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Digestive System Problems

Some babies are born with abnormalities in their digestive system. These are often called congenital abnormalities or birth defects. They form as the result of something going wrong with the development of the baby’s digestive system during pregnancy. It is important to keep in mind that these abnormalities are rare, and can sometimes be treated with surgery.

Abnormalities of the esophagus

Esophageal atresia

This is a condition where the esophagus is not properly connected to the stomach. Babies born with these malformations are fed intravenously with a solution called total parenteral nutrition or, rarely, with a feeding tube directly into the stomach. Esophageal atresia is usually repaired with surgery within the first few days of life.

Tracheoesophageal fistula

This is a small opening, or fistula, between the windpipe and the esophagus. This condition usually occurs in association with esophageal atresia. Tracheoesophageal fistula is caused by a problem with the partitioning of the esophagus from the trachea during development of the unborn baby. One complication of this condition is that stomach contents could pass into the trachea through the fistula and cause pneumonia. Babies born with tracheoesophageal fistula are fed with a feeding tube directly into the stomach. The condition is usually treated with surgery within the first few days of life.

Tracheoesophageal Fistula and Esophageal Atresia
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Tracheoesophageal fistula is a small opening between the windpipe and the esophagus. It often occurs in combination with esophageal atresia, a condition in which the esophagus is not properly connected to the stomach. Babies born with these conditions are fed with a feeding tube directly into the stomach. After several weeks, the malformations are repaired with surgery.
Abnormalities of the stomach

Pyloric stenosis

Pyloric stenosis blocks the passage of food from the stomach to the intestine, which causes severe vomiting. This is one of the most common conditions in babies. Pyloric stenosis occurs when the round end of the stomach enlarges and the lower, tubular portion of the stomach becomes extremely narrow. This narrowing is what blocks food from leaving the stomach. Surgery is used to correct this problem.

Abnormalities of the intestines

Malrotation with volvulus

Malrotation with volvulus happens when parts of the gastrointestinal system end up in the wrong place as a result of a developmental problem. Normally in early pregnancy, the baby’s gastrointestinal tract moves to the umbilical cord to develop and grow. Soon after, the gastrointestinal tract moves back to the baby's abdomen and rotates into its final position. If this process does not occur properly, parts of the gastrointestinal tract can end up in the wrong place. This is called malrotation. Sometimes the gastrointestinal tract ends up looping around itself to an extent that a blockage forms that could reduce the blood supply, and this is called volvulus. Malrotation with volvulus requires emergency surgery to prevent destruction of the intestine.

Malrotation with Volvulus
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If the gastrointestinal tract does not develop properly, parts of it can end up in the wrong place. This is called malrotation. Sometimes the gastrointestinal tract ends up looping around itself to the extent that a blockage forms that could reduce the blood supply, and this is called volvulus. Malrotation with volvulus requires emergency surgery.
Intestinal atresia

This is a malformation where part of the intestine is disconnected from the rest of the gastrointestinal tract. Most often, these occur at a spot just below the stomach called the duodenum. The cause of these conditions is usually a problem with intestinal development, such as malrotation with volvulus. Babies with this condition are often small for their gestational age. Sometimes the problem is a narrowing of the intestine, which is called an intestinal stenosis. Intestinal atresia and stenosis can be repaired with surgery, preferably sooner than later.

Intestinal Atresia
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Intestinal atresia is a malformation where part of the intestine is very narrow or disconnected from the rest of the gastrointestinal tract. Most often, this occurs at a spot just below the stomach called the duodenum. Intestinal atresia can be repaired with surgery.
Hirschsprung’s disease

This is a condition where nerve cells called ganglia are not properly formed on the inner walls of the intestine. This causes the bowel to contract and spasm, and block part of the lower intestine. Surgery is used to correct this condition. The surgeon removes the section of the intestine that is missing the ganglia and then reattaches the healthy intestine to the rectum. The outlook for babies who have gone through this surgery is very good.

Abnormalities of the biliary tract

Choledochal cyst

The biliary tract consists of the gallbladder and bile ducts of the liver, and is responsible for the flow of bile to the small intestine. Choledochal cyst is a cyst in the biliary tract, which obstructs the flow of bile. Choledochal cyst is treated with surgery.

Extrahepatic biliary atresia

Extrahepatic biliary atresia is damage to the ducts of the liver. The ducts do not develop properly, perhaps due to an infection in the baby during pregnancy. As a result, bile is trapped inside the liver, which causes liver damage and scarring. This is a rare condition. About 20% of newborn babies with this condition can have the duct repaired with surgery. The remainder of patients will require a liver transplant.

Other abnormalities of the abdominal organs

Omphalocele

This is a condition where the anterior abdominal wall, that is, the abdominal wall toward the front of the baby's body, has not completely closed. Abdominal organs may protrude through the umbilical region, surrounded by a sac called the amnion. This condition occurs when the gastrointestinal organs do not completely move back from the umbilical cord to the abdomen in early pregnancy. This is a rare but very serious condition. This condition is often associated with heart problems, neural tube defects, and chromosomal abnormalities. Omphalocele is treated with surgery.

Gastroschisis

This is a condition where the abdominal organs protrude through a defect in the anterior abdominal wall. The organs hang outside the body beside and separate from the umbilical cord. The protruding organs are not covered with the protective membrane called amnion, and therefore they may be damaged by exposure to amniotic fluid. Unlike omphalocele, gastroschisis is not associated with heart problems, neural tube defects, or chromosomal abnormalities, but it is associated with intestinal atresias. Surgery is usually quite successful in the treatment of this condition. The survival rate for gastroschisis is excellent.

Abnormalities of the anus and rectum

There are different types of malformations of the anus and rectum. The opening of the anus may be missing, which is a condition called imperforate anus. There may be a small opening called a fistula running from the rectum to the urinary tract or to the vagina. Depending on the nature and severity of these malformations, babies may have full, partial, or no control over their bowel movements. Abnormalities of the anus and rectum can be corrected with surgery. If the opening of the anus is missing, the surgeon will create a new opening. If there is a fistula present, the surgeon will close it.

Nicolette Caccia, MEd, MD, FRCSC

Rory Windrim, MB, MSc, FRCSC

Andrew James, MBChB, MBI, FRACP, FRCPC

9/10/2009




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