背景:在胆道闭锁(BA)患者中,严重的门静脉高压症(HTN)发展,即使成功的胆汁流恢复,提示驱动门静脉HTN的内在因素独立于胆汁阻塞。我们假设BA患者门静脉(PV)发育异常,导致PV发育不全。
方法:在这项观察性队列研究中,我们纳入了2017年至2021年转诊至三级中心的患者,以排除BA.术中胆道造影前将计算机断层扫描(CT)血管造影作为临床常规的新生儿,和腹腔镜开赛肝肠造口术。将PV和肝动脉(HA)的直径与楔形活检中的肝纤维化程度进行比较。黄疸清除,天然肝脏存活率,和临床门静脉高压事件,包括腹水发育和肠出血,被评估。
结果:47例胆汁淤积的新生儿被纳入队列;35例被诊断为BA。BA患者的中位PV直径较小(4.3vs.5.1mm;p<0.001)和更大的中位HA直径(1.4与1.2毫米;p<0.05)与其他形式的胆汁淤积患者相比。中位PV和HA直径与肝纤维化程度无关。在35例BA患者中,29例患者(82.9%)实现黄疸清除,23例患者(65.7%)在2岁时保持天然肝脏存活。7例患者(20%)出现肠出血,7名患者(20%)出现腹水,有一个重叠的病人。
结论:BA患者在诊断时存在PV发育不全,与肝纤维化无关。
BACKGROUND: In patients with biliary atresia (BA), severe portal hypertension (HTN) develops even with successful bile flow restoration, suggesting an intrinsic factor driving portal HTN independent from bile obstruction. We hypothesize that patients with BA have abnormal portal vein (PV) development, leading to PV hypoplasia.
METHODS: In this observational cohort study, we enrolled patients who were referred to a tertiary center from 2017 to 2021 to rule out BA. Newborns who underwent computed tomography (CT) angiogram as a clinical routine before intraoperative cholangiogram, and laparoscopic Kasai hepatoportoenterostomy. The diameter of the PV and hepatic artery (HA) were compared to the degree of liver fibrosis in the wedge biopsies. The jaundice clearance, native liver survival, and clinical portal hypertensive events, including ascites development and intestinal bleeding, were assessed.
RESULTS: 47 newborns with cholestasis were included in the cohort; 35 were diagnosed with BA. The patients with BA had a smaller median PV diameter (4.3 vs. 5.1 mm; p < 0.001) and larger median HA diameter (1.4 vs. 1.2 mm; p < 0.05) compared to the patients with other forms of cholestasis. The median PV and HA diameter did not correlate with the degree of liver fibrosis. Among 35 patients with BA, 29 patients (82.9%) achieved jaundice clearance, and 23 patients (65.7%) were alive with their native liver at two years of age. Seven patients (20%) developed intestinal bleeding, and seven patients (20%) developed ascites, with one overlapping patient.
CONCLUSIONS: PV hypoplasia is present in patients with BA independent of liver fibrosis at the time of diagnosis.