Background: Cystic fibrosis (CF) is the most common inherited life threatening disease in Caucasians. Median life expectancy for CF patients has increased to over 35 years as a result of improvements in medical care including lung transplantation. With increased longevity, more patients with CF are developing gastrointestinal complications including the distal intestinal obstruction syndrome (DIOS).
Methods: The medical records of all patients with CF who underwent lung transplantation at this institution during the 15 year period from August 1989 to August 2004 were reviewed. DIOS was defined as being present in patients with both clinical and radiologic features of distal small bowel obstruction with faecal loading of the caecum and terminal ileum.
Results: 121 patients with CF underwent bilateral sequential lung transplantation during the study period. During a minimum two year follow-up there were 17 episodes of DIOS in 13 (10.7%) patients. The incidence of DIOS in patients who had undergone pre-transplant abdominal operation (6 of 22 patients) was significantly higher than in patients with no previous operation (7 of 99 patients, P=0.013) and was even higher in patients with a history of both meconium ileus in infancy and prior abdominal operation (6 of 15 versus 7 of 106 patients, P=0.001, both Fisher's exact test). Six patients developed DIOS during the transplantation admission. All six had had meconium ileus during infancy and five had undergone pre-transplant laparotomy for CF complications. The development of DIOS was unrelated to age at transplant, sex, need for pancreatic enzyme supplementation, or season. First-line treatment for all patients was medical by a combination of laxatives, stool softeners, bowel preparation formulas, and polyoxyethylene sorbitan monooleate (Tween 80). Medical therapy was successful in 14 of the 17 DIOS episodes after between 1 and 14 days. Four laparotomies were required in 3 patients because of failure of medical therapy. Laparotomy with enterotomy and faecal disimpaction provided definitive resolution of DIOS except in one patient who presented late and died after ileal decompression and ileostomy.
Conclusions: DIOS is a highly morbid condition that develops in approximately ten per cent of patients with CF after lung transplantation. Patients with a history of meconium ileus and/or laparotomy are at very high risk of developing DIOS. These patients should receive prehydration and bowel preparation immediately prior to transplantation. Patients with DIOS require early aggressive management including laparotomy with enterotomy and stoma formation in severe cases.