Osteogenesis imperfecta type I with unusual dental abnormalities

Abstract

A large kindred with dominantly inherited osteogenesis imperfecta was evaluated. Affected individuals had bone fractures, blue sclerae, and hearing loss. In addition, all had dental abnormalities distinct from those previously described in other families with this syndrome. Deciduous teeth were normal in color or blue-grey. On radiographs of an early developing deciduous dentition, pulps were larger than normal. In patients with mixed dentitions, pulp chambers of deciduous teeth were partially obliterated. Increased constriction at the junctions of the crowns and roots was found in some deciduous teeth. One patient had large pulp stones in the pulp chambers of all maxillary deciduous molars. Permanent teeth were normal in color but had oval pulp chambers with apical extensions into the coronal portions of the roots, large coronal pulp stones, narrow root canals, and thin roots. Individuals in this family who did not have osteogenesis imperfecta had normal teeth. In addition, a well circumscribed radiolucency without a sclerotic periphery, involving the apices of all permanent mandibular incisors, was found in the anterior mandible in one patient. These findings support the hypothesis that this family has yet another type I osteogenesis imperfecta "syndrome".

Oral health in prevalent types of Ehlers–Danlos syndromes

“…The present study demonstrates a clear interrelation between deficiency of specific collagen types and clinical manifestations in the different orofacial structures (Table 5). Previous reports on oral manifestations of collagen I deficiency in subjects affected with osteogenesis imperfecta (OI), mainly documented on dentin defects (dentinogenesis imperfecta) in the deciduous and permanent dentition (50–56). A high incidence of class III dental malocclusion, anterior and posterior cross‐bites, and ectopic tooth eruption were also reported in OI, but these characteristics most likely seem related to abnormal growth and development of the craniofacial bones as a result of abnormal collagen I biosynthesis (54).…”

Pulp stones: a review

“…1992), osteogenesis imperfecta type I (Lukinmaa et al. 1987, Levin et al. 1988) and otodental syndrome (Sedano et al.…”

A rare presentation of a simple bone cyst

“…Curettage of the bone walls is the most widely accepted approach to the management of SBCs of the jaws, the goal being to promote the formation of a blood clot and subsequent bone repair. [ 11 16 ] In addition, application of Gelfoam, grafting of allogenic bone with platelet-rich plasma and intralesional injections of a mixture of blood, porous hydroxyapatite, and bone fragments have been reported to produce good results. [ 17 18 19 ]…”