Gastrointestinal Problems

Anus and Rectal Malformations
Atresia, stenosis, or web of the bowel
Biliary Atresia
Bladder Extrophy
Congenital Diaphragmatic Hernia
http://www.familyvillage.wisc.edu/lib_ea-tef.html
Gastroschisis
Hirschprung's Disease
Malrotation
Omphalocele
Hernias and Hydroceles

Atresia, stenosis, or web of the bowel

• In an atresia part of the bowel is thin like a thread and there is no hole in the middle, so that the bowel is blocked or "obstructed". An atresia can be at any part of the large or small bowel but atresias of the upper part of the small bowel are the most common. The amount of bowel that has atresia varies from a very small section to a large part of the bowel. There may be more than one area that has atresia.
• In stenosis the lumen or hole is present but smaller than normal. It may be a small or large portion or in more than one area.
• In a web the hole in one section of the bowel is not complete. It usually occurs in the area where the stomach meets the upper small bowel. The first part of the small bowel is called the duodenum; therefore, the web is a duodenal web.

• If the abnormal area is near the stomach, there may be too much amniotic fluid, called polyhydramnios.
• Too much amniotic fluid may cause premature labor and premature delivery.
• If the abnormal area is lower down there may be no indication there is a problem until after birth when the baby gets sick.

• The baby may have vomiting. Vomiting green material is not normal and is often what alerts one to the problem.
• The baby may have a swollen or full appearing tummy.
• The baby may seem uncomfortable, cry and not want to eat.
• Xrays of the baby's tummy are usually not normal
• Special studies where a dye is put into the bowel may be needed to help find the abnormal part of the bowel.

• S/he may be premature.
• S/he may be small for their number of weeks of pregnancy, called intrauterine growth restriction.
• S/he may have other abnormalities. This occurs about 1/5 of the time. Intestinal atresia is sometimes seen with gastroschisis and with Down syndrome.
• S/he may have problems with nutrition and growth. This is most common if the amount of normal bowel is short or if the surgeon had to remove a large amount of bowel.
• Scar tissue that develops after surgery can cause blockage later on.
• The baby can develop necrotizing enterocolitis.

Gastroschisis

• They may be premature and have the common problems of premature infants
• They may be smaller than normal for their weeks of gestation, see IUGR
• Part of the intestine may not have an opening inside it, called atresia, or a very narrow opening, called stenosis
• The total amount of intestine may be less than normal.
• The baby might aspirate the contents of the amniotic fluid into its lungs at birth, called aspiration pneumonia. This causes breathing problems.
• Occasionally the bowel twists on itself and kinks off its own blood supply while the baby is still in the womb. When this happens the bowel without a blood supply dies and the bowel that is left is very short in length.
• Because the intestines are outside the body , after birth the baby is at increased risk for infection, low body temperature (hypothermia), and rapidly losing too much fluid from the body.
• In boy babies, the testes sometimes stay in the abdomen instead of going to the scrotum. This is called undescended testes.

• The intestines and other organs may be placed back into the abdomen through the hole or through an incision that makes the hole larger. After the intestine is back in the abdomen, the hole is sewn closed.
• If the abdomen is too small for everything to fit back easily, one of three things will be done at surgery:
  • The skin is stretched over the intestines but the other layers of the abdomen are left open to be fixed later in infancy.
  • A patch, made of a special material, may be sewn to the skin edges to cover the intestine. This will be removed in a second surgery later.
  • A "silo" or sack is built out of special material. The edges are attached to the skin and the intestines are placed in it. Over about a week the abdomen stretches and more of the bowel is squeezed from the sack into the abdomen. The baby has a second surgery to remove the sack and close the abdomen.

• Scarring leading to blockage of the intestine - can occur anytime later in life.
• Feeding and digestion problems
• If a large amount of the intestine died, it will be difficult for the baby to grow and absorb nutrients. Nutrition by vein and/or special formulas may be needed make the baby grow. Most of the time the intestines heal and adapt, but it can take many months and sometimes years for this to occur.
• In the first few weeks after surgery the baby is at increased risk for an inflamation of the bowel called Necrotizing Enterocolitis

Malrotation

How does a baby with malrotation act?

• The baby usually gets sick in the first month, often the first few days.
• The baby cries a lot because his/her tummy hurts.
• The baby throws-up frequently; often the vomit is greenish. Vomiting green or yellow-green is not normal spitting up.
• The baby's tummy gets bigger and more firm
• The baby might look sick---pale color, less active, not wanting to eat.

• About 20-25% have another abnormality of the gut, usually atresia.
• Scarring after surgery can cause an obstruction (blockage) later on in life. This is true with any surgery in the abdomen. Another surgery would be needed to fix this problem.
• If a large amount of bowel had to be removed, the baby 's gut may be short. This may make it difficult for a baby or child to grow and absorb nutrients. Nutrition by vein and/or special formulas may be needed make the baby grow. Most of the time the intestines heal and adapt, but it can take many months and sometimes years for this to occur. Some older children and adults with short guts continue to have problems with loose stools and absorbing food.

Omphalocele

• They may be premature and have the common problems of premature infants
• These babies may be smaller or larger than normal for their weeks of gestation, see IUGR
• If the defect is large, the babies may have poor lung development and/or problems breathing.
• The intestines may be slow to handle food.
• Other organs might be abnormal. About 50% of babies with omphalocele have abnormalities in the formation of other organs. Common abnormalities are:
  • Defects in the formation of the heart, occurs in 20%
  • Beckwith-Wiedeman Syndrome, a condition with a large tongue, high insulin and low blood sugar
  • Chromosome problems with many abnormal organs

• In surgery, the intestines and other organs may be placed back in the abdomen and the skin closed.
• If the abdomen is too small for everything to fit back easily, a special material is used to make patch or a "silo" (sack). The edges of the patch or the silo are attached to the skin. If a silo is used, the intestines are placed in it. Over about a week the abdomen stretches and more of the bowel is squeezed from the sack into the abdomen. With both the patch and the silo, the skin is closed in a second operation.
• Surgery may be delayed if the baby is very sick and the membrane over the defect is not open or leaking.

• Scarring leading to blockage of the intestine
• Feeding and digestion problems, especially if the amount of intestine is unusually small.

Hernias and Hydroceles

• smaller babies
• boy babies
• babies who have had lung disease

• Cannot reduce the hernia, that is get the loop of bowel to go back into the tummy. This is the earliest and, therefore, the most important sign.
• Inconsolable crying, pain
• Soreness or tenderness over the hernia
• Redness or bluish color in the groin or the scrotum
• Vomiting