Warning: file_get_contents(https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&id=26933277
&cmd=llinks): Failed to open stream: HTTP request failed! HTTP/1.1 429 Too Many Requests
in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 215
Warning: imagejpeg(C:\Inetpub\vhosts\kidney.de\httpdocs\phplern\26933277
.jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117 Indian+J+Plast+Surg
2015 ; 48
(3
): 236-48
Nephropedia Template TP
gab.com Text
Twit Text FOAVip
Twit Text #
English Wikipedia
Fibrous dysplasia and cherubism
#MMPMID26933277
Bhattacharya S
; Mishra RK
Indian J Plast Surg
2015[Sep]; 48
(3
): 236-48
PMID26933277
show ga
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 in FD is post-natal mutation of GNAS1 gene, which
leads to the proliferation and activation of undifferentiated mesenchymal cells
arresting the bone development in woven phase and ultimately converting them into
fibro-osseous cystic tissue. Cherubism is a hereditary form of fibrous dysplasia
in which the causative factor is transmission of autosomal dominant SH3BP2 gene
mutation. The disease may present in two distinct forms, a less severe and
limited monostotic form, and a more aggressive and more widespread polyostotic
form. Polyostotic form may be associated with various endocrine abnormalities,
which require active management apart from the management of FD. Management of FD
is not free from controversies. While total surgical excision of the involved
area and reconstruction using newer micro-vascular technique is the only
definitive treatment available from the curative point of view, but this can be
only offered to monostotic and very few polyostotic lesions. In polyostotic
varieties on many occasions these radical surgeries are very deforming in these
slow growing lesions and so their indication is highly debated. The treatment of
cranio-facial fibrous dysplasia should be highly individualized, depending on the
fact that the clinical behavior of lesion is variable at various ages and in
individual patients. A more conservative approach in the form of aesthetic
recontouring of deformed bone, orthodontic occlusal correction, and watchful
expectancy may be the more accepted form of treatment in young patients. Newer
generation real-time imaging guidance during recontouring surgery adds to
accuracy and safety of these procedures. Regular clinical and radiological follow
up is required to watch for quiescence, regression or reactivation of the disease
process. Patients must be warned and watched for any sign of nerve compression,
especially visual impairment due to optic nerve compression. Rather than going
for prophylactic optic canal decompression (which does more harm than good),
optic nerve decompression should be done in symptomatic patients only, and
preferably be done via minimal invasive endoscopic neuro-surgical approach than
the conventional more morbid open craniotomy approach. There is growing research
and possibilities that newer generation bisphosphonate medication may change the
management scenario, as these medications show encouraging response in not only
reducing the osteoclastic activity, but simultaneously also stimulating the
osteoblastic and osteocytic activities. The explosion of genetic research and
stem cell therapy may lead to better understanding and subsequently better
treatment of FD in future.
free
free
free
