Long-term intestinal obstruction sequelae and growth in children with cystic fibrosis operated for meconium ileus: expectancies and surprises

Background/purpose In the few studies on intestinal complications and growth of cystic fibrosis (CF) patients with a history of meconium ileus (MI), operated MI has not been investigated separately. We aimed to investigate the incidence of long-term intestinal obstruction sequelae [constipation, distal intestinal obstruction syndrome (DIOS)] and growth in CF patients operated for MI. Methods Retrospective study (1989–2016) including operative diagnoses and procedures, constipation and DIOS events, yearly Body Mass Index (BMI) measurements. Outcomes were examined in subgroups operated for MI only and for MI with atresia and/or volvulus. Results Of 49 patients followed-up for 15 (mean) years, 5 (10.2%) developed constipation and 14 (28.6%) DIOS. BMI was within normal percentiles in 53 patients over a 10-year follow-up. MI only and MI with atresia and/or volvulus did not differ in constipation and/or DIOS incidence (11/34 vs. 7/15, p =0.39) or in BMI ( p =0.47). Cases with ileocecal valve resection (ICV-R) showed lower constipation and/or DIOS incidence than those without ICV-R (0/6 vs. 11/28, p =0.02) and no different BMI ( p >0.05). Conclusions CF patients operated for MI were in long-term risk for constipation/DIOS; their growth was normal. Interestingly, underlying atresia/volvulus neither increased constipation/DIOS risk nor affected growth. Strikingly, ICV-R showed no constipation/DIOS risk and no impact on growth. Type of study Retrospective comparative study. Level of evidence III.