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Surgical considerations in cystic fibrosis: a 32-year evaluation of outcomes.
Surgery 2005; 138(4):560-71; discussion 571-2S

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+Show Affiliations

Section of Pediatric Surgery, Department of Surgery, Indiana University School of Medicine, Indianapolis, Indiana, USA.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

16269283

Citation

Escobar, Mauricio A., et al. "Surgical Considerations in Cystic Fibrosis: a 32-year Evaluation of Outcomes." Surgery, vol. 138, no. 4, 2005, pp. 560-71; discussion 571-2.
Escobar MA, Grosfeld JL, Burdick JJ, et al. Surgical considerations in cystic fibrosis: a 32-year evaluation of outcomes. Surgery. 2005;138(4):560-71; discussion 571-2.
Escobar, M. A., Grosfeld, J. L., Burdick, J. J., Powell, R. L., Jay, C. L., Wait, A. D., ... Rescorla, F. J. (2005). Surgical considerations in cystic fibrosis: a 32-year evaluation of outcomes. Surgery, 138(4), pp. 560-71; discussion 571-2.
Escobar MA, et al. Surgical Considerations in Cystic Fibrosis: a 32-year Evaluation of Outcomes. Surgery. 2005;138(4):560-71; discussion 571-2. PubMed PMID: 16269283.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Surgical considerations in cystic fibrosis: a 32-year evaluation of outcomes. AU - Escobar,Mauricio A, AU - Grosfeld,Jay L, AU - Burdick,Justin J, AU - Powell,Robert L, AU - Jay,Colleen L, AU - Wait,Alyssa D, AU - West,Karen W, AU - Billmire,Deborah F, AU - Scherer,L R,3rd AU - Engum,Scott A, AU - Rouse,Thomas M, AU - Ladd,Alan P, AU - Rescorla,Frederick J, PY - 2005/02/09/received PY - 2005/06/09/revised PY - 2005/06/12/accepted PY - 2005/11/5/pubmed PY - 2005/12/13/medline PY - 2005/11/5/entrez SP - 560-71; discussion 571-2 JF - Surgery JO - Surgery VL - 138 IS - 4 N2 - 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. SN - 0039-6060 UR - https://www.unboundmedicine.com/medline/citation/16269283/Surgical_considerations_in_cystic_fibrosis:_a_32_year_evaluation_of_outcomes_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0039-6060(05)00425-3 DB - PRIME DP - Unbound Medicine ER -