Children with intestinal failure (IF) eventually develop progressive liver failure, known as IF-associated liver disease (IFALD).1 Combined liver-intestine transplantation (Tx) can be indicated for such children, although a suitable size-matching pediatric donor is rarely found.
A 20-month-old boy weighing 6.7 kg who had developed short bowel syndrome due to necrotizing enterocolitis was referred to our institute. The length of his remaining intestine was 18 cm without the ileocecal valve. The laboratory findings revealed hyperbilirubinemia and hepatic synthetic dysfunction with mild coagulopathy. Enhanced abdominal computed tomography revealed splenomegaly and collateral development. The patient’s Pediatric End-stage Liver Disease score was 15, and the liver damage was classified as Child-Pugh grade B. He was placed on the waiting list for a deceased donor intestine Tx; however, Japan’s national organ allocation system does not grant medical priority for obtaining a liver to patients with IFALD (Figure S1, SDC, http://links.lww.com/TP/B779). Thus, we evaluated his father as a potential living donor for living donor liver Tx (LDLT), while considering whether to perform LDLT at the same time as deceased donor intestine Tx or whether to first perform LDLT and then wait for an intestinal graft to become available from a suitable size-matched pediatric donor, as long as his liver graft function was considered tolerable. Before finishing the preparation of his LDLT donor, a no liver-inclusion intestinal graft from a size-matched pediatric donor weighing 10 kg became available. He underwent isolated intestine Tx with an intestinal graft of 200 cm in length; however, there was some difficulty in achieving complete hemostasis was experienced during the operation (Figure 1A). The venous drainage of the intestinal graft was reconstructed to the infrarenal inferior vena cava. The initial immunosuppression protocol consisted of anti-thymocyte immunoglobulin, intravenous tacrolimus, and methylprednisolone. His immediate postoperative course showed an acceptable initial function of the intestinal graft. However, transfusion continued to be required to maintain intravascular volume due to hepatic synthetic dysfunction. Then, the portal vein (PV) flow gradually began to decrease and ultimately showed a hepatofugal flow 1 week later. Accordingly, he underwent LDLT using a left lateral segmental graft of 210 g in weight from his 28-year-old father 12 days after intestine Tx (Figure 1B). Flow cytometry crossmatching was negative before LDLT. During the LDLT procedure, hepatic vein reconstruction was performed by partial clamping of the inferior vena cava with meticulous care to prevent congestion of the intestinal graft. Sufficient PV flow could be obtained by the complete interruption of the collateral vessels, and thus PV reconstruction was performed using the native PV without any correction of the venous drainage of the intestinal graft. Hepatico-duodenectomy was performed as biliary drainage. The explanted liver showed severe cirrhosis. He experienced 2 episodes of T cell-mediated rejection, necessitating anti-thymocyte immunoglobulin rescue therapy. A donor-specific antigen test was negative at 6 months after intestine Tx. His current intestinal and hepatic graft functions are doing well 1 year after intestine Tx. Total parenteral nutrition was completely withdrawn, and full enteral feeding has been established. His current immunosuppression protocol consists of tacrolimus, everolimus, and prednisolone, which are administered orally. The clinical course of the patient is summarized in Figure 1C.
The type of Tx selected for the patients with IFALD depends on the severity of liver damage and potential for intestinal rehabilitation if severe IFALD can be eliminated by liver Tx.2 Sequential liver-intestine Tx has been previously described by other transplant centers,3-5 with a “Liver-First” approach always selected. Combined living donor liver-intestine Tx was another option; however, the donor safety was prioritized at the time. Before we had finished evaluating the patient’s father as a living donor of liver Tx, the patient was able to obtain an intestinal graft from such a perfectly size-matched pediatric donor, so we first performed isolated intestine Tx. As expected, liver replacement was needed immediately after intestinal Tx. If the living donor for LDLT had been completely prepared in this case, simultaneous hybrid liver-intestine Tx might have been a better option than the approach that was undertaken in the present case.
The organ allocation system in Japan should be changed to allow patients with IFALD to obtain a liver organ simultaneously. However, until that is achieved, sequential intestine Tx with a graft from a deceased donor followed by liver Tx with a graft from a living donor, known as hybrid intestine-liver Tx, may be considered a viable alternative as a life-saving rescue procedure for children with IFALD.
1. Kelly DA. Intestinal failure-associated liver disease: what do we know today? Gastroenterology. 2006; 1302 Suppl 1S70–S77
2. Hawksworth JS, Desai CS, Khan KM, et al. Visceral transplantation in patients with intestinal-failure associated liver disease: evolving indications, graft selection, and outcomes. Am J Transplant. 2018; 18:1312–1320
3. Muiesan P, Dhawan A, Novelli M, et al. Isolated liver transplant and sequential small bowel transplantation for intestinal failure and related liver disease in children. Transplantation. 2000; 69:2323–2326
4. Testa G, Holterman M, Abcarian H, et al. Simultaneous or sequential combined living donor-intestine transplantation in children. Transplantation. 2008; 85:713–717
5. Nassar A, Hashimoto K, Shay-Downer C, et al. Sequential split liver followed by isolated intestinal transplant: the “liver-first” approach: report of a case. Transplantation. 2014; 97:e17–e19