Pediatric Surgery and Transplantation Unit.
Anaesthesiology.
Liver Transpl. 2017 Nov;23(11):1440-1450. doi: 10.1002/lt.24850.
Cirrhosis in adults is associated with modifications of systemic and liver hemodynamics, whereas little is known about the pediatric population. The aim of this work was to investigate whether alterations of hepatic and systemic hemodynamics were correlated with cirrhosis severity in children. The impact of hemodynamic findings on surgical management in pediatric living donor liver transplantation (LT) was evaluated. Liver and systemic hemodynamics were studied prospectively in 52 children (median age, 1 year; 33 with biliary atresia [BA]). The hemodynamics of native liver were studied preoperatively by Doppler ultrasound and intraoperatively using invasive flowmetry. Portosystemic gradient was invasively measured. Systemic hemodynamics were studied preoperatively by Doppler transthoracic echocardiography and intraoperatively by using transpulmonary thermodilution. Hemodynamic parameters were correlated with Pediatric End-Stage Liver Disease (PELD) score and the histological degree of fibrosis (collagen proportionate area [CPA]). Cirrhosis was associated with a 60% reduction of pretransplant total liver flow (n = 46; median, 36 mL/minute/100 g of liver) compared with noncirrhotic livers (n = 6; median, 86 mL/minute/100 g; P = 0.002). Total blood flow into the native liver was negatively correlated with PELD (P < 0.001) and liver CPA (P = 0.005). Median portosystemic gradient was 14.5 mm Hg in children with cirrhosis and positively correlated with PELD (P < 0.001). Portal vein (PV) hypoplasia was observed mainly in children with BA (P = 0.02). Systemic hemodynamics were not altered in our children with cirrhosis. Twenty-one children met the intraoperative criteria for PV reconstruction using a portoplasty technique during the LT procedure and had a smaller PV diameter at pretransplant Doppler ultrasound (median = 3.4 mm; P < 0.001). Cirrhosis in children appears also as a hemodynamic disease of the liver, correlated with cirrhosis severity. Surgical technique for PV reconstruction during LT was adapted accordingly. Liver Transplantation 23 1440-1450 2017 AASLD.
成人肝硬化与全身和肝脏血流动力学的改变有关，而小儿患者的相关信息则知之甚少。本研究旨在探讨儿童肝硬化患者肝脏和全身血流动力学的改变是否与肝硬化严重程度相关，并评估血流动力学改变对小儿活体肝移植（LT）手术管理的影响。
我们前瞻性地研究了 52 名儿童（中位年龄 1 岁；33 名胆道闭锁患儿）的肝脏和全身血流动力学。术前通过多普勒超声检查和术中使用有创流量测量仪评估供肝血流动力学。经皮门静脉穿刺测量门腔静脉梯度。术前通过经胸多普勒超声心动图检查和术中通过经肺热稀释法检查评估全身血流动力学。血流动力学参数与小儿终末期肝病评分（PELD）和组织学纤维化程度（胶原比例面积[CPA]）相关。
与非肝硬化肝脏（n=6；中位 86 毫升/分钟/100 克）相比，移植前 46 例肝硬化患儿的总肝血流减少了 60%（中位数 36 毫升/分钟/100 克；P=0.002）。流入供肝的总血流量与 PELD（P<0.001）和肝 CPA（P=0.005）呈负相关。中位门腔静脉梯度为 14.5mmHg，与 PELD 呈正相关（P<0.001）。门静脉（PV）发育不良主要见于胆道闭锁患儿（P=0.02）。
在肝硬化患儿中，我们并未观察到全身血流动力学改变。21 名患儿在 LT 术中符合门静脉成形术重建的标准，且术前多普勒超声检查显示门静脉直径较小（中位数 3.4mm；P<0.001）。
儿童肝硬化也是一种肝脏的血流动力学疾病，与肝硬化严重程度相关。因此，我们相应地调整了 LT 术中 PV 重建的手术技术。
