Orbitocranial Fibrous Dysplasia

Fadle, Khalid Nasser; Hassanein, Ahmed Gaber; Kasim, Abdin K.

doi:10.1097/scs.0000000000003033

Cited by 9 publications

(2 citation statements)

References 38 publications

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“… 10 Initially described as “osteitis fibrosa generalisata” by von Recklinghausen in 1891 in a patient with skeletal deformities due to fibrotic bone changes, the disorder became known as FD in 1938 when Lichtenstein introduced the term. 2 , 15 FD is associated with a gene mutation in the α subunit of the stimulatory G protein encoded by the gene on GNAS1 gene 16 , 17 that affect the cells that produce bone. The germ-line mutation occurs in the early stages of fetal development.…”

Section: Discussionmentioning

confidence: 99%

Orbitocranial Fibrous Dysplasia: Immediate Reconstruction with Titanium 3-dimensional Printing

Pruksapong

Lohwongwatana

2020

Plastic and Reconstructive Surgery - Global Open

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Summary: We present the case of an 18-year-old man who was referred to our department with a suspected tumor in the right orbital region, which caused exophthalmos due to its pressure effect. Preoperative CT imaging revealed an expansile ground-glass appearance of bone mass, with sclerotic bone on the right frontal bone (right superior orbital wall). There was no orbital mass and no enlargement of the extraocular muscles and tendons; the retrobulbar fat and optic nerve appeared unremarkable. A preoperative tissue biopsy confirmed the FD. Complete tumor removal was performed via the right hemicoronal approach. The orbital roof and temporal bone defect were immediately reconstructed using the 3-dimensional titanium printing plate.

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Section: Discussionmentioning

confidence: 99%

Orbitocranial Fibrous Dysplasia: Immediate Reconstruction with Titanium 3-dimensional Printing

Pruksapong

Lohwongwatana

2020

Plastic and Reconstructive Surgery - Global Open

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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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Osteology of the orbit

Dutton

2025

Atlas of Clinical and Surgical Orbital Anatomy

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Orbitocranial Fibrous Dysplasia

Abstract: Radical excision of orbitocranial FD is potentially curative with no extra morbidity. It can achieve good aesthetic and functional results with no recurrence.

Cited by 9 publications

References 38 publications

Orbitocranial Fibrous Dysplasia: Immediate Reconstruction with Titanium 3-dimensional Printing

Orbitocranial Fibrous Dysplasia: Immediate Reconstruction with Titanium 3-dimensional Printing

The Natural History of Fibrous Dysplasia of the Orbit

Osteology of the orbit

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