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儿童肝移植中肝静脉流出道梗阻的诊断、治疗及预后:单中心24年经验

Diagnosis, treatment and outcome of hepatic venous outflow obstruction in paediatric liver transplantation: 24-year experience at a single centre.

作者信息

Galloux Alexis, Pace Erika, Franchi-Abella Stephanie, Branchereau Sophie, Gonzales Emmanuel, Pariente Daniele

机构信息

AP-HP, Bicêtre Hospital, Pediatric Radiology Department, 78 rue du Gal Leclerc, 94270, Le Kremlin Bicêtre, France.

Faculty of Medicine, Paris Sud University, Le Kremlin Bicêtre, France.

出版信息

Pediatr Radiol. 2018 May;48(5):667-679. doi: 10.1007/s00247-018-4079-y. Epub 2018 Feb 21.

Abstract

BACKGROUND

Hepatic venous outflow obstruction after paediatric liver transplantation is an unusual but critical complication.

OBJECTIVES

To review the incidence, diagnosis and therapeutic modalities of hepatic venous outflow obstruction from a large national liver transplant unit.

MATERIALS AND METHODS

During the period from October 1992 to March 2016, 917 liver transplant procedures were performed with all types of grafts in 792 children. Transplants suspected to have early or delayed venous outflow obstruction were confirmed by percutaneous venography or surgical revision findings. Therapeutic intervention, recurrence and outcome were evaluated.

RESULTS

Twenty-six of 792 children (3.3%) experienced post-transplant hepatic venous outflow obstruction. These patients had been diagnosed from 1 day to 8.75 years after transplantation. Six occurred during the early post-transplant period; in three of them, the graft was lost. Seventeen patients were initially treated by balloon angioplasty with success; 11 of these experienced recurrences. Four stents were implanted; one was complicated by definitive occlusion. Three of the five surgical revisions were successful. The initial stenosis involved the inferior vena cava in 10 grafts, in isolation or associated with hepatic vein involvement. Mean follow-up was 79 months after transplantation. Eight grafts were lost.

CONCLUSION

Acute postoperative hepatic venous outflow obstruction was associated with poor prognosis. Diagnostic venography should be performed if there is any suspicion of venous outflow obstruction, even if first-line examinations are normal. Stenosis frequently involved the inferior vena cava. Angioplasty was a safe and efficient treatment for venous outflow obstruction despite frequent recurrence.

摘要

背景

小儿肝移植术后肝静脉流出道梗阻是一种罕见但严重的并发症。

目的

回顾一家大型国家肝移植中心肝静脉流出道梗阻的发生率、诊断及治疗方式。

材料与方法

1992年10月至2016年3月期间,对792名儿童实施了917例各类移植物的肝移植手术。疑似早期或延迟性静脉流出道梗阻的移植手术通过经皮静脉造影或手术探查结果得以确诊。对治疗干预、复发情况及预后进行了评估。

结果

792名儿童中有26名(3.3%)发生了移植后肝静脉流出道梗阻。这些患者在移植后1天至8.75年被确诊。6例发生在移植后早期,其中3例移植物丢失。17例患者最初接受球囊血管成形术治疗并取得成功,其中11例复发。植入了4枚支架,1枚出现最终闭塞并发症。5例手术探查中有3例成功。最初的狭窄累及10例移植物的下腔静脉,可单独出现或合并肝静脉受累。移植后平均随访79个月。8例移植物丢失。

结论

术后急性肝静脉流出道梗阻预后不良。即使一线检查结果正常,只要怀疑有静脉流出道梗阻,就应进行诊断性静脉造影。狭窄常累及下腔静脉。血管成形术是治疗静脉流出道梗阻的一种安全有效的方法,尽管复发频繁。

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