A rare congenital hepatobiliary disorder called Caroli\'s disease is characterized by multifocal segmental dilatation of intrahepatic bile ducts that can affect the entire liver or only specific areas of it. Coexisting conditions with Caroli\'s disease include autosomal dominant polycystic kidney disease (ADPKD) and autosomal recessive polycystic kidney disease (ARPKD). ADPKD results in the development of cysts, which are tiny fluid-filled sacs, in the kidneys. Caroli\'s disease is considered a rare disorder, affecting a small number of individuals worldwide. The symptoms of Caroli\'s disease can vary from person to person and it also may overlap with other liver and biliary disorders. As a result, it may be challenging to diagnose and manage the condition due to limited awareness and expertise. Increased awareness, research, and specialized medical care are crucial in improving outcomes for individuals affected by this rare disorder. This study involves the case of a 60- year-old woman presented with abdominal pain, fever, weight loss, and jaundice. Her imaging test endoscopic retrograde cholangiopancreatography (ERCP) signifies Caroli\'s disease with pancreatic duct (PD) calculi and management involves supportive care with antibiotics. Antibiotics were prescribed to prevent or treat infections such as cholangitis and nutritional supplement was recommended in managing Caroli\'s disease. The patient underwent pancreatic stent placement and was discharged with regular follow-up. So, this case highlights the clinical and diagnostic aspects to improve disease understanding and the progression of Caroli\'s illness along with ADPKD.

Caroli\'s disease is a rare congenital bile duct malformation characterized by intrahepatic bile duct dilatation. This kind of situation is seldom encountered in clinical work. We report such a case who presented to our emergency department with recurrent fever as initial symptom. According to the clinical manifestation and imaging examination, a 13-year-old boy was diagnosed with suppurative cholangitis and sepsis caused by Caroli\'s disease. The symptoms were got relieved after antibiotic therapy upgraded from cephalosporins to carbapenems. After 5 months of follow-up, he did not have fever, abdominal pain or any other discomfort. We believe the present report is of medical significance since it serves as a reminder that Caroli\'s disease may have atypical presentations and be masked by non-specific clinical findings. The report hopes to enlighten our pediatric colleagues by providing more knowledge on such rare congenital disease.

Caroli´s disease is a congenital hepatic disorder characterized by nonobstructive saccular or fusiform dilatation of the intrahepatic bile ducts with the absence of congenital hepatic fibrosis. Caroli´s disease is rare, with few reported cases in the literature, making it hard to distinguish from other liver abnormalities. We present a case of Caroli´s disease discovered indecently in a 16-year-old female who presented with recurrent abdominal pain and intermittent jaundice in the last three years. Abdominal Computed tomography (CT) showed mild liver enlargement with multiple cystic dilatations of the intrahepatic saccular bile ducts cystic dilatations without hepatic fibrosis. The patient was treated conservatively with ursodeoxycholic acid and antibiotic therapy and discharged with regular follow-up. In conclusion, Caroli´s disease should be considered in the differential diagnosis in patients with recurrent abdominal pain and cholangitis without risk factors or relevant history.

暂无摘要。

暂无摘要。

Choledochal cysts are seen commonly in Asian populations, but rarely in Western populations. The pathogenesis of these premalignant lesions is not fully understood yet and the risk of malignant transformation increases with age. The overall malignancy risk is 10%-15% in East Asian countries. In this study, we aimed to present our surgical experience as a hepatobiliary center to the literature.
We retrospectively analyzed the data from the medical records of 70 patients operated for choledochal cyst between 2008-2019.
Sixty-two of the 70 (89%) patients were female and 8 (11%) were male, the mean age was 45.89 ± 15.32 years. Overall, 44 (63%) patients had type I (a+b+c), 20 (28%) type V (Caroli), 2 (3%) type II, 2 (3%) type III and 2 (3%) type IVb cysts. The most common operation was cyst excision combined with hepaticojejunostomy (n: 26, 37%). The median diameter of the resected cysts was 3 cm (min- max: 1-11 cm). Malignancy was observed only in three (4%) patients with type III, type Ib, and type V cyts, who were 19, 38, and 72 years old, respectively. Mortality was not observed, morbidity was determined totally in 30 (43%) cases during early and late postoperative periods.
Type of surgery in choledochal cysts differs according to the type of the cyst. Malignancy was observed at a rate of 4% in all age groups. Although the frequency of malignancy varies, the main treatment of choice should be surgery because malignancy can be seen at a young age.

The cholangipathies are a class of liver diseases that specifically affects the biliary tree. These pathologies may have different etiologies (genetic, autoimmune, viral, or toxic) but all of them are characterized by a stark inflammatory infiltrate, increasing overtime, accompanied by an excess of periportal fibrosis. The cellular types that mount the regenerative/reparative hepatic response to the damage belong to different lineages, including cholagiocytes, mesenchymal and inflammatory cells, which dynamically interact with each other, exchanging different signals acting in autocrine and paracrine fashion. Those messengers may be proinflammatory cytokines and profibrotic chemokines (IL-1, and 6; CXCL1, 10 and 12, or MCP-1), morphogens (Notch, Hedgehog, and WNT/β-catenin signal pathways) and finally growth factors (VEGF, PDGF, and TGFβ, among others). In this review we will focus on the main molecular mechanisms mediating the establishment of a fibroinflammatory liver response that, if perpetuated, can lead not only to organ dysfunction but also to neoplastic transformation. Primary Sclerosing Cholangitis and Congenital Hepatic Fibrosis/Caroli\'s disease, two chronic cholangiopathies, known to be prodrome of cholangiocarcinoma, for which several murine models are also available, were also used to further dissect the mechanisms of fibroinflammation leading to tumor development.

UNASSIGNED: A 4-month-old neutered male Russian Blue kitten had a 4 week history of hypersalivation and failure to thrive. In addition, there was a 2 week history of soft tissue swelling on the ventral abdomen, which had failed to improve with antimicrobial therapy. There were no significant physical examination or neurological deficits on examination; however, the cat had a quiet demeanour for its age. Postprandial bile acids were increased (32 µmol/l; reference interval <25 µmol/l). An abdominal CT scan revealed changes consistent with an extrahepatic portosystemic shunt and inflammation of fat of the ventral abdominal body wall. Surgical biopsy and culture of the subcutaneous swelling identified non-infectious steatitis. Ten weeks following initial presentation, surgical exploration, liver biopsy and ligation of the portosystemic shunt were performed. Liver biopsy was submitted to the Anatomical Pathology Laboratory of Cornell University Animal Health Diagnostic Center, New York, USA. Histopathology revealed a ductal plate malformation (Caroli\'s type), as well as changes consistent with a portosystemic shunt.
UNASSIGNED: Ductal plate malformations are rarely described in the veterinary literature. To our knowledge this is the first reported case of Caroli\'s-type malformation in a cat. There are no biochemical changes that allow for differentiation of ductal plate malformations from other hepatopathies. Liver biopsy is required for a definitive diagnosis.

The aim of this study was to report a Western experience in the diagnosis and management of choledochal cyst disease.
Sixty-seven patients were identified including 15 children and 52 adults; 76.1 % were females. The median age at diagnosis was 3 [inter-quartile range (IQR) = 6.0-0.7] years for children, and 46 [IQR = 55.6-34.3] years for adults. Forty-eight patients (72 %) were symptomatic. Types of choledochal cyst included: I (n = 49, 73.1 %), II (n = 1, 1.5 %), IV (n = 9, 13.4 %), and V (n = 8, 12 %). The median diameter of the type I choledochal cyst was 35 [IQR = 47-25] mm. All 48 patients underwent excision of cyst with Roux-en-Y hepaticojejunostomy, and eight underwent resection with hepaticoduodenostomy. Six patients underwent liver resection, and five patients underwent orthotopic liver transplantation. Malignancy was concomitant in five adult patients, being identified on preoperative imaging in three cases; and atypia was seen in three additional patients. Early morbidity included Clavien-Dindo classification grades III (n = 7) and II (n = 5), while long-term complications consisted of Clavien-Dindo grades V (n = 5), IV (n = 2), III (n = 18), and II (n = 1).
Presentation and management of choledochal cyst is varied. Malignant transformation is often detected incidentally, and so should be the driving source for resection when a choledochal cyst is diagnosed.

BACKGROUND: Caroli\'s disease is a rare congenital condition characterized by non-obstructive dilatation of intrahepatic ducts. In Caroli\'s syndrome, there is additionally an associated congenital hepatic fibrosis.
METHODS: With institutional review board approval, we identified all patients with Caroli\'s disease and syndrome.
RESULTS: Nine patients were identified, seven males and two females, with a median age of 40 years. Final pathological diagnoses included Caroli\'s disease (n = 6) and Caroli\'s syndrome (n = 3). Patients presented with deranged liver function, cholangitis, cholangiocarcinoma, abdominal pain, cirrhosis, or were diagnosed incidentally. Four patients underwent resection and two underwent liver transplantation. Of the resection group, two patients subsequently underwent transplantation for recurrent cholangitis due to anastomotic stricture in one patient and for end-stage liver disease in the other. All patients with Caroli\'s syndrome underwent liver transplantation. Three patients died during follow-up at 26.2, 7.8, and 3 months post-diagnosis with recurrence of cholangiocarcinoma, liver failure, and metastatic cholangiocarcinoma, respectively. Six patients are alive with a median follow-up of 60 months since presentation (range = 10-134 months).
CONCLUSIONS: Caroli\'s disease and syndrome have a varied presentation. Most individuals with Caroli\'s disease may be adequately treated by resection, but transplantation is required for Caroli\'s syndrome patients due to the associated hepatic fibrosis.