Treatment of Meconium Ileus in Newborns
Initial Management Approach
For uncomplicated meconium ileus, the treatment of choice is non-operative management with Gastrografin (diatrizoate meglumine) enema, with surgical intervention reserved for enema failure or complicated cases. 1, 2, 3
Non-Operative Management (First-Line for Uncomplicated Cases)
Gastrografin enema should be attempted first in neonates with uncomplicated meconium ileus (obstruction limited to inspissated meconium in terminal ileum without volvulus, atresia, or perforation). 1, 2, 4
Success rates for Gastrografin enema range from 54-58%, completely relieving obstruction without surgery. 2, 5
Critical pitfall: Gastrografin enema carries a 13% risk of colonic and rectal perforation, requiring careful monitoring during the procedure. 2
Alternative non-operative approach includes enteral N-acetylcysteine, though less commonly used. 3, 4
Non-operative success significantly reduces hospitalization (average 15 days vs 54-111 days for surgical cases). 2
Surgical Management
When non-operative management fails or complications are present, surgical intervention is required. 1, 3, 4
For Uncomplicated Meconium Ileus (After Enema Failure):
Enterotomy with irrigation and primary closure is the preferred single-stage procedure, avoiding the need for stoma creation. 3
Alternative approaches include Bishop-Koop enterostomy, T-tube irrigation, or resection with primary anastomosis. 2, 3, 5
A single surgical procedure is preferable given the high rate of pulmonary involvement in cystic fibrosis patients. 3
For Complicated Meconium Ileus:
Complicated cases present with volvulus (most common), intestinal atresia, perforation, or giant cystic meconium peritonitis. 1
Resection with primary anastomosis is as safe as stoma formation and reduces initial hospital stay in most complicated cases. 4
Primary anastomosis should be performed when feasible (in absence of peritonitis, severe prematurity, or giant cystic meconium peritonitis). 1, 5
Stoma formation (double enterostomy or Bishop-Koop) should be reserved for cases with peritonitis, late diagnosis, prematurity, or associated anomalies. 1, 5
Post-Operative Nutritional Support
Parenteral nutrition may be essential as short-term support following intestinal resection in infants with meconium ileus, particularly when enteral feeding is not immediately possible. 6
Early enteral feeding should be encouraged to reduce risk of cholestasis. 6
Pancreatic enzyme replacement therapy (PERT) must be initiated once enteral feeding begins, as over 90% of meconium ileus patients have pancreatic insufficiency. 6, 7
Fat-soluble vitamin supplementation (A, D, E, K) is necessary with pancreatic insufficiency. 8
Cystic Fibrosis Confirmation and Long-Term Management
Sweat chloride testing (>60 mEq/L) confirms cystic fibrosis diagnosis, though infants often have initial values of 30-59 mEq/L. 6, 8
Meconium ileus occurs in 10-20% of newborns with cystic fibrosis and represents the earliest clinical manifestation (median age of diagnosis 0.2 months). 6, 8, 7
Immediate referral to a cystic fibrosis center for multidisciplinary care is essential once diagnosis is confirmed. 8
Genetic counseling should be provided, as both parents are carriers of CFTR mutations. 3
Chronic airway clearance regimen and antimicrobial prophylaxis protocols must be established. 8