Intestinal transplantation in children: a 18-year single center experience

Sabine Irtan¹, Christophe Chardot¹, Laurent Dupic², Yann Revillon¹, Frédérique Sauvat¹, Yves Aigrain¹, Caroline Telion³, Olivier Goulet¹, Florence Lacaille¹

¹Pediatric Surgery, Necker Enfants Malades Hospital, Paris, France; ²Intensive Care Unit, Necker Enfants Malades Hospital, Paris, France; ³Anesthesiology, Necker Enfants Malades, Paris, France

Aim : To describe medium and long term results of intestinal transplantation (Tx) in children, in order to discuss the indications,  possible improvements, and management.

Patients and methods : From 1994 on, 107 transplantations were performed on 99 children: 59 isolated small bowel, 43 combined liver-small bowel, 4 multivisceral (3 with kidney), 1 modified multivisceral. Indications were short bowel syndrome (n=35), motility disorders (n=28), mucosal defects (n=33), re-transplantation (n=9) and others (n=2). Median follow-up was 8 years [4 months – 17 years].

Results: 53 patients are alive, with a follow-up of 1 to 18.5 years, 40 with a functional graft (1 with a partial parenteral nutrition) including 3 after re-transplantation (1 performed in the adult unit of Beaujon). Overall actuarial patient and graft survival at 10 years is respectively 52% and 33%. Patient and graft survival with a graft including the liver is 49% for both, but respectively 52% and 17% without the liver. The mortality was 42%, at a median of 3 months post-Tx [0-130]: main causes were sepsis (24%) and multi-organ failure (22%). Death more than 5 years post-Tx (10 patients) was due to re-Tx (5), lymphoma (1), chronic rejection and liver failure (2), cardiac arrest (1: hypokaliemia ?), accident (1). Median delay of graft loss was 9 months [0-115], mainly from rejection, either acute (23%) or chronic (35%). Graft loss more than 5 years post-Tx (4 grafts) was due to infection and acute rejection (1) or chronic rejection (3). All 16 patients with a combined liver-small bowel graft for more than 10 years (including 8 more than 15 years) have a functional graft, whereas 75% of isolated small bowel transplants were lost (only 1 patient with a functional graft, at 15 years). 

Conclusion : Like in the Registry, the mortality was high, and the patient and graft survival on the medium and long term is unsatisfactory, although better with grafts including the liver. Continuous improvements are strongly needed in order to decrease rejection-related graft loss and later risk during re-Tx, and infection-related death.