Coats's disease: definition and pathogenesis.

Abstract

IT is remarkable that only one or two writers on Coats's disease appear to have been aware of the fact that Coats had written TWO articles on retinal disease with massive exudation: (1) his primary paper which appeared in the English language (1908), and (2) his final article which was published 4 years later (1912) in the German language. In the second paper, which has apparently been overlooked by most students of Coats's disease, the author gave evidence that his opinion had changed somewhat in the intervening years. In 1908 Coats divided his cases into two groups: those with and those without gross vascular disease and also mentioned "a very peculiar group, perhaps related to these two, which is characterized by the formation of large arteriovenous communications". In his final paper Coats placed his two former groups together because the similarities between the two groups were probably greater than the differences ("die Ahnlichkeiten sind wahrscheinlich gr6sser als die Unterschiede ... ich werde sie zusammengruppieren"), and he eliminated the third group because von Hippel had shown that this condition was in fact a retinal angiomatosis and, therefore, quite different from that described by Coats (".... der Zustand ist in Wahrheit eine Angiomatosis der Retina und daher verschieden von den hier berichteten Fallen. Aus diesen Grund unterlasse ich weitere Betrachtung dieser Gruppe"). In 1912 Coats also emphasized that the disease almost invariably occurred in young people. He mentioned that white masses of exudate below the retinal vessels were often encountered in the macular region in elderly subjects, but that these were mostly connected with senile vascular disease of a different aetiology ("Die Krankheit kommt fast immer bei jungen Menschen vor. Es ist richtig, dass sich weisse Exsudatmassen unter den retinalen Gefiissen nicht selten in der Macula bei alten Personen finden, aber diese Falle sind meist verbunden mit seniler Gefasserkrankung ... ihre Atiologie ist verschieden"). Coats (1912) thus defined one sharply-outlined form of exudative retinitis, clinically characterized as follows: (1) Occurrence in infantile or juvenile male patients; (2) Unilaterality; (3) Absence of systemic diseases; (4) Exudates below the retinal vessels; (5) Retinal haemorrhages; (6) Slow progression to retinal detachment, cataract, atrophy, or glaucoma.

Retinal Telangiectasis in Facioscapulohumeral Muscular Dystrophy With Deafness

“…Ophthalmoscopic exam¬ ination revealed bilateral peripheral telan¬ giectasis but no posterior pole disease. Fluorescein angiographie examination (April 13,1984) confirmed bilateral telan¬ giectasis, with abnormal fluorescein leak¬ age and areas of capillary closure (Fig 2).…”

Coats' syndrome: long term follow up

“…Many investigators studied infectious, inflammatory, metabolic, and other aetiologies, and some suggested different names for this condition 6. Three, several other local and systemic disease processes can produce retinal exudation, and many publications as late as the 1960s included very different disease processes under a slightly different rubric, Coats' disease 7. In addition, Coats' disease has been described as a syndrome in association with several hereditable ocular diseases including retinitis pigmentosa, Hallermann–Streiff syndrome, Senior–Loken syndrome, Cornelia De Lange syndrome, Parry–Romberg syndrome and the epidermal naevus syndrome 8-13…”

Vitrectomy techniques in late-stage Coats'-like exudative retinal detachment

“…This description was later revised to exclude retinal hemorrhage [2][3] and to include bilaterality [4][5][6]. In the earlier stages of the disease externally applied diathermy [4,7], laser photocoagulation or cryotherapy alone may be effective in destroying the retinal telangiectasia [7][8][9][10][11].…”