Gastroschisis

Crying Baby Gastroschisis is a birth defect that causes a baby's intestines to develop outside of the body. This condition is considered rare, but recent studies show that it is becoming more common, especially in younger mothers. According to the Centers for Disease Control and Prevention (CDC), an estimated 1,871 babies are born with this condition each year. Babies with gastroschisis require surgery to place the bowels back into the stomach and must stay in the hospital until they have fully recovered. Most cases of gastroschisis can be successfully treated and usually do not cause any long-term complications, with the survival rate being over 90%. However, some babies may need temporary medical assistance to help them receive proper nutrition while they heal.

What is Gastroschisis?

Gastroschisis is a birth defect that affects the baby's abdominal wall. It occurs when the abdominal wall does not develop correctly and forms an opening near the belly button. This causes the intestines to be pushed out through the hole and develop outside the body in the amniotic fluid. The size of the abdominal hole can vary from small to large, with the average size being 1-2 inches, and is most often found on the right side of the belly button. Gastroschisis can result in the intestines becoming irritated, shortened, twisted, or inflamed because there is no protective sac covering them while being exposed to the amniotic fluid.

Gastroschisis is labeled as either "simple" or "complex". In simple cases, only the intestines form outside the body. For complex cases, the bowels may be damaged from the amniotic fluid and other organs, such as the stomach and liver, also protrude out of the opening. Fortunately, simple cases are far more common than complex ones, but even severe cases can be fixed with surgery. Babies with severe gastroschisis are more likely to experience complications after receiving treatment.

What Causes Gastroschisis?

Currently, the underlying cause of gastroschisis is still unknown. It's been suspected that the condition is linked with changes in the baby's genes or chromosomes. It could also be caused by a combination of factors, including what the mother eats or drinks, what medication she's using, and possibly other environmental factors. The CDC has been conducting research on gastroschisis and recently found that using alcohol or tobacco during pregnancy can increase the risk of the baby developing the condition. Still, there have been no definite conclusions on what is the primary cause.

How is Gastroschisis Diagnosed?

Gastroschisis develops early in pregnancy, but usually is not detected until the 18-20th week of gestation. The most common way it is diagnosed is through a routine ultrasound. Once the ultrasound produces a picture of the baby inside the womb, the bowels can be seen floating freely in the amniotic fluid. This can confirm a diagnosis of gastroschisis, but doctors may want to perform an additional evaluation with other tests, such as an MRI or fetal echocardiogram, to see if the baby is experiencing any other problems. After a mother is diagnosed, it's recommended to have frequent ultrasounds throughout the rest of the pregnancy to monitor the baby's health and how the gastroschisis is progressing.

Can I Prevent Gastroschisis?

There is no way to prevent gastroschisis from occurring besides staying as healthy as possible and avoiding substances that could potentially harm the baby. It's been suggested that folic acid can help prevent birth defects such as gastroschisis. Most prenatal vitamins already have this included in the formula, but you may want to find a vitamin that contains at least 400 micrograms of folic acid.

What Will Happen After Birth With Gastroschisis?

As soon as the baby is born, doctors will begin treatment to protect their organs and keep their vitals stable. The first steps in treating gastroschisis after birth often include:

  • Using a ventilator: A baby might be put on a ventilator machine to help them breathe if they are having troubles breathing on their own.
  • Using a nasogastric tube: Gastroschisis can result in fluid and air being stuck in the baby's abdomen, and a nasogastric tube will be used to relieve the pressure. The tube is placed inside the baby's nose and then guided down into the stomach where suction is used to remove the fluid.
  • Having an IV inserted: After being born, a baby with this condition will have trouble processing nutrition on their own and needs an IV to be directly supplied with food and fluids.
  • Administering antibiotics: Because the bowels are exposed to the environment, it's important that they don't become infected. Antibiotics will be administered through an IV to prevent this from happening.
  • Protecting the intestines: Immediately after birth, doctors will wrap the intestines in gauze and covered in plastic to protect the intestines and prevent fluid loss.
How is Gastroschisis Treated?

Gastroschisis will need to be treated with surgery to put the organs back into place. A baby cannot survive with their bowels outside the body. The type of surgery, however, will depend on the severity of the condition. Surgery for gastroschisis is categorized as either "primary repair" or "staged repair".

Primary repair is used for simple cases of gastroschisis when there is only a small amount of bowel protruding out of the body and there is no damage to the intestines. The surgery for this is relatively simple, doctors will place the bowels back into the baby's belly and repair the opening in the abdomen. This can be performed on the same day or within the next few days after birth. Primary repair is generally not an option for babies with a large amount of bowel outside the body, if the bowel is severely inflamed or damaged, or if there is not enough room in the baby's stomach to hold the intestines.

Staged repair is used for complex cases of gastroschisis. This type of repair often requires multiple surgeries to slowly push the organs back into the abdomen and can take up to two weeks to be completed. In this scenario, doctors will provide a plastic pouch called a silo around the bowel and will slowly tighten it each day to gently push the organs back inside. Once the organs are successfully pushed into place, the silo can be removed.

What is the Prognosis for Gastroschisis?

Babies with gastroschisis tend to be smaller than average and they may develop slower. It can take some time for them to catch up to other babies in terms of developmental milestones. After receiving surgery, a baby will need to stay in the hospital until they have fully recovered. Inpatient care can range from 30-50 days, depending on the severity of the condition.

Each case of gastroschisis is unique and a baby can experience a variety of different effects after treatment, but simple cases of gastroschisis usually do not result in long-term complications. Some babies with complex gastroschisis will have problems feeding after surgery. They may require a feeding tube to absorb nutrition correctly and might experience chronic constipation and abdominal pain. As they grow up, they could possibly develop a food allergy or intolerance.

It's important to follow up with your doctor after you bring your baby home to make sure they are feeding and healing properly.

Sources and Additional Literature
  • Pakarinen, M.P., Koivusalo, A., & Suominen, J. (2019). Gastroschisis and Omphalocele. Journal of Neonatal Surgery, 417-427.
  • Bilibio, J. P., et al. (2019). Gastroschisis during gestation: prognostic factors of neonatal mortality from prenatal care to postsurgery. European Journal of Obstetrics & Gynecology and Reproductive Biology, 237, 79-84.
  • Suominen, J., & Rintala, R. (2018). Medium and long-term outcomes of gastroschisis. Seminars In Pediatric Surgery, 27(5), 327-329.
  • Laje, P., et al. (2018). Complex gastroschisis: Clinical spectrum and neonatal outcomes at a referral center. Journal Of Pediatric Surgery, 53(10), 1904-1907.
  • Salinas-Torres, V. M., et al. (2018). Genetic variants conferring susceptibility to gastroschisis: a phenomenon restricted to the interaction with the environment? Pediatric Surgery International, 34(5), 505-514.
  • Emil, S. (2018). Surgical strategies in complex gastroschisis. Seminars In Pediatric Surgery, 27(5), 309-315.
  • Werler, M. M., & Parker, S. E. (2017). The Gastroschisis Puzzle: Where are We and What is Next? Paediatric And Perinatal Epidemiology, 31(6), 560-562.