Peribiliary Cysts Research Articles

Peribiliary cysts: uncommon mimickers of hepatic and biliary cystic lesions.

Peribiliary cysts (PC) are dilatations of the extramural peribiliary glands, with a very characteristic imaging pattern in the contexts of hepatobiliary diseases, idiopathic portal hypertension, adult-type polycystic disease of the liver and kidneys, solitary nonparasitic cysts, and systemic infections. The clinical relevance of PC is related to the fact that their presence may indicate underlying pathologies (such as those mentioned above) and may be considered as a potential marker of liver disease progression. Although imaging findings are quite characteristic, recognizing their main differential diagnoses, including malignancies, can be challenging but are essential to avoiding diagnostic errors.

Peribiliary cysts masquerading as choledocholithiasis.

Video 1The major papilla was cannulated with a sphincterotome over a straight wire. The polypoid saccular structures were identified by cholangioscopy.

Introduction to the recommendations from the European Association for the Study of the Liver clinical practice guidelines on the management of cystic liver disease

The diagnosis of cystic liver disease has made great progress with the advent of enhanced imaging techniques. At the same time, its management has gradually improved over the past few decades, providing the basis for the development of appropriate diagnostic and treatment guidelines. To this end, the European Association for the Study of the Liver has developed clinical guidelines for the diagnosis and treatment of non-infectious cystic liver disease. This guideline put forward recommendations based on an in-depth review of the relevant literature for addressing clinical issues, including the diagnosis and treament of hepatic cysts, hepatic mucocystic tumors, biliary hamartomas, polycystic liver disease, Caroli disease or Caroli syndrome, biliary hamartomas, and peribiliary cyst.

Detection of Multiple Hepatic Peribiliary Cysts in Captive Lions (Panthera leo)

Background: Hepatic lesions in non-domestic felids are poorly characterized. The present study reports multiple hepatic peribiliary cysts in 9 out of 32 lions (Panthera leo) at Bannerghatta Biological Park, Bengaluru. Methods: The animals were presented for necropsy after the history of clinical signs of lethargy, anorexia, weakness, debilitated body conditions and all animals are in old age (between 15 to 25 years old). Systematic necropsy with gross and histopathological examination was carried out. Result: Post-mortem examination grossly revealed multiple cysts in liver (varying in each case) with the cystic masses filled with clear fluid, sometimes mixed with bile. A diagnosis for the presence of spontaneous peribiliary cysts was made on the basis of gross and microscopic lesions. Histologically, the cysts were surrounded and composed of compact collagenous tissue. The inner cystic single layer resembled biliary mucosa and composed of columnar epithelial lining, while the submucosa was composed of connective tissue with loss of hepatic cord structure.

A Case of Resection of Hepatic Peribiliary Cysts with Repeated Cholangitis Due to Bile Duct Stenosis

A Case of Resection of Hepatic Peribiliary Cysts with Repeated Cholangitis Due to Bile Duct Stenosis

Peribiliary Cysts Mimicking Primary Sclerosing Cholangitis and Cholangiocarcinoma

Peribiliary cysts are cystic dilatations resulting from obstruction of extramural peribiliary glands. They are usually benign, may arise from the intrahepatic and extrahepatic bile ducts, and do not communicate with the biliary tree. They may, however, be confused with alternative diagnoses, resulting in unnecessary investigations with profound clinical implications. We present a case of a 42-year-old male, in whom this entity was misdiagnosed as primary sclerosing cholangitis with cholangiocarcinoma.

Re-Recognizing the Cellular Origin of the Primary Epithelial Tumors of the Liver.

The primary epithelial tumors of the liver (PETL) are composed of a series of heterogeneous tumors. Although the classification of PETLs has been updated several times by the World Health Organization, the cellular origins of some tumors in this family remain to be precisely depicted. In addition, certain tumors in different categories have similar histology, molecular phenotypes and biological characteristics, suggesting that they may have the same cellular origin. In this work, a narrative review method was adopted to review the relevant papers. By comparing the expression profiles of biomarkers of liver epithelium at different lineages and stages of differentiation, the cells-of-origin of some major members of the PETL family were reassessed. We propose that 1) hepatic adenomas, hepatocellular carcinomas (HCCs) and pure fetal hepatoblastomas (HBs) share the same spectrum in their cellular origin including the hepatocytic-committed progenitors (HCP) and their differentiated descendants. 2) Bile duct adenomas, peribiliary cysts and intrahepatic cholangiocellular carcinomas (ICCs) can share the same spectrum in their cellular origin including the cholangiocytic-committed progenitors (CCP) and their differentiated descendants. 3) The cells-of-origin of embryonal HBs include liver stem cells (LSCs), hepatoblasts, and transitional cells between them. Embryonal HB with small cell element, small cell undifferentiated HB and small cell neuroendocrine carcinoma of the liver can have the same or similar cells-of-origin from LSC. Embryonal HB lacking the small cell component of the LSC phenotype and presenting both hepatocytic and bile duct/ductule components may originate from actual hepatoblasts/hepatic progenitor cells (HPCs) as the combined HCC-ICC does. 4) Teratoid hepatoblastoma and mixed epithelial/mesenchymal HBs can be derived from the LSCs or even less committed extrahepatic pluripotent stem cell. 5) Many members of the PETLs family, including those derived from LSCs, hepatoblasts/HPCs, early HCPs and CCPs, have neuroendocrine potentiality. Except for those primary hepatic neuroendocrine tumor (PHNET) exhibit hepatocytic and/or cholangiocytic phenotypes, other PHNETs subtype may be derived from the descendants of LSC that differentiate towards the upper digestive tract, pancreas or other lineages.

Peribiliary cysts: diagnostic features on endoscopic ultrasound and digital cholangioscopy.

Peribiliary cysts: diagnostic features on endoscopic ultrasound and digital cholangioscopy.

Obstructive Jaundice Secondary to Peribiliary Cyst Within the Common Hepatic Duct.

Obstructive Jaundice Secondary to Peribiliary Cyst Within the Common Hepatic Duct.

S2367 Push Enteroscopy-Assisted ERCP for Treatment of Biliary Obstruction and Cholangitis in a Patient With Kasai Porto-Enterostomy

INTRODUCTION: Biliary obstruction and acute cholangitis are late complications in patients with Kasai porto-enterostomy performed for biliary atresia. Patients typically present with fevers and jaundice and can progress to liver failure. Only a few cases have reported successful endoscopic treatment for biliary obstruction in adults with a previous Kasai procedure. Here we describe the case of a 34-year-old female with Kasai porto-enterostomy with recurrent cholangitis. A successful enteroscopy-assisted ERCP was performed with resolution of symptoms. CASE DESCRIPTION/METHODS: A 34-year-old female with a history of biliary atresia status post Kasai procedure done shortly after birth presented with intermittent fevers, refractory pruritus, and recent hospitalization for acute cholangitis. Her workup revealed elevated liver tests and serum bile acids. MRCP showed right intrahepatic bile duct (RHD) dilatation with peribiliary cysts, trace left intrahepatic bile duct (LHD) dilatation and focal stricture at the porto-enterostomy anastomosis. We performed an enteroscopy-assisted ERCP with the patient in supine position. A pediatric colonoscope with a clear cap was used to access the biliary limb. After applying external abdominal pressure, the porto-enterostomy was reached and showed a severely stenosed opening of the bile ducts (Figure 1). The RHD was deeply cannulated using a 0.025” hydrophilic-tipped guidewire. Cholangiogram showed dilated RHD up to 10mm with severe stricture at the porto-enterostomy (Figure 2). The stricture was dilated with a balloon dilator first to 3mm then 4mm. A 5Fr x 4cm plastic stent with a single internal flange and single external pigtail was placed into the RHD. Cannulation of the LHD was unsuccessful due to complete obstruction of the ducts. A submucosal ink injection was used to mark the biliary limb. The patient was discharged home the same day. Both her fever and pruritus resolved and she has not had further episodes of cholangitis. Her liver tests improved markedly (Table 1). On repeat ERCP, the prior stent was exchanged for a 7Fr x 5cm stent. Liver enzymes normalized. No late complications occurred. DISCUSSION: Enteroscopy-assisted ERCP is a feasible treatment for biliary obstruction in patients with Kasai porto-enterostomy. Altered anatomy and limited choice of tools make interventions challenging. This procedure should be considered as an alternative to more invasive percutaneous and surgical techniques and as part of a multidisciplinary approach.Table 1.: Serum Bile Acids, Hepatic Panel, Complete blood count, C-reactive Protein prior to ERCP vs. 4 and 12 weeks post ERCP with stent placementFigure 1.: Severely stenosed bile ducts (black arrow).Figure 2.: Cholangiogram with dilated right intrahepatic bile duct.

Peribiliary glands pathology in a large series of end-stage alcohol-related liver disease.

The peribiliary glands are intramural or extramural structures with multiple functions related to bile secretion. The peribiliary glands can develop cystic alterations in several conditions, such as alcohol addiction. Peribiliary cysts can enlarge till being radiologically visible and mimic cancer. We studied 217 consecutive explanted livers for end-stage alcohol-related liver disease from the Pathology Unit of the Liver Unit at the King's College Hospital in Denmark Hill, with particular focus on peribiliary glands. Our cohort consisted of 31 females and 186 males, with a median age of 51 and of 56years respectively. 92,2% had established cirrhosis, 73,3% had only alcohol-induced liver disease, whilst 26,7% had other co-morbidities. We found a mild ectasia of the peribiliary glands (<2mm) in 37,8% and peribiliary cysts (> = 2mm) in 22,6% of cases. The diameter of the peribiliary glands varied from 1mm to 8mm. Inflammation of the peribiliary glands was found in the majority of cases with dilatation (p value = 0,000). 4,6% of the peribiliary cysts had low-grade intraductal papillary neoplasm of the bile ducts confined to the peribiliary glands. Pancreatic heterotopia was found in 10,6% and associated with the presence of ectasia. Our findings fit with what is reported in literature, such as the alcohol-induced damage at the peribiliary glands. Moreover these results underline the possible role of peribiliary glands in the development of the intraductal papillary neoplasm of the bile ducts, the biliary counterpart of the branch type intraductal mucinous neoplasm of the pancreas.

Specific liver volume parameters in cirrhotic patients with peribiliary cysts

The presence of peribiliary cysts and morphological changes in the volumes of lobes in the livers of patients with cirrhosis are both related to the alteration of portal flow. Our study explored the relationship between these two features in patients with cirrhosis. We retrospectively selected 150 computed tomography (CT) images of cirrhotic livers and 105 CT images of healthy livers. The cirrhotic livers were further classified according to the presence of peribiliary cysts (peribiliary cysts group) or the absence of peribiliary cysts (control cirrhotic group). The characteristic features of liver cirrhosis, including modified caudate-right lobe ratio (mCR ratio), splenomegaly, ascites, and collateral shunts, were reviewed. Liver volume calculations included the sum of the left hepatic volume (LHV) and right hepatic volume (RHV; LHV + RHV) and the ratio of LHV to RHV (LHV/RHV). The two groups did not differ in the presence of splenomegaly, ascites, or collateral shunts. The control cirrhotic group exhibited a significantly higher mCR ratio and LHV/RHV ratio than the peribiliary cysts group did (p < 0.001). The healthy liver group exhibited a significantly higher LHV + RHV value than either the peribiliary cysts group or the control cirrhotic group did (p < 0.001). The peribiliary cysts group and the control cirrhotic group did not differ significantly for LHV + RHV (p > 0.05). The control cirrhotic group exhibited a significantly higher mCR ratio and LHV/RHV ratio than the peribiliary cysts group did, but the two groups were similar for most measurements. Peribiliary cysts might result in reduction of portal flow, causing cirrhotic liver with peribiliary cysts with left-sided dominance not to demonstrate the typical morphological appearance of the common cirrhotic liver.

A Case of Hepatic Peribiliary Cysts with Hepatic Hilar Bile Duct Stenosis due to Enlargement after Total Gastrectomy

A Case of Hepatic Peribiliary Cysts with Hepatic Hilar Bile Duct Stenosis due to Enlargement after Total Gastrectomy

1451 A Caroli Look-Alike

INTRODUCTION: Peribiliary cysts are a rare entity usually found in patients with chronic liver disease and polycystic kidney disease. Patients are mostly asymptomatic therefore it is identified at times incidentally by abdominal imaging. Differential diagnosis include cholangiocarcinoma, Caroli disease and primary biliary cirrhosis. Awareness is essential to avoid misdiagnosis and unnecessary procedures including biopsies and surgeries. CASE DESCRIPTION/METHODS: A 67-year-old female with cirrhosis secondary to NASH and portal hypertension, among other comorbidities was evaluated at GI clinics. Abdominal sonogram performed for hepatocellular carcinoma surveillance showed right and left intrahepatic biliary ductal dilatation. The common bile duct measured 0.35 cm and the gallbladder showed intraluminar posterior acoustic shadows suggestive of cholelithiasis without wall thickening or pericholecystitic fluid collections. Patient denied fever, chills, abdominal pain, nausea or vomits. Abdomen was benign. Laboratories remarkable for baseline thrombocytopenia of 71, normal ALT and AST, elevation of alkaline phosphatase to 171 and hyperbilirubinemia of 3. MRCP was performed for further evaluation which showed multiple hyperintense rounded lesions tracking along the intrahepatic and extrahepatic biliary as seen with peribiliary cysts. DISCUSSION: Peribiliary cyst are usually associated to alcoholic cirrhosis with a prevalence of 9%; while in they have not been described in NASH. Pathophysiology may be due to intrahepatic circulatory disturbances of the portal venous system. Possible complications include biliary tract dilatation, obstructive jaundice and cholangitis.

2232 Peribiliary Cysts Mimicking Primary Sclerosing Cholangitis and Cholangiocarcinoma

INTRODUCTION: Peribiliary cysts are cystic dilatations resulting from obstruction of extramural peribiliary glands. They are usually benign, may arise from the intrahepatic and extrahepatic bile ducts and do not communicate with the biliary tree. They may, however, be confused with alternative diagnoses, resulting in unnecessary investigations with profound clinical implications. CASE DESCRIPTION/METHODS: A 42-year-old male was referred after recent diagnosis of cirrhosis, presumed secondary to primary sclerosing cholangitis (PSC), complicated by suspected cholangiocarcinoma. He presented with jaundice, weight loss and was noted to have nodular, cirrhotic liver with diffusely beaded appearance of the intra-hepatic biliary tree and central hilar prominence suggestive of mass lesion on outside imaging studies. ERCP was also interpreted as PSC with suspected cholangiocarcinoma. Brushing was negative for malignancy. Repeat MRI with MRCP showed multiple grape-like cystic structures surrounding the intrahepatic portal vein branches, resulting in compression of the intrahepatic bile ducts (Figure 1). These findings were consistent with peribiliary cysts. He subsequently underwent deceased donor liver transplantation. Gross examination of the explant revealed multiple benign bile duct cysts, which were focally multi-cystic with an exophytic semitranslucent cystic lesion in the hilum, which likely explains the prior imaging misdiagnosis as cholangiocarcinoma (Figure 2). Histologically, multiple cystic biliary structures were noted, lined by non-dysplastic cuboidal biliary type epithelium (Figure 3). DISCUSSION: Originally described in 1984, Peribiliary cysts are usually asymptomatic and often under- and misdiagnosed. The clinical importance of these structures arises from the fact that they may at times cluster together resembling multi-locular malignancy and mimic a dilated biliary tree. They may consequently be mistaken for entities such as biliary intraductal papillary mucinous neoplasm, cystic metastases, PSC, Caroli’s disease and importantly cholangiocarcinoma. In addition, they may also occasionally grow to compress the neighboring structures, resulting in biliary obstruction. High-resolution imaging with MRCP is a good modality for establishing the diagnosis. As highlighted by our case, given the prevalence of these lesions in patients with cirrhosis, awareness of this entity is critical to avoid misdiagnosis leading to inappropriate exclusion from transplantation in otherwise acceptable candidates.

Three Cases of Peribiliary Cysts in Feeder Pigs

胆管周囲囊胞が肥育豚3頭に認められた．大小の囊胞は症例1では外側左葉のみに，症例2及び症例3では主として外側左葉に形成され，囊胞は左葉に偏って形成されていた．3症例ともに，囊胞は漿液性の液体を貯留していた．症例3では，左右腎臓にも囊胞が認められた．3症例ともに，肝臓の病理組織学的所見は共通していた．囊胞は門脈域に主座していた．囊胞は増生した結合組織によって取り囲まれ，近傍には大型胆管が散見された．囊胞では，一層の上皮細胞で内張りされ，内分泌細胞が介在していた．3症例ともに胆管炎が観察され，このうち2症例では，リゾチーム／ムラミダーゼ陽性細胞数が増加していた．症例3の右腎臓では，囊胞間には糸球体や尿細管を含む腎実質がわずかに残存していた．症例3は胆管周囲囊胞に多発性腎囊胞を併発した最初の症例報告であった．

Sa1437 - Peribiliary Cysts: A Systematic Review and Proposal of a Classification Framework

Sa1437 - Peribiliary Cysts: A Systematic Review and Proposal of a Classification Framework

Peribiliary Cysts Presenting as Primary Sclerosing Cholangitis

Peribiliary Cysts Presenting as Primary Sclerosing Cholangitis

繰り返す胆管炎を来したhepatic peribiliary cystsの1切除例

繰り返す胆管炎を来したhepatic peribiliary cystsの1切除例

Focal lesions in cirrhosis: Not always HCC

Even though most hepatocellular carcinomas (HCC) develop in the setting of cirrhosis, numerous other focal liver lesions and pseudolesions may be encountered. The role of the radiologist is therefore to differentiate these lesions from HCC to avoid under- and overdiagnosis. There are several ways of classifying these lesions: those which predate the development of fibrosis and cirrhosis (cystic lesions, hemangioma), those related to or a consequence of cirrhosis (regenerative nodules, dysplastic nodules, focal fibrosis, peribiliary cysts, shunts, or even cholangiocarcinoma), and those related to the underlying cause of chronic liver disease (lymphoma). Finally, some may develop independently (liver metastases). From an imaging point of view, it is important to remember that the imaging features of pre-existing lesions are not dramatically changed by cirrhosis. Differentiating non-HCC from HCC requires not only an understanding of the multi-step process of hepatocarcinogenesis, but also the importance of medical history, and of complimentary imaging modalities, namely computed tomography (CT) and magnetic resonance imaging (MRI). This review article gives an overview of the imaging features of benign and malignant non-HCC focal liver lesions in the setting of cirrhosis, with a focus on CT and MR imaging.