遗传性牙本质发育缺陷是一组罕见的牙齿结构发育异常。自从Barrett 1883年首次报告这种病例以来,各国学者陆续报道了不少这类病例,近年来就发病机理和病变本质做了一些探讨。这种发育缺陷有几个突出的特点:(1)有明确的遗传因素,为常染色体显性遗传;(2)乳、恒牙皆受累;(3)外层牙本质正常;(4)牙本质小管不同程度地紊乱;(5)髓腔不同程度地被增生的牙本质闭塞。以往所用名词比较混乱,1973年shields把这类发育缺陷分成两大类,每类又分亚型: Hereditary development of dentin defects is a rare group of abnormal tooth structure. Since Barrett first reported this case in 1883, many scholars of this field have reported many cases of this kind. In recent years, some discussions have been made on the pathogenesis and the nature of the disease. There are several prominent features of this developmental defects: (1) a clear genetic factors, autosomal dominant; (2) milk, permanent teeth are involved; (3) the outer dentine normal; (4) teeth The nature of tubules to varying degrees of disruption; (5) the pulp cavity to varying degrees by the proliferation of dentin occlusion. The terms used in the past more confusing 1973 shields the developmental defects into two categories, each sub-sub-sub-sub-type: