the Ophthalmological Clinic of Ghent University as a contribution to an international research programme concerning birdshot chorioretinopathy. The observations reveal that this type of chorioretinopathy is a separate entity and its diagnosis should be based on the binocular occurrence of characteristic lesions in choroid, accompanied by at least two of the following symptoms: vitreous inflammation, vasculitis, macular edema and papilledema.

Natural history and visual outcome in patients with APMPPE.

We undertook a retrospective study of the files of 21 patients (42 affected eyes) with acute posterior multifocal placoid pigment epitheliopathy to obtain details on the natural history of the disease and on the functional and visual outcome (mean follow up: 17 months). The final visual acuities were 8/10 or better in 34 eyes (81.0%), 7/10 to 2/10 in 6 eyes (14.2%) and 1/10 in two eyes (4.8%). Although the final visual acuity was good in most eyes, 24 eyes (57.2%) were still symptomatic due to scotomas (33.3%), metamorphosia (21.4%), decreased vision (16.6%), floaters (4.8%) and chronic redness (2.4%). Recurrences did not occur in this group of patients. Electrophysiological and color vision tests were performed in the initial phase of the disease, but we found no correlation between the test results and the final visual acuity.

Ocular manifestations of Alport's syndrome: a case report.

A review is given of the ocular signs characteristic for Alport's syndrome. The corneal, lenticular and fundus lesions are described. They occur in 15% of the cases. The patient reported here, showed the typical macular spots.

Association of birdshot retinochoroidopathy and HLA-A29 antigen.

In 1980 Ryan and Maumenee described a new clinical entity characterized by hypopigmented spots in a typical patterned distribution in association with vitritis and retinal vasculopathy leading to cystoid macular oedema and papilloedema. In a recent study of a large group of European patients (n = 102) with birdshot retinochoroidopathy, the importance of the different clinical manifestations and their diagnostic value were assessed. Data on HLA-A29 typing were obtained in 49 patients with birdshot retinochoroidopathy. We could confirm the high association between HLA-A29 and this disease, as found by Nussenblatt in 1982. In 47 of these patients (95.5%) the HLA-A29 antigen was present. This association is presently the highest HLA class I disease association reported with a relative risk of 224.35.

[Vascular manifestations in birdshot chorioretinopathy]. / Les manifestations vasculaires dans la choriorétinopathie "birdshot".

The retinal vascular changes and complications are described in 121 patients with birdshot chorioretinopathy. The medical history in these patients did not reveal any relevant correlation with a systemic disorder, although vascular accidents and arterial hypertension in this group were rather high. The antigen HLA-A29 was present in 95.9% of the patients.

Electrophysiologic studies in birdshot chorioretinopathy.

We investigated retinal function in 16 patients with birdshot chorioretinopathy. Consistent abnormalities of dark adaptation, color vision, visual field, electro-oculography, electroretinography, and visual-evoked cortical potentials were found. They included raised thresholds of dark adaptation, acquired dyschromatopsia, mainly of the blue-yellow type, an electroretinogram with reduced amplitude, increased latency of the b-wave and absent oscillatory potentials, an abnormal electro-oculogram, and in many a marked disturbance in the pattern reversal visual-evoked cortical potential. The a-wave of the electroretinogram, the fast oscillations of the standing potential, and the flash visual-evoked cortical potential were well preserved. The nature of the abnormalities suggests that dysfunction was caused by inner retinal disease. Little evidence indicated outer retinal dysfunction resulting from choroidal inflammation.

Birdshot chorioretinopathy: clinical characteristics and evolution.

During the period 1980-6 102 patients from 14 European eye clinics were diagnosed as having birdshot chorioretinopathy (BSCR). All were Caucasian, and the series consisted of 47 men and 55 women, with a mean age of 52.5 years. The major findings in this rare disorder concern the ocular fundus. Most marked are the patterned distribution of depigmented spots without hyperpigmentation, radiation from the optic disc in association with vitritis, retinal vasculopathy with frequent cystoid macular oedema, and involvement of the optic nerve head. The distribution and appearance of the lesions suggest that they are related to the major choroidal veins. Complications of the disease were epiretinal membranes, retinal neovascularisation, recurrent vitreous haemorrhage, subretinal neovascular membranes occurring both in the juxtapapillary and macular regions, and optic atrophy. The medical history was not contributary. HLA testing showed very strong disease association with HLA A29 (95.8%). The evidence suggests that it is a single disease entity rather than a group of disorders because of the remarkable similarity in the ophthalmological appearance and the clinical course, combined with the exceptionally high association with HLA A29.

HLA typing in birdshot chorioretinopathy.

We examined 49 patients with birdshot chorioretinopathy in whom HLA typing had been performed. Of these 49 patients, 47 (95.9%) had the HLA-A29 antigen. The HLA-B12 (B44) antigen was also significantly associated with the disease, but this antigen is known to be in linkage disequilibrium with the antigen HLA-A29. The HLA-A2 antigen was less frequent in patients with birdshot chorioretinopathy than in the control group of normal European individuals (20% as compared to 44.6%).

Subacute sclerosing panencephalitis: fundus changes and histopathologic correlations.

This paper describes the fundus changes in 2 patients with subacute sclerosing panencephalitis (SSPE). In both cases the ocular involvement preceded the neurological symptoms by several months. In one patient a localized serous detachment in the macular region was associated with retinal infiltrates and in the second patient an atypical unilateral macular chorioretinitis was first diagnosed. The diagnosis of SSPE was suspected when neurological signs appeared and it was confirmed by the typical EEG changes and especially by the high titers of measles antibodies in serum and CSF. The affected left eye of the second patient could be studied histopathologically and presented mainly pigment epithelial changes at the level of the scar, edema of the external plexiform layer, retinal folds and a detachment and rupture of the internal limiting membrane.