A report of 42 patients with type Ⅲ biliary atresia treated by hepatojejunostomy / 中华肝胆外科杂志

Objective To determine the clinical effects of type Ⅲ biliary atresia treated by hepatojejunostomy (Kasai procedure).Methods A retrospective study was performed on patients with type Ⅲ biliary atresia treated in our hospital between January 2009 to December 2013.Results The follow-up data were completed in 42 patients out of 48 patients (87.5％) who underwent Kasai procedure for type Ⅲ biliary atresia.The jaundice disappeared in 31 of 42 patients (73.8％).The rate of patients having cholangitis was 47.6％.There were significant differences in the 2-year survival among the groups of patients with mild cholangitis (75.0％,9/12),serious cholangitis (25.0％,2/8) and without cholangitis (86.4％,19/22) (P ＜ 0.05).Conclusions Hepatojejunostomy (Kasai procedure) is an effective operation to treat type Ⅲ biliary atresia.Our data suggested that the survival rates were related to the degree of bile drainage and cholangitis post surgery.

Five-year Survival and Prognostic Factors after Kasai Portoenterostomy for Biliary Atresia

PURPOSE: Long-term survival of biliary atresia patients after Kasai's portoenterostomy is being increasingly reported. Although extended survival has been achieved for many patients, factors influencing outcome have not been defined clearly. The authors investigated 5-year survival rates and prognostic factors for survival after Kasai's portoenterostomy using univariate and multivariable methods. METHODS: The authors analyzed 5-year survival rates and prognostic factors in 56 patients who underwent Kasai's portoenterostomy during the period from 1991 to 2005 by the Kaplan-Meier model and Cox proportional hazards model, respectively. Both clinical factors and histological features of the liver and periportal fibrous mass were analyzed as prognostic factors. RESULTS: Younger ages of less than 90 days at surgery, clearance of jaundice and absence of post-Kasai cholangitis in 6 months were predictive of a favorable outcome. A significant difference between the live and the dead was found with regard to an average of 7 or more bile ductules in periportal fibrous mass in 200 HPF (P=0.013). The external diameter of the longest bile ductule in the periportal fibrous mass is not correlated to the prognosis (P=0.49). Independent factors for good prognosis were jaundice-free in 6 months and over 7 bile ductules density in periportal fibrous mass. CONCLUSION: Favorable outcome after Kasai's portoenterostomy for biliary atresia is determined by bilirubin clearance in 6 months after Kasai operation and seven or more bile ductules in periportal fibrous mass. The 5-year post-Kasai survival rate is 77.2%.

Outcome of Patients Undergoing Kasai Procedure for Biliary Atresia: a Study of Those Surviving More Than 10 Years with Their Native Livers

To evaluate the long-term prognosis of biliary atresia after Kasai operation, a total of 14 patients (of the 41 patients operated upon from 1982 to 1997), who had been followed up for more than 10 years, were included in this retrospective study. Eleven out of 14 patients survived with their native livers, and their data analyzed for age at operation, clearing time of jaundice, histological outcome, postoperative complications, effectiveness after the application of an intussusception anti-reflex valve, and quality of life. Average age at surgery was 62.8 days. Serum bilirubin was normalized within three months in all patients. Six among the eleven long-term survivors had ascending cholangitis as one of the postoperative complications. The application of an intussusception anti-reflux valve did not show any statistical significance in long-term survival. Most of long-term survivors appeared to enjoy good quality of life. Kasai operation might not be the definitive treatment for biliary atresia; however, Kasai operation made it possible to achieve long-term survival for patients with biliary atresia when the patients were detected and treated as early as possible.

Clinical & Nutritional Assessments of Long-term Survivors of Biliary Atresia

To assess the clinical & nutritional status of long-term survivors of biliary atresia, history taking, medical record review, physical examination (height, weight, midarm circumference, triceps skin fold thickness), blood tests (LFT, prothrombin time, platelet count, prealbumin, calcium) and liver needle biopsy were performed in 12 patients in whom Kasai procedure had been performed up to 10 years before at Department of Pediatric Surgery in Seoul National University Hospital. In the height and weight, there was no patient below 5 percentile. Triceps skin fold thickness (TSF) was above 75 percentile in all patients and showed good fat nutritional status. Midarm circumference (MAC) was above 5 percentile in all patients, therefore there was no skeletal muscle protein malnutrition. Serum prealbumin level was abnormal in 2 patients with abnormal liver function and revealed visceral protein malnutrition. Serum calcium level was decreased below normal range in 4 patients with abnormal liver function. One patient had mild ascites. Five patients had abnormal liver function and 7 patients showed clinical manifestation of portal hypertension. Liver needle biopsy was performed in 5 patients and no cirrhotic change was observed. Some patients showed visceral protein malnutrition and vitamin D malabsorption but growth, development and nutritional status were generally satisfactory. Five patients (42%) showed normal liver function and no portal hypertension. In conclusion, Kasai procedure is satisfactory as a primary treatment in biliary atresia but significant portion (7/12) of long-term survivors had abnormal liver function or portal hypertension. Continuous and careful follow-up is needed not to miss the proper time of liver transplantation.