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.

New antireflux plastic stent for patients with distal malignant biliary obstruction.

BACKGROUND: Endoscopic biliary stenting is a well-established palliative treatment for patients with unresectable distal malignant biliary obstruction (MBO). However, the main problem with stent placement is the relatively short duration of stent patency. Although self-expanding metal stents (SEMSs) have a longer patency period than plastic stents (PSs), the higher costs limit the wide use of SEMSs. A PS with an antireflux valve is an attractive idea to prolong stent patency, but no ideal design for an antireflux PS (ARPS) has been proposed. We developed a new ARPS with a "duckbilled" valve attached to the duodenal end of the stent. AIM: To compare the patency of ARPSs with that of traditional PSs (TPSs) in patients with unresectable distal MBO. METHODS: We conducted a single-center, prospective, randomized, controlled, double-blind study. This study was conducted at the West China Hospital of Sichuan University. Consecutive patients with extrahepatic MBO were enrolled prospectively. Eligible patients were randomly assigned to receive either an ARPS or a TPS. Patients were followed by clinic visits or telephone interviews every 1-2 mo until stent exchange, death, or the final study follow-up in October 2018. The primary outcome was the duration of stent patency. Secondary outcomes included the rate of technical success, the rate of clinical success, adverse events, and patient survival. RESULTS: Between February 2016 and December 2017, 38 patients were randomly assigned to two groups, with 19 patients in each group, to receive ARPSs or TPSs. Stent insertion was technically successful in all patients. There were no significant differences between the two groups in the rates of clinical success or the rates of early or late adverse events (P = 0.660, 1.000, and 1.000, respectively). The median duration of stent patency in the ARPS group was 285 d [interquartile range (IQR), 170], which was significantly longer than that in the TPS group (median, 130 d; IQR, 90, P = 0.005). No significant difference in patient survival was noted between the two groups (P = 0.900). CONCLUSION: The new ARPS is safe and effective for the palliation of unresectable distal MBO, and has a significantly longer stent patency than a TPS.

Tannenbaum and metal stents in the palliative treatment of malignant distal bile duct obstruction: a comparative study of patency and cost effectiveness.

BACKGROUND: Stent clogging is the major limitation of palliative treatment for malignant biliary obstruction. Metal stents have much better patency than plastic stents, but are more expensive. Preliminary data suggest that the recently designed plastic (Tannenbaum) stent has better duration of patency than the polyethylene stent. This study aimed to compare the efficacy and cost effectiveness between the Tannenbaum stent without side holes and the uncovered metal stent for patients with malignant distal common bile duct obstruction. METHODS: In this study, 47 patients (median age, 73 years, range, 56-86 years) with inoperable malignant distal common bile duct strictures were prospectively randomized to receive either a Tannenbaum stent (n = 24) or an uncovered self-expandable metal stent (n = 23). The patients were clinically evaluated, and biochemical tests were analyzed if necessary until their death or surgery for gastric outlet obstruction. Cumulative first stent patency and patient survival were compared between the two groups. Cost-effectiveness analysis also was performed for the two study groups. RESULTS: The two groups were comparable in terms of age, gender, and diagnosis. The median first stent patency was longer in the metal group than in the Tannenbaum stent group (255 vs 123.5 days; p = 0.002). There was no significant difference in survival between the two groups. The total cost associated with the Tannenbaum stents was lower than for the metal stents (17,700 vs 30,100 euros; p = 0.001), especially for patients with liver metastases (3,000 vs 6,900 euros; p < 0.001). CONCLUSIONS: Metal stent placement is an effective treatment for inoperable malignant distal common bile duct obstruction, but Tannenbaum stent placement is a cost-saving strategy, as compared with metal stent placement, especially for patients with liver metastases and expected short survival time.

Usual and unusual causes of extrahepatic cholestasis: assessment with magnetic resonance cholangiography and fast MRI.

Cholestasis may result from hepatocellular (intrahepatic) disease or biliary tract (extrahepatic) abnormalities. Etiologies causing extrahepatic cholestasis are extremely diverse and invasive procedures, such as endoscopic retrograde cholangiopancreatography (ERCP) and percutaneous transhepatic cholangiography (PTC), were previously required to establish the diagnosis. Due to refinements of magnetic resonance imaging (MRI) techniques, the patient with extrahepatic cholestasis currently can be evaluated noninvasively, and the information revealed frequently exceeds the findings obtained by ERCP and PTC. In this essay, we illustrate the classic MR cholangiographic (MRC) and MRI features of a variety of disorders causing extrahepatic cholestasis, including non-neoplastic disorders of the biliary tract (congenital abnormalities, infectious processes, iatrogenic disorders, and postsurgical complications) and neoplastic conditions (e.g., tumors of the pancreas, biliary tree, liver, ampulla, and regional lymph nodes). In most cases, familiarity with the key MRC features in addition to information obtained via cross-sectional MR images provide sufficient information for adequate lesion characterization.

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.

The consequences of a major bile duct injury during laparoscopic cholecystectomy.

Bile duct injury is perhaps the most feared complication of laparoscopic cholecystectomy. The focus of this study was on the immediate and short-term outcome of patients who have undergone repair of major bile duct injuries with respect to hospital stay, perioperative interventions, and reoperations. The records of patients who underwent surgery at three academic hospitals in Philadelphia (Hospital of the University of Pennsylvania, Thomas Jefferson University Hospital, and Graduate Hospital) from 1990 to 1995 for repair of a major biliary injury following laparoscopic cholecystectomy were reviewed. A major biliary injury was defined as any disruption (including ligation, avulsion, or resection) of the extrahepatic biliary system. Small biliary leaks not requiring surgery were excluded. Thirty-two patients sustained major bile duct injuries. The injury was recognized immediately in 10 patients. The remaining 22 patients had pain (59%), jaundice (50%), and/or fever (32%) as the symptom heralding the injury. Bismuth classification was as follows: 13% of patients were class I, 63% were class II, 7% were class III, 7% were class IV, and 10% were class V. Biliary reconstruction included a Roux-en-Y hepaticojejunostomy in 30 patients and two were primary repairs. There was one postoperative death from multiorgan system failure. The mean length of hospital stay after repair was 17 +/- 8 days. Over a mean follow-up period of 11.5 +/- 10.5 months, 11 patients (38%) required 19 emergency readmissions, most commonly for cholangitis. Five patients (17%) required postoperative balloon dilatation for biliary stricture. At follow-up 18 patients (62.0%) remain asymptomatic with normal liver function, eight (28%) are experiencing episodic cholangitis, and three (10%) are asymptomatic with persistently elevated liver function values. The consequences of a major biliary tract injury following laparoscopic cholecystectomy include a complex operative repair resulting in a lengthy postoperative stay with an increased risk of death, an excessive number of perioperative diagnostic and therapeutic studies, frequent readmissions (often as emergencies), and a lifelong risk of restricture. The "cost" to these patients remains enormous.

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.

[Metal stents in the biliopancreatic duct system--assessment of current status]. / Metallstents im biliopankreatischen Gangsystem--Standortbestimmung.

By the end of the eighties mesh stents were implanted in malignant stenoses of the biliary and pancreatic ducts for the first time. In the following years, self-expanding as well as balloon-expandable mesh stents were increasingly implanted in malignant stenoses by radiologists or interventional gastroenterologists, either percutaneously or endoscopically retrograde. In randomized comparative studies different teams showed relevant advantages of this new type of prosthesis in comparison to the commonly used plastic stents. Especially the significantly lower rate of late complications and longer patency rates speak in favor of the mesh stents. The higher cost of the mesh stent makes it important to carefully select patients before implantation, to let patients with potentially longer survival time profit from the advantages of the procedure. Expandable mesh stents were successfully implanted in benign stenoses of the biliopancreatic ducts as well, although the lack of randomized comparative studies makes a final assessment difficult. Concluding from already presented results, there might be a new therapeutical option for selected patients with benign stenoses of the biliopancreatic ducts.

Common bile duct obstruction due to malignancy: treatment with plastic versus metal stents.

PURPOSE: To compare the clinical efficacy and treatment costs of plastic versus metal biliary stents. MATERIALS AND METHODS: In a randomized trial, 101 patients with malignant common bile duct obstruction underwent transhepatic stent implantation and were followed up until death. Patients were stratified into risk and nonrisk groups. Forty-nine patients received 12-F plastic stents, and 52 received expandable metal stents. Plastic endoprostheses were placed in a two-step procedure; metal stent, in a single procedure. Kaplan-Meier analyses were used to compare patient survival and stent patency rates. RESULTS: The 30-day mortality rate was significantly lower for metal stents (five of 52 [10%]) than plastic stents (12 of 49 [24%]; P = .05). The obstruction rate was 19% (10 of 52; median patency, 272 days) for metal stents and 27% for plastic stents (13 of 49; median patency, 96 days; P < .01). Median time until death or obstruction was longer for metal stents (122 vs 81 days; P < .01). Placement of metal stents was associated with shorter hospital stay (10 vs 21 days; P < .01) and lower cost ($7,542 vs $12,129; P < .01). CONCLUSION: Use of self-expanding metal stents appears to show substantial benefits for patients and to be cost-effective.

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.