Vaskuläre Erkrankungen der Leber

Kernaussagen

VALDI

• Vaskuläre Lebererkrankungen (VALDI) sind heterogene Erkrankungen.

• Da sie insgesamt eine eher seltene Krankheitsgruppe darstellen, ist nationale und internationale Zusammenarbeit erforderlich, um wissenschaftliche Erkenntnisse von großen Patientenkollektiven zusammenzutragen.

• In diesem Artikel werden das Budd-Chiari-Syndrom (BCS), die nicht zirrhotische, nicht maligne Pfortaderthrombose (PVT), die idiopathische nicht zirrhotische portale Hypertension (INCPH) und das sinusoidale Obstruktionssyndrom (SOS) beschrieben.

Diagnostik

• Eine rasche Diagnostik ist wichtig, da fulminante Verläufe auftreten können.

• Eine lückenlose Ursachendiagnostik ist immer obligat, um Grunderkrankungen, die gut therapierbar sind, aufzudecken.

• Die Standarddiagnostik besteht bei BCS und PVT aus Duplexsonografie und ergänzender CT oder MRT mit Kontrastmittel zur Ausdehnungs- und Ursachendiagnostik, während bei INCPH und SOS eine Leberbiopsie – bei SOS kombiniert mit HVPG-Messung – zur Diagnosestellung erforderlich ist.

Therapie

• Die Therapie sollte rasch an einem spezialisierten Zentrum mit allen therapeutischen Optionen inkl. Lebertransplantationsprogramm erfolgen.

• Die Antikoagulation stellt bei BCS und PVT die Erstlinientherapie dar. Bei Patienten, die hierauf nicht oder ungenügend ansprechen, muss rasch über interventionelle oder operative Therapien entschieden werden.

• Patienten mit INCPH werden in der Regel wie Patienten mit Leberzirrhose behandelt. Die Therapie bei SOS beschränkt sich zumeist auf supportive Maßnahmen.

• Literatur

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