Reduction of the ages at diagnosis and operation of biliary atresia in Taiwan: A 15-year population-based cohort study.

AIM: To describe the ages at diagnosis and operation of biliary atresia (BA) and its incidence over a 15-year period in Taiwan. METHODS: This was a population-based cohort study. BA cases were identified from the Taiwan National Health Insurance Research Database based on the International Classification of Diseases, Ninth Revision (ICD-9) code of BA 751.61 plus Kasai operation (ICD-9 procedure code 51.37) or liver transplantation (LT, ICD-9 procedure code 50.5). The patients' characteristics including sex, age at diagnosis, age at receiving Kasai operation and age at receiving LT were compared among three birth cohorts: (1) 1997 to 2001; (2) 2002 to 2006; and (3) 2007 to 2011. RESULTS: There were a total of 540 BA cases (275 females) with an incidence of 1.62 per 10000 live births. No seasonality of BA was noted. The mean ages at diagnosis of three cohorts were 57.9, 55.6 and 52.6 d. A linear regression model demonstrated a decreasing trend of the mean age at diagnosis (1.27 d per year). The proportion of BA cases that received the Kasai operation within 60 d of age increased from 76% to 81%. A total of 189 (35%) BA patients underwent LT. The mean age at LT was reduced from 3-year-old to 1-year-old. The rates of LT were 25.6% and 32.3% in patients who received the Kasai operation within 60 d or after 60 d of age, respectively. All patients who did not undergo a Kasai operation eventually required LT. CONCLUSION: The ages at diagnosis and operation in BA cases have decreased over time. Kasai operation performed at younger age reduces the need for LT. The incidence of BA in Taiwan fluctuates, but without certain trend.

Biliary atresia - a new derivative method?

BACKGROUND: Biliary atresia is a progressive cholangiopathy in neonates of unknown origin. Surgical intervention - Kasai portoenterostomy - is the only treatment possible. However, only liver transplantation can be considered a definitive solution even in cases of favourable post-operative course, i.e., after bile passage has been recreated. OBJECTIVES: The authors set out to identify ways to optimize the operative treatment in biliary atresia patients. In particular, their objective was to identify a suitable bile derivation area outside porta hepatis, thereby simplifying the surgical procedure of the following liver transplantation. METHODS: The research was conducted from 2006 to 2010 on a set of 30 corrosive casts prepared by the Institute of Anatomy, Medical Faculty, Comenius University Bratislava. The research consisted of an analysis of individual tubular structures of the liver parenchyma, particularly the bile vessels. The authors explored the latter's position and branching out, as well as their relationships in different segments, and their availability in surgical exploration. RESULTS: The analysis of the corrosive casts resulted in selecting an area in liver segments II and III suitable for creating a new type of hepatoenterostomy. The area is sufficiently distant from porta hepatis, conveniently accessible to the surgeon, and has a broad bile duct branch. This type of anastomosis would preserve the anatomic situation in the porta hepatis area intact for the following transplantation. CONCLUSION: While the results of the experiment warrant some optimism, authors will have to wait until this type of derivation operation is applicable in practice. The authors continue their research by performing anastomosis in an animal model (Fig. 5, Ref. 18)

Trends in age for hepatoportoenterostomy in the United States.

BACKGROUND: Biliary atresia is a rare but devastating disease for which hepatoportoenterostomy remains the primary intervention. Increased age at the time of hepatoportoenterostomy is associated with unfavorable outcomes. In this study, we examined trends in age at the time of hepatoportoenterostomy and explored hospital and patient factors associated with more timely diagnosis and treatment. METHODS: Median ages of patients undergoing hepatoportoenterostomy for biliary atresia were compared using the Kids' Inpatients Database from 1997, 2000, 2003, and 2006. The patient and hospital factors associated with later treatment were compared. RESULTS: Of 192 patients, 13.5% had surgery in 1997, 13.5% in 2000, 36.5% in 2003, and 36.5% in 2006. The overall median age was 65.5 days; the median age was 64 days in 1997, 57.5 days in 2000, 69 days in 2003, and 64 days in 2006 (P = .80). Overall, 71% of patients were treated at nonchildren's hospitals, and although the proportion has increased over time, the trend did not reach significance (P = .12). Hispanic and African American patients were more likely to undergo hepatoportoenterostomy after 60 days of life compared with white patients (Hispanic patients: odds ratio, 3.6; 95% confidence interval, 1.1-12.5; P = .04; African American patients: odds ratio, 2.2; 95% confidence interval, 0.8-6.3; P = .14). Compared with specialized children's centers, treatment at nonchildren's hospitals was associated with delayed hepatoportoenterostomy (odds ratio, 3.5; 95% confidence interval, 1.2-9.8; P = .02). CONCLUSION: Although early hepatoportoenterostomy is associated with improved outcomes for children with biliary atresia, our study shows the median age at surgery has not significantly changed over 2 decades. Both hospital and socioeconomic factors play a role in the early treatment of biliary atresia.

Past and future of biliary atresia.

BACKGROUND: With the advent of liver transplantation the outcome of children with biliary atresia (BA) has improved. Is Kasai hepatic portoenterostomy (KHPE) still a valuable option for the treatment of these patients? METHODS: From 1974 to 1998, 77 patients with biliary atresia have been treated at our institution: 50 girls and 27 boys. RESULTS: Seventy-four patients had a KHPE, and 3 patients had no KHPE because of delay in diagnosis. A total of 65 of 74 patients (88%) had undergone KHPE type I, 4 patients (5.4%) KHPE type II, 3 patients (4%) had a Suruga modification, and 2 patients (2.6%) had a portocholecystostomy. Among the 74 patients, 11 were lost to follow-up and their cases were considered failures. Seventeen of our patients are alive at long-term follow-up after KHPE. Among the 77 patients, 33 (43%) had an orthotopic liver transplantation (OLT). Successful KHPE patients underwent transplant at a mean age of 9 years, and KHPE failed at a mean age of 11 months. A total of 25 of 77 (32%) of patients are alive thanks to OLT. In the cohort, the overall survival rate for the KHPE plus OLT is 42 of 77 (55%). Mortality and morbidity rates were more frequent among the younger patients who had early OLT after KHPE failure. In our series, overall survival rate was improved when the patient had a successful KHPE (P < .001). CONCLUSIONS: Kasai hepatic portoenterostomy (KHPE) continues to be a valuable procedure in the treatment of infants with biliary atresia (BA). Successful KHPE permits transplantation at an age at which mortality and morbidity are decreased leading to a better outcome.