Introduction: Intestinal transplantation (IT) remains a challenging procedure. The long term outcome, especially if the graft has been removed, is not well known. We report on clinical and pathological features with and without graft at 10 years or more after IT to discuss possible improvements.
Methods: Of 71 children transplanted between 1989 and 2007, 38 survived more than 10 years after IT: 26 with a functional graft, 9 on home parenteral nutrition (PN), 3 lost to follow-up. The median follow-up was 14.6 years, in our pediatric center, then in Beaujon Hospital for 21 patients. Long term biopsies were available for 21 patients. Data are medians.
Results: Indications of IT were: short bowel syndrome (34%), congenital enteropathy (34%), motility disorder (32%). Age at IT was 4.1 years and at last follow-up 20.1 years. Patient and graft survivals were 53% and 34%. The graft was removed in 12 (34%) for rejection; 5/12 patients were re-transplanted, 3 are well after 9.8-17.4 years follow-up. Five (14%) patients died, 2 transplanted (lymphoma, sepsis), 2 after re-transplantation, one on home PN (sepsis).
The 26 patients with graft were free of PN, 65% had liver-small bowel transplantation. Overall and fat absorptions were 91% and 89%. The height percentile (-0.5 SD) was higher than at IT. The measured renal clearance was 88 ml/min/1.73 m2. Five patients (19%) had a lymphoproliferative syndrome, 42% a graft rejection more than one year post-IT, 42% had DSA (donor specific antibodies) without rejection. On biopsies they had a mild to moderate increase of mononuclear cells in the lamina propria, and of eosinophils for half of them. There was no sign of vascular rejection. 3/7 children had a school delay, 2/19 adults were unemployed, 8 (31%) had psychiatric disorders.
The graft had been removed in 9 patients: all were stable on home PN. On liver biopsy, 2/8 patients had fibrosis ≥ F3, 6 steatosis. Two received GLP-2 with decreased PN needs. 3/4 children had a school delay, 1/5 adults were unemployed, 5 patients (56%) had psychiatric disorders.
Conclusion: IT remains difficult but leads to digestive autonomy and catch-up growth without major complications of immunosuppression. The long-term pathology is reassuring despite the presence of DSA. The prognosis in case of graft removal is acceptable. Re-transplantation carries a significant mortality. Multidisciplinary care with early psychosocial follow-up is essential.
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