Fibrous dysplasia and cherubism

• DOI: 10.4103/0970-0358.173101
• Publication Date: 2019-09-03T19:56:41Z
• Authors: Surajit Bhattacharya; R K Mishra
• Keywords: Polyostotic fibrous dysplasia; Cherubism

ABSTRACT Fibrous dysplasia (FD) is a non-malignant fibro-osseous bony lesion in which the involved bone/bones gradually get converted into expanding cystic and fibrous tissue. The underlying defect FD post-natal mutation of GNAS1 gene, leads to proliferation activation undifferentiated mesenchymal cells arresting bone development woven phase ultimately converting them Cherubism hereditary form causative factor transmission autosomal dominant SH3BP2 gene mutation. disease may present two distinct forms, less severe limited monostotic form, more aggressive widespread polyostotic form. Polyostotic be associated with various endocrine abnormalities, require active management apart from FD. Management not free controversies. While total surgical excision area reconstruction using newer micro-vascular technique only definitive treatment available curative point view, but this can offered very few lesions. In varieties on many occasions these radical surgeries are deforming slow growing lesions so their indication highly debated. cranio-facial should individualized, depending fact that clinical behavior variable at ages individual patients. A conservative approach aesthetic recontouring deformed bone, orthodontic occlusal correction, watchful expectancy accepted young Newer generation real-time imaging guidance during surgery adds accuracy safety procedures. Regular radiological follow up required watch for quiescence, regression or reactivation process. Patients must warned watched any sign nerve compression, especially visual impairment due optic compression. Rather than going prophylactic canal decompression (which does harm good), done symptomatic patients only, preferably via minimal invasive endoscopic neuro-surgical conventional morbid open craniotomy approach. There research possibilities bisphosphonate medication change scenario, as medications show encouraging response reducing osteoclastic activity, simultaneously also stimulating osteoblastic osteocytic activities. explosion genetic stem cell therapy lead better understanding subsequently future.