Klinik Atölye

Karaciğer: diffüz ve vasküler hastalıklar · Patoloji · Yüksek öncelik

Sinüzoidal obstrüksiyon sendromu

Sinusoidal obstruction syndrome

Sinüzoidal obstrüksiyon sendromu: yayımlanmış olgu görüntüsü, aksiyel kesit

4 adım

Görüntü: Kan X, Ye J, Rong X ve ark., “Diagnostic performance of Contrast-enhanced CT in Pyrrolizidine Alkaloids-induced Hepatic Sinusoidal Obstructive Syndrome.”, 2016, Figure 3. PMC5126558 · doi:10.1038/srep37998 · CC BY 4.0. Değişiklik: kırpıldı, yeniden boyutlandırıldı, odak işaretleri eklendi.

Özet

Sinüzoidal obstrüksiyon sendromu (SOS; eski adıyla veno-oklüzif hastalık), sinüzoidal endotel hasarı nedeniyle hepatik venül ve sinüzoidlerde kan akımının tıkanmasıyla oluşan, karaciğer konjesyonu ve portal hipertansiyonla karakterize bir tablodur; hematopoietik kök hücre nakli ve pirrolizidin alkaloit gibi toksik maruziyetler önemli nedenleridir. Kontrastlı BT hepatomegali, periportal ödem ve yamalı parankim kontrastlanmasını gösterebilir; ana hepatik venler açık kalabileceğinden normal büyük damar görünümü SOS'u dışlamaz. Görüntüleme klinik tanıya destek olur ve portal hipertansiyon bulgularını, ayrıca Budd–Chiari gibi makrovenöz tıkanmaları araştırır. Erken tanınmayan ağır SOS çoklu organ etkilenimi ve karaciğer yetmezliğiyle ilerleyebilir; yalnızca hepatik venlerin açık olmasına bakıp tanıyı dışlamak gecikmeye yol açar.

Faz ve pencere

Arteriyel
Arteriyel fazda karaciğer parankiminde yamalı ve düzensiz kontrastlanma görülebilir; bu erken perfüzyon değişikliği tek başına SOS'a özgü değildir ve portal faz bulgularıyla birlikte okunmalıdır.
Portal tanısal
Portal fazda parankimde yamalı düşük atenüasyon ve heterojen azalmış kontrastlanma, periportal ödem ve portal hipertansiyon işaretleri görülebilir. Küçük intrahepatik venler silik veya dar görünebilir; ana hepatik venler patent kalabilir.

Önerilen pencereler: Karaciğer (G 150 / M 30), Batın (G 400 / M 50).

BT bulguları

  • Erken akut dönemde ağrılı hepatomegali, sıvı tutulumu ve asit sık görülür.
  • Portal ven dalları çevresinde ödem ve parankim içinde yamalı düşük atenüasyon/azalmış portal faz kontrastlanması izlenebilir.
  • Safra kesesi duvarında ödem, perihepatik sıvı ve asit portal hipertansiyon ile birlikte ortaya çıkabilir.
  • Küçük intrahepatik hepatik venler dar, düzensiz ya da silik görünebilir; ana hepatik venlerin opasifikasyonu korunabilir.
  • Asit ve safra kesesi duvarı ödemi sık görülürken, dalak büyümesi ve portosistemik kollateraller akut evrede nadirdir; kronik seyirte portal hipertansiyon bulguları gelişebilir.
  • Hepatik venleri çevreleyen pençe biçimli kontrastlanma, venöz ve denge fazlarında yamalı parankim kontrastlanmasıyla birlikte görülebilir.

Normalde

Karşılaştırmada karaciğer parankimindeki yamalı düşük atenüasyon ve venöz-denge fazlarındaki heterojen kontrastlanma ile periportal ödemi, safra kesesi duvarı ödemini ve asiti değerlendir.

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 s0541; organ sınırları veri setinin otomatik segmentasyonundan, birleştirilerek sadeleştirildi. Görüntüler 2,5 mm kesit aralığına yeniden örneklendi.

Ayırıcı tanı

Budd–Chiari sendromu
ana hepatik ven veya IVC'de trombüs, nonopasifikasyon ya da belirgin çıkış darlığı doğrudan makrovenöz tıkanmayı gösterir.
Konjestif hepatopati
sağ kalp büyümesi, geniş IVC/hepatik venler ve sağ atriyumdan venöz sisteme reflü kardiyak kaynaklı basınç artışını destekler.
Akut hepatit
periportal ödem ve safra kesesi duvar ödemi örtüşebilir, ancak venül daralması ve transplantasyon/toksik maruziyet öyküsü SOS yönünde ipucudur.
Hipoksik hepatit
şok bağlamında yaygın silik hipoenhansman ve başka organlarda düşük perfüzyon bulguları daha olasıdır.

Tuzaklar

  • Ana hepatik venlerin açık olması SOS'u dışlamaz; hastalığın yerleşimi sinüzoidler ve küçük venüllerdedir.
  • Asit, safra kesesi duvarı ödemi ve periportal halo özgül değildir; nakil/toksik maruziyet bağlamı ile parankim paternini beraber değerlendir.
  • Budd–Chiari ve SOS benzer hepatomegali-perfüzyon bozukluğu yapabilir; büyük damar lümenini ve olası trombüsü doğrudan araştır.

Kendini dene

  1. Nakil sonrası hastada hepatomegali, yamalı portal faz hipoenhansmanı ve periportal ödem var; ana hepatik venler açık. En olası tanı?

    Cevabı göster

    Sinüzoidal obstrüksiyon sendromu. Nakil bağlamı ve parankimal/portal ödem paterninin, ana venler açıkken görülmesi küçük venül-sinüzoid düzeyindeki SOS'u destekler. Budd–Chiari büyük venleri tıkar; kardiyak konjesyonda geniş IVC ve kalp bulguları beklenir; kolesistit karaciğer parankimini bu biçimde etkilemez.

  2. Yamalı karaciğer kontrastlanmasına ek olarak sağ atriyum ve IVC geniş, hepatik venlere kontrast reflüsü mevcut. Hangi tanı daha uygundur?

    Cevabı göster

    Konjestif hepatopati. Venöz genişleme ve sağ kalpten geriye kontrast akımı kardiyak venöz konjesyonu gösterir. SOS mikrodolaşım hastalığıdır; viral hepatit bu reflüyü, steatoz ise bu venöz bulguları açıklamaz.

İlgili konular

Kaynaklar

Bu sayfadaki 34 cümle ve 2 quiz sorusu aşağıdaki kaynaklardan alıntılarla tek tek karşılaştırıldı; 5 cümle bu karşılaştırmada düzeltildi (2026-10-09).

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