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The uveal effusion syndrome is a rare disease characterized by serous choroidal detachment. The pathogenesis of idiopathic uveal effusion syndrome has not yet been conclusively established. One hypothesis is an abnormality of diffusion of extravascular proteins in the choroid leading to decompensation of the pigment epithelium pumping capacity. Fluid then accumulates in the subretinal space leading to retinal detachment which results in loss of visual acuity. It typically affects males and hypermetropia is another risk factor. When looking at the fundus a circular serous detachment of the choroid and choroidal puckering is typical. The fluorescein angiography shows hyperfluorescence in the form of a leopard-spot pattern. Space-occupying lesions have to be excluded with the help of ultrasound or magnetic resonance tomography. The uveal effusion syndrome is a diagnosis by exclusion. Treatment varies because of the different hypotheses for the pathogenesis. An intraocular tamponade in combination with laser coagulation may for example be an effective treatment.

We present the case of a 41-year-old female patient with acute onset of nyctalopia, xanthopsia and impairment of visual field. The ophthalmologic examination revealed peripheral choroidal elevation with accompanying retinal detachment. Due to the the presence of a nanophthalmic eye without any evidence of inflammation or malignant growth, the diagnosis uveal effusion syndrome could be made.

From 1983 to 1994 enucleated as well as bulbi obtained post mortem from 20 guinea pigs were ophthalmopathologically examined routinely. Eleven cases (one bilateral) with anamneses as hydrophthalmus, exophthalmus or retrobulbar abscess showed histologically osseous choristomas in the stroma of the ciliary body. In one case the osseous choristoma was already macroscopically recognizable. These heterotopic bone formations were causative for a secondary open angle glaucoma. Furthermore, it explains the obscured clinical symptoms of glaucoma together with the resulting exposition keratopathy. The aetiopathogenesis is discussed comparatively.

A six year old domestic cat, blind in the right eye from birth, slowly lost sight in the left eye. An ophthalmological examination of the right eye revealed uveal leucosis and unilateral duplication of the uveal tract. The additional tissues were rotated through 90 degrees resulting in displacement of the lens. In the left eye evidence of a previous retinal detachment and consecutive traumatic cataract were noted. The etiology of these pathological findings is discussed.

A report is given of a case of a unilateral condition of this type in a 30-year-old woman, who was observed and repeatedly photographed for more as 7 years.

This paper reports on two patients with bilateral, spontaneous choroidal and retinal detachment. One patient had scleritis, while in the other no underlying eye disease was found. No signs of orbital venous congestion were detected. Although the pathogenesis of this disease is not fully known, the findings of (1) dilated episcleral veins and (2) elevated episcleral venous pressure suggest an obstruction of venous outflow from the uveal tract. The authors believe that these two findings may have been caused by shunting of venous blood anteriorly from the uveal tract into the episcleral veins due to partial obstruction of the vortex veins.

A mentally and physically retarded 4 1/2 year-old boy with epileptic seizures showed a megalocornea on both sides, a coloboma of the iris in the right eye and a white area at the temporal side of the disc in the left eye. At first a coloboma of the disc was suspected. By further controls at the age of 8 years a typical two diopters elevated nodular opaque white mass was seen in place of the white area in the left eye, in addition two flat tumours were also seen. In the right eye with coloboma of the iris there was also a flat area. Radiographically the right kidney showed two ureters with flat calyces. At the age of 8 years symmetrical face naevi occurred only under atropine medication, and showed at the age of 10 years the typical picture of Pringle's tumours.

A dominantly inherited cleavage of the pigment layer of the iris has been observed throughout three generations. In the two elder generations, luxation and rapidly progressing opacification of the lenses occurred; after cataract extraction, a cleavage of the pigment layer of the ciliary body could also be seen through the iridectomy. The lenses had reduced sagital and spherical diameters (4X7 mm). Furthermore, a peculiar form of glaucoma and a peripheral retinal detachment has been observed in the eldest generation.

It was recently stressed by Scheerer that retinal detachment in a highly myopic eye may be caused by hyperaemia of the choroid, presumably occuring during heavy physical labour which is performed by a non-trained person. Clinical studies have shown, however, that choroidal detachment, resulting from either hemorrhagic, or serous accumulation of fluid during operative procedures or during postoperative periods, is not followed by retinal detachment. Also experimental occlusion of large choroidal vessels, associated with hyperaemia in the choroidal vasculature, does not lead to retinal changes inducing the separation of the neuroretina from the pigment epithelium layer. Thus, it is concluded that choroidal hyperaemia, possibly occuring during heavy physical labour, has no relationship to the development of a retinal detachment in a "disposed" eye.