How Is Meconium Ileus Treated?

Meconium ileus, a serious condition in newborn babies in which the intestines are blocked by the infant’s first stool, often needs surgical intervention and usually indicates the child will struggle with the congenital disease cystic fibrosis throughout their lives.

Meconium ileus isintestinal obstructioncaused by thick, congealed meconium (baby’s first凳子), which does not get evacuated after the birth of a baby.

Cystic fibrosis is aninheriteddisorder caused by a genetic mutation which causes thickening of themucusand primarily affects the respiratory tract and pancreatic function. Cystic fibrosis leads to hardening of meconium and prevents its evacuation.

Meconium ileus may be:

• Simple meconium ileus: Simple obstruction in the smallintestinesresulting in dilatation of the bowel above the obstruction, and bowel wall thickening.
• Complicated meconium ileus: Intestinal obstruction complicated by conditions such as intestinal twisting, perforation and infection.

If thenewbornhas abdominal distension or other symptoms of meconium ileus, the initial medical treatment involves:

• Initiation of resuscitation of the infant
• Administration of IV fluids
• Gastric decompression by removing thegasin the digestive tract
• Administration of antibiotics
• Imaging test to evaluate the cause for abdominal distension
• Meglumine diatrizoate enema

A diagnosis of a simple meconium ileus is most often treated with an enema, though some infants may require surgery. The administration of enema involves the following steps:

• Prepare the infant with adequate hydration, supplementalelectrolytesand antibiotics.
• Administer enema with meglumine diatrizoate (Gastrografin), a water- soluble substance that softens the meconium, and also acts as a contrast medium for continuous X-ray (fluoroscopy) to monitor the process.
• Continue hydration with IV fluids.
• Monitor the baby closely for the next few hours.
• Administer additional enemas if required, until the meconium is completely evacuated.
• Imaging tests to confirm complete evacuation of the meconium.
• Administer supplemental pancreatic enzymes after obstruction is cleared, usually within 48 hours, if the baby is diagnosed with cystic fibrosis.

Potential complications of Gastrografin include:

• Perforation of the bowel
• Hypovolemicshock(shockfrom loss of fluids, which must be carefully monitored and managed with supplemental IV fluids)
• Ischemia (reduced blood supply to the tissues)

Surgical treatment is indicated if the newborn has complicated meconium ileus, with one or more of the following conditions:

• Persistent abdominal distension
• Enlarging abdominal mass
• Volvulus (twisting of a loop of intestine)
• Atresia (malformation of intestine)
• Necrosis (tissue death)
• Perforation
• Meconiumperitonitis(inflammation of peritoneum, the abdominal membrane) and pseudocyst formation from the spillage of meconium through the perforation

手术治疗胎粪性肠梗阻是个体所ualized for each infant. A pediatric surgeon performs the surgery with the baby under general anesthesia.

Preparation

Prior to a surgical procedure

• The infant undergoes imaging tests
• Theneonatalcare team
• Administers IV fluids and antibiotics
• Performs gastric decompression

Procedure

The surgical treatments for meconium ileus may involve one or more of the following procedures:

• Enterostomy: A procedure to make one or more openings (stomas) in the abdomen to insert small tubes into the intestine. The tubes are used to irrigate,decompressthe bowel, and also for feeding. The tubes are removed once the obstruction is completely cleared.
• Resection: Removal of the affected portion of the intestine.
• Anastomosis: Anastomosis is a procedure to connect the severed ends of the bowel after a resection.
• Transplantation: Intestinal and/orlivertransplantation because of cystic fibrosis associated liver damage.

Post-procedure

手术后,婴儿的衔接dration andelectrolyterequirements are managed carefully. Infants who have surgery for simple meconium ileus with cystic fibrosis may receivebreast milkor formula milk, pancreatic enzymes andvitamins.

Babieswho have a complex surgical procedure for complicated meconium ileus may receive diluted, pre-digestedinfant formulathrough the tube, until they are able to feed orally. Pancreatic enzymes are administered orally after the baby starts oral feeding.

Complications

In addition to surgical risks such aswoundinfection and bleeding, complications from surgical procedures for meconium ileus include the following:

• Excessive fluid and sodium loss in infants who undergo removal of a significant portion of the intestines.
• Adverse effects on the development of the colon and the growth of beneficial bacteria in the colon below thestoma, which is nonfunctional until the enterostomy tubes are removed.
• Excessive gastric acid in the shortened intestines can cause malabsorption of nutrients (short bowel syndrome).