Modified multivisceral transplantation with spleen-preserving pancreaticoduodenectomy for patients with familial adenomatous polyposis "Gardner's Syndrome"

Abstract

Background: Liver-sparing "modified" multivisceral transplantation (MMVTx) has recently been more used for patients with diffuse gastrointestinal disorders and preserved hepatic functions. Evisceration techniques with preservation of native spleen were also introduced to reduce risk of posttransplant lymphoproliferative disorders. This study focuses on the indications of MMVTx for patients with familial adenomatous polyposis (FAP) and the technical feasibility of performing spleen-preserving pancreaticoduodenectomy (SPPD).

Methods: Between 1993 and 2009, 10 FAP patients required MMVTx. Nine were adults and one was a child, with a female:male ratio of 1:1.

Results: Short gut with duodenal adenomatosis and extensive desmoid tumors with pancreaticoduodenal involvement dictated need for MMVTx. SPPD was technically feasible in four recipients, and conventional evisceration including splenectomy was performed in remaining six recipients. With an overall cumulative survival of 90% at 1 year and 77% at 10 years, all SPPD recipients were alive with no single example of posttransplant lymphoproliferative disorder, graft-versus-host disease, or chronic rejection. However, SPPD was associated with an increase (P>0.3) in total ischemia time, operative time, and packed red blood cells requirement but with shorter (P=0.6) length of hospital stay. With a mean follow-up of 50±45 months (range 18-128 months), none of the 10 recipients experienced intraabdominal desmoid tumor recurrence or developed de novo visceral allograft neoplasm.

Conclusion: MMVTx is a valuable therapeutic option for FAP patients who are in need for visceral transplantation with pathologic involvement of the pancreaticoduodenal complex. SPPD is technically feasible, and efforts should always be made to preserve native spleen because of the reported herein therapeutic advantages.