|Year : 2002 | Volume
| Issue : 3 | Page : 217-219
The indocyanine green findings in idiopathic uveal effusion syndrome
A Kumar, S Kedar, Rajinder P Singh
Centre for Ophthalmic Sciences, All Institute of Medical Sciences, New Delhi, India
Centre for Ophthalmic Sciences, All Institute of Medical Sciences, New Delhi
Source of Support: None, Conflict of Interest: None
We report two cases clinically diagnosed as idiopathic uveal effusion syndrome, where indocyanine green angiogram suggest non-specific choroidal inflammation as the underlying cause. Treatment with non-steroidal anti-inflammatory drugs was beneficial.
Keywords: Idiopathic uveal effusion, indocyanine green angiogram, choroid, non-steroidal antiinflammatory drugs
|How to cite this article:|
Kumar A, Kedar S, Singh RP. The indocyanine green findings in idiopathic uveal effusion syndrome. Indian J Ophthalmol 2002;50:217-9
|How to cite this URL:|
Kumar A, Kedar S, Singh RP. The indocyanine green findings in idiopathic uveal effusion syndrome. Indian J Ophthalmol [serial online] 2002 [cited 2020 Jun 3];50:217-9. Available from: http://www.ijo.in/text.asp?2002/50/3/217/14781
First described by Schepen and Brockhurst in 1963, Idiopathic uveal effusion syndrome is a rare condition causing non-rhegmatogenous retinal detachment and uveal effusion. It remains a diagnosis of exclusion after excluding the various inflammatory and hydrostatic factors of choroidal detachments. It typically affects males (almost exclusively), who present with a slowly progressive bilateral (65% of all cases) 1 non-rhegmatogenous retinal detachment with shifting subretinal fluid and is often associated with choroidal detachments. The cereboo spinal fluid pressure and proteins are found to be elevated in these patients without pleocytosis. Unlike uveal effusion in nanophthalmic eyes, idiopathic uveal effusion syndrome occurs in normal-sized eyes, which have a variable (often normal) scleral thickness. The pathogenesis of idiopathic uveal effusion syndrome has not been conclusively established and as a result the management these cases remains controversial. We believe that indocyanine Green (ICG) angiogram could shed more light on the pathogenesis of this condition through imaging of the choroid tissue.
| Case 1|| |
A 50-year-old male presented to the vitreoretina services at our center on 24th September 1998, with complaints of rapid, painless decrease of vision in both eyes over a period of 3 months. He was a known diabetic on oral hypoglycemic drugs. At presentation he had a visual acuity of 6/6 (right eye) and 6/9 (left eye). Ocular examination revealed retinal detachment with choroidal detachment in both eyes (left eye worse). The detachment, which was shifting in nature, was confined to the inferior retina in the upright posture and detaching macula in the supine position. The vitreous was clear with no evidence of flare or cells. The optic nerve head, macula and the retinal vasculature were normal in both eyes. The anterior segment, adnexa and intraocular pressure were normal. The pupillary reactions were brisk. The axial length of both eyes was normal and the scleral spike on ultrasonograpy showed no obvious thickening. The MR scan of the eyes also confirmed a normal scleral thickness. Fluorescein angiography (Imagenet systems, TOPCON Inc. Japan) [Figure - 1] showed diffuse choroidal hyperfluorescence, well-defined patches of hyperfluorescence (leakage) in the late venous phases, not obscuring the normal retinal vasculature, and a mild hyperfluorescence of the disc margins suggestive of an early oedema. ICG angiogram [Figure:2a]-[Figure:2c] shows dilated medium to large choroidal vessels with a perivascular leakage of these vessels, starting in the late phases of the angiogram and persisting well beyond 30 minutes. Focal areas of late phase choroidal hyperfluorescence are also evident. He was started on topical prednisolone acetate 1% (q4h) and systemic corticosteroids (prednisolone 60mg daily) but had to be tapered and stopped within 40 days due to their adverse systemic effects. Over the next 6 months the vision in the right eye deteriorated from 6/6 to hand movements with inaccurate projection of rays in one quadrant and in the left eye from 6/9 to counting fingers. An increasing detachment along with the evolution of early retinal folds was seen in both the eyes (right more than left) during the next three months. Repeat fundus fluorescein angiogram (at 3 and 6 months follow-up) showed an exaggeration of the diffuse choroidal hyperfluorescence seen at presentation. A full scatter laser photocogulation was done in both eyes in the attached retina. He was then put on longterm non-steroidal antinflammatory drugs (NSAID) (Cap Indomethacin SR 100 mg 16H). Six months later the right eye showed a marginal improvement in vision to CF (inaccurate projection in 1 quadrant) and the left eye improved to 6/12. The detachment had settled in both eyes but the right eye showed subretinal exudates involving the macula. Fluorescein angiography showed no areas of leakage in both eyes.
| Case 2|| |
This 32-year-old male was referred to the vitreoretina service of our center with a diagnosis of failed RD surgery (right eye); subtotal retinal detachment (left eye). He experienced painless decrease in vision 4 months earlier in the right eye, and a month later in the left. A conventional buckling surgery was attempted at the peripheral center for the right eye, which failed on the second postoperative day. Visual acuity at presentation was hand movements with inaccurate projection in 2 quadrants (right eye) and 6/60 (left eye). The anterior segment, adnexa and the intraocular pressure were normal and the pupillary reactions were brisk. Indirect ophthalmoscopy revealed retinal detachment in both eyes; the right eye had total retinal detachment while left eye had subtotal detachment involving the macula, with shifting subretinal fluids and areas of pigment degeneration. A battery of investigations including haemogram, serology for toxoplasmosis, HIV, tuberculosis and syphilis, thyroid function tests and chest X-ray, were within normal limits. Fluorescein angiography [Figure - 3] revealed well-defined areas of patchy choridal hyperfluorscence in the late phase, with the retinal vessels seen clearly over the patches in both the eyes. Peripapillary leaks were also visible. Indocyanine green [Figure:4a]-[Figure:4b] angiography showed areas of patchy hyperfluorescence around areas of dilated choroidal vessels, starting in the late phases of the angiogram and corresponding to the areas of leakage on the fluorescein angiogram. A diagnosis of exudative retinal detachment due to idiopathic uveal effusion syndrome was made. A full scatter laser photocogulation was done in both eyes in the attached retina and subsequently the patient was put on oral sustained release indomethacin 100mg q4H and topical NSAID eye drops. At the last follow-up (9 months after presentation) the patient has a visual acuity of HM with inaccurate projection in 2 quadrants (right eye) and 6/36 (left eye). Though the detachment had settled in both the eyes at the last follow-up, the fundus in both eyes showed diffuse spotty pigmentation with proliferative vitreo-retinopathy changes in the right eye.
| Discussion|| |
Idiopathic uveal effusion syndrome is often a diagnosis of exclusion. There is as yet no definitive method of investigation to confirm the diagnosis. Even more controversial is the subject of pathogenesis of idiopathic uveal effusion syndrome, which remains a matter of debate. Gass proposed that the effusion results from an increased oncotic pressure in the choroidal tissue subsequent to protein retention in the choroidal tissue. This protein retention results from the impedance to the transcleral flow of fluid and proteins into the general circulation by a thickened sclera. Forrester et al suggest that the thickened sclera in idiopathic uveal effusion syndrome is due to an abnormal accumulation of dermatan sulfates in the sclera, possibly as a manifestation of a form of mucopolysaccharidosis.
Effusion requires an imbalance of the Starling forces acting across a blood vessel, i.e., the interstitial and the intravascular hydrostatic and oncotic pressures, provided the permeability coefficient of the vessel wall is a constant. The theories of Gass and Forrester et al essentially propose that the effusion in idopathic uveal effusion syndrome is a result of the altered hydrostatic forces in the choroidal vasculature due to the thickened sclera, which provides an impedance to the flow of blood from the choroid into the general circulation. These theories could explain those cases of idiopathic uveal effusion syndrome where the scleral thickness was found to be increased but fail to explain those cases where the scleral thickness was normal. We believe that in these cases it is the altered permeability of the choroidal vasculature that causes the effusion. Evidence for this is provided by the ICG angiogram findings in both our cases, which demonstrated focal areas of late phase choroidal hyperfluorescence (suggestive of choroidal hyperpermeability), and dilated and tortuous large choroidal vessels in the first case. The reasons for choroidal vessels hyperpermeabiity is not known. We can only speculate the existence of a nonspecific choroidal inflammation involving these medium to large choroidal vessels.
Treatment of idiopathic uveal effusion syndrome is extremely frustrating. The disease tends to be chronic, often lasting several years. Some cases may go into remission with retinal reattachment but vision is usually poor because of photoreceptor damage as a result of the long duration of retinal detachment. Drainage of subretinal fluid and scleral buckling procedures have not helped. The MRI study in both patients clearly showed a normal sized eye with normal scleral thickness. We believe that in such patients there is no role of vortex vein decompression or scleral resection as described by various authors. [1,6]
Treatment with high dose systemic corticosteroids has been described with rare success. We used longterm oral NSAIDs (slow release indomethacin) and scatter laser photocoagulation in the attached retina in both the cases. Over a period of time not only did the retinal status improve but also the visual acuity. However, from these cases it is difficult to know whether improvement was spontaneous or secondary to the laser treatment, or the longterm NSAIDs. If indeed the concept of choroidal inflammation as indicated by the Indocyanine Green (ICG) angiogram is correct, then the use of longterm NSAIDs should have a beneficial effect on these patients.
The indocyanine green (ICG) angiogram findings in idiopathic uveal effusion syndrome were described in a recent article by Masanobu et al. They demonstrated diffusely granular, marked hyperfluorescence in the choroidal fluorescence in the very early phase, which increased with time and persisted until the late phase as diffuse intense choroidal hyperfluorescence.
This report is the first to describe the choroidal pathology, i.e., patchy hyperfluorescence suggestive of a nonspecific choroidal inflammation, of idiopathic uveal effusion syndrome which responds to longterm NSAID therapy.
| References|| |
Gass JDM, Jallow S. Idiopathic serous detachment of the choroid, ciliary body and the retina (uveal effusion) syndrome. Ophthalmology
Schepens CL, Brockhurst RJ. Uveal effusion. I: Clinical Picture. Arch Ophthalmol
Brockhurst RJ. Discussion of Gass JDM. Uveal effusion syndrome: A new hypothesis concerning pathogenesis and technique of surgical treatment. Trans Am Ophthalmol Soc
Gass JDM. Uveal effusion syndrome. A new hypothesis concerning pathogenesis and technique of surgical treatment. Trans Am Ophthalmol Soc
Forrester JV, Lee WR, Kerr PR, Dua HS. The uveal effusion syndrome and trans-scleral flow. Eye
Johnson MW, Gass JDM. Surgical management of the idiopathic uveal effusion syndrome. Ophthalmology
Uyama M, Takahashi K, Kozaki J, Tagami N, Takada Y, Chkuma H, et al. Uveal effusion syndrome: Clinical features, surgical treatment, histologic examination of the sclera, and pathophysiology. Ophthalmology
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6], [Figure - 7]
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