Intestinal transplantation for total/near-total aganglionosis and intestinal pseudo-obstruction

Whether from anatomical short gut (such as after resection of extensive intestinal aganglionosis) or from a functional cause (such as intestinal pseudoobstruction), intestinal failure is a devastating disease process with profound morbidity and mortality. These patients require total parenteral nutrition (TPN) and are at risk of developing complications such as liver failure, catheter-related sepsis and loss of venous access. Intestinal transplantation, which has advanced markedly over the last 14 years, is now the accepted standard of care for patients failing TPN. Survival outcomes have improved significantly, infectious complications are better controlled, and new immunosuppressive therapies offer great hope for the future. In particular, the results of intestinal transplantation achieved with the motility disorders are equivalent to those experienced with other causes of intestinal failure. In themselves, the motility disorders present their own set of complicating factors, including determining the extent of the disease process (which may involve any part of the gastrointestinal tract), associated urological anomalies, and the type of organ transplantation required. Extensive workup and careful consideration is required before transplantation is undertaken. However, early referral is desirable once complications arise if these patients are to be offered optimal medical care before the chance of transplantation is lost.