Although isolated liver transplantation and pancreas transplantation have become standard procedures for many patients with end-stage liver disease or insulin-dependent diabetes mellitus (IDDM), combined liver and pancreas transplantation is an extremely rare condition, and only a few reports are described in the literature.
During the embryological development of the abdominal organs, the liver and pancreas begin as ventral and dorsal diverticula from that portion of the foregut that later becomes the duodenum. Starzl and colleagues in the early 1990s thought that the radical excision of the foregut might allow complete removal of the primary tumor and of the metastases to the near organs.1 These operations consisted of an upper abdominal exenteration followed by a “cluster” transplantation, including en bloc the liver, the stomach, and the pancreas due to otherwise nonresectable abdominal malignancies such as neuroendocrine tumors, sarcomas, cholangiocarcinomas, or desmoid tumors (Fig. 80-1). This procedure was however abandoned due to the high mortality and poor oncologic results.2
More frequently, combined liver and pancreas transplantation may be indicated for cystic fibrosis (CF). CF is an autosomal recessive condition presenting in early childhood that affects epithelial chloride transports. The ubiquity of these chloride channels throughout the body accounts for the multiorgan involvement, which characterizes this disorder, affecting primarily the lungs, pancreas, gastrointestinal tract, and liver. Although pulmonary disease remains the main cause of mortality, the treatment of the respiratory complications of CF has continued to improve. As a result, life expectancy has increased, and up to 8% of all patients may reach end-stage liver disease, requiring transplantation in selected cases.3 Pancreatic insufficiency is one of the hallmarks of CF and is due to viscous secretions forming plugs and obstructing the pancreatic ductules, leading to destruction of the acinar cell. The resulting IDDM has an incidence reported up to 80% over the age of 25.4 The first multivisceral transplantation described in a patient with CF was a pancreas–liver–kidney transplantation in 1994 for a 21-year-old man with pancreatic insufficiency requiring pancreatic enzymes and insulin, as well as liver cirrhosis and renal failure.5 Thereafter, the number of combined liver and pancreas transplantation has increased.6 Almost all reports outlined the positive effects of the combined transplantation: this benefit not only prevents the long-term complications of diabetes mellitus and improves quality of life but also may prevent pulmonary decline and decrease mortality.7
Liver Cirrhosis and Type 1 Diabetes
Another indication for combined liver and pancreas replacement is in liver transplant candidates who coincidentally suffer from type 1 diabetes. A frequent association is represented by diabetes and primary sclerosing cholangitis (PSC)8 or nonalcoholic steatohepatitis (NASH).9 There are several reasons ...