Progressive hemifacial atrophy

Abstract

Progressive hemifacial atrophy, also known as Parry-Romberg Syndrome, is an uncommon degenerative and poorly understood condition. It is characterized by a slow and progressive but self-limited atrophy affecting one side of the face. The incidence and the cause of this alteration are unknown. A cerebral disturbance of fat metabolism has been proposed as a primary cause. Possible factors that are involved in the pathogenesis include trauma, viral infections, heredity, endocrine disturbances and auto-immunity. The most common complications that appear in association to this disorder are: trigeminal neuralgia, facial paresthesia, severe headache and epilepsy. Characteristically, the atrophy progresses slowly for several years and, it becomes stable. The objective of this work is, through the presentation of a clinical case, to accomplish a literature review concerning general characteristics, etiology, physiopathology and treatment of progressive hemifacial atrophy.

Keywords: Parry-Romberg Syndrome; Progressive hemifacial atrophy; Romberg's disease.

Figure 1

Extra oral photograph showing right side facial atrophy

Figure 2

Intra oral photograph showing edentulous 1^st quadrant

Figure 3

Orthopantomogram showing antegonial notch on right side

Figure 4

Coronal section of CT showing absence of masseter muscle on right side

Figure 5

3D reconstruction CT showing hypoplastic right mandible and condyle

Figure 6

MRI showing absence of parotid and submandibular salivary glands