PMC5426156_01
(a) Clinical photograph showing stout appearance with bowing of upper and lower limbs, (b) clinical photograph showing bony prominence (red arrow) in the mandible extending from 43 to 47
CCD-8-175-g001.jpg
PMC5426156_01_CCD-8-175-g001.jpg
F1
On clinical examination, patient had short stout appearance with bowing of the upper and lower limbs [Figure 1a]. Intraorally, bony prominence of the mandible extending from 43 to mesial aspect of 47 measuring about 2 cm x 1 cm was noticed [Figure 1b].
Orthopantomogram showing mixed radiopaque and radiolucency appearance and deformed condylar region with impacted 33 and missed 45 with over retained 85 and accentuated pulp space in relation to 17, 25, 26, 27, 28, 36, 37, 46, 47
CCD-8-175-g002.jpg
PMC5426156_01_CCD-8-175-g002.jpg
F2
Orthopantomogram revealed mixed radiopaque and radiolucent lesion with ground glass appearance and deformed left condylar region with impacted 33 and missing 45 with over retained 85 and accentuated pulp space in relation to 17, 25, 26, 27, 28, 36, 37, 46, 47 [Figure 2].
(a) Posterior-anterior view of the skull showing bony expansion and lytic areas in the skull vault and mandible, (b) X-ray of the lumbar spine and pelvis showing scoliosis with expansion of pelvic bone with osteolytic and sclerotic areas
CCD-8-175-g003.jpg
PMC5426156_01_CCD-8-175-g003.jpg
F3
Skull radiograph revealed bone expansion and lytic areas in the skull vault and mandible [Figure 3a]. Radiograph of the lumbar spine including pelvis showed scoliosis, expansion of bone in pelvis region, and osteolytic and sclerotic areas [Figure 3b].
(a and b) Photomicrograph showing interconnecting bony trabeculae with irregular cement lines (black arrow) and loose fibrovascular tissue within the intertrabecular space
CCD-8-175-g004.jpg
PMC5426156_01_CCD-8-175-g004.jpg
F4
Histopathological features showed irregular interconnecting trabeculae with prominent basophilic lines arranged irregularly, which are lined by active osteoblasts and few osteoclasts [Figure 4a]. Resorbed bone and marrow spaces were seen to be replaced by fibrovascular connective tissue [Figure 4b].