Fibrous Dysplasia: An Overview of Disease Process, Indications for Surgical Management, and a Case Report
Identifieur interne :
001C80 ( Pmc/Corpus );
précédent :
001C79;
suivant :
001C81
Fibrous Dysplasia: An Overview of Disease Process, Indications for Surgical Management, and a Case Report
Auteurs : Oluwaseun A. Adetayo ;
Samuel E. Salcedo ;
Vedant Borad ;
Sara S. Richards ;
Adrienne D. Workman ;
Andrea O. RaySource :
RBID : PMC:4347360
Abstract
Introduction: First described by Von Recklinghausen in 1891, fibrous dysplasia is a developmental defect of osseous tissue such that bone is produced with an abnormally thin cortex and marrow is replaced with fibrous tissue that demonstrates characteristic ground-glass appearance on x-ray examination. The underlying defect in fibrous dysplasia is a mutation of the GNAS1 gene, which leads to constitutive activation of gene products that preclude the maturation of osteoprogenitor cells and lead to development of abnormal bone matrix, trabeculae, and collagen, produced by undifferentiated mesenchymal cells. There exists a mainly self-limiting form of fibrous dysplasia classified as monostotic, which is characterized by dysplastic bone in a single location that remains relatively stable throughout life and a polyostotic form, which can exhibit aggressive growth placing adjacent structures at risk for compressive sequelae. Methods: We present the surgical management of an unusual case of monostotic fibrous dysplasia, which exhibited aggressive growth with mass effect, and late presentation, both uncharacteristic features for the monostotic form. The authors also performed a comprehensive review of the literature and discuss the disease process, management options, and indications for surgical treatment. Results: An overview of the disease process and management options is presented. The authors also present details of reconstruction in an unusual form of symptomatic monostotic fibrous dysplasia. Conclusion: Conservative management is usually the mainstay of therapy in asymptomatic cases of fibrous dysplasia. In patients fulfilling criteria for surgical management, craniofacial reconstruction offers a viable option in the surgeon's armamentarium, providing good functional and cosmetic outcomes.
Url: PubMed: 25848443
PubMed Central: 4347360
Links to Exploration step
PMC:4347360***** Acces problem to record *****\
Le document en format XML
Pour manipuler ce document sous Unix (Dilib)
EXPLOR_STEP=$WICRI_ROOT/Ticri/CIDE/explor/HapticV1/Data/Pmc/Corpus
HfdSelect -h $EXPLOR_STEP/biblio.hfd -nk 001C80 | SxmlIndent | more
Ou
HfdSelect -h $EXPLOR_AREA/Data/Pmc/Corpus/biblio.hfd -nk 001C80 | SxmlIndent | more
Pour mettre un lien sur cette page dans le réseau Wicri
{{Explor lien
|wiki= Ticri/CIDE
|area= HapticV1
|flux= Pmc
|étape= Corpus
|type= RBID
|clé= PMC:4347360
|texte= Fibrous Dysplasia: An Overview of Disease Process, Indications for Surgical Management, and a Case Report
}}
Pour générer des pages wiki
HfdIndexSelect -h $EXPLOR_AREA/Data/Pmc/Corpus/RBID.i -Sk "pubmed:25848443" \
| HfdSelect -Kh $EXPLOR_AREA/Data/Pmc/Corpus/biblio.hfd \
| NlmPubMed2Wicri -a HapticV1
| This area was generated with Dilib version V0.6.23. Data generation: Mon Jun 13 01:09:46 2016. Site generation: Wed Mar 6 09:54:07 2024 | ![](Common/icons/LogoDilib.gif) |