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Objective: The aim of this analysis was to report a multidisciplinary series comparing choledochoceles to Todani Types I, II, IV, and V choledochal cysts., Summary Background Data: Choledochoceles have been classified as Todani Type III choledochal cysts. However, most surgical series of choledochal cysts have reported few choledochoceles because they are managed primarily by endoscopists., Methods: Surgical, endoscopic, and radiologic records were reviewed at the Riley Children's Hospital and the Indiana University Hospitals to identify patients with choledochal cysts. Patient demographics, presenting symptoms, radiologic studies, associated abnormalities, surgical and endoscopic procedures as well as outcomes were reviewed., Results: A total of 146 patients with "choledochal cysts" including 45 children (31%) and 28 with choledochoceles (18%) were identified, which represents the largest Western series. Patients with choledochoceles were older (50.7 vs. 29.0 years, P < 0.05) and more likely to be male (43% vs. 19%, P < 0.05), to present with pancreatitis (48% vs. 24%, P < 0.05) rather than jaundice (11% vs. 30%, P < 0.05) or cholangitis (0% vs. 21%, P < 0.05), to have pancreas divisum (38% vs. 10%, P < 0.01), and to be managed with endoscopic therapy (79% vs. 17%, P < 0.01). Two patients with choledochoceles (7%) had pancreatic neoplasms., Conclusions: Patients with choledochoceles differ from patients with choledochal cysts with respect to age, gender, presentation, pancreatic ductal anatomy, and their management. The association between choledochoceles and pancreas divisum is a new observation. Therefore, we conclude that classifications of choledochal cysts should not include choledochoceles.

Background: Early endoscopic retrograde cholangiopancreatography (ERCP) should be performed in all patients with acute biliary pancreatitis (ABP) and coexisting acute cholangitis. In patients without cholangitis and predicted mild ABP it is generally accepted that early ERCP should not be performed. Nevertheless, there is a controversy regarding the role of early ERCP in the treatment of patients with predicted severe ABP without cholangitis. We reviewed randomized trials on early ERCP versus conservative management in patients with ABP without acute cholangitis., Methods: Relevant publications in 3 electronic databases (MEDLINE, EMBASE, Cochrane Central Register of Controlled Trials) were systematically reviewed and meta-analyzed., Results: Seven randomized trials on ERCP in acute pancreatitis were found, of which 3 including a total of 450 patients (230 in the invasive arm and 220 in the control arm) qualified for a meta-analysis according to predefined criteria. In all patients with ABP (predicted mild and severe), early ERCP was associated with a nonsignificant reduction in overall complications [risk ratio (RR) 0.76; 95% confidence interval (CI) 0.41-1.04; P = 0.38] and a nonsignificant increase in mortality (RR 1.13; 95% CI 0.23-5.63; P = 0.88). Subgroup analysis based on predicted severity did not affect these outcomes (overall complications: predicted mild: RR 0.86; 95% CI 0.62-1.19; P = 0.36; predicted severe: RR 0.82; 95% CI 0.32-2.10; P = 0.68; mortality: predicted mild: RR 1.90; 95% CI 0.25-14.55; P = 0.53; predicted severe: RR 1.28; 95% CI 0.20-8.06; P = 0.80)., Conclusion: In this meta-analysis, early ERCP in patients with predicted mild and predicted severe ABP without acute cholangitis did not lead to a significant reduction in the risk of overall complications and mortality.

Objective: To analyze the short-term surgical outcome of hepatobiliary resections for perihilar cholangiocarcinoma in the last 5 years., Summary Background Data: Hepatobiliary resection for perihilar cholangiocarcinoma remains a technically demanding procedure, calling for a high level of expertise in biliary and hepatic surgery, and is still associated with significant morbidity or mortality., Methods: Between 2000 and 2004, we surgically treated 102 consecutive patients with perihilar cholangiocarcinoma with a management strategy consisting of preoperative biliary drainage, portal vein embolization (for right-sided and extended left-sided resections), and major hepatobiliary resection. The data on all of the patients were analyzed retrospectively to identify the factors that might significantly affect the postoperative mortality and morbidity., Results: There were no cases of in-hospital mortality or postoperative liver failure. Major complications were encountered in 7 patients (6.9%), and the overall morbidity rate was 50%. Reoperation was required in 2 patients (2%). The overall median length of postoperative hospital stay was 26 days (range, 13-119 days). Univariate analysis in relation to the postoperative morbidity showed significant differences in the preoperative occurrence of segmental cholangitis or cholecystitis (P = 0.015), the severity of postoperative hyperbilirubinemia (P < 0.001), and the total amount of fresh frozen plasma administered (P = 0.002). Multivariate analysis revealed a single independent significant predictive factor for postoperative morbidity, namely, preoperative cholangitis or cholecystitis (odds ratio, 9.08; 95% confidence interval, 1.05-78.56, P = 0.045)., Conclusions: Our experience indicates that hepatobiliary resections for perihilar cholangiocarcinoma can be conducted safely, without a single case of postoperative liver failure or mortality. Occurrence of preoperative cholangitis or cholecystitis is a significant indicator for morbidity of major hepatobiliary resection.

Emergency surgery for patients with severe acute cholangitis carries formidable postoperative morbidity and mortality rates. A retrospective study was conducted on 86 consecutive patients who had exploration for the calculous obstructions to identify the high-risk population to guide better management. Septicemic shock was present in 55 patients before surgery. All patients had ductal exploration under general anesthesia. Additional procedures included cholecystectomy (n = 55), cholecystostomy (n = 5), and transhepatic intubation (n = 2). Complications and deaths occurred in 43 (50%) and 17 (20%) patients, respectively. Multivariate analysis on the 25 clinical (n = 14) and biochemical (n = 11) parameters evaluated yield the following five predictive factors (relative risk): the presence of concomitant medical problems (4.5); pH less than 7.4 (3.5); total bilirubin more than 90 mumol/l (3.1); platelet less than 150 x 10(9)/l (2.9), and serum albumin less than 30 g/L (2.9). In the presence of three or more albumin less than 30 g/L (2.9). In the presence of three or more risk factors, postoperative morbidity and mortality rates were 91% and 55%, respectively, which were significantly higher than those with two or less risk factors (34% and 6%, respectively). As thrombocytopenia developed even with transient hypotension, timely ductal decompression would improve outcome of these patients after surgery. For the high-risk population, application of nonoperative biliary drainage might be considered.

Pyogenic liver abscesses in 106 adult patients at The New York Hospital were reviewed to define optimum treatment. Mortality in the surgically treated patients was 26% (17/65), while those treated nonsurgically had a fatality rate of 95% (39/41). Multiple abscesses treated surgically had a surprisingly low mortality of 29% (5/17). Modern noninvasive tests are highly sensitive in diagnosing liver lesions greater than 2 cm. Difficulty remains in identifying small hepatic abscesses and differentiating large abscesses from tumor. Most liver abscesses have an identifiable source outside the liver. The most common source (31%) was cholangitis secondary to extrahepatic biliary obstruction. Multiple abscesses, mixed organisms, hyperbilirubinemia, and abscess complications are all associated with a significantly increased mortality. However, the lethality of the primary disease process was the most important factor determining survival. Most patients who have the underlying pathogenesis of the abscess controlled will survive surgical treatment. Transperitoneal surgical drainage and antibiotics remain the mainstay of treatment. Percutaneous drainage is recommended for high risk patients only.

Traditional management of patients with primary sclerosing cholangitis (PSC) has included steroids and/or choledochotomy plus prolonged T-tube drainage. The authors have not been convinced, however, that either of these therapies significantly influence the course of the disease. Therefore, a more aggressive surgical approach was adopted that included performance of a choledochoenteric anastomosis in all patients with PSC who had either (1) a major area of extrahepatic blockage, or (2) primary involvement of the extrahepatic bile ducts. Using this approach, 17 of 22 patients (77%) managed surgically at the UCLA Medical Center from 1974 through 1980 have undergone a choledochoenteric anastomosis. Of these 17 patients, 13 (77%) have had an excellent or good result following surgery. Four patients whose disease was confined to the common bile duct have all had excellent results (mean follow-up 49.3 months since surgery). In addition, 18 of the entire group of 22 patients (82%) are still alive, a mean of 52.2 months after operation and 64.7 months following establishment of the diagnosis. These results in 22 patients encourage the authors to continue to pursue an aggressive surgical approach for patients with primary sclerosing cholangitis.

The clinical course and management of 40 patients who underwent operation for acute cholecystitis developing as a postoperative complication were reviewed. Of note was the mortality of 47%, the high incidence of gangrene, perforation, empyema, and cholangitis, and the atypical clinical presentation of acute cholecystitis under these conditions. Awareness of this possible complication, knowledge of its clinical features, and early surgical intervention are important facets of successful management.

In order to identify risk factors in patients with acute cholangitis, 140 clinical, biochemical, etiologic, and pathologic variables of 449 attacks of acute cholangitis seen in one center over a 20-year period were analyzed. Simple regression revealed 24 factors with prognostic significance, but multivariate analysis detected only seven factors with independent significance in predicting mortality (acute renal failure, cholangitis associated with liver abscesses or liver cirrhosis, cholangitis secondary to high malignant biliary strictures or after percutaneous transhepatic cholangiography, female gender, and age). When the presence of each of these factors is weighted proportional to its coefficient of regression, patients with cholangitis could be scored on a scale of 0-27. A score of seven was clinically the most useful cut off--388 attacks of cholangitis associated with a score of less than 7 had a mortality rate of only 1.8%, whereas 61 attacks associated with a score greater than or equal to 7 had a mortality rate of 49%. The value of this scoring system needs to be confirmed in prospective studies, but it may prove useful, for example, in selecting a group of high-risk patients for urgent biliary decompression in an attempt to reduce the mortality associated with this pathology.

Profuse hemorrhage into the biliary tract--major hemobilia,--is an alarming condition which attracts much attention. Minor hemobilia, often caused by gallstones or operative injury, is much more frequent, yet often neglected. Clinical observations indicate that minor hemobilia is not always an innocent condition with the blood remaining fluid and unobtrusively flowing into the intestine. Examples are given where clots from occult hemobilia have caused diagnostic errors or obstructed the bile flow, thus imitating gallstones for which they may be mistaken. Experiments have been performed to elucidate the pathophysiology of this clot formation: 1) A model of the biliary tract was constructed with bile flowing through it. When blood was injected forcefully to simulate a major hemorrhage, mixed clots of blood and bile were formed. When introduced gently, as in minor hemobilia, the blood flowed immiscibly to the lowest level where it formed a clot of pure blood. The clots dissolved under the influence of flowing bile, but remained intact when protected from the flow. 2) Mixed clots were mushy and dissolved spontaneously, while pure clots remained solid and stable. When clots containing increasing amounts of bile were incubated, increasing amounts of cleavage products of fibrinogen and fibrin were formed. 3) Blood clots were produced in gallbladders of 33 dogs. Pure clots remained solid and floating while mixed clots formed a mushy layer, strongly adherent to the mucosa. Both kinds of clots disappeared after two-four weeks, but remained if bile flow was diverted. These findings explain why under certain circumstances minor hemobilia acquires clinical significance by forming clots that may obstruct the flow or cause diagnostic errors. Successful dissolution of "retained stones" may occasionally have the simple explanation that blood clots, mistaken for calculi, have been fibrinolysed. Consequently, in biliary obstruction or when defects are found on cholangiography, the possibility of blood clots in the ducts should be considered even in the absence of overt gastrointestinal hemorrhage.

Thirty patients were identified as having primary common duct stones. Criteria for diagnosis included at least a two year symptom free interval following cholecystectomy; soft, light brown stones or sludge present in the common duct; and the absence of a long cystic duct remnant or a biliary stricture from the previous surgery. The average age of the 30 patients was 66 years. The interval between cholecystectomy and the diagnosis of primary common duct stones averaged 12 years. Acute cholangitis was a frequent mode of presentation. At the time of surgery the bile duct was often dilated out of proportion to the serum bilirubin. In only one of the 30 patients was ampullary stenosis present. Twenty-six of the 30 patients had only stone extraction and insertion of a T tube for treatment of their primary common duct stones. Twenty-two of the 26 were followed for an average of four years and nine months with no evidence of recurrent stones in 82% (18/22). Four developed recurrent primary common duct stones one, five, five, and 7 years later. It is concluded that most patients with primary common duct stones do well after stone extraction alone.

Since 1974, 16 consecutive infants with biliary atresia have been treated by hepatic portoenterostomy employing an exteriorized Roux-en-Y intestinal segment (Miluliez). Simultaneous, sequential analyses of bile pigments and lipids in serum and biliary drainage were performed. In the 11 patients with sustained bile drainage, progressive increases in bile volume, bilirubin and biliary lipid concentrations correlated well with their subsequent return toward normal in the serum. Despite relief of biliary obstruction, four patients have had progressive liver cirrhosis. The other 7 have residual liver damage which has been stable, or in two instances, improved, at late biopsy. The clinical and biochemical results suggest that both obstructive and parenchymal factors are operative in infants with biliary atresia.

Obstructive cholangitis with acute renal failure is a dramatic syndrome which merits individual definition. Twenty-one patients with acute suppurative cholangitis complicated by rapidly developing renal insufficiency were studied, and the severity of the renal failure, an acute interstitial tubulopathy, bore no significant relationship to the serum bilirubin level. The mechanism of renal damage was clearly related to episodes of septicemia. Increasing experience has modified the approach to treatment. The dominant septic problem can often be controlled by vigorous antibiotic and fluid therapy, allowing time for spontaneous improvements in renal function. All patients thus operated at a distance from the septic episode survived. If emergency operation is required because of persistent or recrudescnet sepsis, the necessity for dialysis should be considered first; the circumstances demanding dialysis are defined. The priorities in therapy are then: 1) treatment of the infection, 2) treatment of the renal failure, and finally 3) operation. The amount of the operation depends on the evolution of the sepsis, but should be preceded by dialysis when required.

The clinical, radiologic and pathologic features of 37 patients diagnosed as having primary sclerosing cholangitis (PSC) were reviewed. Sixty-two per cent were men, and 35% had ulcerative colitis. The patients demonstrated considerable variability in their natural history and pathology. It appeared that they could be divided into four fairly distant groups (1) sclerosing cholangitis affecting primarily the distal common bile duct; (2) sclerosing cholangitis occurring soon after an attack of acute necrotizing cholangitis; (3) chronic diffuse sclerosing cholangitis; and (4) chronic diffuse sclerosing cholangitis associated with inflammatory bowel disease. It is suggested that the patients in these groups may have different etiologies, may respond to different treatment regimes, and may have different prognoses.

The cholangiograms of 36 patients with sclerosing cholangitis were reviewed. The mean age of the patient group was 43 years, and the mean disease duration was 4.5 years. Seventeen of the patients had associated inflammatory bowel disease. The mean serum bilirubin was 6.8 mg/dl, the mean SGOT was 105 IU/L, the mean SGPT was 108 IU/L, and the mean serum alkaline phosphatase was 534 IU/L. The cholangiograms demonstrated involvement of the extrahepatic bile ducts in 33 patients, involvement of the hepatic duct bifurcation in 33 patients, and involvement of the intrahepatic bile ducts in 35 patients. The cholangiograms were graded as to the areas of the most severe obstructive involvement. In 24 patients the area of most severe involvement was the hepatic duct bifurcation. In eight additional patients the hepatic duct bifurcation, along with the extrahepatic ducts and/or the intrahepatic ducts, were felt to be the areas most severely affected. This predilection for severe obstructive disease at the hepatic duct bifurcation in sclerosing cholangitis held for both patients with and without inflammatory bowel disease. Thus, most patients with sclerosing cholangitis have cholangiographic evidence of diffuse extrahepatic and intrahepatic biliary tract disease, with the hepatic duct bifurcation being the area generally most severely affected.