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The surgical techniques most frequently used for biliary reconstruction are end-to-end choledocho-choledochostomy over a T-tube, choledocho-jejunostomy to a Roux-en-Y loop and, especially Germany, side-to-side choledocho-choledochostomy [1, 8, 14, 21]. techniques employed in the early years of liver transplantation, e.g., cholecysto-enterostomy of the gallbladder conduit (Waddell-Calne), have now been generally abandoned. We have used end-to-end and side-to-side choledocho-choledochostomy as well as biliodigestive reconstruction with similar results [29]. The technique we currently prefer side-to-side anastomosis.

Yield of Repeat ERCP for Persistently Elevated LFTs After a Previously Normal Cholangiogram in Liver Transplant Patients with Choledochocholedochostomy Anastomosis Evan L. Fogel, Lee McHenry Jr., James L. Watkins, Suzette Schmidt, A. J. Tector, Jonathan A. Fridell, Stuart Sherman, Glen A. Lehman Background: Biliary tract complications occur in 20-30% of patients after orthotopic liver transplantation (OLT), with bile leaks and anastomotic strictures most often identified. ERCP is generally the preferredmethod of evaluation, and is usually performed for persistently elevated or rising LFTs post-OLT. A normal ERC suggests an alternative explanation for the abnormal LFTs (eg. rejection). However, exclusion of other causes may prompt a repeat ERCP. This study examines the yield of a 2nd ERCP, performed after a previously normal exam, in the evaluation of persistently abnormal or rising LFTs. Methods: From 1/99-10/ 03, patients who had a previously normal post-OLT ERC (with or without prior endoscopic intervention) and underwent repeat evaluation for the above indications were identified from our ERCP database. The biliary anastomosis had been established by choledochocholedochostomy in all patients. Complications were graded according to consensus criteria. Results: Overall, 34 patients (who underwent 46 follow-up ERCPs) were identified, 31 of which had biliary continuity established without a T-tube. Twenty-seven ERCPs were performed in 22 patients who had no prior therapy (Group 1), while 19 ERCPs were performed in 12 patients who had prior intervention but had subsequently normalized their cholangiograms (Group 2). Themean interval between the initial normal ERCand the follow-up study was 5.5 months (range 0.5-24.0) in Group 1 and 5.3 months (range 0.3-29) in Group 2. The findings at the 2nd ERC are as noted in the Table. In both groups, 63% of patients had a persistently normal study, while 37% had new pathology identified. Two patients developed pancreatitis (4.3%; 1 mild, 1 moderate) and one patient had a mild post-sphincterotomy bleed (overall complication rate 6.5%). Summary: In over one-third of patients with a previously-normal post-OLT ERC, new findings (strictures, most often) were identified at repeat ERC performed for persistently elevated or rising LFTs. Conclusion: Repeat ERC can identify the cause of persistently abnormal LFTs in a subset of OLT patients despite a previously normal ERC, and should be considered when an alternative explanation is not identified.

Background: Patients with gallbladder stones presenting with jaundice, deranged liver function tests (LFTs), dilated ducts on imaging or pancreatitis as possible indicators of common bile duct (CBD) stones usually undergo ERCP. Except for pancreatitis, it is unclear whether prophylactic ES should be performed in patients with a normal cholangiogram, in whom cholecystectomy is not proposed. Aim: To determine if prophylactic ES can prevent further biliary problems if a cholecystectomy is not planned for patients with suspected CBD stones and a normal cholangiogram. Methods: Endoscopic databases at two centers (Royal Victoria and Ulster Hospitals) were searched for patients with gallbladder stones presenting with jaundice, deranged LFTs or dilated ducts on ultrasound (USS), in whom a cholecystectomy was not planned. Endoscopic findings were noted. A telephone questionnaire was performed on all patients, or their carers if possible, to determine the frequency of recurrent biliary problems or repeat investigations. One center routinely performed prophylactic ES while the other did not. Results from the two groups were compared. Results: 41 patients met the inclusion criteria of whom 20 (7 male) had a ES and 21 (12 male) did not. The frequency of pre-ERCP jaundice (14 v 12; p=0.3), deranged LFTs (18 v 16; p=0.22), abdominal pain (15 v 15; p=0.8), dilated ducts (9 v 12; p=0.39) did not differ between the two groups. Post- ERCP recurrent abdominal pain (5 v 3; p=0.39), jaundice (3 v 1; p=0.28), pancreatitis (0 v 1; p=0.32) and repeat USS (2 v 1; p=0.52) or ERCP (1 v 1; p=0.97) did not differ between the two groups. Conclusions: Patients with gallstones and suspected CBD stones and a normal cholangiogram should not have a prophylactic sphincterotomy as it did not reduce the incidence of recurrent biliary problems.

This paper describes the measurements of the normal cholangiogram as demonstrated by endoscopic retrograde cholangiopancreatography. Two groups of patients were studied. There were 49 patients who had normal livers and no evidence of biliary tract disease. In addition, there were 25 patients with known liver disease and an apparent normal extrahepatic biliary tract. The extrahepatic bile duct was measured in three regions: (1) the common hepatic duct (above the cystic duct and below the bifurcation); (2) the prepancreatic portion (below the cystic duct and above the pancreas); and (3) the interpancreatic portion (bile duct within the pancreas). These measurements ranged from 2.1 to 9.2 mm. Although the common bile ducts were generally quite uniform in caliber, there were instances where each portion was the largest diameter in an individual case. There was a slight increase in bile-duct caliber with age. This study showed somewhat smaller measurements than previously reported and may relate to case selection in this or previous studies.

Endoscopic retrograde cholangiopancreatography (ERCP) is no longer the first choice for diagnostic imaging for many conditions of the biliary tree and pancreas, due to relative risk for complications compared to current noninvasive modalities. However, ERCP remains a firmly established therapeutic modality that offers a wide range of interventions for pancreaticobiliary disease. Skillful execution of diagnostic ERCP remains crucial for the safe and effective deployment of endoscopic therapies. The pancreaticobiliary endoscopist must also master the interpretation of normal and pathologic radiographic findings encountered during ERCP to ensure the highest quality of care for patients. We present an evidence-based review of techniques for safe and efficient diagnostic ERCP. We also review radiographic features of a normal cholangiogram and pancreatogram, as well as findings associated with frequently encountered pathologies of the pancreas and biliary tree.

Background Children with autoimmune hepatitis (AIH) often exhibit particular features. Accordingly, seven pediatric-specific criteria have been proposed. Aim To develop a prediction model based on them, transform it into a scoring system and study its accuracy. Methods 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. Results 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%. Conclusion Pediatric-specific criteria for the diagnosis of AIH can be reliably used as a scoring system.

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.

Patients with cholestatic liver tests and typical histologic features of primary sclerosing cholangitis (PSC) but a normal cholangiogram have been identified as having small-duct PSC. This subgroup of PSC has been less well characterized than the classic large-duct form. Some patients characterized as having small-duct PSC develop cholangiographic features of PSC during follow-up. Three papers published in 2002 on small-duct PSC patients suggested a better prognosis in patients with small-duct PSC than in those with large-duct PSC. However, these studies included a limited number of patients and had a short follow-up. A combined cohort of these patients with a prolonged follow-up recently confirmed these previous observations. However, some patients will suffer from liver-related mortality or will need to undergo liver transplantation. Recurrence of small-duct PSC after liver transplantation has been reported. Cholangiocarcinoma, the most feared complication of PSC, has not been described in a patient with small-duct PSC without progression first to large-duct PSC.

Purpose Two recent meta-analyses support operative CBD exploration (laparoscopic or open) as at least equal to ERCP for the management of choledocholithiasis with the gall bladder in situ. The literature regarding laparoscopic exploration is largely from enthusiasts and may not be transferable across institutions. In our institution both hepatobiliary and general surgeons perform cholecystectomy with variable comfort levels with laparoscopic CBD exploration. ERCP and laparoscopic antegrade transampullary biliary stents are available. We review the management of choledocholithiasis in this setting. Methods A retrospective review of all patients who underwent cholecystectomy during 2004 and 2005 at two Newcastle hospitals was conducted. Results The incidence of choledocholithiasis was 10.3% (70 patients). This was diagnosed pre-operatively in 36 patients; 22 underwent pre-operative ERCP (62.5% clearance) and 14 operative CBD exploration (100% clearance). An additional 22 patients with presumed choledocholithiasis had a normal cholangiogram at pre-operative ERCP. Operative cholangiogram first confirmed choledocholithiasis in 31 patients; CBD exploration was successful in 58.8% (10 of 17). Intra-operative biliary stents were inserted in 15 patients due to a small calibre CBD, failed exploration or lack of equipment and time for exploration. Hepatobiliary surgeons more frequently performed operative CBD exploration and stent placement. Conclusion The management of choledocholithiasis varies with the clinical scenario and local expertise. This series defines a role for intra-operative stent placement, suggests pre-operative ERCP is over-utilised, and that operative CBD exploration is successful with low morbidity.

It has been reported that 10 to 20% of all traumatic diaphragmatic ruptures are missed on initial evaluation, only to be discovered at a later date (1). In the chronic state an abnormal chest X-ray, symptomatic visceral incarceration, or barium contrast studies often lead to the correct diagnosis. This is a case of chronic diaphragmatic injury secondary to trauma being discovered when a diagnostic thoracentesis yielded viscous bile. With the needle left in situ a dye study was performed which demonstrated not only a normal cholangiogram, but also a chronic diaphragmatic rupture with partial herniation of the liver. We feel that this case represents a very unusual presentation of an uncommon injury.

Failure of the contrast medium to enter the duodenum during the operative cholangiography following the common bile duct exploration and instrumentation may be caused by transient obstruction of the common bile duct. It is attributed to spasm and oedema of the sphincter choledochus of Boyden and not of the sphincter of Oddi. The knowledge of the possibility of the pseudo-obstruction of the common bile duct following its exploration may save the surgeon from unnecessary re-exploration and performing sphincterotomy or choledochoduodenostomy. The exploration is unnecessary if, during the common duct exploration, the dilators and the catheter passes easily into the duodenum and on the cholangiogram the obstructed area is smooth and there is no filling defect. A normal cholangiogram performed 8–10 days following the operation proves that the obstruction was transient and not caused by calculus stricture or tumour.

Operative cholangiography is still the most accurate and available method for assessing the presence or absence of stones in the common duct. However, 30 to 40 percent of stones will still be overlooked with cholangiography. To reduce the incidence of overlooked common duct stones we recommend that pressure and flow measurements be obtained before cholangiography. When pressures are high or high normal and flows low, the duct should be explored even in the presence of a normal cholangiogram. Under these circumstances, the incidence of falsepositive pressure flow studies is less than 5 percent even in inexperienced hands.

A patient hospitalized with the clinical diagnosis of acute cholecystitis had a normal cholangiogram and recovered without medical or surgical intervention. During the same week, two adults in an acquainted family had a similar syndrome at home. Echovirus 11 was cultured from the stool of all three patients, as well as from four other members of the same two families who had concurrent diarrheal illness. Serologic evidence confirmed acute echovirus 11 infection in the hospitalized patient. In the absence of evidence of a communitywide epidemic of cholecystitis, the coexistence of viral infection with biliary tract symptoms could represent an ongoing endemic situation. Recognition of this syndrome could make it possible to avoid unnecessary major surgical procedures.

To the Editor.—I wish to offer my solution to the problem of "Spasm and Operative Cholangiography" as described by Chessick et al (Arch Surg110:53, 1975). Some 15 years ago in the role of "kibitzing" anesthesiologist, I placed 0.12 gm of nitroglycerin under the tongue of a patient who had a normal cholangiogram except that the dye did not pass into the duodenum. A short time later the roentgenogram was normal in all respects and dye had entered the intestine. Since then I have used this technique many times to rule out spasm in otherwise normal cholangiograms. Earlier, cyclopropane was usually the anesthetic, but more recently halothane or enflurane have been used, so there was no question of spasm due to morphine-like analgesics.

Background: This retrospective study evaluated the use of diagnostic and therapeutic ERCP in pre- and postoperative patients with hepatic hydatid disease. Methods: For 8 years, ERCP was performed in 39 patients with hepatic echinococcal disease. Indications in the preoperative group of patients (n = 19) included a cholestatic enzyme profile in all cases; jaundice or acute cholangitis also was present in, respectively, 14 and 7 cases. In the postoperative group (n = 20), indications for ERCP included persistent external biliary fistula after surgery in 10 patients, jaundice in 8, acute cholangitis in 7, and right upper quadrant pain in 2 patients. Results: ERCP findings in the preoperative group included cystobiliary fistula (9 patients), external compression of the hepatic biliary system (5), hydatid vesicles and/or membranes within the biliary tract (3), intrahepatic duct stricture (1), and a normal cholangiogram (4). The most common ERCP finding in the postoperative group was external biliary fistula (10 patients); other findings consisted of hydatid cyst material within the bile duct (4), bile duct stenosis (2), cystobiliary fistula and hydatid cyst material in the bile duct (1), cystobiliary fistula (1), hydatid membranes in the gallbladder (1), extrinsic compression to bile ducts (1), and a normal cholangiogram (1). In the preoperative group, endoscopic sphincterotomy was performed in 11 patients, with balloon catheter extraction in 2; complete resolution of findings was achieved in 10 cases. In the postoperative group, sphincterotomy (with balloon or basket extraction as needed) was performed in 19 patients, stents were placed in 2 patients, 1 patient underwent balloon dilatation, and 1 had nasobiliary drainage; there was complete resolution of the findings in 14 of the 20 patients. Conclusions: ERCP and related therapeutic maneuvers are safe and valuable in the pre- and postoperative management of patients with hepatic hydatid disease.

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.

Background: Patients with cholestatic liver function tests and histological features of primary sclerosing 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.

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.

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 post-sphincterotomy 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.

Many hepatobiliary diseases are seen in IBD. PSC is the most common, occurring in 7.5% of patients with UC. The cause of PSC is not well understood, but PSC seems to be associated with genetic susceptibility, sharing some immunologic abnormalities with UC. A characteristic cholangiogram in a patient with abnormal liver function tests usually establishes the diagnosis. Liver biopsy is not essential but can help make the diagnosis of small duct PSC in patients with a normal cholangiogram. There are no medications that treat PSC effectively. Endoscopic dilation of dominant strictures reduces the frequency of cholangitis and may improve survival. OLT remains the only proven treatment of advanced PSC. Cholangiocarcinoma is a feared complication of PSC that is difficult to diagnose. Cholelithiasis, PBC, portal vein thrombosis, and hepatic abscess are hepatobiliary disorders that occur less frequently in IBD patients.

Introduction: Percutaneous nephrolithotomy is generally considered a safe option for the management of large complex or infectious upper urinary tract calculi. Biliary tract injury is a rare and potentially serious complication of percutaneous nephrolithotomy that can even lead to mortality, especially in cases where biliary peritonitis develops. All reported cases of biliary tract injury have been managed by either open or laparoscopic cholecystectomy. Case Presentation: Herein for the first time, we report a 39-year old woman with biliary tract injury following percutaneous nephrolithotomy who was managed less invasively by insertion of a percutaneous cholecystostomy tube. The patient was discharged home shortly thereafter, and the tube was later removed at a follow up visit after a normal cholangiogram. Conclusions: Biliary tract injury is a rare and potentially serious complication of percutaneous nephrolithotomy that can even lead to mortality. If a biliary tract injury is suspected during percutaneous renal procedures, diverting the bile away from the leak may resolve the problem without the need for a cholecystectomy. Ideally this can be done with ERCP and a stent, but in cases where this is not technically feasible; a percutaneous cholecystostomy can be successful at accomplishing the same result.

OBJECTIVE: Cholestasis may result from diverse etiologies. We review chronic cholestatic disorders involving small intrahepatic bile ducts in the adult ambulatory care setting. Specifically, we discuss variant forms of primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) as well as other conditions that may present diagnostic and therapeutic difficulties. METHODS: We conducted a MEDLINE search of the literature (1981–1997) and reviewed the experiences at the Mayo Clinic. All articles were selected that discussed antimitochondrial antibody (AMA)-negative PBC, small-duct PSC (formerly pericholangitis), and idiopathic adulthood ductopenia. RESULTS: The most common chronic cholestatic liver diseases affecting adults are PBC and PSC. Patients without the hallmarks of either syndrome are diagnosed according to their clinical and histological characteristics. Autoimmune cholangitis is diagnosed if clinical and histological features are compatible with PBC but autoantibodies other than AMA are present. Isolated small duct PSC is diagnosed if patients have inflammatory bowel disease, biopsy features compatible with PSC, but a normal cholangiogram. If ductopenia (absence of interlobular bile ducts in small portal tracts) is found histologically in the absence of PSC, inflammatory bowel disease, and other specific cholestatic syndromes such as drug reaction or sarcoidosis, the most likely diagnosis is idiopathic adulthood ductopenia. CONCLUSIONS: Based on these definitions, an algorithm for diagnosis and therapy in patients with laboratory evidence of chronic cholestasis may be constructed, pending results of further investigations into the etiopathogenesis of these syndromes.

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.

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.

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.

Background Persistent small common bile duct (CBD) stones after endoscopic sphincterotomy (EST) and stone extraction may be a nidus for stone growth and could be detected by intraductal US (IDUS). CBD saline solution irrigation may flush out residual stones. Objectives Our purpose was to determine the frequency of residual CBD stones after EST and basket/balloon extraction by using IDUS and to assess the effectiveness of saline solution irrigation in clearing remnant CBD stones. Design Prospective study. Setting General Hospital Singapore. Patients Seventy patients (mean age 62 years, 51% male) were recruited. Interventions In the presence of CBD stones, EST and stone extraction were performed, followed by IDUS. If residual stones were detected, a catheter was inserted into the proximal CBD, saline solution irrigation performed, and IDUS repeated. Main Outcome Measurements (1) The frequency of residual stones detected by IDUS after EST and basket/balloon extraction and (2) the effect of saline solution irrigation in clearing residual CBD stones. Results Cholangiogram showed CBD stones in 38 of 70 patients (median 1 [range 1-5], mean size 7.6 mm [range 3.0-12.0 mm]). IDUS showed CBD stones in 32 of 32 with normal cholangiogram (median 2 [range 1-8], mean size 2.6 mm [range 0.9-7.2 mm]). After EST and stone extraction, IDUS showed persistent stones in 28 of 70 (median: 2 [range 1-5], mean size 2.2 mm [range 1.1-4.6 mm]). The CBD was irrigated with a mean of 48 mL of saline solution. Repeat IDUS showed persistent CBD stones in 2 of 70, and these were flushed out by further saline solution irrigation. Limitations Single-center study. Conclusion IDUS detected small residual CBD stones that persisted after EST and basket/balloon extraction. Saline solution irrigation appeared useful in clearing residual small stones.

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.

Primary sclerosing cholangitis (PSC) is a chronic, progressive, destructive, biliary disease of unknown causes, characterized by multiple, fibrosing, inflammatory strictures of the extrahepatic and intrahepatic bile ducts. The disease often progresses to cirrhosis and leads to premature death caused by liver failure or cholangiocarcinoma. PSC has become a major indication for liver transplantation. 110 The diagnosis of PSC has evolved in recent years from an initially very rigid set of criteria to a diagnosis is based on characteristic clinical, biochemical, histological and—most importantly—radiological features, with irregularity and beading of the intrahepatic or extrahepatic bile ducts. 24,114 Secondary causes of sclerosing cholangitis, such as previous biliary surgery, biliary stone disease, ischemic bile-duct damage caused by treatment with floxouridine, congenital biliary-tree abnormalities, cholangiopathy associated with acquired immunodeficiency syndromes, or bile duct neoplasms must be excluded. 23,61 Cholangiographic features simulating intrahepatic PSC can also be seen in patients with cirrhosis, hepatocellular carcinoma, polycystic liver disease, submassive hepatic necrosis, amyloidosis, intrahepatic portal thrombosis, liver metastases, leukemia and lymphoma, 100 pointing to the importance of combining clinical, histological and cholangiographic features when diagnosing PSC. Some patients with inflammatory bowel disease (IBD), cholestatic biochemical abnormalities and histology consistent with PSC have a normal cholangiogram. These patients are considered to have small bile duct sclerosing cholangitis which may be part of the spectrum of PSC. 59,109

To define the incidence of problematic common bile duct calculi in patients undergoing laparoscopic cholecystectomy.In patients selected for laparoscopic cholecystectomy, the true incidence of potentially problematic common bile duct calculi and their natural history has not been determined. We evaluated the incidence and early natural history of common bile duct calculi in all patients undergoing laparoscopic cholecystectomy with intraoperative and delayed postoperative cholangiography.Operative cholangiography was attempted in all patients. In those patients in whom a filling defect was noted in the bile duct, the fine bore cholangiogram catheter was left securely clipped in the cystic duct for repeated cholangiography at 48 hours and at approximately 6 weeks postoperatively.Operative cholangiography was attempted in 997 consecutive patients and was accomplished in 962 patients (96%). Forty-six patients (4.6%) had at least one filling defect. Twelve of these had a normal cholangiogram at 48 hours (26% possible false-positive operative cholangiogram) and a further 12 at 6 weeks (26% spontaneous passage of calculi). Spontaneous passage was not determined by either the number or size of calculi or by the diameter of the bile duct. Only 22 patients (2.2% of total population) had persistent common bile duct calculi at 6 weeks after laparoscopic cholecystectomy and retrieved by endoscopic retrograde cholangiopancreatography.Choledocholithiasis occurs in 3.4% of patients undergoing laparoscopic cholecystectomy but more than one third of these pass the calculi spontaneously within 6 weeks of operation and may be spared endoscopic retrograde cholangiopancreatography. Treatment decisions based on assessment by operative cholangiography alone would result in unnecessary interventions in 50% of patients who had either false positive studies or subsequently passed the calculi. These data support a short-term expectant approach in the management of clinically silent choledocholithiasis in patients selected for LC.

ERCP is the gold standard for pancreaticobiliary evaluation but is associated with complications. Less invasive diagnostic alternatives with similar capabilities may be cost-effective, particularly in situations involving low prevalence of disease. The aim of this study was to compare the performance of endoscopic ultrasound (EUS) with magnetic resonance cholangiopancreatography (MRCP) and ERCP in the same patients with suspected extrahepatic biliary disease. The economic outcomes of EUS-, MRCP-, and ERCP-based diagnostic strategies were evaluated.Prospective cohort study of patients referred for ERCP with suspected biliary disease. MRCP and EUS were performed within 24 h before ERCP. The investigators were blinded to the results of the alternative imaging studies. A cost-utility analysis was performed for initial ERCP, MRCP, and EUS strategies for these patients.A total of 30 patients were studied. ERCP cholangiogram failed in one patient, and another patient did not complete MRCP because of claustrophobia. The final diagnoses (N = 28) were CBD stone (mean = 4 mm; range = 3-6 mm) in five patients; biliary stricture in three patients, and normal biliary tree in 20. Two patients had pancreatitis after therapeutic ERCP, one after precut sphincterotomy followed by a normal cholangiogram. EUS was more sensitive than MRCP in the detection of choledocolithiasis (80% vs 40%), with similar specificity. MRCP had a poor specificity and positive predictive value for the diagnosis of biliary stricture (76%/25%) compared to EUS (100%/100%), with similar sensitivity. The overall accuracy of MRCP for any abnormality was 61% (95% CI = 0.41-0.78) compared to 89% (CI = 0.72-0.98) for EUS. Among those patients with a normal biliary tree, the proportion correctly identified with each test was 95% for EUS and 65% for MRCP (p0.02). The cost for each strategy per patient evaluated was $1346 for ERCP, $1111 for EUS, and $1145 for MRCP.In this patient population with a low disease prevalence, EUS was superior to MRCP for choledocholithiasis. EUS was most useful for confirming a normal biliary tree and should be considered a low-risk alternative to ERCP. Although MRCP had the lowest procedural reimbursement, the initial EUS strategy had the greatest cost-utility by avoiding unnecessary ERCP examinations.

Background 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.

Chronic cholestatic diseases, whether occurring in infancy, childhood or adulthood, are characterized by defective bile acid transport from the liver to the intestine, which is caused by primary damage to the biliary epithelium in most cases. In this article, approaches to diagnosis and management of the main specific disorders are provided and some of the recent developments in this field are discussed. Major advances in the understanding of the cellular and molecular physiology of bile secretion have led to identification of genetic defects responsible for the different types of progressive familial intrahepatic cholestasis (PFIC). The potential role of the genes involved in PFIC in some adult cholestatic disorders remains to be determined. The majority of adult patients with chronic cholestasis have primary biliary cirrhosis (PBC) or primary sclerosing cholangitis (PSC). Recently, variant forms of PBC have been described. The term autoimmune cholangitis is used to describe patients having chronic non-suppurative cholangitis with negative antimitochondrial antibodies (AMA) but positive antinuclear and/or antismooth muscle antibodies. Autoimmune cholangitis and AMA-positive PBC are quite similar in terms of clinical presentation, survival and response to ursodeoxycholic acid (UDCA) therapy. In contrast, autoimmune cholangitis must be distinguished from PBC-autoimmune hepatitis (AIH) overlap syndrome in which biochemical and histological characteristics of both PBC and AIH coexist. Combination of UDCA and corticosteroids is required in most patients with overlap syndrome to obtain a complete clinical and biochemical response. Long-term UDCA treatment improves survival without liver transplantation in PBC patients. Among the putative mechanisms of the beneficial effects of UDCA, description of anti-apoptotic properties and effect on endotoxin disposal in biliary cells have provided new insights. In patients with incomplete response to UDCA, combination of UDCA with antiinflammatory or immunosuppressive drugs is under evaluation. Variant forms of PSC have also been described, including PSC-AIH overlap syndrome, especially in children or young adults, and small-duct PSC, which is characterized by normal cholangiogram in patients having chronic cholestasis, histologic features compatible with PSC and inflammatory bowel disease. Development of cholangiocarcinoma (CC) is a major feature of PSC, occurring in 10-15% of patients. Early diagnosis of CC is a difficult challenge, although positron emission tomography seems a promising tool. Unlike PBC, effective medical therapy is not yet available in PSC, reflecting the lack of knowledge about the exact pathogenesis of the disease. Currently, liver transplantation is the only effective therapy for patients with advanced disease, although recurrence of PSC in the graft may occur.

The clinical and pathological findings of idiopathic ductopenia were studied in a 30-year-old woman who initially manifested jaundice and pruritus. Serum biochemical tests of liver function indicated severe and progressive cholestasis. Viral hepatitis markers and circulating autoantibodies were absent. The patient had a normal cholangiogram and lacked evidence of inflammatory bowel disease. Histological examination of a liver specimen showed severe cholestasis and absence of interlobular bile ducts. Severe jaundice and intractable pruritus developed in the patient and served as the indications for liver transplantation 4 months after initial examination. Transplantation resulted in prompt and complete resolution of the jaundice and pruritus. Two types of idiopathic adulthood ductopenia associated with different prognoses are recognized. Patients with type 1 idiopathic adulthood ductopenia are asymptomatic or manifest symptoms of cholestatic liver disease. They tend to have less destruction of the intrahepatic bile ducts on liver biopsy specimens. Their clinical course ranges from spontaneous improvement to progression to biliary cirrhosis. In contrast, patients with type 2 idiopathic adulthood ductopenia generally manifest initial symptoms of decompensated biliary cirrhosis, have extensive destruction of the intrahepatic bile ducts on liver biopsy, and frequently require orthotopic liver transplantation.

Background: DACP, as an adjunct to ERCP allows for direct visualization of the biliary duct and pancreatic duct.We aim to assess the technical feasibility of DACP for a variety of clinical indications and to determine its impact on disease management. Methods: 94 consecutive DACP examinations were performed in 57 patients with 3 groups of disorders, choledocholithiasis (CDL), biliary strictures(BS) and pancreatic disease(PD), age range (24 to 91 years; mean 69). All DACPs were performed using the Pentax FCP-8P or 9P. Data pertaining to indication, technical success, and clinical impact of DACP on disease management were collected and analyzed using cMore endoscopic database. Results: CDL (n=53, 27 pts ) Indications included stone fragmentation using EHL (n=34), evaluation of biliary tree post-stone removal (n=14), distinction between stone and stricture, evaluation of biliary tree for stones despite normal cholangiogram (n=5). Technical success was achieved in 29/53 DACPs. The 24/53 failures represent partial fragmentation of stones with EHL in 8 patients with extensive disease. Use of DACP resulted in complete removal of biliary tree stones in 10/34 examinations (10 patients) and demonstrated the presence of stones in 4 patients with normal post-interventional cholangiograms. Furthermore DACP helped to confirm the absence of stones after removal by basket or balloon in 11/12 patients. BS (n=33, 23 pts) Indications included evaluation of stricture(n=21) or Wallstent occlusion (n=4), and monitoring of cholangiocarcinoma progression in a single patient (n=7). Technical success was achieved in 29/33 cases. DACP assisted in confirming or clarifying the nature of a stricture in 16/29 cases, and in stent management in 20/29 cases. PD (n=7) Indications included evaluation of pancreatic duct obstruction in chronic pancreatitis (n=4) and malignancy (n=1), and evaluation of PD for IPMT (n=2). An additional use of DACP was in the evaluation of hemobilia (n=1) where no source was seen. There were no complications reported with the use of DACP. Conclusion: DACP is technically feasible in the management of pancreatico-biliary diseases. Lack of complete technical success in the use of EHL to fragment stones is due to extensive stone disease in a limited number of patients. Otherwise DACP impacts on the management of complicated biliary tract disease by clarifying cholangiograms, assisting in EHL stone fragmentation, and guiding stenting and surgical management.

Endoscopic retrograde cholangiopancreatography (ERCP) has become an essential tool to investigate patients with the postcholecystectomy syndrome. A normal cholangiogram usually rules out the presence of biliary tract disease, and further investigations are directed towards other organ systems. We present a case in which a normal ERCP caused a significant delay in reassessing the biliary tree in a patient who eventually presented with choledocholithiasis. A repeat ERCP should be considered in patients with persistent biliary tract pain, even if the initial ERCP shows no abnormality.

The diameter of extrahepatic and intrahepatic bile ducts was measured on 50 normal retrograde cholangiograms and on the cholangiograms of 109 post-cholecystectomy patients undergoing endoscopic retrograde cholangiopancreatography: 70 of the post-cholecystectomy patients had a normal cholangiogram and 39 had retained stone or biliary stricture, of whom 17 were jaundiced. Biliary diameter at all points measured was greater in the post-cholecystectomy patients with no biliary lesion than in normals, and further increased in the presence of pathology (e.g. retained stone). The extent of overlap in biliary diameter between all these three groups is so great that measurement of bile duct calibre cannot separate patients with retained stone from post-cholecystectomy patients without retained stone, or from patients with an entirely normal biliary tree, even in the presence of obstructive jaundice. Methods of evaluating the biliary tract which rely on the measurement of bile duct diameter (e.g. ultrasound) are therefore of limited value in the investigation of post-cholecystectomy patients.

Percutaneous transhepatic cholangiography has been attempted in 104 patients with jaundice due to a variety of causes. Biliary tract visualization was accomplished in 57, the majority having extrahepatic obstruction due to malignancy or calculi. Nonvisualization (a "negative" study) or a normal cholangiogram, was commonly associated with parenchymal liver disease which was confirmed by percutaneous needle liver biopsy. With an injection technique and television roentgen monitoring, false negative studies have been eliminated in patients with dilated intrahepatic bile ducts, and even normal ducts can be visualized. Needle liver biopsy has become an alternative to unproductive biliary tract exploration.

Only a few studies have attempted to determined the prevalence of long-standing abnormal liver function and primary sclerosing cholangitis (PSC) in patients with Crohn's disease (CD). The aim of the study was to determine the prevalence of long-standing abnormal liver function test results and to describe the clinical, biochemical, and histologic findings in patients with large-duct classic PSC and small-duct PSC (that is, normal cholangiogram) in patients with CD during a 15-year period.Patients with CD and long-standing abnormal liver function results were investigated individually with endoscopic retrograde cholangiography and liver biopsy.Of 262 consecutive patients with CD, 38 (15%) had long-standing increased alkaline phosphatase (ALP) values (mean, 1065 U/l; range, 321-4165 U/l). Of these, 10 patients were classified as having hepatic disease (4%), of which 9 had PSC and 1 had a non-specific reactive hepatitis. Of nine patients with PSC (3.4%), three were classified as having large-duct PSC; five, small-duct PSC; and one, unclassified. In patients with large-bowel CD (n = 102) the prevalence of PSC was 9%. Mean age at diagnosis of PSC was 35 years (22-46 years), and the female to male ratio, 7:2. All PSC patients had large-bowel involvement (P0.00015), and two of them developed colonic carcinoma of the large bowel (P0.01). All cases of small-duct PSC were stage 1, whereas large-duct PSC were stage 2-3. During the observation period (mean, 5.4 years) no PSC patients died.The results of our study indicate that PSC is the major hepatic disease in patients with CD and long-standing abnormal liver function tests and is approximately as prevalent as in ulcerative colitis. Patients with PSC and CD may have a milder liver disease than patients with PSC and ulcerative colitis, perhaps because large-duct PSC is less common in patients with CD. Cholangiograms and liver biopsies are both needed to evaluate the extent of the disease.