A 59 year old non-smoker male presented with a long history of recalcitrant lower extremity ulcers. Forty years ago, he underwent splenectomy for a nonmalignant reason. Shortly after his surgery, he developed multiple collateral veins over his abdomen with extensive lower extremities venous varicosities. He was ultimately diagnosed with Budd-Chiari syndrome (BCS), classified as Budd-Chiari type I whereby the thrombus was limited to the intrahepatic inferior vena cava, not directly compromising the hepatic veins [1,2]. He was initially treated with warfarin but due to bleeding complications, this medication was discontinued. More recently, he has been plagued with chronic recurrent lower extremity ulcers. Although healing promptly with compression therapy, his clinical course was marked by frequent ulcer recurrence. He otherwise denied dyspnea or recent weight changes.Physical examination revealed a 10 cm ulcer involving the medial foreleg of the right lower extremity with hemosiderin staining and dermal sclerosis. Multiple large varices were evident on the inspection of his extremities. There was no evidence of lower extremity deep vein thrombosis on venous Doppler ultrasound. His ECHO revealed a normal left ventricular ejection fraction (68%) and normal right ventricular systolic pressure. Laboratory evaluation was negative for lupus anticoagulant, factor V Leiden and PT 20210 mutation. He had normal levels of protein C and S and there was no evidence of myelodysplasia. His initial evaluation however, also included a chest X-ray that revealed a soft-tissue prominence in the right tracheobronchial angle (Fig. 1) worrisome for malignancy.
DiagnosisThe presence of an indeterminate right paratracheal mass prompted CT of the chest and abdomen to exclude malignancy. We present his CT as a pictorial demonstration of several physiologic and imaging characteristics associated with BCS. The underlying pathology of BCS is represented by occlusive calcified thrombus within the intrahepatic IVC ( Fig. 2a; thick arrow). The intrahepatic IVC occlusion results in development of extensive collateral pathways including a markedly enlarged azygous system as well as prominent hemiazygous (HA) and intercostal collateral systems (thin arrows) [3][4][5]. The indeterminate soft tissue prominence noted on the plain film of the chest was shown to be due to the marked dilatation of the azygous system in the right tracheobronchial angle ( Fig. 2b; arrow). The correlative chest CT confirmed that there was no imaging findings of lymphadenopathy or neoplasm in the chest or abdomen and the chest X-ray findings were shown to be due to physiologic changes from BCS.
DiscussionWith an estimated prevalence of nearly two per million inhabitants, Budd-Chiari syndrome (BCS) is a rare disease [6,7]. BCS may have a high degree of heterogeneity A. Tafur Á W. Wysokinski Á T. Vrtiska Á R. McBane II