Since the success of Kasai portoenterostomies (KPE) in patients with biliary atresia, operative results have improved remarkably, and long-term survivals following the procedure have gradually increased. Despite evidence of a restoration in bile flow, however, liver cirrhosis and portal hypertension have remained as significant life threatening problems throughout patients' life times [1,2]. Herein, we analyzed long-term survival rates and current status of the living patients who underwent KPE over 10 years prior.

We analyzed medical records of the 32 patients with KPE performed for biliary atresia at our institution between 1990 and 2000, retrospectively. Telephone interviews were made when the authors needed further information lacking in the medical records. We analyzed 10-year survival rate by Kaplan-Meier method. Numbers of the death, causes, and the current status of survivals over 10 years were also evaluated. Transplanted cases were considered as death in our study.

Although there has been an increase in the number of long-term survivors of biliary atresia, the disease is still one of the most challenging problems in the field of pediatric surgery because of progressive fibrosis, portal hypertension, and liver cirrhosis [3]. Several investigators have reported that KPE plays a role in gaining time for liver transplantation but is not a curative treatment [3,4]. However, the results of KPE seem to steadily improve, especially in Japanese studies. Altman et al. [5] reported 45% of 5-year survival rates with native liver and 35% for 10-year survival rate in 266 patients in 1997. Davenport et al. [6] showed their experience of 338 infants with biliary atresia, as their current survival rate of 5-years with native liver was 60% and 10-years survival was 41%. In 2003, Nio et al. [7] published 59.7% and 52.8% for 5- and 10-year survival rate, respectively, from the Japanese Biliary Atresia Registry. Furthermore, Shinkai et al. [1] showed improvement of post-KPE survival rate with almost 90% for 5years and nearly 80% for 10 years in patients who were operated on in the 1980s. We also reported increased 5-year survival rate in previous reports [8]. Our results showed 76.2% 10-year survival rates with native liver. As such, these increased post-KPE long-term survival rates suggest that KPE is still considered as first choice of surgery in patients with biliary atresia in the era of liver transplantation.

However, despite initially good surgical outcome of KPE, a few long-term survivals had portal hypertension, esophageal or gastric fundal varices due to hypersplenism, cholangitis, and hepatic failure caused by progressive ongoing cirrhosis and finally were in need of liver transplantation. Portal hypertension is considered the main cause of comorbidity among long-term survivals of biliary atresia. It could be presented as various types of variceal bleeding or hypersplenism. According to two series of long-term follow-up studies in Japan and France, which analyzed native liver after KPE over 20 years, incidence of portal hypertension were 49% and 70%, respectively [1,9]. In our study, one-third of survivals over 10 years had portal hypertensive events, which shows a similar incidence to previous reports [1,9]. Because biliary atresia is not restricted to the extrahepatic bile duct, patients who had KPE successfully performed display abnormalities of the intrahepatic biliary tree, including stenosis, dilatation, and sometimes pseudocyst formation. Intrahepatic change could lead to hepatic cirrhosis and affect portal hypertension [9]. Management of various variceal bleeding has developed according to the site and types of bleeding. In our cases, one esophageal variceal bleeding was controlled with endoscopic band ligation, which is considered as first line of treatment in variceal bleeding with sclerotherapy [9,10]. Another case of gastric fundal variceal bleeding was controlled with BRTO as previously reported [11]. Percutaneous embolization of a branch of jejunal artery was performed in one case of jejuna arteriovenous malformation who had suffered a chronic episode of melena. Another portal hypertensive complication of hypersplenism could be managed with PSE without splenectomy. Nio et al. [12] suggested that PSE should be used rather than splenectomy because it prevents not only the risk of post-splenectomy overwhelming sepsis but also severe intraabdominal adhesion, which could make dissection difficult during liver transplantation. Furthermore, they concluded the long-term efficacy of PSE was 70%. Both patients in our study who suffered hypersplenism were treated successfully with PSE.

With respect to cholangitis, Hadzic et al. [2] reported 41% of patients had cholangitis in 28 long-term survivors over 10 years, and Nio et al. [13] reported 48% in 21 long-term survivors over 20 years. Our series showed 26.3% in 19 survivors over 10 years, all of whom were managed with intravenous antibiotics.

Some authors reported that intrahepatic biliary cyst occurred in 23 to 25% of patients after KPE, and it has been considered that patients with intrahepatic cyst have poor outcomes compared to those who do not [14,15]. In our study, there were 2 patients with intrahepatic cyst. One survived more than 9 years without any procedure or management with constant size. The other case was performed intrahepatic cystojejunostomy as percutaneous transhepatic cholangio-drainage had failed. We have previously reported this case [16], and this patient finally received liver transplantation due to progressive hepatopulmonary syndrome.

The long-term survival rate after KPE in biliary atresia is slightly improving. However, two thirds of patients who survived over 10 years suffer from various complications including portal hypertension, cholangitis, intrahepatic cyst, and intestinal obstruction in spite of successful KPE. One-third of long-term survivals remain alive without any problem. Careful follow-up is needed because of some manifestations of ongoing liver cirrhosis and thus need planning for liver transplantation.