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.
随着增强影像技术的出现，囊性肝病的诊断有了很大进展。与此同时，囊性肝病的管理在过去几十年中逐渐完善，为制定相应诊断和治疗指南提供了依据。对此，欧洲肝病学会为非传染性囊性肝病制定了临床诊疗指南。该指南在深入回顾相关文献的基础上，提出了应对临床问题的推荐意见，涵盖肝囊肿、肝脏黏液囊性肿瘤、胆道错构瘤、多囊性肝病、Caroli病、Caroli综合征、胆道错构瘤和胆周囊肿的诊断和治疗。.

BACKGROUND: Caroli Disease (CD) and Caroli Syndrome (CS) are rare disorders presenting with dilation of the intrahepatic bile ducts. CD/CS are associated with cholangiocarcinoma (CCA). However, the true incidence of CCA is still unclear, although it may serve as an indication for surgery. In this paper, we analyzed (I) the incidence of CCA in German centers, (II) reviewed our single center population together with its clinical presentation and (III) performed a thorough literature review.
METHODS: 17 large HPB-centers across Germany were contacted and their patients after surgical treatment due to CD/CS with histopathology were included. Medline search for all studies published in English or German literature was performed. Patients who underwent surgery at our department between 2012 and 2020 due to CD or CS were analyzed.
RESULTS: In the multicenter study, 79 patients suffered from CD and 119 patients from CS, with a total number of 198 patients. In 14 patients, CCA was found (Overall: 7,1%; CD: 6,3%, CS 7,6%). Between 2012 and 2020, 1661 liver resections were performed at our department. 14 patients underwent surgery due to CD or CS. Histological examination showed synchronous cholangiocarcinoma in one patient. The literature review revealed a CCA-rate of 7,3% in large series, whereas in case reports a rate of 6,8% was found.
CONCLUSIONS: There is risk of malignant transformation and patients with CD might also benefit from resection due to improvement of symptoms. Therefore, resection is strongly advised. As certain patients with CS require transplantation, treatment should not be guided by the relatively low rate of CCA but by the concomitant diseases that come along with hepatic failure.

Background: Biliary tree cysts (BTCs) represent an either localized or multifocal abnormal dilatation of the biliary tree, which entails an increased risk of acute cholangitis and cholangiocarcinoma (2.5%-16%). Its incidence in Western countries is ∼1/100.000-1/150.000, being more frequent in Asia (1/1.000). These cysts are usually classified according to Todani classification, which is based on site and morphology of cysts. Patients and Methods: This is a retrospective multicentric descriptive study of patients surgically treated for BTCs. From 2005 to 2018, 25 cases were collected between Hospital de la Santa Creu i Sant Pau (Barcelona, Spain) and Ospedale San Paolo (Savona, Italy). Clinical presentation was characterized by abdominal pain, jaundice, fever, and sometimes weight loss. Eight patients presented Todani type I, 1 patient Todani type II, 3 patients Todani III, 1 patient Todani type IV, and 12 patients Todani type V. Results: Among the 25 surgically treated patients, 12 patients underwent liver resection, 7 patients underwent resection of the extrahepatic biliary tree, 3 patients underwent BTC removal through a duodenotomy, 1 patient underwent resection of the extrahepatic biliary tree and liver resection, and 2 patients underwent pancreatoduodenectomy. Overall 30-day morbidity rate was 20%, and 90-day mortality was 0%. Pathologic examination confirmed diagnosis of cholangiocarcinoma in 5 patients (20%). After a median follow-up of 59 months, 20 patients are alive and in good conditions, whereas 50% of patients with cholangiocarcinoma died for disease progression. Conclusions: Surgical treatment for BTCs is associated with acceptable postoperative outcomes, with moderate morbidity and null mortality rates. Moreover, the risk of developing cholangiocarcinoma is still high that prompts surgical treatment once diagnosis is made.

OBJECTIVE. The purpose of this multicenter retrospective study was to assess the MRCP features of Caroli disease (CD). MATERIALS AND METHODS. Sixty-six patients were identified from 2000 to 2019. The inclusion criteria were diagnosis of diffuse or localized CD mentioned in an imaging report, presence of intrahepatic bile duct (IHBD) dilatation, and having undergone an MRCP examination. The exclusion criteria included presence of obstructive proximal biliary stricture and having undergone hepatobiliary surgery other than cholecystectomy. Histopathology records were available for 53 of the 66 (80%) patients. Diffuse and localized diseases were compared by chi-square and t tests and Kaplan-Meier model. RESULTS. Forty-five patients had diffuse bilobar CD ((five pediatric patients [three girls and two boys] with a mean [± SD] age of 8 ± 5 years [range, 1-15 years] and 40 adult patients [26 men and 14 women] with a mean age of 35 ± 11 years [range, 20-62 years]) and 21 patients had localized disease (12 men and 9 women; mean age, 54 ± 14 years). Congenital hepatic fibrosis was found only in patients with diffuse CD (35/45 [78%]), as was a \"central dot\" sign (15/35 [43%]). IHBD dilatation with both saccular and fusiform features was found in 43 (96%) and the peripheral \"funnel-shaped\" sign in 41 (91%) of the 45 patients with diffuse CD but in none of the patients with localized disease (p < .001). Intrahepatic biliary calculi were found in all patients with localized disease but in only 16 of the 45 (36%) patients with diffuse CD (p < .001). Left liver atrophy was found in 18 of the 21 (86%) patients with localized disease and in none of the patients with diffuse CD (p < .001). The overall survival rate among patients with diffuse CD was significantly lower than that among patients with localized disease (p = .03). CONCLUSION. Diffuse IHBD dilatation with both saccular and fusiform features associated with the peripheral funnel-shaped sign can be used for the diagnosis of CD on MRCP. Localized IHBD dilatation seems to be mainly related to primary intrahepatic lithiasis.

BACKGROUND: Caroli syndrome (CS) is a rare congenital disorder without pathognomonic clinical symptoms or laboratory findings; therefore, the diagnosis is often delayed. The objective of this study was to investigate the diagnostic delay and associated risk factors in CS patients.
METHODS: This was a retrospective analysis of 16 CS patients admitted to a single tertiary medical center on mainland China. The diagnostic timelines of CS patients were reviewed to demonstrate the initial findings of CS at diagnosis, the risk factors associated with diagnostic delay, and potential clues leading to early diagnosis.
RESULTS: The median diagnostic delay was 1.75 years (range: 1 month to 29 years, interquartile range: 6.2 years) in 16 enrolled CS patients. Sex, age, and initial symptoms were not associated with diagnostic delay. 87.5% of CS patients were diagnosed by imaging, and the accuracies of ultrasonography, computed tomography (CT), and magnetic resonance cholangiopancreatography were 25, 69.2, and 83.3%, respectively. The median diagnostic delays for patients with or without CT performed at the first hospital visited according to physician and radiologist suspicion of the diagnosis were 7.4 months and 6 years, respectively (p = 0.021). Hepatic cysts with splenomegaly were detected by ultrasound in over half of CS patients.
CONCLUSIONS: The majority of CS patients were not diagnosed until complications of portal hypertension had already developed. Recognition and early suspicion of the disease were important factors influencing diagnostic delay of CS. Hepatic cysts plus splenomegaly detected by US might raise the clinical suspicion to include CS in the differential diagnosis.

OBJECTIVE: To assess clinical presentation and long-term results of surgical management of congenital intrahepatic bile duct dilatation (IHBDD) (Caroli disease and syndrome) in a multicenter setting.
BACKGROUND: Congenital IHBDD predisposes to biliary stasis, resulting in intrahepatic lithiasis, septic complications, and cholangiocarcinoma. Although liver resection (LR) is considered to be the treatment of choice for unilobar disease extent into the liver, the management of bilobar disease and/or associated congenital hepatic fibrosis remains challenging.
METHODS: From 1978 to 2011, a total of 155 patients (median age: 55.7 years) were enrolled from 26 centers. Bilobar disease, Caroli syndrome, liver atrophy, and intrahepatic stones were encountered in 31.0%, 19.4%, 27.7%, and 48.4% of patients, respectively. A complete resection of congenital intrahepatic bile ducts was achieved in 90.5% of the 148 patients who underwent surgery.
RESULTS: Postoperative mortality was nil after anatomical LR (n = 111) and 10.7% after liver transplantation (LT) (n = 28). Grade 3 or higher postoperative morbidity occurred in 15.3% of patients after LR and 39.3% after LT. After a median follow-up of 35 months, the 5-year overall survival rate was 88.5% (88.7% after LT), and the Mayo Clinic score was considered as excellent or good in 86.0% of patients. The 1-year survival rate was 33.3% for the 8 patients (5.2%) who presented with coexistent cholangiocarcinoma.
CONCLUSIONS: LR for unilobar and LT for diffuse bilobar congenital IHBDD complicated with cholangitis and/or portal hypertension achieved excellent long-term patient outcomes and survival. Because of the bad prognosis of cholangiocarcinoma and the sizeable morbidity-mortality after LT, timely indication for surgical treatment is of major importance.

BACKGROUND: Treatment requirements in hepatolithiasis may vary and may involve a multidisciplinary approach. Surgical resection has been proposed as a definitive treatment.
OBJECTIVE: This study aimed to evaluate the clinical results of anatomic liver resection among Chilean patients with hepatolithiasis.
METHODS: An historical cohort study was conducted. Patients who underwent hepatectomy as a definitive treatment for hepatolithiasis from January 1990 to December 2010 were included. Patients with a preoperative diagnosis of cholangiocarcinoma were excluded. Preoperative, operative and postoperative variables were evaluated.
RESULTS: A total of 52 patients underwent hepatectomy for hepatolithiasis. The mean ± standard deviation patient age was 49.8 ± 11.8 years (range: 24-78 years); 65.4% of study subjects were female. A total of 75.0% of subjects had a history of previous cholecystectomy. The main presenting symptom was abdominal pain (82.7%). Hepatic involvement was noted in the left lobe in 57.7%, the right lobe in 34.6% and bilaterally in 7.7% of subjects. The rate of postoperative clearance of the biliary tree was 90.4%. Postoperative morbidity was 30.8% and there were no postoperative deaths. Three patients had recurrence of hepatolithiasis, which was associated with Caroli\'s disease in two of them. Overall 5-year survival was 94.5%.
CONCLUSIONS: Anatomic liver resection is an effective treatment in selected patients with hepatolithiasis and is associated with low morbidity and no mortality. At longterm follow-up, anatomic hepatectomy in these patients was associated with a lower rate of recurrence.

OBJECTIVE: In contrast to the improvement in our understanding of the pathogenesis and presentation of autosomal recessive polycystic kidney disease (ARPKD), data regarding the issue of kidney and liver transplantation in patients with ARPKD remain particularly scarce. Here, we report the results and outcome of renal and/or liver transplantation in a series of patients with ARPKD.
METHODS: Fourteen ARPKD patients (age: 3-25 years) who underwent renal transplantation with or without liver transplantation were retrospectively identified in five French nephrology departments. The patients\' medical charts were reviewed and relevant data were collected.
RESULTS: The clinical and radiological presentation of the 14 patients was highly variable illustrating the heterogeneity of ARPKD. Six patients underwent kidney and/or liver transplantation in adulthood. First renal graft survival was 92, 78 and 14% at 1, 5 and 10 years after renal transplantation, respectively. Mortality rate was relatively high (3/14; 21%) in these young patients and was directly related to infectious complications (recurrent angiocholitis) of severe Caroli\'s disease (dilatation of intra- and/or extra-hepatic bile ducts), a typical feature of ARPKD.
CONCLUSIONS: Our data suggest that ARPKD patients evaluated for renal transplantation should be carefully screened for severe Caroli\'s disease. Even though the limited number of patients included in our study precludes any definite recommendation, pre-emptive liver transplantation may be a therapeutic option in ARPKD patients with severe Caroli\'s disease evaluated for renal transplantation.

BACKGROUND: Caroli\'s disease (CD) management is still controversial.
OBJECTIVE: The purpose of this study is to report the most frequent clinical features, treatment options, and outcome obtained after surgical management of CD.
METHODS: A voluntary survey was conducted. Demographic, clinical, surgical, and pathological variables were analyzed.
RESULTS: Six centers included 24 patients having received surgical treatment from 1991 to 2009. Seventeen (70.8%) patients were female, with average age of 48.7 years old (20-71), and 95.5% were symptomatic. There was left hemiliver involvement in 75% of the patients. Surgical procedures included nine left lateral sectionectomies, eight left hepatectomies, and four right hepatectomies for those with hemiliver disease, while for patients with bilateral disease, one right hepatectomy and two Roux-en-Y hepaticojejunostomies were performed. The average length of hospitalization was 7 days. For perioperative complications (25%), three patients presented minor complications (types 1-2), while major complications occurred in three patients (type 3a). No mortality was reported. After a median follow-up of 166 months, all patients are alive and free of symptoms. CD diagnosis was confirmed by histology. Congenital hepatic fibrosis was present in two patients (8.3%) and cholangiocarcinoma in one (4.2%).
CONCLUSIONS: CD in Argentina is more common in females with left hemiliver involvement. Surgical resection is the best curative option in unilateral disease, providing long-term survival free of symptoms and complications. In selected cases of bilateral disease without parenchymal involvement, hepaticojejunostomy should be proposed. However, a close follow-up is mandatory because patients might progress and a transplant should be indicated.

The goal of this study was to determine the prevalence, epidemiology and clinical-therapeutical evolution of hepatolithiasis (HL) in Argentina. With this purpose a survey was conducted sending a questionnaire to ten referencial and interventional radiology centers in the country. Seven centers answered on time. In the last five years a total of 8,736 consecutive patients were examined for cholangiography (endoscopic retrograde cholangiography, PTC). A total of 5,920 (68%) were biliary lithiasis and 53 (0.9%, range 0.5-2.6%) of these were HL. In case of HL the diagnostic procedure was the ERCP in 68% of the cases, and the PTC in the remainder 32%. The patients with HL (53% females, mean age 52, range 23-85) clinically presented cholangitis (79%); pancreatitis (6%) and five (9.4%) showed evolution to a biliary cirrhosis. Associated diseases or abnormalities of the biliary tree were: biliary postsurgical strictures (BPS), 28%; Caroli\'s Syndrome, 20%; and choledocholithiasis, 28%. While a 9.4% presented a \"biliary history\" (that was defined as two or more episodes of biliary surgery) and a 5.7% lacked associated or predisposing diseases. Follow-up was lost in 23% of the cases and in 77% a follow up of 38 months (range 8-60) was observed with 4.8% mortality rate. The treatment was hepatobiliary surgery in 58% of the cases; endoscopic papillotomy in 17% and combined treatments that included extracorporeal shock wave lithotripsy and ursodeoxycholic acid (UDCA) in 15%. Four out of 53 cases (7.5%) received UDCA as the only successful therapy. HL is an entity with high biliary morbidity in 85% of the cases and development in to cirrhosis in 9.4%. When the diagnosis is made in the western world both BPS and Caroli must be discarded first. Combined treatments or only UDCA are new therapeutical alternative in the western world.