Clinical and Radiographic Features of Parry Romberg Syndrome

Abstract

Parry-Romberg syndrome or progressive hemifacial atrophy is a craniofacial disorder characterized by slow and progressive atrophy, generally unilateral, of facial tissues including muscles, bones and skin. The coup de sabre is a clear line of demarcation seen between the normal and abnormal structures. The severity of the facial deformity is dependent on the age of onset of the disease. Cosmetic management is the only available treatment and has to be delayed until facial growth is completed. The present case report deals with a 43-year-old woman with progressive hemifacial atrophy which started from the age of 10 months. Despite almost complete involvement of the right paramedian area and the early age of onset, she had neither eye changes nor any dental malformations.

Parry Romberg Syndrome: A Rare Entity With Possible Association With Periodontitis

“…[11] The term "progressive hemifacial atrophy" was coined by Eulenberg in 1871 to describe a condition characterized by unilateral atrophy of facial structures. [3] this desorder can affect skin-soft tissue, muscles and bones, which were present in our case. [12] More strict definitions of the syndrome include contralateral jacksonian epilepsy, trigeminal neuralgia, and changes in the eyes and hair.…”

“…Isolated cases have been observed with positive serology for Lyme disease. [3,5] The clinical features are unilateral atrophy of the skin, subcutaneous fat and in some cases muscle and bone, resulting in facial asymmetry. Skin is tensed or fibrosed with loss or gain of pigmentation can be observed.…”

“…Parry Romberg syndrome is a degenerative condition characterized by a slow progressive unilateral atrophy of facial tissues including subcutaneous fat, muscles, bone and skin. [1][2][3] This syndrome seems to have higher incidence on Women. [4] It affects individuals who are morphologically normal at birth, Onset is usually in the first or second decade of life.…”

“…More than an esthetic illness, it brings functinnal and psychological problems like trigeminal neuralgia, migraine, seizures, ocular problem and epilepsy. [3,4] The incidence and cause of this alteration Is unknown. Some of the proposed etiologies include heredity, auto-immune disorders, trauma, hypo-or hyperactivity of the sympathetic nervous system, disorders of the trigeminal nerve and infectious diseases and cerebral disturbance on fat metabolism heredity are believed to be associated to the pathogenesis of the disease.…”

Bone Resorption of the Jaws and Occlusal Disturbances in Parry-Romberg Syndrome

“…The prevalence is at least 1/700 000. The incidence is higher in women than men, in a ratio of approximately 3:2 [1], [2]. The disease initiates within the first and second decade of life.…”