Hemifacial Atrophy (Parry-Romberg Syndrome, #141300) With Papillitis, Retinal Alterations, and Restriction of Motility

The essential feature of the Parry-Romberg syndrome is atrophy of the subcutaneous tissues on one side of the face. It is a progressive, rather self-limiting disease whose pathogenesis is still unknown. Ocular manifestations include enophthalmos, eyelid atrophy, pupillary and iris abnormalities, fundus changes, and intraocular inflammations, as well as disturbances of ocular motility. Signs of inflammation in the initial stages of this case suggest that the clinical picture could be the result of a secondary atrophy, following an active inflammatory process. The progressive, acquired type of hemifacial atrophy was first described by Parry in 1825 and later by Henoch and Romberg in 1845, and it was suggested to be a form of localized scleroderma. The French expression for this is sclerodermie en coup de sabre. The disease usually begins in the first 2 decades of life and can proceed rapidly. Females are more often affected than males and most cases appear to be sporadic. Apart from ocular manifestations, bone and cartilage are involved if the disease starts in the period of growth.