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portal vein thrombosis

Etiology: - hypercoagulable state - inherited - JAK2 V617F mutation (50%) - factor V Leiden mutation - prothrombin gene mutation (G20210) - antithrombin 3 deficiency - protein C deficiency - protein S deficiency - acquired - lupus anticoagulant - cirrhosis - disseminated intravascular coagulation (DIC) - burns - sepsis - malignancy - myeloproliferative disorders - pregnancy & postpartum state - oral contraceptives - inflammation - diverticulitis, inflammatory bowel disease, pancreatitis - stasis from reduced blood flow - splenectomy - ingestion of a foreign body (fish bone), with penetration of the duodenum, pancreas, & superior mesenteric vein (case report) [8] - not a consequence of the progression of cirrhosis [6] Pathology: - formation of a thrombus within the portal vein - thrombus can involve segments of the mesenteric veins or splenic vein - portal hypertension - diminished blood supply to the liver - cavernous formation of the portal vein, which represents bridging collaterals around the occlusion Clinical manifestations: - acute form - fever - abdominal pain if mesenteric vein extension Laboratory: - D-dimer level may be elevated - JAK2 V617F mutation Special laboratory: - abdominal ultrasound (doppler) Radiology: - computed tomography (CT) with contrast - magnetic resonance imaging (MRI) * CT or MRI every 3 months for anticoagulation, - continue anticoagulation if partial improvement is seen. Complications: - intestinal ischemia, infarction - does not directly affect progression of cirrhosis [6] - predicts near-term appearance of liver cancer & pancreatic cancer with a poor prognosis [7] Management: - acute form (non-cirrhotic patients) - thrombolysis through the transhepatic route, avoids the need for systemic thrombolysis - tissue-type plasminogen activator (tPA) - follow with warfarin for at least 3 months (indefinitely inpatients with inherited coagulation disorders [3] - rivaroxaban of benefit (rather than warfarin) [11] - shunt surgery with subsequent anticoagulation is an alternative [3] if intestinal infarction suspected - anticoagulation for at least 3 months [4] - chronic form - patient presenting with bleeding varices with imaging revealing cavernous transformation of the portal vein, denoting long-standing disease - anticoagulation - indications: - not advisable [2] - intestinal ischemia [13] - recent (< 6 months) involving intrahepatic portal vein branches or with > 50% occlusion of the main portal vein, or mesenteric veins - patients listed for liver transplantation[13] - contraindications to anticoagulation - recent (< 6 months) involving intrahepatic portal vein branches or with < 50% occlusion of the main portal vein, or mesenteric veins [13] - CT or MRI every 3 months [13] - start anticoagulation if symptoms, progression of thrombosis, candidate for liver transplantation [13] - chronic thrombosis (> 6 months) with complete occlusion & collaterals - anticoagulation with warfarin, DOACs, LMW heparin Child/Pugh class A/B cirrhosis - LMW heparin only Child/Pugh class C cirrhosis [13] - screen for esophagal varices prior to anticoagulation [13] - band ligation if not receiving beta-blocker prophylaxis [13] - anticoagulation may facilitate regression of thrombosis [13] with recanalization & diminish cirrhosis progression without increasing bleeding risk in patients with cirrhosis [10] - variceal banding or sclerotherapy, often requires several sessions to obliterate the bleeding [3] - octreotide infusion has also been used in acute bleeding - propranolol is often used prevent rebleeding in the setting of portal vein thrombosis, but evidence base is lacking [3] - screening of asymptomatic patients with compensated cirrhosis is not advisable - screen for malignancy if portal vein thrombosis incidentally found - portal vein revascularization with transjugular intrahepatic shunting (TIPS) if additional indications for TIPS (refractory ascites or variceal bleeding) or in liver transplant candidates. [13]

Related

portal vein

General

splanchnic vein thrombosis (SVT) liver disease hypercoagulability

References

  1. Wikipedia: Portal vein thrombosis http://en.wikipedia.org/wiki/Portal_vein_thrombosis
  2. Boyer TD Management of Portal Vein Thrombosis Gastroenterol Hepatol (N Y). 2008 October; 4(10): 699-700. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3104181/
  3. Said A and Katz J Medscape: Portal Vein Obstruction http://emedicine.medscape.com/article/182425-overview
  4. Medical Knowledge Self Assessment Program (MKSAP) 16, 17, 18. American College of Physicians, Philadelphia 2012, 2015, 2018
  5. DeLeve LD, Valla DC, Garcia-Tsao G; American Association for the Study Liver Diseases. Vascular disorders of the liver. Hepatology. 2009 May;49(5):1729-64. PMID: 19399912
  6. Nery F et al. Causes and consequences of portal vein thrombosis in 1,243 patients with cirrhosis: Results of a longitudinal study. Hepatology 2015 Feb; 61:660 PMID: 25284616 http://onlinelibrary.wiley.com/doi/10.1002/hep.27546/abstract
  7. Sogaard KK et al. Splanchnic venous thrombosis is a marker of cancer and a prognostic factor for cancer survival. Blood 2015 Jun 18 PMID: 26089394
  8. Gharib SD et al Case 21-2015 - A 37-Year-Old American Man Living in Vietnam, with Fever and Bacteremia. N Engl J Med 2015; 373:174-183. July 9, 2015 PMID: 26154791 http://www.nejm.org/doi/full/10.1056/NEJMcpc1411439
  9. Yonal I et al The clinical significance of JAK2V617F mutation for Philadelphia-negative chronic myeloproliferative neoplasms in patients with splanchnic vein thrombosis. PMID: 22569900
  10. Loffredo L, Pastori D, Farcomeni A, Violi F. Effects of anticoagulants in patients with cirrhosis and portal vein thrombosis: A systematic review and meta-analysis. Gastroenterology 2017 May 4; PMID: 28479379
  11. Smalberg JH, Arends LR, Valla DC Myeloproliferative neoplasms in Budd-Chiari syndrome and portal vein thrombosis: a meta-analysis. Blood. 2012 Dec 13;120(25):4921-8. Review. PMID: 23043069 Free Article
  12. Plessier A et al. Rivaroxaban prophylaxis in noncirrhotic portal vein thrombosis. NEJM Evid 2022 Nov 22; 1:EVIDoa2200104. PMID: 38319842 https://evidence.nejm.org/doi/10.1056/EVIDoa2200104
  13. Davis JPE et al. AGA clinical practice update on management of portal vein thrombosis in patients with cirrhosis: Expert review. Gastroenterology 2025 Feb; 168:396 PMID: 39708000 https://www.gastrojournal.org/article/S0016-5085(24)05664-6/fulltext