Karim A, Laghmari M, Ibrahimy W, Essakali H N, Mohcine Z
Service d'Ophtalmologie A, Hôpital des Spécialités, Rabat, Maroc.
J Fr Ophtalmol. 2005 Oct;28(8):866-70. doi: 10.1016/s0181-5512(05)81008-3.
Progressive hemifacial atrophy (Parry-Romberg syndrome) is a rare entity characterized by severe hemifacial atrophy affecting subcutaneous tissue. Its clinical manifestations are mainly neurological and ocular. The most common ocular finding is enophthalmos with eyelid modifications (ptosis, retraction, atrophy). Neuroretinitis remains a rare symptom in this affection. We report here a new case.
A 22-year-old woman with progressive hemifacial atrophy (Romberg syndrome) is reported. The atrophy had begun 10 years before. At time of presentation, she was suffering from blurred vision in her left eye. She presented with mild enophthalmos associated with eyelid atrophy and loss of cilia. Biomicroscopic examination showed bilateral vitreitis and a typical spectrum of neuroretinitis in her left eye. There was no heterochromia. General examination revealed atrophy of the right part of her body. Etiological investigations showed the presence of antinuclear antibodies and a positive Rose-Waaler latex test without an inflammatory syndrome. Corticotherapy was proposed (1 mg/kg/day) with good progression.
The authors report a new case of progressive hemifacial atrophy (Parry-Romberg syndrome). They emphasize the rarity of this disease, its etiology, which remains controversial, the diversity of its ophthalmologic manifestations, and its relations with scleroderma and autonomic nervous system disorders are discussed. Acute neuroretinitis remains rare and its pathogeny is unknown.
进行性半侧面部萎缩（帕里 - 罗默综合征）是一种罕见的疾病，其特征为严重的半侧面部萎缩，累及皮下组织。其临床表现主要为神经和眼部症状。最常见的眼部表现是眼球内陷伴眼睑改变（上睑下垂、退缩、萎缩）。神经视网膜炎在该疾病中仍然是一种罕见症状。我们在此报告一例新病例。
报告一名22岁患有进行性半侧面部萎缩（罗默综合征）的女性。萎缩始于10年前。就诊时，她左眼视力模糊。表现为轻度眼球内陷，伴有眼睑萎缩和睫毛缺失。生物显微镜检查显示双眼玻璃体炎，左眼有典型的神经视网膜炎表现。无虹膜异色。全身检查发现她身体右侧萎缩。病因学检查显示存在抗核抗体，罗斯 - 瓦勒乳胶试验阳性，但无炎症综合征。建议采用皮质激素治疗（1毫克/千克/天），病情进展良好。
作者报告了一例进行性半侧面部萎缩（帕里 - 罗默综合征）的新病例。他们强调了这种疾病的罕见性、其病因（仍存在争议）、眼科表现的多样性，并讨论了其与硬皮病和自主神经系统疾病的关系。急性神经视网膜炎仍然罕见，其发病机制尚不清楚。
