[Multifocal Vitelliform Retinal Lesion]. / Multifokálna viteliformná retinálna lézia.

The authors present retrospective follow up of patient with bilateral multifocal vitelliform retinal lesion during the 18 years period. At this time, spontaneous improvement of objective picture on retina and subjective visual troubles was observed. It is probable, that this case is a part of the same symptom complex as a variant of Best´s hereditary disease. This conclusion was based on initial stadium of phenotypical expressivity and additional evaluations. The course and outcomes of visual functions were different. The hereditary transmission was not confirmed.

[Clinical variability of Best's disease]. / Klinická rozmanitost Bestovej choroby.

Retrospective view of the various phenotypes 20 persons affected by classic solitary form of vitelliform macular dystrophy, in 3 pedigrees with autosomal dominant transmission and in 4 single cases. Long-term monitoring allows to observe the variability of expression, from classic course to peculiarity of the clinical expression in the disc development and their corresponding functions of the central retina.

[Retinitis septica Roth--a case report]. / Retinitis septica Roth.

We report of a case of retinitis septica in a 37-years old man one month after his tooth's extraction. Because of decreased right eye's central vision and a presence of typical retinal Roth's spots we called internists for a possibility of bacterial endocarditis. Cardiologic examination confirmed this disease together with aortal valve's defect. The course of hearth's disease was weary heavy, with attack of septic fever and cardial decompensation. After acute stage control, defocusation and antibiotic therapy, he underwent a surgical intervention with exchange of aortal valve.

[Spontaneous premacular hemorrhage]. / Spontánne premakulárne krvácanie.

PURPOSE: Among the great amount of etiologically heterogeneous group of general and specific eye diseases, a solitary, spontaneous, unilateral retinal hemorrhage restricted to the macula in young adults can be regarded as an entity. METHODS: Twelve eyes were phakic, emmetropic (including minor non corrected astigmatic errors) and were seen over a period of 5 years. All patients had a complete ophthalmologic examination with special analysis of history of direct or indirect trauma, additional medical work-up to exclude hematological disorders, and fluorescein angiography examinations up to the complete blood resolution. RESULTS: Hemorrhage is presented in 3 pictures: 1) Foveal microhemorrhage, 2) Drop-like hemorrhage limited to the avascular zone of the fovea, 3) Bag-like hemorrhage over the posterior pole. Initially, all patients had complains suggestive of positive central or paracentral scotomas with impaired visual acuity. The resolution of blood was spontaneous in 10 patients, after argon laser treatment in 2 patients. In all of them, the recovery of the visual acuity was complete. Possible pathogenetic mechanism such as Valsalva manoeuvre could be found in 3 cases only. CONCLUSIONS: Spontaneous macular hemorrhage is mostly idiopathic clinical entity in young adults with complete recovery of visual acuity.

[Idiopathic sclerochoroidal calcification]. / Idiopatická sklerochoroidálna kalcifikácia.

Idiopathic sclerochoroidal calcification is an uncommon ocular entity that manifests as calcium deposits in the mid-peripheral choroid and sclera. It is typical for older patients, without disturbances of visual functions, with low progression and almost no complications. Clinical characteristics, diagnostic laboratory tests, ancillary imaging techniques and systemic associations are discussed in this paper.

[Bilateral retinal vasculitis with arterial aneurysms]. / Bilaterálna retinálna vaskulitída s arteriálnymi aneuryzmami.

The authors reported unusual and rare condition of bilateral retinal vasculitis primarily affecting the central retinal artery at the nerve head and its 4 main branches. The most striking feature was the presence of the diffuse vitreous cells, occlusion of branch retinal artery, segmental periarterial infiltration, arterial sheating, retinal arterial aneurysms, disc swelling, peripheral retinal non perfusion and their complications. During 13 year's observation and treatment one eye went blind 3 years after initial examination. Second eye started the same clinical course two years after beginning of the disease. To avoid similar devastating course of the disease we started systemic steroids and immunosuppressive therapy, followed by photocoagulation of nonperfused peripheral retina and vitreoretinal surgery. We achieved stabilization of the disease with decreased visual functions. Comprehensive systemic work-up was unrevealing, no clear etiology was identified and diagnosis of idiopathic retinal vasculitis was made.

[Iris racemose vascular anomalies]. / Cievne anomálie dúhovky racemózneho typu.

Authors present 7 rare iris racemose vascular anomalies that were discovered by biomicroscopy during routine ocular examination. The morphology, clinical features and iris circulation of these cases are documented by iris fluoroangiography. They also report common features, some specific details of arrangement and classification criteria. The iris vascular anomaly appears to be benign stationary condition that has none systemic and ocular associations.

[Retinal and retinal pigment epithelium hamartoma]. / Kombinovaný hamartóm retiny a retinálneho pigmentového epitelu.

Authors present four patients with combined hamartoma of the retina and retinal pigment epithelium who were observed for a period of 10 years. The significance of this entity lies primarily in its resemblance to malignant condition of the retina and choroid and in the vizual reduction. Clinical finding, diagnosis, natural history, histopathology, association with other diseases and therapy are discussed in this report as well.

[Isolated and combined occlusion of the cilioretinal artery and the central retinal vein]. / Izolovaná a kombinovaná oklúzia cilioretinálnej artérie a centrálnej vény sietnice.

Cilioretinal artery occlusion is rare pathological condition because of infrequent occurrence of the cilioretinal artery in the human retina. It presents as an isolated entity, or as a combined cilioretinal artery--central retinal vascular, mostly venous occlusion. Authors present the course, angiographic features, prognosis, risk factors and final outcome of the isolated cilioretinal artery occlusion and combined cilioretinal artery--central retinal vein occlusion. Occlusion of the cilioretinal artery is believed to result from obstruction of the central retinal vein, as a primary process, in the case of combined cilioretinal artery--central retinal vein occlusion.

[Retinal vascular complications in systemic lupus erythematosus]. / Sietnicové cievne komplikácie systémového lupus erythematosus.

An analysis of three women with retinal vaso-occlusive disease in systemic lupus erythematosus produced evidence for this most severe ophthalmic complication during the acute phase of the syndrome. Vaso-occlusive retinopathy appeared 5 years after the onset of systemic symptomatology in all of them. Association of central nervous system lupus and circulating anti-coagulants with increased occurrence of severe retinal vaso-occlusive disease are the subject of the present report. Unilateral extensive photocoagulation of two cases appeared to result in successful therapy of neovascular glaucoma after the central retinal artery occlusion in one patient. Lupus erythematosus is very serious systemic and ocular disease also in present time.

[Kraupa's retinal vein (case reports)]. / Kraupova zila sietnice (kazuistika).

The authors describe four cases of rare and yet little known anomaly of the central retinal vein trunc exit on optical disc. All the anomalies occurred in myopic eyes with higher refractive errors. They did not cause of any retinal and choroidal vascular disturbances.

[Angiography of the iris in the diagnosis of pigmented tumors of the anterior uvea]. / Angiografia dúhovky v diagnostike pigmentových nádorov prednej uvey.

Authors present their observation of 28 pigmented tumors of the iris and iridociliary region during 8 years. While deciding whether to continue in observation or to indicate surgery, they were concentrated on observation of blood supply's abnormalities of pigmented mass and defect of blood-aqueous barrier during the iris angiography. Melanoma--mostly celullar type of high malignity--was confirmed in 11 cases, pigmented meduloepithelioma in 1 case, pigmented naevus in 2 cases out of 14 patients indicated for surgery. Authors correlated angiographical pictures with clinical observation and histopathological findings.

[Surgical treatment of pigmented tumors of the iris and iridociliary region]. / Chirurgická liecba pigmentových nádorov dúhovky a iridociliárnej oblasti.

The authors present a group of six patients with pigmented tumours of the iris and the iridocorneal angle where they performed excision of the tumour by iridectomy (3 patients) or iridiocycletomyy (3 patients). In all six patients with a pigmented tumour of the iris an impaired blood-ocular fluid barrier was found and colouration of the tumour mass by fluorescein. In one patient a heavily pigmented tumour was detected which masked the vascular network. Impaired permeability was found more markedly at the margins. Histological examination confirmed in five instances melanoma of the iris, in one instance a pigmented naevus was involved. During the mean observation period of 32.5 months none of the patients developed a relapse or metastases. Consistent with the extent of the surgical intervention, within 6 months at the latest in all patients the disorder of the blood-ocular fluid barrier improved.

Mutations in the VHL tumor suppressor gene and associated lesions in families with von Hippel-Lindau disease from central Europe.

von Hippel-Lindau (VHL) disease is a dominantly inherited familial cancer syndrome predisposing to retinal, cerebellar and spinal hemangioblastoma, renal cell carcinoma (RCC), pheochromocytoma and pancreatic tumors. Clinically two types of the disease can be distinguished: VHL type 1 (without pheochromocytoma) and VHL type 2 (with pheochromocytoma). We report VHL germline mutations and trends in phenotypic variation in families from central Europe. We identified 28 mutations in 53/65 (81.5%) families with 18 (64%) mutations being unique to this population. Whereas types and distribution of mutations as well as a strong correlation of missense mutations with the VHL 2 phenotype were similar to those identified in other populations, these families have provided new insights into the molecular basis for variability in the VHL 2 phenotype. Seven different missense mutations in exons 1 and 3 varied in their biological consequences from a minimal VHL 2 phenotype with pheochromocytoma only to a full VHL 2 phenotype with RCC and pancreatic lesion. These findings contribute to a better understanding of the fundamental mechanisms of VHL disease and its phenotypic variability. Further, we have provided rapid VHL screening for the families in central Europe, which has resulted in improved diagnosis and clinical management.

[Tortuous retinal vessels]. / Tortuositas vasorum retinae.

The authors observed an occurrence of tortuosity of retinal vessels from the group of 5,000 investigations of an ocular fundus. Elimination of all local reasons or symptoms of general diseases, even in spite of the great possibility of modification of this feature, three characteristic types were found. In two of them the familiar and hereditary occurrence was proved.

[Abnormal vessels in the iris in angiographic imaging]. / Abnormálne cievy dúhovky v angiografickom obraze.

During the examination of 210 eyes with blue irises by fluorescein angiography a few unusual vascular formations of the course, filling and caliber were revealed. Authors have divided them into four groups by similar angiographic pictures. Possible capillary haemangioma in connection with syndrome Sturge-Weber from the third group and angioma racemosum from the fourth group of examined irises are considered to be abnormal vessels. It is possible to state diagnosis by fluorescein angiography in many cases even without histological examination and to follow the dynamics of their possible development.

[Hamartomas of the optic disc and adjacent retina]. / Hamartómy terca zrakového nervu a prilahlej sietnice.

The authors discuss some formations on the optic disk and close neighbourhood resembling tumours. They originate from various cellular elements in that area and are described as hamartomas. A progressive growth was recorded only in vascular hamartomas after unsuccessful therapeutic intervention, the remainder were stationary during observation periods of various lengths.

[Early stages of angiomatosis of the retina and optic nerve disk]. / Vcasné stádiá angiomatózy sietnice a terca zrakového nervu.

Based on 13 years observations of three generations of relatives with v. Hippel-Lindau's disease the authors focus attention on early stages of clinically detectable retinal angiomatosis. They supplement these findings by an angioma on the optic disc in one sporadic case. They consider the ophthalmological diagnosis of priority importance for the patients and risk relatives and recommend a concept of presymptomatic screening to detect other organ sites of the disease. Early detection and treatment of lesions on eyes and other organs improves the prognosis and reduces early mortality.

[Differentiation and prognosis of colloidal retinal dystrophy]. / K diferenciácii a prognóze koloidnej dystrofie sietnice.

The authors investigated in a prospective investigation 27 patients of different age groups for 5 to 12 years with symmetrical, bilateral non-hereditary retinal drusen. The examination was focused on assessment of different types of drusen, on evaluation of the development and incidence of risk factors leading to complications and loss of central vision. The latter include the part of the spectrum of pathological manifestations in higher age groups which are included in the category of senile macular degeneration.

[Familial exudative Criswick-Schepens vitreoretinopathy]. / Familiárna exsudatívna vitreoretinopatia Criswick-Schepens.

The authors describe their own observation of a very rare form of vitreoretinal degeneration in a young man classified as familial exudative Criswick-Schepens vitreoretinopathy. The disease was detected in the second clinical stage on the right eye and in the third clinical stage on the left eye. The transmission of the disease is autosomal dominant with incomplete penetration. In the paper special emphasis is laid on differential diagnostic problems and the possibility of treatment of this disease.