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.

Analysis of clinical characteristics of diagnosis and treatment of 18 patients with Caroli syndrome / 中华消化杂志

Objective:To summarize and analyze the clinical, laboratory and imaging characteristics of patients with Caroli syndrome (CS), so as to deepen the understanding of the disease and explore the possible methods for improving early diagnosis.Methods:From January 2008 to June 2021, the clinical data of 18 CS patients who were hospitalized in Peking Union Medical College Hospital and diagnosed by pathology or by clinical and imaging features were collected. The general data, clinical manifestations, laboratory examination (white blood cell count, hemoglobin, etc.), imaging features (ultrasonography, computed tomography (CT), magnetic resonance imaging (MRI)), and diagnosis and treatment of the patients were retrospectively analyzed. Descriptive method was used for statistical analysis.Results:The median age of 18 CS patients was 18 years old (ranged from 1 to 39 years old); there were 10 male and 8 female patients with a male-to-female ratio of 5∶4. The median time from symptom onset to diagnosis was 24 months (ranged from 1 month to 28 years). At the time of diagnosis, 15 patients had already had portal hypertension-related complications, and 8 patients had biliary infections. The common symptoms included abdominal distension (6 cases), fever with or without abdominal pain (5 cases), and loss of appetite (3 cases). The common abnormal laboratory findings included peripheral white blood cell count, hemoglobin level and platelet count below the normal reference value range, alanine aminotransferase and bilirubin level above the normal reference value range, and 4 patients had positive autoantibodies. Four patients were clearly diagnosed according to the pathology of liver biopsy, 14 patients were clearly diagnosed by imaging. Among which the diagnostic rate of abdominal ultrasonography for CS was 4/18, CT was 11/15, and MRI or magnetic resonance cholangiopancreatography was 12/16. The typical features of abdominal ultrasonography were liver cysts with splenomegaly, typical manifestation of CT was intrahepatic bile duct dilatation with " central dot sign", and MRI typically manifested as multiple cystic dilatations of intrahepatic bile ducts. Among the 18 patients with CS, 1 case underwent right hepatectomy, 3 cases were waiting for liver transplantation, and the other 14 patients chose symptomatic treatment due to economic reasons.Conclusions:The clinical manifestations and laboratory findings of CS patients lack specificity, and the diagnosis of most patients is delayed. The lack of understanding of the disease by clinical and imaging doctors may be one of the reasons affecting the early diagnosis of CS patients. The findings of splenomegaly and liver cysts by abdominal ultrasound may provide clues for the diagnosis of CS for clinical and imaging doctors.

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.

Caroli’s syndrome with ureterovesical calculi in a young child.

An 18-month-old boy was admitted with fever, abdominal pain, and hepatomegaly. CT scan revealed dilatation of intrahepatic biliary radicals with central dot sign, bilateral nephrocalcinosis, multiple ureteric and urinary bladder stones and right-sided obstructive hydroureteronephrosis due to a ureterovesical calculus of about 1.5 cm. Liver biopsy showed early hepatic fibrosis.

Caroli's Syndrome with Autosomal Recessive Polycystic Kidney Disease in a Two Month Old Infant

Caroli's syndrome is a rare congenital disorder that involves intrahepatic bile duct ectasia and congenital hepatic fibrosis, frequently seen with concomitant autosomal recessive polycystic kidney disease (ARPKD). Literature on infants with ARPKD is rare. Here, we present a case of a two month old boy who was diagnosed with Caroli's syndrome and ARPKD.