Pathophysiology, epidemiology, classification and treatment options for polycystic liver diseases

Abstract

Polycystic liver diseases (PLD) represent a group of genetic disorders in which cysts occur in the liver (autosomal dominant polycystic liver disease) or in combination with cysts in the kidneys (autosomal dominant polycystic kidney disease). Regardless of the genetic mutations, the natural history of these disorders is alike. The natural history of PLD is characterized by a continuous increase in the volume and the number of cysts. Both genders are affected; however, women have a higher prevalence. Most patients with PLD are asymptomatic and can be managed conservatively. Severe symptoms can affect 20% of patients who develop massive hepatomegaly with compression of the surrounding organs. Rrarely, patients with PLD suffer from acute complications caused by the torsion of hepatic cysts, intraluminal cystic hemorrhage and infections. The most common methods for the diagnosis of PLD are cross sectional imaging studies. Abdominal ultrasound and computerized tomography are the two most frequently used investigations. Magnetic resonance imaging is more sensitive and specific, and it is a valuable test for patients with intravenous contrast allergies or renal dysfunction. Different treatment modalities are available to physicians caring for these patients. Medical treatment has been ineffective. Percutaneous sclerotherapy, trans-arterial embolization, cyst fenestration, hepatic resection and liver transplantation are indicated to specific groups of patients and have to be tailored according to the extent of disease. This review outlines the current knowledge of the pathophysiology, clinical course, diagnosis and treatment strategies of PLD.

Utilization of Distilled Water Lavage for Localized Fluid Collection After Combined Hepatectomy and Cyst Fenestration for Polycystic Liver Disease

“…15 ) We first planned to place an intraoperative prophylactic drain because ascites necessitating persistent drainage or paracentesis is a common early postoperative complication after CHCF for PLD. [5][6][7] However, fenestration is expected to cause the intracystic fluid outflow into the peritoneal cavity to be absorbed through the peritoneum. Namely, indiscreet drain placement around the liver might isolate the outflow of intracystic fluid and impair the effect of fenestration due to narrowed peritoneal surface exposed to effluent through fenestration orifices.…”

“…However, its effectiveness seemed limited and thus prolonged drainage was usually required. 1,2,[5][6][7] We present a case of massive localized noninfectious fluid collection after CHCF for a patient with PLD, which was relieved by distilled water lavage (DWL). Furthermore, total liver volume (TLV), including an area where massive localized fluid collection treated with DWL existed, continued to reduce up to 12 postoperative months and thereafter retained less than one-third of preoperative volume more than 2 years after surgery.…”

“…[5][6][7] It has been reportedly observed in 20% to 70% cases and seemed to easily cause recurrent symptoms unless properly treated. [5][6][7] Diuretics has been often used for this condition. However, its effectiveness seemed limited and thus prolonged drainage was usually required.…”

“…[1][2][3][4] Thus, considerably invasive procedures, including combined hepatectomy and cyst fenestration (CHCF) and even hepatic replacement, are necessary despite its benign nature. [5][6][7] Ascites necessitating persistent drainage or paracentesis after drain removal is found among early postoperative complications after CHCF for PLD. [5][6][7] It has been reportedly observed in 20% to 70% cases and seemed to easily cause recurrent symptoms unless properly treated.…”

“…[5][6][7] Ascites necessitating persistent drainage or paracentesis after drain removal is found among early postoperative complications after CHCF for PLD. [5][6][7] It has been reportedly observed in 20% to 70% cases and seemed to easily cause recurrent symptoms unless properly treated. [5][6][7] Diuretics has been often used for this condition.…”