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Case Reports
. 2007 Jan;28(1):132-4.

Dental and facial bone abnormalities in pyknodysostosis: CT findings

K W Fleming  1 , G Barest, O Sakai
Affiliations
Case Reports

Dental and facial bone abnormalities in pyknodysostosis: CT findings

K W Fleming et al. AJNR Am J Neuroradiol. 2007 Jan.

Abstract

Pyknodysostosis is an autosomal-recessive disorder of osteoclast dysfunction causing osteosclerosis, with associated maxillofacial anomalies. Multidetector CT with multiplanar and 3D reconstruction illustrated the pathologic findings in this case. Abnormalities included multiple retained deciduous teeth, unerupted teeth with associated follicles, an irregularly expanded alveolus and body of the mandible, and an obtuse mandibular angle. Volume-rendered imaging better delineated the irregular dentition, with crowding and retention of deciduous teeth.

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Figures

Fig 1.
Fig 1.
A 20-year-old man with pyknodysostosis. A, Panorex radiograph reveals multiple disorganized crowded deciduous and permanent teeth within the expanded alveolar processes. Many of the teeth are unerupted or only partially erupted. Evaluation of follicles is limited. B, Scout topogram demonstrates Wormian bones (arrows) within the lambdoid suture. Obtuse gonial angle of the mandible and dental abnormalities are also visualized. C and D, Axial CT images at the level of the mandible (C) and maxilla (D) reveal multiple unerupted crowded deciduous and permanent teeth within the expanded alveolar processes. Lucencies around several of the teeth have well-defined margins, very likely representing follicles (arrows). However, bandlike zones of demineralization suggest an active inflammatory/infectious process (arrowheads). E and F, Coronal (E) and sagittal (F) reformatted CT images demonstrate the crowded teeth in the maxilla and mandible with associated lucencies (arrowheads) surrounding the unerupted teeth. Note the hypoplastic maxillary sinuses bilaterally (arrows). G, Surface-rendered 3D image shows irregularly expanded alveolar processes and mandibular body and a flattened obtuse mandibular angle. The technique limits evaluation for irregular dentition with crowding and retention of deciduous teeth. H, Volume-rendered 3D image better delineates irregular dentition with crowding and retention of deciduous teeth.
See this image and copyright information in PMC

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