• Cyst contents often greater than water density/intensity due to hemorrhage (infection less common)
Calcification in cyst wall often seen due to old hemorrhage
TOP DIFFERENTIAL DIAGNOSES
• Hepatic (bile duct) cysts
Are not distinguishable reliably by imaging or histology from ADPLD
• Biliary hamartomas
Usually numerous and < 15 mm in diameter
• Caroli disease
• Cystic metastases
CLINICAL ISSUES
• Liver progressively enlarges as it is replaced by cysts
• Massive hepatomegaly, compression of stomach, bowel, lungs
• Dull abdominal pain, abdominal distention, dyspnea, cachexia, early satiety
• Liver function is uncommonly impaired
• Treatment: Alcohol ablation, resection or marsupialization of dominant cysts
Orthotopic liver transplantation has excellent long-term results
Usually performed to relieve mass effect of liver on adjacent organs
DIAGNOSTIC CHECKLIST
• Cannot diagnose AD polycystic liver disease just by presence of numerous hepatic cysts
Requires cysts in other organs, family history, or genetic testing
(Left) Gross pathology photograph of a hepatectomy specimen shows numerous cysts replacing liver parenchyma. The cysts ranged in size from microscopic to 5 cm in greatest dimension and contained clear fluid. This liver, which weighed 9 kg, was resected due to intractable patient discomfort and pressure on other organs.
(Right) Coronal T2WI MR shows innumerable high signal intensity cysts of varying size almost completely replacing hepatic parenchyma. Multiple cysts within the left kidney are also visible.
(Left) Axial CECT shows typical findings of AD polycystic liver disease (ADPLD) in a middle-aged man with early satiety. Note the compression of the stomach by a dominant cyst from the left hepatic lobe, which was subsequently marsupialized at surgery with resolution of symptoms.
(Right) Axial CECT in the same patient following surgical drainage of the left lobe cyst shows no residual compression of the stomach . The symptoms of early satiety also resolved.
TERMINOLOGY
Abbreviations
• Autosomal dominant polycystic liver disease (ADPLD) or adult PLD
Definitions
• Uncommon inherited disorder
• Part of fibropolycystic liver disease spectrum
Constitutes group of related lesions of liver and biliary tract caused by abnormal development of embryological ductal plate
IMAGING
General Features
• Best diagnostic clue
Multiple (> 20) cysts of varying size
• Location
Extent of hepatic involvement ranges from limited sporadic areas of cystic disease to diffuse involvement of all lobes of liver
– ± cysts in kidneys and other organs
• Size
Range from < 1 mm to > 12 cm
• Key concepts
Numerous large or small cysts coexist with fibrosis
– Round or oval shape
– Smooth, thin wall (if uncomplicated)
– Absence of internal structures (if uncomplicated)
Radiographic Findings
• ERCP
No communication with biliary tree
Cysts do not opacify
CT Findings
• NECT
Multiple to innumerable, homogeneous, and hypoattenuating cystic lesions
– Cyst contents > water density due to hemorrhage (infection less common)
– Calcification in cyst wall often seen (due to old hemorrhage)
• CECT
No wall or content enhancement
Cysts complicated by infection or hemorrhage may have septations &/or internal debris
– May also have enhancement of walls, but no enhancing nodules
Cysts may contain fluid levels
MR Findings
• T1WI
Uncomplicated cysts have very low signal intensity
– Higher signal in cysts with recent hemorrhage
• T2WI
Hetero- or homogeneous high signal intensity cysts
– Intracystic hemorrhage: Lower signal intensity
• T1WI C+
Nonenhancing after administration of gadolinium contrast material
• MR cholangiography (MRC)
No communication between cysts or with biliary tree
Coexisting Caroli disease will have cysts communicating with bile ducts
Ultrasonographic Findings
• Grayscale ultrasound
Anechoic masses with smooth borders, thin walls, and no septations or mural nodularity
Acoustic enhancement beyond each cyst
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within the left kidney are also visible.
by a dominant cyst 
from the left hepatic lobe, which was subsequently marsupialized at surgery with resolution of symptoms.
. The symptoms of early satiety also resolved.
