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中华肝脏外科手术学电子杂志 ›› 2019, Vol. 08 ›› Issue (06) : 512 -516. doi: 10.3877/cma.j.issn.2095-3232.2019.06.011

所属专题: 文献

临床研究

心脏死亡供体儿童肝移植术后可逆性后部脑病综合征治疗经验并文献复习
邓斐文1, 蒋苏华2, 胡健垣1, 甄作均1, 陈焕伟1,()   
  1. 1. 528000 广东省佛山市第一人民医院肝脏胰腺外科
    2. 528000 广东省佛山市第一人民医院儿科重症监护室
  • 收稿日期:2019-08-18 出版日期:2019-12-10
  • 通信作者: 陈焕伟
  • 基金资助:
    佛山市2018年医学类科技攻关项目(2018AB003501)

Posterior reversible encephalopathy syndrome in child patient after liver transplantation from donation after cardiac death: report of one case and literature review

Feiwen Deng1, Suhua Jiang2, Jianyuan Hu1, Zuojun Zhen1, Huanwei Chen1,()   

  1. 1. Department of Hepatopancreatic Surgery, the First People's Hospital of Foshan, Foshan 528000, China
    2. Pediatric Intensive Care Unit, the First People's Hospital of Foshan, Foshan 528000, China
  • Received:2019-08-18 Published:2019-12-10
  • Corresponding author: Huanwei Chen
  • About author:
    Corresponding author: Chen Huanwei, Email:
引用本文:

邓斐文, 蒋苏华, 胡健垣, 甄作均, 陈焕伟. 心脏死亡供体儿童肝移植术后可逆性后部脑病综合征治疗经验并文献复习[J/OL]. 中华肝脏外科手术学电子杂志, 2019, 08(06): 512-516.

Feiwen Deng, Suhua Jiang, Jianyuan Hu, Zuojun Zhen, Huanwei Chen. Posterior reversible encephalopathy syndrome in child patient after liver transplantation from donation after cardiac death: report of one case and literature review[J/OL]. Chinese Journal of Hepatic Surgery(Electronic Edition), 2019, 08(06): 512-516.

目的

探讨心脏死亡供体(DCD)儿童肝移植术后可逆性后部脑病综合征(PRES)的诊治方法。

方法

回顾性分析2018年7月5日佛山市第一人民医院接受肝移植的1例儿童患者临床资料。受者男,4.5岁,先天性胆道闭锁术后合并肝硬化失代偿期;DCD供者男,6岁。患儿家属签署知情同意书,符合医学伦理学规定。患儿于2018年7月5日接受同种异体原位肝移植术,术后采用他克莫司联合激素免疫抑制方案。观察患儿术后脑部并发症诊治情况。

结果

术后第3天患儿抽搐1次,术后1周出现烦躁不安,右侧肢体偏瘫。头颅CT、MRI和CT血管造影(CTA)示急性左侧脑梗塞,右侧脑出血,脑血管炎性病变,考虑PRES。更换他克莫司为西罗莫司,并给予纠正凝血功能、高血压、脱水等综合治疗,脑部并发症逐渐好转,术后42 d出院。出院后门诊高压氧治疗,言语功能及认知能力逐渐康复,右侧肢体偏瘫情况逐渐改善,截止至投稿时右上下肢肌力恢复为Ⅱ级。

结论

儿童肝移植受者术后PRES发病突然、病情危重、病因复杂,及时调整免疫抑制治疗方案和对症处理是治疗PRES的关键。

Objective

To investigate the diagnosis and treatment of posterior reversible encephalopathy syndrome (PRES) in a child patient after liver transplantation from donation after cardiac death (DCD).

Methods

Clinical data of a child undergoing liver transplantation in the First People's Hospital of Foshan on July 5, 2018 were retrospectively analyzed. The 4.5-year-old male recipient was complicated with liver cirrhosis in decompensation after surgery of congenital biliary atresia. The DCD donor was a boy aged 6 years old. The informed consent of the patient were obtained and the local ethical committee approval was received. The child underwent orthotopic liver transplantation on July 5, 2018. Tacrolimus combined with hormone immunosuppression was delivered postoperatively. The diagnosis and treatment of postoperative cerebral complications were observed.

Results

At postoperative 3 d, the child developed convulsion. At postoperative 1 week, the child presented with dysphoria and right hemiplegia. CT, MRI and CT angiography (CTA) of demonstrated acute left cerebral infarction, right cerebral hemorrhage and cerebral vasculitis. Diagnosis of PRES was considered. Tacrolimus was replaced with sirolimus. Comprehensive treatments was delivered to correct coagulation function, hypertension, dehydration, etc. The syndrome of cerebral complications were gradually improved and the patient was discharged at 42 d after operation. After discharge, the child received hyperbaric oxygen therapy. The speech function and cognitive capability were steadily restored and the right limb hemiplegia was gradually mitigated. Until the date of paper submission, the muscle strength of the right upper and lower limbs was restored to Grade Ⅱ.

Conclusions

Postoperative PRES occurs all of a sudden with severe condition and complex pathogenesis in the pediatric recipient after liver transplantation. Timely adjustment of immunosuppressive therapy and symptomatic treatments play a key role in the treatment of PRES.

图1 一例儿童肝移植受者术后头颅影像学检查
图2 一例儿童肝移植受者术后头颅影像学检查
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