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+---
+title: "Autosomal Dominant Polycystic Liver Disease"
+docid: "c8256fa3-0694-412c-bd0f-8869f336ad90"
+authors:
+ - key: "6c5a9e0e-9dea-461b-9ad4-c00f5c4c2bbf"
+ value: "Atif Zaheer, MD, FSAR"
+ - key: "e987d3d3-1206-48d6-824b-3347c2968855"
+ value: "Michael P. Federle, MD, FACR"
+breadcrumbs:
+ -
+ name: "Gastrointestinal"
+ slug: "gastrointestinal"
+ treeNodeId: "992c2a4d-e0c4-4b82-be00-a05f5f19e3be"
+ -
+ name: "Diagnosis"
+ slug: "diagnosis"
+ treeNodeId: "65118aca-7db6-4b7c-becd-30be67d24e37"
+ -
+ name: "Liver"
+ slug: "liver"
+ treeNodeId: "3a84cf33-306e-40bb-9947-975013d43188"
+ -
+ name: "Congenital"
+ slug: "congenital"
+ treeNodeId: "63f9b1f0-3c9e-43cb-a4f0-ad122f645897"
+ -
+ name: "Autosomal Dominant Polycystic Liver Disease"
+ slug: "autosomal-dominant-polycystic-live-"
+ treeNodeId: null
+category: "Gastrointestinal"
+documentVersionId: "a1ad4728-f525-4085-bd90-704d6d97e19b"
+imageCount: 16
+lastUpdated: "04/21/25"
+pageDescription: "Autosomal Dominant Polycystic Liver Disease"
+pageKeywords: "Gastrointestinal, Diagnosis, Liver, Congenital, Autosomal Dominant Polycystic Liver Disease"
+pageTitle: "Autosomal Dominant Polycystic Liver Disease | STATdx"
+enhancedTitle: "Autosomal Dominant Polycystic Liver Disease"
+type: "DX"
+references: true
+ddx: true
+anatomy:
+ - "{'authors': 'Siva P. Raman, MD', 'bookmarked': False, 'bookmarkUrl': '/document/bookmark/e4fdb09c-d20f-407d-b563-499124541261', 'category': 'Gastrointestinal', 'compareUrl': '/compare/document/e4fdb09c-d20f-407d-b563-499124541261/related-anatomy/treeNode?subContext=Liver', 'documentId': 'e4fdb09c-d20f-407d-b563-499124541261', 'documentType': 'ANATOMY', 'documentUrl': '/document/liver/e4fdb09c-d20f-407d-b563-499124541261', 'enhancedTitle': 'Liver', 'entryDate': '06/27/23', 'imageCount': 114, 'imageUrl': '/image/thumbnail/da574c8c-5d46-46c2-ad9e-75150aa38a0a?size=174&quality=85', 'inCompareCart': False, 'rank': 1, 'referenceCount': 8, 'showCompareButton': False, 'title': 'Liver'}"
+ - "{'authors': 'Aya Kamaya, MD, FSRU, FSAR', 'bookmarked': False, 'bookmarkUrl': '/document/bookmark/236fb145-0c29-4362-be57-06401bf337aa', 'category': 'Ultrasound', 'compareUrl': '/compare/document/236fb145-0c29-4362-be57-06401bf337aa/related-anatomy/treeNode?subContext=Liver', 'documentId': '236fb145-0c29-4362-be57-06401bf337aa', 'documentType': 'ANATOMY', 'documentUrl': '/document/liver/236fb145-0c29-4362-be57-06401bf337aa', 'enhancedTitle': 'Liver', 'entryDate': '05/06/24', 'imageCount': 51, 'imageUrl': '/image/thumbnail/d66e35e7-05f2-4a4d-9ae4-6d913a04de28?size=174&quality=85', 'inCompareCart': False, 'rank': 2, 'referenceCount': 11, 'showCompareButton': False, 'title': 'Liver'}"
+cases: 2
+breadcrumbs:
+ - "Gastrointestinal"
+ - "Diagnosis"
+ - "Liver"
+ - "Congenital"
+ - "Autosomal Dominant Polycystic Liver Disease"
+---
+## KEY FACTS
+
+- ### Terminology
+
+
+ - Part of fibropolycystic liver (and renal) disease spectrum
+ - 2 forms of polycystic liver disease (PLD): Isolated PLD and PLD in association with polycystic kidney disease (PKD)
+- ### Imaging
+
+
+ - Extent of hepatic involvement ranges from scattered cysts to diffuse replacement of liver
+ - ± cysts in kidneys and other organs
+ - 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 autosomal dominant PLD
+ - 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 autosomal dominant PLD just by presence of numerous hepatic cysts
+ - Requires cysts in other organs, family history, or genetic testing
+
+## TERMINOLOGY
+
+- ### Abbreviations
+
+
+ - Autosomal dominant polycystic liver disease (ADPLD)
+- ### Synonyms
+
+
+ - Adult polycystic liver disease (PLD)
+- ### Definitions
+
+
+ - Uncommon inherited disorder
+ - 2 forms of PLD: Isolated PLD and PLD in association with polycystic kidney disease (PKD)
+ - Part of fibropolycystic liver disease spectrum
+ - Constitutes group of related lesions of liver and biliary tract caused by abnormal development of embryologic 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 greater than 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 (MRCP)
+ - 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
+- ### Nuclear Medicine Findings
+
+
+ - Tc-99m DISIDA scintigraphy permits differential diagnosis between Caroli disease and PLD
+ - Caroli disease
+ - Areas of focally ↑ radiotracer accumulation that persist > 120 minutes
+ - PLD
+ - Areas of focally ↓ radiotracer accumulation with normal liver washout and biliary excretion
+- ### Imaging Recommendations
+
+
+ - #### Best imaging tool
+
+
+ - CECT (renal function permitting) or MR
+ - MR is more sensitive for detection of complicated cysts
+
+## DIFFERENTIAL DIAGNOSIS
+
+- [Hepatic (Bile Duct) Cysts](/document/hepatic-cyst/e525e5da-75e8-432c-b398-cb3d18e97771)
+ - Not reliably distinguishable from ADPLD by imaging or histology
+ - Less likely to have hemorrhage
+ - Multiple cysts of different sizes
+- [Biliary Hamartoma](/document/biliary-hamartoma/1b525344-ad66-4381-9ca9-3fb11172744f)
+ - Usually numerous small lesions
+ - Typically < 15 mm in diameter
+ - Varied enhancement based on cystic/solid components of lesions
+ - Predominantly cystic (water density) lesions
+ - No enhancement of contents
+ - Predominantly solid (fibrous stroma) lesions
+ - Enhance and become isodense with liver parenchyma
+ - **Imparts echogenicity of smaller lesions**
+ - T2WI, MRCP: Markedly hyperintense cyst-like lesions, no communication with biliary tree
+- [Caroli Disease](/document/caroli-disease/acb4212f-766e-4766-bd10-1ebd525a467c)
+ - Congenital communicating cavernous ectasia of biliary tract
+ - Multiple small, rounded, saccular dilatations of intrahepatic bile ducts
+ - Central dot sign on CECT
+ - Enhancing portal radicles within cysts
+ - MRCP: Communicating bile duct abnormality
+- [Cystic Metastases](/document/hepatic-metastases-and-lymphoma/248db898-197a-4555-a14d-eef5ca2f8967)
+ - Often from sarcomas, especially gastrointestinal stromal tumor (GIST) after treatment
+ - Have internal debris or mural nodularity
+ - Rarely as numerous as in polycystic liver
+
+## PATHOLOGY
+
+- ### General Features
+
+
+ - #### Etiology
+
+
+ - Due to ductal plate malformation of small intrahepatic bile ducts
+ - Ducts lose communication with biliary tree
+ - #### Genetics
+
+
+ - Autosomal dominant
+ - Gene responsible for PLD
+ - *PRKCSH* (chromosome 19p) and *SEC63* genes
+ - #### Associated abnormalities
+
+
+ - Biliary hamartomas
+ - Congenital hepatic fibrosis
+ - Caroli disease
+ - Often coexists with autosomal dominant or recessive polycystic renal disease
+ - Hepatic cysts are pathologically identical to simple or bile duct cysts
+- ### Staging, Grading, & Classification
+
+
+ - Based on number and size of cysts and remaining liver parenchyma volume
+ - Gigot classification
+ - Schnelldorfer classification
+- ### Gross Pathologic & Surgical Features
+
+
+ - Progressive with massive enlargement and distortion of liver
+ - Liver surrounding cysts frequently contains biliary hamartomas and ↑ fibrous tissue
+- ### Microscopic Features
+
+
+ - Cuboidal and flat monolayer epithelium with no dysplasia in cyst walls
+
+## CLINICAL ISSUES
+
+- ### Presentation
+
+
+ - #### Most common signs/symptoms
+
+
+ - Dull abdominal pain
+ - Abdominal distention
+ - Dyspnea
+ - Cachexia; early satiety
+ - #### Clinical profile
+
+
+ - Other signs/symptoms
+ - Often causes massive hepatomegaly, compression of stomach
+ - Liver volume is often 5-10x higher than normal
+ - Compression of portal vein → portal hypertension
+ - Extrinsic compression of intrahepatic bile ducts
+ - Hepatic venous outflow obstruction due to compression by cysts
+ - Transudative ascites, portal hypertension due to distortion of portal venules by cysts and fibrosis
+ - Laboratory data: ADPLD uncommonly affects liver function
+ - Mild elevations of gamma-glutamyl transferase and alkaline phosphatase
+ - Patients with ADPKD: Renal functional abnormalities
+- ### Demographics
+
+
+ - #### Age
+
+
+ - Adult manifestation
+ - #### Sex
+
+
+ - M < F
+ - More common in women (60-75%) than men (40-60%)
+ - Women often have larger and more numerous cysts than men
+ - #### Epidemiology
+
+
+ - Isolated PLD is distinct genetic disease
+ - Less common than PLD associated with autosomal dominant PKD (ADPKD)
+ - Prevalence: Isolated PLD: 1-10 cases/1,000,000
+ - Variable degrees of fibrosis and cystic anomalies
+ - Hepatobiliary and renal anomalies frequently coexist in various combinations
+ - Suggests common underlying genetic abnormality
+ - ~ 1/2 of all patients with ADPKD have hepatic involvement
+ - ~ 70% of patients with PLD also have PKD
+- ### Natural History & Prognosis
+
+
+ - Liver progressively enlarges as it is replaced by cysts
+ - Isolated liver involvement does not lower life expectancy
+ - Complications: Spontaneous intracystic hemorrhage, rupture, infection
+ - Prognosis: Medical and surgical interventions have inconsistent long-term palliation
+ - Orthotopic liver transplantation has excellent long-term results
+ - Usually performed to relieve mass effect of liver on adjacent organs
+- ### Treatment
+
+
+ - Medical therapy
+ - Somatostatin analogues reduce cyst volume temporarily
+ - Mammalian target of rapamycin (mTOR), ursodeoxycholic acid, vasopressin-2 receptor antagonist
+ - Surgical therapy
+ - Simple unroofing, cyst fenestration alone, or fenestration combined with resection
+ - Percutaneous aspiration and alcohol sclerotherapy
+ - Combination of marsupialization and resection of dominant cysts
+ - Total hepatectomy and orthotopic liver transplantation for patients with severe ADPLD
+ - Combined liver and kidney transplantation if both organs are severely involved
+ - 1-year survival rate: 89% with excellent symptomatic relief and improved quality of life
+
+## DIAGNOSTIC CHECKLIST
+
+- ### Consider
+
+
+ - Isolated ADPLD is underdiagnosed and genetically distinct from PLD associated with ADPKD
+ - All other aspects of disease are similar
+- ### Image Interpretation Pearls
+
+
+ - Cannot diagnose ADPLD just by presence of numerous hepatic cysts
+ - Requires cysts in other organs, family history, or genetic testing
+
+ 6e192ea4-428d-407c-ab7d-8f7db7aabb7e
+
+## References
+
+## Selected References
+
+1. [Vicente TP et al: Liver transplantation for polycystic disease: experience and results of a single-center series. Transplant Proc. 57(1):48-51, 2025](http://www.ncbi.nlm.nih.gov/pubmed/?term=39788795%5Bpmid%5D)
+1. [Limaiem F et al: Polycystic liver disease: an uncommon genetic condition. Clin Case Rep. 12(5):e8892, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=38711839%5Bpmid%5D)
+1. [Zhang ZY et al: Polycystic liver disease: classification, diagnosis, treatment process, and clinical management. World J Hepatol. 12(3):72-83, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32231761%5Bpmid%5D)
+1. [Aussilhou B et al: Treatment of polycystic liver disease. Update on the management. J Visc Surg. 155(6):471-81, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30145049%5Bpmid%5D)
+1. [Lanktree MB et al: Prevalence estimates of polycystic kidney and liver disease by population sequencing. J Am Soc Nephrol. 29(10):2593-600, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30135240%5Bpmid%5D)
+1. [Mamone G et al: Hepatic morphology abnormalities: beyond cirrhosis. Abdom Radiol (NY). 43(7):1612-26, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29043403%5Bpmid%5D)
+1. [van Aerts RMM et al: Clinical management of polycystic liver disease. J Hepatol. 68(4):827-37, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29175241%5Bpmid%5D)
+1. [Kim JA et al: Pancreatic cysts in autosomal dominant polycystic kidney disease: prevalence and association with PKD2 gene mutations. Radiology. 280(3):762-70, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27046073%5Bpmid%5D)
+1. [Benzimra J et al: Hepatic cysts treated with percutaneous ethanol sclerotherapy: time to extend the indications to haemorrhagic cysts and polycystic liver disease. Eur Radiol. 24(5):1030-8, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24563160%5Bpmid%5D)
+1. [Abu-Wasel B et al: Pathophysiology, epidemiology, classification and treatment options for polycystic liver diseases. World J Gastroenterol. 19(35):5775-86, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=24124322%5Bpmid%5D)
+1. [Fedeles SV et al: A genetic interaction network of five genes for human polycystic kidney and liver diseases defines polycystin-1 as the central determinant of cyst formation. Nat Genet. 43(7):639-47, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21685914%5Bpmid%5D)
+1. [Gevers TJ et al: Somatostatin analogues for treatment of polycystic liver disease. Curr Opin Gastroenterol. 27(3):294-300, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21191289%5Bpmid%5D)
+1. [Venkatanarasimha N et al: Imaging features of ductal plate malformations in adults. Clin Radiol. 66(11):1086-93, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21840516%5Bpmid%5D)
+1. [Everson GT et al: Advances in management of polycystic liver disease. Expert Rev Gastroenterol Hepatol. 2(4):563-76, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=19072404%5Bpmid%5D)
+1. [Morgan DE et al: Polycystic liver disease: multimodality imaging for complications and transplant evaluation. Radiographics. 26(6):1655-68; quiz 1655, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=17102042%5Bpmid%5D)
+1. [Brancatelli G et al: Fibropolycystic liver disease: CT and MR imaging findings. Radiographics. 25(3):659-70, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=15888616%5Bpmid%5D)
+1. [Qian Q et al: Clinical profile of autosomal dominant polycystic liver disease. Hepatology. 37(1):164-71, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12500201%5Bpmid%5D)
+1. [Mortele KJ et al: Cystic focal liver lesions in the adult: differential CT and MR imaging features. Radiographics. 21(4):895-910, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11452064%5Bpmid%5D)
+1. [Steinberg ML et al: MRI and CT features of polycystic liver disease. N J Med. 90(5):398-400, 1993](http://www.ncbi.nlm.nih.gov/pubmed/?term=8506104%5Bpmid%5D)
+1. [Wan SK et al: Sonographic and computed tomographic features of polycystic disease of the liver. Gastrointest Radiol. 15(4):310-2, 1990](http://www.ncbi.nlm.nih.gov/pubmed/?term=2210203%5Bpmid%5D)
+1. [Wilcox DM et al: MR imaging of a hemorrhagic hepatic cyst in a patient with polycystic liver disease. J Comput Assist Tomogr. 9(1):183-5, 1985](http://www.ncbi.nlm.nih.gov/pubmed/?term=3881489%5Bpmid%5D)
+1. [Segal AJ et al: Computed tomography of adult polycystic disease. J Comput Assist Tomogr. 6(4):777-80, 1982](http://www.ncbi.nlm.nih.gov/pubmed/?term=7119198%5Bpmid%5D)
+
+## Differential diagnosis
+
+### Cystic Hepatic Mass
+DDX:85bb9a0b-2d25-457d-a131-20ff07cb552b
+
+### Dysmorphic Liver With Abnormal Bile Ducts
+DDX:1cb8d652-5e83-4ee2-a762-bab56e2d107b
+
+### Focal Liver Lesion With Hemorrhage
+DDX:5e6be44f-07d3-4569-abe3-798d7513fe41
+
+### Multiple Hypodense Liver Lesions
+DDX:5178ae9c-1ea9-4e06-9e8a-91e98d8708f6
+
+## Anatomy
+
+### Liver
+Gastrointestinal/ANATOMY:e4fdb09c-d20f-407d-b563-499124541261
+
+### Liver
+Ultrasound/ANATOMY:236fb145-0c29-4362-be57-06401bf337aa
+
+## Cases
+
+- {'cases': [{'authors': [{'key': '3d84d682-9451-4b02-99b2-e34970a5b440', 'value': 'Michael P. Federle, MD, FACR'}], 'caseVersionId': 'a96a5ff2-7908-45be-96de-ebcd711da86d', 'description': 'Typical MR case of extensive hepatic cysts of varying intensity due to repeated episodes of intracystic hemorrhage.\n\nT1WI GRE opposed phase images (#1-3) show numerous hepatic cysts of varying intensity. Some are nearly "black" (open arrow, #2, 3), as one would expect for simple fluid content, while others are of intermediate intensity, and at least one has septations and heterogeneous contents (arrow, #2, 3). The complex nature of the contents of some of the hepatic cysts is evident on the T2WI MR images (#4-6) as well.', 'history': 'Polycystic liver disease with episodes of abdominal pain and fever', 'imagePoolId': '112574a9-2d77-4aed-ab72-295651f7f638', 'name': 'Cysts of varying intensity due to hemorrhage', 'teachingPoint': None, 'demographics': '55 Years old male'}, {'authors': [{'key': '3d84d682-9451-4b02-99b2-e34970a5b440', 'value': 'Michael P. Federle, MD, FACR'}], 'caseVersionId': 'a9f45729-7864-4367-af95-63b4576245ee', 'description': 'Typical MR findings of polycystic liver disease.\n\nThere are innumerable cysts throughout the liver. Most have typical findings of water intensity on both T1 and T2WI MR, being very hypointense on T1WI and very bright on T2WI. At least one cyst (arrow, #1, 2), however, is of intermediate intensity on both in phase and opposed phase GRE MR T1WI, indicating complex fluid content. \n\nComment: The cysts in autosomal dominant polycystic disease of the liver (and kidney) often undergo spontaneous hemorrhage, usually into the cyst, but rarely extravisceral. T1WI GRE images are particularly well-suited to displaying complexity of cyst fluid.', 'history': 'Asymptomatic adult with family history of polycystic disease.', 'imagePoolId': 'b8338fe6-5936-4f8d-a1f6-04d429e53993', 'name': 'Extensive but asymptomatic involvement', 'teachingPoint': None}, {'authors': [{'key': '3d84d682-9451-4b02-99b2-e34970a5b440', 'value': 'Michael P. Federle, MD, FACR'}], 'caseVersionId': '604eb984-8636-46a1-9b19-b7cf33c2c379', 'description': 'Axial T1WI shows an enlarged liver with numerous large and small cysts of differing intensity. Those with high intensity fluid (arrows, #1,2) on the T1- and T2WI have blood products within the fluid. The cysts that are dark on T1WI and bright on T2WI (curved arrows, #1,2) have simple fluid within them. The kidneys are also enlarged and distorted by innumerable cysts with the same characteristics. Sagittal US shows a few of the hepatic cysts as sonolucent masses (arrows, #3).', 'history': 'Middle aged woman with failing kidneys and intermittent abdominal pain. ', 'imagePoolId': 'a7da59fa-5649-42eb-bc8c-8b7c98cd9db6', 'name': 'Polycystic liver & kidneys with old hemorrhage', 'teachingPoint': None, 'demographics': '51 Years old female'}, {'authors': [{'key': '3d84d682-9451-4b02-99b2-e34970a5b440', 'value': 'Michael P. Federle, MD, FACR'}], 'caseVersionId': '57faed90-4c93-442f-86cb-cfafccfbc9d1', 'description': 'Extensive replacement of liver shown on MR.\n\nNonenhanced T1WI (#1) and T2WI (#2-4) MR show extensive replacement of the liver with water intensity cysts. The massive enlargement of the liver by the cysts is even more evident on the T2WI coronal MR images (#3, 4). In spite of the extensive involvement of the liver, patients with autosomal dominant polycystic disease rarely have significant loss of hepatic function. When intervention is needed, it is usually because of intolerable pain from the massive hepatomegaly or mass effect on adjacent organs. If dominant cysts are present, these may be drained. Even orthotopic liver transplantation has been employed in severe cases.', 'history': 'Abdominal discomfort and early satiety with normal liver function.', 'imagePoolId': '001c6943-8cd1-4239-abdb-e05debf978bb', 'name': 'Extensive liver disease without liver dysfunction', 'teachingPoint': None}, {'authors': [{'key': '3d84d682-9451-4b02-99b2-e34970a5b440', 'value': 'Michael P. Federle, MD, FACR'}], 'caseVersionId': '87be736d-a564-47f5-9816-50505471995a', 'description': 'Typical CT findings, with dominant cysts compressing stomach.\n\nCECT shows innumerable water density cysts throughout the liver. Note the compression of the stomach by several large, exophytic cysts (arrow, #1, 2). There was no clinical or biochemical evidence of hepatic dysfunction. There were only a few small cysts in the kidneys and no renal dysfunction.\n\nComment: Because of the early satiety and abdominal discomfort, the patient had surgical decompression of several of the larger cysts with a reduction of symptoms.', 'history': 'Middle-aged man with known polycystic disease and early satiety and abdominal discomfort.', 'imagePoolId': '7f367467-4093-42d3-a8e2-c76405d2932e', 'name': 'Isolated involvement of liver with gastric compression', 'teachingPoint': None, 'demographics': '63 Years old male'}], 'caseType': 'typical', 'name': 'TYPICAL'}
+- {'cases': [{'authors': [{'key': '3d84d682-9451-4b02-99b2-e34970a5b440', 'value': 'Michael P. Federle, MD, FACR'}], 'caseVersionId': '7abbbdaf-4cfe-4e68-8f56-f62e731d9ca6', 'description': 'Extensive, but asymptomatic cysts found on CT for unrelated reason.\n\nCECT images (#1-6) show numerous hepatic cysts, some with a diameter in excess of 20 cm. Only a few small renal cysts are evident. The infra-renal aorta (open arrow, #6) is aneurysmally dilated; the left kidney (curved arrow, #6) is displaced into the pelvis by the enlarged liver; and there is end-stage hydronephrosis.\n\nComment: Had the extensive and individually enlarged hepatic cysts been discovered earlier in life, it would have been worth considering elective surgical decompression (usually by a process called marsupialization).', 'history': 'Elderly woman having CT evaluation for abdominal aortic aneurysm.', 'imagePoolId': '1f01926d-8d99-4d19-abd4-b6dd437f76b3', 'name': 'Dominant cysts in elderly asymptomatic woman', 'teachingPoint': None, 'demographics': '88 Years old female'}, {'authors': [{'key': '3d84d682-9451-4b02-99b2-e34970a5b440', 'value': 'Michael P. Federle, MD, FACR'}], 'caseVersionId': '3777ba4c-79f2-433b-bbbf-a4db5cb32e27', 'description': 'A series of axial (#1-5) and coronal (#6-7) NECT images show innumerable water attenuation cysts within the liver and kidneys (arrows, #1-7). Many of the renal cysts are of high attenuation (curved arrows, #2-6), indicative of intracyst hemorrhage with the acuity of hemorrhage felt to correlate to some extent with the degree of hyperdensity of cyst contents. At least 1 of the hyperdense cysts in the right kidney has ruptured, resulting in hemorrhage into the adjacent retroperitoneal spaces (open arrows, #5, 7).\n\t\nComment: Spontaneous hemorrhage may occur into renal cysts that are otherwise simple or those that develop in patients with ADPKD or acquired cystic disease of uremia. Any of these cysts may also rupture into the retroperitoneum and may rarely result in considerable blood loss.', 'history': 'Acute right flank pain in a patient with ADPKD.\n', 'imagePoolId': 'ba05368e-54d4-4ce2-8ad4-5b7f6b79adc7', 'name': 'ADPKD with hemorrhage', 'teachingPoint': None, 'demographics': '49 Years old male'}], 'caseType': 'variant', 'name': 'VARIANT'}
+
+
+## Images
+
+
+### Selected Images
+
+
+*Gross pathology photograph of a hepatectomy specimen shows numerous cysts replacing liver parenchyma. 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.*
+
+
+*Gross pathology photograph of a hepatectomy specimen shows numerous cysts replacing liver parenchyma. 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.*
+
+
and mixed signal due to hemorrhagic component 
. Note multiple liver cysts 
.](images/app.statdx.com_image_thumbnail_07246e10-5a1c-40c6-af23-57344515f46f_annotated_true_size_900_quality_90_d8dcf6b7612018bd739189512746a14f758aaa0f.jpg)
+*Coronal T2 MR shows polycystic kidney disease with multiple cysts in the kidneys with fluid signal (bright) and mixed signal due to hemorrhagic component . Note multiple liver cysts .*
+
+
by a dominant cyst 
from the left hepatic lobe, which was subsequently marsupialized at surgery with resolution of symptoms.](images/app.statdx.com_image_thumbnail_3850ee14-00dc-4a19-9ba5-a085ea2dbdd7_annotated_true_size_900_quality_90_16e69dc2678b2f380df92d20f97f0541353e5dfe.jpg)
+*Axial CECT shows typical findings of autosomal dominant 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.*
+
+
+*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.*
+
+
+*CECT in a middle-aged woman shows multiple large cysts in polycystic kidneys and liver . Women with polycystic liver disease (PLD) tend to develop larger cysts than men, attributed by some to a hormonal effect on cyst development.*
+
+
+*Coronal CT in a 36-year-old man with autosomal dominant polycystic kidney disease (ADPKD) shows associated hepatic cysts . Dilated left ventricle and pacer are from dilated cardiomyopathy. ADPKD is associated with ↑ prevalence of idiopathic dilated cardiomyopathy.*
+
+
+*Axial NECT shows innumerable, homogeneous and hypoattenuating cystic lesions with smooth, thin walls and an absence of internal structures. Some cysts have higher than water-density contents and others have peripheral calcification in cyst walls due to prior episodes of intracystic hemorrhage.*
+
+
+*Axial CECT shows a liver that is grossly enlarged and nearly replaced by innumerable cysts. Note also the involvement of both kidneys. Most patients with PLD also have ADPKD.*
+
+
+*Coronal T2 MR in a 60-year-old woman with PLD in association with PKD shows multiple hepatic , renal , and pancreatic cysts . The patient had PKD2 mutation, which has a higher incidence of pancreatic cysts and a later onset of renal failure compared to PKD1 mutation.*
+
+
+*Axial T2 MR in the same patient shows the complex nature of the contents of some of the hepatic cysts with uncomplicated cysts being homogeneously bright and complex cysts having heterogeneous contents .*
+
+
+### Additional Images
+
+
+*Axial CECT shows innumerable cysts on portal venous phase. These are uncomplicated cysts with no wall or content enhancement and an absence of internal debris/septations. Pancreatic cysts are also seen.*
+
+
+*Longitudinal ultrasound of the right upper quadrant demonstrates anechoic, multiple, hepatic cysts with smooth borders, thin walls, and no septations or mural nodularity in a patient with ADPLD.*
+
+
+*Axial CECT through the liver and kidneys shows lack of involvement of the kidneys in a patient with isolated ADPLD.*
+
+
+*Axial T2WI MR shows a hemorrhagic cyst in a patient with ADPLD as less hyperintense than its neighboring uncomplicated cyst .*
+
+
+*Axial T1WI GRE opposed-phase MR shows numerous hepatic cysts of varying intensity. Some are nearly black , as one would expect for simple fluid content, while others are of intermediate intensity. At least 1 has septations and heterogeneous contents from prior hemorrhage.*
+
+
+*Axial CECT shows numerous hepatic cysts, some > 20 cm in diameter. Only a few small renal cysts are evident. The right kidney was displaced caudally and was hydronephrotic due to compression of the renal pelvis (not shown).*
+
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