Ann Liver Transplant.  2021 Nov;1(2):165-173. 10.52604/alt.21.0021.

Emergency living donor liver transplantation under extracorporeal membrane oxygenation in an infant with biliary atresiapolysplenia syndrome

• Namgoong JM
• Hwang S
• Park GC
• Kwon H
• Kim KM
• Oh SH

• KMID: 2560868
• DOI: http://doi.org/10.52604/alt.21.0021

Abstract

Biliary atresia-polysplenia syndrome (BAPS) is diagnosed in a small number of patients with biliary atresia (BA). We present a case of emergency living donor liver transplantation (LT) successfully performed in an infant with BAPS undergoing extracorporeal membrane oxygenation. The recipient was a 10-month-old boy who did not undergo Kasai portoenterostomy due to rapid progression of liver cirrhosis. Co-existing malformations included heterotopic inferior vena cava without hepatic communication, direct hepatic vein drainage into the right atrium, polysplenia, intestinal malrotation, truncated pancreas, and preduodenal portal vein and annular pancreas. Patient condition deteriorated rapidly after pulmonary hemorrhage, and thus emergency living donor LT was performed after starting veno-venous extracorporeal membrane oxygenation (ECMO) with a pediatric end-stage liver disease score of 32. A left lateral section graft obtained from his father showed a graft-to-recipient weight ratio of 3.2%. The recipient surgery was performed according to standard procedures of pediatric LT. The graft hepatic vein was directly anastomosed with the suprahepatic confluence of the recipient hepatic veins. An external iliac vein homograft was interposed for portal vein reconstruction. Multiple portal collateral veins were ligated and intraoperative portography was performed to secure portal vein inflow. The patient was weaned off ECMO and ventilator were weaned off on 17 days and 65 days respectively after transplantation. The patient stayed at the intensive care unit for 3 months before and after transplantation. Our pediatric patient with BAPS manifested various anatomical malformations. Successful LT requires comprehensive preoperative and intraoperative assessment of these anomalies, adoption of customized reconstruction techniques of LT, and careful posttransplant monitoring.

Keyword

Preduodenal portal vein; Inferior vena cava; Intestinal malrotation; Vascular complication; Pediatric transplantation

Figure

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  Figure 1 Pretransplant computed tomography (CT) scans. (A) CT taken at 3 months of age reveals overt liver cirrhosis. (B) CT taken at 6 months of age shows progression of liver cirrhosis. (C, D) CT taken at 10 months of age indicates marked progression of liver cirrhosis with formation of ascites.

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  Figure 2 Peritransplant simple x-ray images. (A) Pulmonary hemorrhage occurred before liver transplantation, interfering pulmonary oxygenation. (B) Veno-venous extracorporeal membrane oxygenation (arrow) was initiated through the jugular vein catheters before transplantation and continued until recovery of pulmonary function.

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  Figure 3 Images of vascular reconstruction of the retransplant computed tomography taken 3 days before liver transplantation. (A) Two anomalous superior mesenteric veins (arrows) are joined to make a single portal vein. (B) Umbilical collateral veins (arrow) are visibly drained at the upper abdominal wall. (C) Anomalous course of the hepatic artery (arrow) is visible. (D) The heterotopic inferior vena cava (arrow) has no hepatic vein branch from the liver.

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  Figure 4 Intraoperative photographs showing the combined vascular anomalies. (A) The liver was markedly cirrhotic and shows large umbilical collateral veins (arrow). (B) The portal vein runs along the ventral surface of the duodenum, forming the preduodenal portal vein (arrow). (C) The isolated portal vein (blue loop) and hepatic artery (yellow loop) are visible after completion of hilar dissection. No common bile duct was identified. (D) The retrohepatic inferior vena cava was absent (arrow) and the hepatic vein stump is directly drained into the right atrium.

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  Figure 5 Intraoperative photographs showing reconstruction of the graft hepatic vein. (A) The suprahepatic hepatic vein stump was exposed after deep clamping of the right atrium. (B, C) The septum at the hepatic vein stump was cut to make a single orifice (arrow). (D) The graft hepatic vein stump was unified (arrow) to match with the recipient hepatic vein stump. (E, F) Graft hepatic vein was reconstructed through a size-matched anastomosis.

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  Figure 6 Intraoperative photographs showing reconstruction of the graft portal vein. (A) The interposed iliac vein conduit (arrow) connected to the preduodenal portal vein appears to be excessively redundant. (B) Portal vein anastomosis was performed after removal of the majority of the interposed iliac vein homograft. (C, D) A small segment of interposed iliac vein homograft (arrow) was left to enlarge the diameter of the native portal vein.

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  Figure 7 Findings of intraoperative portography. (A) Multiple retrosplenic collateral veins were ligated immediately after portal reperfusion. (B) Two anomalous superior mesenteric veins (arrows) are joined to make a single portal vein. No anastomotic stenosis of the portal vein is detected. (C, D) Multiple collateral veins are visible around spleen, but all of them were interrupted by multiple sutures.

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  Figure 8 Gross photograph of the explant liver.

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  Figure 9 Posttransplant computed tomography scan taken 21 days after transplantation. (A, B) Graft hepatic vein outflow (arrows) was patent. (C, D) The preduodenal portal vein and graft portal vein (arrows) were reconstructed without anastomotic stenosis.

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