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ABSTRACT Regional odontodysplasia is a rare developmental anomaly involving dental tissues. The precise etiology is not yet known. The affected teeth are usually small with atypical morphology with yellow or brown discoloration. One hundred and seventy six cases of regional odontodysplasia have been published to-date. Analysis of the published cases shows that the age at the time of diagnosis ranges from 1-23 years. Maxilla is more commonly affected with predilection for anterior teeth. Radiographic images of the involved teeth show a ghost-like appearance. Histologically, almost all dental tissues are altered. Enamel and dentin appear as hypoplastic and hypocalcified and the pulp is larger than normal. Treatment of regional odontodysplasia needs to be individualized and requires a multidisciplinary approach. Although regional odontodysplasia is known for more than six decades, the literature is almost limited to case reports only.
Further epidemiological, experimental and genetic studies are needed.
Key Words: Odontodysplasia, Regional, overview.
INTRODUCTION Regional odontodysplasia (RO) is defined as a localized developmental anomaly involving both deciduous various terms, such as arrested tooth development"4, shell teeth"5, unilateral dental malformation"6, and ghost teeth"7. Zegarelli et al. (1963)10 coined the most widely used term "odontodysplasia". Later in 1970, Pindborg8 added the term, "regional" emphasizing the localized nature of this disease. It is usually found isolated, however, it can be associated with some developmental anomalies such as ectodermal dysplasia, hypophosphatasia, unilateral facial hypoplasia, neurofibromatosis, and vascular and epidermal nevi.9 The purpose of the present article was to review the English-language literature and to provide an overview of various aspects of RO. The etiology, pathogenesis, epidemiology, clinical presentation, radiographic, macroscopic, ultrastructural, light microscopic, immunohistochemical features, and treatment of RO are discussed.
ETIOLOGY and PATHOGENESIS The exact etiology of odontodysplasia is not clear, although several theories have been suggested. Some authors suggest a correlation with trauma and infection;6,10 however, this correlation was not proved, since the correlation was based on patient history alone. The distribution of affected teeth in all four quadrants makes some authors suggest that somatic mutation may be the causative factor.11 Witkop also suggested that a somatic mutation in early development could cause alterations in the ontogenesis.12 Nagai et al.13 had transplanted tooth germs in mice and found alterations in the tooth formation after inducing polyoma virus. Thus, viral...