Abstract
Background: Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited kidney disease in humans. It is a multisystemic disorder characterized by progressive cystic dilatation of both kidneys, with variable extrarenal manifestations in the gastrointestinal tract (hepatic cysts), cardiovascular system, reproductive organs and brain. An important issue of patients with hepatorenal cystic disease is the fact that complications can arise due to the growing of the cysts: local kidney complications (intracystic infection, intracystic hemorrhage) and local liver complications (portal hypertension as a result of portal vein compression by cysts, bile duct compression, ruptures and bleedings of the cysts, obstruction of the liver veins).
Objective: The main purpose of our case presentation is to emphasize the fact that ADPKD can be an aggressive disease with multiple complications, which requires an early diagnosis in order to properly avoid possible complications.
Material and Method: We present the case of a 61 years old male, known with polycystic hepatorenal disease, chronic kidney disease (CKD) in chronic hemodialysis since 2010, renal hypertension, hypertensive cardiomyopathy, renal anemia and stage 1 chronic lymphatic leukemia. The patient was diagnosed with hepatorenal polycystic disease 20 years ago and 9 years ago he started undergoing renal replacement therapy by hemodialysis. Three months ago, the patient presented a suddenly installed ascitic syndrome for which an MRI was performed. The imagistic investigation revealed multiple kidney and liver cysts, with secondary compression of the portal vein and a tumoral mass that may suggest an adenocarcinoma.
Results: The investigations performed confirm the diagnosis of portal hypertension secondary to compression due to cystic formations.
Conclusions: Hepatorenal polycystic disease has numerous clinical variations, so it needs to be followed in a multidisciplinary way with rapid therapeutic measures to prevent complications. Further investigations are needed when dealing with suspicious cystic formations with an uncertain substrate.
Extracardiac compression of right atrium by hepatic cysts in a patient with polycystic kidney disease
