Section of Pediatric Surgery, Pediatric Liver and Gut Research Group and Pediatric Research Center, Children's Hospital, Helsinki University Hospital.
HUS Medical Imaging Center, Children's Hospital, Helsinki University, Helsinki, Finland.
J Pediatr Gastroenterol Nutr. 2019 Apr;68(4):488-494. doi: 10.1097/MPG.0000000000002243.
Cholangitis and bile lakes are incompletely understood complications after portoenterostomy (PE). We investigated relationships between recurrent cholangitis, bile lakes, and clinical outcomes as well as surgical management of bile lakes.
In this retrospective observational single institution study medical records and imaging studies of all patients who had undergone PE for biliary atresia during 1987 to 2016 (N = 61) were reviewed. We related occurrence of cholangitis episodes with the presence of intrahepatic bile lakes, patient characteristics, and PE outcomes. Risk factors for recurrent cholangitis and bile lakes, and management of bile lakes were analyzed.
Despite routine antibiotic prophylaxis median of 3.0 cholangitis episodes (0.75 episodes/year) occurred in 48 (79%) patients. Intrahepatic bile lakes were discovered in 8 (13%) patients by 16 months after PE. Overall, 54% had survived with their native liver at median age of 7.3 years and 28 (46%) patients had ≥1 cholangitis episodes/year. Number and frequency of cholangitis episodes were >5 times higher among patients with bile lakes (P < 0.001). Six patients underwent Roux-en-Y bile lake-jejunostomy, resulting in regression/disappearance of bile lakes and normalization of serum bilirubin in 5 with reduction of median yearly cholangitis rate from 8.8 to 1.1 (P = 0.028) and native liver survival of 6.3 (range, 1.3-17) years after the operation.
Bile lakes are a significant risk factor for recurrent cholangitis after PE and efficiently treated by operative intestinal drainage providing prolonged jaundice-free native liver survival. Bile lakes should be actively screened among patients presenting with recurrent cholangitis after PE.
胆系炎症和胆湖是门腔分流术后不完全了解的并发症。我们研究了复发性胆系炎症、胆湖与临床结果之间的关系，以及胆湖的手术处理。
在这项回顾性观察性单中心研究中，我们回顾了 1987 年至 2016 年间接受门腔分流术治疗胆道闭锁的所有患者（n=61）的病历和影像学检查。我们将胆管炎发作的发生与肝内胆汁湖的存在、患者特征和门腔分流术结果联系起来。分析了复发性胆管炎和胆湖的危险因素，以及胆湖的处理方法。
尽管常规使用抗生素预防，仍有 48 例（79%）患者在中位 3.0 年后发生 3.0 次胆管炎发作（0.75 次/年）。8 例（13%）患者在门腔分流术后 16 个月发现肝内胆汁湖。总的来说，54%的患者在中位年龄为 7.3 岁时仍保留其自身肝脏，28 例（46%）患者每年发生≥1 次胆管炎发作。有胆湖的患者胆管炎发作次数和频率要高出 5 倍以上（P<0.001）。6 例患者接受 Roux-en-Y 胆湖空肠吻合术，5 例患者的胆湖消退/消失，血清胆红素正常化，中位每年胆管炎发生率从 8.8 降至 1.1（P=0.028），术后自身肝脏存活率为 6.3（范围，1.3-17）年。
胆湖是门腔分流术后复发性胆管炎的一个重要危险因素，通过手术肠道引流可有效治疗，从而延长无黄疸的自身肝脏存活率。在门腔分流术后出现复发性胆管炎的患者中，应积极筛查胆湖。
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