Early management of meconium ileus in infants with cystic fibrosis: A prospective population cohort study

• Published in: Journal of pediatric surgery Vol. 56; no. 8; pp. 1287 - 1292
• Main Authors: Long, Anna-May, Jones, Ian H, Knight, Marian, McNally, Janet, Saeed, Atif, Lopes, Joana, Garrett-Cox, Robin, Sherwood, William, Besarovic, Sanja, Jones, Ceri, Owusu-Ankomah, Rachel, Eradi, Bala, Jones, Abigail, Walker, Gregor, Zaman, Azrina, Craigie, Ross, Murthi, Govind V S, Johal, Navroop, Njere, Ike, Mortell, Alan, Shrestha, Ram, Aslam, Adil, Kenny, Simon, Jester, Ingo, Cusick, Eleri, Clarke, Simon, McCabe, Amanda, Kufeji, Dorothy, Gupta, Samir, Johnson, Paul, Patel, Shailesh, Sugarman, Ian D, Rajimwale, Ashok, Tsang, Thomas T M, Motiwale, Sandeep, Driver, Chris, Paramalingam, S, Marshall, David, Charlesworth, Paul, Jaffray, Bruce, Marven, Sean S, Keys, Charlie, Giuliani, Stefano, Mortell, Mr Alan
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01-08-2021
• Subjects: BAPS-CASS; Cystic fibrosis; Meconium ileus; Outcomes; Population-based; Cystic fibrosis; Population-based; BAPS-CASS; Meconium ileus; Outcomes
• ISSN: 0022-3468; 1531-5037
• DOI: 10.1016/j.jpedsurg.2021.02.047

Contemporary early outcome data of meconium Ileus (MI) in cystic fibrosis (CF) are lacking on a population level. We describe these and explore factors associated with successful non-operative management.
A prospective population-cohort study using an established surveillance system (BAPS-CASS) was conducted October 2012–September 2014. Live-born infants with bowel-obstruction from inspissated meconium in the terminal ileum and CF were reported. Data are described as median (interquartile range, IQR).
56 infants were identified. 14/56(25%) had primary laparotomy (13/23 complicated MI, 1/33 simple), the remainder underwent contrast enema. Twelve, (12/33 (36%) with simple MI) achieved decompression. 8/12 (67%) who decompressed had >1 enema vs 3/20 (15%) with simple MI who had laparotomy after enema. The number of enemas per infant (1–4), contrast agents and their concentration, were highly variable. Enterostomy was formed at 24/44(55%) of laparotomies. In infants with simple MI, time to full enteral feeds was 6 (2–10) days in those decompressing with enema vs 15 (9–19) days with laparotomy after enema. Case fatality was 4% (95% CI 0.4–12%). Two infants, both preterm died, both in the second month after birth.
Infants with simple MI achieving successful enema decompression were more likely to have had repeat enemas than those who proceeded to laparotomy. Successful non-operative management was associated with a shorter time to full feeds. The early management of infants with MI is highly variable and not standardised across the UK and Ireland.