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

被引：0

作者：

Omameuda, Takahiko ^{[1
]}

Sanada, Yukihiro ^{[1
]}

Sakuma, Yasunaru ^{[1
]}

Onishi, Yasuharu ^{[1
]}

Wakiya, Taiichi ^{[1
]}

Hirata, Yuta ^{[1
]}

Horiuchi, Toshio ^{[1
]}

Takadera, Kiichiro ^{[1
]}

Akimoto, Ryosuke ^{[1
]}

Sata, Naohiro ^{[1
]}

机构：

[1] Jichi Med Univ, Dept Surg, Div Gastroenterol Gen & Transplant Surg, 3311-1 Yakushiji, Shimotsuke, Tochigi 3290498, Japan

来源：

CLINICAL JOURNAL OF GASTROENTEROLOGY | 2024年 / 17卷 / 06期

关键词：

Extrahepatic portal vein obstruction; Liver transplantation; Pericholedochal varix; Portal cavernoma cholangiopathy; Vanishing bile duct syndrome; PERICHOLEDOCHAL VARIX; VEIN; MANAGEMENT; ADULT;

D O I：

10.1007/s12328-024-02041-7

中图分类号：

R57 [消化系及腹部疾病];

学科分类号：

摘要：

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.

引用

页码：1080 / 1086

页数：7

共 18 条

[1]

Bharathy KGS, 2017, J CLIN EXP HEPATOL, V7, P63, DOI 10.1016/j.jceh.2016.10.007

[2] Portal cavernoma cholangiopathy: Update and recommendations on diagnosis and management [J].