Introduction: 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.
Case report: 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.
Discussion: 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.