Korean J Transplant.  2021 Mar;35(1):66-70. 10.4285/kjt.20.0039.

Pediatric split liver transplantation for congenital factor X deficiency: first 10-year follow-up of a case with portal vein stenting

• Namgoong JM
• Hwang S
• Kim DY
• Ha TY
• Song GW
• Dong-Hwan DH
• Kim KM
• Oh SH

• KMID: 2514411
• DOI: http://doi.org/10.4285/kjt.20.0039

Abstract

Congenital factor X (FX) deficiency is a rare autosomal-recessive disease that induces bleeding disorder. Herein, we present the 10-year posttransplant course of a pediatric patient who underwent liver transplantation (LT) with portal vein (PV) stenting for correction of severe congenital FX deficiency, with focus on long-term maintenance of coagulation function and patency of PV stenting. A 17-month-old infant with recurrent hemorrhagic episodes due to FX deficiency underwent split LT using a left lateral sec- tion graft. The graft-recipient weight ratio was 2.2%. The graft implantation procedures were performed by following the standard pediatric split LT procedure. Nevertheless, a wall stent was inserted due to PV anastomotic stenosis on posttransplant day 1. Graft function recovered slowly because of partial parenchyma infarct, and the patient was discharged at 46 days after LT operation. The FX activity started to increase soon after LT and gradually normalized; the coagulation profiles have been maintained well for the past 10 years. The patient has been doing well for the past 10 years after LT without any episodes of abnormal bleeding. Due to the risk of vascular complications owed to PV stenting, life-long follow-up is mandatory with special attention until attainment of complete physical growth to adolescent and adulthood.

Keyword

Coagulation factor; Autosomal-recessive genetic disease; Portal vein; Stent; Split liver transplantation

Figure

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  Fig. 1 Pretransplant computed tomography scan showing normal looking hepatic parenchyma (A) and perihilar vascular anatomy (B).

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  Fig. 2 Gross photographs of the explant liver showing normal-looking hepatic parenchyma.

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  Fig. 3 Posttransplant computed tomography scan taken at 1 day after transplantation. Heterogeneous low attenuation of the hepatic parenchyma (A) and segmental stenosis of the portal vein anastomosis site (B) are identified. Arrows indicate the site of anastomotic stenosis.

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  Fig. 4 Posttransplant portal vein stenting performed at 1 day after transplantation. (A) A significant stenosis portal vein anastomosis site is identified. (B) A 10 mm×40 mm-sized wall stent is inserted across the stenosis. (C) The stenotic site is expanded after stenting. Arrows indicate the site of anastomotic stenosis.

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  Fig. 5 Posttransplant computed tomography scan taken at 2 days after transplantation. (A) Heterogeneous low attenuation of the hepatic parenchyma was slightly improved. (B) The configuration of the reconstructed portal vein with an internal stent implicates the presence of tension and acute angle bending at the anastomosis site before stenting.

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  Fig. 6 Follow-up Doppler ultrasonography taken at 9 years after transplantation. The intrahepatic portal blood flow is well maintained (up to 20 cm/sec) with slight narrowing of the intrastent diameter. The intrastent diameter of the portal vein was about 6 mm, which remained unchanged during last 5 years despite gradual physical growth of the patient.

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