Orbital kemikte fibröz displazi

Özet

Kraniofasiyal fibröz displazi, normal kemik iliğinin fibröz doku ve düzensiz örgü kemikle yer değiştirdiği, çoğu kez yavaş seyirli benign bir kemik lezyonudur. Orbita duvarı veya sfenoid tutulduğunda kemik ekspansiyonu glob konumunu değiştirebilir ve optik kanalı daraltabilir; tanıda ve yayılım haritasında BT temel yöntemdir. BT’de tipik buzlu cam matriks ve komşu kemiğe geçişli sınırlar görülür, ancak matriks görünümü yaşla ve lezyonla değişken olabilir. Optik kanal daralmasını tek başına görme kaybı olarak yorumlamak veya eşlik eden yeni ekspansil lezyonu kaçırmak görsel işlev tehdidini yanlış sınıflandırabilir.

Faz ve pencere

Kontrastsız tanısal

İnce kesit kemik serisinde ekspansil kemik içi buzlu cam dansitesi, kortikal incelme ve sfenoid/optik kanal çevresinde daralma izlenir; damar içi kontrast tipik kemik matriksini göstermek için gerekmez.

Önerilen pencereler: Kemik (G 1800 / M 400), Yumuşak doku (G 400 / M 40).

BT bulguları

• Buzlu cam matriks: kemik iliği yerine geçen homojen veya hafif heterojen yüksek dansiteli mineralize doku.
• Ekspansil remodelling: sfenoid kanat, frontal kemik veya maksilla dış konturu düzgünce genişler.
• Kortikal kabuk incelir; çoğu tipik lezyonda dış kontur korunur ve keskin litik sınır beklenmez.
• Lezyon komşu kemikle silik geçiş yapabilir; ayrı, kapsüllü bir yumuşak doku kitlesi gibi görünmez.
• Optik kanal lümeni daralabilir veya çevresindeki kemik kalınlaşarak daralabilir; bulgu karşı taraftaki optik kanal ile kıyaslanmalıdır.
• Orbita duvarı kalınlaşması orbital hacmi azaltıp globda öne yer değiştirme veya distopi oluşturabilir.
• Yeni kistik/litik odak ya da hızlı kemik genişlemesi, tipik stabil matriksten farklı davranış gösterir ve ek değerlendirme gerektirir.

Ölçütler ve sınıflamalar

Radyografik kraniofasiyal FD paterni

BT'de homojen yoğun (buzlu cam) görünüm; hastanın yaş ilerledikçe gelişebilen karışık radyo-lusent ve radyo-dens alanlar; santral lusensi ve ince sklerotik kenarı olan kistik görünüm. Paternler değişken olabilir ve tek başına klinik evre değildir.

Normalde

Normal sfenoid kanat, frontal kemik ve maksilla trabeküler kemik görünümünü sürdürür; optik kanal kortike sınırları düzenli ve karşı tarafla yaklaşık simetriktir. Aynı aksiyel ve koronal düzeylerde kemik konturunun dışa bombeleşmesine, matriksin buzlu cam yoğunluğuna ve kanal çapındaki taraf farkına bak; yalnız kanalın dar görünmesi klinik optik nöropatiyi kanıtlamaz.

Ayırıcı tanı

Ossifiye fibrom

genellikle daha iyi sınırlı, ekspansif lezyon; radyolojik ve histolojik değerlendirmelerle fibröz displaziden ayırt edilmesi gereklidir.

Paget hastalığı, fibröz displazinin olası taklitçilerinden biridir.

Osteom

kemik yüzeyinden köken alan, kompakt/kancellöz kemik proliferasyonuyla karakterize, iyi sınırlı ve yavaş büyüyen lezyon; fibröz displazinin geniş matriks yapısından farklıdır.

Metastaz veya miyelom

litik/destrüktif odaklar ve korteks aşımı; tipik geçişli FD matriksi beklenmez.

Anevrizmal kemik kisti

ekspansil, çoğunlukla lusent ve septalı lezyon; FD zemininde ikincil gelişebilir.

Tuzaklar

• Optik kanal daralması otomatik olarak görme kaybı anlamına gelmez; bu görüntü bulgusu klinik görme fonksiyonunun değerlendirilmesinin yerini tutmaz.
• Buzlu cam görünümünün her olguda aynı homojenlikte olmasını bekleme; yaşla veya lezyon bileşimiyle karışık lusent alanlar oluşabilir.
• Hızlı büyüyen kistik değişikliği sıradan FD matriksi kabul etme; ikincil kist veya başka agresif süreç olasılığını araştır.

Kendini dene

1. Yavaş gelişen tek taraflı glob distopisi olan kişide sfenoid kemik genişlemiş ve buzlu cam matriksli. En olası tanı nedir?

   • Ossifiye fibrom
   • Fibröz displazi
   • Osteom
   • Metastatik lezyon
   Cevabı göster

   Fibröz displazi. Kemik boyunca geçişli buzlu cam matriks ve düzgün ekspansiyon fibröz displaziyi destekler. Ossifiye fibrom genellikle daha keskin sınırlıdır; osteom fokal yoğun kemik oluşumudur, metastaz ise çoğu kez destrüktif odak yapar.

2. Sfenoid fibröz displazide BT'de optik kanal dar, ancak hasta görme yakınması tarif etmiyor. Görüntü tek başına neyi kanıtlamaz?

   • Kemik ekspansiyonunu
   • Kanal daralmasını
   • Optik nöropatiyi
   • Orbital duvar kalınlığını
   Cevabı göster

   Optik nöropatiyi. BT kanal lümeni ve kemik anatomisini gösterir; görme siniri işlev kaybı klinik ve oftalmolojik değerlendirmeyle belirlenir. Diğer seçenekler doğrudan kemik görüntüsünde gözlenebilir.

İlgili konular

• Glob pozisyonu ve proptoz ölçümü
• Ekstraoküler kasların normal BT ölçümleri

Kaynaklar

Bu sayfadaki 38 cümle ve 2 quiz sorusu aşağıdaki kaynaklardan alıntılarla tek tek karşılaştırıldı; 7 cümle bu karşılaştırmada düzeltildi (2026-10-08).

1. Clinical guidelines for the management of craniofacial fibrous dysplasiapmc.ncbi.nlm.nih.gov
2. Best practice management guidelines for fibrous dysplasia/McCune-Albright syndrome: a consensus statement from the FD/MAS international consortiumpmc.ncbi.nlm.nih.gov
3. Simultaneous decompression of the orbital lateral wall and optic canal for fibrous dysplasia in early adolescencepmc.ncbi.nlm.nih.gov
4. Minimally invasive resection of a spheno-orbital tumor: Review of surgical techniques and a case reportpmc.ncbi.nlm.nih.gov
5. Small ethmoid mucocele with intracranial extension associated with fibrous dysplasia: A rare case reportpmc.ncbi.nlm.nih.gov
6. Craniofacial Fibrous Dysplasia Involving the Orbit: A Case Report and Literature Review.pubmed.ncbi.nlm.nih.gov
7. Surgical Simulation and Custom-Made Implant for Cranial Fibrous Dysplasia.pubmed.ncbi.nlm.nih.gov
8. Might Trauma Be a Triggering Factor for Craniofacial Fibrous Dysplasia?pubmed.ncbi.nlm.nih.gov
9. The Natural History of Fibrous Dysplasia of the Orbit.pubmed.ncbi.nlm.nih.gov
10. Complex skull defects reconstruction with САD/САМ titanium and polyetheretherketone (PEEK) implants.pubmed.ncbi.nlm.nih.gov
11. Isolated orbital fibrous dysplasia associated with ipsilateral keratoconus.pubmed.ncbi.nlm.nih.gov
12. Simultaneous decompression of the orbital lateral wall and optic canal for fibrous dysplasia in early adolescence.pubmed.ncbi.nlm.nih.gov
13. Cherubism: report of three cases and literature review.pubmed.ncbi.nlm.nih.gov
14. The indication and application of computer-assisted navigation in oral and maxillofacial surgery-Shanghai's experience based on 104 cases.pubmed.ncbi.nlm.nih.gov
15. Orbital Involvement In Sinonasal Diseases.pubmed.ncbi.nlm.nih.gov
16. [Application of computer-assisted navigation in oral and maxillofacial surgery: retrospective analysis of 104 consecutive cases].pubmed.ncbi.nlm.nih.gov
17. Transconjunctival Orbital Decompression in Cherubism.pubmed.ncbi.nlm.nih.gov
18. Innovative Surgical Treatment of Severe Cherubism.pubmed.ncbi.nlm.nih.gov
19. Orbital tumours and tumour-like lesions: exploring the armamentarium of multiparametric imaging.pubmed.ncbi.nlm.nih.gov
20. Small ethmoid mucocele with intracranial extension associated with fibrous dysplasia: A rare case report.pubmed.ncbi.nlm.nih.gov
21. Intracranial aneurysmal bone cysts in association with fibrous dysplasia: Case report and scoping review of case reports.pubmed.ncbi.nlm.nih.gov
22. Massive Mandibular Fibrous Dysplasia Mimicking Calcifying Epithelial Odontogenic Tumor Radiologically: A Case Report.pubmed.ncbi.nlm.nih.gov
23. Pediatric triad of craniofacial fibrous dysplasia, Chiari malformation type I and syringomyelia: a case report.pubmed.ncbi.nlm.nih.gov
24. Unusual Manifestations of Sinonasal Osteomas: A Narrative Review.pubmed.ncbi.nlm.nih.gov
25. Polyostotic fibrous dysplasia: Unveiling the complexity of a rare bone disorder through a case report.pubmed.ncbi.nlm.nih.gov
26. A Case Series and Literature Review of Craniofacial Fibrous Dysplasia.pubmed.ncbi.nlm.nih.gov
27. Protein kinase A is a dependent factor and therapeutic target in mouse models of fibrous dysplasia.pubmed.ncbi.nlm.nih.gov
28. Imaging findings of arrested pneumatisation and differentiation from other skull base lesions.pubmed.ncbi.nlm.nih.gov
29. Orbital pseudotumor and skull base infiltration as the sole manifestation of granulomatosis with polyangiitis.pubmed.ncbi.nlm.nih.gov
30. Cancer predisposition syndromes: an imaging review.pubmed.ncbi.nlm.nih.gov
31. Salvaging Vision: A Study of Non-Traumatic Optic Neuropathies.pubmed.ncbi.nlm.nih.gov
32. When plasmacytoma defies expectations: An unusual maxillary presentation with mixed imaging features in a young patient.pubmed.ncbi.nlm.nih.gov
33. Ossifying fibroma of the maxilla: A case report with literature review.pubmed.ncbi.nlm.nih.gov
34. Osteomyelitis of the Jaw Bones and Its Mimics: Resolving the Diagnostic Enigma.pubmed.ncbi.nlm.nih.gov
35. The solitary skull mass: An atypical presentation of anaplastic large cell lymphoma.pubmed.ncbi.nlm.nih.gov
36. Benign and malignant lesions of the mandible and maxilla: imaging findings and systematic approach.pubmed.ncbi.nlm.nih.gov
37. Malignant Transformation of Musculoskeletal Lesions with Imaging-Pathology Correlation-Part 1: Bone Lesions.pubmed.ncbi.nlm.nih.gov
38. Ossifying Fibroma of the Frontal Sinus: A Case Report and Literature Review.pubmed.ncbi.nlm.nih.gov
39. Craniofacial fibrous dysplasia: Molecular insights, long-term institutional experience, and evolving surgical strategies.pubmed.ncbi.nlm.nih.gov
40. Diagnostic and Treatment Strategies for Adult Non-Ossifying Fibromas: A Case Report and Literature Review.pubmed.ncbi.nlm.nih.gov
41. Review of imaging modalities and radiological findings of calvarial lesions.pubmed.ncbi.nlm.nih.gov
42. Surgical Management of a Case of Frontoparietal Fibrous Dysplasia.pubmed.ncbi.nlm.nih.gov
43. Improving diagnostic clarity in fibro-osseous lesions using modified Gallego's, alizarin red, and von kossa stains: A pilot comparative study.pubmed.ncbi.nlm.nih.gov
44. Benign Fibro-Osseous Lesions of the Head and Neck.pubmed.ncbi.nlm.nih.gov
45. Histological Diversity, Diagnostic Challenges, and Surgical Treatment Strategies of Fibrous Dysplasia of Upper and Mid-Thirds of the Craniomaxillofacial Complex.pubmed.ncbi.nlm.nih.gov
46. Multidisciplinary approach and clinical management of McCune-Albright syndrome.pubmed.ncbi.nlm.nih.gov
47. Polyostotic Fibrous Dysplasia: A Case Report.pubmed.ncbi.nlm.nih.gov
48. Foramen Magnum Stenosis in Achondroplasia: Imaging-Based Surgical Indications, Synchondrosis Fusion, and the Role of Ventriculomegaly.pubmed.ncbi.nlm.nih.gov
49. Craniofacial fibrous dysplasia: Surgery and literature review.pubmed.ncbi.nlm.nih.gov
50. Computer guided resection and reconstruction of intra-osseous zygomatic hemangioma: Case report and systematic review of literature.pubmed.ncbi.nlm.nih.gov
51. Endoscopic management of uncommon benign maxillary sinus lesions: a case series.pubmed.ncbi.nlm.nih.gov
52. Mucinous cystic neoplasms of the liver: current insights into epidemiology, diagnosis, and treatment.pubmed.ncbi.nlm.nih.gov
53. Case Report: Novel <i>ADAMTSL2</i> compound heterozygous mutations in geleophysic dysplasia with bilateral glaucoma and keratoconus-like corneal ectasia.pubmed.ncbi.nlm.nih.gov
54. Fibrous dysplasia of the head and neck in Southern Finland: a retrospective study on clinical characteristics, diagnostics, and treatment.pubmed.ncbi.nlm.nih.gov
55. In Vivo Assessment of Retinal Phenotypes in Axenfeld-Rieger Syndrome.pubmed.ncbi.nlm.nih.gov
56. Advances in origin, evolution, and pathogenesis of optic disc drusen: A narrative review.pubmed.ncbi.nlm.nih.gov
57. Craniofacial disorders and dysplasias: Molecular, clinical, and management perspectives.pubmed.ncbi.nlm.nih.gov
58. Expression of Beta-Catenin, Cadherins and P-Runx2 in Fibro-Osseous Lesions of the Jaw: Tissue Microarray Study.pubmed.ncbi.nlm.nih.gov
59. Modality-specific quantitative radiologic characterization of diffuse sclerosing osteomyelitis of the mandible: a pilot study.pubmed.ncbi.nlm.nih.gov
60. Performance of large language models on image-based oral pathology questions from the Japanese National Dental Examination.pubmed.ncbi.nlm.nih.gov
61. Surgically Treated Benign Bone Tumors and Tumor-like Conditions in the Pediatric Population-A 10-Year Institutional Experience.pubmed.ncbi.nlm.nih.gov
62. Lateral orbitotomy approach for removing hyperostosing en plaque sphenoid wing meningiomas. Description of surgical strategy and analysis of findings in a series of 88 patients with long-term follow up.pubmed.ncbi.nlm.nih.gov
63. Long-term safety and effectiveness of the "OptEase" vena cava filter.pubmed.ncbi.nlm.nih.gov
64. Orbital clock concept: A novel anatomical zoning system for endoscopic vs. external orbital approaches.pubmed.ncbi.nlm.nih.gov
65. Modern technology complements classic technique: 3D-printed inter-locking cutting guides for frontal sinus osteoma resection and reconstruction with split-calvarial bone graft.pubmed.ncbi.nlm.nih.gov
66. Xanthogranulomatous Osteomyelitis as a Bone Tumor Mimic: A Systematic Review of Pre-biopsy Diagnostic Clues and Management Decision Points.pubmed.ncbi.nlm.nih.gov
67. Decoding calvarial lesions: Contemporary neurosurgical management integrating radiological and histopathological features.pubmed.ncbi.nlm.nih.gov
68. A rare calvarial aneurysmal bone cyst in an adult: Case report and literature review.pubmed.ncbi.nlm.nih.gov
69. Journey and Roadblocks in the Management of Aneurysmal Bone Cysts in Patients with Unusual Presentations.pubmed.ncbi.nlm.nih.gov
70. The genetic basis of hydrocephalus: genes, pathways, mechanisms, and global impact.pubmed.ncbi.nlm.nih.gov
71. Neoplasms and tumor-like lesions of the sellar region: imaging findings with correlation to pathology and 2021 WHO classification.pubmed.ncbi.nlm.nih.gov
72. Management of Tibial Adamantinoma: Balancing Oncologic Cure and Reconstructive Longevity.pubmed.ncbi.nlm.nih.gov
73. Fibrous dysplasia: Clinical features, deformities, management, and outcomes.pubmed.ncbi.nlm.nih.gov
74. Aggressive Juvenile Trabecular Ossifying Fibroma in a 4-Year-Old: Case Report Bringing Diagnostic Challenges and Surgical Management.pubmed.ncbi.nlm.nih.gov
75. Common cystic pancreatic neoplasms: A comprehensive magnetic resonance imaging-based review of typical and atypical imaging features.pubmed.ncbi.nlm.nih.gov
76. Craniofacial Ossifying Fibromas in Children: Clinical Variability and Surgical Outcomes in a Case Series.pubmed.ncbi.nlm.nih.gov
77. Conventional radiography for the assessment of focal bone lesions of the appendicular skeleton: fundamental concepts in the modern imaging era.pubmed.ncbi.nlm.nih.gov
78. Magnetic resonance imaging features of craniofacial fibrous dysplasia.pubmed.ncbi.nlm.nih.gov
79. Special Issue: EACR 2026 Congress: Innovative Cancer Science, 8-11 June 2026.pubmed.ncbi.nlm.nih.gov
80. Intraoperative navigation in craniofacial surgery.pubmed.ncbi.nlm.nih.gov
81. Surgical Measures Improving Functional Limitations of the Masticatory System, Aesthetic Deficits, and Skeletal Malformations in Neurofibromatosis Type 1-associated Hemifacial Diffuse Plexiform Neurofibroma Complicated by Rapidly Growing Midfacial Peripheral Nerve Sheath Tumor.pubmed.ncbi.nlm.nih.gov
82. Current concepts of craniofacial fibrous dysplasia: pathophysiology and treatment.pubmed.ncbi.nlm.nih.gov
83. Giant Fibrous Dysplasia of the Sixth Rib Managed in a Rural, Resource-Limited Setting: A Case Report.pubmed.ncbi.nlm.nih.gov
84. Integration of Clinical, Radiographic, Histologic, and Molecular Findings to Diagnose Craniofacial Fibrous Dysplasia.pubmed.ncbi.nlm.nih.gov
85. Imaging of the skull base and orbital tumors.pubmed.ncbi.nlm.nih.gov
86. Cholesterol granuloma of the sphenoid wing with long-term growth dynamics: illustrative case.pubmed.ncbi.nlm.nih.gov
87. A Review of Posteromedial Lesions of the Chest Wall: What Should a Chest Radiologist Know?pubmed.ncbi.nlm.nih.gov
88. Osseous Manifestations of Primary Hyperparathyroidism: Imaging Findings.pubmed.ncbi.nlm.nih.gov
89. Osteoblastic and hyperostotic craniofacial lesion detected by 99mTc-labeled methylene diphosphonate bone scintigraphy and single-photon emission computed tomography/computed tomography: a pictorial essay.pubmed.ncbi.nlm.nih.gov
90. Radiological review of skull lesions.pubmed.ncbi.nlm.nih.gov
91. Apparent diffusion coefficient can assist in differentiating between benign and malignant primary bone tumors in pediatric patients.pubmed.ncbi.nlm.nih.gov
92. Benign incidental do-not-touch bone lesions.pubmed.ncbi.nlm.nih.gov
93. Sclerotic Lesions of the Jaw: A Pictorial Review.pubmed.ncbi.nlm.nih.gov
94. Imaging Features of Mandibular Ramus Osteoma on Computed Tomography: A Case Report With Literature Analysis.pubmed.ncbi.nlm.nih.gov
95. Intraosseous tumours of the knee.pubmed.ncbi.nlm.nih.gov
96. Radiologic and Pathologic Insights into Medication-Related Osteonecrosis of the Jaw in Myeloma Patients: A Report of 3 Cases.pubmed.ncbi.nlm.nih.gov
97. Special Considerations in Pediatric Endoscopic Skull Base Surgery.pubmed.ncbi.nlm.nih.gov
98. What's new in pediatric bone sarcomas and benign bone tumors.pubmed.ncbi.nlm.nih.gov
99. Fibrous Dysplasia Presenting as an Exophytic Gingival Mass: A Rare Clinical Presentation.pubmed.ncbi.nlm.nih.gov
100. Craniofacial Fibrous Dysplasia to Affect or Not the Optic Nerve in Long-Term Follow-Up of Three Cases.pubmed.ncbi.nlm.nih.gov
101. Jansen metaphyseal chondrodysplasia: analysis of craniofacial manifestations.pubmed.ncbi.nlm.nih.gov
102. Non-Arteritic Anterior Ischemic Optic Neuropathy (NA-AION): A Comprehensive Overview.pubmed.ncbi.nlm.nih.gov
103. PET/CT and PET/MRI in ophthalmic oncology (Review).pubmed.ncbi.nlm.nih.gov
104. Pagetoid polyostotic fibrous dysplasia.pubmed.ncbi.nlm.nih.gov
105. Osteoid osteoma of the triquetrum: A rare cause of ulnar-sided wrist pain in an adolescent female.pubmed.ncbi.nlm.nih.gov
106. Intraosseous lipoma of the sphenoid bone causing optic nerve compression: A case report.pubmed.ncbi.nlm.nih.gov

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