By: Antoinette Adjowa Amevor, MD, FAAP
When a baby is developing in the womb, everything must form in a specific way. With gastroschisis (Gas- TRO- Skee- SIS), the intestines and sometimes other organs move outside of the body through a hole near the belly button. The organs float in the amniotic fluid, which causes them to become twisted, swollen and shorter than usual.
Read how two moms found strength by connecting with others after their children were diagnosed with gastroschisis.
Most children with gastroschisis need surgery after birth to put the organs inside the belly and close the opening. They may need ongoing care to manage their condition and help them grow and thrive. Read on to learn about the types of gastroschisis, treatment and care needs.
What causes gastroschisis?
We do not know what causes gastroschisis. It may be more common in people who are young when they become pregnant. People who smoke or use alcohol or drugs while pregnant are also at an increased risk of having a baby with gastroschisis.
Types of gastroschisis
There are two types of gastroschisis: simple and complex.
Simple gastroschisis means that the intestines are outside of the abdomen at delivery.
Complex gastroschisis means that the intestines are outside of the abdomen and there is a related problem that affects the intestines. For example: part of the intestines are not formed properly (called atresia), twisting of the bowel (called volvulus), narrowing (called stricture) or tissue death (called necrosis/ischemia).
A rare and severe form of complex gastroschisis is called
vanishing gastroschisis (or closing gastroschisis). In this case, the belly wall closes over the intestines while the baby is still developing. This can cause serious damage to the bowel and may lead to intestinal failure or short bowel syndrome, which affects how the body absorbs nutrition and fluids.
How is gastroschisis diagnosed and treated?
Gastroschisis is detected during pregnancy or soon after a baby is born. It can be diagnosed before birth using
prenatal tests and an ultrasound during pregnancy.
After delivery, the baby will need surgery to put the organs back into the body or a sterile wrap or bag placed around the intestines (called a silo). The silo slowly moves the intestines back into the belly. Then, the surgeon can close the belly, either that same day or a few days later.
It will take time for the intestines and the hole to heal. Your baby will need to stay at the hospital and receive nutrition through the veins. Later, they will be fed through their gut. If some of the intestines must be removed, your baby may also need medications and IV nutrition for a bit longer.
Gastroschisis care for life
After hospital discharge, schedule your baby's next health visit. A baby with gastroschisis may continue to have problems with eating, digesting food, absorbing nutrients, moving food and waste through the intestines (dysmotility), and growth over time.
Parents should watch for problems like loose stools, slow weight gain, weight loss, abdominal pain or vomiting. A gastroenterologist or intestinal rehabilitation specialist can closely monitor for infection, fluid and nutrition status, and liver status.
Long-term management includes regular visits to your child's pediatrician, gastroenterologist, and sometimes a surgeon. The health care team can make sure your child is getting the right treatment so they can grow and lead a healthy life.
Finding support for gastroschisis
By: Christine Fowler & Gaby Luna
Life with gastroschisis has changed our families in every way and will forever be part of our lives. Through it all, we have found strength within our family and by connecting with other parents.
Emersyn's story, shared by her mother Christine Fowler
At my 20-week ultrasound, I learned that my baby girl had gastroschisis. If she survived, doctors told me that she would need multiple surgeries. Emersyn was born with many organs on the outside including her small bowel, stomach, liver and bladder. She needed surgery to put her
organs back into place. The organs that could not be put back right away were placed into a
silo bag. After three surgeries and 43 days in the NICU, my baby girl was able to come home.
Five years later, Emersyn needed surgery again. She started having severe stomach pain, so we went to the emergency department. After hours of X-rays and ultrasounds, we were sent home with a diagnosis of constipation. Less than 12 hours later, her pain came back so we returned to the emergency department for more tests. The doctor eventually ordered a
CT scan. Minutes later, she was taken for emergency surgery. Emersyn had a life-threatening condition called volvulus, in which the intestine twists around itself. Emersyn is now 8 years old. Every day, I worry that she might have another complication from gastroschisis.
Damian's story, shared by his mother Gaby Luna
Damian was born with a complex form of gastroschisis that left him with 10% of his small intestine. Since he was just a few days old, Damian has lived on nutrition daily through a long tube (called a central line) that is inserted into a vein in his chest. He also has a feeding tube in his stomach for medication and specialized food.
We quickly realized that we needed to seek out a center of excellence—experts who specialize in intestinal rehabilitation and have experience treating children with vanishing gastroschisis. Time is critical in these cases, and the right team can make all the difference. We knew we had to give him the best chance in life. Specialists who dedicate their careers and research to these rare conditions are life changing. They don't have all the answers, but they are always willing to partner with us for my son.
Damian is now 9 years old. Having a medical complexity like gastroschisis means life is filled with constant doctor's appointments, hospital admissions, therapies and the ongoing fight to ensure he has access to accommodations at school and daily activities.
Both of our families found support through the Global Gastroschisis Foundation. We connected with other parents through online communities. That support has given us knowledge, courage and strength. It taught us how to advocate for our child's survival and quality of life.
To any parent navigating this path: You are not alone. We encourage you to connect with other families. You are doing an incredible job, and it's okay to ask for help, to seek better care, and to fight for joy amidst the challenges.
Christine Fowler and Gaby Luna are members of the Global Gastroschisis Foundation. To learn more and get involved, visit
https://averysangels.org.
More information
About the author
Antoinette Adjowa Amevor, MD, FAAP, is a board-certified pediatrician, board-certified pediatric gastroenterologist, and board-certified physician nutrition specialist. She is an assistant professor of pediatrics at Emory University and medical director of the intestinal rehabilitation program at Children's Healthcare of Atlanta. She sub-specializes in advanced nutrition and intestinal rehabilitation.
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This article was supported by the cooperative agreement Number NU01DD000032, between the American Academy of Pediatrics and the Centers for Disease Control and Prevention.