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Condition: Meconium Ileus
Overview (“What is it?”)
- Meconium: The stool (intestinal contents) that collects in the intestines of fetuses (unborn babies). When the babies are born, they evacuate this stool for the first 24-48 hours, which tends to be thicker and darker than normal baby poop. Afterward, the stools tend to be thinner and lighter.
- Meconium ileus: Blockage of intestine due to abnormally thick meconium at the point where small bowel transitions to large bowel.
- Blockage can happen because the thick meconium does not allow intestinal fluid to go through. Alternatively, blockage can cause the intestine to rupture, twist or not be in continuity.
- Sometimes complications resulting from the blockage may be seen on ultrasounds done to monitor the growing baby inside the mother.
- Epidemiology: Meconium ileus is seen mostly in two populations:
- Cystic fibrosis (CF) patients. CF patients tend to have thick secretions in their intestines as a result of their disease.
- Preterm infants. Sometimes associated with medications to slow down labor.
Signs and Symptoms (“What symptoms will my child have?”)
- Inability to keep feedings down, vomiting, swollen belly, no stool. Sometimes, large intestinal loops may have been seen on prenatal ultrasound, abdominal distension, failure to pass stool. If there is a rupture of the intestine, the baby may be sicker with low blood pressure.
- Conditions that mimic this condition: Other conditions can also cause intestinal blockage including narrowing or discontinuity of intestine, abnormal rotation of intestine, Hirschsprung disease (a disease where the nerves that control intestinal movement do not form completely), incarcerated hernia (intestine caught in a connection between the abdomen and the groin).
Diagnosis (“What tests are done to find out what my child has?”)
- Blood tests: Blood tests to check for CF may be obtained.
- Other tests: Sweat may be tested for concentration of chloride. This will help determine whether someone has CF.
- X-rays: Plain X-rays may give clues as to the cause of intestinal blockage.
- Contrast enema: Water-soluble contrast is instilled through the anal opening and X-rays are obtained to see the anatomy of the large intestine and where the blockage may be. Sometimes, the contrast mixes with the thick stool and allows the stool to be evacuated.
Treatment (“What will be done to make my child better?”)
- Non-operative management
- Non-operative management of meconium ileus can be tried if there are no complications from the blockage (such as intestinal twist, rupture, or discontinuity). Attempts to dissolve the thick meconium can be tried. Water soluble contrast can be instilled into the anus. The contrast can mix and soften the thick meconium, allowing it to be pooped out.
- The infant will have X-rays performed every 8-12 hours to observe progress. If the meconium does not pass completely the first time, but the infant remains stable, the enema can be repeated safely, and after exam by a physician.
- Surgery is needed if an infant fails to respond to enemas and the bowel remains blocked. Surgery is also required if there are complications resulting from meconium ileus such as intestinal rupture, twist or discontinuity.
- The goal of surgery is to relieve the intestine of blockage. If an infection exists, the goal is also to control the infection (for instance, repair a hole in the intestine). What is done in the operating room depends on many factors including the status of the baby (stable vs unstable), size of the baby, and damage to the intestine. Options may include simple flushing of the intestine, removal of an intestinal segment with re-establishment of continuity, creation of a stoma. The surgeon will make a decision of what is the best and safest alternative for the baby while in the operating room.
- Preoperative preparation involves giving fluids and antibiotics.
- Postoperative care: Infants are managed in the neonatal intensive care unit. The infant will likely need the support of a breathing machine (ventilator). It will take days or sometimes weeks for the intestine to gain full function so nutrition will have to be provided through the veins.
- Risks: Risks of operation including anesthesia, bleeding, postoperative infection, damage to internal structures which may require further operation, and recurrence of meconium ileus.
- Benefits: The bowel obstruction from meconium ileus and accompanying complications make the infant very ill. Often surgery is needed to relieve the blockage and help control any existing infection.
Home Care (“What do I need to do once my child goes home?”)
- Diet: The infant’s formula will be determined by many factors such as whether or not s/he has CF and the length of intestine that remains if intestine required resection.
- Activity: Activity should be normal.
- Wound care: By the time the baby is released, s/he should be able to have regular baths. If a stoma remains, you will be trained in stoma care prior to discharge.
- When to call a doctor: Not able to keep down feeds, vomiting (in particular, green or bright yellow vomiting), swelling of the belly, no bowel movements, belly pain, redness or drainage from wound.
- If the infant is diagnosed with cystic fibrosis, they will likely be referred to a specialized group of doctors (lung and gut specialists) to address all aspects of CF care including dietary supplements, exposure restrictions, lung care.
Long-Term Outcomes (“Are there future conditions to worry about?”)
The outcome for meconium ileus treatment has improved to almost 100% survival. If the child required an operation but does not have CF, there is a lifetime risk of intestinal blockage from scarring of about 10-15%. Children with cystic fibrosis are at a much higher likelihood of having pulmonary and gastrointestinal complications as they get older. These will not be discussed here.
Author: Joanne E. Baerg, MD
Editors: Patricia Lange, MD; Marjorie J. Arca, MD