J. J. De Laey

Polypoidal choroidal vasculopathy in Caucasians.

Abstract 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.

Clinicopathological correlation in exudative age related macular degeneration: histological differentiation between classic and occult choroidal neovascularisation.

AIMS To analyse the histopathology of classic and occult choroidal neovascular membrane surgical specimens in age related macular degeneration. METHODS 35 membranes, from a consecutive series of surgically removed choroidal neovascular membranes in age related macular degeneration, were classified as classic or occult following the guidelines of the Macular Photocoagulation Study. Membranes with classic as well as occult components were considered as mixed membranes. The membranes were serially sectioned and stained with haematoxylin and eosin, Masson trichrome, periodic acid-Schiff, and phosphotungstic acid haematoxylin stain. The correlation has been made in a masked fashion. RESULTS 31 membranes (19 classic, 10 occult, and two mixed membranes) could be analysed histologically. 18 classic choroidal neovascular membranes had a major subretinal fibrovascular component and 10 of these had an additional, minor fibrovascular component under the retinal pigment epithelium. The 10 occult membranes contained a fibrovascular component under the retinal pigment epithelium and the two mixed membranes contained fibrovascular tissue on both sides of the retinal pigment epithelium. Fibrin and remains of outer segments tended to occur at the lateral edges of classic membranes and to cover the inner surface of occult membranes. CONCLUSION Classic choroidal neovascularisation in age related macular degeneration is predominantly composed of subretinal fibrovascular tissue while occult choroidal neovascularisation is composed of fibrovascular tissue at the choroidal side of the retinal pigment epithelium.

Neovascularization after Argon Laser Photocoagulation of Macular Lesions

In three patients treated for macular degeneration with argon laser photocoagulation choroidal neovascularization occurred four to six weeks later at the photocoagulation site. Two of the patients were treated for angioid streaks of the macular region, the third patient was treated for disciform macular degeneration. Argon laser photocoagulation also induced neovascularization experimentally in pigmented rabbits.

Indocyanine Green Angiography of Choroidal Neovascular Membranes

The choroidal circulation was studied in 27 eyes with choroidal neovascular diseases by means of fluorescein and indocyanine green (ICG) angiography. In 10 of the cases the disciform response was seen to be associated with either watershed zones of the choroidal circulation or with areas of choroidal circulatory disturbance. This is consistent with the theory that hypoxic regions of the choroid are the underlying cause of macular disciform response.

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 is of value for the diagnosis of chronic central serous chorioretinopathy in elderly patients

Abstract · 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.

Indocyanine Green Angiography of Submacular Choroidal Vessels in the Human Eye

Indocyanine green angiograms of the macular region in 40 human eyes were evaluated in order to demonstrate the characteristics of the circulatory patterns of the submacular choroid. During both the early and the late phases of choroidal fluorescence nonuniform fluorescence was found. This implies the existence of watershed zones between choroidal arteries and between choroidal veins. A horizontal line running through the fovea was found to form a boundary between arterial systems, and also, in the majority of cases, between venous system.

Subtraction ICG angiography in Harada's disease.

BACKGROUND/AIM The significance of indocyanine green (ICG) angiography (ICGA) in Harada’s disease still awaits clarification in many respects. This study investigates the details of choroidal lesions observed in Harada’s disease by the subtraction method. METHODS Eight patients with Harada’s disease were followed with ICGA. ICG angiograms were obtained with a Topcon high resolution digital fundus camera and processed with a Topcon IMAGEnet computer system. Image subtraction was conducted for analysing serial angiograms taken at about 2 second intervals during the dye transit phase and those taken in the early and middle phases of angiography. RESULTS Standard ICG images of acute stage disease showed delayed choroidal filling in the early phase. Mid phase angiograms showed areas with bright fluorescence of variable intensity, indicating intrachoroidal ICG leakage. With image subtraction of angiograms with an interval of seconds the choroidal vessels could be imaged sequentially, with the choroidal arteries visualised first, followed by the definition of the choriocapillaris and then the choroidal veins. The choroidal veins with delayed filling were visualised as positive images in serial subtraction angiograms. Subtraction with an interval of minutes showed uneven background fluorescence and bright fluorescence corresponding to the areas of intrachoroidal ICG leakage. After the disease subsided with steroid therapy, angiography revealed an improvement in delayed choroidal filling. Image subtraction by the second allowed a clear visualisation of improved choroidal venous filling, while subtraction by the minute showed homogeneous background fluorescence, eliminating brighter areas. CONCLUSION Subtraction ICGA demonstrated that delayed filling of the choroidal veins of varying severity occurs in association with hyperpermeability of the choroidal vessels in the course of Harada’s disease.

Dominant optic nerve atrophy with progressive hearing loss and chronic progressive external ophthalmoplegia (CPEO)

This paper describes a family where chronic progressive external ophthalmoplegia is associated with dominant optic atrophy and progressive sensorineural deafness. This may be a possible association in the same family of two diseases: progressive external ophthalmoplegia and dominant optic atrophy with progressive hearing loss. However, we believe that this family represents an unusual manifestation of ophthalmoplegia plus.

Clinical and electrophysiological findings in autosomal dominant vitreoretinochoroidopathy : report of a new pedigree

Abstract.Purpose: To report the clinical and electrophysiological findings in a three-generation pedigree with autosomal dominant vitreoretinochoroidopathy. Methods: Sixteen members of a three-generation pedigree with autosomal dominant vitreoretinochoroidopathy were examined clinically, including measurement of the corneal diameter. In 14 persons, Goldmann perimetry, axial length determination and electro-oculography were carried out. Electroretinography, according to ISCEV standards, was performed in 11 of 12 affected persons. Results: Characteristic annular peripheral pigmentary changes were present in all affected members, as well as chorioretinal atrophy varying from a tigroid aspect to marked atrophy. Four patients presented a microcornea and shallow anterior chamber without microphthalmia. The visual fields appeared to narrow with ageing. The electro-oculography was pathological in the affected patients and normal in the unaffected. The electroretinographic amplitude responses tended to worsen with age, with maintenance of near normal latencies. Conclusion: The clinical presentation of autosomal dominant vitreoretinopathy is variable. Electro-oculography seems to be a discriminative test. The condition may be associated with anterior segment abnormalities other than presenile cataract, such as microcornea, shallow anterior chamber and angle closure glaucoma.