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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.
Abstract:
Meconium ileus (MI) affects 15% of neonates with cystic fibrosis (CF). The authors reviewed the management and outcome of 51 neonates presenting to a single institution between 1976 and 1995 with MI secondary to CF. Clinical presentation included abdominal distension (96%), bilious vomiting (49%), and delayed passage of meconium (36%). A family history of CF was present in 4 cases (8%). Twenty-three neonates presented with MI and evidence of volvulus, atresia, or perforation (complicated MI). Of these, 16 underwent stoma formation, 1 appendicectomy, and 6 resection with primary anastomosis. Twenty-eight neonates presented with uncomplicated MI. Of these, 11 were managed non-operatively by Gastrografin enema (10) or enteral N-acetylcysteine (1). The remainder required stoma formation (15) or bowel resection with primary anastomosis (2). Early postoperative complications occurred in 2 neonates (4%). In this hospital the 1-year survival for this condition has increased from 49% (1953-1970) to 98% (1976-1995) irrespective of the surgical procedure performed or the presence of volvulus, atresia, or perforation. In our experience, bowel resection with primary anastomosis is as safe as stoma formation and is associated with a reduced length of initial hospital stay.
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