Introduction: Recent improvements in intestinal rehabilitation programs allowed avoiding intestinal transplantation in many cases. Conversely, improved outcomes prompt the use of intestinal transplantation for complex intestinal pathologies not included in the classical list of indications. Our aim was to analyze our experience in some of these unusual indications for transplantation and to review their outcome when compared to the usual indications.
Methods: Retrospective review of our historical series of intestinal and multivisceral transplants (1999-2018) identifying those cases in which the initial diagnosis was not among the classic indications (short gut syndrome, motility disorders, untreatable diarrheas). Their general outcome (complications, immunosuppression, graft loss, survival) were compared with the rest of the cases, considering statistically significant a P value under 0.05.
Results: We reviewed 107 transplants, finding 9 unusual cases transplanted with an average age of 4 years (1-10). Their initial diagnoses were: 3 benign cystic retroperitoneal teratomas with SMA lesion during surgery, 1 myofibroblastic tumor involving main vessels, 1 multifocal postext IV hepatoblastoma with tumoral portal thrombus reaching beyond the splenic mesenteric junction, 1 vascular accident in an appendectomy, 1 Martínez-Frias syndrome, 1 mitochondrial disease and 1 Alagille syndrome with ileal atresia. Eight patients received a multivisceral graft and 1 an isolated small bowel. Surgical and infectious complications did not shown statistical differences with our main series. PTLD was only seen in 11% (1) and GVHD in 22% (2), being less common than in the main series but not statistically significant. The rate of rejection and retransplantation was also similar. Only the case who had an isolated small bowel graft presented rejection (11%) requiring multivisceral retransplantation. Overall patient survival was 82% with a mean follow-up of 7 years [0-13], with no differences between the groups. No tumor recurrence was observed.
Conclusion: Multivisceral transplant is a safe therapeutic option in unconventional and challenging cases in which the integrity of the intestine is affected. According to our study, these transplants present the same prognosis as the classic indications. Emerging indications, like the 9 cases reported in this work, would appeared in the future. Intestinal transplantation should be at least considered to treat these patients attended in IRUs.