Liver Transplantation for Children With Budd-Chiari Syndrome: A Case Report From Vietnam and Literature Review.

IF 1.4 4区医学 Q3 PEDIATRICS

Pediatric Transplantation Pub Date : 2025-03-01 DOI:10.1111/petr.70038

Thanh Tri Tran, Thi Yen Nhi Truong, Hong Van Khanh Nguyen, Nguyen An Thuan Luu, Trong Thien Than, Hai Trung Bui, Phi Duy Ho, Thierry Pirotte, Raymond Reding

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Abstract

Budd-Chiari syndrome (BCS) presents with various degrees of liver damage, and the choice of treatment depends on the type and extent of hepatic injury. Liver transplantation (LT) is considered as the final treatment option when other interventions are not feasible and when the liver injury is irreversible. We report a case of a pediatric patient with BCS who underwent liver transplantation from a living donor in the context of thrombophilic disorder.

Case presentation: A 14-month-old girl was admitted to the hospital with ascites. She was malnourished, and an abdominal CT scan confirmed significant ascites with no visualization of the hepatic veins and retrohepatic inferior vena cava (IVC). A liver biopsy revealed fibrosis, necrosis, and parenchymal hemorrhage. Patient's portal hypertension was managed with prophylactic beta-blocker Propranolol and endoscopic esophageal variceal ligation. However, she was hospitalized four times due to gastrointestinal bleeding from ruptured esophageal varices. Protein C deficiency was found as probable etiology of BCS. The patient underwent liver transplantation at 3 years and 8 months old with a liver from a parental living donor. The surgery and postoperative course were uneventful, and the patient was discharged 25 days after the transplant.

Clinical discussion: Hypercoagulability is often the underlying cause of BCS. Maintaining anticoagulation/thrombophilic balance postoperatively contributed to the successful liver transplantation in this pediatric patient.

Conclusion: Liver transplantation is a safe and effective treatment for pediatric patients with BCS who meet the criteria for the procedure.

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肝移植治疗布-恰利综合征儿童：越南一例报告及文献复习。

Budd-Chiari综合征（BCS）表现为不同程度的肝损伤，治疗的选择取决于肝损伤的类型和程度。当其他干预措施不可行且肝损伤不可逆转时，肝移植（LT）被认为是最后的治疗选择。我们报告一例小儿BCS患者在嗜血栓性疾病的背景下接受活体供体肝移植。病例介绍：一名14个月大的女婴因腹水入院。她营养不良，腹部CT扫描证实了明显的腹水，没有肝静脉和肝后下腔静脉（IVC）的可见。肝活检显示纤维化、坏死和实质出血。患者的门脉高压症是通过预防性受体阻滞剂普萘洛尔和内镜食管静脉曲张结扎。然而，她因食道静脉曲张破裂导致胃肠出血而住院四次。蛋白C缺乏可能是BCS的病因。患者在3岁零8个月大时接受了肝脏移植手术，肝脏来自父母的活体供体。手术和术后过程顺利，患者在移植后25天出院。临床讨论：高凝往往是BCS的根本原因。术后维持抗凝/亲血栓平衡有助于该患儿肝移植的成功。结论：肝移植是一种安全有效的治疗小儿BCS患者符合手术标准。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Pediatric Transplantation 医学-小儿科

CiteScore

2.90

自引率

15.40%

发文量

216

审稿时长

3-8 weeks

期刊介绍： The aim of Pediatric Transplantation is to publish original articles of the highest quality on clinical experience and basic research in transplantation of tissues and solid organs in infants, children and adolescents. The journal seeks to disseminate the latest information widely to all individuals involved in kidney, liver, heart, lung, intestine and stem cell (bone-marrow) transplantation. In addition, the journal publishes focused reviews on topics relevant to pediatric transplantation as well as timely editorial comment on controversial issues.