|Year : 1973 | Volume
| Issue : 1 | Page : 5-15
Fluorescein angiography in Eale's disease
S.R.K Malik, B Patnaik
Maulana Azad Medical College and Associated Hospitals, New Delhi, India
Maulana Azad Medical College and Associated Hospitals, New Delhi
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Malik S, Patnaik B. Fluorescein angiography in Eale's disease. Indian J Ophthalmol 1973;21:5-15
The clinical picture and course of Eale's disease is characteristic enough to constitute a clinical entity (DUKE ELDER  ). The primary pathology is thought to be a vasculitis affecting the veins. Capillary abnormalities of beading and micro-aneurysms, abnormal vascular communications, recurrent retinal and vitreous haemorrhages and retinitis proliferans are secondary to phlebitis. Occasionally arteritis, uveitis, papillitis and neuroretinitis are associated. The etiology of the disease is unknown. Venous inflammation of sufficient severity due to any cause may develop into the clinical picture of Eale's disease.
Being essentially a vascular disease, the various manifestations of Eale's disease can be ideally studied by fluorescein angiography. Fluorescein circulation in the fundus has been studied in Eale's disease using a blue filter in an ophthalmoscope (JUTTE AND LEMKE,  ) and in a biomicroscope (EISNER  ). Isolated fluorescin fundus photographs have been recorded (MITSUI AND MATSUBARA,  ). However, serial fluorescein fundus photography in Eale's disease has not been often attempted (WESSING AND VON NOORDEN  THEODOSSIADIS,  ).
The present paper presents the characteristic lesions of Eale's disease as obtained from the study of 30 suitable cases by serial fluorescein fundus photo-angiography.
| Methods|| |
The patients selected for this study were showing the following clinical features:
1. Evidence of vitreous or retinal haemorrhages and/or
2. History suggestive of vitreous haemorrhage and
3. Active or healed signs of venous inflammation and/or
4. Other retinal lesions of Eale's disease.
The cases were thoroughly investigated to exclude any active systemic disorder. The media were sufficiently clear for the area to be satisfactorily studied in all cases. Thirty cases were studied by fluorescein angiography using the technique of NOVOTNY AND ALVIS  at Irwin Hospital between July 1968 to July 1969. To study different areas of the retina, repeated injections of the fluorescein were made in many cases. Every time 5 ml. of 10% sodium fluorescein was injected rapidly into a suitable vein in the forearm.
Twenty four out of thirty cases were males and had the first sign or symptom of the disease between 15 to 30 years of age. Only one case was a female who had the first attack at the age of 40.
Cases with vitreous or retinal haemorrhages or having a history of such haemorrhages within prior six months were grouped as active cases. Those with no evidence of fresh haemorrhages or having no history of haemorrhage within past six months but showed healed lesions characteristic of the disease were grouped under inactive cases.
| Results|| |
The findings of fluorescein angiography in various cases have been described under the following headings: (1) Common features; (2) Features of active cases; (3) Features of inactive cases and (4) miscellaneous features.
The affected area was often the one drained by a vein showing one of the following ophthalmoloscopic abnormalities: (i) a kink [Figure - 1],[Figure - 4],[Figure - 6]; (ii) apparent narrowing of the calibre with a patch of exudates or gliosis [Figure - 4]; (iii) a patch of chorioretinal lesion underneath the vein [Figure - 2],[Figure - 6] (iv); a complete obliteration of a small segment of a vein with collateral communication with the distal part of the same or surrounding veins [Figure - 5]. On one occasion no obvious ophthalmoscopically detectable lesion could be found. It was only on fluorescein angiography that the site of lesion was detected [Figure - 3].
There was a considerable delay in filling up of the affected vein. Delay in the venous circulation was detected in 3 ways: (i) when compared with veins of equal calibre in the same general area the affected vein was found to receive the dye much later than its, normal counterparts [Figure - 1]. (ii) The affected vein draining blood without fluorescein at a time when normal tributaries drain the dye to the main trunk resulted in a unilateral laminar fluorescein flow in the main trunk [Figure - 2]. (iii) The time between the arterial and the venous phase was prolonged to as much as 1.2 seconds.
However, once the veins filled up with the dye no localised narrowing of lumen could be detected at the site of venous abnormality [Figure - 1],[Figure - 2],[Figure - 3],[Figure - 4]. The ophthalmoscopic impression of narrowing of the calibre of the affected veins showing sheathing were repeatedly proved to be erroneous on fluorescein angiography. The part of the vein receiving the dye late retained it for a longer period [Figure - 1],[Figure - 2] , indicating a slowing of the circulation.
Features in Active cases
There was a marked diffuse fluorescence over the area involved. The fluorescence was maximum during the arteriovenous phase [Figure - 6]A, indi cative of increased general vascularity at the capillary level. The fine vessels showed varying degree of dye leakage in the affected area. The leakage could be prompt and marked as in a case of fresh vitreous haemorrhage [Figure - 6]A or it may be slow in showing up, as in a case of a fine superficial retinal haemorrhage [Figure - 7]. Another interesting feature seen in active cases was multiple microaneurysms [Figure - 3],[Figure - 6] and patches of neovascularisation [Figure - 6]. Difrent parts of the same fundus may show different types of picture. The inflammed large calibre veins showed fluorescein uptake of their walls over a small [Figure - 8] or large [Figure - 9]B part of their course. Often there was a frank extravasation of the dye which stained the surrounding tissue [Figure - 8],[Figure - 9]C.
Features in Inactive cases
There were no signs of increased retinal fluorescence or of micro-aneurysms or of fluorescein leakage. There was a reduced fluorescence in the arteriovenous phase. The larger veins were somewhat engorged and tortuous. [Figure - 1],[Figure - 4]. The small calibre veins showed extreme tortuosities, loops and kinks [Figure - 10],[Figure - 11]. Most of these dilated tortuous vessels appeared to be veno-venous capillary shunts. The arteries as far as could be visualised were apparently unaffected. Occasionally there were prominent collateral channels [Figure - 2].
Often there were multiple focal chorioretinal lesions. Their distribution was characteristic in that they were found singularly under the veins [Figure - 2],[Figure - 6]A,[Figure - 12] except in one instance where the lesions were found along a large vein [Figure - 13] . No such lesion was seen anywhere in the fundus unrelated to the veins. The chorioretinal lesions in the inactive case show ed significant fluorescence in the early arterial phase. However, the fluorescence increased in intensity through arteriovencus and venous phases. There was a residual fluorescence confined to the depigmented centre of the focal lesion [Figure - 13]. In one case of the active group, the dye leakage was marked and spreaded out beyond the limits of the focal lesion [Figure - 6]A.
There was profuse leakage of dye through the blood vessels over an area of retinitis proliferans. The dye not only stained the scar tissue, but leaked into the vitreous to form a diffuse cloud of fluorescence [Figure - 14].
Often the affected area stood out with increased fluorescence in the early arterial phase indicating an increased transmission of choroidal fluorescence through the pigment epithelium [Figure - 13]A &[Figure - 14]A. Very often the choroidal vascular fluorescence was seen with unusual' clarity [Figure - 12].
| Discussion|| |
Fluorescein angiegraphy reveals the affected veins and the site of the lesion. It demonstrates the sluggish state of venous circulation and the precise nature of altered haemodynamics. Ophthalmoscopy could miss relatively gross vascular lesions. They were often seen for the first time in their actual extension during photocoagulation when the background became pale (MEYER-SCHWICKERATH  ). On the other hand the finest of vascular anomalies could be demonstrated by fluorescein angiography.
The normal retinal vessels are impermeable to the fluorescein in circulation. The barrier seems to be at the level of the intima. Fluorescein leakage through the retinal vasculature is always pathological. It occurs through inflammed, new formed or otherwise damaged blood vessels. Fluorescein angiography therefore seems to be the only available method to study the vascular permeability as a sign of pathology.
The active areas were noted by marked increased vascularity [Figure - 6]A, extravasation of the fluorescein [Figure - 6]A &[Figure - 7]B and development of micro-aneurysms [Figure - 3],[Figure - 6]. They may be taken as the cardinal signs of activity of the disease. None of -these findings were seen in the inactive cases.
The selective distribution of the chorioretinal lesions under or close to the involved veins [Figure - 2],[Figure - 5]B,[Figure - 11],[Figure - 12] would indicate that the focal chorioretinitis patches were secondary to phlebitis. The patch would be already fluorescent during early arterial phase [Figure - 12]A. The fluorescence was - obviously a window- phenomenon of the choroidal fluorescence. However, the intensity of fluorescence. increased with time. Occasionally it did spread beyond the limit of the lesion [Figure - 5]A & [Figure - 12]B. There was a residual fluorescence [Figure - 12]C. The process may suggest that there was a dye leakage in the area, the source o' which may be either choroidal or retinal or both. The residual fluorescence may be due to staining of the sclera or the retina or both.
In general the description of active and inactive pictures conformed well to the respective groups. However, in the same fundus two different areas may show two different pictures; while one area was active other may have gone to the inactive stage.
Whatever may be the etiology, the acute inflammation of the veins appears to be the initial pathology. The inflammed vessel walls take up the fluorescein and there is frank extravasation of the dye at that site [Figure - 8]. The vascular lumen get partially or completely occluded. It is followed by retinal oedema and retinal haemorrhages. The venous obstruction leads to engorgements of the vascular bed. The development of microaneurysms and neovascularisation in the area may be due to tissue anoxia. Neovascularisation round the disc was common in cases with massive or repeated vitreous haemorrhages. It may be a nonspecific response to ocular tissue anoxia and unknown toxic factors associated with vitreous haemorrhages.
If the initial lesion is comparatively mild and massive haemorrhage has not complicated the picture the continued anoxia may lead to atrophy of the tissue with regression of the capillary bed. That may explain the relatively poor vascularity, absence of microaneurysms and new formed vessels in the inactive cases. What re mains are dilated tortuous channel which could be dilated capillaries. While we have not got any evidence of a true arterio-venous shunt as reported by JUTTE AND LEMKE  , we have demonstrated veno-venous shunts [Figure - 11]. Similarly, WESSING AND VON NoORDEN  has failed to detect a single instance of arterio-venous shunt. The continued delay in venous return in the area in such cases may be due to poor tissue circulation besides the organic obstruction to the venous drainage in the main trunk at the site of old phlebitis.
The part of the retina showing increased vascularity, microaneurysms, neovascularisation and extravasation of the dye can be treated with photocoagulation. It would achieve a similar stage of tissue atrophy and regression of the vascular bed as attained by nature in favourable cases. Fluorescein angiography provides an unique opportunity to define the extent and nature of vascular anomalies in the retina. As such it would help in precise and rational application of photocoagulation. There seems to be no justification in photocoagulating the larger veins affected by inflammation.
The fluorescein leakage through the new formed blood vessels of retinitis proliferans is understandable. Similarly, in our experience there was always a dye leakage from a rete mirabile formation. However, Jutte and Lemke (1965) report no leakage through such lesions. Its clinical significance is not clear.
The increased visibility of the choroidal fluorescence through the pigment epithelium in the area may mean some degenerative change of the pigment epithelium.
| Summary|| |
The characteristic features of Eale's disease as obtained by fluorescein photoangiographic study in 30 cases have been presented. Increased vascularity, extravasation of fluorescein and microaneurysms appear to be the cardinal signs of activity of the disease. The dilated turtuous vessels have been found to be either veins or veno-venous communications. The role of fluorescein angiography in planning and executing photocoagulation has been discussed.
| References|| |
Duke-Elder, S. and Dobree, J. H.: System of Ophthalmology Vol. XDiseases of the Retina, London, Henry Kimpton. 1967, p. 222.
Eisner, G.: Biomikroskopische Untersuchungen nach Fluoresceininjektion. Ophthalmologica. 150: 371.
Jutte, A. and Lemke, L.: Kapillardefekte imnetzhautkreislauf beiperiphlebitis retinae Klin. Mbl. Augenheilk. 149: 334, 1966.
Meyer-Schwickerath. G.: Eale's disease, Treatment with light-coagulation. Acta XIX Concilium Ophthalmologicum. 2: 862, 1962.
Mitsui, Y. and Matsubara: Clinical picture of Eale's disease and findings of fluorescein fundus and iris photographs. Jap. J. Clin. Ophth. 22: 195, 1968.
Novotny. H. R. and Alvis, D. L.: A method of photographing fluorescene in circulating blood in human retina. Circulation (NY) 24: 82, 1961.
Theodossiadis, G.: Fluorescein Angiography in Eale's disease. Amer. J. Ophth. 69: 271, 1970.
Wessing, A. and Von Noorden. G. K.: Fluorescein angiography of the retina, St. Louis, C. V. Mosby. 1969, pp. 76-85.
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6], [Figure - 7], [Figure - 8], [Figure - 9], [Figure - 10], [Figure - 11], [Figure - 12], [Figure - 13], [Figure - 14]
[Table - 1]