PDF | Familial gigantiform cementoma is an exceedingly rare but distinct subtype of cemento-osseous-fibrous lesion. Undocumented. Very few cases of gigantiform cementoma have been reported, and those associated with a positive family history are especially rare. Confusion exists about the. Familial gigantiform cementoma is a rare benign fibrocemento-osseous lesion of the jaws that can cause severe facial deformity. It has an.
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A The adolescent patient with a huge mass extending along the mandible body. Familial gigantiform cementoma with brittle bone disease, pathologic fractures, and osteosarcoma: Gigantifoem Lateral view of FGC. OMIM is intended for use primarily by physicians and other professionals concerned with genetic disorders, by genetics researchers, and by advanced students in science and medicine.
Report of a gigabtiform documented with computed tomography and 3D imaging. In concurrence with the boy and his parent’s wishes, we decided to perform a bilateral segmental mandibulectomy and to tentatively reconstruct the corresponding defect with vascularized iliac bone flaps. Open in a separate window.
EllisDerek G. However, owing to such diversion of calcium supply, bone fragility was manifested in the other anatomic regions, especially in lower extremities. Surgery with recontouring and shaving intent was initially attempted in a local institution about 7 months ago, but in vain.
Views Read Edit View history. E Technetium scintigraphy showed increased tracer uptaker in the chin area. Fibro-osseous lesions of the head and neck.
Familial gigantiform cementoma: classification and presentation of a large pedigree.
Among all the reports available, Rossbach et al 3 was the first to postulate the correlation of a brittle bone disorder with FGC. The cause of this tumor is currently unknown. Loss of lamina dura, decreased skull bone density, and sporadic bony gigantifom all served as convincing evidence of the serious osseous fragility.
Lam Dento maxillo facial radiology Unfortunately, it is not free to produce. Published online Mar 7. Besides, the informed consent was obtained from these patients.
To keep both practitioners and patients informed of the overall bony changes and corresponding risks of fractures, we contended that DEA should be routinely tested since initial clinic visit. This article has been cited by other articles in PMC. We are determined to keep this website freely accessible.
Despite the paucity of information regarding FGC, DEA, as we believe, carries diagnostic and therapeutic implications, along with other radiographic examinations. All these fractures, which occurred in diaphyseal locations, were categorized to a minor-trauma or spontaneous causes owing to poor evidence of outside forces.
Gigantiform cementoma is characterized by diffuse radioopaque masses scattered throughout the jaws.
Familial gigantiform cementoma with Ehlers – Danlos syndrome: A report of 2 cases
Large vascular malformation in a child presenting with vascular steal phenomenon managed with pial synangiosis. Topics Discussed in This Paper. Cementoma Search for additional papers on this topic. Besides, his pelvis was also considered to be susceptible to fractures because both cortical and trabecular bones were undergoing a progeric or osteopenic conversion signifying an unexpected calcium loss.
Gigantiform cementoma in a child. This is an open access article distributed under the Creative Commons Attribution License 4.
From This Paper Figures, tables, and topics from this paper. References Publications referenced by this paper. His chief concern was dysmasesis and dysphagia caused by a protuberant mandible since 10 years old.