The surgery of "correctable" biliary atresia

Seven of 114 (6%) patients treated in Denver for biliary atresia had a variant of the disease in which only microscopic bile ducts or ductules were identified at the porta hepatis, hepatic lesions were present, even in the neonatal example, and intrahepatic biliary hypoplasia was uniformly present. The excised bile duct cysts consisted of fibrosis or scar tissue and with little or no epithelial lining. Six patients had corrective surgery. The first patient was treated by choledochoenterostomy and became totally obstructed. Definitive operation in this patient and all subsequent patients consisted of excision of all extrahepatic duct structures and Roux-en-Y portoenterostomy. Two patients died, the one who did not have surgical correction and a second who had correction at age 35 weeks. The other five patients have been followed for 18 to 158 months and are anicteric but have some degree of residual liver damage. We conclude that so-called correctable biliary atresia occurs in less than 10% of cases, that because of coincident biliary hypoplasia, "cure" is not a possible outcome, and that the treatment of choice is complete surgical excision of the extrahepatic bile ducts and Roux-en-Y portoenterostomy.     

The pharmacokinetics of the biliary excretion of ciprofloxacin

The pharmacokinetics of ciprofloxacin excretion have been studied in 54 patients undergoing biliary and pancreatic operations with and without obstruction of the common bile duct. High concentrations were achieved in common duct bile within 20 minutes of intravenous injection and persisted for over 3 hours after 100 mg and for over 8 hours after 200 mg. The concentration of ciprofloxacin in the bile of functioning gall bladders was much greater than that in the common duct bile. Remarkably, it was identified in therapeutic concentrations in the bile of obstructed ducts. This and the rapid fall from initially high venous concentrations probably reflect diffusion from the circulation as a result of the exceptional tissue penetration. A unique feature of this study was the finding of clinically significant concentrations in the bile of obstructed ducts. Two patients developed wound infection and no side effects were observed. The broad spectrum antibiotic ciprofloxacin has potential as a useful agent for prophylaxis in biliary surgery maintaining biliary and venous concentrations in excess of the MIC90 for most biliary pathogens for more than 8 hours.     

Endoscopic biliary endoprosthesis in the palliation of malignant obstruction of the distal common bile duct: a randomized trial

A total of 52 jaundiced elderly patients who had malignant obstruction of the distal common bile duct and who required palliative biliary decompression were randomized to receive either an endoscopically placed biliary endoprosthesis (10 French gauge) or conventional surgical bypass. Patients within the two treatment groups were well matched and 51 were followed until their death. Patients treated with endoprosthesis had a significantly shorter initial hospital stay than those treated surgically. In the long term, overall survival in the two groups was similar and jaundice was relieved in over 90 per cent of patients. Despite more re-admissions to hospital for those patients treated endoscopically, the total time spent in hospital still remained significantly shorter in this treatment group compared with those subjected to surgery. The endoscopically placed biliary endoprosthesis is a valuable alternative to conventional surgical bypass in the palliation of extrahepatic biliary obstruction.     

Clinico-pathological studies on a transitional type between extrahepatic biliary atresia and paucity of the interlobular bile ducts

Among the neonatal and infantile cases of obstructive jaundice seen at Niigata and Yamagata University Hospitals between 1976 and 1990, extrahepatic bile ducts were visualized in 19 cases by either preoperative endoscopic retrograde cholangiopancreaticography (ERCP) or intraoperative cholangiography. Neonatal hepatitis was diagnosed in 3 of these cases by clear images of the bile duct system extending from the common bile duct to the intrahepatic bile duct. In 7 cases, the common bile duct was able to be seen, while the common hepatic duct was only slightly visualized. Four of these 7 cases were consistent with paucity of the interlobular bile ducts (PILBD) based on hepatic histology, while the remaining 3 showed fibrosis, bile ductular proliferation, and many bile plugs in the bile ductuli of the portal areas, concurrent with histological changes in extrahepatic biliary atresia (EHBA), not PILBD. In 9 cases, only the common bile duct was visualized while the common hepatic duct was not seen, 7 of these 9 cases being consistent with type III-al EHBA. In 2 cases, neither fibrosis nor proliferation of the bile ductuli was observed in the portal areas, and portal areas without any bile ductuli were also seen, in accordance with findings for PILBD. Three cases which showed similar hepatic histological findings to EHBA despite the presence of patent extrahepatic bile ducts, and 2 cases which had obstructed extrahepatic bile ducts and hepatic histological findings similar to PILBD, were thought to be of a transitional type between EHBA and PILBD. Thus, it is postulated that the entire bile duct system covering hepatocytes to extrahepatic bile ducts is affected by a certain factor, but the expression of individual disease types may be dependent upon the location of the site most seriously affected.     

梗阻性黄疸肝内增生胆管上皮细胞凋亡的实验研究

目的：研究梗阻性黄疸肝内胆管上皮细胞凋亡对增生胆管的影响。方法：应用末端脱氧苷酸转移酶介导的脱氧三磷酸尿苷（dUTP）缺口末端标记技术（TUNEL）观察大鼠胆道梗阻及胆肠内引流术后肝内胆管上皮细胞凋亡与胆管增生的关系。结果：胆道梗阻后，肝内胆管明显增生，胆管上皮细胞凋亡明显增加；早期胆肠内引流术后，增生的胆管及胆管上皮细胞凋亡明显减少。结论：胆道梗阻胆管上皮细胞凋亡增加可能与清除过度增生的胆管有关，是机体维持自身组织稳定的一种重要机制；早期胆肠内引流术后，随着增生的胆管减少，胆管上皮细胞凋亡明显减少。     

Biliary complications after liver transplantation: with special reference to the biliary cast syndrome and techniques of secondary duct repair.

In 93 consecutive cases of orthotopic liver transplantation, there were 24 example of biliary obstruction and eight of bile fistula formation. Six of the obstructed livers developed biliary cast formation so extensive that the smaller intrhepatic ducts became plugged to an extent that they could no longer have been treated by surgical mena. In each of the six cases, the most important causative factor was neglected obstruction of the large bile ducts with the intrahepatic lesions apparently being late and secondary. Stone and/or cast formation also occurred in other obstructed livers in the presence of bile fustulas, but these deposits were limited to the large ducts where they could have been or were removed. Although homograft bile undoubtedly has increased lithogenicity at certain posoperative times, the data from the present study have shown that biliary sludge formation essentially is always associated with defective bile duct reconstruction, and the observations have underscored the urgency with which reoperation must be considered. Techniques of secondary intervention have been described, with emphasis on conversion of cholecystojejunostomy to choledochojejunostomy. This operation has permitted salvage of homografts in eight of nine trials and the survival of seven patients.     

Agenesis of the extrahepatic bile ducts: report of five cases

Five patients were encountered in whom agenesis either of the proximal extrahepatic biliary ducts (four patients) or total absence of the extrahepatic bile ducts and gall bladder (one patient) were evaluated. Jaundice was diagnosed from at birth to 3 weeks of age (average, 1.2 weeks of age) in these five patients. The patients' ages ranged from 2 to 8 weeks at the time of surgical exploration. Findings at surgery showed either absence of the entire extrahepatic biliary ducts or proximal bile duct remnants, no evidence of an inflammatory process, and no fibrous mass present at the portahepatis. Liver biopsy specimens showed histological evidence of cholestasis, minimal bile duct proliferation and fibrosis, and nearly complete absence of inflammation. In three patients in whom a portocholecystostomy was performed, no bile flow was obtained. Two patients underwent surgical exploration and liver biopsy only. One patient died as a result of severe congenital heart disease at 3 months of age. Four patients have undergone successful hepatic transplantation. These patients are now 10 months to 6 years of age. In our review of the literature, we were unable to find any reports of bile duct agenesis despite the fact that it appears to be a known phenomenon. We conclude that patients with biliary agenesis have early onset of jaundice when compared with patients with biliary atresia, absence of inflammation at the portahepatis at the time of surgical exploration, as well as on biopsy of the liver. Portoenterostomy or portocholecystostomy are likely to fail. We believe that liver transplantation is the treatment of choice for this rare entity.     

Biliary Atresia: histopathologic observations and reflections upon its natural history

Operative liver biopsies and specimens of the extrahepatic ducts and porta hepatis have been studied in 12 cases of biliary atresia. In all cases, the liver showed giant cell transformation and inflammation with mononuclear cells and neutrophils. Most had other features of neonatal hepatitis, including necrosis of hepatocytes. In the intrahepatic bile ducts of all cases but one, the hepatic ducts and glands at the porta hepatis, and in the extrahepatic ducts where epithelium remained, there was degeneration of the epithelium and intramural inflammation. In the ducts at the porta hepatis and in 6 of 8 extrahepatic ducts where epithelium remained, there was extensive mural fibrosis compromising the diameter of the duct lumens. Three cases showed the inflammatory changes distal to sites of closure of the extrahepatic ducts. These findings demonstrate that in biliary atresia, hepatitis, intrahepatic cholangitis, and sclerosing cholangitis of the extrahepatic ducts all interact to produce acquired obstruction to bile flow.     

The reliability and clinical limitations of sonographic scanning of the biliary ducts.

Sonographic scanning of the biliary ducts has been successfully used as a screening test to distinguish between patients with surgical and medical jaundice, with an accuracy of 90%. However, there is no consensus in the literature on what numerically defines a dilated biliary duct. To clarify this problem a prospective study of 102 consecutive patients was initiated to determine the sonographic size range of bile ducts in patients with and without extrahepatic ductal obstruction. The ultrasonic measurements were compared with direct measurements of the common bile duct, at surgery. The extrahepatic ductal system was visualized sonographically in 62% of the patients, while the intrahepatic ducts were found in 81% of the population. Direct measurements at operation agreed with the ultrasonic measurements in 84% of the patients. Analysis of the size range of the biliary ducts in patients with and without extrahepatic obstruction, by chi square analysis and the Student's t-test, allowed the following guidelines to be established. Extrahepatic bile duct obstruction was present if the extrahepatic bile ducts was 1 cm or wider (p less than 0.001) or if the intrahepatic bile duct was in excess of 0.5 cm (p less than 0.001). Similarly if the extrahepatic bile duct measured less than 0.8 cm sonographically, and the intrahepatic bile duct was 0.4 cm or less than bile duct, obstruction was not present (p less than 0.001).     

Should endoscopic stenting be the initial treatment of malignant biliary obstruction?

Forty-two patients with biliary obstruction caused by a stricture had a diagnostic ERCP with subsequent insertion of a straight 10G endoprosthesis. These patients represented 70% of a cohort in which stent insertion had been attempted. The majority (63%) had pancreatic carcinoma, but 22% had malignant hilar obstruction. Five patients (12%) died within a few days of stent insertion; ERCP may have contributed to two deaths. Jaundice was relieved in all survivors. Median hospital stay was 6 days (range 2-32 days). After further investigation, nine patients were thought to be potentially curable and underwent laparotomy. Late complications after stent insertion alone included cholangitis (26%) and recurrent jaundice (28%). Only one patient developed gastric outlet obstruction and needed a gastroenterostomy. Median survival in the endoprosthesis group was 11 weeks (range 2-84 weeks). Survival was longer for patients with bile duct (14 weeks) rather than hilar strictures (6 weeks). Median survival after subsequent surgery was 40 weeks (range 4-80 weeks) with two long-term survivors. This study confirms that ERCP and stent insertion is a useful initial treatment for obstructive jaundice due to a biliary stricture, being both diagnostic and therapeutic. Subsequent evaluation for curative surgery is not precluded and in the majority of cases worthwhile palliation may be achieved by stenting alone.     

Incidence of intrahepatic biliary cysts in biliary atresia after hepatic portoenterostomy and associated histopathologic findings in the liver and porta hepatis at diagnosis.

BACKGROUND/PURPOSE: The association of intrahepatic biliary cysts in biliary atresia (BA) after hepatic portoenterostomy has drawn the attention of many pediatric surgeons and has become an important subject in clinical management, but the incidence and causes of this association are not well known. METHODS: During the last 14 years, we operated on 33 BA patients by hepatic portoenterostomy. Five patients could not be followed up over a 1-year period, so the remaining 28 patients had their intrahepatic structure investigated by periodic magnetic resonance imaging (MRI), and were confirmed at autopsy. The authors also reviewed the preoperative conditions of these patients and the histopathologic changes in the liver biopsy and the porta hepatis specimens taken at portoenterostomy. RESULTS: (1) Intrahepatic biliary cysts were found in 6 of the 28 patients studied (21%). Of 16 patients who were operated on after 1989 and are undergoing follow-up by MRI, 4 (25%) had cysts. (2) Five of the 6 patients with cysts had episodes of cholangitis before or at the time of discovery of the cysts, and 4 of them have had their jaundice reappear since the discovery of the cysts. (3) The intralobular spaces (ILS) with fibrosis and the number of bile ducts had increased in the group with cysts compared with those in the group without cysts. (4) The bile duct maximum size in the porta hepatis (PH) was similar in the groups with and without cysts, but a denudation of the mucosal lining cells in the duct, and a fibrosis and inflammation around the duct had increased in the group with cysts compared with those in the group without cysts. (5) Statistically, the fibrosis found in the ILS and the inflammation around the bile duct found in the PH were induced as a correlative factor affecting cyst formation. CONCLUSIONS: The association of intrahepatic biliary cysts in BA after portoenterostomy is frequent. The fibrous change in the ILS and the inflammatory process around the bile ducts might be potential causes of cyst formation.     

Forme fruste choledochal cyst

Four patients had the characteristic features of choledochal cyst except for the cystic component. All patients had stenosis of the distal common bile duct, a "long common channel" secondary to a proximal junction of the common bile and pancreatic ducts, cholecystitis and the classic pathological microscopic features of choledochal cyst in the wall of the common bile duct. Three children had coexisting intrahepatic duct cysts and/or stenosis and one had intrahepatic choledocholithiasis. The clinical presentations were cholangitis (2), pancreatitis (1) and biliary obstruction (1). In all cases the common bile duct was resected and biliary reconstruction was carried out by choledochojejunostomy (Roux-en-Y). Morbidity was minor except in one patient with ductal disease extending far into the intrahepatic ducts. This child developed an anastomotic stricture requiring revision of the anastomosis and long-term "U" tube stenting. Forme fruste choledochal cyst appears to be another variation in the spectrum of pancreaticobiliary malformations of choledochal cyst. Treatment is identical, that is, excision of all malformed ductal tissue.     

Percutaneous-endoscopic placement of endoprostheses for relief of jaundice caused by inoperable bile duct strictures.

Fifty-three patients with biliary obstruction caused by unresectable malignancy were treated by attempted insertion of an endoprosthesis by the percutaneous-endoscopic route. This was successful in 50 patients. A single endoprosthesis was inserted in each case. Both right and left hepatic duct decompression were obtained in 31 patients, but only unilateral or segmental drainage was achieved in 19 patients. Procedure-related complications occurred in 18 (36%) patients, and 15 (30%) patients died within 30 days of the procedure. Satisfactory resolution of jaundice was obtained in 26 (84%) patients with bilateral decompression and in 12 (63%) of those with unilateral drainage. The 30-day mortality rate was 26% for patients with bilateral and 37% for those with unilateral drainage. The morbidity rate from cholangitis after endoprosthesis insertion was 10% after bilateral and 32% after unilateral drainage. None of these differences was statistically significant. Surviving patients with satisfactory bile drainage were relieved of symptoms such as pruritus. The combined percutaneous-endoscopic technique enables difficult biliary strictures to be intubated. Although bilateral duct drainage is preferable, the palliation is often worthwhile even when segmental ducts alone are drained.     

Presence of multiple bile ducts in the liver graft increases the incidence of biliary complications in pediatric liver transplantation.

We studied the impact of multiple bile duct anastomosis on the development of biliary complications after liver transplantation in children. A total of 101 patients received a primary liver transplant and were divided into 2 groups: those with a single bile duct (n = 77) and those with multiple bile ducts (n = 24). Mean follow-up was 39.8 +/- 20.8 months. A total of 27 patients presented with biliary complications (26.7%), 18 patients (18.7%) presented with early complications (12 leaks and 6 strictures), and 9 patients (8.9%) had late strictures. Hepatic artery thrombosis (HAT) and multiple bile ducts were significant risk factors for the development of biliary complications, and the presence of multiple bile ducts was an independent risk factor. Patients with multiple bile ducts had a significantly greater incidence of total biliary complications compared to those with single ducts. Patients with multiple ducts had a higher incidence of leaks when compared to those in the single duct group, but the incidence of strictures, both early and late, was similar in both groups. One-year patient and graft survivals were not statistically different in the 2 groups. In conclusion, the presence of more than one bile duct in the graft is an independent risk factor for the development of biliary complications after pediatric liver transplantation.     

Hepatic portocholecystostomy for biliary atresia.

Four infants with biliary atresia had gross obliteration of the common hepatic duct but residual patency of the gallbladder, cystic duct and common bile duct. The patients were treated by hepatic portocholecystostomy utilizing the extant bile ducts for biliary reconstruction. Bile drainage was achieved in all four infants. There was a conspicuous absence of postoperative cholangitis. Subsequent obstruction of the distal ducts in two patients necessitated reoperation and construction of a standard biliointestinal conduit. The other two children are surviving, jaundice-free, 5 1/2 and 5 years after operation with minimal sequelae of biliary atresia. Hepatic portocholecystostomy is a feasible surgical alternative to intestinal reconstruction in patients with biliary atresia in whom the disease is limited to the proximal extrahepatic bile ducts.     

经内镜胆道支架选择方式对恶性胆总管梗阻的疗效分析

目的观察各胆道支架选择方式对不同病因恶性胆总管梗阻的疗效、术后并发症及治疗费用。方法选择2010年7月至2015年12月在我院肝胆外科因恶性胆总管梗阻行ERCP术置入胆道支架治疗的47例患者作为研究对象,按照置入胆道支架的不同,所有病例分为金属支架组(A组,n=16)、单塑料支架组(B组,n=17)和多塑料支架组(C组,n=14),观察三组患者支架通畅时间、并发症及手术相关费用。结果对于胰腺癌型,A组和C组的平均支架通畅时间明显比B组长(P0.05),且A组优于C组,但差异无统计学意义(P0.05);对于胆管癌型和壶腹周围癌型,A组和C组的平均支架通畅时间明显比B组长,差异有统计学意义(P0.05);其中C组优于A组,但差异无统计学意义(P0.05)。结论针对不同病因的恶性胆总管梗阻患者,选择个性化胆道支架治疗方案,既提高治疗效果,减少并发症,又降低医疗费用,为患者的后续治疗节省成本。     

The etiology of "white bile" in the biliary tree

"White bile" is the colorless fluid occasionally found in occluded biliary systems. The absence of pigments in this "bile" was not satisfactorily explained. The objectives of this study were to assess its etiology. In dogs, "white bile" developed whenever both the common bile duct and the cystic duct were ligated. In comparison, dark green ("black") bile occurred when only the common bile duct was ligated leaving the gallbladder in communication with the obstructed ducts. The pressure in extrahepatic ducts containing "white bile" was significantly higher than in those filled with "black bile." Flow in the extrahepatic ducts was assessed by the aid of radioiodinated human serum albumin (RIHSA). When "black bile" was present, the direction of flow was from the extrahepatic ducts into the gallbladder. Whenever "white bile" developed, a reverse flow from the extrahepatic ducts into the liver was observed. Thus, the role of the gallbladder appears to be decompression of the biliary system allowing bile flow from the liver even in obstruction. In the absence of the gallbladder water absorption activity, the colorless secretion of the bile ducts seems to "back wash" into the liver and replace the bile present in the ducts at the time of occlusion.     

In biliary atresia duct histology correlates with bile flow

Three basic types of microscopic biliary structures at the portahepatis were distinguishable in infants with biliary atresia: bile ducts, collecting ductules of biliary glands, and biliary glands. Correlation between the type of biliary structure and the quantity and quality of post-operative bile flow was possible in 23 instances. At 2 weeks after operation, the 11 patients in whom a bile duct was identified had a daily bile flow of 68.0 +/- 11.5 mL. Bilirubin concentration in the bile was 13.6 +/- 3.3 mg/dL and total daily bilirubin excretion was 8.77 +/- 2.74 mg. In contrast, bile flow in 12 patients having only collecting ductules and/or biliary glands in the porta hepatis was 19.1 +/- 3.9 mL and bilirubin concentration in bile was 1.7 +/- 0.3 mg/dL. Thus, total daily bilirubin excretion was 0.34 +/- 0.08 mg (P less than 0.001). Postoperative cholangitis occurred only in patients with ducts. It is concluded that only bile ducts communicate with the intrahepatic biliary system and drain bile after hepatic portoenterostomy.     

Technical considerations to maintain a low frequency of postoperative biliary stent-associated infections

Abstract. Background/Purpose: In patients with malignant biliary obstruction, preoperative biliary tract manipulation and stent drainage has been associated with increased infectious complications and mortality. Methods: Between October 1996 and September 2000, 36 patients underwent bilioenteric anastomosis by a single surgeon. Diagnoses included pancreatic and other periampullary cancers (67%), benign obstruction (14%), hilar cholangiocarcinoma (8%), and other malignancies (11%). Preoperative bile duct manipulation had been done in 72%, and a biliary stent had been placed in 58%. Two-thirds of patients underwent major resection and the remainder were treated with internal biliary bypass. All patients had received preoperative bowel cleansing and perioperative antibiotics. Bile ducts were left clamped after incision until anastomotic completion to avoid biliary spillage, and drains were generally not placed. Results: Intraoperative bile cultures were positive in 73%. This was strongly linked to the presence of a stent (P = 0.0004), or prior duct manipulation (P = 0.002). There were 3 postoperative, unrelated deaths in patients after palliative bypass (overall mortality rate, 8.3%). Postoperative infections occurred in 7 patients (19%), of which three were due to a similar organism. There was one biliary leak, no pancreatic leak, and no intraabdominal abscess. Conclusions: Appropriate preoperative antibiotic coverage, preventing intraoperative peritoneal bile contamination through temporary bile duct occlusion, and avoiding routine drain placement, are strongly suggested for patients in whom preoperative biliary manipulation has taken place. Received: June 07, 2001 / Accepted: November 16, 2001