Congenital Horner's syndrome observed in an adult

Abstract

A 55-year-old woman reported, since infancy, right-sided ptosis (Fig. 1A, 1B), anhidrosis, exertional pallor (Fig. 1B), and lightly pigmented iris with miosis (Fig. 1C vs 1D). There was no history of birth trauma, malignancy, or vascular abnormality. Examination was otherwise normal other than a reversal of anisocoria with apraclonidine (before apraclonidine administration: Fig. 2A; after apraclonidine administration: Fig. 2B). These features indicate congenital Horner's syndrome, in which sympathetic innervation controlling Müller's muscle of the eyelid, iris dilator muscles, iris pigmentation, sweat glands, and vasomotor activity is disrupted. Ipsilateral anhidrosis implies a pre-superior cervical ganglionic lesion. Unlike acquired Horner's syndrome, congenital Horner's syndrome is generally a benign, idiopathic entity that does not require routine imaging beyond examination during infancy to exclude masses along the sympathetic chain. Figure 2Before apraclonidine administration (A) and after apraclonidine administration (B). View Large Image Figure Viewer Download Hi-res image