American Journal of Neuroradiology 28:132-134, January 2007
© 2007 American Society of Neuroradiology
Case Report
HEAD & NECK
Dental and Facial Bone Abnormalities in Pyknodysostosis: CT Findings
a From the Department of Radiology, Boston University Medical Center, Boston, Mass
Please address correspondence to Keith W. Fleming, MD, New York Presbyterian Hospital/Weill Cornell Medical Center, 525 E. 68th St, New York, NY 10021; e-mail: keithwfleming{at}gmail.com
SUMMARY: 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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