Portal vein aneurysm after liver transplantation: report of two cases and literature review
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摘要:
目的 总结肝移植术后门静脉瘤的诊疗经验。 方法 回顾性分析2例肝移植术后门静脉瘤患者的临床资料，结合文献复习总结其临床特点、诊断、治疗及预后。 结果 两例肝移植术后门静脉瘤均为肝内型，同时合并门静脉血栓、门静脉高压表现。例1患者给予针对性内科保守治疗，患者拒绝行再次肝移植手术，出院后病情加重，最终因移植肝衰竭、肾衰竭、肺部感染、感染性休克死亡。例2患者给予大剂量糖皮质激素冲击治疗，患者肝功能无改善，接受二次肝移植后恢复顺利。 结论 肝移植术后远期合并门静脉瘤（尤其是肝内型）可能预示预后不良，需正确认识、密切随访、积极处理，适时考虑再次肝移植是可供选择的治疗方案。 Abstract:Objective To summarize the diagnosis and treatment experience of portal vein aneurysm after liver transplantation. Methods Clinical data of two recipients with portal vein aneurysm after liver transplantation were retrospectively analyzed. Clinical features, diagnosis, treatment and prognosis were summarized based on literature review. Results Both two cases were diagnosed with intrahepatic portal vein aneurysm complicated with portal vein thrombosis and portal hypertension after liver transplantation. Case 1 was given with targeted conservative treatment and he refused to undergo liver retransplantation. Physical condition was worsened after discharge, and the patient eventually died from liver graft failure, kidney failure, lung infection, and septic shock. Case 2 received high-dose glucocorticoid pulse therapy, whereas liver function was not improved, and the patient was recovered successfully after secondary liver transplantation. Conclusions Long-term complication of portal vein aneurysm (especially intrahepatic type) after liver transplantation probably indicates poor prognosis. Correct understanding, intimate follow-up and active treatment should be conducted. Liver retransplantation may be a potential treatment regimen. -
图 1 例1患者肝移植术后PVA的影像学表现
注：A图为CT平扫水平位显示门静脉左右支瘤样扩张，最大直径为40.7 mm，并可见TIPS支架影（箭头所示）；B图为CT平扫冠状位显示肝内门静脉左右支瘤样扩张，并可见TIPS支架影（箭头所示）；C图为超声多普勒显示肝内形状不规则的无回声病变与门静脉系统相连。瘤内可见血流充盈缺损，另可见强回声影，为TIPS支架（箭头所示）；D图为超声多普勒显示该病变内部为源自静脉系统的湍流血流信号，诊断为肝内门静脉左右支静脉瘤。瘤内血流充盈缺损未见血流信号，诊断为门静脉血栓。
Figure 1. Imaging findings of PVA after liver transplantation in case 1
图 2 例2患者肝移植术后PVA的影像学表现
注：A图为CT平扫水平位显示门静脉左右支瘤样扩张，最大直径34 mm；B图为CT平扫冠状位显示肝内门静脉左右支瘤样扩张，并可见门静脉支架影（黑色箭头所示）；C图为超声多普勒显示该肝内形状不规则的无回声病变与门静脉系统相连，其内可见源自静脉系统湍流血流信号，诊断为肝内门静脉左右支静脉瘤；D图为二次肝移植术中见门静脉左右支呈瘤样扩张，肝门区门静脉吻合口狭窄，肝脏淤血肿胀。
Figure 2. Imaging findings of PVA after liver transplantation in case 2
表 1 文献报道的5例肝移植术后PVA患者临床资料
Table 1. Clinical data of 5 patients with PVA after liver transplantation reported in literature
研究者 年份 受者年龄 原发病 手术方式 门静脉吻合
特点发现PVA时间
及方式报道时PVA
直径PVA
位置疾病转归 Ferraz-Neto BH, et al[3] 2004年 59岁 丙型病毒性肝炎肝硬化 经典原位
肝移植供受者不匹配，门静脉整形式吻合 术后3年常规超声复查发现 5.7 cm×
5.7 cm×
5.2 cm肝内 术后6年，疾病进展，患者因移植肝衰竭死亡 Francesco FD, et al[4] 2010年 60岁 丙型病毒性肝炎肝硬化 经典原位
肝移植供受者端端吻合 术后10年因肠梗阻、结肠肿瘤入院时CT检查发现 最大直径
6.3 cm肝外 随访至术后17年，无PVA相关症状及并发症 Quan JR,
et al[5]2012年 46岁 乙型病毒性肝炎肝硬化 经典原位
肝移植供受者端端吻合 术后7年常规超声复查发现 －① 肝外 随访至术后10年，无PVA相关症状及并发症 Molinares B,
et al[6]2013年 6岁 急性甲型病毒性肝炎 减体积
肝移植供受者端端吻合 术后2年常规超声复查时发现 14 mm×
18 mm肝外 随访至术后4年无PVA相关症状及并发症 Nassafi EM,
et al[7]2022年 25岁 糖原贮积病Ⅳ型 背驮式
肝移植－ 术后4年超声复查发现 最大直径
35 mm肝内 术后19年，发现胆肠吻合口狭窄并肝内胆管扩张，可能与PVA相关。经胆道扩张治疗后，病情好转 注：①－为文献未提及。 
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