Portal Vein Thrombophlebitis in Behcet’s Disease
Is Catheter Directed Thrombolysis Warranted?


Greg Schwartzman,M.D., Jeff Geschwind,M.D., &
George Hartnell, M.D.
Johns Hopkins Hospital

Figs. 1. Magnified image from superior mesenteric arteriogram showing segmental narrowing of SMA branches consistent with a vasculitis.

Fig. 2.Ultrasound showed portal vein thrombus and dilatation to 21 mm.

Case Reference No. CC-0100-04

A 22-year-old African-American male presented to an outside hospital two years previously complaining of malaise, fatigue, weight loss, abdominal pain, arthralgias, headache, and intermittent fevers. His past medical history was significant for uveitis and meningitis. On the basis of this history he was diagnosed as having Behcet's disease.

After two years of waxing and waning symptoms, an arteriogram at another hospital showed "diffuse central arterial stenoses of the SMA branches only visualized when priscoline was administered", suggesting vasculitis (Figure 1). No contrast was identified in the portal vein suggesting portal vein thrombosis. Ultrasound showed portal vein thrombus extending into the splenic vein. The portal vein was dilated to 21 mm (Figure 2). There was cavernous transformation. Ultrasound at another hospital a month earlier had reportedly shown a patent portal vein. Abdominal CT showed portal vein thrombosis and marked distension suggesting infectious thrombophlebitis or neoplastic thrombus. There were multiple low attenuation areas consistent with abscesses (Figure 3). There was an elevated sedimentation rate of 130 and a WBC count of 25,000. Ultrasound guided fine-needle aspiration of the portal vein revealed pus, which grew E. coli. The patient was referred for possible thrombolysis of the portal venous thrombosis.

Fig.3. Abdominal CT showing multiple low attenuation areas consistent with abscesses.

Fig.4.Direct portogram showing dilated and occluded portal vein with saccular dilatation of multiple intrahepatic portal branches.

Using ultrasound and fluoroscopic guidance, a 21-gauge needle was used to access the portal vein and contrast was injected. This showed an extensively expanded and obstructed portal venous system that was aneurysmal and saccular in appearance. Thrombus extended into the SMV and distal splenic vein. The proximal splenic and inferior mesenteric veins were patent. A Cragg-MacNamara infusion catheter was placed in the portal vein and SMV and r-TPA (Alteplase) infused overnight at 0.02 mg/kg/hr. Angiography the next day showed minimal clearing of thrombus but significant residual thrombus. R-TPA was continued at the same rate. Angiography later that day revealed no significant change. The infusion rate of r-TPA was increased to 0.04 mg/kg/hr but after another 10 hours there was no further improvement. Therefore an Angiojet device was deployed but only had the effect of causing thrombus to develop in the splenic vein and the IMV. Therefore r-TPA was continued for another 12 hours but final images after 72 hours of infusion there was no further clot lysis. The patient has been managed subsequently on heparin, coumadin, and three intravenous courses of antibiotics.

Questions:

1) Was catheter directed thrombolysis wise in this patient with Behcet’s disease? Given the propensity for venous thrombosis in Behcet’s disease, was catheter manipulation too risky?

2) What is the best therapeutic option for a patient of this age?

3) Would higher doses of thrombolytics or different agents have benefited this patient? What about the use of mechanical thrombolysis?

Related Cases:

Case 397_05 -- Portal vein thrombosis

Case 797_02 -- Pancreatitis with portal vein thrombosis after biopsy

Case 1097_02 -- Portal vein thrombosis in the setting of acute pancreatitis: treatment with portal reconstruction