Sanford’s Maternal-Fetal Care team, Pediatric Surgeons and Level III Newborn Intensive Care Unit provide advanced care and treatment for babies diagnosed with Gastroschisis.
What is Gastroschisis?
Gastroschisis (gas-troh-skee-sis) is a congenital birth defect in which the baby’s abdominal wall fails to close completely during early development. As a result, an opening, usually to the right of the umbilical cord, allows the baby’s intestines to protrude out of the abdomen and float in the amniotic fluid. This unprotected exposure to the amniotic fluid can cause the intestines to swell or shorten, and at times, blood flow to the intestines may be slowed or the bowel may twist around itself. This can result in complications and poor bowel function after the baby is born.
How Does Gastroschisis Happen?
Fetal Gastroschisis occurs approximately in 1 of 5,000 births. The exact cause is unknown and it is not known to be related to chromosomal abnormalities or genetic factors. It occurs most often in mothers who are in their teens and early twenties. Having one baby diagnosed with Gastroschisis does not increase the chances of it occurring in future pregnancies.
How is Gastroschisis Diagnosed?
This birth defect is most commonly diagnosed during the second trimester, but may be detected as early as 14 weeks. Mothers often have excessive amniotic fluid, elevated alpha-fetoprotein (AFP) levels, and the condition can be seen on ultrasound. Gastroschisis must be differentiated from other abdominal defects such as omphalocele – a congenital malformation where the abdominal contents protrude at the base of the umbilical cord into a sac outside the abdomen and multiple birth defects may be present.
Tests You May Undergo:
- Repeated, detailed ultrasounds - every 1 to 2 weeks to monitor the condition of the baby’s intestines
- Magnetic Resonance Imaging (MRI) - to detect intestinal damage
- Amniocentesis - for chromosome testing
- Maternal Serum Screening - for elevated AFP levels
What Does it Mean for My Baby?
If your baby is diagnosed with Fetal Gastroschisis, there is an increased risk of complications during the third trimester such as fetal bowel dilatation (stretching), decreased fetal growth and amniotic fluid volume, preterm delivery and very rarely, fetal death. Your Maternal-Fetal Care specialist will closely monitor your baby via a series of ultrasounds and fetal non-stress tests to observe the severity of the Gastroschisis, the condition of the baby’s intestines and overall well-being.
If My Baby is Diagnosed with Gastroschisis, What Happens Next?
You and your baby will be closely monitored for the remainder of your pregnancy. Because treatment involves surgery for your baby after birth and an extended stay in Intensive Care, you will need to plan to deliver your baby at a hospital equipped with a Newborn Intensive Care Unit (NICU) and an experienced pediatric surgical team. Your baby may be in the NICU for one month or more, depending on severity of the gastroschisis and recovery progress. Delivery may be scheduled as early as 36 weeks by C-section; however, the majority of women are able to have safe vaginal deliveries.
How is Gastroschisis Treated?
- Complete Surgical Repair
- When the amount of intestines outside of the abdomen is small and they are not inflamed or swollen, complete repair can often be done in one surgery, often immediately following birth.
- Staged Repair
- If the intestines are damaged, swollen or inflamed, your baby may need to have repair done in several stages. This usually takes three to 10 days, but can take longer depending on the baby’s condition. A protective silastic (“silo”) pouch will be placed over your baby’s intestines. Each day the silo will be tightened to gently push the intestines back into the abdomen. Once they are back in place, the pouch is removed and a surgeon will close the opening. It is common for babies to need help breathing with a ventilator during this time.
- Recovery in NICU
- After the intestines are back in the abdomen and the opening has been closed, your baby will need to continue to be closely monitored in the Newborn Intensive Care Unit for several weeks or more. Parents are encouraged to participate in the baby’s care as much as possible.
- In the NICU, your baby will be fed for the first few weeks intravenously (through an IV tube). Once the baby has a bowel movement, the intestines are considered to be functioning normally. Feedings through a naso-gastric (NG) tube will then be introduced along with the IV feedings. The NG feedings will be slowly increased as tolerated until the baby is able to have oral feedings. Mothers are encouraged to pump breast milk for the NG and oral feedings.
- Going Home
- Your baby will be released from the hospital when he or she is able to take all feedings by mouth, has proper bowel functioning, is gaining weight and has no signs of complications.
- Follow-Up Care
- Most babies born with Gastroschisis require follow up visits with a team of pediatric surgeons, pediatric gastroenterologists, nutritionists and primary care providers during the first year or more.
What is the Long-term Prognosis of Gastroschisis?
- Minimal to Zero Intestinal Damage
- Eight out of ten babies diagnosed with Gastroschisis have minimal to no damage to their intestines and are successfully treated after birth. They typically go on to develop normally.
- Moderate to Severe Intestinal Damage
- Survival rates for babies with gastroschisis is 95%. However, in rare cases, the baby may have significant damage to the intestines which can be life-threatening and may require multiple surgeries and a prolonged NICU stay. At times, surgeons may need to remove damaged parts of the intestine, and in the worst cases, there may not be enough intestine left to absorb nutrition. Babies most severely affected may not survive. Additionally, some babies may develop "short gut syndrome" - characterized by diarrhea, slow weight gain and difficulty absorbing essential vitamins and minerals. These babies may require prolonged IV nutrition and close monitoring by the pediatric surgeon, pediatric gastroenterologist and primary care physician team.