Project description:IntroductionEmbryonal Rhabdomyosarcoma (ERMS) is a malignant soft tissue musculoskeletal tumor which constitutes about 0.06% of all malignancies affecting children. Biliary tract ERMS is still rare, though it is considered the most common cause of malignant obstructive jaundice in children.Case presentationA report of a 2-year-old boy, who was presented with recurrent episodes of scleral icterus of three months duration, is added to the related literature. His labs went with obstructive jaundice and the radiological investigations were consistent with a diagnosis of choledochal cyst. The found mass was suspected to be an ERMS of common bile duct and turned out to be so by the histopathology. He was managed totally by laparoscope, both excision and hepaticojejunostomy reconstruction, which is an extremely uncommon entity.ConclusionCommon Bile Duct Rhabdomyosarcoma is rare and diagnosis at this anatomical site is difficult. Our case highlights the feasibility of laparoscopic resection and hepaticojejunostomy reconstruction with very good results at 16-month follow up and parents' gratitude as well.
Project description:Background and study aimsBile stones represent a highly prevalent condition and abnormalities of the biliary tree predispose to stone recurrence due to development of biliary stasis. In our study, we assessed the importance of an altered bile duct course for stone formation.Patients and methods1,307 patients with choledocholithiasis in the absence of any associated hepatobiliary disease who underwent endoscopic retrograde cholangiopancreatography (ERCP) between 2002 and 2009 were analysed. The angle enclosed between the horizontal portion of the common bile duct (CBD) and the horizontal plane was measured (angle ?). Oblique common bile duct (OCBD) was defined as a CBD with angle ? < 45°.Results103 patients (7.9%) were found to harbour OCBD and these were compared to 104 randomly selected control subjects. Compared to controls, OCBD patients were (i) significantly older (72 ± 13 vs. 67 ± 13, p<0.00001); (ii) more frequently underwent a cholecystectomy (p = 0.02) and biliary surgery (p = 0.003) prior to the diagnosis and (iii) more often developed chronic pancreatitis (p = 0.04) as well as biliary fistulae (p = 0.03). Prior to and after ERCP, OCBD subjects displayed significantly elevated cholestatic parameters and angle ? negatively correlated with common bile duct diameter (r = -0.29, p = 0.003). OCBD subjects more often required multiple back-to-back ERCP sessions to remove bile stones (p = 0.005) as well as more ERCPs later on due to recurrent stone formation (p<0.05).ConclusionOCBD defines a novel variant of the biliary tree, which is associated with chronic cholestasis, hampers an efficient stone removal and predisposes to recurrence of bile duct stones.
Project description:Mucoepidermoid carcinoma (MEC) is the most common salivary gland carcinoma; however, hepatobiliary MEC is extremely rare. A 74-year-old patient was diagnosed with hepatobiliary MEC after hepatectomy. We considered its origin could be the peribiliary glands. Its genome profile was similar to salivary MEC rather than standard biliary tract carcinoma.
Project description:Physical examination is becoming a lost art. We describe a case of a patient who was referred to us with an initial diagnosis of ventricular septal defect. Discordance between imaging findings and the physical examination led to a diagnosis of an accessory left ventricle, a rare but benign congenital cardiac condition. (Level of Difficulty: Beginner.).
Project description:Introduction and importanceIntraductal papillary neoplasm of the bile duct (IPNB) is a tumour with a very low incidence in the Western world, characterised by a high risk of malignant transformation and unknown prognosis. It is a new entity which was adopted by the WHO in 2010 as a precursor lesion of cholangiocarcinoma. Intrahepatic bile duct is the most common site of origin for IPNB.Case presentationHereby, we present a case of an asymptomatic 63- year-old man, referred to our department after routine ultrasonography showing a multifocal cystic lesion on the left hepatic lobe. Further screening modalities (CT, MRI abdo) confirmed a complex cystic liver lesion with atypical features. The patient underwent left hepatectomy. Histopathology showed a cystic type intrahepatic IPNB, which was completely resected (R0). The follow up in 2 yrs post-operation showed no signs of recurrence.Clinical discussionThe diagnosis and management of IPNB remain challenging. A multimodality imaging approach is essential in order to diagnose IPNB, assess tumour location and extent and plan the optimal treatment strategy.ConclusionComplete surgical resection (R0) with close postoperative follow-up offers long-term survival.
Project description:BackgroundLiver metabolites are used to diagnose disease and examine drugs in clinical pharmacokinetics. Therefore, development of an in vitro assay system that reproduces liver metabolite recovery would provide important benefits to pharmaceutical research. However, liver models have proven challenging to develop because of the lack of an appropriate bile duct structure for the accumulation and transport of metabolites from the liver parenchyma. Currently available bile duct models, such as the bile duct cyst-embedded extracellular matrix (ECM), lack any morphological resemblance to the tubular morphology of the living bile duct. Moreover, these systems cannot overcome metabolite recovery issues because they are established in isolated culture systems. Here, we successfully established a non-continuous tubular bile duct structure model in an open-culture system, which closely resembled an in vivo structure. This system was utilized to effectively collect liver metabolites separately from liver parenchymal cells.ResultsTriple-cell co-culture of primary rat hepatoblasts, rat biliary epithelial cells, and mouse embryonic fibroblasts was grown to mimic the morphogenesis of the bile duct during liver development. Overlaying the cells with ECM containing a Matrigel and collagen type I gel mixture promoted the development of a tubular bile duct structure. In this culture system, the expression of specific markers and signaling molecules related to biliary epithelial cell differentiation was highly upregulated during the ductal formation process. This bile duct structure also enabled the separate accumulation of metabolite analogs from liver parenchymal cells.ConclusionsA morphogenesis-based culture system effectively establishes an advanced bile duct structure and improves the plasticity of liver models feasible for autologous in vitro metabolite-bile collection, which may enhance the performance of high-throughput liver models in cell-based assays.
Project description:BackgroundAlthough laparoscopic living donor left lateral section liver procurements represents an established and safe procedure, there remains much discussion on this topic. In particular, the issue of whether laparoscopic living donor liver procurement increases the difficulty of the surgery and potential complications for recipients continue to confound experts in this field.MethodsIn this report, data from 180 cases of living donor left lateral section liver transplantation patients were analyzed retrospectively. Of these 180 cases, 106 grafts were procured by open surgery and 74 by pure laparoscopic surgery.ResultsWhile surgery durations and blood loss were decreased in donors from the laparoscopic surgery group, increased biliary openings of grafts and relatively high peak aspartate aminotransferase (AST) levels were present in both donors and recipients with this procedure.ConclusionsLaparoscopic living donor left lateral section liver procurement represents a safe and effective procedure for both donors and recipients. However, laparoscopic surgery can more frequently lead to multiple biliary tracts in the graft and its impact on the prognosis of recipients remains uncertain. Use of routine X-ray based intraoperative cholangiography may help to reduce this problem.
Project description:A 40-year-old man was admitted with necrotising fasciitis of the right thigh. Further workup to evaluate for an infectious source revealed an incidental finding of persistent left superior vena cava via transesophageal echocardiography. This finding was confirmed by cardiac MRI. During his hospitalisation, he also developed altered mental status which was found to be secondary to systemic emboli by a head MRI. The primary source of infection was likely his dental abscesses. He underwent intravenous antibiotic therapy for a total of 6 weeks and was then referred to a specialised vascular centre for further management of his congenital anomaly.