title: Surgical experience in patients with cystic fibrosis: a 25-year perspective.
abstract: 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.
authors.affiliation: Department of Surgery, University of Minnesota, Minneapolis, Minnesota 55455, USA. saltzooztec.umn.edu
authors.forename: Daniel A
authors.initials: DA
authors.lastname: Saltzman
authors.forename: Eric M
authors.initials: EM
authors.lastname: Johnson
authors.forename: Brad A
authors.initials: BA
authors.lastname: Feltis
authors.forename: Catherine
authors.initials: C
authors.lastname: Wilienski
authors.forename: Warren J
authors.initials: WJ
authors.lastname: Warwick
authors.forename: Arnold S
authors.initials: AS
authors.lastname: Leonard
mesh_terms: D000005:Abdomen / Q000601:surgery; D017677:Age Distribution; D003550:Cystic Fibrosis / Q000150:complications / Q000503:physiopathology / Q000601:surgery*; D005260:Female; D059685:Herniorrhaphy; D006801:Humans; D008297:Male; D011183:Postoperative Complications / Q000453:epidemiology; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D012307:Risk Factors; D013514:Surgical Procedures, Operative / Q000379:methods / Q000706:statistics & numerical data*; D019616:Thoracic Surgical Procedures; D013997:Time Factors; D016896:Treatment Outcome