Obstructive Gastrointestinal Tract Disorders in cystic fibrosis

Meconium ileus (MI) – is a disorder of the neonate caused by the obstruction of the small intestines at the level of the terminal ileum with inspissated meconium. Epidemiology: Approximately 10 percent of patients with cystic fibrosis (CF) present as neonates with meconium ileus. Conversely, most series suggest that 80 to 90 percent of infants with MI have CF. Infants with MI are at increased risk for developing cholestasis. Approximately 40 percent of MI in newborns with CF is complex (complicated by gastrointestinal pathology, including intestinal perforation, meconium peritonitis, atresia, or volvulus).Clinical manifestations: In most patients, manifestations appear during the first three days of life with abdominal distension and failure to pass meconium, with or without vomiting. An abdominal plain film should be performed to look for evidence of dilated bowel loops, perforation, calcifications, or other abnormalities. Affected infants are initially stabilized with nasogastric decompression and correction of fluid and electrolyte abnormalities.Diagnosis: All infants with MI should have a definitive diagnostic test for CF. Infants with MI are likely to have a negative newborn screen for CF, for unclear reasons. If there is no evidence of perforation, hyperosmotic contrast enema radiography is performed to confirm the diagnosis, using a water-soluble agent. In patients with MI, the contrast radiograph typically reveals a small-caliber colon (microcolon of disuse) and meconium pellets in the terminal ileum. The ileum proximal to the obstruction is dilated.Management: Nonoperative management – Affected infants are initially stabilized with nasogastric decompression and correction of fluid and electrolyte abnormalities. If simple MI is confirmed by the diagnostic enema, it is usually managed by administration of hyperosmolar enema (Gastrografin), closely monitored by fluoroscopy. . Patients should be well hydrated and have an intravenous line in place before performing the procedure.Operative management – Surgical approaches are used for complicated MI and for some cases of simple MI that do not clear with nonoperative intervention.