Ann Liver Transplant.  2024 Nov;4(2):134-140. 10.52604/alt.24.0023.

Portal vein interposition in living donor liver transplantation for a pediatric patient with hepatoblastoma invading the portal vein

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

• KMID: 2561861
• DOI: http://doi.org/10.52604/alt.24.0023

Abstract

Liver transplantation is accepted as an effective therapeutic option for unresectable hepatoblastoma. We present a pediatric case of hepatoblastoma patient with portal vein (PV) tumor thrombosis (PVTT) that occluded the main PV completely despite aggressive neoadjuvant chemotherapy. The patient was a 4-year and 2-month-old 17-kg boy suffering from hepatoblastoma with PVTT, which was partially regressed by neoadjuvant chemotherapy. Viable PVTT remained after right trisectionectomy and repetition of systemic chemotherapy. To remove the tumor completely, we performed living donor liver transplantation (LDLT) using his father’s left liver graft. The native PV was completely occluded with PVTT and abundant pericholedochal collaterals developed. The native PV was meticulously dissected to fully expose the superior mesenteric vein-splenic vein confluence. This confluence portion was deeply clamped and opened to use as a viable PV inflow source. A cold-stored fresh external iliac vein homograft was anastomosed to the confluence portion as an end-to-end fashion and passed through the native retropancreatic PV tract after mechanical dilatation of the tract. Left liver graft implantation was performed using standard procedures of pediatric LDLT. The patient recovered uneventfully. He will undergo scheduled adjuvant chemotherapy. Customized design for PV reconstruction using interposition vein graft with viable PV inflow source can result in successful PV reconstruction with complete removal of PVTT.

Keyword

Interposition; Venoplasty; Pediatric transplantation; Portal vein tumor thrombosis; Neoadjuvant chemotherapy

Figure

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  Figure 1 Preoperative computed tomography findings. (A,B) At 30 months of age, large tumors occupied the right liver. (C,D) Tumors were reduced after neoadjuvant chemotherapy and portal vein tumor thrombosis remained at the age of 34 months.

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  Figure 2 Gross photograph of the resected right liver at the age of 35 months.

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  Figure 3 Pretransplant computed tomography findings at the age of 48 months. (A-C) Extensive intrahepatic and extrahepatic portal vein thromboses are identified. (D) Portal vein tumor thrombosis was extended to the superior mesenteric vein-splenic vein confluence with obliterated native portal vein (arrow).

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  Figure 4 Preplanned design to obtain portal blood flow at the superior mesenteric vein-splenic vein confluence portion. A dotted circle indicates the boundary of vein conduit anastomosis.

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  Figure 5 Intraoperative photographs showing PV interposition and reconstruction. (A) The neck of the pancreas was lifted after dissection of the obliterated native PV vein. (B,C) The dissected the SMV and SV confluence portion was deeply clamped and opened (arrow). (D,E) A cold-stored fresh external iliac vein homograft was anastomosed to the SMV-SV confluence portion as an end-to-end fashion. (F) The native PV tract posterior to the pancreas neck meticulously dilated with Hegar cervical dilators. (G,H) The interposed PV conduit was passed through the native retropancreatic PV tract. PV, portal vein; SMV, superior mesenteric vein; SV, splenic vein.

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  Figure 6 Intraoperative photograph showing portal vein reconstruction using an interposition vein graft.

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  Figure 7 Gross photograph of the explanted liver.

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  Figure 8 Post-transplant computed tomography finding taken at 5 days after transplantation. (A,B) Uneventful hepatic vein reconstruction was identified. (C,D) A large-sized streamline shape of the portal vein conduit is connected between the graft portal vein and the confluence portion of the superior mesenteric vein and the splenic vein (arrows).

Reference

1. Parkin DM, Stiller CA, Draper GJ, Bieber CA. 1988; The international incidence of childhood cancer. Int J Cancer. 42:511–520. DOI: 10.1002/ijc.2910420408. PMID: 3170025.

2. Perilongo G, Maibach R, Shafford E, Brugieres L, Brock P, Morland B, et al. 2009; Cisplatin versus cisplatin plus doxorubicin for standard-risk hepatoblastoma. N Engl J Med. 361:1662–1670. DOI: 10.1056/NEJMoa0810613. PMID: 19846851.

3. Trobaugh-Lotrario AD, Katzenstein HM. 2012; Chemotherapeutic approaches for newly diagnosed hepatoblastoma: past, present, and future strategies. Pediatr Blood Cancer. 59:809–812. DOI: 10.1002/pbc.24219. PMID: 22648979.

4. Kasahara M, Ueda M, Haga H, Hiramatsu H, Kobayashi M, Adachi S, et al. 2005; Living-donor liver transplantation for hepatoblastoma. Am J Transplant. 5:2229–2235. DOI: 10.1111/j.1600-6143.2005.01003.x. PMID: 16095502.

5. Kanazawa H, Sakamoto S, Matsunami M, Sasaki K, Uchida H, Shigeta T, et al. 2014; Technical refinement in living-donor liver transplantation for hepatoblastoma with main portal vein tumor thrombosis - a pullout technique. Pediatr Transplant. 18:E266–E269. DOI: 10.1111/petr.12357.

6. Otte JB, de Ville de Goyet J, Reding R. 2005; Liver transplantation for hepatoblastoma: indications and contraindications in the modern era. Pediatr Transplant. 9:557–565. DOI: 10.1111/j.1399-3046.2005.00354.x. PMID: 16176410.

7. Roebuck DJ, Aronson D, Clapuyt P, Czauderna P, de Ville de Goyet J, Gauthier F, et al. International Childrhood Liver Tumor Strategy Group. 2005 PRETEXT: a revised staging system for primary malignant liver tumours of childhood developed by the SIOPEL group. Pediatr Radiol. 2007; 37:123–132. DOI: 10.1007/s00247-006-0361-5. PMID: 17186233. PMCID: PMC1805044.

8. Moon DB, Lee SG, Ahn CS, Hwang S, Kim KH, Ha TY, et al. 2014; Restoration of portal flow using a pericholedochal varix in adult living donor liver transplantation for patients with total portosplenomesenteric thrombosis. Liver Transpl. 20:612–615. DOI: 10.1002/lt.23850. PMID: 24677674.

9. Namgoong JM, Hwang S, Park GC, Kwon H, Ha S, Kim KM, et al. 2021; Portal vein interposition in living donor liver transplantation for a pediatric hepatoblastoma patient with portal vein tumor thrombosis. Ann Liver Transplant. 1:180–186. DOI: 10.52604/alt.21.0030.

10. Hwang S, Kim DY, Ahn CS, Moon DB, Kim KM, Park GC, et al. 2013; Computational simulation-based vessel interposition reconstruction technique for portal vein hypoplasia in pediatric liver transplantation. Transplant Proc. 45:255–258. DOI: 10.1016/j.transproceed.2012.05.090. PMID: 23375311.

11. Namgoong JM, Hwang S, Oh SH, Kim KM, Park GC, Ahn CS, et al. 2020; Living donor liver transplantation with total pancreatectomy and portal vein homograft replacement in a pediatric patient with advanced pancreatoblastoma. Ann Hepatobiliary Pancreat Surg. 24:78–84. DOI: 10.14701/ahbps.2020.24.1.78. PMID: 32181434. PMCID: PMC7061052.

12. Sharshar M, Yagi S, Iida T, Yao S, Miyachi Y, Macshut M, et al. 2020; Liver transplantation in patients with portal vein thrombosis: a strategic road map throughout management. Surgery. 168:1160–1168. DOI: 10.1016/j.surg.2020.07.023. PMID: 32861438.

13. Hwang S, Bae JH, Kim IO, Hong JJ. 2021; Current vascular allograft procurement, cryopreservation and transplantation techniques in the Asan Medical Center Tissue Bank. Ann Liver Transplant. 1:79–85. DOI: 10.52604/alt.21.0016.