Polycystic liver disease: Hepatic venous outflow obstruction lesions of the noncystic parenchyma have major consequences.

In patients with polycystic liver disease (PLD), development of cysts induces hepatic venous outflow obstruction (HVOO) and parenchymal modifications, challenging the paradigm of a normal noncystic liver parenchyma. The aims were to reappraise the pathology of the noncystic parenchyma, by focusing on HVOO lesions; and to investigate the association with outflow obstruction at imaging and perioperative course after liver resection. This is a retrospective study conducted in one tertiary center between 1993 and 2014. PLD patients (n = 125) who underwent resection (n = 90) or transplantation (n = 35) were included. HVOO parenchymal lesions were assessed for all patients and a liver congestion score was built. Imaging was analysed for 45 patients with computed tomography scan, and perioperative course was assessed in resected patients. At pathology, 92% of patients had HVOO lesions, with sinusoidal dilatation being the most common feature. HVOO was more severe in patients who underwent transplantation compared to liver resection, as assessed by the congestion score. At imaging, all patients had HVOO with at least two hepatic veins involved. Mosaic enhancement pattern of the parenchyma was associated with the severity of hepatic vein obstruction (P = 0.045) and the compression of the inferior vena cava (P = 0.014). In case of liver resection, intraoperative course was characterized by hemorrhage, related to HVOO at imaging. Ascites (44%) and liver failure (9%) in the postoperative period were associated with blood losses and transfusions.

CONCLUSION: Hepatic venous outflow obstruction, including development of venous collaterality and parenchymal changes, is frequent in PLD and has major consequences on intraoperative bleeding and postoperative ascites and liver failure. Hepatic venous outflow obstruction should be taken into account to choose the most appropriate surgical treatment. (Hepatology 2017).