Balan Preethi, Gogineni Subhas Babu, Shetty Shishir Ram, D'souza Deepa
Department of Oral Medicine and Radiology, AB Shetty Memorial Institute of Dental Sciences, Nitte University, Mangalore, India.
Imaging Sci Dent. 2011 Dec;41(4):183-7. doi: 10.5624/isd.2011.41.4.183. Epub 2011 Dec 19.
Progressive hemifacial atrophy, also known as Parry-Romberg syndrome, is an uncommon degenerative condition which is poorly defined. It is characterized by a slow and progressive atrophy affecting one side of the face. The onset usually occurs during the first two decades of life. Characteristically, the atrophy progresses slowly for several years, and then it becomes stable. Ophthalmic involvement is common, with progressive enophthalmos which is a frequent finding. Cutaneous pigmentation is common in such conditions, however its extension to the conjunctiva is rarely reported. We report a case of Parry Romberg syndrome with characteristic clinical and radiographic presentation accompanied with rare ocular findings. The clinical features, radiological findings, and differential diagnoses to be considered, and the available treatment options are discussed in this report.
进行性半侧面部萎缩,又称帕里-罗姆伯格综合征,是一种定义不明确的罕见退行性疾病。其特征为一侧面部缓慢进行性萎缩。发病通常发生在生命的前二十年。典型的情况是,萎缩会缓慢进展数年,然后趋于稳定。眼部受累很常见,进行性眼球内陷是常见表现。这种情况下皮肤色素沉着很常见,但累及结膜的情况鲜有报道。我们报告一例具有典型临床和影像学表现并伴有罕见眼部表现的帕里-罗姆伯格综合征病例。本报告讨论了其临床特征、影像学表现、需考虑的鉴别诊断以及现有的治疗选择。