1. Introduction

Gastroschisis is a birth defect of the abdominal wall, in most cases located to the right of the umbilicus and typically smaller than 2 cm, with the herniation of abdominal organs into the amniotic cavity [1, 2].

intestinal atresia, perforation, necrotic segments or volvulus. Simple gastroschisis was defined

Gastroschisis: Prenatal Diagnosis and Outcome http://dx.doi.org/10.5772/intechopen.74270 233

In addition, having good, early prenatal diagnosis of gastroschisis gives the possibility of following of the intrauterine development of fetus, identifying possible growth restriction or gastrointestinal obstruction. This could be helpful in making the strategy for perinatal treatment in a tertiary referral hospital. Having in mind that the prognosis of the infant born with gastroschisis depends primarily on the condition of the bowel at birth, researchers have tried to find sonographic predictors of bowel damage [4] which can be used to make decisions about the timing of delivery [12]. Recent researches show different results: some of them have shown connection between the dilatation of the herniated bowel and thickening of the bowel wall with postnatal gastrointestinal complications and poor outcomes [13]. Other studies, on the other hand, show that the dilatation of herniated bowel is not the predictor of poor outcome [14]. The results of our study did not show that there was a statistically significant difference in outcome between patients without prenatally detected bowel dilatation and those one who had dilated bowel prenatally identified. Future researches should be focused on discovering some other prenatal sonographic parameters in patients with gastroschisis which could be

as gastroschisis in absence of any of these conditions causing additional bowel damage.

Figure 2. Complex gastroschisis—gastroschisis associated with intrauterine necrosis and perforation of the bowel.

useful for poor outcome prediction.

Figure 1. Simple gastroschisis.

This anomaly occurs in approximately 0.01–0.06% of births and it could be detected prenatally in up to 95% of cases using the obstetric sonography. Identifying the gastroschisis by fetal echosonography is possible as early as in 12 weeks of gestation by observing the ventral wall defect with loops of bowel protruding outside the abdomen, floating freely in the amniotic fluid [3, 4].

Researchers still have not discovered the precise cause of gastroschisis [5]. Some of them claim that it is probably the results of mesenchymal damage and failure of the epidermis to differentiate at the site of the defect, which is caused by premature atrophy or abnormal persistence of the right umbilical vein [6]. Others have proposed that the condition may be caused by ischemia of the base of the cord due to intrauterine disruption of the right omphalomesenteric artery, which than lead to herniation of the gut through this infarcted area [7].

Furthermore, it is very important to make the distinction between various ventral wall defects, especially between gastroschisis and omphalocele [8]. Omphalocele is characterized by a midline defect of abdominal muscles, fascia, and skin at the umbilicus, resulting in herniation of intraabdominal structures into the base of the umbilical cord. The herniated organs in omphalocele are always covered by an amniotic membrane, unlike to gastroschisis where the abdominal organs float freely in amniotic cavity, exposed to potentially negative influence of amniotic fluid [1]. However, it is proved that gastroschisis has got much better prognosis, because it is, on contrary to omphalocele, not associated with chromosomal abnormalities and usually not associated with other structural anomalies. Survival rates of gastroschisis are more than 90% [9, 10].

On the other hand, infants with gastroschisis are at high risk of postnatal complications, especially gastrointestinal ones. The most common gastroschisis-associated gastrointestinal anomaly is intestinal atresia [3]. Besides, we can identify intestinal stenosis, as one of the most common bowel complication, probably caused by lack of the blood flow as a result of compression of eviscerated bowel at the site of the abdominal wall defect. Intestinal atresia as well as other intestinal anomalies could lead to bowel dysmotility, peritonitis, small-bowel pseudo obstruction, perforation, necrotizing enterocolitis, cholestatic jaundice, short-bowel syndrome, vomiting, and fistulas. In fact, the most significant factor determining the prognosis is the condition of the bowel at birth. The more the bowel is damaged, the worse the long-term outcome is. If the bowel distension, atresia, or necrosis is identified, primary repair with the resection of necrotic segment could be usually done; however, staged repair is sometimes required. These infants often have long-term morbidity caused by severe intestinal hypoperistalsis and poor absorptive capacity, requiring prolonged or permanent parenteral nutrition with its associated risks of infection, growth restriction, metabolic disturbances, and liver disease.

Regarding to all above-mentioned parameters, researchers Molik et al. [11] proposed classification of gastroschisis into simple (Figure 1) and complex (Figure 2) cases. Complex gastroschisis was defined as gastroschisis associated with at least one of the following intestinal pathologies: intestinal atresia, perforation, necrotic segments or volvulus. Simple gastroschisis was defined as gastroschisis in absence of any of these conditions causing additional bowel damage.

In addition, having good, early prenatal diagnosis of gastroschisis gives the possibility of following of the intrauterine development of fetus, identifying possible growth restriction or gastrointestinal obstruction. This could be helpful in making the strategy for perinatal treatment in a tertiary referral hospital. Having in mind that the prognosis of the infant born with gastroschisis depends primarily on the condition of the bowel at birth, researchers have tried to find sonographic predictors of bowel damage [4] which can be used to make decisions about the timing of delivery [12]. Recent researches show different results: some of them have shown connection between the dilatation of the herniated bowel and thickening of the bowel wall with postnatal gastrointestinal complications and poor outcomes [13]. Other studies, on the other hand, show that the dilatation of herniated bowel is not the predictor of poor outcome [14]. The results of our study did not show that there was a statistically significant difference in outcome between patients without prenatally detected bowel dilatation and those one who had dilated bowel prenatally identified. Future researches should be focused on discovering some other prenatal sonographic parameters in patients with gastroschisis which could be useful for poor outcome prediction.

Figure 1. Simple gastroschisis.

1. Introduction

232 Congenital Anomalies - From the Embryo to the Neonate

amniotic cavity [1, 2].

Gastroschisis is a birth defect of the abdominal wall, in most cases located to the right of the umbilicus and typically smaller than 2 cm, with the herniation of abdominal organs into the

This anomaly occurs in approximately 0.01–0.06% of births and it could be detected prenatally in up to 95% of cases using the obstetric sonography. Identifying the gastroschisis by fetal echosonography is possible as early as in 12 weeks of gestation by observing the ventral wall defect with loops of bowel protruding outside the abdomen, floating freely in the amniotic fluid [3, 4]. Researchers still have not discovered the precise cause of gastroschisis [5]. Some of them claim that it is probably the results of mesenchymal damage and failure of the epidermis to differentiate at the site of the defect, which is caused by premature atrophy or abnormal persistence of the right umbilical vein [6]. Others have proposed that the condition may be caused by ischemia of the base of the cord due to intrauterine disruption of the right omphalomesenteric

Furthermore, it is very important to make the distinction between various ventral wall defects, especially between gastroschisis and omphalocele [8]. Omphalocele is characterized by a midline defect of abdominal muscles, fascia, and skin at the umbilicus, resulting in herniation of intraabdominal structures into the base of the umbilical cord. The herniated organs in omphalocele are always covered by an amniotic membrane, unlike to gastroschisis where the abdominal organs float freely in amniotic cavity, exposed to potentially negative influence of amniotic fluid [1]. However, it is proved that gastroschisis has got much better prognosis, because it is, on contrary to omphalocele, not associated with chromosomal abnormalities and usually not associated with

On the other hand, infants with gastroschisis are at high risk of postnatal complications, especially gastrointestinal ones. The most common gastroschisis-associated gastrointestinal anomaly is intestinal atresia [3]. Besides, we can identify intestinal stenosis, as one of the most common bowel complication, probably caused by lack of the blood flow as a result of compression of eviscerated bowel at the site of the abdominal wall defect. Intestinal atresia as well as other intestinal anomalies could lead to bowel dysmotility, peritonitis, small-bowel pseudo obstruction, perforation, necrotizing enterocolitis, cholestatic jaundice, short-bowel syndrome, vomiting, and fistulas. In fact, the most significant factor determining the prognosis is the condition of the bowel at birth. The more the bowel is damaged, the worse the long-term outcome is. If the bowel distension, atresia, or necrosis is identified, primary repair with the resection of necrotic segment could be usually done; however, staged repair is sometimes required. These infants often have long-term morbidity caused by severe intestinal hypoperistalsis and poor absorptive capacity, requiring prolonged or permanent parenteral nutrition with its associated risks of infection,

Regarding to all above-mentioned parameters, researchers Molik et al. [11] proposed classification of gastroschisis into simple (Figure 1) and complex (Figure 2) cases. Complex gastroschisis was defined as gastroschisis associated with at least one of the following intestinal pathologies:

artery, which than lead to herniation of the gut through this infarcted area [7].

other structural anomalies. Survival rates of gastroschisis are more than 90% [9, 10].

growth restriction, metabolic disturbances, and liver disease.

Figure 2. Complex gastroschisis—gastroschisis associated with intrauterine necrosis and perforation of the bowel.

Although numerous articles considering the optional management of gastroschisis are available in the professional literature, it still remains controversial. There are two preferable methods in operative approaches: primary closure and staged closure using customized silo [15]. Primary surgical repair is the method of choice of gastroschisis treatment at our Institution, whenever it is feasible. During the last decade, we have moved toward staged reduction of the herniated intestines (without intestinal anomalies) into the abdominal cavity using a silo (Figure 3), which is then followed by elective abdominal wall closure (Figure 4). We also reviewed differences of the outcome of newborn with simple and complex gastroschisis treated at our Institution over the past 15 years and tried to identify factors associated with mortality [9]. In majority of published studies reported mortality of gastroschisis is less than 10%, which is mostly the result of development of the modern neonatal intensive care, advanced pediatric surgery, and new prenatal diagnostic procedures [4, 16]. All this provides better results in overcoming the gastroschisis-associated complications such as extensive intestinal loss, short bowel syndrome, prolonged total parenteral nutrition, liver failure, sepsis, and early baby death.

Several studies have examined the different outcomes in fetuses with gastroschisis [14], but most of them have included all abdominal wall defects, not just gastroschisis [17, 18]. In future, more researches should be focused on discovering the factors which could indicate the presence of complex gastroschisis. Besides, new studies should provide improvement of prenatal diagnosis and postnatal management.

> The aim of this study was to investigate and identify prenatal predictors of perinatal outcomes of gastroschisis. Also, we tried to compare the outcomes in infants with simple and complex

> Figure 4. Eviscerated organs removed form a silastic bag, preparing for reducing into abdominal cavity and abdominal

Gastroschisis: Prenatal Diagnosis and Outcome http://dx.doi.org/10.5772/intechopen.74270 235

We performed a retrospective cohort study where we included all the patients treated at the Institute for Mother and Child Health Care of Serbia "Dr. Vukan Čupić" in period 2001–2017 with the diagnosis of gastroschisis (n = 70). The exclusion parameters were prematurity (<34 weeks of gestation) and birth weight less than 1500 g, so the five patients were excluded (n = 5). We used patient records and neonatal intensive care unit database to obtain infant birth

Antenatal data included extra-abdominal bowel dilatation (EABD) (bowel diameter ≥ 18 mm) and in particular intra-abdominal bowel dilatation (IABD). Perinatal data included birth age, gestational age, sex, and birth weight. Surgical records included presence of intestinal atresia, necrosis, perforation, strictures, and method of closure. Outcome data included duration of mechanical ventilation, duration of total parenteral nutrition, pseudoobstruction, sepsis (cen-

history, demographic and clinical parameters that were necessary for this research.

tral line infection), reoperations, length of hospital stay, and mortality rates.

gastroschisis.

wall defect closure.

2. Material and methods

Figure 3. Eviscerated organs placed into silastic bag.

The aim of this study was to investigate and identify prenatal predictors of perinatal outcomes of gastroschisis. Also, we tried to compare the outcomes in infants with simple and complex gastroschisis.
