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 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. This can be result of a trophic malformation of cerebral sympathetic nervous system. Possible factors that are involved in the pathogenesis are trauma, viral infections, heredity, endocrine disturbances, and autoimmunity, among others. Characteristically, atrophy progresses slowly for several years and, soon after, it become stable. The purpose of this work is, through the presentation of a clinical case, to accomplish a literature review concerning general characteristics, etiology, pathophysiology, differential diagnosis, and treatment of progressive hemifacial atrophy.
Congenital hemifacial hyperplasia (CHH) is a rare congenital malformation characterized by marked unilateral overdevelopment of hard and soft tissues of the face. Asymmetry in CHH is usually evident at birth and accentuated with age, especially at puberty. The affected side grows at a rate proportional to the nonaffected side so that the disproportion is maintained thr oughout the life. Multisystem involvement has resulted in etiological heterogeneity including heredity, chromosomal abnormalities, atypical forms of twinning, altered intrauterine environment, and endocrine dysfunctions; however, no single theory explains the etiology adequately. Deformities of all tissues of face, including teeth and their related tissues in the jaw, are key findings for correct diagnosis of CHH. Here an attempt has been made to present a case of CHH with its archetypal features and to supplement existing clinical knowledge.
Ewing's sarcoma is a malignant, small, round cell neoplasm that normally affects the long bones of the limbs or the pelvis. It is a relatively frequent malignant bone tumor in children. The occurrence of the primary tumor in the facial region is approximately 2%, with most of the cases affecting the mandible. Primary zygoma involvement is rare and as per the available literature only three cases have been reported.A case of Ewing's sarcoma that originated in the zygoma of a 15-year-old girl in reported. Bearing in mind its neuroectodermal origin, immunohistochemical studies are essential to distinguish Ewing's sarcoma from other small round cell tumors.
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