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CASE REPORT
Year : 2018  |  Volume : 8  |  Issue : 1  |  Page : 44-47

Orofacial manifestations of Pyle's metaphyseal dysplasia: A case report and review of literature


Department of Dentistry, N. R. S. Medical College and Hospital, Kolkata, West Bengal, India

Correspondence Address:
Dr. Tathagata Bhattacharjee
Kamalpur, Chakdaha, Nadia - 741 222, West Bengal
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijmd.ijmd_64_17

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Pyle's disease, commonly known as metaphyseal dysplasia, is an extremely rare genetic disorder with autosomal recessive mode of transmission. A case of Pyle's metaphyseal dysplasia in a 9 year old boy presented with a complaint of retained milk teeth and discharging pus from oral cavity with genu vulgum clinically and abnormal broad metaphyses of tubular bone radiologically. Cone-beam computerized tomography of maxilla and mandible showed decayed teeth, multiple retained deciduous teeth, and unerupted permanent teeth. We also found bilaterally permanent tooth with dwarf roots and large pulp chamber (taurodontism) as well as discontinuation of labial cortex of mandible.


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