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Burun ve paranazal sinüsler · Patoloji · Orta öncelik

Sinonazal fibroz displazi

Sinonasal fibrous dysplasia

Sinonazal fibroz displazi: yayımlanmış olgu görüntüsü, aksiyel kesit
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4 adım

Görüntü: Elhend SB, Belfquih H., “Fibrous dysplasia: Rare sphenoidal and parietal involvement.”, 2025, Figure 1:. PMC12530758 · doi:10.25259/sni_510_2025 · CC BY-NC-SA 4.0. Değişiklik: yeniden boyutlandırıldı.

Özet

Kraniofasiyal fibroz displazi, normal kemik dokusunun fibro-osseöz matriksle yer değiştirdiği benign bir kemik hastalığıdır ve sinüs çevresindeki kemikleri genişleterek hava boşluklarını daraltabilir. Kontrastsız ince kesit BT, buzlu cam veya karma matriksi, kemik genişlemesini ve sinüs anatomisine etkisini gösterir. Fibroz displazi, radyografik özellikleri diğer fibro-osseöz lezyonlarla örtüşebildiğinden kesin tanı için histopatoloji gerekli olup genişleyici ve destrüktif olmayan seyir gösterir. Optik kanal, orbital duvar veya kafa tabanı komşuluğundaki daralma atlanırsa işlevsel etkilenim ve hastalık yayılımı eksik tarif edilebilir.

Faz ve pencere

Kontrastsız tanısal
Kemik algoritmalı ince kesitlerde genişlemiş kemik içinde buzlu cam, sklerotik ya da karma fibro-osseöz matriks ve sinüs lümeninde biçim değişikliği görülür.

Önerilen pencereler: Kemik (G 1800 / M 400).

BT bulguları

  • Maksilla veya başka kraniofasiyal kemik segmentinde düzgün ekspansiyon ve kortikal kabukta korunma ya da incelme.
  • Medüller boşluğun homojen buzlu cam görünümünde yoğun matriksle silinmesi.
  • Olgunlaşma ve kemik içeriğine bağlı sklerotik veya yamalı karma yoğunluk alanları.
  • Lezyon, kaynaklarda belirtildiği üzere genişleyici ve non-destrüktif seyir izler; radyografik özellikler lezyonun boyutuna ve derecesine göre değişkenlik gösterir.
  • Maksiller sinüs tabanının veya yan duvarının içe itilmesiyle sinüs hacminde azalma ve asimetri.
  • Fibroz displaziye bağlı kemik genişlemesi, etmoid hücrelerin, sfenoid sinüs duvarlarının veya frontal resesin daralmasına ve sinüs ostiumlarının kemiksel deformasyona uğramasına yol açabilir.
  • Orbita tabanı, optik kanal veya kafa tabanı açıklıklarında komşuluk nedeniyle oluşan daralma.

Normalde

BT’de lezyonun yerleşimini ve matriksini tanımlayın; uzanımını ve çevre yapılarla ilişkisini değerlendirin.

Normal BT ile kıyasla

Aynı bölgenin, kaynak veri setinde patoloji içermediği belirtilen bir BT incelemesini kesit kesit kaydırın; organ sınırlarını açarak anatomiyi hasta görüntüsüyle karşılaştırın. Küçük rastlantısal bulgular tümüyle dışlanmamıştır.

Kaynak: TotalSegmentator v2.01 veri seti (Wasserthal ve ark., Radiology: Artificial Intelligence 2023), CC BY 4.0, vaka s0849; organ sınırları veri setinin otomatik segmentasyonundan, birleştirilerek sadeleştirildi. Görüntüler 2,5 mm kesit aralığına yeniden örneklendi.

Ayırıcı tanı

Ossifiye fibrom
keskin sınırlı, çoğu kez kapsüllü ve kemiği dışa doğru iten ekspansil odak oluşturur.
Osteoma
sınırlı, çok yoğun kemik odağı olarak sinüs lümenine projekte olur; kemiğin geniş bir segmentini tutmaz.
Düşük dereceli osteosarkom
korteks yıkımı, düzensiz periostal reaksiyon veya ekstraosseöz yumuşak doku bileşeni varsa benign displaziden uzaklaşılır.

Tuzaklar

  • Buzlu cam görünümünü tek başına tanı kabul etmeyin; lezyonun kemiği genişletme biçimi ve sınırının medullaya geçişi ossifiye fibromdan ayrımda önemlidir.
  • Sinüs lümenindeki opasifikasyon yalnızca mukozal hastalık olarak yorumlanmamalı; fibroz displazide karakteristik kemik genişlemesi ve duvarların itilmesi ayrıntılı incelenmelidir.
  • Belirgin asimetriyi agresif destrüksiyonla eşitlemeyin; düzgün konturlu genişleme fibroz displazide görülebilir, korteks dışına çıkan yumuşak doku kitlesi ise atipiktir.

Kendini dene

  1. Genç erişkinin sinüs BT’sinde maksiller kemik düzgün genişlemiş, buzlu cam matriksli ve sinüs tabanı içe itilmiş. En olası tanı nedir?

    Cevabı göster

    Fibroz displazi. Kemiğin segmental ve düzgün genişlemesiyle buzlu cam matriks fibroz displaziyi destekler. Ossifiye fibrom daha keskin sınırlı bir ekspansil odak oluşturur; osteit duvar kalınlaşmasıdır, osteom ise fokal yoğun kemiktir.

  2. Buzlu cam matriksli kraniofasiyal kemik lezyonunda sinüs anatomisinin etkisini belirlemek için hangi kıyas en yararlıdır?

    Cevabı göster

    Karşı maksiller sinüs hacmi. Karşı maksiller sinüs aynı anatomik düzeyde kontrol sağlar; taban ve duvarların yer değiştirmesi ile lümen daralmasını görünür kılar.

İlgili konular

Kaynaklar

Bu sayfadaki 30 cümle ve 2 quiz sorusu aşağıdaki kaynaklardan alıntılarla tek tek karşılaştırıldı; 7 cümle bu karşılaştırmada düzeltildi (2026-10-08).

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