Isolated intestinal transplantation is the proper choice for patients with intestinal failure without liver cirrhosis. Mild-to-moderate liver dysfunction with periportal hepatic fibrosis is not contraindication for isolated intestinal transplant particularly in patients without synthetic or vascular decompensation. Isolated intestinal graft has been more frequently used with a higher incidence in adults (55%) than children (37%) [2]. This could be partly due to the greater need for a combined liver-intestinal transplant in children as a result of a higher incidence of end-stage liver disease associated with total parenteral nutrition in this age group.

The indications for this type of transplant can be collectively divided into short bowel syndrome, motility disorders, malabsorption syndromes, and gastrointestinal neoplastic disorders. In patients with concomitant pancreatic insufficiency and intestinal failure, such as patients with cystic fibrosis, chronic pancreatitis, or diabetes mellitus, an en bloc intestine and pancreas transplant may be considered [10].

The combined liver-intestine transplantation is usually indicated for patients with intestinal failure who developed end-stage liver disease due to long-term parenteral nutrition [10, 11]. The procedure may also be indicated for patients with liver failure combined with portomesenteric venous thrombosis when isolated liver transplantation is not technically feasible.

The organs can be transplanted in a simultaneous or consequent fashion. The en bloc allograft includes the pancreaticoduodenal axis along with liver and small bowel to maintain continuity of gastrointestinal tract and integrity of axial blood supply. Pediatric or small candidates requiring combined liver and intestinal transplants may benefit from a “reduced-size liver”—small bowel graft [12] which may include left, right, or extended right lobes of the liver (Fig. 39.2).

In 2009, the senior author [3] proposed and implemented a “domino transplant procedure ” in which patients with recurrent chronic rejection after isolated intestinal transplantation would receive a combined liver-intestine graft from the same donor even if they have a fully functioning native liver. The recipient native liver will be given to another candidate of liver-only transplant.

Full or modified multivisceral transplantation comprises nearly 24 % of adult and 13 % of pediatric intestinal transplants [10, 13]. It is indicated for patients with complex abdominal pathology including massive gastrointestinal polyposis, traumatic loss of the abdominal viscera, extensive abdominal desmoid tumors, locally aggressive non-metastasizing neoplasms, advanced generalized hollow visceral myopathy/neuropathy, and complete thrombosis of the splanchnic arterial or portomesenteric venous systems with hepatic decompensation [3].

From an immunological standpoint, multivisceral or combined liver-intestine transplant may have an advantage over isolated intestine transplant. The achieved better long-term engraftment with liver-contained graft compared to liver-free graft was reported by the senior author [3]. The improved outcome is mostly related to the immunoprotective effect of the concomitantly transplanted liver. This observation can be partially explained by the recently published data showing that liver-contained allografts were associated with significant clearance of preformed alloantibody and low induction of de novo donor-specific antibodies along with better survival in liver-contained allografts [14]. The study also demonstrated the important role of alloantibody in chronic visceral allograft injury and the liver can be immunoprotective with less favorable outcome in recipients with persistent alloantibodies.

Optimal donor selection is imperative to successful transplant outcome in intestine-contained transplantation. Prolonged downtime and the requirement for high-dose or multiple inotropes compromise the quality of visceral grafts. Other important factors include size disparity especially for recipients who lost the abdominal domain or large component of the abdominal wall. Allograft reduction in conjunction with efforts to increase abdominal domain including abdominal wall transplant and pre-transplant implementation of tissue expander in subcutaneous layer have been performed to facilitate graft coverage with newly created abdominal wall [12, 15, 16]. It is imperative to obtain arterial and venous vascular segments from the same donor to facilitate visceral allograft implantation. Accordingly, prompt inititation of communication with other abdominal organ-sharing programs is essential to facilitate smooth retrieval.

With an increase in the gap between organ donation and demand, a procurement procedure is needed that permits multiple-organ retrievals for separate recipients waiting for liver, pancreas, and intestinal allografts [4]. A recent advance in organ retrieval technique made it feasible to share these organs among three different recipients (Fig. 39.3). When multivisceral transplantation is required for patients with preserved liver function, modification of the technique made it possible to utilize the donor liver for one recipient and the remaining visceral organs to a second patient (Fig. 39.4). The term “modified’ multivisceral transplantation was first introduced in 1993 and recently published by the senior author [17–19].

Upon entering the abdominal cavity, the intestine should be carefully examined. Thin mesentery with less adipose tissue is preferable because fat component is susceptible to ischemic-reperfusion injury, resulting in fat necrosis and subsequent mesenteric sclerosis after transplantation. Intestine with pneumatosis or portal venous gas is unacceptable for transplant. Iliac arteries and veins are accessed for the suitability for interposition grafts. Once quality of organ(s) is found to be satisfactory for transplant, direct communication between donor and recipient team prompts the recipient operation to minimize the cold ischemic time.

The first step of organ retrieval is performing the organ dissection with Cattel maneuver to mobilize small bowel and right colon that facilitates exposure of the vena cava and aorta [20, 21]. The left renal vein is identified with isolation of the SMA origin. Then the abdominal aorta is encircled distally for the eventual insertion of an infusion cannula. The supraceliac aorta is also encircled for later cross clamp.

After the colon and intestine are mobilized from retroperitoneum, the ileum is divided with the gastrointestinal stapler near the ileocecal valve when donor colon is not procured with the visceral organs. Right and transverse colons are detached from ileocolic vessels by taking down right and middle colic vessels. The remaining steps of the procedure are dictated by the type of required visceral allograft.

In preparation for visceral transplantation, a first stage surgical exploration has been increasingly utilized. The primary purpose of the approach is to eradicate intra-abdominal infections by surgical methods including debridgement, repair of fistulae, and restoration of gastrointestinal continuity. Upon referral, these patients often have intra-abdominal infection with enteric leak, abscesses, enterocutaneous fistulas, infected foreign materials including ventral hernia mesh, and venting tube drainage with colonized multidrug-resistant organisms. Because of the need for heavy maintenance immunosuppression after visceral transplant, successful treatment of these infections is necessary for successful outcome.

Another valuable purpose of the initial surgical exploration is to restore gastrointestinal continuity. Foregut and midgut reconstruction, particulary in patients with prior bariatric surgery, reduces the need for composite visceral allograft by salvaging the native stomach and may eliminate the need for isolated intestinal transplantation in selected cases after successful rehabilitative surgery including bowel lengthening.

When such an ambitious goal of achieving natural autonomy is not reached, restoration of upper gastrointestinal continuity temporally improves quality of life and more importantly reduce the number of required visceral organs with the need in most cases for intestine-only allograft (Fig. 39.5). Accordingly, the pancreatic gland from the same donor can be retrieved and utilized for another recipient. Another important advantage of the technique is utilizing the native conduit as an end stoma in patients who require allograft enterectomy due to graft failure.