A Case of Parry-Romberg Syndrome in Neonate

Chang, Chun Hyuk; Jung, Jin Hwa; Cho, Sung Min; Choi, Dae Seop

Abstract

Parry-Romberg syndrome(Progressive hemifacial atrophy), described in the last century by Parry(1825) and Romberg(1846), is a very rare disorder characterized by a slowly progressive and self-limited unilateral(rarely bilateral) atrophy of the faces affecting variably the skin, subcutaneous fat tissues, musculature, connective tissue, cartilage and bones. And this disorder is usually accompanied by contralateral Jacksonian epilepsy, trigerminal neuralgia, and changes in the eyes and hair. The onset is slow and progressive, starting at 5-15 years of age and lasting from 2-10 years, ending with the face being "burned out". There are a few cases of this disease which presented during the neonatal period. This disorder seems to affect females more than males, and its etiology and incidence has yet to be determined. Trauma, infection with a slow virus, sympathetic dysfunction, immunological abnormality and cranial vascular malformation are proposed causes. No typical or consistent neuropathologic findings occur. No specific treatment for the syndrome exists; however, various reconstructive surgical procedures can have in reasonably good cosmetic effects, as well as antiinflammatory or immunosuppressive treatment. We report a case of Parry-Romberg syndrome, which was presented at 1 month of age, and has progressd to contralateral hemiparesis.