An unusual presentation of portal biliopathy manifesting as chronic abdominal pain as a delayed post-splenectomy complication: a case report.

Introduction and importance: Portal biliopathy is a rare biliary complication resulting from chronic extrahepatic portal vein obstruction and cavernous transformation, often presenting with obstructive jaundice or cholangitis. It is typically associated with longstanding portal hypertension and often develops insidiously. Case presentation: A 45-year-old woman with a history of Banti's syndrome treated by splenectomy presented with diffuse abdominal pain of 12 hours' duration. She reported intermittent abdominal pain over the previous 2 years, increasing in intensity and frequency. Laboratory workup revealed normal liver enzymes and bilirubin levels. Magnetic resonance cholangiopancreatography showed moderate intrahepatic biliary dilation with mild narrowing suggestive of external compression. Magnetic resonance imaging confirmed portal vein thrombosis with cavernous transformation in the porta hepatis compressing the common bile duct. These findings were consistent with portal biliopathy. A liver biopsy ruled out malignancy. Anticoagulation therapy was initiated with low molecular weight heparin followed by warfarin, then transitioned to rivaroxaban upon discharge. Clinical discussion: This report presents an unusual case of symptomatic portal biliopathy in a post-splenectomy patient, with a unique clinical presentation. This case is noteworthy for the absence of classic biliary symptoms and biochemical abnormalities despite significant anatomical changes. Unlike most cases in the literature, the patient's primary symptom was chronic abdominal pain without jaundice or cholangitis. The delayed onset of symptoms post-splenectomy and diagnosis through advanced imaging highlight the importance of considering portal biliopathy in similar clinical contexts. Conclusion: Early recognition and imaging-based diagnosis of portal biliopathy in post-splenectomy patients with vague symptoms are essential for preventing complications. This case adds to the growing spectrum of atypical presentations of portal biliopathy.