儿童肝移植术后门静脉狭窄的诊断与治疗

Diagnosis and treatment of portal vein stenosis after pediatric liver transplantation

目的分析儿童肝移植术后门静脉狭窄(PVS)的可能危险因素,并探讨不同治疗方式的临床疗效。方法回顾性分析2013年6月至2017年12月首都医科大学附属北京友谊医院肝移植中心396例儿童肝移植受者临床资料(年龄≤14周岁)。随访至2018年6月,有26例发生PVS(6.6%)。对于超声怀疑PVS的儿童受者,本中心多选用门静脉血管造影确诊。术后采用超声监测门静脉直径及流速,观察血管通畅情况。采用随访观察并口服药物抗凝治疗、球囊扩张、门静脉支架置入或Meso-Rex分流术治疗PVS。监测肝功能变化,评估有无门静脉相关的移植物损伤,并观察有无门静脉高压相关的症状或体征。结果26例儿童受者术后发生PVS中位时间为9.5个月(1.3~50.0个月),其中3个月以内发生者占26.9%(7/26),3个月以后占73.1%(19/26)。行介入球囊扩张和支架置入或Meso-Rex分流术共47例次,均未因PVS死亡。2例儿童受者动态随访,期间口服抗凝药物;23例行门静脉球囊扩张术,1例因门静脉冗长行1次门静脉球囊扩张+支架置入术,10例经1次门静脉球囊扩张术后无效后行二次球囊扩张,7例经二次门静脉球囊扩张术后无效行门静脉支架置入术,2例经门静脉支架置入术后再次狭窄,行Meso-Rex手术。1例口服药物抗凝治疗的儿童受者,随访期间超声提示门静脉流速偏快,其余随访至今未见PVS复发。结论超声是监测儿童肝移植术后门静脉情况、早期发现PVS的有效办法。发生PVS时,轻症儿童受者可动态随访,期间口服抗凝药物;中重度儿童受者首选门静脉球囊扩张、门静脉支架置入术。Meso-Rex分流术是对门脉支架置入术后PVS复发或发生门静脉闭塞的一种可选择的手术方式。

Objective To analyze the possible risk factors which were related to portal vein stenosis (PVS) after pediatric liver transplantation, and to explore the clinical efficacy of different therapeutic method. Methods The clinical data of 396 pediatric liver transplantation recipients ( age ≤14 years old) who got transplantation at the liver transplantation center of Beijing friendship hospital from June 2013 to December 2017 were retrospectively analyzed. All the recipients were followed up until June 2018, during which 26 recipients (6.6%) were diagnosed with PVS. For pediatric recipients who were suspected of PVS by ultrasound, portal vein angiography was used to make a definite diagnosis. Doppler ultrasound was used to monitor the caliber and velocity of the portal vein after transplantation. PVS was treated with follow- up and oral anticoagulant therapy,balloon angioplasty, portal vein stent placement, or Meso- Rex bypass surgery. Change of liver function was monitored. Portal- related graft injury was assessed. Symptoms or signs associated with portal hypertension was observed. Results The median time of diagnosis of postoperative PVS in 26 pediatric recipients was 9.5 months (1.3~50.0 months), among which 26.9%(7/26) occurred within 3 months , and 73.1%(19/26) occurred after 3 months. Forty- seven person- time including medical intervention, balloon dilation, stent placement or Meso- Rex surgery were carried out, and no recipient died from PVS. Two recipients were followed up dynamically, during which oral anticoagulant drugs were taken. Twenty- three recipients got portal vein balloon dilatation, 1 recipient got a portal balloon dilation and stent placement because of portal vein lengthy, 10 recipients accepted second portal vein balloon angioplasty due to the failure of the balloon angioplasty, 7 recipients accepted portal vein stent placement due to the failure of the second balloon angioplasty, 2 recipients occured with portal vein stenosis again after portal vein stent placement, and then they received Meso- Rex surgery. For 1 recipient who received oral anticoagulant therapy during follow- up, the result of ultrasound examination suggested that the portal vein velocity was too fast, and no PVS recurrence was found in the remaining patients. Conclusions Doppler ultrasound is an effective method to monitor the portal vein and detect PVS early in pediatric liver transplantation .In the case of PVS, children with mild clinical symptoms could be followed up dynamically, during which oral anticoagulant drugs should be taken. Portal vein balloon dilatation and portal vein stent implacement are the first choice for moderate and severe recipients. Meso- Rex bypass surgery is an optional procedure for recurrence after portal stent implacement or portal vein occlusion.