Literature DB >> 24528967

Gastrointestinal surgery in cystic fibrosis: a 20-year review.

Paul J Farrelly1, Caroline Charlesworth1, Sophie Lee1, Kevin W Southern2, Colin T Baillie3.   

Abstract

OBJECTIVES: The purpose of this study was to evaluate outcomes of the surgical management for meconium ileus (MI) and Distal Intestinal Obstruction Syndrome (DIOS) in Cystic Fibrosis (CF).
METHODS: Children born between 1990 and 2010 were identified using a regional CF database. Retrospective case note analysis was performed. Outcome measures for MI were mortality, relaparotomy rate, length of stay (LOS), time on parental nutrition (TP), and time to full feeds (TFF). Outcome measures for DIOS were: age of onset, number of episodes, and need for laparotomy.
RESULTS: Seventy-five of 376 neonates presented with MI. Fifty-four (92%) required laparotomy. Contrast enema decompression was attempted in nineteen. There were no post-operative deaths. Thirty-nine (72%) neonates with MI were managed with stomas. LOS was longer in those managed with stomas (p=0.001) and in complex MI (p=0.002). Thirty-five patients were treated for DIOS. Twenty-five patients were managed with gastrograffin. Ten patients underwent surgical management of DIOS. Overall, MI did not predispose to later development of DIOS. There was a significantly greater incidence of laparotomy for DIOS in children who had MI.
CONCLUSION: The proportion of neonates with complex meconium ileus was high (49%) and may explain the infrequent utilisation of radiological decompression. Complex MI or management with stomas both significantly increase LOS. Re-laparotomy rate is high (22%) in MI irrespective of the type of management. DIOS is not a benign condition, particularly when the child has had previous abdominal surgery. Early referral to a surgical team is essential in these children.
Copyright © 2014 Elsevier Inc. All rights reserved.

Entities:  

Keywords:  Cystic fibrosis; Distal intestinal obstruction syndrome; Meconium ileus; Surgery

Mesh:

Substances:

Year:  2013        PMID: 24528967     DOI: 10.1016/j.jpedsurg.2013.11.038

Source DB:  PubMed          Journal:  J Pediatr Surg        ISSN: 0022-3468            Impact factor:   2.545


  7 in total

Review 1.  Beyond pancreatic insufficiency and liver disease in cystic fibrosis.

Authors:  Stephanie Demeyer; Kris De Boeck; Peter Witters; Katrien Cosaert
Journal:  Eur J Pediatr       Date:  2016-04-07       Impact factor: 3.183

Review 2.  Lung transplantation for cystic fibrosis: results, indications, complications, and controversies.

Authors:  Joseph P Lynch; David M Sayah; John A Belperio; S Sam Weigt
Journal:  Semin Respir Crit Care Med       Date:  2015-03-31       Impact factor: 3.119

3.  Altered Stool Microbiota of Infants with Cystic Fibrosis Shows a Reduction in Genera Associated with Immune Programming from Birth.

Authors:  Katherine M Antosca; Diana A Chernikova; Courtney E Price; Kathryn L Ruoff; Kewei Li; Margaret F Guill; Natalie R Sontag; Hilary G Morrison; Shuyu Hao; Mitchell L Drumm; Todd A MacKenzie; Dana B Dorman; Lynn M Feenan; Molly A Williams; John Dessaint; Irene H Yuan; Brian J Aldrich; Lisa A Moulton; Lily Ting; Ana Martinez-Del Campo; Edward J Stewart; Margaret R Karagas; George A O'Toole; Juliette C Madan
Journal:  J Bacteriol       Date:  2019-07-24       Impact factor: 3.490

4.  Intraoperative intraluminal injection of N-acetylcysteine allowing treatment of distal intestinal obstruction syndrome without the need for enterotomy.

Authors:  Nicholas Js Chilvers; James Wheeler
Journal:  BMJ Case Rep       Date:  2018-05-16

Review 5.  Animal models of gastrointestinal and liver diseases. Animal models of cystic fibrosis: gastrointestinal, pancreatic, and hepatobiliary disease and pathophysiology.

Authors:  Alicia K Olivier; Katherine N Gibson-Corley; David K Meyerholz
Journal:  Am J Physiol Gastrointest Liver Physiol       Date:  2015-01-15       Impact factor: 4.052

Review 6.  Pancreatic pathophysiology in cystic fibrosis.

Authors:  Katherine N Gibson-Corley; David K Meyerholz; John F Engelhardt
Journal:  J Pathol       Date:  2015-10-01       Impact factor: 7.996

Review 7.  Meconium Ileus.

Authors:  John H T Waldhausen; Morgan Richards
Journal:  Clin Colon Rectal Surg       Date:  2018-02-25
  7 in total

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