Ann Liver Transplant.  2023 Nov;3(2):118-127. 10.52604/alt.23.0016.

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

• Namgoong JM
• Hwang S
• Park GC
• Kang S
• Kim KM
• Oh SH

• KMID: 2560933
• DOI: http://doi.org/10.52604/alt.23.0016

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 3-year-old boy was diagnosed with CAPV at the age of 2 years. Portal hypertension with collateral vein formation was progressed, thus we decided to perform LDLT. The graft was a left lateral section graft from the 35-year-old father of the patient. Recipient hepatectomy was performed according to the standard procedures of pediatric LDLT with isolation of two pericholedochal collateral veins. These collateral veins were unified and integrated with an iliac vein homograft using a modified patch-conduit venoplasty. The left lateral section graft was implanted with direct ligation of the coronary and splenorenal collateral veins. However, luminal thrombus was formed within the reconstructed portal vein conduit, thus the conduit was removed and graft portal vein was directly anastomosed with the pericholedochal collateral veins. The patient recovered from the LDLT operation, but the reconstructed portal vein was showed marked anastomotic stenosis. At 40 days after LDLT, percutaneous transhepatic balloon angioplasty was performed and the anastomotic stenosis was expanded. This patient has been doing well for 3 months after the LDLT. In conclusion, given the diverse presentations of portocaval shunt in CAPV patients, it is imperative to tailor the portal vein reconstruction approach based on a comprehensive anatomical assessment both prior to and during liver transplantation operation.

Keyword

Portal vein agenesis; Portocaval shunt; Left lateral section graft; Direct anastomosis; Percutaneous transhepatic angioplasty

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 along the common bile duct. (D) The hepatic arteries appeared normal without noticeable anatomical variation.

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  Figure 2 Computed tomography findings of the donor before donation surgery (A) and at one week after donation of the left lateral section (B).

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  Figure 3 Intraoperative photographs of the recipient hilar dissection. (A) The portal cavernous collateral veins were isolated with closure of the common bile duct. (B–D) Two collateral veins were incised and unified to make a left half wall of the portal vein conduit.

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  Figure 4 Intraoperative photographs of modified patch-conduit venoplasty to make a portal vein conduit. (A–C) An iliac vein homograft was attached to make a right half wall of the portal vein conduit. (D) The portal vein conduit was clamped after excision of the redundant vein homograft.

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  Figure 5 Intraoperative photographs of graft outflow vein reconstruction (A–C) The hepatic vein orifices were unified to make a large single orifice. (D) The graft outflow vein was anastomosed using 5-0 PDS continuous sutures.

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  Figure 6 Intraoperative photographs of graft portal vein reconstruction using the newly made conduit. (A, B) The portal vein conduit was anastomosed using a 6-0 PDS. (C, D) The reconstructed portal vein conduit showed a large diameter.

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  Figure 7 Intraoperative photographs of graft portal vein reconstruction using a new orifice using the unified pericholedochal collateral veins. (A) The portal vein conduit was complexly excised (bidirectional arrow) and two pericholedochal collateral veins were unified to make a single orifice. (B) A newly created orifice was used for portal vein reconstruction. (C, D) The reconstructed portal vein showed an anastomotic stenosis.

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  Figure 8 Doppler ultrasonography taken at one day after transplantation. (A) A long anastomotic stenosis was identified (arrow). (B) High velocity difference was measured across the portal vein anastomosis.

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  Figure 9 Gross photograph of the explant liver.

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  Figure 10 Fig. 10 . Posttransplant computed tomography scan taken at four days after transplantation. (A) Uneventful anastomosis of the graft hepatic vein was identified. (B–D) A marked anastomotic stenosis was identified at the reconstructed portal vein (arrows).

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  Figure 11 Fig. 11 . Percutaneous transhepatic angioplasty taken at 40 days after transplantation. (A) A puncture was made through the segment III porta vein branch. (B) The remained portal collateral veins were markedly engorged. (C) A marked anastomotic stenosis was identified (arrow). (D) Balloon angioplasty was performed to dilate the anastomotic stenosis (arrow).

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