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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.
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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.
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Autosomal dominant. Polycystic liver disease
occurs either alone or in association with autosomal dominant polycystic
kidney disease.
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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.
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Magnetic resonance imaging may be the most sensitive
method to differentiate a complicated cyst from an uncomplicated one.
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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.
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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.
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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 ...