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A Case of Parry-Romberg Syndrome in Neonate

Journal of the Korean Pediatric Society 1999;42(11):1589-1593.
Published online November 15, 1999.
A Case of Parry-Romberg Syndrome in Neonate
Chun Hyuk Chang1, Jin Hwa Jung1, Sung Min Cho,1, Dae Seop Choi2
1Departments of Pediatrics, College of Medicine, Dongguk University, Kyungju, Korea
2Departments of Radiology, College of Medicine, Dongguk University, Kyungju, Korea
신생아기에 시작된 Parry-Romberg 증후군 1례
장준혁1, 정진화1, 조성민1, 최대섭2
1동국대학교 의과대학 소아과학교실
2동국대학교 의과대학 방사선과학교실
Correspondence: 
Chun Hyuk Chang, Email: 1
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.
Key Words: Parry-Romberg syndrome, Neonate


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