Thomas E. Starzl Transplantation Institute, Department of Surgery, University of Pittsburgh Medical Center, Pittsburgh, PA 15213, USA.
Transplantation. 2011 Jun 27;91(12):1417-23. doi: 10.1097/TP.0b013e31821ab93b.
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).
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.
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.
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.
肝保留“改良”多器官联合移植（MMVTx）最近更多地用于治疗弥漫性胃肠道疾病和保留肝功能的患者。还引入了保留固有脾脏的去内脏技术，以降低移植后淋巴组织增生性疾病的风险。本研究侧重于 MMVTx 用于家族性腺瘤性息肉病（FAP）患者的适应证，以及进行保留脾脏的胰十二指肠切除术（SPPD）的技术可行性。
1993 年至 2009 年期间，10 例 FAP 患者需要 MMVTx。9 例为成人，1 例为儿童，男女比例为 1:1。
短肠伴有十二指肠腺瘤病和广泛的纤维瘤病，胰腺十二指肠受累，需要 MMVTx。4 例受者行 SPPD 技术可行，其余 6 例受者行常规去内脏术，包括脾切除术。总体 1 年累积生存率为 90%，10 年累积生存率为 77%，所有 SPPD 受者均存活，无一例发生移植后淋巴组织增生性疾病、移植物抗宿主病或慢性排斥反应。然而，SPPD 与总缺血时间、手术时间和红细胞压积需求增加（P>0.3）相关，但住院时间缩短（P=0.6）。平均随访 50±45 个月（范围 18-128 个月），10 例受者均未发生腹腔内纤维瘤病复发或新发内脏移植物肿瘤。
对于需要进行胰腺十二指肠复合体病理受累的内脏移植的 FAP 患者，MMVTx 是一种有价值的治疗选择。SPPD 技术可行，应始终努力保留固有脾脏，因为本文报告了治疗优势。
