Li Peilin, Hidaka Masaaki, Hamada Takashi, Ikeda Satoshi, Ono Shinichiro, Maruya Yasuhiro, Kugiyama Tota, Hara Takanobu, Yoshimoto Tomoko, Adachi Tomohiko, Tanaka Takayuki, Miyoshi Takayuki, Murakami Shunsuke, Huang Yu, Kanetaka Kengo, Eguchi Susumu
Department of Surgery, Nagasaki University Graduate School of Biomedical Science, 1-7-1 Sakamoto, Nagasaki, 852-8102, Japan.
Department of Cardiovascular Medicine, Nagasaki University Graduate School of Biomedical Science, 1-7-1 Sakamoto, Nagasaki, 852-8102, Japan.
Surg Case Rep. 2020 Jun 29;6(1):153. doi: 10.1186/s40792-020-00916-8.
Congenital absence of portal vein (CAPV) is a rare structural anomaly in which the portal vein (PV) blood that normally flow into the liver directly drains into the systemic venous system through other collateral circulation. Congenital portal vein shunts (CPSs) is classified into types I and II according to the absence or presence of the intrahepatic portal vein, respectively. The CPS type I is also known as CAPV. The liver transplantation may be the only treatment option for CAPV. The key point of liver transplantation for CAPV is the reconstruction of the PV.
A 29-year-old man was diagnosed with CAPV with splenomegaly and gastroesophageal varix when being treated for pancytopenia and liver dysfunction. A living donor liver transplantation was performed for him using the right lobe which had been donated by his mother. The PV was reconstructed using his own great saphenous vein (GSV) as a graft vein. The end of the GSV graft was anastomosed to the inferior mesenteric vein while the other end was anastomosed to the vein graft of the right hepatic vein from the explanted liver.
Using the patient's own GSV for PV reconstruction during living donor transplantation in the patient with CAPV seems to be an effective method.
先天性门静脉缺如（CAPV）是一种罕见的结构异常，正常情况下流入肝脏的门静脉（PV）血通过其他侧支循环直接引流至体静脉系统。先天性门静脉分流（CPS）根据肝内门静脉的有无分别分为I型和II型。CPS I型也称为CAPV。肝移植可能是CAPV的唯一治疗选择。CAPV肝移植手术重点在于门静脉重建。
一名29岁男性在治疗全血细胞减少症和肝功能不全时被诊断为CAPV伴脾肿大和胃食管静脉曲张。为其进行了活体供肝肝移植，供肝为其母亲捐献的右半肝。采用患者自身大隐静脉（GSV）作为移植血管重建门静脉（PV）。GSV移植血管一端吻合至肠系膜下静脉，另一端吻合至切除肝脏右肝静脉移植血管。
在CAPV患者活体供肝移植术中，采用患者自身GSV重建门静脉（PV）似乎是一种有效的方法。
