Noncirrhotic Portal Hypertension and Diagnostic Pitfalls in Severe Polycystic Liver Disease: A Case Report

Polycystic liver disease (PLD) is a rare inherited disorder characterized by progressive hepatic cyst formation and, in severe cases, noncirrhotic portal hypertension.Although ascites is a recognized complication of advanced PLD, diagnostic frameworks for ascites and spontaneous bacterial peritonitis (SBP) were developed primarily in patients with cirrhosis and may not be directly applicable to noncirrhotic disease.We present the case of a 60-year-old male with severe PLD who developed recurrent massive ascites following hepatic cyst surgery.Imaging demonstrated extensive hepatic cystic disease with portal vein compression and hepatic venous outflow obstruction.Ascitic fluid demonstrated a high serum-ascites albumin gradient and elevated protein, consistent with noncirrhotic portal hypertension.Despite a polymorphonuclear leukocyte (PMN) count of only 16 cells/mm³, broth-only growth of three oropharyngeal commensal organisms prompted treatment for presumed SBP; subsequent negative cultures and the absence of neutrocytic ascites favored contamination or transient bacterascites.Point-of-care ultrasound (POCUS) facilitated rapid identification of free fluid despite extensive cystic hepatic anatomy and enabled safe paracentesis by identifying appropriate fluid pockets.This case highlights the importance of contextualizing cirrhosisderived diagnostic frameworks when evaluating noncirrhotic ascites and demonstrates the value of POCUS as a practical diagnostic and procedural tool in patients with severe PLD.