Budd-Chiari Syndrom, Review und Illustration der Bildgebung

Zusammenfassung

Das Budd-Chiari-Syndrom ist eine seltene vaskuläre Erkrankung, die durch eine Obstruktion des hepatischen venösen Abflusses gekennzeichnet ist. Ätiologisch spielen diverse Erkrankungen, die eine Gerinnungsstörung verursachen, wie beispielsweise myeloproliferative Erkrankungen eine Rolle. Der akute Gefäßverschluss kann zu einer akuten Phlebitis mit Fieber und der klassischen Trias des akuten Leberversagens aus Aszites, Hepatomegalie und abdominellen Schmerzen führen. Allerdings kommen auch subakute Verlaufsformen vor. Aufgrund der wechselnden Symptomatik und Ausprägung, abhängig vom zeitlichen Verlauf und dem Ausmaß der betroffenen Gefäße, gestaltet sich eine Diagnose oft schwierig. Die Sonografie als ubiquitär verfügbares und kosteneffizientes diagnostisches Mittel spielt dabei eine führende Rolle. Mithilfe der Doppler-Sonografie lässt sich zusätzlich die Hämodynamik visualisieren. Beim akuten thrombotischen Verschluss lassen sich die betroffenen Lebervenen meist nicht oder nur teilweise darstellen. Bei nicht okkludierenden Thromben können turbulente Flussmuster im Bereich der venösen Abflussstörung entstehen, im Bereich der Stenose ist die Flussgeschwindigkeit dann erhöht. Die Kontrastmittelsonografie bietet hinsichtlich einer Diagnosestellung eine höhere Spezifität als die Dopplersonografie. Durch die Computertomografie (CT) und Magnetresonanztomografie (MRT) können Thromben beziehungsweise die Ursache für eine Obstruktion teilweise direkt visualisiert werden. Sobald die Diagnose gesichert ist, muss eine Antikoagulation eingeleitet werden, aber auch eine Therapie des zugrunde liegenden Krankheitsbildes begonnen werden. Reichen symptomkontrollierende Maßnahmen nicht aus, kann eine Angioplastie/ein Stenting zur Wiedereröffnung kurzstreckiger Stenosen oder eine Transjuguläre intrahepatische portosystemische Shunt-Anlage (TIPSS) erwogen werden. Als Ultima Ratio bleibt die Lebertransplantation. Die kontroverse Studienlage zur Präzision der diagnostischen Methoden und Charakteristika der Bildgebung werden in diesem Review anhand zahlreicher Fallbeschreibungen zusammengefasst.

Abstract

Budd-Chiari syndrome is a rare vascular disorder characterized by obstruction of the hepatic venous outflow. Various diseases causing coagulopathy play a role in aetiology, such as myeloproliferative disorders. Acute vascular occlusion may lead to acute phlebitis with fever. The classic triad of acute liver failure may be present with ascites, hepatomegaly, and abdominal pain. However, subacute courses of disease were also observed. Because of the variable symptoms and severity extent, depending on the acuity of the course and the extent of the affected vessels, diagnosis is often difficult. Sonography, as a ubiquitously available and cost-effective diagnostic tool, plays a leading role. Doppler ultrasonography can be used to visualize hemodynamics in particular. In acute thrombotic occlusion, the affected hepatic veins usually cannot or only partially be visualized. In non-occluding thrombi, turbulent flow patterns may develop in the area of venous outflow obstruction, and flow velocity is then increased in the area of stenosis. Contrast enhanced ultrasound offers even better specificity of diagnosis. Computed tomography and magnetic resonance imaging can directly visualize thrombi and the cause of obstruction. Once the diagnosis is confirmed, anticoagulation must be initiated, but therapy of the underlying disease must also be started. If symptom-controlling measures are not sufficient, angioplasty/stenting to reopen short-segment stenoses or implantation of a TIPSS device may be considered. Liver transplantation remains ultima ratio. As studies on the precision of diagnostic methods are controversial, the characteristics of imaging for BCS are therefore summarized in this review on the basis of several illustrating case reports.

Schlüsselwörter

Budd Chiari - BCS - Sonografie - Doppler - Kontrastmittel - CEUS - Stent - TIPS - CT - MRT - Leber

Keywords

Budd Chiari - BCS - Ultrasound - Doppler - Contrast agent - CEUS - Stent - TIPSS - CT - MRT - Liver

Publication History

Received: 01 May 2021

Accepted after revision: 14 September 2021

Article published online:
24 November 2021

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