Intestinal transplantation for patients with short gut syndrome and hypercoagulable states.

Between May 2.1990 and August 18. 1999. a total of DO patients received primary intestinal transplantation at our center. Fifty-five were adults and splanchnic vascular thrombosis was the cause of loss of the native intestine in 17 (31%). Of these 17 patients. 8 (47%) received isolated intestine. and 9 (53%) received composite visceral grafts that contained liver (5 liveriintestine. 4 muItivisceral). Twelve (71 %) recipients were male and five (29%) were female with a mean age of 37 :!: 13 years. The preoperative work-up included a full hypercoagulable laboratory profile and visceral angiography for all patients. The thrombosis was arterial in 12 (70.5%). venous (portomesenteric) in 4 (23.5%). and combined in the remaining case (6%). Concomitant extensive central venous thrombosis was documented preoperatively in 8 (47%) patients. The etiology of vascular thrombosis was hypercoagulable state in 9 (53%). TIP in I (5.8%), myeloproliferative disorder in I (5.8%). and undetermined in the remaining 6 (35%) patients. The hypercoagulable syndrome was due to isolated/combined deficiency of proteins C and S and antithrombin III in all 9 patients, with concomitant factor V mutation in 2 cases. Composite visceral grafts were given only to patients with combined liver and intestinal failure and/or total vascular occlusion of the splanchnic circulation. Anticoagulant therapy was continued postoperatively as a lifelong treatment for all patients with superior vena cava syndrome and those who were hypercoagulable and received isolated intestinal transplantation. The donor characteristics. full details of donor/ recipient operations. and perioperative management. including immunosuppressive protocol. have been described e1sewhere. I 3