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Gastrointestinal surgery in cystic fibrosis: a 20-year review.
J Pediatr Surg. 2014 Feb; 49(2):280-3.JP

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.

Authors+Show Affiliations

Department of Paediatric Surgery, Alder Hey Children's NHS Foundation Trust, West Derby, Liverpool, L12 2AP, United Kingdom.Department of Paediatric Surgery, Alder Hey Children's NHS Foundation Trust, West Derby, Liverpool, L12 2AP, United Kingdom.Department of Paediatric Surgery, Alder Hey Children's NHS Foundation Trust, West Derby, Liverpool, L12 2AP, United Kingdom.Institute of Child Health, Alder Hey Children's NHS Foundation Trust, West Derby, Liverpool, L12 2AP, United Kingdom.Department of Paediatric Surgery, Alder Hey Children's NHS Foundation Trust, West Derby, Liverpool, L12 2AP, United Kingdom. Electronic address: colin.baillie@alderhey.nhs.uk.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

24528967

Citation

Farrelly, Paul J., et al. "Gastrointestinal Surgery in Cystic Fibrosis: a 20-year Review." Journal of Pediatric Surgery, vol. 49, no. 2, 2014, pp. 280-3.
Farrelly PJ, Charlesworth C, Lee S, et al. Gastrointestinal surgery in cystic fibrosis: a 20-year review. J Pediatr Surg. 2014;49(2):280-3.
Farrelly, P. J., Charlesworth, C., Lee, S., Southern, K. W., & Baillie, C. T. (2014). Gastrointestinal surgery in cystic fibrosis: a 20-year review. Journal of Pediatric Surgery, 49(2), 280-3. https://doi.org/10.1016/j.jpedsurg.2013.11.038
Farrelly PJ, et al. Gastrointestinal Surgery in Cystic Fibrosis: a 20-year Review. J Pediatr Surg. 2014;49(2):280-3. PubMed PMID: 24528967.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Gastrointestinal surgery in cystic fibrosis: a 20-year review. AU - Farrelly,Paul J, AU - Charlesworth,Caroline, AU - Lee,Sophie, AU - Southern,Kevin W, AU - Baillie,Colin T, Y1 - 2013/11/18/ PY - 2013/10/31/received PY - 2013/11/10/accepted PY - 2014/2/18/entrez PY - 2014/2/18/pubmed PY - 2014/11/7/medline KW - Cystic fibrosis KW - Distal intestinal obstruction syndrome KW - Meconium ileus KW - Surgery SP - 280 EP - 3 JF - Journal of pediatric surgery JO - J. Pediatr. Surg. VL - 49 IS - 2 N2 - 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. SN - 1531-5037 UR - https://www.unboundmedicine.com/medline/citation/24528967/Gastrointestinal_surgery_in_cystic_fibrosis:_a_20_year_review_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0022-3468(13)00898-1 DB - PRIME DP - Unbound Medicine ER -