Scheutzel P
Department of Restorative Dentistry, Dental Hospital, Westfälische, Wilhelms-Universität, Münster, Germany.
Dentomaxillofac Radiol. 1991 Aug;20(3):175-8. doi: 10.1259/dmfr.20.3.1808004.
The clinical and radiographic changes in a case of oculodentodigital syndrome are presented. The characteristic features of this rare developmental disorder, microphthalmus with microcornea and iris anomalies, hypertelorism, thin nose with hypoplastic alae and anteverted nostrils, syndactyly with camptodactyly and clinodactyly of the fourth and fifth fingers associated with bony anomalies of the middle phalanges of the fifth fingers and toes, were all present. In addition, histological examination of a lateral incisor showed the enamel dysplasia to be due to enamel hypoplasia; the dentine also showed marked hypocalcification. There were widespread pulp denticles and hypercementosis throughout the dentition. Both the patient and his mother had coronoid hypoplasia.
本文介绍了一例眼牙指综合征的临床和影像学变化。这种罕见的发育障碍的特征包括小眼合并小角膜和虹膜异常、眼距过宽、鼻瘦伴鼻翼发育不全和鼻孔前倾、并指(趾)合并第四和第五指屈曲指和斜指,以及第五指和趾中节指(趾)骨的骨异常。此外,一颗侧切牙的组织学检查显示釉质发育不全是由于釉质发育不良所致;牙本质也显示出明显的钙化不足。整个牙列中普遍存在牙髓牙本质小体和牙骨质增生。患者及其母亲均有冠状突发育不全。