Korean J Transplant.  2021 Mar;35(1):59-65. 10.4285/kjt.20.0038.

Pediatric liver transplantation using a hepatitis B surface antigen-positive donor liver graft for congenital absence of the portal vein

• Namgoong JM
• Hwang S
• Kim DY
• Ha TY
• Song GW
• Jung DH
• Kim KM
• Oh SH

• KMID: 2514410
• DOI: http://doi.org/10.4285/kjt.20.0038

Abstract

Congenital absence of the portal vein (CAPV) is a rare venous malformation in which mesenteric venous blood drains directly into systemic circulation. Herein, we report a case of pediatric deceased donor liver transplantation (DDLT) for symptomatic CAPV with whole liver graft from a hepatitis B surface antigen (HBsAg)-positive donor. A 4-year-old boy suffered from CAPV and secondary portal hypertension. He was also diagnosed with DiGeorge syndrome and heart anomalies. After waiting for 4 months, a 5-year-old donor weighing 19 kg with positive HBsAg was allocated to this 4-year-old patient weighing 15 kg. Recipient operation was performed according to the standard procedures of pediatric DDLT. Portal vein reconstruction was performed using interposition of a vascular homograft conduit to the superior mesenteric vein-splenic vein confluence. The patient recovered uneventfully from DDLT. He has been administered with lamivudine to prevent hepatitis B virus infection. This patient has been doing well for 5 years after DDLT without growth retardation. In conclusion, CAPV patients can have various vascular anomalies, thus combined vascular anomalies should be thoroughly assessed before and during liver transplantation operation. The most effective reconstruction techniques should be used to achieve satisfactory results following liver transplantation.

Keyword

Portal vein agenesis; Portacaval shunt; Portal hypertension; Hepatitis B virus; Preemptive therapy

Figure

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  Fig. 1 Pretransplant computed tomography scan. (A-C) There is agenesis of the portal vein with cavernous transformation and secondary portal hypertension with gastric and esophageal varix. Any large communication vein to the inferior vena cava or left renal vein is not visible. (D) The anatomy of the hepatic artery appears to be normal.

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  Fig. 2 Surgical technique of end-to-side conduit vein interposition. (A) Anatomy of the superior mesenteric vein-splenic vein confluence is visualized. (B) An external iliac vein graft (cylinder) was anastomosed to the superior mesenteric vein-splenic vein confluence after deep clamping of this confluence portion (blue line). (C) The interposed vascular conduit (cylinder) is located between the superior mesenteric vein-splenic vein confluence and graft portal vein.

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  Fig. 3 Gross photographs of the explant liver. (A) The liver parenchyma does not show cirrhotic changes. (B, C) Magnification of the portal triad area shows increased vascularity of portal venous structures with variable shapes and intimal fibrosis.

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  Fig. 4 Posttransplant computed tomography scan taken at 7 days after transplantation. The portal vein reconstruction appears to be smooth streamlined with resolution of variceal collaterals. An arrow indicates the anastomosis site of the interposed vascular conduit and the superior mesenteric vein-splenic vein confluence.

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  Fig. 5 Posttransplant computed tomography scan taken at 1 month after transplantation. (A) The intrahepatic portal vein appears normal. (B) The extrahepatic portal vein is fully expanded with collapse of the collateral veins.

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