Portal cavernoma cholangiopathy treated with living donor liver transplantation: a case report and review of the literature.

IF 0.8 Q4 GASTROENTEROLOGY & HEPATOLOGY

Clinical Journal of Gastroenterology Pub Date : 2024-12-01 Epub Date: 2024-09-23 DOI:10.1007/s12328-024-02041-7

Takahiko Omameuda, Yukihiro Sanada, Yasunaru Sakuma, Yasuharu Onishi, Taiichi Wakiya, Yuta Hirata, Toshio Horiuchi, Kiichiro Takadera, Ryosuke Akimoto, Naohiro Sata

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引用次数: 0

Abstract

Portal cavernoma cholangiopathy (PCC) is a complex condition associated with portal hypertension, particularly in patients with extrahepatic portal vein obstruction (EHPVO). Herein, we present a case of liver failure with PCC in a 55-year-old male successfully treated with living-donor liver transplantation (LDLT). The patient had a history of gastrointestinal bleeding and recurrence of cholangitis. Imaging studies confirmed cavernous transformation and pericholedochal varices. Preoperative angiography verified hepatopetal flow in the pericholedochal varix, which facilitated successful anastomosis with the donor's portal vein during LDLT. Histological examination of the explanted liver confirmed vanishing bile duct syndrome (VBDS) and secondary bile stasis was considered to have caused liver failure. No postoperative complications were observed within 13 months of LDLT. We report the first case of VBDS in the PCC resulting from EHPVO that was successfully managed with LDLT. Careful management of similar cases should involve angiography and long-term postoperative monitoring of portal vein complications.

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用活体肝移植治疗门静脉海绵状瘤胆管病：病例报告和文献综述。

门静脉海绵状瘤胆管病（PCC）是一种与门静脉高压症相关的复杂病症，尤其适用于肝外门静脉阻塞（EHPVO）患者。在此，我们介绍了一例肝衰竭合并 PCC 的病例，患者是一名 55 岁的男性，成功接受了活体肝移植（LDLT）治疗。患者曾有消化道出血和胆管炎复发病史。影像学检查证实了海绵体转化和胆总管静脉曲张。术前血管造影验证了胆总管静脉曲张处的肝瓣血流，这有助于在LDLT过程中与供体的门静脉成功吻合。取出肝脏的组织学检查证实了胆管消失综合征（VBDS），继发性胆汁淤积被认为是导致肝衰竭的原因。LDLT术后13个月内未发现术后并发症。我们报告了首例通过 LDLT 成功治疗的 EHPVO 引起的 PCC VBDS 病例。类似病例的谨慎处理应包括血管造影和术后长期监测门静脉并发症。

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

Clinical Journal of Gastroenterology GASTROENTEROLOGY & HEPATOLOGY-

CiteScore

2.00

自引率

0.00%

发文量

182

期刊介绍： The journal publishes Case Reports and Clinical Reviews on all aspects of the digestive tract, liver, biliary tract, and pancreas. Critical Case Reports that show originality or have educational implications for diagnosis and treatment are especially encouraged for submission. Personal reviews of clinical gastroenterology are also welcomed. The journal aims for quick publication of such critical Case Reports and Clinical Reviews.