Strategies to improve the outcomes of patients with biliary atresia

Abstract

The objective of this thesis is to derive strategies that can improve the outcomes of patients with biliary atresia (BA) based on the clinical review of 185 BA patient treated in Queen Mary Hospital since 1980. BA is one of the most common surgical diagnoses for patients with prolonged jaundice during the neonatal and early infantile period. Despite an uneventful Kasai operation, some patients continue to develop progressive liver failure or complications of the disease. The success rate of the Kasai operation has been reported to be related to several factors. The finding in this thesis, however, did not support the traditional belief that an early surgery is essential for the best outcomes. Operation performed between day 61 to 80 was associated with the best 1-year native liver survival (82.7%). In addition, the outcomes of laparoscopic Kasai operation have been shown to be inferior to that of open operation. Adjuvant steroid treatment is generally believed to promote the patency of the transected microscopic ducts due to its anti-inflammatory and choleretic effect. Moderate to high dose steroid (4mg/kg/day) has been found to improve post-Kasai biliary drainage rate (73.2%) as well as 1-year native liver survival (78.0%) based on our local experience. The occurrence of repeated cholangitis was considered as a prognostic marker for adverse short and long term outcomes ( RR: 3.16 for short term failure ,p =0.001 and RR: 2.07 for long term failure, p = 0.004). Therefore, an aggressive approach with potent and adequate duration of antibiotics should be considered to prevent cholangitis. Based on these evidences a standardized treatment protocol has been introduced into clinical practice with promising early results. An improved post-Kasai biliary drainage (60.0% vs 82.1%, p = 0.07) as well as short-term native liver survival (69.0% vs 85.7%, p = 0.05) were found in patients treated with this protocol. When disease progression occurs despite an uneventful surgery and the proper use of adjuvant medications, liver transplant becomes the only salvage treatment option. Some surgeons hypothesize that the outcomes of transplant surgery are worse in patients with BA than in patients with other diagnoses due to previous Kasai operation. However, the evidence in this thesis did not support this hypothesis. The recipient survival of BA recipients was comparable to that of non-BA recipients (86.4% vs 84.2%, p = 0.9). Liver transplant referral is occasionally delayed due to the misconception that small-sized recipients have a poor outcome. The study in this thesis has provided the evidence that the recipient and graft survivals were also satisfactory in recipients with the smallest body size. Referral to liver transplant should not be withheld when it is required by the patient to reduce morbidity. In conclusion, this thesis has highlighted important issues that would optimize the management of BA based different clinical and laboratory studies. The outcomes of patients with BA can be improved by implementing proper strategies before, during and after the Kasai operation.