Bile duct embryology

Left-sided gallbladders are rare anatomical variations and a result of an abnormal embryological process. The most frequent cause for a sinistroposition gallbladder is the presence of situs inversus . We present a case of a 51-year-old male referred to the General Surgery consult due to cholelithiasis with a history of occasional post-prandial abdominal pain in the left hypochondrium and nausea associated with the ingestion of lipid-rich meals. The ultrasound revealed a gallbladder filled with calculous but without inflammatory signs or bile duct dilation, in the sinistroposition...

BACKGROUND: Portal vein (PV) duplication is a rare developmental anomaly but has an important role in the diagnosis and management of disease for radiologists and surgeons. MATERIALS AND METHODS: A new variant of PV duplication with vein fenestration leading to choledochal stenosis and dilatation and thrombus was identified by computed tomography angiography (CTA) on a 59-year-old woman with a history of gallstones. RESULTS: A second PV originated from the superior mesenteric vein (SMV), split into two branches that encircling the common bile duct to form a vein fenestration, leading to choledochal stenosis and dilatation, with thrombus formation at the confluence...

Earlier data on liver development demonstrated that morphogenesis of the bile duct, portal mesenchyme and hepatic artery is interdependent, yet how this interdependency is orchestrated remains unknown. Here, using 2D and 3D imaging, we first describe how portal mesenchymal cells organize to form hepatic arteries. Next, we searched for intercellular signalling active during portal area development and found that axon guidance genes are dynamically expressed in developing bile ducts and portal mesenchyme. Using tissue-specific gene inactivation in mice, we show that the Repulsive guidance molecule BMP co-receptor A (Rgma)/Neogenin 1 (Neo1) receptor/ligand pair is dispensable for portal area development, but that deficient Roundabout 2 (Robo2)/Slit2 signalling in the portal mesenchyme causes reduced maturation of the vascular smooth muscle cells that form the tunica media of the hepatic artery...

The Haematopoietically expressed homeobox transcription factor (Hhex) is a transcriptional repressor that is of fundamental importance across species, as evident by its evolutionary conservation spanning fish, amphibians, birds, mice and humans. Indeed, Hhex maintains its vital functions throughout the lifespan of the organism, beginning in the oocyte, through fundamental stages of embryogenesis in the foregut endoderm. The endodermal development driven by Hhex gives rise to endocrine organs such as the pancreas in a process which is likely linked to its role as a risk factor in diabetes and pancreatic disorders...

Hagfish (Myxinoidea) are a deep-sea taxon of cyclostomes, the extant jawless vertebrates. Many researchers have examined the anatomy and embryology of hagfish to shed light on the early evolution of vertebrates; however, the diversity within hagfish is often overlooked. Hagfish have three lineages, Myxininae, Eptatretinae, and Rubicundinae. Usually, textbook illustrations of hagfish anatomy reflect the morphology of the Myxininae lineage, especially Myxine glutinosa, with its single pair of external branchial pores...

Congenital duplication of the extrahepatic bile duct (DEBD) is an unusual anomaly of the biliary system. It occurs due to inability of the embryological duplex biliary system to regress. DEBD has various subtypes depending on the morphology and opening of the aberrant common bile duct. It can have distinct complications. We encountered a 38-year-old lady who experienced pain in the right upper abdomen along with a low-grade fever. Magnetic resonance cholangiopancreatography revealed DEBD with multiple calculi in the right hepatic duct (ductolithiasis) and joining of the right hepatic duct with the left hepatic duct in the intrapancreatic region...

The two main biliary pathologies in paediatric practice, biliary atresia and choledochal malformations (CM), have their origins within prenatal life. Nevertheless, the actual mechanisms remain elusive with many unanswered questions. The extrahepatic bile duct develops as a funnel-like structure emerging from the foregut from about 3-4 weeks of gestation into the mesenchyme of the septum transversum. The cranial elements of this contain hepatoblasts - the precursors to the two key cell lines that will become hepatocytes and biliary epithelial cells...

BACKGROUND: Triple gallbladder is a rare congenital anomaly of the biliary tract that can be associated with heterotopic tissue. Gallbladder triplication results from the failure of rudimentary bile ducts to regress during embryological development, and can be difficult to distinguish from Todani type II choledochal cysts and biliary duplication cysts. CASE PRESENTATION: A 2-year-old patient presented to our institution with intermittent abdominal pain for 1 year...

Hepatocellular carcinoma (HCC) is the main primary liver malignancy, being associated with both health and economic burden worldwide. Recently, novel molecular markers and possible therapeutic targets were identified. Different adhesion molecules, as well as possible angiogenesis-associated targets can be prime candidates when investigating novel therapies. Considering these premises, our goal was to study the co-existence of E-cadherin and aquaporin 1 (AQP1) in a series of HCC diagnosed patients. Utilizing archived tissue fragments from 17 patients diagnosed with well-to-moderate and poorly differentiated HCC, as well as four samples of normal liver tissue and using a highly specific biotin-free tyramide amplification technique, we have assessed here the expression of E-cadherin and AQP1 during HCC carcinogenesis...

Ductal plate malformations (DPM) arise from abnormal remodeling of the embryologic ductal plate of the liver. Malignant transformation of DPMs to intrahepatic cholangiocarcinoma (iCCA) has been reported in very rare instances but is viewed with some skepticism. We report the clinicopathological findings in five cases of iCCA, occurring in liver with DPM-like features. All tumors were less than 5 cm, often presented as stage T1a tumors. Histologically, a typical tumor showed a vague multinodular architecture with larger, irregular, tortuous glandular structures with microcystic dilation, intraluminal fibroepithelial projection, and bridge/island formation...

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The biliary tract is notorious for its variable anatomy. A persistent hepaticocystic duct with agenesis of common bile duct is a rare biliary anomaly that creates a diagnostic dilemma and can add to the operative difficulties. It is important to diagnose this anomaly preoperatively since the gallbladder forms an integral part of bilioenteric continuity and an inadvertent cholecystectomy can lead to a surgical catastrophe. If diagnosed, surgeons can plan definitive treatment in the form of biliary diversion...

The liver consists of parenchymal hepatocytes and non-parenchymal cells. Non-parenchymal cells, Kupffer cells, hepatic stellate cells and cholangiocytes have crucial roles in liver homeostasis and liver pathology. To establish baseline data, this study investigated immunohistochemically the distribution of non-parenchymal cells in perivenular areas (PV), periportal areas (PP) and Glisson's sheath (GS) of adult rat liver. Liver tissues were collected from the left lateral lobe of rats. CD163-positive macrophages were seen along the sinusoid of PV and PP areas, indicating Kupffer cells...

Ductal plate malformations are abnormalities in the liver that arise from inappropriate or incomplete remodeling of the embryologic ductal plate. Various types of ductal plate malformations are reported in the human and veterinary literature, most commonly affecting domestic mammalian species but also fish. We investigated the occurrence and described the histopathologic features of ductal plate malformations in captive snakes. Malformations were identified in 18 snakes: 10 colubrids, 6 vipers, and 2 boids...

Familiarity with the anatomy is paramount for conduct of safe surgery for a surgeon. Anomalies with the biliary and hepatic arterial system are common but portal vein anomalies are least common. A preduodenal portal vein has been reported in about 100 cases but a combination of preduodenal portal vein with preduodenal common bile duct is extremely rare with only 9 cases being reported. We report a one such case and discuss the embryological basis for the peculiar anatomy observed in our case which has never been reported earlier...

the bile duct system and pancreas show many similarities due to their anatomical proximity and common embryological origin. Consequently, preneoplastic and neoplastic lesions of the bile duct and pancreas share analogies in terms of molecular, histological and pathophysiological features. Intraepithelial neoplasms are reported in biliary tract, as biliary intraepithelial neoplasm (BilIN), and in pancreas, as pancreatic intraepithelial neoplasm (PanIN). Both can evolve to invasive carcinomas, respectively cholangiocarcinoma (CCA) and pancreatic ductal adenocarcinoma (PDAC)...

Congenital cystic lesions of the bile ducts represent a spectrum of liver and biliary system lesions, resulting from abnormal embryologic development of the ductal plate. These disorders include Caroli disease, choledochal cysts, autosomal dominant polycystic liver disease, congenital hepatic fibrosis, and biliary hamartomas. Each disorder carries a peculiar clinical presentation, prognosis, and risk of complications. Knowledge of radiological findings of fibropolycystic liver diseases is crucial for their appropriate detection and for differential diagnosis with other similar hepatic cystic lesions, in order to avoid relevant misdiagnosis...

INTRODUCTION: Congenital agenesis of the gallbladder is a rare embryological defect of the biliary system. While occurring equally in men and women, gallbladder agenesis is found clinically twice as often in women. Patients present with symptoms suggesting biliary colic. Abdominal ultrasound and cholecintigraphy or HIDA scan are usually inconclusive and, in some cases, may be read as positive for biliary colic. Patients can undergo surgery based on characteristics of pain. PRESENTATION OF CASE: We report the case of a 60-year-old female presenting with symptoms of recurrent biliary colic and subsequently undergoing laparoscopic cholecystectomy after inconclusive workup...

INTRODUCTION: Left-sided gallbladder without situs viscerum inversus (LSG-woSVI) is defined as a gallbladder located under the left lobe of the liver; to the left of the round/falciform ligament, but with all other viscera maintaining normal anatomical relationships. This is a rare congenital anomaly with a reported prevalence that ranges from 0.04% to 1.1%. It is usually an incidental intraoperative finding, and can be associated with anatomic abnormalities of the biliary tree, portal system and vasculature...

PURPOSE: We aimed to evaluate the frequency of bile duct branching pattern variations at the hepatic confluence in patients with pancreas divisum (PD). METHODS: A search was performed through the hospital database using the keyword "pancreas divisum" to identify patients. The magnetic resonance cholangiopancreatography (MRCP) images of 137 patients who were diagnosed with PD between August 2011 and November 2016 were retrospectively analyzed for the presence of bile duct variations...