Abstract
BackgroundCongenital 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.Case presentationA 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.ConclusionUsing the patient’s own GSV for PV reconstruction during living donor transplantation in the patient with CAPV seems to be an effective method.
Highlights
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
The use of the donor’s ovarian vein and the patient’s hepatic vein (HV) as graft veins has been reported [7, 8], but the use of the patient’s own great saphenous vein (GSV) as the graft vein for a patient with CAPV has never been described, the GSV was reportedly used as graft in living donor liver transplantation (LDLT) for other diseases [9, 10]
We report a case of CAPV, in which LDLT was performed for the patient and the patient’s own GSV was used to reconstruct the PV
Summary
The key to LDLT in patients with CAPV is PV reconstruction. The present findings proved that using the GSV to reconstruct the PV in CAPV patients is an effective method.
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