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Odontodysplasia. Report of three cases with vascular nevi overlying the adjacent skin of the face.

Three cases of odontodysplasia are described, and the literature is reviewed with reference to proposed etiologic mechanisms underlying the pathogenesis of the disorder. The distribution of affected teeth in all four quadrants of one patient is offered as evidence against a somatic mutation as an etiologic mechanism. As infants all three patients had vascular nevi in the facial skin overlying the area where defective teeth developed. Three cases from the literature involved similar vascular lesions. Hypoplastic teeth radiographically similar to odontodysplastic teeth occur in the linear sebaceous nevus syndrome. These findings strongly suggest that local vascular defects are involved in the pathogenesis of odontodysplasia.

Child

Regional odontodysplasia.

Regional odontodysplasia was diagnosed in a girl aged 6 with lesions of a number of deciduous and permanent teeth in the right lower and upper quadrants. The literature comprises reports on some 50 cases of regional odontodysplasia, including only one patient who also showed involvement of both the maxilla and the mandible.

Child

Odontodysplasia.

Results of a comprehensive dental examination, including a histologic evaluation, of a patient showing unilateral odontodysplasia revealed no hint as to the origin of the abnormality. A complete medical examination also resulted in negative findings, with the excetion of congenital bilateral talipes equinovarus. The genetic history was negative, and results of chromosomal studies of the child and her mother revealed no abnormalities. Neither had an anatomic or a functional abnormality, with the exception of the odontodysplasia in the child. Results of laboratory studies including complete blood counts, urinalyses, ferric chloride tests of the urine, and electrolyte tests also were negative. The special considerations given in this case in an attempt to establish a possible causation of the abnormality were inconclusive. We think, however, that the management of the patient was successful in regard to the prosthetic appliance, and every attempt will be made to follow up the patient to ensure a good prognosis of the remaining developing dentition.

Denture, Partial, Removable

Odontodysplasia. Report of two cases and review of the literature.

Odontodysplasia is a rare developmental anomaly affecting the tooth structures in both deciduous and permanent dentitions. The enamel is thin and uneven in thickness, and the detinal tissue surrounds very large pulp chambers. Denticles are present in the pulp organ. The maxilla is involved twice as frequently as the mandible. Most of the affected teeth are in the anterior segments; however, all other teeth can be affected. The cause is unknown. Because of the tendency of the affected teeth to develop abscesses, the most common treatment is extraction. Two additional cases are reported, and the literature is reviewed.

Abscess

Abnormal tooth tissue in human odontodysplasia.

The affected teeth in this case of odontodysplasia exhibited abnormal hypoplastic enamel, abnormal dentin containing extensive interglobular regions and completely lacking a peritubular matrix, and an abnormal irregular tissue consisting of highly calcified and partially fused granules located within the dentin of the tooth tips. Electron micrographs of the irregular tissue showed that the granules consisted of crystallites densely and radically packed in a noncollagenous amorphous matrix. Granules were surrounded by a slightly calcified, irregularly arranged, collagenous matrix. The irregular tissue formed in the pulp of the tooth tip befofe and independently of the dentin. It was at least partially formed by pulpal calcification. Abnormal dentin matrix was formed by odontoblasts which were less differentiated than normal. Odontodysplastic teeth showed abnormal differentiation of odontoblasts and ameloblasts resulting in defective enamel and dentin and, in extreme cases, in extensive pulpal calcification.

Child

Ultrastructural, electron-probe, and microhardness studies of the controversial amorphous areas in the dentin of regional odontodysplasia.

The amorphous areas, which are gray in sections stained with hematoxylin and eosin and which are a prominent feature in the coronal dentin of the more severely affected cases of regional odontodysplasia, were studied ultrastructurally and with an electron probe and a Vickers microhardnesss tester. The ultrastructural findings confirmed previous histochemical evidence that the amorphous material consists of glycosaminoglycans and represents collagen-free dentinal matrix, that is, ground substance. Phosphoproteins may also present. The ultrastructural examination of nondecalcified material and electron-probe and microhardness tests supported previous microradiographic evidence that the amorphous areas are more heavily mineralized than normal dentin. The boundaries of the amorphous areas were found on ultrastructural examination to be distinct but very irregular.

Child

[A rare dental abnormality: "the phantom tooth" or "odontodysplasia". Apropos of a case].

"Phantom tooth" or "odontodysplasia" is one of the most unusual and rare of dental abnormalities, being characterised: Clinically, by the topography of the lesions, the involvement of both sets of teeth and abnormalities in the size and form of the teeth which remain unrupted or only partially evolved. Radiologically, by transparency of the teeth to X-rays. Histologically, by disorganisation of the dental structures and a highly characteristic appearance of the enamel and, in particular, dentine. On the basis of one case, the authors review in detail each aspect of these characteristics, paying special attention to histology and the information obtained using the sweep electron microscope.

Amelogenesis Imperfecta

Structural changes in odontodysplasia.

Report is made of a histologic and scanning electron microscopic (SEM) study of teeth affected by odontodysplasia. The enamel was found to be thinner than normal, and the entire surface showed numerous depressions. The prismatic orientation, diameter, and density were found to be normal. Although the mantle dentin remained normal, the rest of this tissue showed a tendency to globular formation. The orientation, density, size, and shape of the dentinal tubules appeared to be normal. The cementum, in general, was normal, but in some instances appeared to be scalloped or globular. The pulp consisted of normal tissue but was found to be larger than normal. The presence of denticles was an outstanding feature.

Dental Enamel

[Odontodysplasia].

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Child, Preschool

Focal odontodysgenesis of the maxillary second premolars in a child.

A few months before her sixth birthday, a Caucasian girl suffered progressively worse episodes of painful, nontender, erythematous swellings of the palate lingual to apparently healthy primary second molars. Neither of the developing maxillary second premolars was discerned clearly in radiographs, but, upon surgical exploration, incomplete crowns were found in both follicles. All the other premolars appeared to be developing normally. Histologic, histochemical, and chemical analyses showed that the enamel was hypocalcified, apparently as a result of hypomaturation; the dentin was essentially normal; and morphogenesis had ceased when the heights of contour of the crowns had not yet been reached. This pattern of developmental defects differs from those of well-characterized odontogenic disorders, such as regional odontodysplasia and amelogenesis imperfecta. We suggest that focal odontodysgenesis might be an appropriate term for it. The bilateral symmetry suggests a genetic component in the etiology.

Bicuspid

A classification of dysplastic forms of dentin.

Considerable effort has been made in the past to describe the morphologic changes in dentin which characterize various developmental disturbances of teeth. However, there appears to be no previous attempt to describe the spectrum of changes which may be exhibited by abnormal dentin. This paper presents a classification of dysplastic forms of dentin with definitions and illustrations of each abnormality. The classification was derived from reviewing the literature and from a study of 82 odontomas, examples of specific developmental disorders of dentin, regional odontodysplasia, and systemic conditions affecting dentin.

Dentin

Enamel matrix proteins in normal and abnormal amelogenesis.

Histochemical techniques have been used to study three stages of enamel maturation in normal (fetal calf, newborn human, adult human) and abnormal amelogenesis (odontodysplasia, fluorosis, a compound-complex odontome, and an invaginated odontome). The amount of Type I matrix secreted decreases as amelogenesis progresses. Pockets of Type II matrix may be left at the dentino-enamel junction after maturation of enamel to Type III. Fluorosed enamel contains pockets which may extend the width of the enamel. All stages of enamel maturation may be seen in the compound-complex odontoma.

Amelogenesis

Histopathologic findings in third molar opercula.

This review of 130 opercula revealed the following findings: 1. In nine cases, salivary gland tissue was present in variable amounts. 2. The oral epithelium was unremarkable, except for eleven cases of atypia, most likely benign atypia resulting from trauma and inflammation. 3. Odontogenic-type epithelium was found in sixty cases. None showed unusual proliferation, but two cases were of a clear-cell type. 4. The lining epithelium was variably present and was either simple squamous, cuboidal, columnar, or stratified squamous in type. 5. Fifty-four cases were found which showed either bone, dentin, osteoid, or focal odontodysplasia in the tissue, including one case that showed a structure identical to a periodontal membrane encircling it. 6. The lining epithelium on the ISO was often completely absent. This was true with almost no inflammation as well as with marked inflammation present. The soft-tissue overlying erupting third molar teeth contains a host of different tissues. Many of these have the propensity to undergo proliferation, neoplastic change, or cause postoperative complications and delay healing. Therefore, it is my opinion that all such tissue removed be subjected to histopathologic review. Furthermore, surgeons should be aware of the possible neoplastic potential and the detrimental healing effects of these various tissues if an operculum is used for primary closure.

Epithelium