The role of gastrointestinal endoscopic workup in double lung transplanted cystic fibrosis patients – A prospective study involving 25 consecutive patients

Background: Cystic fibrosis (CF) patients are at a higher risk of developing digestive tract disorders, especially gastrointestinal (GI) cancer. However, optimal criteria, timing and screening interval for GI pathology is not yet established in this patient population.

Aims/Methods: This prospective study aimed to evaluate the role of gastrointestinal endoscopic workup in transplanted CF patients. Study subjects underwent esophagogastroduodenoscopy and ileocolonoscopy with segmental biopsy assessment for cancer screening, regardless of presence or absence of GI symptoms (group A or group B). The diagnostic yield for clinically relevant findings (defined as those requiring medical intervention or endoscopic surveillance) was compared among groups (Fisher's exact test).

Results: 25 CF patients (13 male, 12 female, median age 35 years) were included. All but one had undergone double lung transplantation. GI symptoms were present in 13 patients (group A), above all, abdominal pain (n = 11), 12 patients had no GI related symptoms (group B). The diagnostic yield was 6/13 in patients with GI symptoms (8 pathologies detected) and 7/12 in patients without GI symptoms (10 pathologies detected; 46 vs. 58%, OR 0.61, 95% CI 0.13 – 2.98; P = 0.70). Findings included four cases of non-dysplastic short-segment Barrett's esophagus, three cases of esophageal candidiasis, three cases of atrophic gastritis with intestinal metaplasia, two cases of erosive gastritis, one case of erosive esophagitis, three cases of colonic adenoma (tubulous: n = 2, tubulovillous: n = 1) and one case each of tubular adenoma of the terminal ileum and post-transplant lymphoproliferative disorder. The median Boston Bowel Preparation Scale was 1+2+3 = 6 (9), including four cases with fecaliths trapped in the appendiceal orifice.

Conclusion: In this prospective study (pre-)malignant GI lesions were common in lung transplanted CF patients, irrespective of the presence of GI symptoms. These data further support the necessity of GI endoscopic screening in these patients. Prior to colonoscopy 2-days of bowel-cleansing is suggested.