The morphological and histopathological assessment of Alagille syndrome with extrahepatic bile duct obstruction: the importance of the differential diagnosis with subgroup "o" biliary atresia.

PURPOSE: The differential diagnosis between Alagille syndrome (AGS) with extrahepatic bile duct obstruction (EHBDO) and biliary atresia (BA) is difficult. We report a case series of AGS with EHBDO with detailed validation of the morphological and histopathological features for the differential diagnosis of BA. METHODS: Six liver transplantations (LTs) were performed for AGS with EHBDO. All patients were diagnosed with BA at the referring institution and the diagnosis of AGS was then confirmed based on a genetic analysis before LT. We verified the morphological and histopathological findings of the porta hepatis and liver at the diagnosis of BA and at LT. RESULTS: All patients had acholic stool in the neonatal period and were diagnosed with BA by cholangiography. The gross liver findings included a smooth and soft surface, without any cirrhosis. The gross findings of the porta hepatis included aplasia of the proximal hepatic duct, or subgroup "o", in five patients. The histopathological examination of the EHBD also revealed obstruction/absence of the hepatic duct. There were no patients with aplasia of the common bile duct. CONCLUSIONS: Aplasia of the hepatic duct and the macroscopic liver findings may help in to differentiate between AGS with EHBDO and BA.

Cost Efficacy of Metal Stents for Palliation of Extrahepatic Bile Duct Obstruction in a Randomized Controlled Trial.

BACKGROUND & AIMS: Endoscopic stents are placed for palliation of extrahepatic bile duct obstruction. Although self-expandable metal stents (SEMS) remain patent longer than plastic stents, they are more expensive. We aimed to evaluate which type of stent (plastic, uncovered SEMS [uSEMS], or partially covered SEMS [pcSEMS]) is the most effective and we assessed costs. METHODS: We performed a multicenter randomized trial in 219 patients at 18 hospitals in The Netherlands from February 2008 through February 2013. Patients were assigned randomly for placement of a plastic stent (n = 73), uSEMS (n = 75), or pcSEMS (n = 71) during endoscopic retrograde cholangiopancreatography. Patients were followed up for up to 1 year. Researchers were not blinded to groups. The main study end points included functional stent time and costs. RESULTS: The mean functional stent times were 172 days for plastic stents, 288 days for uSEMS, and 299 days for pcSEMS (P < .005 for uSEMS and pcSEMS vs plastic). The initial placement of plastic stents (€1042 or $1106) cost significantly less than placement of SEMS (€1973 or $2094) (P = .001). However, the total cost per patient at the end of the follow-up period did not differ significantly between plastic stents (€7320 or $7770) and SEMS (€6932 or $7356) (P = .61). Furthermore, in patients with short survival times (≤3 mo) or metastatic disease, the total cost per patient did not differ between plastic stents and SEMS. No differences in costs were found between pcSEMS and uSEMS. CONCLUSIONS: Although placement of SEMS (uncovered or partially covered) for palliation of extrahepatic bile duct obstruction initially is more expensive than placement of plastic stents, SEMS have longer functional time. The total costs after 1 year do not differ significantly with stent type. Dutch Clinical Trial Registration no: NTR1361.

Intraductal US in the assessment of tumor involvement to the orifice of the cystic duct by malignant biliary obstruction.

BACKGROUND: Tumor involvement to the orifice of the cystic duct (OCD) is a risk factor for cholecystitis after metallic stent (MS) placement. OBJECTIVE: ERCP and intraductal US (IDUS) were used to assess tumor involvement to the OCD. DESIGN: A prospective consecutive study. SETTING: The University of Tokyo Hospital, Japan. PATIENTS AND INTERVENTIONS: Between January 2003 and May 2007, 34 patients with resectable malignant biliary obstruction underwent an ERCP and an IDUS before preoperative biliary drainage. MAIN OUTCOME MEASUREMENTS: ERCP and IDUS findings were confirmed via a histopathologic analysis of surgical specimens. RESULTS: An ERCP showed that the cystic duct was opacified in 22 of 34 patients (64.7%). Eight patients were diagnosed with OCD tumor involvement. In the remaining 12 patients, tumor involvement was impossible to evaluate via an ERCP. An IDUS identified tumor involvement to the OCD in all 34 patients. Tumor involvement to the OCD was diagnosed in 12 patients, including 4 patients with previously negative results during an ERCP. Tumor involvement was confirmed by histopathology of surgical specimens. The sensitivity and specificity of detection were 66.7% (8/12) and 63.6% (14/22), respectively, via ERCP, and 100% (12/12) and 100% (22/22), respectively, via IDUS. LIMITATION: The small sample size. CONCLUSIONS: IDUS, in addition to ERCP, provides an accurate evaluation of tumor involvement to the OCD in patients with malignant biliary obstruction.

Incidence and management of Mirizzi syndrome during laparoscopic cholecystectomy.

BACKGROUND: Benign extrinsic obstruction of the hepatic duct, known as "Mirizzi syndrome" (MS), is an uncommon complication of longstanding cholelithiasis. Since laparoscopic cholecystectomy (LC) replaced the open approach, Mirizzi syndrome has regained the interest of biliary surgeons. METHODS: The Swiss Association for Laparoscopic and Thoracoscopic Surgery (SALTS) prospectively collected the data on 13,023 patients undergoing LC between 1995 and 1999. This database was investigated with special regard to patients with Mirizzi syndrome. RESULTS: There were 39 patients (14 men and 25 women; mean age, 61 years) with MS (incidence, 0.3%). Thirty-four patients had type 1 MS and five had type 2. A gallbladder carcinoma was found in four patients (incidence, 11%). In the type 1 group, 23 patients underwent cholecystectomy only, 10 patients had a bile duct exploration and T-tube insertion, and one patient had a Roux-en-Y reconstruction. In three patients with type 2, a hepaticojejunostomy was performed; two others underwent simple closure and drainage (via T-tube) of the biliary fistula. The conversion rate was 74% (24 of 34 patients) in the type 1 group and 100% (five of five patients) for type 2. The overall complication rate was 18%. There were no deaths. CONCLUSIONS: Although MS is rarely encountered during LC, it must be recognized intraoperatively. Conversion to an open approach is often needed, and prior to any surgical intervention, gallbladder cancer must be excluded.

A randomized trial of endoscopic drainage methods for inoperable malignant strictures of the common bile duct.

BACKGROUND: Although metallic stents remain patent longer than plastic stents, the optimal palliation of inoperable malignant biliary strictures remains controversial because of the high cost of metallic stents and short patient survival. METHODS: A total of 101 patients (mean age 72.5+/-12.9 years) with malignant strictures of the common bile duct were included in this study, after three exclusions for technical failure (n = 3) and one for noncompliance with study design. The etiology of the strictures included pancreatic cancer (65), cholangiocarcinoma (21), ampullary tumor (3), and metastatic lymph nodes (12). Patients were randomized to receive either an 11.5F polyethylene stent to be exchanged in case of dysfunction (group 1, n = 33), an 11.5F stent to be exchanged every 3 months (group 2, n = 34), or a self-expanding metallic Wallstent (group 3, n = 34). RESULTS: Endoscopic procedures were successful (including complete relief of jaundice) in 97.1 % of cases. Procedure-related morbidity was 11.9%, and mortality was 2.9%. Bilirubinemia after 48 hours (37.2%+/-21.7% decrease from the preoperative level) did not differ between groups. Patients were followed for a mean of 166 days (median 143, range 0 to 596 days). Overall survivals were not different between groups, but complication-free survival for groups 2 and 3 was longer than that of group 1 (p < 0.05). Cumulated hospital days were 7.4+/-1.5, 10.6+/-1.7, and 5.5+/-1.4 (groups 1, 2, and 3, respectively) (p < 0.05; analysis of variance). Cost analysis showed that metallic stents were advantageous in patients surviving more than 6 months, whereas a plastic stent was advantageous in patients surviving 6 months or less. CONCLUSIONS: Metallic stents and plastic stents exchanged every 3 months are valuable alternatives for increasing complication-free survival in patients with malignant strictures of the common bile duct. Metal stents are advantageous in patients with the longest life expectancy.

Renal functions in obstructive jaundice: a pre and post operative assessment.

Surgery in patients with surgical obstructive jaundice is known to be associated with increased risk of post-operative acute renal failure. A prospective study was carried out to evaluate the renal function in patients with obstructive jaundice. Renal functions of thirty two patients with jaundice secondary to mechanical obstruction of the biliary tract were evaluated pre-operatively and 7 days after surgical biliary decompression. Although no significant difference was seen in the mean values of pre and post operative renal function variables, two patients had overt renal failure, one with pre-operative cholangitis and acute tubular necrosis and another with carcinoma of the pancreas and postoperative acute renal failure. The overall satisfactory outcome in our obstructive jaundice patients may be related to pre-operative and intraoperative preparation with intravenous fluids and mannitol. It is concluded that patients with obstructive jaundice can be satisfactorily treated with special preoperative care including good hydration and mannitol therapy during anaesthesia and surgery.

[IODIDA scanning for functional and morphologic assessment of the liver and bile ducts before and following surgical interventions in bile duct obstruction]. / IODIDA-Scanning für die funktionelle und morphologische Beurteilung von Leber und Gallenwegen vor und nach chirurgischen Eingriffen bei Gallenwegobstruktion.

PROBLEM: Full pre- and postoperative assessment is mandatory in the management of complex cases of incomplete biliary obstruction. Investigations should not only define the level of extrahepatic bile duct obstruction but also detect intrahepatic obstruction, give some index of liver function and of the dynamics of biliary flow. Computed tomography, ultrasonography and direct cholangiography are very valuable. IODIDA-scanning provides a non-invasive method which not only complements other studies but also gives information otherwise unobtainable. CLINICAL MATERIAL: In an initial retrospective study 36 patients, 12 of whom had previously undergone operation for biliary obstruction, were fully investigated with particular reference to the use of IODIDA-scanning. PROCEDURE: 2-5 mCi of 99mTc labelled IODIDA were injected intravenously and the liver and upper abdomen scanned at 1 minute intervals and displayed at 5 minute intervals during the first hour. RESULTS: All patients were studied on admission and then postoperatively at intervals. In 31 of 36 patients IODIDA-scanning gave reliable evidence of the level of obstruction of biliary flow and of the patency of biliodigestive anastomosis. Assessment of liver function before and after biliary reconstruction was also possible. CONCLUSION: IODIDA-scanning has proved a valuable non-invasive method for the assessment of liver parenchymal function, intrahepatic abnormalities and of bile flow in cases of complex biliary obstruction. This is particularly valuable with the Roux-Y biliary reconstruction since ERCP is impossible and PTC an invasive method which, although supplying an accurate picture of major biliary obstruction, fails to characterize hepatic function and bile flow.

Urinary sodium levels related to jaundice duration in the assessment of postoperative risk of hepatorenal syndrome in elderly patients undergoing surgery for biliary obstruction.

The early diagnosis of hepatorenal syndrome (HRS) is based chiefly on biochemical alterations and in particular, the relationship between urinary sodium levels and duration of jaundice seems to offer the most reliable prognostic index. Urinary sodium levels were determined in 32 elderly patients, who underwent surgery for benign or malignant biliary obstruction, without, major organ resection; in this group 4 patients died of HRS (12.5 per cent). The diagnostic accuracy of the method reached 97 per cent.