title: Surgical considerations in cystic fibrosis: a 32-year evaluation of outcomes.
abstract: BACKGROUND
  Information concerning long-term operative outcomes in patients with cystic fibrosis (CF) is relatively sparse in the operative literature.
  
  
  METHODS
  A retrospective review of CF patients with operative conditions was performed (1972-2004) at a tertiary children's hospital to analyze outcomes including long-term morbidity and survival.
  
  
  RESULTS
  A total of 226 patients with CF presented with an operative diagnosis (113 men, 113 women). A total of 422 operations were performed in 213 patients (94%). The mean age at operation was 4.1 +/- 6.2 years (range, 1 d to 26 y) and 109 were neonates. Fifteen of 42 (36%) babies with simple meconium ileus (MI) were treated nonoperatively with hypertonic enemas, 27 of 42 and all 45 patients with complicated MI required operation, including 15 with jejunoileal atresia (17%). Seventeen of 27 (63%) patients with meconium ileus equivalent had MI as neonates; 7 of 27 (26%) required operation. Eight of 9 (89%) with fibrosing colonopathy required operation. Organ transplantation was required in 21 patients. Follow-up evaluation was possible in 204 of 213 (96%) patients. The duration of follow-up evaluation was 14.9 +/- 8.5 years (range, 2 mo to 35 y). Operative morbidity was 11% at 1 year, 2% at 2 to 4 years, 1% at 5 to 10 years, and less than 1% at more than 10 years. There were 24 deaths (11%); 22 followed CF-related pulmonary complications and included 8 of 16 (50%) children with pneumothorax.
  
  
  CONCLUSIONS
  Long-term survival in CF patients has improved significantly (89%), with many surviving into the fourth decade. MI may predispose to late complications including meconium ileus equivalent and fibrosing colonopathy. Pneumothorax in CF patients is an ominous predictor of mortality. Children with CF are living longer and are good candidates for operation, but require long-term follow-up evaluation because of ongoing exocrine dysfunction.
authors.affiliation: Section of Pediatric Surgery, Department of Surgery, Indiana University School of Medicine, Indianapolis, Indiana, USA.
authors.forename: Mauricio A
authors.initials: MA
authors.lastname: Escobar
authors.forename: Jay L
authors.initials: JL
authors.lastname: Grosfeld
authors.forename: Justin J
authors.initials: JJ
authors.lastname: Burdick
authors.forename: Robert L
authors.initials: RL
authors.lastname: Powell
authors.forename: Colleen L
authors.initials: CL
authors.lastname: Jay
authors.forename: Alyssa D
authors.initials: AD
authors.lastname: Wait
authors.forename: Karen W
authors.initials: KW
authors.lastname: West
authors.forename: Deborah F
authors.initials: DF
authors.lastname: Billmire
authors.forename: L R
authors.initials: LR
authors.lastname: Scherer
authors.forename: Scott A
authors.initials: SA
authors.lastname: Engum
authors.forename: Thomas M
authors.initials: TM
authors.lastname: Rouse
authors.forename: Alan P
authors.initials: AP
authors.lastname: Ladd
authors.forename: Frederick J
authors.initials: FJ
authors.lastname: Rescorla
mesh_terms: D000005:Abdomen / Q000601:surgery; D000293:Adolescent; D000328:Adult; D001649:Bile Duct Diseases / Q000209:etiology / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000150:complications* / Q000378:metabolism / Q000401:mortality; D005260:Female; D006801:Humans; D045823:Ileus / Q000209:etiology / Q000601:surgery*; D007223:Infant; D007231:Infant, Newborn; D007410:Intestinal Diseases / Q000209:etiology / Q000601:surgery*; D007443:Intussusception / Q000209:etiology / Q000601:surgery; D008107:Liver Diseases / Q000209:etiology / Q000601:surgery*; D008297:Male; D008470:Meconium / Q000378:metabolism; D011030:Pneumothorax / Q000209:etiology / Q000601:surgery*; D011183:Postoperative Complications; D012189:Retrospective Studies; D016019:Survival Analysis; D019616:Thoracic Surgical Procedures / Q000009:adverse effects; D016896:Treatment Outcome