Fibrous dysplasia of the orbital region: Series of 12 cases and review of the literature

Bertin, Hélios; Huon, Jean‐François; Guillot, Pascale; Longis, Julie; Corre, Pierre; Bordereau, Sylvain; Lebranchu, Pierre

doi:10.1016/j.jfo.2019.10.012

Cited by 11 publications

(3 citation statements)

References 35 publications

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“…Expansion of FD involving the orbital bones may lead to deformities such as vertical dystopia or proptosis (Fig. 3c), which if severe can result in visual disturbances [10]. Narrowing of the optic canal may rarely lead to nerve compression and vision loss, particularly in patients with MAS-associated growth hormone excess [11].…”

Section: Clinical Featuresmentioning

confidence: 99%

Fibrous dysplasia in children and its management

Gun,

Arif,

Boyce

2023

Current Opinion in Endocrinology, Diabetes &Amp; Obesity

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Purpose of review The purpose of this review is to provide a comprehensive overview into the diagnosis and management of fibrous dysplasia (FD) in children. Recent findings FD is a mosaic disorder arising from somatic Gαs variants, leading to impaired osteogenic cell differentiation. Fibro-osseous lesions expand during childhood and reach final disease burden in early adulthood. The mainstay of treatment focuses on surgical correction of skeletal deformities, physiatric care, and medical management of associated hyperfunctioning endocrinopathies. Bisphosphonates may be helpful to treat bone pain, but do not alter lesion quality or progression. Emerging evidence suggests that the RANKL inhibitor denosumab may be effective in improving lesion activity and mineralization, however further studies are needed to determine the potential utility of this and other novel therapies, particularly in children with FD. Summary Management of children with FD has unique challenges related to skeletal growth and age-related lesion progression. Inclusion of children in clinical research is critical to develop effective treatment strategies to treat FD lesions and prevent their development.

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Section: Clinical Featuresmentioning

confidence: 99%

Fibrous dysplasia in children and its management

Gun,

Arif,

Boyce

2023

Current Opinion in Endocrinology, Diabetes &Amp; Obesity

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“…Na região craniofacial, o complexo zigomático-maxilar e o mais afetado pela DF monostótica (Assiri, 2020;Bertin et al, 2020;Pontes-Madruga et al, 2020;Verma et al, 2013). Clinicamente, uma DF causa expansão óssea, sendo geralmente diagnosticada na infância, adolescência ou adulto até terceira década de vida.…”

Section: Introductionunclassified

Reconstrução de região infraorbitária após ressecção de displasia fibrosa com enxerto ósseo bovino liofilizado

Puricelli

Gália

Quevedo

et al. 2021

RSD

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Introdução: A displasia fibrosa monostótica envolvendo a região maxilo-facial é mais frequente na maxila, podendo ocorrer em outros ossos. Na maioria dos casos, a condição é assintomática e tem um crescimento lento, com evolução autolimitada, associada à maturação esquelética. O tratamento cirúrgico conservador é a conduta de eleição, sempre que possível. Objetivo e relato do caso: O artigo tem por objetivo apresentar o relato de um caso clínico de paciente jovem, com diagnóstico de displasia fibrosa monostótica zigomática-maxilar. O tratamento proposto foi a ressecção da lesão e reconstrução imediata do defeito com enxerto ósseo bovino liofilizado. O enxerto foi mantido com microplaca e parafusos de titânio. O paciente permaneceu em controle clínico e imaginológico sem evidências de recidiva no período pós-operatório de seis anos. Considerações finais: Na reconstrução imediata, o enxerto ósseo bovino liofilizado atuou como arcabouço osteocondutor e favoreceu a recuperação volumétrica da área, proporcionando suporte para reorganização do defeito ósseo.

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“…Fibrous dysplasia (FD) is a benign fibro-osseous tumor of growing bone whereby fibrous tissue replaces normal medullary bone. 1,2 FD likely arises from somatic activating mutations in the Gsα gene, leading to abnormal proliferation of skeletal progenitor cells. 3 FD exists along a spectrum of severity, affecting one (monostotic) or many (polyostotic) bones of the axial and appendicular skeleton, 4 with symptoms varying depending on location and disease burden.…”

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confidence: 99%

The Natural History of Fibrous Dysplasia of the Orbit

Blum

Cho

Villavisanis

et al. 2023

Plastic &Amp; Reconstructive Surgery

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Fibrous dysplasia (FD) is a benign fibroosseous tumor of growing bone whereby fibrous tissue replaces normal medullary bone. 1,2 FD likely arises from somatic activating mutations in the Gsα gene, leading to abnormal proliferation of skeletal progenitor cells. 3 FD exists along a spectrum of severity, affecting one (monostotic) or many (polyostotic) bones of the axial and appendicular skeleton, 4 with symptoms varying depending on location and disease burden. FD may exist in isolation or within the characteristic triad of symptoms associated with McCune-Albright syndrome (MAS), including polyostotic fibrous dysplasia, café-au-lait spots, and precocious puberty. 5 FD usually presents in childhood and may progress throughout puberty and adolescence, ultimately "burning out" around skeletal maturity with apoptosis of mutation-bearing mesenchymal cells. 6 Devastating sequelae of FD include optic neuropathy secondary to ischemic Background: Orbital fibrous dysplasia (FD) is a disease of disordered fibroosseous proliferation secondary to altered osteogenesis, with potential sequelae including compressive neuropathy and irreversible vision loss. The purpose of this study was to evaluate the natural history and longitudinal outcomes of 37 patients with orbital FD who underwent stratified surgical management. Methods: All patients treated for FD from 2015 to 2021 were identified, yielding 185 patients, 39 with orbital involvement. Impressions from head computed tomographic scans were analyzed by a craniofacial radiologist to evaluate location and timing of tumor growth. Operative records were reviewed to determine surgical approach (partial excision/contouring, complete excision, or partial excision with optic nerve decompression). Results: The average patient age at diagnosis was 10.7 ± 4.5 years, and the average follow-up was 5.2 ± 4.7 years. Of the 37 patients with orbital involvement, 28 (75.7%) had optic canal involvement. Of those with optic canal involvement, 13 (46.4%) required partial excision with optic nerve decompression, whereas 15 (53.6%) did not. Of those without optic canal involvement, two patients (22.2%) underwent partial excision/contouring of the anterior orbit to correct dystopia and/or proptosis, and four patients (44.4%) underwent complete excision of the orbital component and reconstruction with bone graft or mesh. Younger age at diagnosis was associated with an increased number of surgical interventions (P = 0.011), younger age at first optic canal decompression (P = 0.003), and worse visual outcomes (P = 0.009). Conclusion:In the authors' cohort, patients diagnosed at a younger age required more operations, underwent decompression earlier, and had worse visual outcomes.

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Fibrous dysplasia of the orbital region: Series of 12 cases and review of the literature

Cited by 11 publications

References 35 publications

Fibrous dysplasia in children and its management

Fibrous dysplasia in children and its management

Reconstrução de região infraorbitária após ressecção de displasia fibrosa com enxerto ósseo bovino liofilizado

The Natural History of Fibrous Dysplasia of the Orbit

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