Gastroschisis

 Gastroschisis

 Overview

Gastroschisis is a birth defect where a hole in the abdominal (belly) wall beside the belly button allows the baby’s intestines to extend outside of the baby’s body. The hole can be small or large and sometimes other organs, such as the stomach and liver, can be found outside of the baby’s body as well.

Gastroschisis occurs early during pregnancy. The hole is usually to the right side of the belly button. Because the intestines are not covered in a protective sac and are exposed to the amniotic fluid, they can become irritated, causing them to shorten, twist, or swell.

Gastroschisis is a birth defect where your baby’s intestines (stomach, large or small intestines) exit their body from a 2 to 5-centimeter hole beside their belly button during fetal development. This condition happens early during pregnancy when your baby’s abdominal wall doesn’t form correctly, leaving an opening for their organs to escape. Your baby’s organs float in amniotic fluid inside of your uterus, which causes irritation and swelling. Surgery is necessary to replace your baby’s organs inside their body.

Symptoms

During pregnancy, you will not feel any symptoms of your baby’s gastroschisis. Some findings for the baby on ultrasound may include:

Stomach, large or small intestines located outside of your baby’s body.

Swollen intestines.

Twisted intestines.

Low body temperature (hypothermia)

Babies born with gastroschisis have some of their intestines on the outside of their belly. They will lose heat and water very quickly from the intestines, causing:

too much water loss (dehydration)

a body temperature that gets too low (hypothermia)

Other organs may stick out along with the intestines, including the baby's:

stomach

gallbladder

uterus

ovaries

testes

bladder

Causes

Doctors don't know why gastroschisis happens. It is probably due to a combination of genetic and environmental factors.

Gastroschisis is more likely when the mother:

is under age 20

smokes during her pregnancy

drinks alcohol

Diagnosis

Diagnosing gastroschisis during pregnancy

You may find out your baby has gastroschisis from a routine prenatal test (medical tests you get during pregnancy) that checks your baby for birth defects. Your health care provider also may check your baby for gastroschisis if you have a condition called polyhydramnios during pregnancy. Polyhydramnios is when you have too much amniotic fluid.

Your provider may use these tests to diagnose gastroschisis during pregnancy:

Fetal echocardiogram (also called fetal echo). This test uses ultrasound to make a picture of your baby’s heart while still in the womb. Some babies with gastroschisis also have heart problems.

Maternal blood screening (also called quad screen). This screening test is called a quad screen because it measures four substances in your blood: alpha-fetoprotein (also called AFP), estriol, human gonadotropin (also called hCG) and inhibin A. The test is done at 15 to 22 weeks of pregnancy. If you have high levels of AFP, your baby may have gastroschisis.

Magnetic resonance imaging (also called MRI). This is a medical test that uses a large magnet and radio waves to make a detailed picture of the inside of the body.

Ultrasound. This test uses sound waves and a computer screen to show a picture of your baby inside the womb. If your baby has gastroschisis, an ultrasound may show the bowels floating outside of your baby’s belly. Health care providers may find gastroschisis as early as 10 weeks of pregnancy on ultrasound, but it’s most often diagnosed between 18 and 20 weeks. You may need several ultrasounds during your pregnancy so your provider can monitor the intestines and other organs as your baby grows, checking for any changes to them.

Diagnosing gastroschisis after your baby is born

If gastroschisis isn’t found during pregnancy, your provider can diagnose it at birth.

Your health care provider may recommend you give birth at a hospital that has a newborn intensive care unit (also called NICU). The NICU is the nursery in a hospital where sick newborns get medical care. Most of the time, gastroschisis can be fixed with one or two surgeries. After recovering from surgery, most babies with gastroschisis live normal lives. Some children may have problems with digestion later in life.

Treatment

Once the diagnosis is made, most parents plan to deliver the baby in a hospital with a neonatal intensive care unit, where he or she can receive special care. A Caesarian delivery may be advised at about 36 weeks of pregnancy, if the baby's lungs are mature enough. (The maturity of the lungs is also shown on ultrasound tests.) The early delivery helps prevent further irritation of the intestine. Gastroschisis surgery will be done as soon as the infant's condition is stabilized, usually within 12 to 24 hours after delivery.

Gastroschisis surgery 

When babies have only a small amount of intestine outside the abdominal wall, one operation can often correct the problem. About two-thirds of gastroschisis patients fall into this category. The surgeons simply return the intestine to the abdominal cavity and close the hole.

If the amount of bowel outside the abdomen is large or if the baby's condition is unstable, the surgery for the gastroschisis may need to be staged (done in several steps) over three to ten days. A Silastic (silicone plastic) pouch is first placed over the baby's exposed bowel and anchored to the surrounding muscle. Each day, the pouch is tightened to push the intestine back into the abdominal cavity. When the intestine is back in its proper place within the abdomen, the pouch is removed and the opening is surgically closed. Some babies may need the help of a breathing machine (ventilator) during this time.

Gastroschisis prognosis

In most babies, the long-term prognosis is very good. However, some babies may develop a condition known as short gut. These babies usually have sustained injuries to the bowel due to direct contact between the intestines and amniotic fluid, or due to the intestine being twisted or damaged in some way during fetal development or delivery. Diarrhea, slow weight gain, and deficiencies of essential vitamins and minerals characterize this condition. Babies with short gut may require a prolonged course of intravenous nutrition (total perenteral nutrition, or TPN) delivered at the hospital or at home, and will need to be closely monitored by a pediatric surgeon, pediatric gastroenterologist, and primary care physician.

 Type of Doctor Department : A neonatologist and a pediatric surgeon