AD Polycystic Liver Disease

Published on 18/07/2015 by admin

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Last modified 18/07/2015

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 ± cysts in kidneys and other organs

• Cyst contents often greater than water density/intensity due to hemorrhage (infection less common)

image Calcification in cyst wall often seen due to old hemorrhage

TOP DIFFERENTIAL DIAGNOSES

• Hepatic (bile duct) cysts

image Are not distinguishable reliably by imaging or histology from ADPLD
• Biliary hamartomas

image 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

image Orthotopic liver transplantation has excellent long-term results
image 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

image Requires cysts in other organs, family history, or genetic testing
image
(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.

image
(Right) Coronal T2WI MR shows innumerable high signal intensity cysts of varying size almost completely replacing hepatic parenchyma. Multiple cysts image within the left kidney are also visible.
image
(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 image by a dominant cyst image from the left hepatic lobe, which was subsequently marsupialized at surgery with resolution of symptoms.

image
(Right) Axial CECT in the same patient following surgical drainage of the left lobe cyst shows no residual compression of the stomach image. 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

image Constitutes group of related lesions of liver and biliary tract caused by abnormal development of embryological ductal plate

IMAGING

General Features

• Best diagnostic clue

image Multiple (> 20) cysts of varying size
• Location

image 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

image Range from < 1 mm to > 12 cm
• Key concepts

image 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

image No communication with biliary tree
image Cysts do not opacify

CT Findings

• NECT

image 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

image No wall or content enhancement
image Cysts complicated by infection or hemorrhage may have septations &/or internal debris

– May also have enhancement of walls, but no enhancing nodules
image Cysts may contain fluid levels

MR Findings

• T1WI

image Uncomplicated cysts have very low signal intensity

– Higher signal in cysts with recent hemorrhage
• T2WI

image Hetero- or homogeneous high signal intensity cysts

– Intracystic hemorrhage: Lower signal intensity
• T1WI C+

image Nonenhancing after administration of gadolinium contrast material
• MR cholangiography (MRC)

image No communication between cysts or with biliary tree
image Coexisting Caroli disease will have cysts communicating with bile ducts

Ultrasonographic Findings

• Grayscale ultrasound

image Anechoic masses with smooth borders, thin walls, and no septations or mural nodularity
image Acoustic enhancement beyond each cyst
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