Introduction
The baby's development in the mother's womb is a crucial and critical process during which the baby attains all the necessary growth and development intended to be present in a newborn baby. However, the baby's development in the womb is influenced by diverse factors predisposing the baby to develop impairment. All the abnormalities that arise in the baby while in the womb are generally categorized as birth defects or congenital defects. There is a wide spectrum of birth defects. However, each differs from one another and possesses unique clinical presentations. Such birth defects often necessitate an interdisciplinary and integrative management approach to improve longevity and quality of life.
What Is Omphalocele and Gastroschisis?
Omphalocele and gastroschisis are two distinct birth defects, with an incidence of around 0.025 percent among live births. However, recent research has emphasized a dramatic rise in the incidence of omphalocele and gastroschisis globally, which raises concern. Innovations in prenatal diagnostic strategies and interventions result in early detection of the condition, even before the baby is born. The sequelae of omphalocele and gastroschisis tend to differ individually based on the severity and intensity of the condition and the extent of structural and functional impairment precipitated by the defect. In both omphalocele and gastroschisis, the baby acquires defects in the abdominal wall.
In omphalocele, impaired muscle strength at the point of the belly button (navel) due to a faulty abdominal wall provides room for the stomach content to obtrude out through the defective point. As a result of the obliterated abdominal wall, the intestine, and other vital organs get exposed out of the abdomen. A thin protective layer encapsulates the protruded internal structures. The severity of the condition is assessed by the degree of the defect size. Syndromic associations are reported in around 30 percent of omphalocele cases, where the condition closely correlates with syndromes like Beckwith-Weidemann syndrome (aberrant and abnormal overgrowth of body structures).
In gastroschisis, the abdominal structures obtrude out just like omphalocele. However, the obtrusion is not through the navel. The defect often lies right and in line with the belly button. Through the defect, the bowel jets out. No intact protective sheath envelops the protruded bowel. About 25 percent of the babies born with gastroschisis exhibit associated gastrointestinal issues. The size and dimension of the defect vary, and it influences the severity of the condition. Abdominal viability and the impairment of the abdominal wall are critical in assessing the success rate.
Smaller defects restrict the jet out of the larger organs, like the liver, through it. Gastroschisis is categorized into two categories, taking into account the extent of internal structures that protrude out: simple and complicated gastroschisis. When only the intestine is bulged out through the defect, it is considered simple. In some cases, the obtruded bowel segment may lose its viability, occlude, or exhibit extensive bulging of the internal organs like the liver, posing complications. Those types of gastroschisis are considered to be complicated gastroschisis. Gastroschisis babies tend to elicit growth restriction.
How Are Omphalocele and Gastroschisis Managed?
Omphalocele and gastroschisis entail a multifaceted and integrative treatment approach. A panel encompassing neonatologists, fetal medicine specialists, and pediatric surgeons together work to deal with babies detected with omphalocele and gastroschisis.
1. Management Strategies of Gastroschisis:
With the advancement in prenatal monitoring techniques, most gastroschisis cases are detected before birth. Ultrasound scans clearly show the obtruded bowel suspended in the amniotic fluid. An obvious diagnosis of the condition is often made during the second trimester. However, the defect may occasionally get diagnosed early within the first trimester. Once the baby in the womb is detected with gastroschisis, scrutiny and monitoring of the fetus (baby in the womb) is critical.
Babies diagnosed with gastroschisis are more susceptible to fetal death or demise within the mother’s uterus (womb). Proper delivery planning brings more favorable results than spontaneous birth in babies with gastroschisis. The susceptibility for fetal death is appreciably more with babies upon attaining the full term of pregnancy. Hence, planned delivery, particularly during the late preterm stage, at around 37 weeks, is advised. The obtruded bowel segment is exposed to the amniotic fluid, which induces certain changes in the bowel. At times, it precipitates inflammation and death of the cells in the exposed bowel segments. Bowel cell death (necrosis) depreciates bowel viability and directly impacts fetal outcomes.
Babies born with stick-out bowels tend to elicit dramatic bodily fluid loss. To tackle excessive evaporative loss of fluid, a bowel bag is employed to safeguard the exposed bowel segment. Babies are often supported with fluid supplementation through the vein (intravenous). The standard and hallmark treatment strategy for gastroschisis is surgical repair by accommodating the exposed abdominal structures into the abdominal cavity without inflicting damage to any of the internal structures. It can be performed as a primary or delayed closure technique.
2. Management Strategies of Omphalocele:
The treatment strategies for omphalocele are analogous to that of gastroschisis. The exposed bowel is shielded with sterile wrapping to check fluid loss and intravenous fluid introduction to counterbalance the lost fluid from the body. The baby should be given antibiotics to extend protection against infections. The surgical consideration for omphalocele is determined after considering the defect size. Primary closure is the recommended treatment choice for smaller defects, which must be initiated before 72 hours post-delivery. For moderately larger defects, the repair is done through a silo bag (a mesh-like bag that guides the herniated segment of the bowel to move inside the baby’s abdomen). It is achieved through a delayed closure and may last a week to get all the bowel content into the abdomen. Hence, the exposed bowel advancement into the abdominal cavity occurs step by step. Larger defects often require complex surgical intervention, necessitating a patch to seal the defect.
Conclusion
Omphalocele and gastroschisis are inborn conditions arising from impairment and aberration during the organogenesis stage (during which the baby’s organs are formed). Both are two different forms of abdominal wall defect. In omphalocele, the herniated and protruded intestinal segments are enclosed by a skinny covering, while gastroschisis completely lacks covering. Instead, it directly exposes the intestinal segments to the external environment. The treatment intervention is often determined by considering the severity of the condition. Untreated omphaloceles and gastroschisis are potentially life-threatening. Therapeutic intervention should be initiated right from the moment of diagnosis of the condition. Proper prenatal care aids in checking intrauterine mortality associated with these conditions.
