Ann Liver Transplant.  2021 Nov;1(2):180-186. 10.52604/alt.21.0030.

Portal vein interposition in living donor liver transplantation for a pediatric hepatoblastoma patient with portal vein tumor thrombosis

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

• KMID: 2560870
• DOI: http://doi.org/10.52604/alt.21.0030

Abstract

Liver transplantation is accepted as an effective therapeutic option for unresectable hepatoblastoma. We present a pediatric case of hepatoblastoma patient with portal vein tumor thrombosis (PVTT) that occluded the main portal vein completely despite neoadjuvant chemotherapy. The patient was a 36-month-old 17-kg girl suffering from hepatoblastoma with PVTT, which was partially regressed by neoadjuvant chemotherapy. Viable PVTT remained after left hepatectomy and repetition of systemic chemotherapy due to partial treatment response. To remove the tumor completely, we performed living donor liver transplantation using her mother’s left lateral section graft. The blood flow from the native portal vein was greater than that from the pericholedochal collaterals, thus we used the native portal vein as the source of portal inflow. A cold-stored fresh external iliac vein homograft was anastomosed to the portal vein stump. Graft implantation was performed using standard procedures of pediatric liver transplantation. The patient recovered uneventfully. She has been undergoing scheduled adjuvant chemotherapy to date. Meticulous design for portal vein reconstruction using interposition vein graft with the remaining splanchnic vein stumps resulted in successful portal vein reconstruction with complete removal of PVTT.

Keyword

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

Figure

• 

  Figure 1 Pretransplant computed tomography findings. (A) At 20 months of age, multiple tumors occupied the whole liver. (B) Tumors were reduced after neoadjuvant chemotherapy, but viable tumor with portal vein tumor thrombosis (arrow) remained. (C, D) Left hepatectomy was performed to remove viable tumors (arrows) at 1 year before liver transplantation.

• 

  Figure 2 Pretransplant computed tomography (CT) findings. (A) Portal vein thrombosis with cavernous transformation was visible at 2 months after left hepatectomy. (B) At 6 months after left hepatectomy, filling defects were detected at the portal vein enlarged (arrow). (C, D) CT scan just before transplantation showed the pericholedochal collaterals (yellow arrow) and the occluded confluence portion of the superior mesenteric vein and the splenic vein (red arrow).

• 

  Figure 3 Intraoperative photograph of the recipient liver after mobilization. The blue and yellow vessel loop each encircled the thrombosed native portal vein and the pericholedochal collateral, respectively.

• 

  Figure 4 Intraoperative photographs showing transection of the pericholedochal varices. (A) The longitudinal axis of the pericholedochal varices is marked. (B) The common bile duct opening (arrow) is exposed. (C) Weak blood flow (arrow) is identified from the collateral veins. (D) The pericholedochal varices are temporarily clamped.

• 

  Figure 5 Intraoperative photographs showing preparation of the native portal vein. (A, B) The thrombosed native portal vein is extensively dissected to the confluence level of the superior mesenteric vein and the splenic vein, and transected at this level. (C, D) A cold-stored fresh external iliac vein homograft is anastomosed to the portal vein stump.

• 

  Figure 6 Intraoperative photographs of the graft implantation. (A–C) Hepatic vein reconstruction is performed. (D–G) Portal vein reconstruction is performed after careful length adjustment of the interposed vein conduit. (H) The pericholedochal collaterals including the common bile duct are securely ligated after graft reperfusion.

• 

  Figure 7 Gross photograph of the explanted liver. Arrow indicates the portal vein tumor thrombus.

• 

  Figure 8 Post-transplant computed tomography finding taken at 10 days after transplantation. (A) A left lateral section graft is well regenerated. (B) Collateral veins (arrow) are visible around the pancreas. (C, D) The portal vein conduit is anastomosed at 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. PMID: 25213021.

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. 2007; 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. 37:123–132. quiz 249-250. DOI: 10.1007/s00247-006-0361-5. PMID: 17186233. PMCID: PMC1805044.

8. Katzenstein HM, Langham MR, Malogolowkin MH, Krailo MD, Towbin AJ, McCarville MB, et al. 2019; Minimal adjuvant chemotherapy for children with hepatoblastoma resected at diagnosis (AHEP0731): a Children's Oncology Group, multicentre, phase 3 trial. Lancet Oncol. 20:719–727. DOI: 10.1016/S1470-2045(18)30895-7.

9. 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.

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. 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.