• Humans
• Liver Transplantation
• Hepatitis B, Chronic/complications
• Caroli Disease/surgery
• Male
• Liver Cirrhosis/surgery
• Liver Cirrhosis/virology
• Adult
• Treatment Outcome

• Congenital
• Egypt
• Liver diseases
• Orphan
• Rare

• Humans
• Egypt/epidemiology
• Liver Diseases/epidemiology
• Liver Diseases/diagnosis
• Rare Diseases/epidemiology
• Rare Diseases/diagnosis

• Adult
• Humans
• Child
• Contrast Media
• Cholangiopancreatography, Endoscopic Retrograde
• Choledochal Cyst/diagnosis
• Choledochal Cyst/pathology
• Magnetic Resonance Imaging/methods
• Gallbladder Diseases
• Bile Ducts, Intrahepatic/pathology
• Bile Duct Neoplasms/pathology

• Cysts/diagnosis
• Cysts/therapy
• Hamartoma
• Humans
• Liver Diseases/diagnosis
• Liver Diseases/therapy

• Caroli disease
• MRI
• bile ducts
• congenital lithiasis

• Caroli disease (CD)
• Caroli syndrome (CS)
• Fibrocystic liver disease (FLD)
• biliary fibrosis
• congenital hepatic fibrosis (CHF)
• polycystic kidney hepatic disease 1 (PKHD1)

Liver resection is the treatment of choice for patients with localised Caroli disease. While liver resection was traditionally performed as open procedure, this case series aims to evaluate the safety and efficacy of minimally invasive, laparoscopic liver surgery in these patients.

A systematic review of electronic case files of patients seen between April 2015 and December 2017 at the Department of Surgery, Charité University Hospital Berlin, was conducted. Patients with Caroli disease in whom laparoscopic liver resection had been performed were identified and analysed in this single-centre case series.

Seven patients who underwent laparoscopic liver surgery for Caroli syndrome were identified and presented with a median age of 49 (range = 44–66) years, of which four (57%) were female. Preoperatively, six patients were classified as the American Society of Anaesthesiologists (ASA) 2 and one patient as ASA 3. Two operations were performed as single-incision laparoscopic surgery, whereas the others were done as multi-incision laparoscopic surgery. One patient required a conversion to an open procedure. The length of operation varied between patients, ranging from 128 to 758 min (median = 355). The length of stay in the intensive care unit ranged from 0 to 2 days. Two patients presented with post-operative complications (Clavien–Dindo Grade ≥3a), whereas no patient died. In histopathological analysis, all patients demonstrated characteristic findings of Caroli disease and no cholangiocarcinoma was found.

These results indicate that minimally invasive, laparoscopic liver surgery is a safe and efficacious treatment option for patients with Caroli disease who require liver resection.

• Caroli syndrome
• laparoscopic liver resection
• liver resection
• minimally invasive liver resection

• Caroli's syndrome
• hematemesis
• pediatrics
• portal hypertension

Congenital intrahepatic bile duct dilatation without fibrosis is called Caroli disease (CD), and is called Caroli syndrome (CS) when it has fibrotic and cirrhotic liver morphology. The development of intrahepatic carcinoma is described in both conditions, but the reported incidence varies extensively. Potential risk factors for the malignant transformation were not described. Furthermore, conservative or surgical treatment is performed depending on the extent of cystic malformation, hepatic dysfunction and structural hepatic changes, but little is known about which treatment should be offered to patients with CD or CS and cancer.

To further investigate the malignant transformation in these conditions.

A systematic review of the current literature until January 2019 was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement. A search using Medline (PubMed) was performed using a combination of Medical Subject Headings terms “caroli disease”, “caroli syndrome”, “tumor”, “malignant”, and “cholangiocarcinoma”. Only human studies published in English were used for this systematic review. The following parameters were extracted from each article: year of publication, type of study, number of patients, incidence of malignant tumor, duration of symptoms, age, sex, diagnostics, identification of tumor, surgical therapy, survival and tumor recurrence.

Twelve retrospective studies reporting the courses of 561 patients (53% females) were included in this systematic review. With a mean age of 41.6 years old (range 23 to 56 years old), patients were younger than other populations undergoing liver surgery. Depending on the size of the study population the incidence of cholangiocarcinoma varied from 2.7% to 37.5% with an overall incidence of 6.6%. There were only few detailed reports about preoperative diagnostic work-up, but a multimodal work-up including ultrasound of the liver, computed tomography, magnetic resonance imaging and endoscopic retrograde cholangiopancreatography was used in most studies. Disease duration was variable with up to several years. Most patients had episodes of cholangitis, sepsis, fever or abdominal pain. Tumor detection was an incidental finding of the surgical specimen in most cases because it is currently often impossible to detect tumor manifestation during preoperative diagnostics. Liver resection or liver transplantation was performed depending on the extent of the biliary pathology and additional alterations of the liver structure or function. No postoperative adjuvant chemotherapy was reported, but chemotherapy was administered in selected cases of tumor recurrence. Overall survival rates after one year were low at 36% and a high recurrence rate of up to 75% during the observation period.

Only few retrospective studies reported a low tumor incidence. Despite the high rate of mortality and tumor recurrence, definite surgical treatment should be offered as soon as possible.

• Malignancy
• Caroli disease
• Caroli syndrome
• Tumor
• Cholangiocarcinoma
• Review

• Adult
• Bile Duct Neoplasms
• Bile Ducts, Intrahepatic
• Caroli Disease/diagnostic imaging
• Caroli Disease/epidemiology
• Caroli Disease/surgery
• Female
• Humans
• Male
• Middle Aged
• Neoplasm Recurrence, Local
• Retrospective Studies
• Young Adult

• Caroli disease
• Caroli syndrome
• Cholangiocarcinoma
• Liver resection
• Liver transplantation

• Bile Duct Neoplasms/surgery
• Caroli Disease/surgery
• Cholangiocarcinoma/surgery
• Female
• Follow-Up Studies
• Hepatectomy/methods
• Hepatectomy/statistics & numerical data
• Humans
• Liver Cirrhosis/surgery
• Liver Transplantation/statistics & numerical data
• Male
• Middle Aged
• Retrospective Studies
• Symptom Assessment
• Syndrome
• Time Factors
• Treatment Outcome