The Path of Least Resistance: A Rare Case of Enterocutaneous Fistula (ECF) Due to Pressure From Massive Polycystic Liver

Abstract

Introduction: Polycystic liver disease (PLD) is a condition where there are more than 20 cysts in the liver, causing hepatomegaly; presentation is often asymptomatic. We present a case of PLD exerting mass effect and resulting in an ECF. A 53-year-old with isolated PLD and ventral hernia repair 4 years prior presented for evaluation. Over the preceding year, he experienced increasing abdominal girth. Immediately prior to presentation, he observed feculent material coming from the surface of his abdominal wall. On presentation, CT of the abdomen showed innumerable liver cysts and an ECF between the small bowel and the abdominal wall. Bidirectional endoscopy revealed no GI malignancy, and barium enema confirmed ECF between the terminal ileum and abdominal wall. Other findings included coagulopathy, ascites, and encephalopathy. Complications of PLD include abdominal pain and distension. Advanced liver synthetic dysfunction and portal hypertension are rare. To our knowledge, the development of ECF from increased intra-abdominal pressure exerted by PLD has not been reported. We present an unusual case of PLD in which hepatomegaly and resultant mass effect in the abdominal compartment precipitated ECF formation.Figure 1: Barium enema: ECF.Figure 2: Caput medusa, ECF.Figure 3: Coronal CT: liver cysts.