Polycystic Liver Disease

A form of hepatobiliary fibropolycystic disease characterized by an overgrowth of biliary epithelium and supportive connective tissue. Multiple cysts are present in the liver parenchyma. The condition is usually associated with polycystic kidney disease and congenital biliary duct disease.

PCLD.

Of patients referred for ultrasonographic examination of the abdomen, 2 to 5% have hepatic cysts, of which fewer than one-third are multiple with one to three cysts. Hepatic cysts are exceptionally rare in children; frequency increases from 0% in first two decades of life to 7% in the eighth decade of life.

Autosomal dominant. Polycystic liver disease occurs either alone or in association with autosomal dominant polycystic kidney disease.

Alterations in the development and differentiation of bile ducts and intralobular bile ductules with development of biliary microhamartomas and cysts. Cystic dilatation of peribiliary glands around the large intrahepatic bile ducts may also give rise to the cysts.

Magnetic resonance imaging may be the most sensitive method to differentiate a complicated cyst from an uncomplicated one.

Despite the great differences in the number and size of the cysts, the volume of the noncystic liver parenchyma remains normal; therefore, most patients with polycystic liver disease are asymptomatic and have normal liver function tests. Symptoms may be caused by complicated hepatic cysts, by the mass effect of one or several cysts, or by a massively enlarged liver. Hemorrhage or rupture of the hepatic cysts are rare complications that present as an acute abdomen. Infection of hepatic cyst may be indistinguishable from acute cholecystitis. Percutaneous drainage of the infected cyst is usually effective and safe and surgical drainage is reserved for patients with relapses. Mass effects of large cysts include symptoms related to the increased intraabdominal pressure, obstruction of the hepatic veins, inferior vena cava, and obstruction of the bile ducts. When indicated, surgical treatment for polycystic liver disease is directed at reducing the volume of the cystic tissue. Cystic aspiration followed by instillation of a sclerosing agent may be performed for symptomatic cysts that are strategically located. Superficial cysts may be treated by laparoscopic fenestration. Combined hepatic resection and fenestration have met with limited success. In those patients incapacitated by severe hepatomegaly secondary to massive cystic replacement without areas of parenchymal sparing, liver transplantation is a therapeutic option.

Concomitant renal evaluation is mandatory to exclude polycystic kidney disease. Complex hemostatic abnormalities may be present. H2 antagonists can be given to reduce the risk of gastrointestinal bleeding and to reduce the risk of aspiration of gastric contents from the delay of gastric motility and hyperacidity. Investigations: Complete cell blood count, coagulation studies, ECG, chest radiograph, electrolytes, urea, and creatinine levels. Check for the presence of portal hypertension and ascites.

Passage of nasogastric tubes should be done with care in patients with esophageal varices. If coagulation function is compromised, particular care should be paid to insertion of invasive lines and intubation. Regional techniques are worth considering if there is no coagulopathy. Otherwise, intubation and controlled ventilation should be employed, especially in patients with ascites or pleural effusion, and to minimize the risk for pulmonary aspiration. Patients with significant hepatic impairment and hyperbilirubinemia (>180 mmol/L) are at higher risk for renal failure; these patients should be adequately hydrated (guided by central venous pressure if necessary), and maintenance of diuresis before, during, and after operation with fluid loading, mannitol, or furosemide. Partial hepatectomy is usually associated with massive blood loss, with subsequent massive blood transfusion and its attendant complications. Close monitoring of blood loss is necessary, as is the need to maintain adequate venous access, normothermia, electrolytes, and acid-base balance.

Choice of anesthetic drugs according to the presence and importance of liver fibrosis, portal hypertension, cirrhosis, and/or renal insufficiency. Isoflurane has the least effect on the liver circulation.

Caroli Disease and Caroli Syndrome: Congenital cystic dilatation of the intrahepatic biliary tree associated with periportal fibrosis, portal hypertension, and, eventually, liver failure. Associated with polycystic kidneys in 40 to 50% of cases.