[The 80th anniversary of Professor Custodis, 23 March 1978].
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Biomedical subjects
Publications and source records attributed to H Pau.
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Mooren's ulcer has not yet been treated successfully with drugs. Cautery, cryocoagulation, surgical diathermy, lamellar or perforating keratoplasty, and conjunctivoplasty usually have no or only little success. We have been able to cure our last five cases of Mooren's ulcer by an extensive and deep thermocoagulation both of the ulcer, the swollen corneal margins and the bordering conjunctiva. Then followed dilatation of the conjunctival vessels with Dionine and Priscol ointments.
In a 33-year-old patient discrete conjunctivitides occured over a period of 3 years. Grey-white, fibrinous, spongy conjunctival swellings and white areas of cartilage-like consistency occured in relation with these. Microscopically these lesions showed parakeratotic cornified stratified epithelium, leukoplakias, chronic inflammation, round cell infiltrates, and vessel sprouts. Immunofluorescence showed, in the preparations incubated with antihuman compliment (C3), what could be described as characteristic for allergic vasculitis.
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The way in which a varying amount of blood runs past an almost obliterating yellowish-grey, homogenous "embolus" in a branch of the artery is shown. Finally although the "embolus" remained, there was an almost normal blood flow in amount. Retinal arteries running downwards showed changing vascular spasms, sometimes at the origin, sometimes further away from the central artery. This produces reversible scotomas. A small artery running nasally is sometimes better and sometimes worse supplied with blood. Temporary and permanent scotomata develop. Haemorrhages appearing here vary in extent.
In the lens, disorders of the metabolism occur, and, with them, active permeability (the cation pump with uptake of K and release of Na) changes to passive permeability and consequently Na ions enter with water. As a result, the lens increases in weight and a subcapsular (permeability) cataract develops. It is shown that the cattle lens in vitro increases in weight the lower the pH (6.5 greater than 7.5 greater than 8.5) of the surrounding fluid becomes. In a further experiment, 1 ml of buffered liquids with different pH were injected into the anterior chamber of the eyes of freshly slaughtered cattle. Here, too, the mechanically undamaged, untouched lens increased in weight more greatly as the pH (5.5 greater than 6.5 greater than 7.5 greater than 8.5) of the injected fluid was lowered. The significance of the lowering of the pH, e.g., in local inflammation (iritis, cyclitis, retinitis, etc.) or general acidoses (diabetes mellitus, galactosemia, hunger, extracorporeal circulation for atrophic kidney. Albright-, Love-, Fanconi-syndrome) for the appearance of incipient subcapsular clouding of the lens is pointed out.
Latticed or dendritic lines (true white lines) on the periphery of the retina. Vitreo-retinal adherences between the ora and equator correspond to former embryonal blood vessels to the vitreous (or retinal vessels) attaching to the retina. Between attachments of vitreous consolidations coming from in front and behind as a congenital anomaly of the retina, the latter is either loosened or replaced by connective tissue, or there is a defect, wedge-shaped from within outwards (colobomatous). These are obviously very frequently comgenital. The vitreous between and above the vitreous-retinal attachments is always liquefied and structureless. With increasing age, there is a reactive proliferation of the connective tissue (vessel-vitreous medullary cells) and pigmented epithelium. This new tissue either replaces the retina or rests on it distinctly localized, and has a strong tendency to hyalinization and sclerosis (sclerotic degenerative area). The latticed or dendritic lines (true white lines) on the periphery of the retina which for preference appear in the region of the degenerative sclerotic area sometimes consist of obliterated vessels with hyalinized walls and sometimes of hyaline connective tissue threads which are independent of the vessels. Frequently the vitreous fibre strands (vitreo-retinal adherences) may also continue through the inner retinal layers as far as these dendritic hyaline (white) retinal threads of connective tissue.
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V-shaped retinal defects which are usually parallel to the equator, develop as a congenital anomaly at the insertion of vitreous fibrils. The vitreous overlying these retinal areas is without structure. Progressive degenerative-sclerotic areas, equatorial degeneration-or lattice degeneration can develop in the region of these vitreo-retinal adhesions.
The picture of the grey retinal coagulation focus after light coagulation due to observation of an eclipse of the sun corresponds to the picture of the sun's disc not covered by the moon.
1. Lange's fold of the ora frequently found at autopsy in fetuses and infants is a purely postmortal change. 2. The 'honeycomb ora' occurring in infants and small children may sometimes be present in vivo. The extensive and larger cavities however may well have appeared only postmortally here too (they are not seen in older children). 3. Both the typical or external and the reticular or internal cystoid degeneration of the peripheral retina increase in frequency and extent with increasing age. Both occur in vivo. Biomicroscopically cystoid degenerations apparently can be demonstrated in any living eye after indentation of the ora. Basal membrane fragments and microfibrils have sometimes been shown by staining in the cyst walls (when cyst and vitreous body are connected) and mucopolysaccharides in the cystoid cavities. But there are also cystoid degenerations occurring postmortally. The picture of internal cystoid degeneration can also be seen as a fixation artefact (only) in fetuses in not yet vascularised sections of the retina. In the cystoid cavities one finds histologically sometimes reaction-free cell debris, torn fibres and isolated cell nuclei of the granular layers. Whereas the biomicroscopically found cystoid degenerations almost always end (well) in front of the equator of the eyeball, the histologically found cystoid degenerations are seen relatively often considerably behind the equator. Scotomas to be expected in vivo in these cases have not been observed so far. The periphery of the retina is histologically often torn off the ora without any reaction and displaced far forward. The picture of cystoid degeneration can also be produced quite artificially by bulging of the retina due to fixation in histology (different treatment of the two halves of the eyeball). Even without any tissue participation a picture like cystoid degeneration below the peripheral retina, between it and the pigmented epithelium, may develop in celloidin itself if too quickly hardened. 4. Retinal cysts found relatively often histologically are predominantly extensive cystoid degenerations. Exceptionally these are apparently identical with the rare cysts or retinoschisis cases visible ophthalmoscopically. 5. Small to large-cystic retinal degenerations are mostly secondary reactive changes. The situation of Lange's fold, honeycomb ora and especially cystoid degenerations depends postmortally (but also apparently in vivo) on the pull of the zonule and vitreous body fibrils as regards extent and direction. The intravitally present cavities in (honeycomb ora and) cystoid degeneration are considerably increased postmortally and apparently still more by histological preparation. In addition there seems to be an age-specific alteration in the ratio of the pull of zonule-vitreous body fibrils on the one hand and the (age-dependent) tearability of the retina on the other: in fetuses and infants we get Lange's fold, in infants and small children honeycomb ora and in later childhood increasingly extensive cystoid degenerations.
In two patients with mycosis fungoides the following were found in the region of the eye: Infiltration of the eyelid (sometimes board-like), infiltrations and swellings of the connective tissue, orbital infiltrations (hard as a board) with secondary glaucoma, atrophy of the optic nerve, amaurosis, Descemet spots, cells in the aqueous humour, preretinal opacities of the vitreous body), intussusception of retinal vessels, grey infiltration of the retina.
Incipient siderosis of the lens is reflected by an extremely fine granular, almost homogenous closely subcapsular brown discoloration between the anterior capsule of the lens and the epithelium. Advanced siderosis of the lens leads to subcapsular "rust spots" of varying size, especially in the region below the pupil; to increasing permeability cataract with protein breakdown and also to brown discoloration of the developing hollow spaces and clefts containing protein. In extensive siderosis of the lens, there was the following histological evidence of iron infiltration colour reactions: Between epithelium and capsule: (fusiformly) changed epithelia, intercellular substance formed by metaplasia of epithelia with connective tissue fibrillae (birefractive capsular cataract), in large, balloon-like epithelial cells freely occurring in the liquefied capsule, by decomposition of these cells in all fluid cavities containing protein (water clefts, etc.). On the other hand, there were no siderous granulations of protein: in normal epithelial cells of the lens, in unchanged fibres of the lens, in Wedl's cells and in Morgagni's or myelin droplets.
On classifying 1000 senile cataracts it was found that in pure cataract forms - starting from the cortex - the typical grey senile cataract (cataracta supranuclearis) mostly occurs with a clear nucleus. The dense permeability cataract (cataracta subcapsularis) most frequently shows a dense grey clouding of the nucleus and in addition a still smaller increase in frequency with a clear nucleus. The cortex was also frequently clear when the nucleus of the lens was a dense cloudy grey. Conversley, starting from pure nuclear cataract forms, the dense grey clouded lens nucleus most frequently accompanies a permeability cataract (cataracta subcapsularis) and - with a second peak - with a clear cortex. The brown (black) nucleus (cataracta brunescens [nigral]) quite predominantly shows a clear cortex. If all the cataracts, including the mixed forms between the various cortical and nuclear cataracts are so classified that the percentage participation of the individual forms of cataract in the entire collective (including the mixed forms) is summarised, then the distribution is: Typical grey senile cataract (cataracta supranuclearis) in 23%. Permeability cataract (cataracta subcapsularis) in 36%. Light brown-grey nuclear cataract (mainly nuclear sclerosis) in 13%, grey nuclear cataract in 37%, brown nuclear cataract in 5%, mature cataract in 4%, hypermature cataract in 1%, intumescent cataract in 11%.
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