Meconium ileus secondary to cystic fibrosis. The East London experience

I Mushtaq1, V M Wright, D P Drake

  • 1Department of Paediatric Surgery, Queen Elizabeth Hospital for Children, Hackney Road, London, UK.

Insights

Meconium ileus (MI) in cystic fibrosis (CF) neonates has improved survival rates to 98%. Bowel resection with primary anastomosis offers a safe alternative to stoma formation, reducing hospital stays.

Area of Science:

  • Pediatric Surgery
  • Neonatology
  • Gastroenterology

Background:

  • Meconium ileus (MI) is a significant early manifestation of cystic fibrosis (CF), affecting approximately 15% of neonates with the condition.
  • Early diagnosis and effective management are crucial for improving outcomes in neonates with MI secondary to CF.

Purpose of the Study:

  • To review the management strategies and outcomes of neonates with meconium ileus secondary to cystic fibrosis.
  • To evaluate the safety and efficacy of different surgical interventions, including stoma formation and bowel resection with primary anastomosis.
  • To assess the trends in survival rates for meconium ileus over a defined period.

Main Methods:

  • Retrospective review of 51 neonates diagnosed with meconium ileus secondary to cystic fibrosis between 1976 and 1995.
  • Analysis of clinical presentations, management approaches (operative and non-operative), and postoperative complications.
  • Comparison of outcomes, including survival rates and length of hospital stay, based on treatment modalities.

Main Results:

  • Common clinical presentations included abdominal distension (96%), bilious vomiting (49%), and delayed meconium passage (36%).
  • Complicated MI (with volvulus, atresia, or perforation) was present in 23 neonates, while 28 had uncomplicated MI.
  • The 1-year survival rate increased significantly from 49% (1953-1970) to 98% (1976-1995). Bowel resection with primary anastomosis was associated with a reduced initial hospital stay compared to stoma formation.

Conclusions:

  • Management of meconium ileus in cystic fibrosis has evolved, leading to dramatically improved survival rates.
  • Bowel resection with primary anastomosis is a safe and effective surgical option for meconium ileus, comparable to stoma formation, with the added benefit of a shorter hospital stay.

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