Significance of total liver volume on hepatic symptoms and complications

Abstract

Autosomal dominant polycystic kidney disease (ADPKD) is the most prevalent potentially lethal monogenic disorder. It is characterized by the development of renal cysts and various extra-renal manifestations (1, 2). Although the most common extra-renal manifestation is hepatic cysts (3), morbidity in ADPKD patients is primarily determined by the severity of the underlying renal cystic disease and loss of renal function. Furthermore, most patients with hepatic cysts are thought to be asymptomatic (4). Therefore, physicians generally do not consider hepatic cysts as significant as polycystic kidney disease in ADPKD patients. The prevalence of hepatic cyst involvement ranges from 70% to -94% detected by MRI studies (2, 3). The risk factors for severe hepatic cysts include advanced age, female sex, pregnancy, exogenous use of female steroid hormones, the degree of renal cystic disease, and the severity of renal dysfunction (5). The reported symptoms and signs of hepatic cysts include abdominal pain, dyspnea, early satiety, gastro-esophageal reflux, nausea, vomiting, and mechanical lower back pain (4, 6). The complications of hepatic cysts include ascites, cyst hemorrhage, cyst infection, and compression of adjacent structures such as hepatic veins (Budd-Chiari syndrome), IVC, portal veins or bile ducts. These symptoms and complications may be related to a progressive increase in the size and number of liver cysts. Therefore, disease management has been focusesd on decreasing the liver volume (6, 7). However, determining which patients are affected, the optimal treatment timing, and effective interventions for hepatic cysts are unresolved issues. Although some treatment guidelines for hepatic cysts have been proposed (8), specific data required to resolve the aforementioned issues are limited. Furthermore, the associations of total liver volume with the symptoms and complications of hepatic cysts are poorly understood. Moreover, there is no standard definition of severe liver disease. Therefore, this study aimed to elucidate the significance of height-adjusted total liver volume (htTLV) in pressure-related symptoms and complications in ADPKD. In particular, htTLV was measured by using a 3-D reconstruction method. The distribution of htTLV, as well as inter- and intra-familial variations in Korean ADPKD patients were also investigated. In addition, the associations of htTLV with hepatic symptoms and complications were elucidated.