The Conjunctival Sign of Sickle-Cell Disease

The purpose of this paper is to report a characteristic appearance of the bulbar conjunctival circulation as a reliable sign in the diagnosis of clinically significant sickle-cell disease. Although general physical examination can offer many clues, it is proposed here that no other clinical observation establishes that diagnosis with as much certainty. Even routine laboratory tests are occasionally inconclusive, for sickled red blood cells are found in patients with the asymptomatic sickle-cell trait. In 1957, Goodman, von Sallmann, and Holland1described for the first time vascular loops and segments which were found near the limbus in several patients with heterozygous sickle-cell disease. Bulbar conjunctival changes have since been commented on by Lieb, Geeraets, and Guerry2in their monograph on sickle-cell retinopathy. In that report, the following conjunctival abnormalities were noted: icterus, telangiectasis, vascular stasis, and sausagelike dilatations. Their observations indicated a rather low incidence: Of 51 patients with