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Management and survival of meconium ileus. A 30-year review
C A Del Pin1, C Czyrko, M M Ziegler
1Department of Surgery, Children's Hospital of Philadelphia, PA 19104.
Abstract:
Cystic fibrosis patients born with meconium ileus (MI) have had an improved outcome over the last three decades. The authors reviewed the impact of surgical management and long-term nutritional care on the survival of patients with MI. Of the 59 cases of MI seen from 1959 to 1989, 48 cases were managed operatively using either the Bishop-Koop ileostomy (BK), the Mikulicz ileostomy, primary resection and anastomosis (RA), or ileostomy. Six-month survival of MI has improved from 37% to 100%. Nonoperative cases (n = 11) had 100% long-term survival. The RA survivors required less late operative intervention (20%) as compared with other surgical patients (81%). A comparison of serial growth percentiles of CF patients with MI with those of their non-MI CF peers showed similar long-term decreases. These data confirm: (1) There is an improved survival for MI independent of the surgical procedure; (2) The BK ileostomy is an effective and time-tested MI treatment; (3) Primary resection and anastomosis in selected cases may have a lower surgical morbidity rate; and (4) Meconium ileus does not adversely affect the long-term nutritional outcome of CF patients.
Insights
Outcomes for cystic fibrosis patients with meconium ileus have significantly improved, with survival rates reaching 100% regardless of surgical approach. Long-term nutritional status in these patients is comparable to their peers without meconium ileus.
Area of Science:
- Pediatric Surgery
- Cystic Fibrosis Research
- Gastroenterology
Background:
- Meconium ileus (MI) is a significant early complication of cystic fibrosis (CF).
- Historically, MI presented a poor prognosis, but recent decades show improved outcomes.
Purpose of the Study:
- To evaluate the impact of surgical management and long-term nutritional care on survival in CF patients with MI.
- To compare the effectiveness of different surgical interventions for MI.
Main Methods:
- Retrospective review of 59 CF patients with MI diagnosed between 1959 and 1989.
- Analysis of surgical outcomes (Bishop-Koop ileostomy, Mikulicz ileostomy, primary resection and anastomosis, ileostomy) and nonoperative management.
- Comparison of long-term growth percentiles between CF patients with and without MI.
Main Results:
- Six-month survival for MI improved from 37% to 100% over the study period.
- Nonoperative management (11 cases) resulted in 100% long-term survival.
- Primary resection and anastomosis (RA) group showed lower late operative intervention rates (20%) compared to other surgical methods (81%).
- Long-term growth percentiles were similar between CF patients with and without MI.
Conclusions:
- Improved survival for MI is independent of the specific surgical procedure used.
- Bishop-Koop ileostomy remains an effective treatment for MI.
- Primary resection and anastomosis may offer lower surgical morbidity in selected MI cases.
- Meconium ileus does not negatively impact the long-term nutritional outcomes of cystic fibrosis patients.