Corticosteroid therapy in biliary atresia

Sixteen patients with biliary atresia had 44 steroid courses for treatment of cholangitis or diminution of bile flow following Kasai hepatic portoenterostomy operations. A "blast" type (high dose/short duration) steroid administration was employed to potentiate the choleretic and anti-inflammatory effect. There was a significant augmentation of bile flow and a reduction in maximum temperature, serum bilirubin, and alkaline phosphatase.     

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.     

Hepatolithiasis after hepatic portoenterostomy for biliary atresia

Background

Hepatolithiasis after hepatic portoenterostomy for biliary atresia has been paid little attention, with only 22 reported cases.

Patients and Methods

Fifteen patients underwent living-related liver transplantation for biliary atresia after hepatic portoenterostomy in our hospital between 1998 and 2004. The resected livers were examined for the existence and location of hepatolithiasis, composition of the calculi, and bacterial infection of bile. The relation between a history of cholangitis and the presence of hepatolithiasis was analyzed.

Results

Intrahepatic calculi were found in 8 (53%) of 15 patients. The calculi consisted of almost 100% calcium bilirubinate. Calculi were found in bile lakes in 8 patients. Bacteria were present in the bile in 8 (53%) of the 15 patients. Of the 8 patients, 7 (88%) had a history of ascending cholangitis.

Conclusions

Hepatolithiasis occurs after hepatic portoenterostomy for biliary atresia more frequently than previously thought. Bile stasis and possibly bile infection are the main causes of calculi formation.     

Long-term outcome in type I biliary atresia

Purpose

This retrospective study reviews the long-term outcome of type I biliary atresia (BA).

Methods

Three hundred twenty-three patients with BA, including 50 with type I, underwent corrective surgery. The surgical results, role of cholangiograms during the corrective surgery, late complications, and current statuses were evaluated.

Results

The overall survival rate of the nontransplant type I patients was better than that of the type II/III patients (52% vs 33%, P = .0009). Cholangiograms of 32 patients were classified into 3 types: cloudy (48%), treelike (13%), and mixed (39%). Of 26 patients who underwent corrective surgery in 1972 or later, 7 (50%), 7 (78%), and 3 (100%) patients of the cloudy type, mixed type, and treelike type, respectively, have survived without liver transplantation (LTx). Of 18 type I patients who survived more than 20 years without LTx, 7 developed severe late complications. Two of them eventually required LTx after 20 years old.

Conclusions

Use of cholangiograms during corrective surgery might have a long-term prognostic value. The overall survival rate of type I BA was better than that of type II/III. The incidence of late complications was, however, considerably high in the type I survivors. All patients required careful long-term follow-up.     

A study of patients with long-term bile flow after hepatic portoenterostomy for biliary atresia

Thirty-five cases of biliary atresia that demonstrated the creation of internal fistula between intrahepatic bile ducts at the portahepatis and intestine and showed long-term bile flow after our hepatic portoenterostomy were investigated from various aspects. These 35 cases were divided into Group A cases, in which jaundice disappeared within three months after surgery, and Group B cases, in which persistent jaundice was seen more than three months after surgery. (1) Severe liver fibrosis and degeneration of intrahepatic bile ducts were more often seen in Group B cases than in Group A cases. (2) The age at the time of surgery and the size of intrahepatic bile ducts at the portahepatis did not have much influence on the operative results in those 35 cases. (3) Measurement of the amount of bile flow and bile acid excretion obtained from Suruga II enterostomy is useful for evaluating the postoperative results of biliary atresia patients. (4) Reoperation that includes curettage and rehepatic portoenterostomy, and serious postoperative ascending cholangitis were each closely related to postoperative persistent jaundice.     

The intussusception antireflux valve is ineffective for preventing cholangitis in biliary atresia: a prospective study

Purpose: Cholangitis after Kasai portoenterostomy for biliary atresia (BA) strongly influences outcome, and an intussusception antireflux valve (IAV) in the Roux-en-Y limb has been advocated for the prevention of cholangitis without definitive evidence. The authors report a prospective study to evaluate the effectiveness of IAV in BA.Methods: Twenty-one consecutive patients with uncorrectable-type BA were the subjects for this study. IAV was included based on informed parental request to give 2 groups: IAV− (n = 11) and IAV+ (n = 10). The incidence/duration of episodes of cholangitis (temperature > 38°C, elevated serum bilirubin level, and leukocytosis) during the first 6 months after Kasai were statistically compared between the 2 groups (P > .05 was regarded as not significant).Results: Technical variations in surgical technique were minimized because the same surgeon supervised all procedures. There were no significant differences between the 2 groups for age and weight at the time of Kasai, mean size of bile ductules at the porta hepatis, and degree of liver cirrhosis present. Protocols for antibiotic and steroid therapy were the same for both groups. The total number of patients who had cholangitis in the IAV− group was 6 of 11 and 5 of 10 in the IAV+ group (P = NS). The total number of episodes of cholangitis was 16 in the IAV− group versus 17 in the IAV+ group (P value, not significant). The mean duration of an episode of cholangitis (number of days per number of episodes) was 12 ± 6 days in the IAV− group versus 11 ± 7 days in the IAV+ group (P value, not significant).Conclusions: IAV does not make any significant impact on the incidence or duration of cholangitis in the early post-Kasai period.     

Determinants of survival after Kasai's operation for biliary atresia using actuarial analysis.

Survival after Kasai's operation for "noncorrectable" biliary atresia is influenced by (1) age, (2) large bile ducts, and (3) concentration of the bile bilirubin. Critical values are: age less than 10.5 wk, any evidence of a large bile duct, and a bile bilirubin concentration greater than 8.8 mg/100 ml. Using these 3 factors, a predictive model is able to identify a favorable group with an 89% expected 4 yr survival.     

Excellent long-term outcome of hepaticojejunostomy for biliary atresia with a hilar cyst

Background

Biliary atresia (BA) with hilar cyst is an uncommon variant, which constitutes less than 10% of all types of BA, and the operative procedure for this type of BA remains controversial.

Methods

We have had 200 cases with BA from 1963 to 2008 in our institute and our branch hospitals, in which 12 cases (6%) were BA with a hilar cyst. The clinical records of all cases were evaluated retrospectively.

Results

Twelve BA patients with a hilar cyst included 2 boys and 10 girls. The diagnosis of BA was confirmed by intraoperative cholangiography (cloudy or treelike pattern). In all 12 cases, a hepaticojejunostomy was performed at a median age of 71.6 days (range, 24-136 days). The follow-up periods were 1.2 to 23.2 years. The current mean total bilirubin level was 0.8 mg/dL (range, 0.2-3.5 mg/dL), and the mean direct bilirubin level was 0.2 mg/dL (range, 0.0 to 0.8 mg/dL). Methylprednisolone or oral prednisolone was administered in 8 cases after operation, and 10 of 12 cases achieved a jaundice free state. The postoperative complications were cholangitis (n = 10), gastroesophageal varices (n = 7), splenomegaly (n = 3), ileus (n = 1), and pulmonary hypertension (n = 1). The overall survival rate with a native liver was 10 (83.3%) of 12 cases, of which 9 cases were jaundice-free and only 1 case showed recurrent jaundice. The other 2 cases underwent living-donor liver transplantation at age of 2 and 20 years, respectively, and they are currently doing well.

Conclusions

Most cases of BA with a hilar cyst achieved excellent clinical outcome after a hepaticojejunostomy.     

Early outcomes of laparoscopic surgery for biliary atresia

Aim

The aim of the study was to present early outcomes of the laparoscopic technique for biliary atresia with some technical modifications.

Materials and Methods

We reviewed charts of all patients with biliary atresia who underwent laparoscopic portoenterostomy from July to December 2008. There were 11 patients with biliary atresia, including 5 boys and 6 girls. The operation was carried out using 4 trocars. The liver was elevated by 2 transcutaneous stay sutures: one on the round ligament and the other on the gallbladder remnant. The left and right hepatic arteries and portal veins were dissected and retracted laterally by 2 transcutaneous sutures to expose the liver hilum. A stay suture was placed on fibrotic tissue at the liver hilum to facilitate its maximal removal. A jejunal end-to-side anastomosis was constructed extracorporeally. Portoenterostomy was carried out laparoscopically.

Results

Mean operative time was 245 ± 31 minutes. No patient required conversion. There were no operative deaths. Blood loss during operation was minimal. One patient died on day 65 after operation because of intractable hepatic liver. Follow-up after discharge from 10 to 16 months revealed that 6 patients still survived and 4 patients died. One patient died because of milk aspiration at 12 months of age. Three patients died because of repeated cholangitis and liver failure at 10, 10, and 14 months, respectively.

Conclusion

With a modified laparoscopic technique, good early outcomes of laparoscopic surgery for biliary atresia were achieved.     

Prognostic implications of bile lakes after surgery for biliary atresia

Purpose

The aim of the study was to establish the natural history of bile lakes (BLs) in patients with postportoenterostomy biliary atresia (BA) and assess their prognostic implications, in particular, if they are a risk factor for cholangitis.

Methods

One hundred twenty-one postsurgical BA patients were divided into 2 groups as follows: period 1, or preliver transplantation era group (n = 56), and period 2, or liver transplantation era group (n = 65). Presence of BL and outcome were examined in relation to the incidence of cholangitis.

Results

In period 1, there were more multiple BLs (MBLs) in nonsurvivors than survivors. Non-BL survivors had significantly lower mean total number of episodes of cholangitis than MBL survivors (P < .01).In period 2, 58 survived (native liver in 35; transplanted liver in 23). Bile lakes were present in 9/58 (16%) survivors (single in five, multiple BLs in four), and 1/7 (14%) nonsurvivors had MBLs. Mean total number of episodes of cholangitis in the 4 MBL survivors and the 49 non-BL survivors were not significantly different.

Conclusion

Our results suggest that presence of BL is a risk factor for cholangitis in the long-term but not in the short-term.     

Five- and 10-year survival rates after surgery for biliary atresia: a report from the Japanese Biliary Atresia Registry

Purpose

The aim of this study was to elucidate the epidemiology and short- and long-term results of biliary atresia in Japan analyzing the data of the Japanese Biliary Atresia Registry (JBAR).

Methods

In 1989, the Japanese Biliary Atresia Society started a nationwide registry, JBAR, to investigate all aspects of biliary atresia. A total of 1,381 patients, 863 girls, 507 boys, and 11 unknown, were registered between 1989 and 1999. JBAR includes an initial and follow-up questionnaires. Using these patients’ data, the incidence, sex distribution, associated anomalies, the type of obstruction, the type of operation, and the surgical results were evaluated. The 5- and the 10-year results of 735 patients who were registered initially in or before 1994 also were analyzed.

Results

The incidence of biliary atresia was 1 in 9,640 live births. One hundred sixty-four patients (11.9%) had type I atresia of the common bile duct, 34 (2.5%) had type II atresia of the hepatic ducts, and 1,162 (84.1%) had type III atresia at the porta hepatis. Congenital associated anomalies were found in 19.6% of the patients including 33 cases associated with polysplenia. Impact of the age at operation on bile flow was not clear until 90 days of age, and after 90 days the bile flow rate worsened. The original Roux-en-Y procedure had been used in more than 50% of the patients since 1995. In 1999, 96% of the patients underwent the original Roux-en-Y procedure or the Roux-en-Y with an intestinal valve, and only 3 patients (3.5%) underwent other modifications. There were no significant differences in either the rate of disappearance of jaundice or the incidence of cholangitis among these 3 procedures. Of the 735 patients registered in or before 1994, 19 patients (2.6%) were lost to follow-up. The 5-year survival rates of patients registered in 1989, 1990, 1991, 1992, 1993, and 1994 were 62%, 64.5%, 61.3%, 59.0%, 58.7%, and 52.7% without liver transplantation (LTx), and 69.4%, 74.2%, 75.2%, 79.5%, 78%, and 78.3% with LTx, respectively. Although the overall 5-year survival rate changed from 69.4% to 78.3%, the difference was not statistically significant. According to the 10-year follow-up results of the 108 patients initially registered in 1989, 72 (66.7%) and 57 (52.8%) survived with and without the aid of LTx, respectively.

Conclusions

The overall 5- and 10-year survival rates were 75.3% (553 of 734) and 66.7% (72 of 108), respectively. In spite of the increasing number of survivors after LTx, there was no significant improvement in the 5-year survival rate. It was shown that the JBAR system was functioning well with only 19 patients lost to follow-up among the 743 patients registered from 1989 to 1994.     

Long-term results of splenomegaly after surgery for biliary atresia in the native liver

BackgroundBiliary atresia (BA) is a rare disorder characterized by obstructive jaundice in infants, shortly after birth. Postoperatively, some patients exhibit portal hypertension and progressive liver fibrosis. Splenomegaly is a symptom of portal hypertension. We aimed to investigate splenomegaly as a marker for complications of portal hypertension and the relationship between splenomegaly and liver fibrosis in the long-term native liver (NL).MethodsBetween 1977 and 2018, 71 patients underwent hepaticojejunostomy. We included 54 patients (34 NL group, 20 liver transplant (LT) group) who fulfilled the eligibility criteria. Spleen volume (SV), total bile acids, hyaluronic acid, type IV collagen, and aspartate aminotransferase-to-platelet ratio index (APRi) were measured. Data were analyzed using Student's t-test, regression analysis, and receiver operating characteristic (ROC) curve analysis (P < 0.05).ResultsTotal bile acids, hyaluronic acid, type IV collagen, and APRi increased in NL patients with a large SV at >25 years. SV and type IV collagen were correlated with NL for >25 years (r = 0.79 [P = 0.006], y = 1.1 - [0.03 × type IV collagen] [P = 0.008]). In the ROC curve analysis, the cutoff value for type IV collagen was 165 ng/mL (P = 0.07).ConclusionsWe suggest that SV as a prognostic index for End-Stage Liver Disease may be useful in biliary atresia. Long-term follow-up is necessary because the clinical course may be favorable in childhood but worsen during adulthood.     

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.     

胆道闭锁诊治体会

目的　分析胆道闭锁诊断、治疗及预后情况 ,探讨胆道闭锁的早期诊断和治疗策略。方法　回顾性分析我院近 5年收治的 2 8例胆道闭锁患儿诊断手段、手术年龄、手术方式及术后生存率。结果　胆道闭锁早期诊断率为 1 4 % (4 / 2 8) ,手术时年龄 1 3～ 2 70d ,6 0d以内 4例 (1 4 % ) ;6 1～ 90d 1 3例 (4 6 % ) ;91～ 1 2 0d 8例 (2 9% ) ;1 2 0d以上 3例 (1 1 % )。没有患儿接受肝移植 ,1～ 5年生存率为35 % (9/ 2 5 )。结论　密切观察临床表现、掌握胆道闭锁的影像学特点和及时采用腹腔镜探查可提高胆道闭锁的早期诊断率。完善手术方式、让更多的患儿接受肝移植、开辟新的治疗方法如抑制肝胆管细胞凋亡、抗体及基因治疗可提高胆道闭锁的长期治愈率