BILE DUCT SYSTEM MALFORMATION - EMBRYOLOGICAL AND PATHOLOGICAL ASSOCIATION. TREATMENT /REVIEW ARTICLE (original) (raw)
Related papers
Management of Congenital Bile Duct Cysts
Digestive Surgery, 2010
lying chronic liver (congenital hepatic fibrosis) and kidney disease. Late postoperative cholangitis secondary to intrahepatic strictures and lithiasis, as well as the risk of metachronous cholangiocarcinoma that can occur throughout the whole biliary tree even after complete cyst excision, represent serious complications justifying careful long-term follow-up.
A CASE REPORT OF CONGENITAL CYSTIC DILATATION OF INTRAHEPATIC BILIARY TREE (CAROLI’S DISEASE)
2017
Caroli’s disease is a rare autosomal recessive congenital disorder characterized by multifocal, non-obstructive, saccular or fusiform dilatation of the large-sized intrahepatic bile ducts, which communicate with the biliary tree. . The estimated incidence of Caroli’s disease is 1 in 1 000 000 population, males and females are equally affected. The disease usually remains asymptomatic during the first 20 years, and may also remain so throughout life. The diagnosis is radiological and relies on the demonstration of continuity between the cystic lesions and the biliary tree. We report a case of Caroli’s disease, which was revealed at male patient of 44 years old.
Veterinary Pathology, 2003
Hepatic fibrosis with bile duct ectasia and hyperplasia associated with polycystic kidney disease, analogous to Caroli syndrome in humans, was observed in a rat used as a control in a subchronic toxicity study. Light microscopy of liver sections showed multiple cystic and segmental saccular dilatations and hyperplasia of the intrahepatic bile ducts associated with overgrowth of portal connective tissue; the kidneys had diffuse cystic dilatation of cortical renal tubules. The lesions resembled those of human cases of the fibropolycystic disease termed as Caroli syndrome, which is thought to be the result of a pathologic developmental process known as ductal plate malformation. Recently, an animal model of Caroli syndrome has been described in mutant rats from a colony that constantly showed renal and hepatic cysts and an autosomal recessive mode of inheritance. The finding in our case of identical hepatorenal lesions suggests that the same mutation has occurred incidentally in a stan...
Development of the bile ducts: Essentials for the clinical hepatologist
Several cholangiopathies result from a perturbation of developmental processes. Most of these cholangiopathies are character-ised by the persistence of biliary structures with foetal configuration. Developmental processes are also relevant in acquired liver diseases, as liver repair mechanisms exploit a range of autocrine and paracrine signals transiently expressed in embryonic life. We briefly review the ontogenesis of the intra-and extrahepatic biliary tree, highlighting the morphogens, growth factors, and transcription factors that regulate biliary development, and the relationships between developing bile ducts and other branching biliary structures. Then, we discuss the ontogenetic mechanisms involved in liver repair, and how these mechanisms are recapitulated in ductular reaction, a common reparative response to many forms of biliary and hepatocel-lular damage. Finally, we discuss the pathogenic aspects of the most important primary cholangiopathies related to altered bili-ary development, i.e. polycystic and fibropolycystic liver diseases, Alagille syndrome.
Congenital Cystic Lesion In Extra Hepatic Biliary Tract
2019
Objective: To illustrate congenital cystic lesions of the extra hepatic biliary tract on the idea of information of embryologic development through the magnetic resonance cholangiopancreatography (MRCP). Methods: Patients that were suspected to have been suffering from biliary disease were referred to the MRI units, there the patients were narrowed done to confirm with the inclusion and exclusion criteria, thereafter written and informed consent was obtained from them and the procedure was explained to them in detail. MRCP was performed on a 1.5 Tesla in MR unit, using phased-array coil for signal detection. Heavily T2 weighted images were obtained with SSF-SE technique. The axial sections were used for pancreatic and bile ducts whereas the coronal sections were used for the pancreatobiliary tract, axial and coronal source images and reformatted images were evaluated together for the possibility of any anomaly in extra hepatic biliary tract. This was an observational, cross sectiona...
Bile duct cyst type V (Caroli's disease): surgical strategy and results
HPB, 2007
Background. Caroli's disease (CD) is a benign congenital disorder characterized by segmental cystic dilatation of the intrahepatic biliary ducts. Therapeutic strategy includes medical treatment, percutaneous, endoscopic or surgical drainage of the affected bile ducts, liver resection or transplantation. The aim of this study was to analyse the results and long-term follow-up of a consecutive series of patients who underwent surgical treatment for CD. Patients and methods. Between 1995 and 2005, 10 patients were surgically treated for CD. Variables evaluated were: age, gender, clinical presentation, diagnostic procedures, percutaneous and surgical treatments, histopathological analysis and outcome. Results. The average age of the patients was 45.8 years. Recurrent cholangitis was the main clinical manifestation (70%). In unilateral CD a liver resection was performed in nine patients (left lateral sectionectomy in seven, left hepatectomy in one and right hepatectomy in one). In bilateral disease a cholecystectomy, duct exploration, hepaticojejunostomy and liver biopsy of both lobes were performed. Average follow-up was 60 months. All the patients are alive and free of symptoms without recurrence in the remnant liver. Discussion. Liver resection is the preferred therapeutic option for unilateral CD, demonstrating good results in long-term follow-up. In bilateral disease, hepaticojejunostomy could be considered as an alternative or a previous step to liver transplantation, which still remains the ultimate option.