A 29 year old male presented to the Emergency Department with a one week history of painful jaundice, nausea, lethargy, fevers and rigors. He had latent tuberculosis but had otherwise been well and did not consume alcohol. Upon examination he was febrile, icteric and tender in the right upper quadrant with a temperature of 39 degrees Celsius. Liver function tests were abnormal as follows: bilirubin 212 mumol/L (RR 2-14 muumol/L), alkaline phosphatase 242 U/L (RR 40-130 U/L), alanine transaminase 75 U/L (RR 8-40 U/L), gamma glutamyl transferase 295 U/L (RR 0-50 U/L). International Normalised Ratio was 1.4. Full blood examination demonstrated anaemia with a haemoglobin of 119 g/L (RR 130-180 g/L) and thrombocytopenia with platelets 109 x 109/L (RR 150-400 x 109/L). Electrolytes were normal and serological tests for viral hepatitides, human immunodeficiency virus, schistosomiasis, strongyloides, malaria and fasciola were negative. An abdominal ultrasound showed gross biliary duct dilatation with sludge in the gallbladder. The portal vein was mildly dilated at 1.4 cm diameter but flow within the lumen was anterograde with no sign of portal venous obstruction or thrombosis.
A subsequent contrast-enhanced computed tomography (CT) scan of the abdomen showed dilatation of the portal vein in the region of the bifurcation, with collateral portal varices causing compression of the proximal common bile duct (CBD) (Fig. 1). A magnetic resonance cholangio-pancreatogram (MRCP) confirmed these findings and demonstrated intrahepatic bile duct dilatation with a beaded appearance, especially in the left lobe (Figs. 2 and 3). Splenic vein dilatation was also noted with concomitant splenomegaly, and the sludge previously visualised in the gallbladder was seen to extend into the CBD. A liver biopsy showed non specific changes consistent with biliary obstruction, periportal fibrosis and no cirrhosis.
This patient had cholangitis secondary to biliary tract obstruction. While biliary sludge likely contributed to the obstruction, this patient also exhibited radiological evidence of portal hypertension resulting in varicosed portal venous collaterals compressing adjacent intrahepatic and extrahepatic bile ducts:a rare phenomenon termed 'portal biliary ductopathy' (PBD). The cause of portal hypertension in this patient is believed to be idiopathic portal hypertension. He has undergone multiple placements of biliary stents via endoscopic retrograde cholangio-pancreatography (ERCP) since his initial presentation complicated by recurrent structuring, cholangitis and ERCP-related pancreatitis. Furthermore, as a result of portal hypertension, he has developed oesophageal varices necessitating endoscopic banding and internal haemorrhoids causing intermittent per-rectal bleeding. He is currently planned for insertion of a portosystemic shunt.