Gastroschisis: 7 Critical Facts on Congenital Abdominal Wall Defect

Gastroschisis: 7 Critical Facts on Congenital Abdominal Wall Defects

Gastroschisis is a dramatic congenital defect characterized by a full-thickness opening in the abdominal wall. In most cases, this opening is located to the right of the umbilical cord insertion. Through this gap, the baby’s intestines—and sometimes other organs like the stomach or liver—protrude outside the body. 

Unlike a similar condition called omphalocele, the herniated organs in gastroschisis are not covered by a protective membrane or sac. This leaves the delicate intestines exposed directly to the amniotic fluid during pregnancy, which causes them to become swollen, thickened, and covered with a fibrous "peel." This exposure makes the management of gastroschisis a complex surgical and neonatal challenge.

1. Core Features and Physical Appearance

The hallmark of gastroschisis is the visible protrusion of bowel loops at birth. Because there is no sac, the intestines are at high risk for infection, temperature loss, and dehydration. The surrounding skin of the umbilicus is usually normal, with the defect strictly located to the right side.

  • The hole is typically located to the right of the umbilicus.
  • Organs are not covered by a protective sac (unlike omphalocele).
  • Exposed organs are prone to swelling and infection due to fluid contact.

2. Exploring Potential Causes and Risk Factors

While the exact cause remains unknown, gastroschisis is thought to result from a vascular disruption of the omphalomesenteric artery during early fetal development. Interestingly, statistical data shows a strong correlation between this condition and young maternal age.

  • Maternal age under 20 years.
  • Maternal smoking or substance use.
  • Nutritional deficiencies during the first trimester.
  • Environmental toxins or specific medications.

3. Prenatal Diagnosis and Monitoring

Advancements in prenatal care allow most cases of gastroschisis to be diagnosed well before birth. Routine ultrasounds at 18–20 weeks can clearly identify the bowel loops floating in the amniotic fluid. Early diagnosis is crucial for planning the delivery in a specialized center equipped with a Neonatal Intensive Care Unit (NICU).

  • Prenatal ultrasound (detected at 18–20 weeks).
  • Elevated Alpha-Fetoprotein (AFP) in maternal blood tests.

4. Immediate Postnatal Stabilization

As soon as the baby is born, the exposed intestines must be protected. The lower half of the infant is placed in a sterile plastic bag (bowel bag) to maintain moisture and heat. Aggressive IV fluid resuscitation is started immediately to compensate for the fluid lost through the exposed bowel wall. A nasogastric tube is also used to decompress the stomach.

5. Surgical Management: Primary vs. Staged Repair

A pediatric surgeon determines the best method to return the organs to the abdomen. If the abdominal cavity is large enough, a Primary Repair is performed. If the intestines are too swollen, a Staged Repair is necessary.

  • Primary repair: Returning organs and closing the gap in one surgery.
  • Staged repair : Gradually returning organs over several days using a Silo bag to avoid high abdominal pressure.

6. Overcoming Intestinal Dysmotility

The biggest challenge after surgery is often the "paralyzed" state of the intestines (ileus). Because the bowel was irritated by amniotic fluid, it may take weeks for it to start moving and absorbing nutrients. During this time, the baby receives nutrition through an IV (Total Parenteral Nutrition).

7. Long-Term Prognosis

Despite the dramatic start, the long-term survival rate for gastroschisis is excellent, often exceeding 90%. Most children grow up without any digestive issues and develop normally, catching up to their peers within the first few years of life. 250464