A Case of Extensive polyostotic fibrous dysplasia.
- Author:
Byeong Do LEE
1
;
Eui Hwan HWANG
;
Sang Rae LEE
Author Information
1. Department of Oral and Maxillofacial Radiology, School of Dentistry, Wonkwang University, Korea. eebydo@wonkwang.ac.kr
- Publication Type:Case Report
- Keywords:
Polyostotic;
extensive type;
bilateral;
shepard's crook deformity
- MeSH:
Adolescent;
Congenital Abnormalities;
Extremities;
Facial Bones;
Femur;
Fibrous Dysplasia, Polyostotic*;
Fractures, Spontaneous;
Glass;
Humans;
Humerus;
Mandible;
Ribs;
Sclerosis;
Skin Pigmentation;
Tibia;
Transplants
- From:Korean Journal of Oral and Maxillofacial Radiology
2000;30(2):117-122
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Fibrous dysplasia is a benign disorder of bone consisting of intramedullary proliferation of fibrous tissue and irregularly distributed, poorly developed bone. The disease manifests itself in the monostotic form in which only one bone is involved and the polyostotic form in which multiple bones at different sites are affected. We reported a extensive case of polyostotic fibrous dysplasia with involvement of craniofacial bones, mandible, ribs, extremities. A 18-year-old man showed remarkable right facial swelling who had been treated on right femur 3 years ago with a bone graft for pathologic fracture and he recognized facial swelling 5 years ago. Extraoral radiograms and computed tomogram showed diffuse sclerosis with a ground glass appearance of the most calvarial bones, facial bones. The right mandibular lesion showed very expansile lesion with mottled appearance. Bone scans showed mutifocal increased uptakes in craniofacial bones, right mandible, bilaterally in ribs, humerus, femur, tibia and characteristic varus deformity of right femur (shepherd's crook defomity). This case showed exceptionally bilateral, extensive nature of bone lesion and didn't show any features of skin pigmentation and endocrine disturbances.
