4. Discussion

patients depended primarily on characteristics of each case, which were quite heterogeneous. Routine silastic bag closure was performed in 21 patients (42%) with simple gastroschisis, and

EABD diameter (mm) Complex gastroschisis n = 15 Simple gastroschisis n = 50

Complexity group (n = total in group) Simple (n = 50) Complex (n = 15)

Number (% of complexity group) 16 (32%) 11 (73%)

Number (% of complexity group) 7 (14%) 10 (66%)

Number (% of complexity group) 4 (8%) 6 (40%)

Number (% of complexity group) 3 (6%) 1 (7%)

Number (% of complexity group) 7 (14%) 3 (20%)

<10 0 4 (5%) 10 to <18 7 (47%) 10 (20%) ≥18 11 (73%) 16 (32%)

<10 0 0

10 to <18 3 (20%) 11 (27.5%) ≥18 12 (82%) 29 (72%)

IABD diameter (mm) Complex gastroschisis, n = 15 Simple gastroschisis, n = 50

Number (% in group) Number (% in group)

Number (% in group) Number (% in group)

Four patients with simple gastroschisis (8%) did not survive to be discharged. We identified gangrene of the bowels which were placed into silastic bag in two patients. Abdominal compartment syndrome was documented in two patients who were treated with primary closure. The need for repeated laparotomies was a result of various complications: sepsis, persistent metabolic acidosis, respiratory compromise, low urine output, and poor perfusion.

19 patients (90.47%) of them had no complications.

Table 4. Degree of extra-abdominal bowel dilatation (EABD).

IABD at last scan

IABD at ≥30 weeks to ≤34 GA

Table 2. All intra-abdominal bowel dilatation (IABD).

238 Congenital Anomalies - From the Embryo to the Neonate

Table 3. Degree of intra-abdominal bowel dilatation (IABD).

IABD at <30 weeks GA

Resolved IABD

Never had IABD

Recent studies have shown significant increase in the incidence of gastroschisis during the past 20 years [19]. The incidence of gastroschisis is as high as 4.4 per 10,000 live births, and it is proved that it depends on the mother's age [1, 20]. In fact, the incidence is several times higher in women younger than 20 years than in women 25–29 years old [1].

In our study, we classified gastroschisis cases into simple and complex, according to the presence of associated bowel damage such as atresia, perforation, and necrosis, as these factors may have influence on the way of treatment and outcomes. Reviews in large national databases in Great Britain and the United States have shown that complex gastroschisis represents 11.5% and 10.9% of all cases, respectively [21]. The prevalence of complex gastroschisis in recent publications has been reported as 11–31% [21, 22]. In our cohort study, 77% of infants had simple gastroschisis, in other words almost 1 in 4 patients had complex gastroschisis. Researchers have proved that the only factor that has been consistently shown to predict poorer outcomes of this anomaly is the presence of complex gastroschisis [23, 24]. The results we obtained in our study show the same. Also, the presence of complex gastroschisis could lead to prolonged duration of total parenteral nutrition, ventilation support, and hospital stay. On the other hand, in our study, as well in some others, mortality did not differ significantly between simple and complex gastroschisis [9, 10, 22]. Some previous researches, on contrary, claim that intestinal complications are associated with higher mortality, as high as 28% [10, 11]. We also try to investigate possible relationship between intra-abdominal and extra-abdominal bowel dilatation with poor outcome. Some studies have shown that antenatal bowel dilatation (bowel diameter ≥ 18 mm) and in particular intra-abdominal bowel dilatation (IABD) is a useful predictor for impending necrosis or atresia and for bad outcome [25, 26]. On the other hand, one study shows [12] that 19% of complex patients never had IABD and the other one shows [27] that 75% never had extra-abdominal bowel dilatation (EABD). Therefore, the absence of bowel dilatation cannot fully exclude complex patients with gastroschisis. However, combined IABD/EABD or IABD/collapsed extra-abdominal bowel is highly suggestive to complex gastroschisis [28].

atresia who developed a terminal ileum perforation. The echogenic material spillage at the margin of a bowel loop near the site of the abdominal wall defect led to diagnosis of an acute intrauterine bowel perforation. The perforation has been identified proximal to an atretic segment of ileum. Finally, the patient was treated by resection of the 14 cm of bowel and ileostomy, which were followed by primary fascial repair. The operation outcome was favorable [28]. Furthermore, a case of child with gastroschisis and fetal eviscerated gastric perforation was reported by Tseng and Chou. The antenatal sonography identified a mural thickening of the triple layered gastric segment, a concave deformity of the inner layer, and a small nodule on the outer surface. Firstly, the small perforation over the greater curvature of the stomach was repaired and then staged operations were performed in the aim to reduce the exposure of the bowel loops out of the abdominal cavity [31]. Next case was published by Yang et al.: a patient where the gastric perforation was diagnosed during surgical repair of complex gastroschisis (associated with colon atresia) [25]. Marinović et al. reported case of gastroschisis with gastric perforation and intestinal stenosis in male newborn. Prenatal sonographic findings were inconclusive. Large gastric perforation was diagnosed during surgical repair of

Gastroschisis: Prenatal Diagnosis and Outcome http://dx.doi.org/10.5772/intechopen.74270 241

Having diagnosed some of above-mentioned factors prenatally may lead to consideration of an early delivery with the aim to salvage necrotic bowel, although these antenatal findings may indicate that the bowel damage has already occurred. On the other hand, early delivery is associated with prolonged time to achieve a full enteral feeding (FEF) and prolonged length of hospital stay (LHS), suggesting that elective delivery earlier than 37 weeks of gestation is not

Closing gastroschisis is rare, but potentially extremely complicated type of this anomaly. Houben et al. [35] published a research with the largest series of infants born with various stages of closing gastroschisis where 6% of infants present with closing abdominal ring. Recent literature shows low survival rate of an infant with closing gastroschisis [36–39]. In our research one patient (6.66%) presented with closing gastroschisis. Identifying progressive intra-abdominal bowel dilatation using prenatal ultrasonography could be indicative of a presence of closing abdominal ring complication. If there is a suspicion of a closing ring, early

There are many different methods of surgical treatment of gastroschisis. Reducing the evaporative and thermal loss is crucial primary goal. This could be accomplished by primary closure or staged closure using a silastic bag [15]. We found that the only factor that has been shown to predict poorer outcomes of gastroschisis is the presence of complex gastroschisis. Therefore, the strategy of surgical treatment should be focused on management of gastroschisis-associated conditions such as perforation, necrosis, stenosis, atresia, and short bowel syndrome. Our experience confirms the safety of an early restoration of bowel continuity and primary fascial closure. We found that much better outcome is associated with this way of treatment. Others have reported both early and late primary anastomosis as the safe options for treatment of gastroschisis associated with atresia [40]. By experience, surgeons may identify which patients have fascial defects more amenable to primary closure [30]. Our study favored primary closure because it is associated with significant reduction in length of hospital stay, total parenteral

gastroschisis [30].

beneficial.

delivery must be urgently considered [37].

Neonates with gastroschisis have delayed beginning of enteral feeding and prolonged time to achieve full enteral feeding (FEF), possibly due to bowel exposure to amniotic fluid. The research done by Yang et al. shows that IABD is associated with prolonged time to achieve full enteral feeding (FEF) and prolonged length of hospital stay (LHS) [25]. On the other hand, systematic review of isolated gastroschisis reported by Helen Carnaghan et al. [12] does not support those results; this study data on contrary show that neither EABD nor IABD could be predictors of increased poor neonatal outcomes [29]. However, it is proved that if the IABD and collapsed extra-abdominal bowel or both IABD/EABD are identified earlier than 30 weeks of gestation that could be more accurate predictor of poor outcome. Our study has shown that antenatal bowel dilation does not predict the poor outcome in infants with gastroschisis.

A PubMed literature search revealed fetal gastroschisis cases with intrauterine eviscerated bowel or stomach perforation in five reports [25, 27, 28, 30, 31]. There was one case with gastric perforation identified in prenatal period [28] and the other one where the gastric perforation was diagnosed during surgical repair of complex gastroschisis [27]. Two cases of gastroschisis had bowel perforation and intestinal atresia at the same time [25, 31].

The prenatal sonographic findings of bowel or gastric perforation are variable. According to literature, prenatal sonographic findings depend on the extent of meconium leakage, time when the bowel or gastric perforation occurred, the underlying bowel disorder, the stage of the disease, and the site of perforation [32]. The mechanism of gastric and bowel perforation in gastroschisis is multifactorial. Firstly, an amniotic fluid has got toxic effect on the muscle cells of bowel and stomach. Secondly, the amniotic fluid exerts negative effects on the vascular structures of mesentery causing mesenteric shortening which then leads to bowel ischemia. Besides, the lack of blood supply may be additionally caused by previously occurred vascular insult, as well as by constriction of the abdominal wall defect [33, 34]. All this could result in muscle layer thinning and interruption. Furthermore, if the perforation occurs, chemical irritation caused by dissipated meconium leads to an inflammatory reaction which initiates bowel mural thickening (causing stenosis) and adhesions creation (causing external bowel obstruction).

Grundy et al. presented a case of an infant with gastroschisis and intrauterine bowel perforation. The suspicion of fetal bowel perforation was indicated by the presence of calcifications on the surface of the extra-abdominal bowel as well as by the presence of the extra-abdominal intramesenteric pseudocyst. Unfortunately, the neonate died third day after birth [27]. Another case was presented by Haberman et al.: a neonate with gastroschisis associated with bowel atresia who developed a terminal ileum perforation. The echogenic material spillage at the margin of a bowel loop near the site of the abdominal wall defect led to diagnosis of an acute intrauterine bowel perforation. The perforation has been identified proximal to an atretic segment of ileum. Finally, the patient was treated by resection of the 14 cm of bowel and ileostomy, which were followed by primary fascial repair. The operation outcome was favorable [28]. Furthermore, a case of child with gastroschisis and fetal eviscerated gastric perforation was reported by Tseng and Chou. The antenatal sonography identified a mural thickening of the triple layered gastric segment, a concave deformity of the inner layer, and a small nodule on the outer surface. Firstly, the small perforation over the greater curvature of the stomach was repaired and then staged operations were performed in the aim to reduce the exposure of the bowel loops out of the abdominal cavity [31]. Next case was published by Yang et al.: a patient where the gastric perforation was diagnosed during surgical repair of complex gastroschisis (associated with colon atresia) [25]. Marinović et al. reported case of gastroschisis with gastric perforation and intestinal stenosis in male newborn. Prenatal sonographic findings were inconclusive. Large gastric perforation was diagnosed during surgical repair of gastroschisis [30].

We also try to investigate possible relationship between intra-abdominal and extra-abdominal bowel dilatation with poor outcome. Some studies have shown that antenatal bowel dilatation (bowel diameter ≥ 18 mm) and in particular intra-abdominal bowel dilatation (IABD) is a useful predictor for impending necrosis or atresia and for bad outcome [25, 26]. On the other hand, one study shows [12] that 19% of complex patients never had IABD and the other one shows [27] that 75% never had extra-abdominal bowel dilatation (EABD). Therefore, the absence of bowel dilatation cannot fully exclude complex patients with gastroschisis. However, combined IABD/EABD or IABD/collapsed extra-abdominal bowel is highly suggestive to

Neonates with gastroschisis have delayed beginning of enteral feeding and prolonged time to achieve full enteral feeding (FEF), possibly due to bowel exposure to amniotic fluid. The research done by Yang et al. shows that IABD is associated with prolonged time to achieve full enteral feeding (FEF) and prolonged length of hospital stay (LHS) [25]. On the other hand, systematic review of isolated gastroschisis reported by Helen Carnaghan et al. [12] does not support those results; this study data on contrary show that neither EABD nor IABD could be predictors of increased poor neonatal outcomes [29]. However, it is proved that if the IABD and collapsed extra-abdominal bowel or both IABD/EABD are identified earlier than 30 weeks of gestation that could be more accurate predictor of poor outcome. Our study has shown that antenatal bowel dilation does not predict the poor outcome in infants with gastroschisis.

A PubMed literature search revealed fetal gastroschisis cases with intrauterine eviscerated bowel or stomach perforation in five reports [25, 27, 28, 30, 31]. There was one case with gastric perforation identified in prenatal period [28] and the other one where the gastric perforation was diagnosed during surgical repair of complex gastroschisis [27]. Two cases of gastroschisis

The prenatal sonographic findings of bowel or gastric perforation are variable. According to literature, prenatal sonographic findings depend on the extent of meconium leakage, time when the bowel or gastric perforation occurred, the underlying bowel disorder, the stage of the disease, and the site of perforation [32]. The mechanism of gastric and bowel perforation in gastroschisis is multifactorial. Firstly, an amniotic fluid has got toxic effect on the muscle cells of bowel and stomach. Secondly, the amniotic fluid exerts negative effects on the vascular structures of mesentery causing mesenteric shortening which then leads to bowel ischemia. Besides, the lack of blood supply may be additionally caused by previously occurred vascular insult, as well as by constriction of the abdominal wall defect [33, 34]. All this could result in muscle layer thinning and interruption. Furthermore, if the perforation occurs, chemical irritation caused by dissipated meconium leads to an inflammatory reaction which initiates bowel mural thickening (causing stenosis) and adhesions creation (causing external bowel

Grundy et al. presented a case of an infant with gastroschisis and intrauterine bowel perforation. The suspicion of fetal bowel perforation was indicated by the presence of calcifications on the surface of the extra-abdominal bowel as well as by the presence of the extra-abdominal intramesenteric pseudocyst. Unfortunately, the neonate died third day after birth [27]. Another case was presented by Haberman et al.: a neonate with gastroschisis associated with bowel

had bowel perforation and intestinal atresia at the same time [25, 31].

complex gastroschisis [28].

240 Congenital Anomalies - From the Embryo to the Neonate

obstruction).

Having diagnosed some of above-mentioned factors prenatally may lead to consideration of an early delivery with the aim to salvage necrotic bowel, although these antenatal findings may indicate that the bowel damage has already occurred. On the other hand, early delivery is associated with prolonged time to achieve a full enteral feeding (FEF) and prolonged length of hospital stay (LHS), suggesting that elective delivery earlier than 37 weeks of gestation is not beneficial.

Closing gastroschisis is rare, but potentially extremely complicated type of this anomaly. Houben et al. [35] published a research with the largest series of infants born with various stages of closing gastroschisis where 6% of infants present with closing abdominal ring. Recent literature shows low survival rate of an infant with closing gastroschisis [36–39]. In our research one patient (6.66%) presented with closing gastroschisis. Identifying progressive intra-abdominal bowel dilatation using prenatal ultrasonography could be indicative of a presence of closing abdominal ring complication. If there is a suspicion of a closing ring, early delivery must be urgently considered [37].

There are many different methods of surgical treatment of gastroschisis. Reducing the evaporative and thermal loss is crucial primary goal. This could be accomplished by primary closure or staged closure using a silastic bag [15]. We found that the only factor that has been shown to predict poorer outcomes of gastroschisis is the presence of complex gastroschisis. Therefore, the strategy of surgical treatment should be focused on management of gastroschisis-associated conditions such as perforation, necrosis, stenosis, atresia, and short bowel syndrome. Our experience confirms the safety of an early restoration of bowel continuity and primary fascial closure. We found that much better outcome is associated with this way of treatment. Others have reported both early and late primary anastomosis as the safe options for treatment of gastroschisis associated with atresia [40]. By experience, surgeons may identify which patients have fascial defects more amenable to primary closure [30]. Our study favored primary closure because it is associated with significant reduction in length of hospital stay, total parenteral nutrition, and days with ventilation support. On the other hand, primary closure may cause abdominal hypertension and abdominal compartment syndrome which then could lead to an ischemia and necrosis of the bowel, renal failure, respiratory distress, and sepsis [41]. Abdominal hypertension is defined as prolonged or repeated increase in intraperitoneal pressure above 12 mmHg [42]. In fact, when the bowels are reduced into the non-sufficiently developed abdominal cavity, the raise of intraperitoneal pressure leads to compression of the blood vessels causing mesenteric ischemia and bowel necrosis. This kind of ischemia occurs very fast because the bowels in gastroschisis have got, in most cases, primarily lower perfusion than the bowels of a healthy infant [43]. Besides, an increased intra-abdominal pressure reduces the blood flow in big veins leading to diminished heart preload which then causes lower cardiac output and global hypoperfusion. This has an influence on renal blood flow making the renal hypoperfusion, anuria, and renal failure. We identified two patients in our study who received primary fascial repair and had abdominal compartment syndrome (Figure 5). So, it is very important that surgeon considers many different parameters when making the decision regarding the way of treatment of various cases of gastroschisis. In case when the primary closure without risk of abdominal compartment syndrome is not possible, surgeon should perform stage closure using a silastic bag which use becomes routine in many healthcare centers around the world. The criterion which strictly indicates that the silastic bag treatment should be performed is the presence of antenatal type of gastroschisis. Regarding to the duration of the exposure of the eviscerated bowels to amniotic fluid and the degree of abdominal cavity development, Moore classified gastroschisis into antenatal and prenatal types [44]. In antenatal type, the abdominal wall defect appears in early pregnancy so the bowels are exposed to negative influence of the aminotic fluid for a long time. Postnatally, the bowels are covered with gelatinous matrix, with a lot of adhesions and shortened mesentery. This type is also characterized by non-sufficiently developed abdominal cavity and the fascial defect which tends to become smaller and more tight during the intrauterine development. The closing of the fascial defect leads to an intrauterine bowel ischemia, which causes thickening of the intestinal wall, creating of the intestinal peel, stenosis, necrosis, and perforation. On the other hand, surgeon is not allowed to apply silastic bag if there is a possibility that tight fascial defect

could compromise eviscerated organs' blood flow. In that case, it is very important that surgeon performs an urgent fasciotomy before placing the eviscerated organs into silastic bag. The bad evaluation of fascial defect size and skipping the urgent fasciotomy may lead to bowel ischemia and necrosis. In our study, we identified two patients who had developed gangrene of the bowels placed in the silastic bag without having fasciotomy previously performed (Figure 6). On contrary, in prenatal type of gastroschisis, the abdominal wall defect appears later in pregnancy, so the bowels are not as damaged as in antenatal type, and the abdominal cavity is much more developed. This type of gastroschisis could be treated by the

Gastroschisis: Prenatal Diagnosis and Outcome http://dx.doi.org/10.5772/intechopen.74270 243

Finally, the length of hospital stay in patients with complex gastroschisis is in most cases based on additional intestinal complications they have, so it is often prolonged, regardless to way of

The only factor that has been shown to predict poorer outcomes of gastroschisis is the presence of complex gastroschisis. Therefore, the strategy of surgical treatment should be focused on the management of gastroschisis-associated conditions such as perforation, necrosis, stenosis, atresia, and short bowel syndrome. Our experience confirms the safety of an early restoration of bowel continuity and primary fascial closure. We found that much better outcome is associated with this way of treatment. More researches should be focused on finding of complex gastroschisis predictors, improvement of prenatal diagnosis, and

Current available evidence suggests that antenatal bowel dilatation is not associated with increased risk of adverse perinatal outcome in infants with gastroschisis. Also, the absence of bowel dilatation cannot fully exclude complex patients. However, a randomized controlled

primary closure.

Figure 6. Necrosis of the bowel placed into silastic bag.

5. Conclusions

postnatal management.

trial is urgently needed.

closure [9].

Figure 5. Massive necrosis of the bowel due to abdominal compartment syndrome.

Figure 6. Necrosis of the bowel placed into silastic bag.

could compromise eviscerated organs' blood flow. In that case, it is very important that surgeon performs an urgent fasciotomy before placing the eviscerated organs into silastic bag. The bad evaluation of fascial defect size and skipping the urgent fasciotomy may lead to bowel ischemia and necrosis. In our study, we identified two patients who had developed gangrene of the bowels placed in the silastic bag without having fasciotomy previously performed (Figure 6). On contrary, in prenatal type of gastroschisis, the abdominal wall defect appears later in pregnancy, so the bowels are not as damaged as in antenatal type, and the abdominal cavity is much more developed. This type of gastroschisis could be treated by the primary closure.

Finally, the length of hospital stay in patients with complex gastroschisis is in most cases based on additional intestinal complications they have, so it is often prolonged, regardless to way of closure [9].
