Surgical experience in patients with cystic fibrosis: a 25-year perspective

The spectrum of surgical diseases in patients with cystic fibrosis (CF) has not been comprehensively studied. A retrospective review of 792 consecutive patients with CF presenting over a 25 year period (1970-1994) was made to determine the incidence of operations, procedures performed, complications encountered, and impact on physical development and pulmonary function tests (PFTs). A total of 191 operations were performed on 130 (16%) of the 792 patients; 98 operations (51%) were abdominal, 58 (30%) thoracic, and 31 (16%) hernias; 64 were male, and 66 female; average age was 14 +/- 10 years. Complications occurred in 12 (16%); 9 deaths were from progressive respiratory failure, 2 from superficial wound infections, and 1 from an episode of line sepsis. In the first 15 years, 9 complications occurred in 126 operations vs. 3 in 73 operations during the last 10 years. Operations were classified as emergent, urgent, or elective. Of the 9 deaths, 8 occurred after emergent or urgent operations (4 abdominal and 4 thoracic), while 1 death occurred following elective herniorrhaphy. For each subgroup, (abdominal, thoracic, and hernia), there was no difference in height/weight indicies, peak flow, forced vital capacity (FVC), forced expired volume in 1 sec (FEV(1)), or FEV(1)/FVC ratio when comparing 1 year preoperation and 1 year postoperation. In conclusion, patients in this high-risk population were operated on with few complications, but when a complication occurred it tended to be pulmonary and fatal (4.7% of all operations). Furthermore, operations did not cause significant deteriorations in PFTs and they did not cause these children to fall off their expected age-adjusted growth curves.