An 89-year-old female presented to the emergency department with jaundice, severe pruritis, and low- grade fever of 100° F that had persisted for 3 weeks. Patient had multiple underlying co-morbidities, most notably untreated non-Hodgkin's lymphoma, chronic renal dysfunction from diabetes mellitus, and myelofibrosis. Significant findings on clinical examination included splenomegaly. Initial laboratory analysis revealed total serum bilirubin of 7.5 mg/dl (normal 0.3 to 1.9 mg/dl) (conjugated bilirubin 6 mg/dl), alkaline phosphatase 762 U/L (normal <126 U/L), Aspartate Aminotransferase (AST) 141 U/L (normal <41 U/L), and Alanine Aminotransferase (ALT) 274 U/L (normal <45 U/L). There was thrombocytopenia, platelet count of 90 000/ mm3. The other parameters such as complete blood count, INR (International normalized ratio), and serum albumin were normal. Hepatitis workup including hepatitis A IgM antibodies, hepatitis B surface antigen, hepatitis B IgM core antibody, and hepatitis C antibodies were also negative. In view of the patient's advanced age and clinical presentation, the working diagnosis was that of Klatskin tumor (hilar cholangiocarcinoma) versus lymphoma extending up to the porta hepatis.
An abdominal CT scan, without intravenous contrast at an outside facility was performed (due to underlying renal (dysfunction, estimated glomerular filteration rate (GFR) of 38 ml/min), revealed a 4.8 cm soft tissue attenuation mass at the porta hepatis adjacent to the pancreatic head. There was no intrahepatic biliary ductal dilatation reported at the time of initial presentation. Given her multiple co-morbidities, she was transferred to the university hospital for consideration of ERCP (endoscopic retrograde cholangiopancreatography) with biliary stenting. In view of the patient's history of lymphoma, the possibility of confluent periportal lymphadenopathy causing extrinsic biliary narrowing was raised, and an abdominal MRI and magnetic resonance cholangiopancreatography (MRCP) were performed (Siemens Magnetom Avanto 1.5 Tesla, Siemens Medical Solutions, Erlangen, Germany). The scans showed mild intrahepatic biliary dilatation. On axial and coronal T1- and T2-weighted images, a large elliptical flow void was noted at the porta hepatis which on post gadolinium dynamic sequences showed enhancement similar to adjacent venous structures [Figures and ]. Findings were consistent with an aneurysm of the extra hepatic portal vein. Incidentally noted was an early cirrhotic appearance of the liver with surface nodularity and splenomegaly related to portal hypertension (spleen craniocaudal dimension was 15 cm). No ascites was present. The portal vein aneurysm caused a smooth extrinsic mass effect upon the common hepatic duct at the porta hepatis with significant narrowing of the common bile duct (CBD) to a diameter of 3 mm.
Figure 1 An 89-year-old female with portal vein aneurysm. (a) Coronal Half Fourier Acquisition Single shot Turbo spin Echo (HASTE) image and (b) magnetic resonance cholangiopancreatography (MRCP) image show a 4.8 cm long smooth narrowing of the common bile duct (more ...)
Figure 2 An 89-year-old female with portal vein aneurysm. (a) Axial T2-weighted and (b) post gadolinium T1-weighted MR images show a large enhancing aneurysm of the main portal vein (enhancing to the same degree as the IVC (inferior vena cava) and splenic vein) (more ...)
Color Doppler and duplex US were performed at the request of the referring gastroenterology service to confirm the suspicion of a portal vein aneurysm compressing the common hepatic duct (CHD). Transverse color Doppler US images demonstrated a large aneurysm of the portal vein measuring 3.2 cm in diameter and extending over a length of 4.8 cm, with turbulent flow noted within the aneurysm . The aneurysm causes significant extrinsic compression upon the adjacent common hepatic duct (CHD) and common bile duct (CBD) with subsequent mild intrahepatic biliary dilatation, better appreciated on real time imaging. Marked splenomegaly was again noted. No ascites was present.
An 89-year-old female with portal vein aneurysm. (a) Color Doppler and (b) duplex US images show a large elliptical aneurysm of the main portal vein with turbulent to and fro flow (Ying-Yang sign) within the aneurysm.
Subsequent ERCP showed mild intrahepatic biliary ductal dilatation with a short segment of smooth significant narrowing of the CHD and proximal CBD secondary to extrinsic mass effect from the portal vein aneurysm . A sphinterotomy was performed and a biliary stent was placed with resolution of intrahepatic biliary ductal dilatation. There was slow normalization of elevated serum bilirubin (likely related to underlying myelofibrosis), liver enzymes, and overall improvement of patient's clinical symptoms over the next week. Total serum bilirubin was 0.6 mg/dl (normal 0.3 to 1.9 mg/dl) after 7 days of biliary stent placement. The other liver function tests were also normal.
Figure 4 An 89-year-old female with portal vein aneurysm. Endoscopic retrograde cholangiopancreatogram (ERCP) images (a) pre- and (b) post biliary stent placement show a smooth long segment narrowing of the common bile duct (arrow) secondary to the portal vein (more ...)