Meconium Ileus Study

A prospective, national observational study to determine the incidence, presentation, current management strategies and outcomes for Meconium Ileus in association with Cystic Fibrosis in the UK and Ireland, at one year after diagnosis

Key points

  • MI is not reported in any UK registry and there is currently limited information about the incidence and mode of presentation of MI in the UK and Ireland. To date there are no prospective, population based observational studies of MI.
  • Operative outcomes for MI are limited to case series collected over prolonged periods at single centres rather than population based data.
  • By limiting the study to Cystic Fibrosis (CF) patients with MI we will be able to utilise CF registries to cross reference the cases and ensure high levels of case ascertainment.
  • The MI study will collect accurate data on the incidence, mode of presentation, genotypes, clinical characteristics and outcomes for this group of infants.
  • Outcome data at one year will be collected to allow critical analysis of the different management strategies which will aid the development of management guidelines and provide a benchmark against which paediatric surgeons can assess their own operative outcomes.

Surveillance Period

October 2012 – September 2014

Background

Meconium Ileus (MI) is defined as small bowel obstruction in the newborn period caused by inspissated meconium within the terminal ileum[1][2]. There is a well-recognised association of MI with cystic fibrosis (CF). 10% - 15% of infants with CF will present with MI[1]. A Dutch study identified that in their patient population CF infants were more likely to present with complicated MI[3]. There is currently limited information about the incidence and mode of presentation of MI in the UK and Ireland. The proposed study will collect accurate data on the incidence, mode of presentation, genotypes, clinical characteristics and outcomes for this group of infants and will provide valuable information which will aid in the counselling of parents with infants diagnosed with MI and CF.

In addition, outcome data at one year will be collected. This will allow critical analysis of the different management strategies which will aid the development of management guidelines and facilitate the planning of resource allocation and service provision.

The study will be the first national population based study with prospective follow-up of a birth cohort of infants with MI and CF. It will provide accurate data regarding the incidence of MI and CF in the UK and Ireland, the current non-surgical and surgical management of MI, the complications of MI and the specialist service utilization of these patients in the first year of life. The data will also provide a benchmark against which paediatric surgeons can assess their own operative outcomes.

Research questions

  • What is the incidence of Meconium Ileus (MI) with Cystic Fibrosis (CF) in the UK and Ireland?
  • What is the mode of presentation, genotypes, current diagnostic strategies and clinical characteristics for MI with CF in the UK and Ireland?
  • What are the current management strategies for patients with MI with CF in the UK and Ireland and the incidence of immediate and short-term complications of treatment?
  • What are the outcomes at one year after diagnosis?

Case definition

Any live-born infant presenting between 01/10/2012 and 30/09/2014 with Meconium Ileus and Cystic Fibrosis. Meconium Ileus is defined as bowel obstruction caused by inspissated meconium in the terminal ileum.

Ethics approval

The study has been approved by the NRES Committee South Central-Oxford A (Ref: 12/SC/0416)

Study Lead

Ms Janet McNally, Bristol Royal Hospital for Children.

Download the Data Collection Form (DCF)

BAPS-CASS Meconium Ileus DCF

PLEASE NOTE: The data collection form is for your information only. Please DO NOT use it to report a case or to provide us with information on a case you have reported. If you have reported a case to us, a form with the BAPS-CASS Case ID written on it will be sent to you. If you wish to report a case, or require an additional form please contact us. Thank you.

References

  1. a, b Carlyle BE, Borowitz DS, Glick PL. A review of pathophysiology and management of foetuses and neonates with meconium ileus for the pediatric surgeon. J Pediatr Surg 2012;47: 772–781.
  2. ^ Copeland DR, St Peter SD, Sharp S et al. Diminishing role of contrast enema in simple meconium ileus. J Pediatr Surg 2009; 44: 2130-2132.
  3. ^ Burke MS, Ragi JM, Karamanoukian HL et al. New strategies in the nonoperative management of meconium ileus. J Pediatr Surg 2002; 37: 760-764.