A narrative review of the literature on the pediatric orthopedic management of fibrous dysplasia
Fibrous dysplasia is a congenital, non-inherited, benign intramedullary bone lesion in which the normal bone marrow is replaced by abnormal fibro-osseous tissue. The disorder can be monostotic (involving a single bone) or polyostotic (involving multiple bones). As the abnormal fibro-osseous tissue c...
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Frontiers Media S.A.
2025-01-01
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Online Access: | https://www.frontiersin.org/articles/10.3389/fped.2024.1502262/full |
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author | Elio Paris Giacomo De Marco Oscar Vazquez Christina Steiger Sana Boudabbous Romain Dayer Dimitri Ceroni |
author_facet | Elio Paris Giacomo De Marco Oscar Vazquez Christina Steiger Sana Boudabbous Romain Dayer Dimitri Ceroni |
author_sort | Elio Paris |
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description | Fibrous dysplasia is a congenital, non-inherited, benign intramedullary bone lesion in which the normal bone marrow is replaced by abnormal fibro-osseous tissue. The disorder can be monostotic (involving a single bone) or polyostotic (involving multiple bones). As the abnormal fibro-osseous tissue compromises the mechanical strength of bone, it can result in pain, deformity, fractures, or abnormalities in bone mechanics with inappropriate bone alignment. This narrative review attempts to summarize more than 20 years of observations of patients with FD to help pediatric orthopedists establish a care framework that can improve its identification, understand the impact that endocrinopathies can have on its clinical presentation, and optimize the management of bone disorders. Our focus is specifically on orthopedic manifestations of FD and modern management alternatives. The past 20 years have provided major advances in understanding of fibrous dysplasia (FD), and it is clear that the pediatric orthopedist's role remains highly relevant in the management of all types of FD. Surgical treatment remains appropriate when pain is unresponsive to other medical treatments, when a pathological fracture is impending or has happened, when a deformity is worsening or has formed, or when there is a suspicion of malignant transformation. The pediatric orthopedist must be aware, therefore, of the particularities of the different bones on which they may be called to intervene, and they should give very careful consideration to their operative strategy, which must be adjusted to the biological and static characteristics of the bone. |
format | Article |
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institution | Kabale University |
issn | 2296-2360 |
language | English |
publishDate | 2025-01-01 |
publisher | Frontiers Media S.A. |
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series | Frontiers in Pediatrics |
spelling | doaj-art-009a80b891f34927939a93eae632e2c22025-01-31T06:40:02ZengFrontiers Media S.A.Frontiers in Pediatrics2296-23602025-01-011210.3389/fped.2024.15022621502262A narrative review of the literature on the pediatric orthopedic management of fibrous dysplasiaElio Paris0Giacomo De Marco1Oscar Vazquez2Christina Steiger3Sana Boudabbous4Romain Dayer5Dimitri Ceroni6Faculty of Medicine, University of Geneva, Geneva, SwitzerlandPediatric Orthopedic Unit, Pediatric Surgery Service, Geneva University Hospitals, Geneva, SwitzerlandPediatric Orthopedic Unit, Pediatric Surgery Service, Geneva University Hospitals, Geneva, SwitzerlandPediatric Orthopedic Unit, Pediatric Surgery Service, Geneva University Hospitals, Geneva, SwitzerlandRadiology Department, Geneva University Hospitals, Geneva, SwitzerlandPediatric Orthopedic Unit, Pediatric Surgery Service, Geneva University Hospitals, Geneva, SwitzerlandPediatric Orthopedic Unit, Pediatric Surgery Service, Geneva University Hospitals, Geneva, SwitzerlandFibrous dysplasia is a congenital, non-inherited, benign intramedullary bone lesion in which the normal bone marrow is replaced by abnormal fibro-osseous tissue. The disorder can be monostotic (involving a single bone) or polyostotic (involving multiple bones). As the abnormal fibro-osseous tissue compromises the mechanical strength of bone, it can result in pain, deformity, fractures, or abnormalities in bone mechanics with inappropriate bone alignment. This narrative review attempts to summarize more than 20 years of observations of patients with FD to help pediatric orthopedists establish a care framework that can improve its identification, understand the impact that endocrinopathies can have on its clinical presentation, and optimize the management of bone disorders. Our focus is specifically on orthopedic manifestations of FD and modern management alternatives. The past 20 years have provided major advances in understanding of fibrous dysplasia (FD), and it is clear that the pediatric orthopedist's role remains highly relevant in the management of all types of FD. Surgical treatment remains appropriate when pain is unresponsive to other medical treatments, when a pathological fracture is impending or has happened, when a deformity is worsening or has formed, or when there is a suspicion of malignant transformation. The pediatric orthopedist must be aware, therefore, of the particularities of the different bones on which they may be called to intervene, and they should give very careful consideration to their operative strategy, which must be adjusted to the biological and static characteristics of the bone.https://www.frontiersin.org/articles/10.3389/fped.2024.1502262/fullnarrative reviewpediatricorthopedicfibrous dysplasiabone disorder |
spellingShingle | Elio Paris Giacomo De Marco Oscar Vazquez Christina Steiger Sana Boudabbous Romain Dayer Dimitri Ceroni A narrative review of the literature on the pediatric orthopedic management of fibrous dysplasia Frontiers in Pediatrics narrative review pediatric orthopedic fibrous dysplasia bone disorder |
title | A narrative review of the literature on the pediatric orthopedic management of fibrous dysplasia |
title_full | A narrative review of the literature on the pediatric orthopedic management of fibrous dysplasia |
title_fullStr | A narrative review of the literature on the pediatric orthopedic management of fibrous dysplasia |
title_full_unstemmed | A narrative review of the literature on the pediatric orthopedic management of fibrous dysplasia |
title_short | A narrative review of the literature on the pediatric orthopedic management of fibrous dysplasia |
title_sort | narrative review of the literature on the pediatric orthopedic management of fibrous dysplasia |
topic | narrative review pediatric orthopedic fibrous dysplasia bone disorder |
url | https://www.frontiersin.org/articles/10.3389/fped.2024.1502262/full |
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