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Parry-Romberg syndrome or progressive hemifacial atrophy is vary rare, uncommon, degenerative, poorly understood condition characterized by a slow and progressive atrophy affecting one side of the face. The incidence and the causes of this alteration are unknown. Possible factors that are involved in the pathogenesis include disturbance of fat metabolism, trauma, viral infections, heredity, endocrine disturbances and auto-immunity. The most common complications are: trigeminal neuritis, facial paresthesia, severe headache and epilepsy. Characteristically, the atrophy progresses slowly for several years and become stable after certain time period. After stabilization of the disease multi specialty approach including physician, orthodontic treatment and reconstructive surgery with autogenous fat graft can be performed to correct the deformity. The objective of this article is to accomplish a literature review concerning general characteristics, etiology, physiopathology, differential diagnosis and treatment of progressive hemifacial atrophy.

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