[Coat's disease and Leber's miliary aneurysm retinitis].
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Biomedical subjects
Publications and source records attributed to M Spitznas.
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The paper describes new instruments for cutting of vitreous strands (vibrotome) and for removal of vitreous tissue (hyalophage). In addition, a motorized stereotaxic micromanipulator is presented.
Light coagulation of a retinal defect or of the whole buckle following successful retinal detachment surgery, can be replaced by cryopexy. This is particularly useful in the case of opacity of the refracting media, or where too contracted a pupil hinders light coagulation. The technique and advantages of this proceeding are discussed.
Analyzing the findings of 45 patients with primary epiretinal gliosis in 51 otherwise entirely healthy eyes, the clinical features of the disease are elaborated. The disorder is characterized by the occurance of a glial membrane covering the retina in the area of the posterior pole and leading to a distorsion of the posterior pole and leading to a distorsion of the subjacent and surrounding tissues. Most frequently, the foveal region is involved, but the foveola is often spared. With increasing distance from the center the frequency of involvement decreases. Only in very rare cases the membrane extends beyond a distance of 5,5 mm from the center of the retina. The density of the membrane varies from case to case. Average visual acuity of the eyes evaluated in this study was 0,45. In no case it was worse than 0,05. The incidence of the disease increased with both increasing myopia and hyperopia. The average age of the patients was 55 years. There was no predominance of either men or women. The glia cells which compose the membrane originate from the innermost layers of the retina, from where they extend on the retinal surface through a defect in the inner limiting membrane. The reason for the formation of the defects is not yet understood. It is also unknown if the presence of such defects is absolutely necessary for the development of this condition.
The HLA linkage group, located on chromosome 6, is composed of serologically defined (SD-) loci (HLA-A, -B and -C) and of a MLC (= mixed lymphocyte culture-) or lymphocyte defined (LD-) locus (HLA-D). Different alleles of these loci are linked together as well as with loci for the synthesis of some complement components and the properdin factor B (= Bf-system) by strong positive linkage disequilibria (gametic association). The indication of a HLA linked genetic control of immunoresponsiveness makes the HLA system one of the biologically most important immunogenetic systems in man. The associations, found between single HLA determinants and diseases, can be explained in different ways, but may contribute to a better understanding of the etiopathogenesis of diseases and of the biological role of the HLA linkage group.
In order to gain information on the possible role of lactate in the development of neovascularization following retinal malperfusion, the lactate content in vitreous-retina-choroid specimens of 02-treated kittens and streptozotocin-diabetic rats was determined. In both experiments there was no significant difference of lactate content between the diseased eyes and healthy controls.
71 eyes with Coats' disease were treated with photocoagulation. By coagulation of the abnormal vessels the disease process could be halted in 64 eyes over a mean observation time of 2 years and 3 months after completion of treatment. In 51 of these eyes the lipoid deposits regressed. In only 7 eyes a progression of the disease could not be prevented. Complications of photocoagulation treatment were not observed.
The internal portion of the capsule of the spinal ganglia of rabbit is composed of continuous superimposed layers of flattended cells (perineurium). In replicas, extensive meshworks of strands, presumably forming zonulae occludentes, are found between the perineurial cells of the same layer. Adjacent layers are joint by maculae occludentes located on the lateral processes of the perineurial cells. Occasionally small gap junctions lie within the meshes of the zonulae occludentes.
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Description of a new holder for experimental intraocular surgery on human autopsy eyes. Stability of shape and position of the experimental eyes is achieved by a vacuum surrounding the posterior segment.
A soft, self-adhering, biconcave silicone contact lens is presented. After surface anesthesia the lens can be placed on the cornea without additional contact fluid and a fundus area of about 35 degrees can be viewed. For vitreous surgery a modification with a flat or slightly convex surface is recommended.
The fluorescein angiographic picture of sympathetic ophthalmia is described in an instructive case, which first started at the posterior pole. In the acute phase there was a picture of an exudative process with multiple subretinal enlarging hyperfluorecent spots and some dye-pooling. The retinal vasculature was unaffected. In the healing phase a transitory involvement of the optic nerve head was observed. The cicatricial phase was characterized by a coarsening of the pigment pattern of the fundus. In addition to fluorescein angiography the degree of transitory hyperopia was an excellent indicator of the activity of the disease process.
Coats' disease represents a disorder of the retinal vascular walls. In a series of 112 consecutive eyes with Coats' disease the involvement was unilateral in 90.2%. The majority (72%) of patients were male. 57.8% were under 20 years of age. There was, however, a second peak of the disease (30.4%) between the age of 41 and 60. Severe cases were found mainly among juvenile patients, whereas in the senile group milder cases prevailed. Fluorescein angiographic findings comprised vascular aneurysms in almost all cases, rarefaction and coarsening of the capillary bed in 71%, capillary occlusions in 70%, occlusions of arterioles or arteries in 27%, shunt formations in 27%, extensive widening of arteries or veins in 27% and leakage of dye mainly in advanced cases. The participation of the central retina in the disease process was variable. Evidence is presented that Leber's military aneurysm retinitis is not a separate entity but a special form of Coats' disease.
The clinical findings in 316 eyes with Eales' disease and 41 eyes true idiopathic periphlebitis were analyzed. Eales' disease differed clearly from idiopathic periphlebitis in a predominance of male patients, a marked tendency toward bilateral disease in males, the aspect of vascular sheathing and the absence of inflammatory signs from the vitreous body. The clinical picture of Eales' disease was characterized by avascular areas in the retinal periphery, followed posteriorly by microaneurysms, rope-ladder-like dilations of capillary channels, tortousity of neighbouring vessels and spontaneous choriortinal scars. The more pronounced finds were neovascularizations (84%) hemorrhages (58%), obliterated vessels (39%) and vascular sheathing (34%). In contrast to idiopathic periphlebitis, Eales' disease is considered a primary, non-inflammatory disorder of the walls of the peripheral retinal vessels, namely the shunt vessels.
224 eyes with Eales' disease were subjected to photocoagulation. In 205 (=91%) the disease could be brought to a morphological standstill over a mean observation time of 5 years after completion of treatment. Visual acuity remained unchanged or improved in 185 eyes (=87%). Postcoagulative macular puckering occured in 6 eyes (=3%).
Ridges and grooves composing extensive zonulae occludentes are revealed by the freeze-fracture method on split myelin lamellae in the nerve fibre layer of the retina and in the optic nerve of the rabbit. The junctions are located immediately internal to the outer loop of the myelin sheath and in corresponding areas of deeper myelin layers. They follow a straight or gently undulating course along the axis of the fibres. Only at the paranodal region of nodes of Ranvier do they deviate and assume a transverse course, The strands of these zonulae occludentes probably represent the radial thickenings of the intraperiod line described in thin sections.
Replicas of the perineurium of rabbit sciatic nerve fascicles show extensive zonulae occludentes between the flattened cells of each perineuriallamella. The junctions formed by the zonulae occludentes are of the morphologically 'tight' type. Therefore, theyare likely to represent the anatomical site of the diffusion barrier previously demonstrated by other morphological and physiological methods. Neighbouring perineurial lamellae are connected to one another by numerous maculae occludentes, some of which lie on thelateral processes of the perineurial cells. Within the meshes of the maculae occludentes and close tothem, small gap junctions can be observed.
Replicas of freeze-fractured endothelial cells of human choroidal capillaries show fenestrations with a central thickening, as described in thin-section studies. Between the endothelial cells there are discontinuous zonulae occludentes (i.e., maculae or fasciae occludentes), composed of ridges on face A and grooves on face B of the split-cell membrane. They are also present along the sutures of the endothelial cells of venules. Close to the intercellular suture gap junctions, probably connecting capillary endothelial cells and pericytes, are found.