CASE REPORT |
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Year : 2018 | Volume
: 8
| Issue : 1 | Page : 44-47 |
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Orofacial manifestations of Pyle's metaphyseal dysplasia: A case report and review of literature
Tathagata Bhattacharjee, Mrinmoy Kerketa, Debjit Roy, Santosh Shukla, Debarpita Roy
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
 Source of Support: None, Conflict of Interest: None  | Check |
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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