Laparoscopic hepp couinaud for the treatment of caroli's disease: a case report / Hepp Couinaud laparoscópica para el tratamiento de la enfermedad de Caroli: reporte de caso

Caroli disease is a rare congenital disorder characterized by dilatation of intrahepatic bile ducts. The treatment could correspond to hepatic resection for localized disease. This paper describes a case of Caroli's disease in a 60 years old female patient, who presented jaundice and diffuse abdominal pain. Further examinations suggested the diagnosis of Caroli disease associated with cholestatic syndrome. We decided to perform a Hepp Couinaud biliodigestive bypass by video laparoscopy with Roux-en-Y reconstruction. There were no immediate complications and no longterm complications after 6 months of follow-up. The VLP biliodigestive bypass in patients with associated cholestatic syndromes should be considered a treatment prior to a more aggressive approach.

La enfermedad de Caroli es un raro trastorno congénito que consiste en una dilatación de las vías biliares intrahepáticas. Su tratamiento definitivo debe consistir en resección hepática por la enfermedad localizada. Este artículo describe un caso de enfermedad de Caroli en una paciente de sexo femenino de 60 años de edad, quien presentó ictericia y dolor abdominal difuso. Exámenes posteriores sugirieron el diagnóstico de enfermedad de Caroli asociada con síndrome colestásico. Los médicos decidieron realizar un bypass biliodigestivo de Hepp Couinaud mediante videolaparoscopia con reconstrucción en Y de Roux. No hubo complicaciones inmediatas ni complicaciones a largo plazo después de 6 meses de seguimiento. El bypass biliodigestivo VLP en pacientes con síndromes colestásicos asociados debe considerarse un tratamiento previo a un abordaje más agresivo.

An Excerpt of 2022 European Association for the Study of the Liver Clinical Practice Guidelines on the Management of Cystic Liver Diseases / 胃肠病学

The advent of enhanced radiological imaging techniques has facilitated the diagnosis of cystic liver lesions. Concomitantly, the evidence base supporting the management of these diseases has matured over the last decades. As a result, comprehensive clinical guidance on the subject matter is warranted. This guideline covers the diagnosis and management of hepatic cysts, mucinous cystic neoplasms of the liver, polycystic liver disease, caroli disease, caroli syndrome, biliary hamartomas and peribiliary cysts. On the basis of in⁃depth review of the relevant literature, this guideline provides recommendations to navigate clinical dilemmas followed by supporting text. The recommendations are graded according to the Oxford Centre for Evidence⁃based Medicine system and categorized as "weak" or "strong". This guideline aims to provide the best available evidence to aid the clinical decision⁃making process in the diagnosis and treatment of patients with cystic liver diseases, and presents the readers with translations and summarizations of the above mentioned recommendations.

An excerpt of 2022 European Association for the Study of the Liver clinical practice guidelines on the management of cystic liver diseases / 中华消化杂志

The advent of enhanced radiological imaging techniques has facilitated the diagnosis of cystic liver lesions. Concomitantly, the evidence base supporting the management of these diseases has matured over the last decades. As a result, comprehensive clinical guidance on the subject matter is warranted. This guideline covers the diagnosis and management of hepatic cysts, mucinous cystic neoplasms of the liver, biliary hamartomas, polycystic liver disease, caroli disease, caroli syndrome, biliary hamartomas and peribiliary cysts. On the basis of in-depth review of the relevant literature, this guideline provide recommendations to navigate clinical dilemmas followed by supporting text. The recommendations are graded according to the Oxford Centre for Evidence-Based Medicine system and categorized as "weak" or "strong" . This guideline aims to provide the best available evidence to aid the clinical decision-making process in the management of patients with cystic liver disease, and presents the readers with translations and summarizations of the above mentioned recommendations.

Progress in the diagnosis and treatment of Caroli disease / 国际外科学杂志

Caroli disease is a relatively rare genetic disease, also known as congenital intrahepatic cystic cholangiectasis, which is mainly manifested as non-obstructive segmental dilation of large, intrahepatic bile ducts, which is manifested as cysts in imaging and histopathological examination. The pathogenesis of Caroli disease is still unclear, and it is mainly believed to be related to PKHD1 gene mutation. Mutations in this gene often lead to autosomal recessive polycystic kidney disease (ARPKD), so Caroli disease is commonly associated with polycystic kidney disease. Caroli disease usually develops during adolescence and is characterized by recurrent cholangitis, which is diagnosed mainly by imaging. This article reviews the progress of diagnosis and treatment of Caroli disease by reading related literature.

Dilataciones quísticas congénitas de las vías biliares (primera parte). Vías biliares intrahepáticas / Congenital bile duct cystic dilatation (part 1). Intrahepatic bile ducts

RESUMEN La enfermedad de Caroli es una enfermedad infrecuente que requiere un alto índice de sospecha para su diagnóstico. Puede afectar un segmento hepático, un lóbulo o todo el hígado; suele generar episodios repetidos de colangitis. Existe una amplia gama de propuestas terapéuticas que oscilan desde el tratamiento médico hasta el trasplante de hígado. En este trabajo presentamos 3 casos, realizamos una revisión de la literatura y proponemos una ampliación de la clasificación de Alonso-Lej modificada por Todani que, a nuestra manera de ver, tiene implicaciones a la hora de seleccionar un tratamiento.

ABSTRACT Caroli's disease is a rare condition, and its diagnosis requires high level of suspicion. The disease may affect one segment, one lobe or the entire liver, and may result in repeated episodes of cholangitis. The disease can be managed using different therapeutic approaches ranging from medical treatment to liver transplantation. In this paper we report 3 cases with review of the literature and propose a modification of the classification by Alonso-Lej modified by Todani which we believe may be useful to guide treatment.

Enfermedad de Caroli: revisión sistemática de la literatura / Characterization of patients with Caroli's disease: Systematic review

Resumen Introducción: La enfermedad de Caroli es un trastorno congénito poco usual, el cual cursa con dilatación segmentaria multifocal de los conductos biliares intrahepáticos. Fue descrita por Jacques Caroli en 1958 como una dilatación sacular, segmentada o fusiforme de los ductos biliares intrahepáticos. Objetivo: Caracterizar a la población que ha padecido la enfermedad de Caroli. Metodología: Revisión sistemática de la literatura. Resultados: Se analizaron 66 artículos, el grupo de edad más afectado es los menores de 10 años, en el que se evidenció una mayor prevalencia en varones, y se encontró asociado con otras comorbilidades como el riñón poliquístico (20 %). La manifestación más frecuente fue la hepatomegalia (44,7 %), seguido de la fiebre (42,4 %) y el dolor abdominal en el hipocondrio derecho (41,2 %). Como método diagnóstico más utilizado se encontraba la resonancia magnética (73,8 %); dentro de los hallazgos predominó la dilatación intrahepática (76,5 %) y el manejo más empleado fue la antibioticoterapia para tratar las recurrencias por colangitis. Conclusión: La enfermedad de Caroli tiene una baja prevalencia, se presenta con mayor frecuencia en el continente americano, afecta principalmente a la primera década de la vida, tiene predilección por el sexo masculino y se caracteriza por una dilatación de los conductos intrahepáticos que pueden afectar a otros órganos como los riñones, lo que produce quistes renales.

Abstract Caroli's disease (CD) is a rare congenital disease, which presents with multifocal segmental intrahepatic bile duct dilatation. It was first described by Jacques Caroli in 1958 as a saccular or fusiform dilatation of the intrahepatic bile ducts. Objective: To characterize the population that has been diagnosed with Caroli's disease. Materials and methods: Systematic review. Results: 66 articles were analyzed. The age group most affected was children under 10 years old, where a higher prevalence was evidenced in males. This condition was associated with other comorbidities such as polycystic kidney in 20%. The most frequent manifestation was hepatomegaly (44.7%), followed by fever (42.4%), and abdominal pain in the right upper quadrant (41.2%). The most used diagnostic method was magnetic resonance imaging in 73.8% of the sample. The findings showed predominance of intra-hepatic dilatation in 76.5%. The most widely used treatment was antibiotic therapy to treat recurrences due to cholangitis. Conclusion: Caroli's disease has an extremely low incidence and occurs more frequently in the American continent, affecting mainly patients in the first decade of life, with a predilection for the male sex. It is characterized by a dilatation of the intrahepatic ducts that can affect other organs such as the kidneys, causing renal cysts.

Enfermedad de Caroli: a propósito de un caso / Carolis disease: report of one case

Resumen La enfermedad de Caroli es una rara patología caracterizada por la existencia de dilataciones saculares segmentarias del árbol biliar intrahepático, que, por lo general, afectan a todo el parénquima hepático, aunque es poco frecuente el compromiso biliar extrahepático. Presentamos el caso de un varón que ingresó con un cuadro clínico de 45 días de evolución, consistente en ictericia colestásica, baja ponderal y prurito. La colangiorresonancia informó múltiples dilataciones saculares en las vías biliares intrahepáticas.

Abstract Caroli disease is a condition characterized by cystic dilatation of the intrahepatic biliary tree that usually affects the entire liver parenchyma, although extrahepatic biliary involvement is rare. The following is the case of a male patient who was admitted due to cholestatic jaundice, low weight, and pruritus for 45 days. Magnetic resonance cholangiopancreatography reported multiple cystic dilatations in the intrahepatic bile ducts.

Clinical features of Caroli disease: An analysis of 41 cases / 临床肝胆病杂志

ObjectiveTo investigate the clinical features of patients with Caroli disease. MethodsThe clinical data were collected from 41 patients who were diagnosed with Caroli disease in The Fifth Medical Center of Chinese PLA General Hospital from April 2015 to January 2020, and the patients were divided into type I group with 16 patients and type Ⅱ group with 25 patients. A retrospective analysis was performed for general information, laboratory markers, and clinical features. The independent samples t-test was used for comparison of normally distributed continuous data between groups, and the Mann-Whitney U test was used for comparison of non-normally distributed continuous data between groups; the chi-square test was used for comparison of categorical data; a Spearman correlation analysis was also performed. ResultsThe type Ⅰ group had a significantly higher level of albumin (Alb) than the type Ⅱ group (t=0.976, P=0.048), and the type Ⅱ group had a significantly higher prothrombin time (PT) than the type I group (Z=3.115, P=0.001). Compared with the type I group, the type Ⅱ group had significantly higher incidence rates of esophageal and gastric varices, upper gastrointestinal bleeding and/or tarry stool, and portal hypertension (χ2=6.077, 5.468, and 2.403, P=0.002, 0.019, and 0.028). In the patients with type Ⅱ Caroli disease, the level of cholinesterase was negatively correlated with the incidence rates of esophageal and gastric varices and portal hypertension (r=-0.468 and -0.436, P=0.018 and 0.029); Alb level was negatively correlated with the incidence rate of esophageal and gastric varices (r=-0.561, P=0.004); red blood cell count was negatively correlated with the incidence rates of esophageal and gastric varices, upper gastrointestinal bleeding and/or tarry stool, and portal hypertension (r=-0.662, -0.566, and -0.436, P＜0.001, P=0.003, and P=0.029); hemoglobin count was negatively correlated with the incidence rates of esophageal and gastric varices, upper gastrointestinal bleeding and/or tarry stool, and portal hypertension (r=-0.605, -0.590, and -0.510, P=0.001, 0.002, and 0.009); PT was positively correlated with the incidence rates of esophageal varices and portal hypertension (r=0.488 and 0.520, P=0.013 and 0.008). ConclusionCompared with the patients with type I Caroli disease, the patients with type Ⅱ Caroli disease have a higher incidence rates of esophageal and gastric varices, upper gastrointestinal bleeding and/or tarry stool, and portal hypertension, with the changes in clinical indicators such as the decrease of Alb level and the increase of PT level, and they tend to have poor prognosis.

Caroli syndrome in full brothers caused by new mutation of PKHD1 gene / 中华普通外科杂志

Objective@#To explore the new gene mutation and clinical manifestations of Caroli syndrome in 2 blood brother cases.@*Methods@#The clinical data of the 2 brothers with clinical diagnosis of Caroli syndrome were analyzed, and the related genes were detected by second-generation sequencing technology.@*Results@#The PKHD1 gene of both brothers had the mutation of c. 5059A>T(exon32). c. 4358(exon 32)delC shift mutation, neither of which has been reported in OMIM database.@*Conclusion@#After analysis, c. 4358(exon32) delC and c. 5059A>T(exon32) may be the new pathogenic mutation of this disease, with the same mutant genotype but completely different phenotype.

Clinicopathological features of Caroli’s disease: An analysis of 5 cases / 临床肝胆病杂志

@#ObjectiveTo investigate the clinical features, pathological features, differential diagnosis, and prognosis of Caroli’s disease (CD). MethodsA retrospective analysis was performed for the clinicopathological data of 5 parents with CD who were treated in Beijing Tsinghua Chang Gung Hospital from January 2014 to May 2019, and a literature review was also performed. ResultsThere were 3 male and 2 female patients, with a mean age of 20 years. All of them attended the hospital due to the presence of intermittent upper abdominal pain, and among these patients, 4 had pyrexia, 2 had splenomegaly and portal hypertension, 1 had gallstones, 4 had bilateral renal cysts, and 4 had abnormal hepatic function. Imaging examination showed varying degrees of cystic dilatation of the intrahepatic bile ducts which communicated with the biliary tree, and fibrous bands were observed in these cysts. Pathological examination showed intrahepatic bile duct dilatation with fibrous tissue proliferation and chronic inflammatory cell infiltration on the bile duct wall, and fibrovascular bundles were observed in the bile duct cavity. In addition, fibrosis and proliferation of the portal area and abnormal small bile ducts were observed in type II CD. ConclusionCD is often seen in children and adolescents, and its clinical manifestations lack specificity. A confirmed diagnosis of CD relies on imaging and pathological examinations. Histopathological features of this disease include cystic dilatation of the intrahepatic bile ducts communicating with the biliary tree and the bands consisting of fibrous tissue and vessels in these cysts.

Caroli syndrome in full brothers caused by new mutation of PKHD1 gene / 中华普通外科杂志

Objective To explore the new gene mutation and clinical manifestations of Caroli syndrome in 2 blood brother cases.Methods The clinical data of the 2 brothers with clinical diagnosis of Caroli syndrome were analyzed,and the related genes were detected by second-generation sequencing technology.Results The PKHD1 gene of both brothers had the mutation of c.5059A ＞ T (exon32).c.4358 (exon 32)delC shift mutation,neither of which has been reported in OMIM database.Conclusion After analysis,c.4358 (exon32) delC and c.5059A ＞ T(exon32) may be the new pathogenic mutation of this disease,with the same mutant genotype but completely different phenotype.

Laparoscopic hepatectomy for the treatment of Caroli's disease: a case report

Caroli disease is a rare congenital disorder characterized by nonobstructive dilatation of intrahepatic ducts. In cases with symptomatic intrahepatic manifestations, treatment should correspond to the type with hepatic resection for localized disease and transplantation for diffuse forms. If possible, complete resection of the cysts can cure the symptoms and avoid the risk of malignancy. A 66-year-old woman presented to Wuxi Xishan People's Hospital with recurrent intermittent upper quadrant abdominal pain. Further examinations suggested the diagnosis of Caroli disease limited to the left hepatic lobe. She underwent laparoscopic hepatectomy. Pathological examination confirmed the diagnosis of Caroli disease, and no malignancy was found. There were no immediate complications and no long-term complications after one and one-half years of follow-up. Laparoscopic hepatectomy could be a feasible, safe treatment option for localized Caroli disease.

Mechanism of PP242 inducing autophagy and apoptosis in cholangiocytes of polycystic kidney rats / 中国药理学通报

Aim To investigate the molecular mecha-nism of mTORC1/2 inhibitor PP242, which inhibiting cholangiocyte cell preliferation and cystic diliatation via inducing apoptosis and autophagy in the polycystic kid-ney ( PCK ) rats. Methods The expression of p-mTOR and p-Akt in the bile duct epithelial cells was examined by immunohistochemistry. The inhibiting effect of rapamycin and PP242 on cell proliferation ac-tivity on bile duct epithelial cells, the effect of gene si-lence on LC3, Beclin-1 and the effect of the authoph-agy-specific inhibitor 3-methyladenine (3-MA) on cell proliferation were respectively analyzed by WST-1 as-say. The expression of PI3K/Akt signaling pathway re-lated proteins, autophagy-related proteins LC3, Bec-lin-1 and clevead caspase-3, which were treated by PP242 were determined by Western blot. The effect of PP242 on apoptosis was detected by Annexin V/PI double staining and ELISA. The expression of LC3 in cytoplasm was detected by immunofluorescence. The a-bility of rat bile duct epithelial cells spheroid formation was detected by 3D cell culture method, and the cells were treated by single applied with rapamycin and ap- plied rapamycin combined with Rictor gene silencing respectively. Results The protein levels of p-Akt and p-mTOR markedly increased in the bile duct epitheli-um of PCK rats. PP242 inhibited the proliferation of bile duct epithelial cells more effectively than rapamy-cin and showed a dose-and time-dependent manner ( P<0.05 ) . PP242 significantly reduced the levels of PI3K/Akt signaling pathway-related proteins in PCK rat cholangiocytes. PP242 induced apoptosis and auto-phagy, up-regulated the levels of cleaved caspase-3, Beclin-1 and increased the ratio of LC3-II/LC3-I. The combination of Rictor gene silencing and rapamycin was more effective than rapamycin alone in inhibiting cholangiocytes in PCK rats. The inhibitory effect of PP242 on the cell viability was significantly weakened by treatment with 3-MA and knockdown of LC3 and Beclin-1 ( P <0.05 ) . Conclusions PP242 inhibits the proliferation of PCK rat cholangiocytes through PI3K/Akt/mTOR signaling pathway, and the mecha-nism is closely related with autophagy and apoptosis.

Diffuse-Type Caroli Disease with Characteristic Central Dot Sign Complicated by Multiple Intrahepatic and Common Bile Duct Stones

Caroli disease (CD) is a rare congenital malformation of the liver characterized by non-obstructive, segmental, cystic dilatation of the intrahepatic bile ducts (IHDs). The clinical course is usually asymptomatic for the first 5–20 years, and symptoms may seldom occur throughout the patient’s life. Bile stagnation leads to recurrent episodes of cholangitis, stone formation, or liver abscesses, and biliary cirrhosis usually occurs years later. Here we report on a 42-year-old man diagnosed with diffuse-type CD with a characteristic central dot sign, who had multiple intrahepatic and common bile duct (CBD) stones. CBD stones were treated successfully with endoscopic retrograde cholangiopancreatography (ERCP).

Clinical features of congenital hepatic ifbrosis in children / 临床儿科杂志

Objective To explore the clinical features of congenital hepatic fibrosis (CHF) in children.MethodsA total of 111 patients diagnosed with CHF during January 2002 to June 2015 were included and divided into children group and adult group according to the age at diagnosis. The clinical data including gender, age, clinical symptoms, signs, laboratory examinations, and imaging characteristics were retrospectively analyzed and compared between two groups.ResultsThere were high incidences of splenomegaly and liver cirrhosis in both children and adult groups, and no difference was found between two groups (P>?0.05). The fatigue, jaundice, and hepatosplenomegaly were more common in children group while the renal cyst and liver cyst were more common in adult group, and the differences were statistically signiifcant (P all?

Doenças císticas das vias biliares / Cystic diseases of the bile ducts

JUSTIFICATIVA E OBJETIVOS: Os ductos biliares podemapresentar dilatações congênitas ou adquiridas. As lesões congênitaspodem ser hipoplásticas (atresias) e císticas. O primeiroestudo descrito na literatura foi realizado por Vater and Ezler in1723. Os cistos biliares são isolados ou múltiplos, e aparecemem qualquer idade, mas 75% deles são antes dos 20 anos. Elespodem ocorrer em toda árvore biliar, sendo, as intra-hepáticasrestritas, conhecida como doença de Caroli. O objetivo desseestudo foi fazer uma revisão sobre as Doenças císticas das viasbiliares. CONTEÚDO: As dilatações císticas extra-hepáticaatingem diversos tamanhos e são classificadas segundo sua formae localização. São elas: fusiforme, divertículos e dilataçõessaculares. Háuma incidência variada dessas lesões com câncer,entre 2,5% e 17,5%. O quadro clínico mais comum éa colestaseneonatal, correspondendo a 80% dos pacientes. Na idadeadulta, podem ser assintomáticos ou apresentar apenas sintomasinespecíficos como náuseas,vômitos. A dificuldade de drenagemda colestase obstrutiva pode lavar a cirrose biliar secundária.CONCLUSÃO: O ultrassom de abdome pode confirmar odiagnóstico. No tocante ao laboratório, observa-se uma hiperbilirrubinemiae elevação de enzimas canaliculares. A terapêuticaempregada na maioria dos casos écirúrgica, com excisão do cistoe reconstrução da drenagem biliar, devendo-se evitar a drenagemisolada, pois tais pacientes tendem a evoluir com complicaçõescomo fístulas, estenose, colangite e carcinoma.(AU)

BACKGROUND AND OBJECTIVES: The bile duct dilatationmay have congenital or acquired. Congenital lesions can behipoplásticas (atresia) and cystic. The first study described in theliterature was performed by Vater and Ezler in 1723. Biliary cystsare isolated or multiple and appear at any age, but 75% of themare before age 20. They can occur throughout the biliary tree,and the intrahepatic restricted, known as Caroli disease. Theaim of this study was to review about the cystic biliary diseases.CONTENTS: Dilated cystic extrahepatic reach various sizesand are classified according to their shape and location. Theyare: fusiform, saccular dilatation and diverticula. There is anincidence of these lesions with cancer varied between 2.5% and17.5%. The most common symptom is a neonatal cholestasis,corresponding to 80% of patients. In adulthood, may beasymptomatic or show only nonspecific symptoms such as nausea,vomiting. The difficulty of draining obstructive cholestasis canwash the secondary biliary cirrhosis. CONCLUSION: Theabdominal ultrasound can confirm the diagnosis. Regarding thelaboratory, there is a hyperbilirubinemia and elevated GGT. Thetherapy used in most cases is surgical excision of the cyst andreconstruction of biliary drainage, should be avoided drainagealone because these patients tend to develop complications suchas fistula, stricture, cholangitis and carcinoma.(AU)

Enfermedad de Caroli: Presentación de un caso / Caroli's Disease: Presentation of a case

La enfermedad de Caroli es una condición congénita poco frecuente, cuya incidencia se estima en 1 caso por millón. Se caracteriza por dilataciones saculares o fusiformes de las vías biliares. Entre las manifestaciones clínicas se encuentran dolor en hipocondrio derecho, fiebre e ictericia, estas crisis de colangitis secundarias a estasis biliar, y sepsis. Su diagnóstico se basa en el hallazgo de lesiones quísticas en árbol biliar, pueden observarse por ecografía, tomografía computarizada. Presentamos a paciente femenino de 37 años, acude a emergencia de Medicina Interna del Hospital Escuela, de la ciudad deTegucigalpa, Honduras, manifestando signos y síntomas de colangitis aguda e ictericia. Al examen físico presenta abdomen globoso y doloroso a expensas de una hepatomegalia marcada. Nuestra paciente presento episodios de colangitis recurrente. La tomografía computarizada revela múltiples espacios quísticos en ambos lóbulos hepáticos y se encontró lesiones quísticas en el árbol biliar. Excluyendo, colangitis esclerosante, quiste hidatídico, quiste del colédoco y dilatación ductal hereditaria, y confirmando la sospecha diagnostica de enfermedad de Caroli.

Caroli disease is a rare congenital condition, which incidence is estimated at 1 case per million. Characterized by saccular or fusiform dilation of the bile. Clinical manifestations include right upper quadrant pain, fever and jaundice, these crisis of cholangitis are secondary to biliary stasis, and sepsis. Diagnosis is based on the discovery of cystic lesions in biliary tree, it may be seen by ultrasound, computed tomography. We present a case of female patient with 37-year-old, who attends to the emergency service of internal medicine of the Hospital Escuela Universitario of the city ofTegucigalpa, Honduras, manifesting signs and symptoms of acute cholangitis and jaundice. Physical examination reveals globose and painful abdomen at the expense of a marked he-patomegaly. Our patient presented episodes of recurrent cholangitis, the computed tomography reveals multiple cystic spaces in both lobes and was found lesions in the biliary tree. Excluding, sclerosing cholangitis, hydatid cyst, bile duct and hereditary ductal dilatation, and confirmed the suspected diagnosis of Caroli's disease.

Caroli's disease misdiagnosed as intraductal papillary neoplasm of the bile duct

Caroli's disease is a rare autosomal-recessive disorder caused by malformation of the ductal plate during embryonic development. Although it is present at birth, Caroli's disease is typically not diagnosed until between the second and fourth decades of life, as it was in the present patient. Here we report a rare case of Caroli's disease limited to one liver segment, which was initially misdiagnosed as an intraductal papillary neoplasm of the bile duct. The asymptomatic patient was treated with liver segmentectomy.

Monosegmental Hepatobiliary Fibropolycystic Disease Mimicking a Mass: Report of Three Cases

Hepatobiliary fibropolycystic diseases are a unique group of entities involving the liver and biliary tract, which are caused by abnormal embryologic development of the ductal plates at various stages. We experienced strange hepatobiliary fibropolycystic diseases with a complex mass composed of malformed ducts and biliary cysts, which did not belong to, and were different from, previously known malformations. They were unique in imaging and histologic features. We herein report three cases of monosegmental hepatobiliary fibropolycystic disease mimicking a mass.