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AJNR Am J Neuroradiol. 2007 Jan;28(1):132-4.

Dental and facial bone abnormalities in pyknodysostosis: CT findings.

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Department of Radiology, Boston University Medical Center, Boston, MA, USA.


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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