Ann Liver Transplant.  2022 Nov;2(2):144-150. 10.52604/alt.22.0019.

Living donor liver transplantation in a pediatric patient having intrahepatic portocaval shunt with congenital absence of the intrahepatic portal vein

• Namgoong JM
• Hwang S
• Park GC
• Ha S
• Gang S
• Park J
• Oh SH

• KMID: 2560910
• DOI: http://doi.org/10.52604/alt.22.0019

Abstract

Congenital absence of the portal vein (CAPV) is a rare venous malformation in which the mesenteric venous blood drains directly into systemic circulation. We report the case of a pediatric living donor liver transplantation (LDLT) for CAPV with an intrahepatic portosystemic shunt. A 3-year-old boy was diagnosed with CAPV at the age of 1 year. There was no evidence of portal hypertension due to complete diversion of the portal blood flow into the retrohepatic inferior vena cava. The patient suffered from hepatic encephalopathy; therefore, we decided to perform LDLT. The graft was the left liver from the 31-year-old mother of the patient. The recipient hepatectomy was performed according to standard procedures of pediatric LDLT. Portal vein reconstruction was performed using a branch patch of the native portal vein. The patient recovered uneventfully from the LDLT. The reconstructed portal vein was maintained well without any hemodynamic abnormalities. In conclusion, the features of CAPV and portocaval shunt may vary among CAPV patients; thus, portal vein reconstruction should be customized according to anatomical variations.

Keyword

Portal vein agenesis; Portocaval shunt; Portosystemic shunt; Living donor liver transplantation; Hyperammonemia

Figure

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  Figure 1 Pretransplant computed tomography findings of the recipient. The extrahepatic portal vein anatomy appeared to be normal (A, C), but portocaval shunting through the inferior right hepatic vein (arrows) was identified (B, D). The anatomy of the major hepatic veins and hepatic artery appeared normal (E, F).

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  Figure 2 Intraoperative photographs of dissection of the retrohepatic inferior vena cava and hepatoduodenal ligament dissection. The enlarged inferior hepatic vein was identified and encircled (arrow) (A), and the hepatic arteries were enlarged (B). After transection of the hepatic arteries, the portal vein was traced via hepatic parenchymal transection (C, D).

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  Figure 3 Intraoperative photographs showing removal of the recipient’s native liver. The right and left portal vein branches were clamped, leaving the portocaval shunt (arrow) patent (A, B). After the recipient’s native liver was removed, the supra- and infra-hepatic inferior vena cava portions were clamped (C). The communicating inferior right hepatic vein was also clamped and ligated (D).

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  Figure 4 Intraoperative photographs of graft implantation. The right and left portal vein stumps were opened to create a branch patch (A). The graft hepatic vein orifice of the left liver graft was enlarged by unification venoplasty of the left and middle hepatic vein openings (B). After the native liver was removed, the three hepatic vein orifices at the recipient inferior vena cava were unified (C). The graft hepatic vein was then reconstructed by continuous suturing with 5-0 polydioxanone (D).

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  Figure 5 Intraoperative photographs of graft implantation. The graft hepatic vein was reconstructed by continuous suturing with 5-0 polydioxanone (A). The recipient portal vein branch patch was anastomosed with the graft portal vein using 6-0 polydioxanone suture (B, C). After graft reperfusion, the reperfusion status appeared normal (D).

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  Figure 6 Gross photographs of the explant liver.

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  Figure 7 Posttransplant computed tomography scan obtained at 5 days (A) and 2 weeks (B) after transplantation. The graft portal vein was perfused well without noticeable stenosis.

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