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Long-term outcome after neonatal meconium obstruction
1Department of Surgery, Washington University School of Medicine, St Louis, Missouri, USA.
Insights
Children with cystic fibrosis (CF) presenting with meconium ileus have similar long-term outcomes as those without, but a higher risk of surgical complications. Infants with meconium obstruction but without CF show an excellent prognosis.
Area of Science:
- Pediatric Gastroenterology
- Neonatal Surgery
- Cystic Fibrosis Research
Background:
- Neonatal meconium ileus is a common initial manifestation of cystic fibrosis (CF).
- The long-term implications of meconium ileus in CF patients compared to those without meconium ileus or non-CF meconium obstruction are not well-defined.
Purpose of the Study:
- To compare the long-term outcomes of children with CF who presented with meconium ileus versus those with CF without meconium ileus.
- To evaluate the prognosis of neonates with meconium obstruction without CF (meconium plug syndrome).
Main Methods:
- A retrospective comparative study analyzed data from three groups: CF patients with meconium ileus (n=35), CF patients without meconium ileus (n=35), and infants with meconium plug syndrome (n=12).
- Outcomes assessed included pulmonary, gastrointestinal, nutritional, and functional status, along with surgical complications.
Main Results:
- CF patients with meconium ileus had a higher incidence of meconium ileus equivalent and significantly more long-term surgical complications (27%) compared to CF patients without meconium ileus.
- Complicated meconium ileus and surgical management involving resection or enterostomy were associated with increased surgical complication rates.
- Infants with meconium plug syndrome (no CF) demonstrated superior growth and functional status with fewer pulmonary and gastrointestinal issues.
Conclusions:
- Long-term outcomes for CF patients with and without meconium ileus are comparable, barring a slightly increased risk of meconium ileus equivalent and a notably higher rate of surgical complications in the meconium ileus group.
- Complicated meconium ileus and specific surgical interventions increase the risk of adverse surgical outcomes.
- Neonates with meconium obstruction but without CF have a favorable long-term prognosis, crucial information for family counseling.
Objective:
It is unclear whether children with cystic fibrosis (CF) who present with neonatal meconium ileus have a different long-term outcome from those presenting later in childhood with pulmonary complications or failure to thrive. We examined a cohort of patients with meconium ileus, and compared their long-term outcome with children who had CF without meconium ileus and neonates who had meconium obstruction without CF (meconium plug syndrome).
Study Design:
Comparative study using retrospective and follow-up interview data.
Patients:
Group 1 consisted of 35 surviving CF patients who presented with meconium ileus between 1966 and 1992. Two control groups were also studied: 35 age- and sex-matched CF patients without meconium ileus (group 2), and 12 infants presenting with meconium plug syndrome during the same time period (group 3).
Outcome Measures:
Pulmonary, gastrointestinal, nutritional, and functional status were reviewed, and surgical complications were recorded.
Results:
Mean follow-up was 12.6 +/- 6, 12.6 +/- 6, and 9. 3 +/- 4 years in groups 1, 2, and 3, respectively. Patients without CF (group 3) demonstrated better growth and functional status, and had a lower incidence of pulmonary and gastrointestinal problems. Although the presence of meconium ileus among CF patients was associated with an earlier diagnosis, there were no significant differences between groups 1 and 2 with respect to hepatobiliary, nutritional, functional, or respiratory status. Meconium ileus was associated with a higher risk of meconium ileus equivalent (20% vs 6%), although this difference was not statistically significant. Long-term surgical complications (adhesive small bowel obstruction and blind loop syndrome) were seen in 27% of children with meconium ileus; there were no long-term surgical complications in groups 2 or 3, because these infants did not have any neonatal surgical procedures. Children presenting with complicated meconium ileus had a higher rate of long-term surgical complications than those with uncomplicated meconium ileus (36% vs 17%), and those managed with resection or enterostomy had more complications than those treated by enterotomy and lavage (33% vs 0%).
Conclusions:
Long-term outcome is similar in CF patients who present with meconium ileus and those who do not, except for a slightly higher incidence of meconium ileus equivalent, and a significantly higher rate of surgical complications. The risk of surgical complications is highest in those presenting with complicated meconium ileus and those undergoing resection or enterostomy. Patients with meconium obstruction who do not have CF have an excellent long-term prognosis. This information will be useful in counseling the families of infants presenting with neonatal meconium obstruction.