Spontaneous Pylephlebitis of the Portal Vein Mimicking Acute Ascending Cholangitis: A Rare Case

Abstract

Pylephlebitis, a suppurative endophlebitis of the portal vein, is a rare but serious condition associated with a variety of underlying disease processes, most commonly intraabdominal pathologies like diverticulitis, appendicitis and inflammatory bowel disease. However, a spontaneous pylephlebitis mimicking acute cholangitis is rarely observed. A 60 year old man with a history of hypertension presented with right upper quadrant pain, jaundice and intermittent fevers for one month. He denied any history of venous thrombosis, malignancy, recent abdominal surgery or procedures. On exam he was febrile to 101.1 degrees F. He had tender hepatomegaly and icterus. Labs were remarkable for WBC of 17.8 x 10 cells per L and elevated liver enzymes (total bilirubin of 4.2 mg/dl, direct bilirubin 2.8 mg/dl, ALP 164 U/L, AST 64 U/L and ALT 44 U/L). On MRI and MRCP of the abdomen, he was found to have multiple hepatic abscesses with the largest measuring 11 mm, and right and left portal vein thrombosis. ERCP and hypercoagulable workup were unremarkable. The patient was diagnosed with spontaneous pylephlebitis complicating into multiple hepatic abscesses. No provoking etiology was established. The patient was started on intravenous piperacillin-tazobactam, then transitioned to amoxicillin-clavulanate for 6 weeks upon clinical remission, in addition to warfarin for six months. On post hospital appointment, the patient demonstrated clinical and biochemical improvement. The difficulty in managing pylephlebitis stems from its elusive diagnosis, as it presents with nonspecific symptoms such as fever, abdominal tenderness, and nausea. Pylephlebitis is a clinical and radiological diagnosis and in the setting of cholestatic liver enzymes with no precipitating intraabdominal source. It may even mimic acute ascending cholangitis. Charcot's triad is 95% specific for ascending cholangitis, however, Pylephlebitis may complicate into hepatic abscesses and present as classic Charcot's triad - pain, fever, and jaundice. Delay in the diagnosis of pylephlebitis may lead to complications like septic shock, disseminated intravascular coagulation (DIC) and bowel infarction secondary to extension of the thrombus into the superior mesenteric vein. Antibiotic therapy targeting gram negative aerobes and anaerobes is the mainstay of treatment. Additionally, anticoagulation has been associated with lower mortality, better recanalization and lower incidence of portal hypertension.2263 Figure 1. MRI abdomen showing right and left portal vein thromboses (green arrows) and multiple hepatic abscesses (red arrows).