Splanchnic vein thrombosis

Zusammenfassung

Die Venenthrombose im Splanchnikusgebiet – einschließlich Thrombosen der Pfortader, der Mesenterial-, Milz- und suprahepatischen Venen – ist eine unterdiagnostizierte Erkrankung mit einem heterogenen klinischen Bild und einer nicht unbeträchtlichen Quote an Zufallsbefunden.

Die Hauptrisikofaktoren umfassen abdominelle Erkrankungen oder Eingriffe (z.B. Infektionen, Zirrhose, abdominelle Krebserkrankungen oder chirurgische Maßnahmen), hämatologische

Störungen (insbesondere myeloproliferative Neoplasien), hereditäre Thrombophilien und hormonelle Störungen. Kürzlich wurden neue Biomarker für subklinische Erkrankungen identifiziert: die JAK2-Mutation und die Durchflusszytometrie für CD55 und CD59. Die klinische Manifestation ist gewöhnlich unspezifisch. In der akuten Phase können als wichtigste Symptome Bauchschmerzen, gas-trointestinale Blutungen und Aszites auftre-ten; zu den langfristigen Folgen hingegen gehören die Leberzirrhose und die portale Hypertonie.

Fortschritte bei der nicht invasiven Gefäßdarstellung (Doppler-Ultraschall, Gefäß-Computertomografie und Magnetresonanztomogra-fie) haben die Diagnostik der splanchnischen Venenthrombose verbessert. Auffällige Blutbefunde können auf eine zugrunde liegende hämatologische oder hepatische Störung hinweisen.

Die optimale Behandlung der splanchnischen Venenthrombose ist noch eine offene Frage, da große klinische Studien fehlen. Experten empfehlen übereinstimmend, die akute, symptomatische, nicht zirrhotische Pfortaderthrombose im akuten Stadium mittels parenteraler Antikoagulation zu behandeln und an-schließend über mindestens 3 Monate orale Antikoagulanzien zu geben; bei persistieren-den prothrombotischen Faktoren wird jedoch eine lebenslange Behandlung empfohlen. Bei einem Budd-Chiari-Syndrom wird für alle Pa-tienten ohne größere Kontraindikationen eine Antikoagulation empfohlen. Allerdings muss das Nutzen-/Risikoverhältnis einer gerinnungshemmenden Therapie sowohl für die Akutbehandlung als auch für die langfristige Sekundärprävention noch besser untersucht werden.

Summary

Splanchnic vein thrombosis (SVT) – including mesenteric, portal, splenic and supra-hepatic veins thrombosis – is an underdiagnosed disease, with heterogeneous clinical presentations and a non-negligible rate of incidental findings.

The main risk factors include abdominal diseases or interventions (e.g. infections, cirrhosis, abdominal cancer or surgical procedures), haematological disorders (mainly myeloproliferative neoplasms), inherited thrombophilic states and hormonal imbalances. New biological markers of subclinical disorders have recently been identified: JAK2 mutation and flow cytometry for CD55 and CD59.

Clinical manifestations are generally aspecific. During the acute phase, main symptoms can be abdominal pain, gastrointestinal bleeding and ascites; while long-term consequences include liver cirrhosis and portal hypertension.

Advances in non-invasive vascular imaging (Doppler ultrasound, angio-computed tomography and magnetic resonance imaging), have improved the diagnosis of SVT. Alterations in blood tests may suggest an underlying haematological or hepatic disorder.

The optimal treatment of SVT remains an open issue, since large clinical trials are lacking. Expert consensus recommend to treat acute symptomatic non-cirrhotic portal vein thrombosis with parenteral anticoagulation during the acute phase, followed by oral anticoagulants for at least 3 months, though lifelong treatment is recommended in case of persistent prothrombotic factors. In Budd-Chiari syndrome, anticoagulation is recommended for all patients in the absence of major contraindications. However, the risk to benefit-ratio of anticoagulant therapy, both in the acute phase and for the long-term secondary prevention, still needs to be better assessed.

Schlüsselwörter

Splanchnikus-Venenthrombose - Risikofaktoren - Behandlung - Klinische Historie

Keywords

Splanchnic vein thrombosis - risk factors - treatment - clinical history

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