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Background: There has been a progressive increase in the use of percutaneous cholecystostomy (PC) in acute cholecystitis (AC) over the last decades due to population aging, and the support of guidelines (Tokyo Guidelines (TG), World Society of Emergency Surgery (WSES) Guidelines) as a valid therapeutical option. However, there are many unanswered questions about the management of PCs. An international consensus on indications and PC management using Delphi methodology with contributions from experts from three surgical societies (EAHPBA, ANS, WSES) have been performed. Methods: A two-round Delphi consensus, which included 27 questions, was sent to key opinion leaders in AC. Participants were asked to indicate their 'agreement/disagreement' using a 5-point Likert scale. Survey items with less than 70% consensus were excluded from the second round. For inclusion in the final recommendations, each survey item had to have reached a group consensus (≥ 70% agreement) by the end of the two survey rounds. Results: 54 completed both rounds (82% of invitees). Six questions got > 70% and are included in consensus recommendations: In patients with acute cholecystitis, when there is a clear indication of PC, it is not necessary to wait 48 h to be carried out; Surgery is the first therapeutic option for the TG grade II acute cholecystitis in a patient suitable for surgery; Before PC removal a cholangiography should be done; There is no indication for PC in Tokyo Guidelines (TG) grade I patients; Transhepatic approach is the route of choice for PC; and after PC, laparoscopic cholecystectomy is the preferred approach (93.1%). Conclusions: Only six statements about PC management after AC got an international consensus. An international guideline about the management of PCs are necessary. [ABSTRACT FROM AUTHOR]

Biliary stent insertion is a well-established treatment of bile duct obstruction. Stent migration is a late-onset complication and can be life-threatening. We present a case of proximal biliary stent migration to the portal vein. An upper endoscopy was performed, and the biliary stent was retracted. Bleeding was seen from the papilla of Vater. The hepatoduodenal ligament was explored, and the common bile duct was found fully adherent to the portal vein, with a fistula between the common bile duct and the portal vein. The patient underwent surgery with extrahepatic bile duct resection and hepaticoduodenostomy. The patient survived and was discharged without surgical postoperative complications on postoperative Day 8. [ABSTRACT FROM AUTHOR]

Postoperative biliary complications following extensive hepatic resections are complex, often requiring a multidisciplinary team approach. We describe a case of a free bile duct leak following extended right hepatectomy and surgical hepaticojejunostomy treated with percutaneous transhepatic hepaticojejunostomy in which a radiofrequency guidewire was used to gain enteral access. A modified internal/external biliary catheter was left in place. The patient was enrolled in a benign biliary stricture protocol, and 8 months later, the catheter was removed following a normal cholangiogram and biliary manometric perfusion testing. At 3-month follow-up after catheter removal, the patient is asymptomatic with no clinical, biochemical, or radiographic evidence of biliary leak or obstruction. [ABSTRACT FROM AUTHOR]

Background Endoscopic retrograde cholangiopancreatography (ERCP) is currently the method of choice for the postoperative evaluation of suspected bile duct stones seen on intraoperative cholangiogram (IOC); however, the sensitivity of IOC for identifying biliary pathology is unclear, with studies reporting false positive rates between 30% and 60%. Objective Evaluate the sensitivity of IOC for biliary pathology, using ERCP with sphincterotomy and balloon sweep as gold standard. Design Retrospective cohort study. Setting Tertiary medical centre. Patients 130 consecutive patients (age 51.3 ±1.7 years, 69.2% women) who underwent ERCP for the evaluation of abnormalities identified on IOC between 2005 and 2013. Interventions Endoscopic retrograde cholangiopancreatography. Main outcome measurements Sensitivity of IOC, identify predictors of positive postoperative ERCP and ERCP-related complications. Results ERCP was successful in all 130 subjects. ERCP-related adverse events occurred in six (4.3%) patients, including self-limited postsphincterotomy bleeding in three (2.3%) and mild post-ERCP pancreatitis in three (2.3%). Overall, 41 (31.5%) patients had normal cholangiogram at time of ERCP. Finding of a filling defect on IOC was the only predictor for the presence of common bile duct stones on postoperative ERCP (OR 3.3, 95% CI 1.0 to 10.8, p=0.05). Limitations Retrospective study design. Conclusions Nearly one-third of patients with abnormal IOC had a normal postoperative ERCP. Significant pathology could have been missed in 1/130 patients. Based on these findings, we believe the use of less-invasive diagnostic modalities may be used in place of ERCP in patients with suspected choledocholithiasis on IOC. [ABSTRACT FROM AUTHOR]

Autoimmune hepatitis (AIH) is a chronic, relapsing and remitting, immune-mediated liver disease that progresses to cirrhosis if left untreated. A significant number of patients may present with acute hepatitis or acute liver failure, which are often misdiagnosed as toxic liver injury. AIH shows a preponderance in young women but may be seen in children and the elderly. Diagnosis requires the integration of clinical, biochemical, and serologic parameters, along with supportive liver histology and exclusion of other causes of liver disease. Liver biopsy is a prerequisite for diagnosis of AIH, to assess severity and stage of disease, exclude other entities, and recognize any concurrent morbidities. No single biomarker or histologic feature is pathognomonic for AIH. The diagnostic and histologic criteria have undergone several modifications since the original scoring system was proposed by the International Autoimmune Hepatitis Group (IAIHG) in 1993. Recently, the IAIHG has proposed consensus recommendations for histologic criteria, relevant for both acute and chronic AIH. This review article will describe the evolving diagnostic criteria for AIH, with their limitations and utility, and with an emphasis on the role of liver histology in the diagnosis and management of AIH. [ABSTRACT FROM AUTHOR]

Inflammatory bowel disease (IBD) is frequently associated with extraintestinal manifestations such as hepatopancreatobiliary manifestations (HPBMs), which include primary sclerosing cholangitis (PSC), pancreatitis, and cholelithiasis. PSC is correlated with IBD, particularly ulcerative colitis (UC); 70-80% of PSC patients in Western countries and 20-30% in Japan have comorbid UC. Therefore, patients diagnosed with PSC should be screened for UC by total colonoscopy. While symptoms of PSC-associated UC are usually milder than PSC-negative UC, these patients have a higher risk of colorectal cancer, particularly in the proximal colon. Therefore, regular colonoscopy surveillance is required regardless of UC symptoms. Administration of 5-aminosalicylic acid or ursodeoxycholic acid may prevent colorectal cancer and cholangiocarcinoma. While PSC is diagnosed by diffuse multifocal strictures on cholangiography, it must be carefully differentiated from immunoglobulin G4 (IgG4)-associated cholangitis, which shows a similar cholangiogram but requires different treatment. When PSC is suspected despite a normal cholangiogram, the patient may have small-duct PSC, which requires a liver biopsy. IBD patients have a high incidence of acute and chronic pancreatitis. Most cases are induced by cholelithiasis or medication, although some patients may have autoimmune pancreatitis (AIP), most commonly type 2 without elevation of serum IgG4. AIP should be accurately identified based on characteristic image findings, because AIP responds well to corticosteroids. Crohn's disease is frequently associated with gallstones, and several risk factors are indicated. HPBMs may influence the management of IBD, therefore, accurate diagnosis and an appropriate therapeutic strategy are important, as treatment depends upon the type of HPBM. [ABSTRACT FROM AUTHOR]

Children with autoimmune hepatitis (AIH) often exhibit particular features. Accordingly, seven pediatric-specific criteria have been proposed. To develop a prediction model based on them, transform it into a scoring system and study its accuracy. A cohort of children under study for liver disease was consecutively selected. AIH diagnosis was based on classical criteria. Already proposed pediatric criteria were recorded. The best possible regression model was selected, and the beta coefficient of each criterion was translated into a whole number (points). Total scores were obtained following the points system and the best cut-off was calculated. Subsequently, accuracy of the diagnostic score was studied in the validation set. Among 212 included patients, 100 had AIH. The score included 5 criteria: autoantibodies (0–2 points), hypergammaglobulinemia, exclusion of viral hepatitis, exclusion of Wilson's disease (1 point each) and liver histology (3 points). In addition, a normal cholangiogram is mandatory. The validation set was formed of 70 patients (24 with AIH). In this subsample, a score of ≥6 renders a sensitivity/specificity of 95.8%/100%. The area under the receiver operating characteristic curve was 97.1%. Pediatric-specific criteria for the diagnosis of AIH can be reliably used as a scoring system. [ABSTRACT FROM AUTHOR]

Background and aims: Information about the natural history of small duct primary sclerosing cholangitis (SDPSC) remains scant despite literature suggesting that it constitutes 6-16% of all cases of primary sclerosing cholangitis (PSC). We combined clinical data on SDPSC cases from two tertiary care institutions with liver transplantation programs with the aim of studying the natural history of SDPSC. Methods: Medical records of 25 individuals with SDPSC were reviewed. Diagnosis of SDPSC was based on liver biopsy findings consistent with PSC, a normal cholangiogram, and elimination of known causes of secondary sclerosing cholangitis. Demographic information, symptoms, past medical history, laboratory values, and histologic data were evaluated. Our primary outcome measure was liver transplantation or death. Secondary outcome measures included evidence of end-stage liver disease, development of cholangiocarcinoma, and/or the development of classic PSC on a repeat cholangiogram. Results: Data on 25 individuals (13 males, 12 females; mean age 40 ± 15 years) diagnosed with SDPSC were analyzed. Upon presentation, 11 patients had symptoms including abdominal pain, fatigue, and pruritus. Inflammatory bowel disease was present in 14 patients (56%) at diagnosis. On initial liver biopsy, 60% had early-stage disease (I or II) and none had cirrhosis. On follow-up (1-168 months, median 17 months), malignancy or progression to classic large duct PSC was not noted. Two (8%) patients had evidence of varices and one of the two also developed ascites; one of these patients underwent liver transplantation and the other one died due to sepsis. Conclusions: SDPSC, a mild disease at presentation typically runs a benign course and likely is not an early stage of classic PSC. Further studies with a control group of classic PSC and longer follow-up are needed to study the natural history of SDPSC. [ABSTRACT FROM AUTHOR]

Jejunal access loop is fashioned in patients who undergo Roux en Y hepaticojejunostomy and biliary intervention is anticipated on follow up. Post-operative study of the biliary tree through the access loop is usually done under fluoroscopic guidance. We present a series of 20 access loop cholangiograms performed in our institution between August 2004 and November 2008. We aimed to evaluate the safety and efficacy of the procedure and to highlight the role of CT guidance in procuring access. Access loop was accessed using CT ( n = 13), ultrasound ( n = 3) or fluoroscopic guidance ( n = 4). Fluoroscopy was used for performing cholangiograms and interventions. Twelve studies had balloon plasty of the stricture at anastomotic site or high up in the hepatic ducts. Seven studies showed normal cholangiogram. Plasty was unsuccessful in one study. Technical success in accessing the jejunal access loop was 100%; in cannulation of anastomotic site and balloon plasty it was 95%. One case required two attempts. Procedure-related complications were not seen. All patients who underwent balloon plasty of the stricture were doing well for variable lengths of time. Access loop cholangiogram and interventions are safe and effective. CT guidance in locating/procuring the access loop is a good technique. [ABSTRACT FROM AUTHOR]

The utility of 1H MR spectroscopy in detecting chronic cholestasis has been investigated. The amide proton region of the 1H MR spectrum of human bile plays a major role in differentiating cholestatic (Ch) patterns from the normal ones. Bile obtained from normal bile ducts contains both taurine and glycine conjugates of bile acids - cholic acid (CA), chenodeoxycholic acid (CDCA), and deoxycholic acid (DCA). Absence of a glycine-conjugated bile acid glycochenodeoxycholic acid (GCDCA) has been observed in bile samples obtained from primary sclerosing cholangitis (PSC) patients. A total of 32 patients with various hepatobiliary diseases were included in the study. Twenty-one patients had PSC and 11 had normal cholangiograms. One PSC patient was excluded from the study because of a bad spectrum. Seventeen out of the 20 PSC patients showed an absence of GCDCA in their 1H MR spectrum of bile. Six of the 11 reference patients with normal cholangiogram also showed spectra similar to those of PSC, indicating the possibility of cholestasis. DQF-COSY and TOCSY experiments performed on bile samples from PSC patients also revealed absence of phosphatidylcholine (PC) in some of the bile samples, suggesting possible damage to the cholangiocytes by the toxic bile. These observations suggest that analysis of human bile by 1H MRS could be of value in the diagnosis of chronic Ch liver disorders. Copyright © 2008 John Wiley & Sons, Ltd. [ABSTRACT FROM AUTHOR]

Background: Patients with cholestatic liver function tests and histological features of primary scleroslng cholangitis (PSC) but a normal cholangiogram are considered to have small duct PSC. The natural history of this condition is unknown. Methods: Thirty three patients with small duct PSC were identified among patients admitted for diagnostic workup of cholestatic liver function tests in one centre in the UK (Oxford) and one centre in Norway (Oslo). A total of 260 patients with large duct PSC were compared, and prognosis in terms of death, cholangiocarcinoma, biochemical features, histological features, and symptoms analysed. Results: Mean age at diagnosis was 38 years and 39 years in small duct and large duct PSC, respectively. Mean follow up was 106 months in small duct and 105 months in large duct patients. Four patients originally considered to have small duct developed large duct PSC. Two of these underwent liver transplantation during follow up. Of the remainder who did not develop large duct PSC, two patients died during follow up: one of liver failure and the other of cardiac death unrelated to her liver disease. A total of 122 (47%) large duct patients either required liver transplantation (34 patients) or died (88 patients). Small duct patients had a significantly better survival compared with large duct patients. Among small duct patients, none developed cholangiocarcinoma compared with 28 of 260 (11%) large duct patients. Conclusions: Patients with small duct PSC seem to have a good prognosis in terms of survival and development of cholangiocarcinoma. Small duct PSC progresses to large duct PSC in a small proportion of patients. [ABSTRACT FROM AUTHOR]

AbstractBackground and Aim: The role of endoscopic retrograde cholangiopancreatography (ERCP) is not yet fully established in children. The purpose of this study was to assess the use of ERCP in the diagnosis and management of various pancreaticobiliary disorders in children. Methods: Eighty-four ERCPs were performed over 5.5 years in 72 children with suspected pancreaticobiliary tract disorders with an adult-type duodenoscope. In all cases, indications, procedure time, ERCP findings, complications, patients course and therapeutic intervention (if any) were recorded. Results: The mean (± SD) age of these children was 8.8 ± 3.3 years. Successful cannulation was possible in 70 (97%) cases. Of the 44 cases with suspected biliary tract disease, 14 had a choledochal cyst, 13 had portal biliopathy, two each had CBD stones, primary sclerosing cholangitis and a bile leak, one had biliary ascariasis, eight had a normal cholangiogram, and CBD cannulation failed in two. Eight of the 28 children with suspected pancreatic disorders had chronic pancreatitis, five had pancreatic duct disruption, three had pancreas divisum and the rest had a normal pancreatogram (including all eight children with unexplained abdominal pain). Therapeutic ERCP was performed in 22 children, endoscopic nasobiliary or a nasocystic drain was placed in 16, biliary stenting was conducted in two, pancreatic duct stenting was conducted in three, and minor papilla dilation was conducted in one child. Six children had mild procedure-related complications. Conclusion: Endoscopic retrograde cholangiopancreatography is very useful in the treatment of cholangitis, bile leak, pseudocyst and pancreatic fistulae in children. However, its role in unexplained abdominal pain is doubtful. [ABSTRACT FROM AUTHOR]

Background: To improve knowledge on endoscopic retrograde cholangiopancreatography (ERCP) in children, we aimed to study the proportion of indications, success rate and complication of ERCP. Methods: We performed a systematic search of all articles published up to December 2022 in the following databases: Cochrane Library, PubMed (MEDLINE) and Scopus. The meta-analysis was performed using a random-effects model. Heterogeneity was determined by the I2 statistics and the Cochrane Q test. The included data were analyzed to identify the proportion of indications, success rate and complications of ERCP in children. Results: Based on data from 52 studies with a total of 5624 participants, the most common indications for ERCP in children were biliary [48% (95% CI: 0.40 - 0.57; I2 = 98.17%, P < 0.001)] and both biliary and pancreatic [41% (95% CI: 0.33 - 0.49; I2 = 98.27%, P < 0.001)]. The success rate of ERCP was 95% (95% CI: 0.94 - 0.96; I2 = 82.53%, P < 0.001) with the overall complication rate of 7% (95% CI: 0.05 - 0.09; I2 = 82.06%, P < 0.001). The pooled estimate for the incidence of post ERCP pancreatitis was 4% (95% CI: 0.03 - 0.06; I2 = 85.46%, P < 0.001) and the bleeding was 0% (95% CI: 0.0 - 0.0; I2 = 28.21%, P = 0.03). Conclusions: ERCP appears to be performed safely in children with a similar success rate as in the adult population. [ABSTRACT FROM AUTHOR]

Twelve of 178 (7%) liver transplant patients underwent endoscopic retrograde cholangiopancreatography (ERCP) after transplantation. The indications for ERCP were persistent or late onset cholestasis, recurrent cholangitis, and suspected biliary leaks or strictures. The time between transplantation and ERCP ranged from 44 to 330 days (median 153 days). Biliary complications diagnosed by ERCP included biliary sludge in the form of casts, calculi, or debris (n = 7); bile leaks (n = 2); a biliary stricture (n = 1), and complete biliary obstruction (n = 1). One patient had a normal cholangiogram after transplantation. Biliary sludge was detected by ultrasound before ERCP in only one of six patients. Eight patients underwent endoscopic papillotomy, followed by clearance of biliary sludge in four and dilatation of a biliary stricture in one. Two patients bled after papillotomy but neither required surgical intervention. At a median follow up of 1.2 years (range 0.5-2.8 years), nine patients are well and three have died. ERCP provides both accurate diagnosis of biliary complications after liver transplantation and treatment that obviates the need for additional surgery in selected patients. [ABSTRACT FROM PUBLISHER]

In order to clarify the pathogenesis and process of the formation of intrahepatic cholesterol gallstones, we examined the clinical features, cholangiograms and pathological findings of eight patients with intrahepatic cholesterol gallstones. When examining the clinical features, one patient was found to have developed intrahepatic cholesterol gallstones 3 years after a complete lithotomy. The cholangiograms of two patients revealed small gallstones in the peripheral bile ducts of the lateral segment of the liver, and these bile ducts showed localized cystic dilatation and were tightly filled with gallstones. Conversely, their other bile ducts which contained no gallstones showed an entirely normal cholangiogram. Pathologically, these two cases showed mild chronic cholangitis, and cholesterol crystals in the peripheral bile ducts. The other six cases showed moderate or severe dilatation of the bile duct and severe chronic proliferative cholangitis. From the above results, we proposed the following theory to explain the pathogenesis and process of the formation of intrahepatic cholesterol stones: The cholesterol crystals in the peripheral intrahepatic bile ducts may be a primitive form of intrahepatic cholesterol gallstones, and the formation of intrahepatic cholesterol gallstones may precede and cause such deformities of the bile ducts as strictures or dilatations. [ABSTRACT FROM AUTHOR]

Thirty-nine patients (58%) received surgical management, 19 patients (28%) had surveillance, 7 patients (10%) had medical therapy and 2 patients (3%) had no further follow up. HP020P ESTABLISHING A PILOT ENHANCED RECOVERY PATHWAY FOR COMPLEX UGI/HPB PATIENTS: A SINGLE CENTRE... Georges Tinawi, Nick Rogers and Yukai Lim Wellington Regional Hospital, Wellington, Aotearoa New Zealand B Introduction b : Enhanced recovery pathways in complex UGI/HPB surgery have been shown to improve patient outcomes. PREOPERATIVE DIABETES MELLITUS AND KEY PANCREATODUODENECTOMY OUTCOMES: IS THE TIMING OF THE D... Daniel Croagh and Raw Study Collaborators Monash Health, Victoria B Background: b Pancreatoduodenectomy (PD) remains a high-risk operation. Four patients had a lesion associated with the ductal system ( I n i = 4), three patients had a cystic liver lesion ( I n i = 3), and one patient had a FDG-avid hepatic lesion ( I n i = 1). Recurrent disease was identified in 11.6% of all patients, but notably, no patient scoring clearly for cholecystectomy suffered recurrent pancreatitis following surgery. [Extracted from the article]

Patients with cholestatic liver function tests and histological features of primary sclerosing cholangitis (PSC) but without the typical cholangiographic changes are considered to have small-duct PSC. The incidence of small-duct PSC and the natural history still is not known. We performed a retrospective search for patients diagnosed with small-duct PSC between January 1997 and December 2003. The diagnosis of small-duct PSC was based on biochemical features of chronic cholestasis, liver biopsy findings consistent with PSC, and a normal cholangiogram on endoscopic retrograde cholangiography. Six patients fulfilled the diagnostic criteria for small-duct PSC. All patients received medical therapy. After a mean follow-up time of 26.0 ± 29.8 months (range, 7–84 months), all patients are alive. Repeated liver biopsy was performed in one patient, 58 months after the initial one, and disclosed amelioration of histological findings (reduction in the Ludwig fibrosis score from 4 to 2). During follow-up symptoms disappeared in all patients who were symptomatic at diagnosis; none of those who were asymptomatic at diagnosis developed symptoms. At the time of last follow-up all patients showed significant improvement of their biochemical variables compared to baseline. Administration of aminosalicylates seemed to be of benefit irrespective of the presence of inflammatory bowel disease. No patients underwent liver transplantation or developed cholangiocarcinoma. Even though our study included a low number of patients and the follow-up time was relatively short, we can suggest that small-duct PSC rarely progresses to large-duct PSC and does not seem to be associated with development of cholangiocarcinoma. It thus seems to represent a separate entity with a favorable prognosis. [ABSTRACT FROM AUTHOR]

Spontaneous perforation of the biliary tract is rare in infants & children with less than 100 cases reported in English Literature till date. We report two cases of this rare clinical entity in a 9-month-old boy and other of a 2-year old boy. Both patients presented with clinical features of acute peritonitis. Laparotomy revealed sterile biliary peritonitis with a normal cholangiogram in the infant and biliary perforation in other child. Simple drainage in the former and cholecystostomy with external biliary drainage in later resulted in a satisfactory outcome. [ABSTRACT FROM AUTHOR]

This article discusses the use of endoscopic retrograde cholangiopancreatography (ERCP) in patients with abnormal intraoperative cholangiograms (IOC) during laparoscopic cholecystectomy for gallstone disease. The study found that ERCP is not necessary in all patients with abnormal IOC findings, as approximately one-third of ERCPs performed for abnormal IOC were normal. The authors suggest that performing magnetic resonance cholangiopancreatography (MRCP) prior to ERCP may be a more prudent approach, as MRCP was found to be an independent predictor of positive ERCP. The study also highlights the risks associated with ERCP, including serious complications such as pancreatitis. [Extracted from the article]

Background and Aims: Patients with pediatric‐onset primary sclerosing cholangitis (PSC) are at risk of developing hepatic complications with liver transplantation as only curative treatment. Complications usually occur over many years, underlining the need for reliable surrogate markers to predict the clinical course. Recently, gamma‐glutamyl transferase (GGT) has been suggested to allow prediction of the clinical course. In a single‐center cohort study, we tested the potency of GGT in this respect. Methods: We used longitudinal data of patients from our academic center, diagnosed with pediatric‐onset PSC between 2000 and 2020. Patients with a GGT decrease from baseline >25% (n = 36) were compared with those who did not have this decrease (n = 7). We performed Kaplan–Meier analysis and log‐rank testing to assess the occurrence of portal hypertensive or biliary complications, hepatobiliary malignancies, liver transplantation, or death. Results: The median age diagnosis was 15.2 years and 12.1 years in the group with ≤25% decrease of GGT and the group with >25% decrease, respectively (p = 0.078). The probability of developing ≥1 complications in the first 5 years after diagnosis was 50% in the group with ≤25% decrease of GGT and 20% in the group with >25% decrease of GGT (p = 0.031). The use of medication was not associated with the development of complications. Conclusion: In a retrospective cohort study, we report that a GGT decrease of >25% within 1 year of diagnosis of pediatric‐onset PSC is associated with a lower occurrence of complications within 5 years. Our results provide further support for the recently hypothesized predictive value of first‐year GGT change in predicting the disease course in pediatric‐onset PSC. [ABSTRACT FROM AUTHOR]

Background/Aims Small duct primary sclerosing cholangitis is a rare clinical condition that may remain unrecognized. Diagnosis rests on clinical, biochemical, and histologic features consistent with primary sclerosing cholangitis but with normal cholangiogram findings. We report here a case of small duct primary sclerosing cholangitis in a patient who presented with jaundice. Methods Clinical Presentation. A 22-year-old Filipino gentleman with no known comorbids came in due to a 2-week history of jaundice with elevated transaminases, bilirubin and alkaline phosphatase. No other symptoms were noted. Hepatitis markers were all normal. Physical examination was unremarkable except for jaundice. Results Management. Magnetic resonance cholangiopancreatography was normal with no evidence of dilated intra- and extrahepatic ducts. Liver biopsy showed intrahepatic cholestasis with periductal fibrosis. Ursodeoxycholic acid was started which showed significant decrease in jaundice with improvement in biochemistries. Conclusions Recommendation. Small duct primary sclerosing cholangitis appears to have a more favorable prognosis than classic primary sclerosing cholangitis. However, further studies with long-term follow up are recommended to determine disease progression. [ABSTRACT FROM AUTHOR]