[Heterochromia complicata Fuchs]

Abstract

We are indebted to I. Loewenfeld and her ophthalmologist colleague H. S. Thompson for having called for an explanation of the clinical picture of Fuchs's heterochromic cyclitis in their "Critical Review." In the present author's view, the reason why so many different opinions are held concerning the clinical picture and the etiology is that only a few authors (Franceschetti, François, Georgiades, Hollwich, Huber, Kimura, Hogan and Thygeson, Perkins, Sugar and others) have been able to follow up collectives of their own, of up to 50 patients and more, for many years. None of the investigators deny the presence of typical precipitates, observed both under direct light and retroillumination; typical because they are only found in Fuchs's heterochromia and its variation Posner-Schlossman syndrome. The course is inflammation-free, since, in contrast to all other forms of diseases of the anterior uvea, neither external signs of inflammation nor posterior synechiae occur. Etiologically, according to the behavior of the pupil (François 1949, 1954), there is sympathicoparalysis, while according to Amsler and Huber as well as Verrey, Franceschetti and Herrmann there is a corresponding pathologically increased fluorescein permeability of the vessels in the anterior segment and an extreme tendency to bleeding, as shown by the filiform bleeding when the anterior chamber is opened. The sympathicoparalysis also explains the inflammation-free vascular fragility, with escape of cell elements, primarily protein (albumins) and lymphocytes (Verrey, Matteucci, Franceschetti and Hermann, and François) into the aqueous and vitreous. Therefore, the syndrome should no longer be termed "heterochromic cyclitis" but rather "heterochromia complicata" as proposed by E. Fuchs. In view of the somatic features, amounting to a status dysraphicus, the condition is probably connected with a congenital developmental anomaly of the sympathetic nerve (François); these features have been described by Franceschetti, Hollwich, Passow, Perkins, Sugar, Huber and many others. There may also be immunologic factors (Loewenfeld and Thompson); however, research into these is still only at an early stage.