Ann Liver Transplant.  2023 Nov;3(2):128-135. 10.52604/alt.23.0013.

Living donor liver transplantation with direct collateral portal vein anastomosis in a pediatric patient with congenital absence of the portal vein

• Namgoong JM
• Hwang S
• Park GC
• Lee DY
• Kim KM
• Oh SH

• KMID: 2560934
• DOI: http://doi.org/10.52604/alt.23.0013

Abstract

Congenital absence of the portal vein (CAPV) is a rare venous malformation in which the mesenteric venous blood drains directly into the systemic circulation. We report a case of pediatric living donor liver transplantation (LDLT) for CAPV with a portal collateral vein of cavernous transformation. A 12-year-old boy was diagnosed with CAPV at the age of 11 years. Portal hypertension with collateral vein formation was rapidly progressed, therefore, we decided to perform LDLT. The graft was a modified right liver graft from the 41-year-old father of the patient. The recipient hepatectomy was performed according to the standard procedures of pediatric LDLT with isolation of the portal collateral vein. This portal collateral vein was gently manipulated and its branches were used to form a branch patch. A modified right liver graft was implanted with direct ligation of the coronary and splenorenal collateral veins. The patient recovered from the LDLT operation. The reconstructed portal vein was maintained well without hemodynamic abnormality. This patient has been doing well for 2 months after the LDLT. In conclusion, since CAPV patients show various types of portocaval shunt, individualized portal vein reconstruction should be performed after thorough anatomical assessment before and during the liver transplantation operation.

Keyword

Portal vein agenesis; Portocaval shunt; Modified right liver graft; Direct anastomosis; Esophageal varix

Figure

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  Figure 1 Pretransplant computed tomography findings of the recipient. (A–C) The native portal vein was absent with the development of cavernous, transformed collateral veins. (D) The hepatic arteries were normal without anatomical variation.

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  Figure 2 Preoperative endoscopic varix ligation performed at three months before transplantation. (A, B) Esophageal and gastric fundic varices were identified. (C) Endoscopic band ligation of the esophageal varix was performed. (D) Regression of varix was identified at 1 week after band ligation.

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  Figure 3 Computed tomography findings of the donor before donation surgery (A) and at one week after right liver donation (B).

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  Figure 4 Intraoperative photographs of recipient hilar dissection. (A) The portal cavernous collateral vein was isolated. (B, C) This collateral vein was transected with distal cutting of the collateral vein branches. (D) The transected vein was gently clamped to prevent iatrogenic injury.

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  Figure 5 Intraoperative photographs of graft implantation. (A) The right liver graft was prepared after vein interposition of the middle hepatic vein branches and patch venoplasty of the right hepatic vein orifice. (B) The right hepatic vein was reconstructed under total clamping of the inferior vena caca. (C) The interposed vein conduit was reconstructed to the left-middle hepatic vein trunk stump. (D) A branch patch was made at the end of the portal cavernous collateral vein. (E–G) The posterior was anastomosed with a running suture with insertion of a vein patch to prevent stitch-inducing wall tearing. (H) The anterior wall was redundantly anastomosed to provide a sufficient growth factor.

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  Figure 6 Gross photograph of the explant liver.

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  Figure 7 Posttransplant computed tomography scan taken at four days after transplantation. (A, B) Uneventful anastomosis of the graft portal vein was identified. (C, D) Unusual running course of the portal collateral vein was identified (arrows).

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