Extrapancreatic Bile Duct Research Articles

Biliary and duodenal complications after «en bloc» liver-small bowel transplantation in children. A single center cohort study.

The technique of « en bloc» liver and small bowel transplantation (L-BT) spares a biliary anastomosis, but does not protect against biliary complications. We analyze biliary and duodenal complications (BDC) in our pediatric series. Between 1994 and 2020, 54 L-BT were performed in 53 children. The procurement technique included in situ vascular dissection and pancreatic reduction to the head until 2009 (group A). Thereafter, the whole pancreas was recovered (group B). Nine BDCs occurred in 8/53 (15%) patients (7 in group A and 1 in group B): leak of the donor's duodenal stump (2), stenosis of the extra-pancreatic bile duct (5), and intra-pancreatic bile duct stenosis (2). Median delay for diagnosis of stricture was 8months (4-168). Interventional radiology was successful in one child only, the others required reoperations. Two patients died, of biliary cirrhosis or cholangitis, 15-month and 12-year post-L-BT. One was listed and liver re-transplanted 13years post-L-BT. At last follow-up, two patients only had normal liver tests and ultrasound. BDC after L-BT can cause severe morbidities. Pancreatic reduction might increase this risk. Early surgical complications or chronic pancreatic rejection might be co-factors. Early diagnosis and treatment are key to the long-term prognosis.

Immunoglobulin G-4-Related Retroperitoneal Fibrosis.

Retroperitoneal fibrosis is caused by the replacement of normal retroperitoneal tissue with fibrosis. The majority of the cases are idiopathic, but some secondary causes include malignancy, infection, drugs, and radiotherapy. Immunoglobulin G-4 (IgG-4) related disease is a relatively newer disease and one of the rarer causes of retroperitoneal fibrosis. It usually involves the pancreas, lungs, kidneys, aorta lacrimal and salivary glands, or extrapancreatic bile duct. Elevated serum IgG-4 is the biomarker of the disease and its levels correlate with disease activity. High-dose glucocorticoid is the treatment of choice.

Usefulness of peroral cholangioscopy in the differential diagnosis of IgG4-related sclerosing cholangitis and extrahepatic cholangiocarcinoma: a single-center retrospective study

BackgroundIn the diagnosis of IgG4-related sclerosing cholangitis (IgG4-SC), differentiation from extrahepatic cholangiocarcinoma (ECC) is extremely important but is still a clinical challenge. This study aimed to elucidate the usefulness of peroral cholangioscopy (POCS) for the differential diagnosis between IgG4-SC and ECC.MethodsPOCS findings for bile duct stricture were retrospectively evaluated in 17 patients with IgG4-SC diagnosed at the Hiroshima University Hospital and 53 patients with surgically resected infiltrating ECC. Mucosal surface, dilated vessels (tortuosity, caliber alteration, and disruption), and easily bleeding were compared between the groups.ResultsThe stricture sites of IgG4-SC evaluated by POCS were 10 extrapancreatic bile ducts and 9 intrapancreatic bile ducts. In patients with IgG4-SC, smooth mucosal surface was observed in 89% (17/19), dilated vessels in 58% (11/19) [tortuosity 82% (9/11), caliber alteration 18% (2/11), and disruption 9% (1/11)], and easily bleeding in 0%. Irregular mucosal surface and easily bleeding were observed significantly more frequently in ECC (both P < 0.001). The frequency of caliber alteration and disruption of dilated vessels was significantly less in IgG4-SC (P < 0.001 and 0.005, respectively). The sensitivity and specificity of POCS in the diagnosis of ECC were 96 and 89%, respectively. Dilated vessels in IgG4-SC were observed significantly more frequently in the extrapancreatic bile duct, especially the hilar bile duct (P = 0.006). Concerning image evaluation, the interobserver agreement was κ = 0.719, and the intraobserver agreement was κ = 0.768 and 0.754.ConclusionsCharacteristic POCS findings of the stricture sites in IgG4-SC were smooth mucosal surface, dilated vessels without caliber alteration and disruption, and lack of easily bleeding. These POCS findings are extremely useful for distinguishing between IgG4-SC and ECC.

Pancreaticobiliary involvement in treated type 1 autoimmune pancreatitis: Imaging pattern and risk factors for disease relapse

PurposeTo evaluate the imaging pattern of pancreaticobiliary lesions in patients with treated type 1AIP, to determine the incidence of disease relapse and malignancy, and to identify the risk factors. MethodThe institutional review board approval was acquired. All patients gave written informed consent. From a prospective clinico-radiological database since 2012, consecutive patients with type 1 AIP who were treated and followed up (≥18 months) were identified. The presence/absence of pancreaticobiliary lesion(s) development during follow-up were assessed. The etiology was determined and the imaging pattern was compared to the initial attack. Risk factors were identified by univariate and multivariate analysis. ResultsAmong 103 patients with treated type 1 AIP, 44 (42.7%) developed pancreaticobiliary lesions during follow up (median time interval to initial diagnosis: 17 months, range 3–62 months), mostly after steroid discontinuation (63.6%) or during maintenance therapy (29.5%). All lesions were disease relapse, which responded to steroid treatment. At relapse, pancreatic involvement was less frequent (81.8% vs 100%, p = 0.003), and the pancreas size was smaller (p < 0.01), whereas extra-pancreatic bile duct (ExPanBD) involvement was more severe and extensive (both p < 0.01). Multivariate analysis revealed ExPanBD involvement at initial diagnosis (hazard ratio 2.437, 95% CI 1.343–7.402, p = 0.002) and serum IgG4 response ratio at the induction phase (hazard ratio 0.357, 95% CI 0.055-0.804, p = 0.011) as significant independent predictors of relapse. ConclusionsIn treated type 1 AIP, although imaging pattern may differ, pancreaticobiliary lesions are usually manifestations of disease relapse. ExPanBD involvement and poor serum response suggests high risk of relapse.

18F- FDG PET/CT helps differentiate autoimmune pancreatitis from pancreatic cancer

Background18F-FDG PET/CT could satisfactorily show pancreatic and extra-pancreatic lesions in AIP, which can be mistaken for pancreatic cancer (PC). This study aimed to identify 18F-FDG PET/CT findings that might differentiate AIP from PC.MethodsFDG-PET/CT findings of 26 AIP and 40 PC patients were reviewed. Pancreatic and extra-pancreatic lesions related findings, including maximum standardized uptake values (SUVmax) and patterns of FDG uptake, were identified and compared.ResultsAll 26 patients with AIP had increased pancreatic FDG uptake. Focal abnormal pancreatic FDG activities were found in 38/40 (95.00%) PC patients, while longitudinal were found in 18/26 (69.23%) AIP patients. SUVmax was significantly different between AIP and PC, both in early and delayed PET/CT scans (p < 0.05). AUCs were 0.700 (early SUVmax), 0.687 (delayed SUVmax), 0.683 (early lesions/liver SUVmax), and 0.715 (delayed lesion/liver SUVmax). Bile duct related abnormalities were found in 12/26 (46.15%) AIP and 10/40 (25.00%) PC patients, respectively. Incidentally, salivary and prostate gland SUVmax in AIP patients were higher compared with those of PC patients (p < 0.05). In males,an inverted “V” shaped high FDG uptake in the prostate was more frequent in AIP than PC patients (56.00%, 14/25 vs. 5.71%, 2/35). Increased FDG activity in extra-pancreatic bile duct was present in 4/26 of AIP patients, while was observed in none of the PC patients. Only in AIP patients, both diffuse pancreatic FDG accumulation and increased inverted “V” shaped FDG uptake in the prostate could be found simultaneously.Conclusions18F-FDG PET/CT findings might help differentiate AIP from PC.

Impact of intrapancreatic or extrapancreatic bile duct involvement on survival following pancreatoduodenectomy for common bile duct cancer

The clinicopathological factors that influence survival following pancreatoduodenectomy (PD) for common bile duct (CBD) cancer are not well known. This study aimed to investigate the effect of tumour involvement of the intrapancreatic versus extrapancreatic CBD on margin status, overall (OS) and disease-free (DFS) survival. This was a retrospective study of patients who underwent PD for CBD cancer between 2001 and 2009. Pathological examination was performed according to a previously described standardized protocol based on axial slicing. Clinicopathological data and outcome in terms of margin status, DFS and OS were compared between cancers involving exclusively the intrapancreatic CBD (CBDin) and those involving the extrapancreatic CBD, in isolation or combined with invasion of the intrapancreatic part of the duct (CBDex). A total of 66 patients were enrolled. Most CBD cancers were locally advanced (97 per cent pathological (p) T3, 76 per cent pN1). Microscopic margin involvement (R1) was more frequent in CBDex than in CBDin cancers (34 of 39 versus 13 of 27; P = 0.001), more often multifocal (P < 0.001) and more frequently affected the periductal margin (P = 0.005). Venous resection was more often required for CBDex cancers (P = 0.009). CBDex cancers were associated with worse OS (median 21 versus 28 months; P = 0.020) and DFS (14 versus 31 months; P = 0.015), but the rate and site of recurrence did not differ. Metastasis to more than two lymph nodes was an independent predictor of OS and DFS. CBDex cancer is associated with a higher rate of R1 resection and venous resection after PD, and has a worse outcome than CBDin cancer.

Clinical Significance of Extrapancreatic Lesions in Autoimmune Pancreatitis

To clarify the frequency and clinical significance of extrapancreatic lesions in autoimmune pancreatitis (AIP). The frequency and clinical characteristics of extrapancreatic lesions during the clinical course of AIP were investigated retrospectively in 64 patients with AIP. The predictive factors for relapse of AIP at clinical onset were also examined. Extrapancreatic lesions occurred in 95% (61/64) during the clinical course of AIP. The frequencies of sclerosing cholangitis, sclerosing sialadenitis, retroperitoneal fibrosis, and mediastinal or hilar lymphadenopathy were 84% (54/64), 23% (15/64), 16% (10/64), and 77% (27/35), respectively. Patients with sclerosing sialadenitis or extrapancreatic bile duct sclerosing cholangitis had a significantly higher serum immunoglobulin G concentration than those without (P = 0.005 and P = 0.016, respectively). Univariate analysis revealed that sclerosing sialadenitis (P = 0.005), diffuse pancreatic ductal changes (P = 0.028), and a high serum immunoglobulin G concentration (P = 0.030) at clinical onset of AIP were significant predictive factors for relapse. Multivariate analysis revealed that diffuse pancreatic ductal changes (P = 0.005) and sclerosing sialadenitis (P = 0.012) were significant independent predictive factors for relapse of AIP. The frequency of extrapancreatic lesions with AIP during the clinical course was high. The presence of sclerosing sialadenitis at clinical onset is a significant predictive factor for relapse of AIP.

Pancreatitis autoinmune asociada a fibrosis retroperitoneal: evolución tras dos años de seguimiento

Autoimmune pancreatitis is a kind of chronic pancreatitis characterized by the presence of lymphoplasmacytic infiltration and severely elevated serum IgG and IgG4, which has been associated to many extrapancreatic lesions and other autoimmune disorders, leading to the theory of an autoimmune mechanism involved in the pathogenesis of this disease. We report the case of a man who simultaneously presented with autoimmune pancreatitis associated with retroperitonal fibrosis, and a lesion of the extrapancreatic bile duct, with total response to corticosteroid treatment for 4 months and absence of recurrence after 24 months of follow-up. Autoimmune pancreatitis is a kind of chronic pancreatitis that is probably a part of a systemic autoimmune disease, with retroperitoneal fibrosis and extrapancreatic bile duct lesion being the most commonly associated extrapancreatic lesions. A correct diagnosis and early treatment of this disease may aid in the total resolution of lesions, especially in cases with a low activity grade.

A case of sclerosing cholangitis with autoimmune pancreatitis evaluated by FDG-PET

The extrapancreatic bile duct lesions in autoimmune pancreatitis are termed sclerosing cholangitis (SC with AIP), which is known to complicate AIP somewhat more frequently than other extrapancreatic lesions. In cases of SC with AIP, differentiation from primary SC, pancreatic cancer, and bile duct cancer is often difficult. In our patient, pancreatic cancer had to be ruled out at admission, given the findings of obstructive jaundice, pancreatic duct stenosis, and swelling of the pancreas. Fluorine-18-fluorodeoxyglucose positron emission tomography was useful in checking for the presence of extrapancreatic lesions, including SC, and was also useful in the evaluation of the response to steroid therapy for following the course of AIP.

Long-term prognosis of autoimmune pancreatitis with and without corticosteroid treatment

Objectives:Autoimmune pancreatitis (AIP) is a unique form of chronic pancreatitis, and has a favourable response to corticosteroid treatment (CST). Little is known, however, about the long-term outcome of AIP. This...

Prevalence and distribution of extrapancreatic lesions complicating autoimmune pancreatitis

Autoimmune pancreatitis is a unique form of chronic pancreatitis characterized by high serum IgG4 concentrations and abundant IgG4-bearing plasma cell infiltration in the pancreatic lesion, and it has been reported to be associated with a variety of extrapancreatic lesions, leading us to postulate the concept of a systemic inflammatory disease. To confirm this, we clarified the exact distribution of these extrapancreatic lesions and provide a panoramic view of them. The frequency, distribution, clinical characteristics, and pathology of five extrapancreatic lesions were determined in 64 patients with autoimmune pancreatitis by examining clinical and laboratory findings. The most frequent extrapancreatic lesion was hilar lymphadenopathy (80.4%), followed by extrapancreatic bile duct lesions (73.9%), lachrymal and salivary gland lesions (39.1%), hypothyroidism (22.2%), and retroperitoneal fibrosis (12.5%). No patients had all five types of lesions. Patients with hilar lymphadenopathy or lachrymal and salivary gland lesions were found to have significantly higher IgG4 levels than those without (P = 0.0042 and 0.0227, respectively). Patients with three lesions were found to have significantly higher IgG4 levels than those with no lesion, suggesting that patients with multiple extrapancreatic lesions have active disease. Similar to pancreatic lesions, extrapancreatic lesions have a characteristic histological finding of abundant IgG4-bearing plasma cell infiltration, and they respond favorably to corticosteroid therapy. Autoimmune pancreatitis was recognized as a systemic inflammatory disease. Furthermore, recognition of these characteristic findings will aid in the correct diagnosis of this disease.

Extrapancreatic Lesions in Autoimmune Pancreatitis

To examine extrapancreatic lesions associated with autoimmune pancreatitis. Autoimmune pancreatitis is a unique clinical entity proposed recently, and is reported to occasionally be associated with other autoimmune diseases. Extrapancreatic lesions were examined clinically, radiologically, and histologically in 25 patients with autoimmune pancreatitis. Stenosis of the bile duct was observed in 22 patients (lower bile duct [n = 19], upper bile duct [n = 1], intrahepatic bile duct [n = 2]). Marked extrapancreatic bile duct wall thickening not associated with obvious cholangiographic abnormality was seen on ultrasound in 3 patients. Enlargement of the salivary glands and cervical lymph nodes was detected in 4 patients. Abdominal lymphadenopathy was observed in 5 of 8 patients at laparotomy. Retroperitoneal fibrosis was noted in 2 patients. Obliterative phlebitis of the pancreatic and peripancreatic veins was observed histologically in all 6 resected specimens. Marked stenosis of the portal vein and encasement of the peripancreatic arteries was observed in 4 and 8 of 14 patients who underwent abdominal angiography, respectively. Diabetes mellitus was diagnosed in 13 patients. All associated extrapancreatic lesions except diabetes mellitus improved after steroid therapy. Extrapancreatic lesions found to be occasionally associated with autoimmune pancreatitis were stenosis of the bile duct, enlargement of the salivary glands, abdominal or cervical lymphadenopathy, retroperitoneal fibrosis, stenosis of the peripancreatic arteries or portal vein, and diabetes mellitus. It is possible that these lesions are induced by the same inflammatory mechanisms as autoimmune pancreatitis.

Involvement of the biliary system in autoimmune pancreatitis: a follow-up study

Background & Aims : The aim of this study was to define the bile duct changes associated with autoimmune pancreatitis. Methods : Eight patients with autoimmune pancreatitis were followed for a mean of 4 years. The clinical features of these patients, including extrapancreatic bile duct changes, were examined by using biochemical parameters and several imaging modalities. Pathologic features of the pancreas and liver were examined by using the biopsy specimens of 7 patients. Results : Diffuse or focal narrowing of the main pancreatic duct was observed in all patients. Histologic examination of the pancreas showed lymphoplasmacyte infiltration with severe fibrosis and acinar cell depletion. In 6 patients extrapancreatic bile duct changes such as stricture of the bile duct at hilus or intrahepatic area were observed. In 2 patients abnormalities in the bile duct and pancreas were detected simultaneously at diagnosis, and changes in the bile duct were observed later in 4 patients. Lymphoplasmacyte infiltration and fibrosis were observed in the portal area of all 7 liver biopsy samples. Five of the patients with bile duct changes received steroid therapy, and the pathological changes improved. Conclusions : Extrapancreatic bile duct changes are frequently associated with autoimmune pancreatitis. Similar pathogenic mechanism might produce the biliary tract and pancreatic abnormalities in autoimmune pancreatitis resulting in a similar histopathology in the liver and pancreas and response to steroid therapy.

Clinico-pathological features of extrapancreatic bile duct abnormalities observed in autoimmune pancreatitis

Clinico-pathological features of extrapancreatic bile duct abnormalities observed in autoimmune pancreatitis

Cell proliferation kinetics of the bile duct epithelium of the rat

Cell proliferation kinetics of the extrahepatic bile duct were studied by flash and cumulative labelling methods and immunohistochemical techniques. We compared the cell kinetics of the epithelium of the intra- and extra-pancreatic bile ducts and of the bile duct of the ampulla in rats administered intraperitoneally with bromodeoxyuridine (BrdUrd). After a single injection of BrdUrd (flash labelling), labelled cells appeared in the lower portion of the downgrowths of the epithelium in the intra- and extra-pancreatic bile ducts. A gradual accumulation of the labelled cells at the surface epithelium was observed during the cumulative labelling. After cumulative labelling the labelled cells gradually decreased in number and were finally confined to the degenerative cell zone of the surface epithelium 30 days later. Similarly, after a single injection of BrdUrd, the labelled cells in the bile duct of the ampulla appeared at the lower half of the crypt from where they migrated to the upper portion during cumulative labelling. These findings indicate that epithelial cells of the bile duct are renewed at the lower portion of the downgrowths of the epithelium, or crypt, and shed from the surface epithelium or upper portion of the fold. The labelling indices reached 23.83 +/- 7.47% in the intra-pancreatic bile duct, 14.74 +/- 7.99% in the extra-pancreatic bile duct and 43.42 +/- 4.40% in the bile duct of the ampulla at the end of 70 h cumulative labelling. The fluctuating values of the labelling index were higher in the bile duct of the ampulla than in the intra- or extra-pancreatic bile ducts. These results indicate that the bile-duct epithelium undergoes a slower renewal rate than the other parts of the gastrointestinal tract, and that the renewal time of the epithelial cells is shorter at the bile duct of the ampulla than at the intra- or extra-pancreatic bile ducts.