Polyostotic fibrous dysplasia: report of a patient

Polyostotic fibrous dysplasia is a benign fibro-osseous lesion in which the normal structure of the bone is replaced by fibrous tissue and by nonfunctional bony structures that show a trabecular aspect and have an unknown etiology. It is a very rare and infrequent disease on which there is little in...

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Main Authors: Esteban Hernández Ruiz, Margarita Hernández García
Format: Article
Language:Spanish
Published: Editorial Ciencias Médicas 2013-10-01
Series:Acta Médica del Centro
Subjects:
Online Access:http://www.revactamedicacentro.sld.cu/index.php/amc/article/view/26
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author Esteban Hernández Ruiz
Margarita Hernández García
author_facet Esteban Hernández Ruiz
Margarita Hernández García
author_sort Esteban Hernández Ruiz
collection DOAJ
description Polyostotic fibrous dysplasia is a benign fibro-osseous lesion in which the normal structure of the bone is replaced by fibrous tissue and by nonfunctional bony structures that show a trabecular aspect and have an unknown etiology. It is a very rare and infrequent disease on which there is little information in the literature. This situation has motivated us to report this case. It is hypothesized that there is a gene mutation encoding G alpha protein which is responsible for the condition. The surgical management of these cases is a challenge and it is also controversial. In this patient, it was reported an osteolytic lesion in both femoral necks that prevented proper ambulation and it was decided to conduct an inter-consultation with the Orthopedic Specialist for a better management. The patient underwent a hip computed tomography that confirmed the lesion and, subsequently, he underwent a biopsy that reported a polyostotic fibrous bone dysplasia. The dysplasia increases in size until general body growth ceases at the end of puberty.
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spelling doaj.art-cc0d08ad41464a25be8e7845d439af5b2022-12-22T03:55:50ZspaEditorial Ciencias MédicasActa Médica del Centro2709-79272013-10-0174525726Polyostotic fibrous dysplasia: report of a patientEsteban Hernández Ruiz0Margarita Hernández García1Hospital Pediátrico “José Luis Miranda”, Santa Clara, Villa ClaraHospital Pediátrico “José Luis Miranda”, Santa Clara, Villa ClaraPolyostotic fibrous dysplasia is a benign fibro-osseous lesion in which the normal structure of the bone is replaced by fibrous tissue and by nonfunctional bony structures that show a trabecular aspect and have an unknown etiology. It is a very rare and infrequent disease on which there is little information in the literature. This situation has motivated us to report this case. It is hypothesized that there is a gene mutation encoding G alpha protein which is responsible for the condition. The surgical management of these cases is a challenge and it is also controversial. In this patient, it was reported an osteolytic lesion in both femoral necks that prevented proper ambulation and it was decided to conduct an inter-consultation with the Orthopedic Specialist for a better management. The patient underwent a hip computed tomography that confirmed the lesion and, subsequently, he underwent a biopsy that reported a polyostotic fibrous bone dysplasia. The dysplasia increases in size until general body growth ceases at the end of puberty.http://www.revactamedicacentro.sld.cu/index.php/amc/article/view/26displasia fibrosa poliostótica
spellingShingle Esteban Hernández Ruiz
Margarita Hernández García
Polyostotic fibrous dysplasia: report of a patient
Acta Médica del Centro
displasia fibrosa poliostótica
title Polyostotic fibrous dysplasia: report of a patient
title_full Polyostotic fibrous dysplasia: report of a patient
title_fullStr Polyostotic fibrous dysplasia: report of a patient
title_full_unstemmed Polyostotic fibrous dysplasia: report of a patient
title_short Polyostotic fibrous dysplasia: report of a patient
title_sort polyostotic fibrous dysplasia report of a patient
topic displasia fibrosa poliostótica
url http://www.revactamedicacentro.sld.cu/index.php/amc/article/view/26
work_keys_str_mv AT estebanhernandezruiz polyostoticfibrousdysplasiareportofapatient
AT margaritahernandezgarcia polyostoticfibrousdysplasiareportofapatient