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.

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Introduction:Odontogenic tumors (OTs) are a group of heterogeneous lesions derived from epithelial or ectomesenchymal tissues or both, which are part of the tooth-forming apparatus. They range from hamartomatous or nonneoplastic tissue proliferations to malignant neoplasms with metastatic capacity. OTs are comparatively rare, comprising about 4.79% of all oral and maxillofacial biopsy specimens diagnosed. Several retrospective studies carried out in Africa, Asia, Europe and America, show that differences exist in the relative frequency of the various histologic types. Very few studies are reported among Asians, especially from the Indian subcontinent. Hence, the present study is designed to determine the frequency of the OTs and compare them with reports of various other part of the world.Study Design:A retrospective study was carried out with the ethical clearance and permission from the authority. The histopathology records from the Department of Oral Pathology and Microbiology, within the period from January 1992 to June 2012 were obtained. A total number of OTs were analyzed for age, gender, site of the tumor and histopathological type. The odontogenic keratocyst now considered as kerato cysticodontogenic tumor (KCOT) was also included in the present study.ResultsTotally, 2652 tissue specimens were received for histopathologic examination out of which 127 were OTs. All these reported cases were benign except two cases of malignancy. Among these male: female ratio of 1.04:1 with an overall mandible: maxilla ratio of 1.01:1. The most common benign odontogenic tumor was KCOT (44.9%). Ameloblastomas were the second most common benign tumors (35.43%), followed by odontome (7.08%) and adenomatoid odontogenic tumor (4.72%). Age distribution showed a peak occurrence of the odontogenic tumor in the fourth decade (31.49%).Conclusion:OTs are rare lesions in the studied population and are represented mainly by the KCOT, ameloblastoma and odontoma. Data from the reviewed cases have shown a possible geographic variation of OTs. With the introduction of the KCOT in the 2005 WHO classification, this neoplasm is now one of the most prevalent OT types.

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.

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