Konjenital trakeal stenoz ve tam halka

Özet

Konjenital trakeal stenoz, sıklıkla trakea arka membranöz duvarının bulunmadığı tam kıkırdak halkalarla ilişkili sabit daralmadır ve çocukta stridor, solunum sıkıntısı ya da tekrarlayan enfeksiyonla ortaya çıkabilir. BT anormal segmentin uzunluğunu ve en dar yerini haritalar; BT anjiyografi eşlik eden vasküler halka veya sol pulmoner arter slingini hava yoluyla birlikte gösterir. O biçimli trakea ve uzun segment daralma tam halka için güçlü ipucu olsa da BT tek başına kıkırdak halkasının histolojik yapısını doğrulamaz. Darlığın uzunluğunu veya bronş-vasküler anomalileri atlamak hava yolu tıkanıklığının kapsamını ve klinik ağırlığını olduğundan az gösterir.

Faz ve pencere

BTA tanısal

İnce kesit toraks BT anjiyografide lümenin trakea boyunca sabit daraldığı segment, O biçimli kesit görünümü, dar segmentin üst-alt devamı ve pulmoner arterlerin seyri değerlendirilir. Sol pulmoner arterin sağ pulmoner arterden çıkıp trakea ile özofagus arasından sola geçmesi sling lehinedir; vasküler ilişki için kontrastlı anjiyografi gerekir.

Kontrastsız

Hava yolu lümeni ve akciğer parankimi, eldeki kontrastsız ince kesitlerde daralma, distal mukus birikimi, atelektazi ve eşlik eden bronş anomalileri açısından izlenebilir; damar halkası/sling anatomisini dışlamak için kontrastsız inceleme yeterli değildir.

Önerilen pencereler: Mediasten (G 350 / M 50), Akciğer (G 1500 / M -600), Anjiyo (G 600 / M 150).

BT bulguları

• Sabit trakeal daralma — aksiyel görüntülerde lümen kısa veya uzun bir segmentte düzenli biçimde küçük kalır; boyun ve toraks boyunca devamlılık kontrol edilir.
• O biçimli trakea — aksiyel kesitte posterior membranöz duvarın yerini kıkırdak halka almış izlenimi veren dairesel lümen tam halka şüphesi doğurur; tek başına kesin kanıt değildir.
• Uzun segment daralma — stenozun üst ve alt sınırları koronal/sagittal reformasyonlarda izlenir; eşlik eden ana bronş stenozu ayrıca kaydedilir.
• Pulmoner arter sling — sol pulmoner arter sağ pulmoner arterden çıkar ve trakea ile özofagus arasından sola yönelerek distal trakeayı veya sağ ana bronşu sıkıştırabilir.
• Vasküler halka veya ark anomalisi — aortik ark ve dallarının trakea/özofagus çevresindeki seyri lümeni dıştan daraltabilir.
• Trakeobronşiyal dallanma anomalisi — trakeal bronş, trakeal trifurkasyon veya bridging bronchus eşlik edebilir; bronşların her akciğer lobuna gidişi izlenmelidir.
• Distal obstrüksiyon bulguları — dar segmentin altındaki mukus, segmental atelektazi, havalanma farkı veya tekrarlayan enfeksiyonla uyumlu parankim opasitesi görülebilir.
• Cantrell ve Guild sınıflamasında konjenital trakeal stenoz yaygın, huni biçimli veya segmental olarak sınıflandırılır.
• BT, trakeal lümen çapının ve dar segmentin uzunluğunun değerlendirilmesini sağlayabilir.

Normalde

Normal trakeada aksiyel kesitte kıkırdak ön-yan duvarı at nalı biçimindedir ve posterior membranöz duvar lümeni tamamlar; çap toraks boyunca sabit bir uzun segmentte daralmaz. Aynı düzeyde trakea şeklini, üst-alt lümen kalibresini ve pulmoner arterlerin özofagusa göre seyrini karşılaştırın.

Ayırıcı tanı

Vasküler halka/slinge bağlı dıştan bası

lümen damar yapısına komşu noktada yassılaşır veya yer değiştirir; kontrastlı BT anormal damar seyrini ortaya koyar.

Edinsel postentübasyon stenozu

çoğu kez kaf veya trakeostomi düzeyinde kısa, fokal skar daralması vardır; doğuştan uzun segment O biçimli lümen beklenmez.

Trakeomalazi

inspiryumda kalibre daha geniş, ekspiryum/dinamik görüntüde lümen belirgin çöker; konjenital sabit daralmadan fazlar arası değişkenlikle ayrılır.

Trakeobronşiyal tümör

fokal endolüminal yumuşak doku kitlesi oluşturup hava yolunu daraltabilir; kontrastlı BT’de kitle görülebilir.

Tuzaklar

• O biçimli trakeayı BT’de tam halka kesin tanısı gibi sunmayın; morfoloji kuşku oluşturur, mukozal ve kıkırdak değerlendirmesi bronkoskopiyle tamamlanır.
• Dar segmenti tek aksiyel kesitten ölçüp tanımlamayın; koronal ve sagittal planda stenozun tüm uzunluğunu ve karina/ana bronş uzanımını izleyin.
• Pulmoner arter slingini yalnız trakea darlığına bakarak dışlamayın; sol pulmoner arterin kökenini ve trakea-özofagus arasındaki seyrini kontrastlı anjiyografide takip edin.
• Solunum fazı ve entübasyon tüpü lümen şeklini etkileyebilir; klinik olarak mümkünse tüp ucunun konumunu, görüntüleme fazını ve gerçek duvar sınırını hesaba katın.

Kendini dene

1. Bebekte uzun segment sabit daralma ve dairesel trakea lümeni var; sol pulmoner arter trakea-özofagus arasından geçiyor. Birliktelik nedir?

   • Tam halka ile pulmoner arter sling
   • Trakeomalazi ve çift aort arkı
   • Postentübasyon stenozu ve web
   • Trakeal bronş ve bronş atrezisi
   Cevabı göster

   Tam halka ile pulmoner arter sling. Sabit uzun segment daralma, dairesel lümen ve sol pulmoner arterin trakea-özofagus arasındaki sling seyri ring-sling birlikteliğini düşündürür. Trakeomalazi dinamik çöker; postentübasyon darlığı tipik olarak kısa ve edinilmiş; trakeal bronş dallanma varyantıdır.

2. Aynı trakea düzeyinde inspiryumda lümen açık, ekspiryumda belirgin çöküyor; sabit uzun segment daralma yok. En olası durum nedir?

   • Trakeomalazi
   • Konjenital tam halka stenozu
   • Trakeal web
   • Endobronşiyal tüberküloz
   Cevabı göster

   Trakeomalazi. Solunum fazları arasında belirgin lümen çökmesi dinamik duvar zayıflığını, yani trakeomalaziyi destekler. Tam halka stenozu sabit ve genellikle uzun segmenttir; trakeomalazi ise ekspirasyonda dinamik çökme yapar.

İlgili konular

• Trakeal bronş ve aksesuar bronşlar

Kaynaklar

Bu sayfadaki 40 cümle ve 2 quiz sorusu aşağıdaki kaynaklardan alıntılarla otomatik olarak karşılaştırıldı; 5 cümle bu karşılaştırmada düzeltildi (2026-10-09).

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