Triple Localized Cranio-Facial Fibrous Dysplasia: A Case Report

Mahmoud Hamou,Adil Eabdenbitsen,Fahd Elayoubi,Mohamed Rachid Ghailan

doi:10.15342/ijms.v6ir.257

Mahmoud Hamou, Adil Eabdenbitsen + Show 2 more

Open Access

https://doi.org/10.15342/ijms.v6ir.257

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Abstract

Introduction: fibrous dysplasia of bones is a non-hereditary congenital benign bone disorder, where normal bone is replaced by pseudofibrous tissue containing immature osteogenesis.Case report: a 29-year-old patient with chronic hemodialysis who had a swollen mouth and hard palate that had been evolving for a year, impeding chewing and swallowing and causing facial asymmetry. Cranio-facial CT revealed multiple osteolytic bone-blast lesions, the histopathological study of which favored polyostotic fibrous dysplasia.Discussion: fibrous dysplasia lesions may be single or multiple and may be responsible for pain and fragility, causing neurological complications in craniofacial localization. Imaging and, when a biopsy is needed, histology can establish the diagnosis. The treatment is based on bisphosphonates or, in special cases, surgical excision.

Highlights

Fibrous dysplasia (FD) is a rare congenital disorder; it may be single in the monostotic form or multiple in the polyostotic form [1]
FD is due to a postzygotic mutation of the Gnas1 gene responsible for immature osteogenesis in a bone weakened by fibrosis [3]
We report the case of a polyostotic FD in chronic hemodialysis with craniofacial involvement

Summary

INTRODUCTION

Fibrous dysplasia (FD) is a rare congenital disorder; it may be single in the monostotic form or multiple in the polyostotic form [1]. CASE REPORT Mrs B.L, aged 29, followed for 8 years for chronic renal failure at the hemodialysis stage on indeterminate nephropathy, who consulted for a tumefaction of the buccal floor and the hard palate evolving since a year, hindering the mastication and the swallowing and causing right eye pain. The second was a localized mass at the level of the hard palate, bilobed, well-defined extending posteriorly to the osteo-membranous junction of the palate, leading to a decrease in nasal flow on the right side (fig B) and the third in front of the rising branch of the right mandibular bone, which is responsible for obvious facial asymmetry. A cranio-facial CT revealed multiple osteolytic lesions of the right maxillary bone, the buccal floor and the right mandibular angle, blowing the bone especially around the maxillary sinus, with an overall osteo-condensed appearance of the cranial vault (fig).

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