Clinical Investigation On A Case Of Hereditary Dentine Dysplasia Of Deciduous And Permanent Teeth

Dentin dysplasia type Ⅰ (DD-Ⅰ) is a very rare autosomal dominant disease which exhibits dentine abnormal development. Its clinical manifestations are the normal shape, size and color of the clinical crown, great degree of tooth mobility, easy occurrence of late sprouting and early tooth loss phenomena. X-ray showed short root, obliteration of pulp chambers in the primary teeth, the crescent shaped chambers paralleling to the cementoenamel junction in the permanent teeth, and the visible periapical image transmission in non-carious tooth. Histopathological examinations showed that the pulp chambers and root canals disappeared completely and dentinal tubules disorderly arranged, in which were filled with large amounts of spherical calcifications to form the typical structure which the water flowing around pebbles, and visible osteoid dentin. Because the features of the disease are short root and great degree of tooth mobility, in previous reports the therapeutic approaches were more taken conservative symptomatic treatment, the reports on improving the patient’s oral function by orthodontic treatment are very few.This paper reports a case of dentin dysplasia of primary and permanent teeth found in our clinical. The diagnosis was confirmed through the clinical, imaging and histopathological examinations, the changes in permanent teeth eruption were observed by further regular follow-up, the occurred problems related to the oral cavity were treated timely, and the discussion was performed with combined the related literature. All of these supplemented data for further studies on this disease.