Ann Hepatobiliary Pancreat Surg.  2020 Feb;24(1):78-84. 10.14701/ahbps.2020.24.1.78.

Living donor liver transplantation with total pancreatectomy and portal vein homograft replacement in a pediatric patient with advanced pancreatoblastoma

• Namgoong J
• Hwang S
• Oh SH
• Kim KM
• Park GC
• Ahn CS
• Kwon H
• Cho YJ
• Kwon YJ

• KMID: 2471194
• DOI: http://doi.org/10.14701/ahbps.2020.24.1.78

Abstract

Pancreatoblastoma is a malignant exocrine pancreatic tumor that is usually present in childhood. We herein present one case of pediatric living donor liver transplantation (LDLT) combined with spleen-preserving regional total pancreatectomy and portal vein homograft interposition in a 4-year-old boy with advanced pancreatoblastoma invading the portal and superior mesenteric veins. The size of the pancreatoblastoma was gradually reduced along systemic chemotherapy, thus we decided to perform surgery to remove it completely. A cold-stored fresh iliac vein homograft was prepared. Initially, a spleen-preserving distal pancreatectomy was performed. Thereafter, a completion regional total pancreatectomy was performed under superior mesenteric vein-vena cava bypass. A left liver graft from his mother was implanted according to the standardized procedures with portal vein interposition. This patient recovered uneventfully and is currently undergoing scheduled adjuvant chemotherapy. To our knowledge, this is the world-second case of pediatric LDLT for advanced pancreatoblastoma. Availability of fresh vein homografts is helpful to expand the indication of pediatric LDLT.

Keyword

Homograft; Pancreatectomy; Pediatric transplantation; Pancreatoblastoma

MeSH Terms

Allografts*
Chemotherapy, Adjuvant
Child, Preschool
Drug Therapy
Humans
Iliac Vein
Liver Transplantation*
Liver*
Living Donors*
Male
Mesenteric Veins
Mothers
Pancreatectomy*
Portal Vein*
Transplants
Veins

Figure

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  Fig. 1 Pretransplant computed tomography findings. The size of pancreatoblastoma was gradually reduced according to the neoadjuvant chemotherapy, showing serial size changes as follows: at diagnosis (A), five months before transplantation, (B) one month before transplantation, and (C) one week before transplantation (D). An arrow indicates the proximal end of the superior mesenteric vein.

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  Fig. 2 Intraoperative photographs of the recipient's abdomen. No peritoneal seeding was identified at the mesenteric side (A) and around the pancreas (B).

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  Fig. 3 Intraoperative photographs of spleen-preserving distal pancreatectomy. The pancreas body was transected to facilitate pancreatic resection (A). The celiac axis area was completely dissected (B).

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  Fig. 4 Intraoperative photographs of the superior mesenteric vein-vena cava bypass using a cold-stored fresh iliac vein homograft. The vein graft is anastomosed to the retrohepatic inferior vena cava (A and B). The vein conduit was anastomosed to the superior mesenteric vein as an end-to-side fashion (C and D).

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  Fig. 5 Intraoperative photographs of spleen-preserving regional total pancreatectomy. The common hepatic artery was preserved with extensive lymph node dissection (A). Superior mesenteric vein-caval bypass was maintained during pancreatectomy (B).

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  Fig. 6 Intraoperative photographs of the left liver graft implantation. The graft hepatic vein orifices were unified with an iliac vein patch (A). The recipient's native liver was removed with clamping of the superior mesenteric vein-caval bypass (B). The venous bypass was clamped (C). The iliac vein conduit was anastomosed to the graft portal vein (D).

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  Fig. 7 Intraoperative photographs of the recipient's abdomen after graft implantation. The extent of dissection was visualized. An arrow indicates the interposed iliac vein homograft.

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  Fig. 8 Gross photographs of the resected specimens including the liver, pancreas and duodenum.

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  Fig. 9 Posttransplant computed tomography finding showing streamlined reconstruction figures of the graft hepatic and portal veins taken one week after transplantation. An arrow indicates the interposed iliac vein homograft.

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