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Q J Med 2000; 93: 531-534
© 2000 Association of Physicians

Clinical features, diagnosis and outcome of acute portal vein thrombosis

C.L. Sheen, H. Lamparelli, A. Milne, I. Green and J.K. Ramage

From the North Hampshire Hospital, Basingstoke, UK

Received 9 March 2000 and in revised form 1 June 2000


    Summary
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 Methods
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 Discussion
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The clinical features of acute portal vein thrombosis (APVT) are poorly defined in the literature. The proportion that progress to chronic PVT and the influences of various treatments are unknown. Between 1996 and 1998, nine patients presented to our hospital with varying upper gastrointestinal symptoms. They were found to have APVT by colour flow Doppler ultrasound, which was confirmed by CT scanning. All were tested for procoagulant tendencies and then treated with intravenous heparin for 7 days and warfarin for 3 months. Colour flow Doppler ultrasound or CT was done regularly to assess response to treatment. There was complete resolution of thrombus in five patients. Four patients had procoagulant tendencies identified; of these the thrombus resolved in two cases and in two cavernous transformation occurred. In most cases, the thrombus disperses on heparin and warfarin, although the effect of this therapy is unknown. A randomized trial of thrombolytic therapy may be appropriate, in an attempt to reduce the rate of progression to chronic PVT.


    Introduction
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Portal vein thrombosis is an important condition because of its serious long-term complications. Few reports of the clinical features and consequences of acute portal vein thrombosis have been made. The difficulty in clinical diagnosis occurs because of the non-specific nature of the symptoms and signs.1,2 Using ultrasound, recent thrombosis may be missed since it is echo-poor and may be indistinguishable from normal blood flow.3–5 In those cases in which the B-mode ultrasound raises suspicion, colour flow Doppler is required, since it can clinch the diagnosis.6

When portal vein thrombosis in its acute state goes unrecognized, symptoms resolve as collateral channels become established, and the end result may be portal hypertension.5–7 It is desirable to avoid this scenario, and previous studies have demonstrated resolution of acute thrombosis using a variety of techniques. These include selective portal venography with infusion of streptokinase8 or urokinase,2,9 open surgical thrombectomy and intravenous heparinization followed by oral anticoagulation.6

We describe a prospective study on the diagnosis, treatment and outcomes of patients admitted to a District General Hospital with a diagnosis of acute portal vein thrombosis.


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Between 1996 and 1998, nine patients presented with varying gastrointestinal symptoms. Most commonly these were anorexia, right upper quadrant or epigastric pain, and vomiting. On admission, before the diagnosis of APVT all patients had blood tests for full blood count, coagulation screen, renal and liver function. APVT was diagnosed by colour flow Doppler ultrasound scanning and confirmed by contrast enhanced CT scanning of the abdomen. The thrombus was clearly seen on CT and Doppler ultrasound in all cases, and it was not felt necessary to proceed to angiography in any of the cases.

Once diagnosed, patients were assessed for a prothombotic tendency by history, examination and blood thrombophilia screen. In addition, selected patients had a Ham's test, bone-marrow aspirate and trephine The thrombophilia screen included tests for protein C, protein S, antithrombin III, lupus anticoagulant, anticardiolipin antibodies and activated protein C resistance. In those with reduced activated protein C resistance, molecular analysis for factor V Leiden defect was performed. The patients were then commenced on intravenous heparin infusion to achieve an activated partial thromboplastin time 1.5 to 3 times that of normal. They were also loaded with warfarin, and the dose then adjusted to obtain an international normalized ratio (INR) of 2 to 4. Once the INR was in range, the heparin was stopped and the warfarin was continued for 3 months. Those patients with other indications for prolonged anticoagulation (e.g. mutiple embolic events) were continued on warfarin as indicated by their underlying conditions.

Patients underwent repeat imaging by CT or Doppler ultrasound several times after diagnosis to ascertain whether the thrombosis had resolved. Doppler ultrasound was performed serially by an experienced operator (IG), and in all cases the diagnosis was confirmed by portal-phase CT scanning, hence the presence of APVT is virtually certain in all nine cases. The shortest follow-up period to date was 49 days, and the longest was 532 days, both demonstrating clearance of thrombus. Although APVT is more common in patients with cirrhosis due to reduced hepatopetal blood flow, none of these patients gas cirrhosis clinically or radiologically. Cirrhosis was excluded clinically by the absence of signs of chronic liver disease, and radiologically by the absence of nodularity of the liver substance on CT and ultrasound scans.


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These are summarized in Table 1Go. Thrombus resolved in five out of the nine cases at a median of 197 days after diagnosis. Four cases had prothrombotic tendencies, and in two of these the thrombus resolved. Five patients had abnormal liver function tests, and this correlated with signs of abdominal sepsis in four of these, hence these patients probably had portal pyaemia. In total five cases had evidence of abdominal sepsis, of which thrombus resolved in two. A combination of a prothrombotic state and abdominal sepsis was seen in only one case, and the thrombus did not resolve. There were no reported differences in the initial CT and ultrasound appearances in those cases in which the thrombus resolved and those in which it did not.


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Table 1 Patient characteristics and outcomes

 


    Discussion
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Acute portal vein thrombosis is a difficult clinical diagnosis because of the wide variety of clinical presentations. If the thrombus remains, portal venous hypertension and varices may result.5–7 In our series, 5/9 resolved using heparin and warfarin. It is not known whether the therapy is beneficial, although clinically it is logical. It is also unclear as to the most appropriate duration of therapy and whether this should be adjusted depending on risk factors such as identified thrombophilia or strong family history of thromboembolism

Colour flow Doppler ultrasonography can accurately diagnose fresh thrombus,6 and CT may be complementary in the diagnosis.4,6 Although there is dual blood supply to the liver, APVT can lead to ischaemic areas that resolve as the portal vein thrombosis resolves (Figure 1Go).



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Figure 1. CT scans (after i.v. contrast) of the liver before (left), and after (right) resolution of thrombus.

 
Procoagulant states are established risk factors for PVT.10–13 As more abnormalities are identified, the percentage with an underlying number of possible coagulation disorders may increase.14 (In our series thrombus resolved in over 50% after treatment.) However, intra-abdominal infection is the commonest cause of APVT,6,15 and in adults this is often diverticulitis causing a portal pyaemia. Most patients with abdominal sepsis and/or abnormal liver function tests will have a standard ultrasound. However, colour flow Doppler scanning of the portal vein should be considered for any patient with intra-abdominal sepsis who develops an unexplained fever or abnormal liver function. If APVT is found, we suggest that these patients be treated with anticoagulant therapy, although the long-term benefits of this therapy remain to be ascertained.

In any patient with unexplained acute abdominal symptoms, especially in those with signs of sepsis or abnormal liver function tests, the possibility of an acute portal vein thrombosis should be considered and investigated by colour flow Doppler ultrasound scanning. With increasing use of colour Doppler ultrasound and contrast-enhanced CT scanning, it is likely that more cases of acute portal vein thrombosis will be diagnosed, as it is likely that many are missed currently. The prevalence of chronic portal vein thrombosis in the population is unknown, but the prevalence of diagnosed cases remains low. We have shown that 50% of acute cases do not progress to chronic occlusion, hence the acute disease is more common than was previously thought. This is a condition that presents frequently in district hospitals, and clinicians dealing with acute medical and surgical emergencies should be aware of the symptoms, signs and means of diagnosis.


    Notes
 
Dr J.K. Ramage, North Hampshire Hospital, Aldermaston Road, Basingstoke, RG24 9NA Back


    References
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 Summary
 Introduction
 Methods
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 Discussion
 References
 
1. Jaffe V, Lygidakis NJ, Blumgart LH. Acute portal vein thrombosis after right hepatic lobectomy: successful treatment by thrombectomy. Br J Surg1982; 69:211.[ISI][Medline]

2. Bilbao JI, Rodriguez-Cabello J, Longo J, Zornoza G, Paramo J, Lecumberri FJ. Portal thrombosis: percutaneous trans-hepatic treatment with urokinase—a case report. Gastrointest Radiol1989; 14:326–8.[ISI][Medline]

3. Mori H, Hayashi K, Uetani M, Matsuoka Y, lwao M, Maeda H. High-attenuation recent thrombus of the portal vein: CT demonstration and clinical significance. Radiology1987; 163:353–6.[Abstract/Free Full Text]

4. Miller VE, Berland LL. Pulsed Doppler duplex sonography and CT of portal vein thrombosis. Am J Roentgenol1985; 145:73–6.[Abstract/Free Full Text]

5. Albertyn LE. Case report: acute portal vein thrombosis. Clin Radiol1987; 38: 645–8.[ISI][Medline]

6. Cohen J, Edelman RR, Chopra S. Portal vein thrombosis: a review. Am J Med1992; 92: 173–82.[ISI][Medline]

7. Gertsch P, Matthews J, Lerut J, Luder P, Blumgart LH. Acute thrombosis of the splanchnic veins. Arch Surg1993; 128: 341–5.[Abstract]

8. Paquet KJ, Raschke E, Popov S, Bucheler E. The fibrinolytic therapy of thrombosed portal veins and shunts in liver cirrhosis with portal hypertension and prehepatic block. J Cardiovasc Surg1971; 12: 147–51.[Medline]

9. Miyazaki Y, Shinomura Y, Kitamura S, Hiraoka S, Tomoda K, Nezu R, Kamiike W, Nakarmura H, Matsuzawa Y. Portal vein thrombus associated with active ulcerative colitis: percutaneous transhepatic recanalization. Am J Gastroenterol1995; 90: 1533–4.[ISI][Medline]

10. Levoir D, Aubertin JM, Becheur H, Bloch F, Alhenc Gelas M, Petite, JP. A new hereditary cause of portal vein thrombosis (abstract). Gastroenterology1996; 110: A1250.[ISI]

11. Pertuiset E, Tribout B, Wechsler B, Bellin M F, Godeau P, Jian R, Rambaud JC. Systemic lupus erythematosus presenting with portal vein thrombosis. Am J Med1989; 86: 501–2.[ISI][Medline]

12. Fidler H, Booth A, Hodgson HJF, Calam J, Luzatto L, Hughes JM. Portal vein thrombosis in myeloproliferative disease. Uses of thrombolytic and antiplatelet treatments. Br Med J1990; 300: 590–2.

13. Green D, Ganger DR, Blei AT. Protein C deficiency in splanchnic venous thrombosis. Am J Med1987; 82: 1171–4.[ISI][Medline]

14. Bergenfeldt M, Svensson PJ, Borgström A. Mesenteric vein thrombosis due to factor V Leiden gene mutation. Br J Surg1999; 86:1059–62.[ISI][Medline]

15. Babcock DS. Ultrasound diagnosis of portal vein thtombosis a complication of appendicitis. Am J Roentgenol1979; 133:317–19.[ISI][Medline]


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