Error traps and culture of safety in biliary atresia.

Biliary atresia is a progressive inflammatory sclerosing disease of the bile ducts in the neonatal liver. Without surgical intervention these patients are destined to succumb to the disease. The development of the hepatoportoenterostomy in 1959 and liver transplantation in 1963 ushered a new era of success treating these patients. While many surgical modifications and adjuncts to treatment have been attempted over the last 50 years, the mainstay of treatment to give the child the best chance at prolonged survival with the native liver is a properly performed operation, in a timely fashion, with minimal post-operative complications. This review presents the authors current practice guideline to achieve these goals.

Technical standardization of Kasai portoenterostomy for biliary atresia.

BACKGROUND: The purpose of this study was to assess the clinical outcome of patients treated with the current technique of Kasai procedure compared with that of those treated with previous procedures. METHODS: We retrospectively assessed the outcomes of 256 patients with biliary atresia who underwent Kasai portoenterostomy at our hospital between 1972 and 2014. Patients were divided into four groups: group 1 (1972-1981, double Roux-en Y, n=91), group 2 (1982-1991, Suruga II, n=80), group 3 (1992-2000, double-valve Roux-en Y, n=46), and group 4 (2001-2014, total removal of the extrahepatic biliary remnants at hepatic capsule and Roux-en Y reconstruction with a spur valve, n=39). Clinical outcomes were compared between the four groups. RESULTS: In groups 1, 2, 3, and 4, the rate of jaundice clearance was 65.9%, 77.5%, 63.0%, and 87.2%, respectively; incidence of early cholangitis was 60.4%, 53.8%, 37.0%, and 23.1%, respectively; requirement for redo Kasai surgery was 15.4%, 37.5%, 17.4%, and 5.1%, respectively; 10-year native liver survival rate was 53.8%, 60.1%, 44.1%, and 73.7%, respectively; and 10-year overall survival rate was 55.0%, 72.3%, 86.7%, and 97.3%, respectively. CONCLUSION: The standardized Kasai procedure was associated with favorable outcomes. Long-term outcomes remain to be evaluated. LEVEL OF EVIDENCE: Case-control/treatment study, level III.

Kasai hepatoportoenterostomy in South Australia: a case for 'centralized decentralization'.

BACKGROUND: Recent follow-up studies have demonstrated significant improvement in overall survival as well as survival with native liver following geographic centralization of services to three centres in the UK. However, this model has not been replicated in countries with relatively low population density such as Australia and Canada. METHODS: Retrospective evaluation of all patients born with biliary atresia (BA) in South Australia from 1989 to 2010 was performed. Thirty-one patients with BA were discovered. Two patients were excluded because the initial Kasai procedure (KP) was performed interstate. Outcome parameters measured were (i) clearance of jaundice (bilirubin of less than 20 µmol/L, by 6 months); (ii) survival with native liver; and (iii) overall survival. Kaplan-Meier survival curves were plotted for both survival with native liver and overall survival. RESULTS: The incidence of BA in South Australia between 1989 and 2010 was 7.48 per 100,000 live births. Following KP, clearance of jaundice was achieved in 42.9% of patients. Five-year actuarial survival with native liver was 55.2%, and overall 5-year actuarial survival was 89.3%. CONCLUSIONS: The results of KP performed at Women's and Children's Hospital from 1989 to 2010 can be considered comparable with international benchmarks. Based on these results, we propose the creation of a 'centralized' pool of surgeons in Australia to help continue providing 'decentralized' care of BA.

Scottish outcomes for extra hepatic biliary atresia post-rationalisation of services.

AIMS: To evaluate the outcome of Scottish children with extra hepatic biliary atresia (EHBA) since rationalisation of Kasai services to three English centres in 2002 (The 'Group A' centres). METHODS: All Scottish children with EHBA diagnosed between 2002 and 2009 were identified via the Scottish Society of Paediatric Gastroenterology, Hepatology and Nutrition (SSPGHAN) clinicians. A case-note review was conducted with demographics, presentation and outcome data recorded. These data were compared with historical Scottish data and data published previously by the supraregional liver units. RESULTS: 25 patients were identified, of whom 22 were referred for Kasai in the group A centres, and of whom 19 had a Kasai. 2 year transplant-free survival (TFS) was significantly lower in the SSPGHAN 2002-2009 group than the group A centres in (1) (6/18 (33%) vs 36/57 (63%), p=0.023). CONCLUSIONS: These postrationalisation data are disappointing. The emphasis for care will now focus on improved communication between, primary care, general paediatricians and surgical centres through regional and national managed clinical networks, aiming to improve future outcomes for Scottish children with BA.

Biliary atresia in England and Wales: results of centralization and new benchmark.

INTRODUCTION: Biliary atresia (BA) is a rare, potentially life-threatening condition of the newborn presenting with conjugated jaundice. Typically, it is treated by an initial attempt to restore bile flow (the Kasai portoenterostomy [KP]) as soon as possible after diagnosis and, if this fails, liver transplantation. Since 1999, the treatment of BA has been centralized to 3 centers in England and Wales able to offer both treatment options. The aim of this study was to review the outcome of this policy change and provide a national benchmark. METHODS: The management of all infants born within England and Wales during the period January 1999 to December 2009 was assessed using 3 key performance indicators such as median time to KP, percentage clearance of jaundice (≤20 mol/L) post-KP, and 5- and 10-year native liver and true survival estimates. Data are quoted as median (range), and P < .05 was considered significant. RESULTS: A total of 443 infants had confirmed BA; and of these, most were isolated BA (n = 359), with 84 having other significant anomalies (but predominantly BA splenic malformation syndrome). Four infants died before any biliary intervention. Kasai portoenterostomy was performed in 424 infants (median age, 54 [range 7-209] days), and a primary liver transplant was performed in 15. Clearance of jaundice post-KP was achieved in 232 (55%). There were 41 deaths, including 4 (10%) without any intervention, 24 (58%) post-KP usually because of end-stage liver disease and mostly on a transplant waiting list, and 13 (32%) post-LT usually because of multiorgan failure. Overall, the 5- and 10-year native liver survival estimates were 46% (95% confidence interval [CI], 41-51) and 40% (95% CI, 34-46), respectively. The 5- and 10-year true patient survival estimates were 90% (95% CI, 88-93) and 89% (95% CI, 86-93), respectively. Outcome was worse for those with other anomalies (lower clearance of jaundice post-KP [43% vs 57%; odds ratio, 1.7; 95% CI, 1.04-2.8]; P = .02) and an increased mortality overall (eg, at 5 years, 72 [95% CI, 64-83] vs 94 [95% CI, 91-96]; χ(2) = 33; P < .0001). CONCLUSIONS: National outcome measures in BA appear better than those from previously published series from comparable countries and may be attributed to centralization of surgical and medical resources.

Post-portoenterostomy triangular cord sign prognostic value in biliary atresia: a prospective study.

The triangular cord sign (TC sign) is a sensitive and specific tool in prompt diagnosis of extrahepatic biliary atresia. The objective of this study is to evaluate post-operative TC sign presence in outcome prediction of infants with biliary atresia after Kasai hepato-portoenterostomy 27 infants and children with biliary atresia underwent 122 ultrasound examinations using both 5 MHz and 7 MHz convex linear transducers in 33 months follow up. For all infants TC sign identification was included pre-operatively, ultrasound was done 2 weeks post-operatively then bimonthly for 3 months, monthly for 2 months and every 3 months thereafter. 14 (53.8%) had post-operative TC sign. Once post-operatively positive, it remained positive throughout the study. It did not reappear in an initially post-operatively TC sign negative infant. Those having post-operative TC sign had statistically worse outcomes (0 became anicteric, 2 improved, 7 had progressive disease and 6 died) than those with a negative TC sign (p = 0.04) (3 became anicteric, 5 improved, 2 progressed and 1 died). Presence of TC sign post-operatively correlated with measure of removal of all fibrous cone at porta-hepatis during portoenterostomy (p = 0.026). Post-portoenterostomy TC sign is associated with more morbidity and mortality; and reflects inadequate surgical technique.