Overview

Gastroschisis represents a herniation of abdominal contents through a paramedian fullthickness abdominal fusion defect. The abdominal herniation is usually to the right of the umbilical cord. No genetic association exists. A gastroschisis usually contains small bowel and has no surrounding membrane. The herniated bowel is not rotated and is devoid of secondary fixation to the posterior abdominal wall. [1] (See image below.)

Diagram of the transverse section of the fetal abdomen showing gastroschisis. Note the bowel herniation in the right paramedian/paraumbilical region. The cord is inserted in the normal location to the left of the herniation. No membranous covering exists over the herniated bowel.

Because the herniated bowel is bathed by amniotic fluid, both maternal serum and amniotic fluid alpha-fetoprotein (AFP) levels are elevated, more so than in exomphalos. Thus, gastroschisis is found incidentally or because of an elevated maternal AFP level, a finding in 77-100% of cases. Rarely, polyhydramnios may prompt an antenatal sonographic examination. Fetal growth restriction is a frequent association. Oligohydramnios is rare. Chromosomal anomalies are not associated with gastroschisis, and familial occurrence is exceptionally rare. Gastroschisis usually is detected in the second trimester using antenatal sonography. [2, 3, 4, 5, 6, 7, 8, 9, 10] The diagnosis can often be made by using antenatal sonography before 20 weeks' gestation. With transvaginal sonograms, the diagnosis has been made as early as 12 weeks' gestation. In early pregnancy, the bowel loops can be seen floating in the amniotic fluid. The thickness and the diameter of the bowel are normal. Later in pregnancy, bowel obstruction, peritonitis, bowel perforation, and fetal growth restriction may occur. Intrauterine growth restriction (IUGR) occurs in 38-77% of fetuses and is usually secondary to nutrient loss through exposed bowel. Approximately 48% of infants with gastroschisis are small for their gestational age.[11] A bowel diameter greater than 17 mm usually represents significant bowel dilatation, and diameters greater than 11 mm are usually associated with a greater number of postnatal bowel complications. Sonographic findings of bowel abnormalities are associated with difficult abdominal wall repair and an increased incidence of complications. Approximately 50% of fetuses with gastroschisis are small for their gestational dates. Fetal abdominal circumference, which is regarded as a standard reference for

assessment of fetal size, does not apply to this group of fetuses; therefore, obstetric management may be difficult. The mortality rate of gastroschisis is approximately 17%. Surgical repair should be offered within the first day after delivery to avoid infection. The outcome is no different in infants delivered in tertiary obstetric centers than in infants delivered in smaller peripheral hospitals, although delivery within easy access of a neonatal surgical unit is advised. Cesarean delivery is performed in many mothers of fetuses with gastroschisis, although this does not convey any advantage over vaginal delivery.

Preferred examination
Antenatal sonography is the key imaging examination available, with detection rates of 70-72%. Prenatal sonography is the primary imaging modality in pregnancy because it is noninvasive, is rapid, and allows real-time fetal examination. Plain radiographs and bowel contrast studies may be indicated in the postnatal postoperative period to assess bowel complications. With the use of antenatal sonography, the diagnosis of a surgically treatable malformation is made before birth in an increasing number of fetuses. This allows fetal intervention, in utero transfer, planned delivery in a specialized unit, and antenatal counseling of the parents regarding the likely prognosis and outcome.[12]

Limitations of techniques
Sonography remains operator dependent, and artifacts are a problem. Despite the straightforward nature of the defect, a diagnosis of gastroschisis can be missed. Misdiagnosis of exomphalos as gastroschisis has occurred in 5% of patients. This misdiagnosis has serious implications because exomphalos is often associated with chromosomal and other severe anomalies and karyotyping is not performed in patients with gastroschisis. In one case series, gastroschisis was misdiagnosed as exomphalos at a rate of 14.7%. This misdiagnosis results in unnecessary amniocentesis, which exposes the fetus to the risks involved in amniocentesis and which also exposes the mother to psychological trauma. Assessment of fetal size by using abdominal circumference measurements is difficult in the presence of gastroschisis. Postnatal plain radiographs and bowel-contrast studies lack specificity and expose the infant to a radiation burden. However, Siemer et al have developed a sonographic weight formula for fetuses with abdominal wall defects.[11] The authors evaluated their formula in a group of 97 fetuses with either gastroschisis or omphalocele and concluded that it provided a significantly greater accuracy in estimating fetal weight than a more commonly used formula. More data will be necessary to determine the utility of Siemer et al's formula.

Abstract and Introduction

Abstract

Gastroschisis is a congenital defect of the abdominal wall involving evisceration of abdominal contents. The incidence of gastroschisis is approximately 1.4 per 10,000 pregnancies and is rarely associated with other congenital anomalies. To provide appropriate care for these infants, the newborn/infant nurse must have a clear understanding of the embryology, etiology, and pathophysiology of the defect, and the ability to differentiate between gastroschisis and omphalocele. The purpose of this article is to provide the reader with a basic understanding of management strategies. Parents need to be provided with timely and accurate education and support as they cope with the stress of having an infant with gastroschisis. With appropriate care, these infants do well and have an essentially normal outcome.
Introduction

Gastroschisis, formally thought to be a variant of omphalocele, was first described in the 1940s. It was not recognized as having a different embryologic origin for almost 20 years after the first reported case. Until the 1970s, survival of infants with gastroschisis was relatively poor.[1] With increasing technology, mortality has been reduced, and survival is an expected outcome. Infants with gastroschisis present the health care team with numerous challenges from delivery to postdischarge. This article reviews the embryology, incidence, diagnosis, clinical manifestations, associated anomalies, management, complications, and follow-up needs for newborns and infants with gastroschisis. Gastroschisis is a defect in the abdominal wall causing evisceration of abdominal contents.[2] The defect usually occurs on the right side of the umbilical cord and is never enclosed in a peritoneal sac.[2,3] The vertical opening is approximately 2 to 5 cm in size, with the umbilicus normally developed and properly positioned.[4,5] The small and large intestines are usually the only organs protruding outside the abdominal wall (Fig 1). The spleen and liver may also be involved, but with a much lower incidence. [6] Malformations of other major organ systems are infrequently associated with gastroschisis; however, if malformations occur, they are commonly related to infarction or atresia of the herniated bowel.

Etiology

The etiology of gastroschisis is uncertain, but it has been reported to be of nongenetic origin.[8] Four hypotheses for the etiology of the defect have been proposed. The first is that gastroschisis may result from a vascular disruption of the right lateral fold allowing the abdominal contents to herniate outside the abdominal cavity.[2] The second is that the defect results from occlusion of the omphalomesenteric artery in utero. This occlusion may weaken the abdominal wall causing it to rupture.[4] The third hypothesis is that premature atrophy or abnormal persistence of the right umbilical vein leads to mesenchymal damage and failure of the epidermis to differentiate. [9] This damage or differentiation failure results in a defect of the abdominal wall. The fourth and last hypothesis is that a gastroschisis defect may be the end result of an intrauterine rupture of a small omphalocele with the absorption of the sac.

Incidence
During the last two decades, the overall incidence of gastroschisis is on the rise.[10] Although the exact reason is unknown, the rise may be secondary to improved case ascertainment, the increased accuracy of diagnosing gastroschisis from other abdominal wall defects,[11] and environmental risk factors.[10] The incidence of gastroschisis ranges from 1.4 to 2.5 per 10,000 live births and has no gender predilection.[7,9,10,12] Factors associated with an increased risk for gastroschisis include maternal age, parity, and maternal use of selected drugs. The incidence of gastroschisis is higher in young mothers and declines markedly with increasing maternal age.[2,13] Women less than 20 years of age are 11 times more likely to have an affected infant.[5] Low parity has also been shown to increase the risk for gastroschisis.[2] A study conducted by Sharp et al in 2000 found that 66% of mothers who delivered an infant with gastroschisis were in their first pregnancy, 26.4% were in their second, and 3.8% were in their third or greater pregnancy.[14] Drugs taken during the first trimester including nicotine, pseudo-ephedrine alone or in combination with acetaminophen, phenylpropanolamine, cocaine, aspirin, and acetaminophen are associated with an increased incidence of gastroschisis.[5,15-1

Embryological Basis
The embryologic development of gastroschisis involves maldevelopment of the abdominal wall. The abdominal wall is normally formed from the fusion of the cephalic, caudal, and right and left lateral folds of the mesoderm. These four folds usually meet and fuse in the center of the embryo to form the abdominal wall. Each of the

mesodermal folds creates different portions of the abdominal wall. The cephalic fold forms the upper abdomen; the caudal fold forms the lower abdomen; and the lateral folds transform into both sides of the abdominal wall. As the folds are fusing, rotation occurs forming the umbilical ring. These folds are approaching the midline of the abdomen as the intestinal tract is beginning to form. By the 10th week of gestation, the anatomical positions of the abdominal wall contents should be complete. A defect of the abdominal wall can result if any of these four folds do not develop correctly.

Differential Diagnoses
With advancements in prenatal testing and ultrasonography, abdominal wall defects are commonly diagnosed in utero as early as 12 to 14 weeks gestation. The differential diagnosis of abdominal wall defects includes gastroschisis and omphalocele. Gastroschisis is a defect in the abdominal wall lateral to the umbilical cord, whereas omphalocele is a defect in which the intestines are enclosed within the umbilical cord. It may be difficult to distinguish between the two diagnoses if the protective sac of the omphalocele has been ruptured.[3,19] It is important to remember that gastroschisis defects do not involve the umbilical cord. It is also essential to distinguish between the two defects because there is a higher incidence of major congenital/chromosomal anomalies associated with omphalocele.[7] The incidence of chromosomal anomalies associated with gastroschisis is less than 5%

Clinical Manifestations
The infant with gastroschisis typically presents with a small, underdeveloped abdominal cavity caused by evisceration of the intestines. Although the distal portions of the colon, liver, and other solid organs have the potential to protrude through the abdominal wall defect, these organs usually remain in the abdominal cavity.[19]Malrotation occurs almost universally because of the protrusion of the intestines outside the abdominal wall.[20] Exposure to amniotic fluid can cause the uncovered bowel to become inflamed, thickened, and edematous. The affected bowel can also appear as a matted mass with no identifiable loops. A peel over the serosal surface of the bowel can occur as a result of amniotic fluid exposure. This, in conjunction with a chemical peritonitis, may impede reduction of the intestine into the abdominal cavity.

Associated Anomalies

There is a lower incidence of associated anomalies with gastroschisis compared with other abdominal wall defects. A 10-year review of infants with gastroschisis found a 30% incidence of associated anomalies with intestinal atresia and cryptorchidism or undescended testes being the most common.[22] Intestinal atresia was noted in 22% of affected infants, while cryptorchidism was noted in 55%.[22] In a second 10-year review of infants with gastroschisis, ileal atresia occurred in 5.4% of affected infants and cryptorchidism occurred in 24%.[14] Cryptorchidism in infants with gastroschisis has an estimated occurrence of 31%.[23] Cryptorchidism is considered a minor anomaly that usually requires conservative management

Management
Management of the newborn with gastroschisis often occurs before birth, with the decision on the mode of delivery. Postnatal management includes presurgical stabilization and evaluation, surgical repair, and postsurgical care and follow-up. While numerous articles are available in the professional literature, no randomized clinical trials on any aspect of management have been published.

Mode of Delivery
While the goal of delivery of the newborn with gastroschisis is to optimize their outcome by minimizing trauma to the exposed gastrointestinal contents, the best mode of delivery for these infants remains controversial. From a theoretical standpoint, one might assume delivery by cesarean section would be more advantageous than vaginal delivery for several reasons. The first reason is a cesarean delivery is thought to produce less compromise to the mesenteric circulation because there may be less compression and twisting of the bowel during uterine contractions and passage through the birth canal. Another reason is that the risk of infection to the exposed bowel is decreased by cesarean delivery with intact membranes. The last theoretical disadvantage to vaginal delivery is if a large defect is present with possible liver involvement, there may be an increased risk for avulsion injury. While the rationale to promote cesarean delivery of the newborn with gastroschisis makes sense from a theoretical standpoint, none of these assumptions have been confirmed by clinical data.[24] No significant differences in outcomes between cesarean and vaginal delivery were noted in several studies of morbidity associated with gastroschisis and type of delivery.[22,25,26] The measures of morbidity in these studies included time to full oral feedings, duration of parenteral nutrition, age at discharge,

incidence of complications, and number of hospital days.[22,25] A recent meta-analysis of 15 clinical studies also concluded there is no significant relationship between mode of delivery and infant outcomes.[27] In addition to the outcomes previously listed, the metaanalysis also found no significant relationship between mode of delivery and rate of primary repair, neonatal sepsis, and pediatric mortality.[27]
Presurgical Management

Stabilization and preoperative management of the newborn with gastroschisis must take into consideration many factors, including thermoregulation, fluid volume status, gastric distention and intestinal compromise, infection, respiratory status, and preparation for surgery. Stability of the aforementioned factors is necessary before the impending surgical repair to optimize the infant's outcome. On delivery, the infant's trunk and lower extremities should be placed in a sterile plastic "bowel bag." The infant should be placed under a preheated radiant warmer and dried to prevent heat loss.[28] It is also important to ensure that the infant has a patent airway and a naso/orogastric tube in place to prevent gastric distention.[3]Endotracheal intubation may be indicated if the infant appears to be in distress, although decompression of the gastrointestinal tract may help reduce respiratory compromise.[4] The infant must be monitored for signs of hypothermia, respiratory distress, and shock. A thorough physical examination should also be performed to determine the presence of other anomalies.[3,7] Delivery room management of the infant with gastroschisis has included the use of sterile bowel bags and/or saline-soaked gauze dressings to prevent damage to the exposed intestines.[29] Sterile, moist gauze dressings, covered by a transparent plastic film have also been used to cover the exposed tissue. The use of moistened gauze dressings is associated with several problems including hypothermia caused by increased insensible water loss and radiant heat loss from the larger surface area of the exposed bowel, tissue trauma, and infection. The bowel bag is the most appropriate alternative.[28] Bowel bags provide a sterile environment for the exposed intestine and reduce the risk for contamination and tissue trauma. In addition, the bowel bag helps to prevent evaporative heat and fluid losses and enables pooling of fluid within the bag. This pooling of fluid can be measured to provide a more accurate assessment of fluid loss.[28] Once initial stabilization in the delivery room is achieved, the newborn is admitted to the neonatal intensive care unit (NICU) for further evaluation and stabilization before

surgical repair. Because the newborn with gastroschisis is at an increased risk for fluid loss because of the large surface area of exposed bowel, the newborn may present with symptoms of shock.[30] Fluid resuscitation with isotonic solutions such as normal saline or Ringer's lactate is recommended for the newborn in shock.[31,32] Fluid resuscitation is usually continued until the infant's urine output normalizes and/or blood gases indicate normal acid-base balance.[31] The maintenance fluid requirement for infants with gastroschisis is increased 2- to 3-fold because of the excessive losses through the exposed bowel.[31,32] When fluid orders are written, it is important to keep in mind the glucose load associated with the intravenous fluids. Placing the newborn with a gastroschisis on a dextrose solution at two to three times the usual maintenance rate increases the likelihood of hyperglycemia. The newborn with gastroschisis is at constant risk for hypothermia because of the large surface area of exposed bowel. The use of warm, saline-soaked gauze to cover the defect may increase the risk of hypothermia as the saline cools.[28] Care must be taken to prevent cold stress by keeping the infant on a radiant warmer or other external heat device, and by minimizing fluid and heat loss from the exposed bowel. [19] The infant must be continually assessed for signs of gastrointestinal compression before surgical repair. A naso/orogastric tube should be inserted and placed to intermittent suction to keep the bowel and stomach decompressed.[29,33] Decompression is important because it helps to prevent partial or total obstruction of blood flow and oxygenation to the bowel. If decompression does not occur, there is an increased risk for bowel necrosis secondary to the constriction of the exteriorized intestine through the small visceral defect. Decompression will also reduce the infant's risk for emesis and thus aspiration.[33] Bowel compromise can occur during positioning of the infant. Infants with gastroschisis should be positioned on their right side in a lateral decubitus position to enhance venous blood return from the gut.[29] The right lateral decubitus position also decreases the risk of decreased perfusion caused by compression or kinking of mesenteric vessels.[20] It is important to make frequent assessments of perfusion to the intestines with minimal handling of the exposed tissue. Diagnostic testing and antibiotic prophylaxis are the last two areas of presurgical management. While the specific tests may vary from NICU to NICU, the most common presurgical studies ordered include a baseline chest x-ray, complete blood count (CBC) with differential and platelets, arterial blood gas, serum electrolytes, blood glucose level, total protein, and a blood type and cross match.[3] Broad-spectrum antibiotics such as

ampicillin and gentamicin are started to decrease the risk of infection from bacterial contamination of the exposed bowel.[3

Surgical Management
Surgical management of the infant with gastroschisis remains controversial. While primary closure of the abdominal defect is the preferred surgical approach, each pediatric surgeon must subjectively assess the degree of abdominal wall tension anticipated before deciding the nature of the repair.[8,22] If primary closure cannot be obtained, the alternative management strategy is a staged silo repair. [31] Because of the increased risk of sepsis and hypovolemic shock, primary closure is considered in all cases where reduction does not cause hemodynamic or respiratory compromise.[7,33] Airway and intra-abdominal pressures should be kept less than 25 and 20 mm Hg, respectively, to prevent adverse hemodynamic consequences to other organs and tissues.[7] Strategies to achieve primary repair include stretching of the abdominal wall, evacuating the contents of the stomach and small bowel, irrigating meconium from the intestines, and enlarging the defect by leaving a fascial hernia. [20,35]If primary closure is attempted without sufficient space in the abdominal cavity, potential complications secondary to abdominal compartment syndrome may occur. [36,37] These complications are listed in Table 1 . If the surgeon is unable to achieve primary closure or if a primary closure leads to hemodynamic and/or ventilatory compromise, an alternative method of closure must be used. Currently, most surgeons use a silastic silo for gradual reduction of herniated abdominal contents (Fig 2). Secondary closure occurs at a later time when the intestinal contents fit within the abdominal cavity.[7,20]Closure of the silo is usually performed in stages over 7 to 10 days, with reduction of the silo occurring one to two times daily.

Figure 2. Silo reduction.

(Enlarge Image)

A variety of methods including umbilical tape ties, sutures, clamps, or staples are used for silo reduction.[20,38]While slow reduction of the silo reduces the risk of abdominal

compartment syndrome, the nurse must remember that the infant remains at risk for complications associated with abdominal compartment syndrome. The infant should be carefully assessed during and immediately after the reduction for complications. A major concern for nurses and family members is the issue of pain associated with the reduction process. To date there is only one published study addressing this topic.[39] Kimble et al prospectively collected data on 35 newborns with gastroschisis born between 1999 and 2001. They concluded analgesia for reduction is "safe if strict selection criteria are adhered to."[39] Infants with noticeable atresia, bowel perforation or ischemia, or clinical instability were excluded from the study. The investigators of a pilot study involving 14 infants with gastroschisis who underwent midgut reduction without anesthesia, concluded that the practice "appeared safe, carrying no additional morbidity or mortality."[40] Davies et al conducted a meta-analysis of the available literature from 1966 to 2002 on the issue of reduction with or without general anesthesia.[41] Although these investigators were unable to find any studies that meet their inclusion criteria, they concluded "there is no evidence from randomized clinical trials to support or refute the practice of ward reduction...." [41] The authors of both of these studies are from England. Silo reduction in the NICU in the United States is seldom performed under anesthesia, so the relevance of these studies to American practice is questionable. The issue of concern is whether the silo reduction is painful and warrants pain management. Based on clinical experience, the authors recommend that each infant undergoing a reduction procedure must be assessed for pain before, during, and after the reduction. Appropriate pharmacologic and nonpharmacologic pain therapies should be used for those infants who exhibit signs of pain or distress with the reduction.

Postsurgical Management
Initial postsurgical management of the infant with gastroschisis includes monitoring of vital signs, cardiovascular and respiratory status, fluid and electrolyte balance, and pain. After the repair, intra-abdominal pressure increases and can result in venous compression. Venous compression may compromise renal blood flow and the glomerular filtration rate, resulting in decreased urine output. A urinary catheter may be necessary to relieve bladder distention and to allow for a more accurate measurement of urine output.[4] Maintenance fluid requirements may need to be increased because of third spacing into the distended bowel and abdominal cavity. [29-31] Alterations in electrolyte balance may ensue from this shift of fluids. The postoperative infant may

require anywhere from 120 to 170 mL/kg/d of a crystalloid solution that is adjusted to provide for adequate tissue perfusion and urine output.[20] A large-bore naso/orogastric tube placed to intermittent suction is needed to prevent gastrointestinal distention caused by hypoperistalsis. Hypoperistalsis or adynamic ileus is frequently seen in the postoperative period and may persist for several weeks.[29] The initial gastrointestinal drainage is characteristically green because of the back up of biliary and pancreatic secretions in the immediate postoperative period. As gut motility improves, the drainage becomes clear in appearance. Volume loss from the gastric tube must be monitored, because it is possible for the infant to lose up to 100 mL/kg/d.[3] Replacement of these losses is necessary to maintain homeostasis. Because of the increased intra-abdominal pressure, close monitoring of respiratory status is essential for the first 48 to 72 hours postsurgery. Respiratory support, as indicated, is provided to optimize oxygenation and ventilation. The increase in abdominal pressure may interfere with optimal expansion of the diaphragm and venous return impeding both ventilation and oxygenation.[42] Some infants may benefit from mechanical ventilation or continuous positive airway pressure (CPAP) to maximize lung expansion, lung volume, and oxygenation.[33]Central venous pressure (CVP) must be monitored constantly during CPAP because of its hindering effect on the CVP. [33] Other infants may not tolerate CPAP because of increased abdominal distention from the increased airflow to the gastrointestinal track. A properly functioning naso/orogastric tube will minimize this risk. In the immediate postsurgical period, the infant with gastroschisis is usually returned to the NICU from the operating room intubated and on mechanical ventilation. While most infants can be extubated within 24 to 48 hours after surgery, infants who are small for gestational age, preterm, and/or have significantly increased intra-abdominal pressure may require a longer period of ventilator support.[31] High peak inspiratory pressures (PIP), usually greater than 25 cm H2O, should be avoided if possible to minimize adverse effects to the renal and intestinal perfusion.[4,29] High-frequency ventilation can be an acceptable alternative because its effects on intra-abdominal pressure are not as great.[4] After the initial stabilization period, the main goal of management is to provide adequate nutrition and pain management. Initially, the infant will require parenteral nutrition. Gut motility is delayed because of the chemical peritonitis that occurred when the intestinal contents were exposed to amniotic fluid. Delayed gut motility may persist for weeks after surgical repair and is often influenced by the severity of the defect and other

associated anomalies such as intestinal atresia.[29,31] Because of the postoperative ileus, total parenteral nutrition (TPN) is needed in all infants with gastroschisis and is usually initiated within 24 to 48 hours after surgery.[29,33] Because these infants may require TPN for weeks after surgery,[31] a central line is recommended. The minimal daily requirements for postoperative TPN are 90 to 100 kcal/kg/d, 3 g/kg/d of protein, 3 to 4 g/kg/d of intravenous lipids, and dextrose to maintain euglycemia.[43] Because of protein losses from the surgical stress, wound healing, and/or third spacing, additional protein in the TPN may be necessary.[43] Once gut motility returns, it is important to be proactive with the initiation of enteral feedings. A retrospective study found the age of initial enteral feeding was positively correlated with the time of discharge.[14] These investigators also noted that for every additional day enteral feedings were delayed, hospital length of stay was increased by 1 day. Infants with gastroschisis have a tendency toward malabsorption of substrates and possible allergies secondary to gut inflammation. The use of elemental formulas, expressed human milk, or preterm formulas are indicated because they are more easily digested.[3,4,43] Typically, small volume feedings are initiated and advanced by 10 to 20 mL/kg/d as tolerated.[43] TPN is usually decreased as the feedings increase. Manipulation of bowel and the increase in intra-abdominal pressure postrepair may increase the need for analgesia in the first 48 to 72 hours after surgery.[30] Infants should be routinely assessed for pain using a validated pain assessment tool, and analgesia should be provided as needed according to established pain guidelines.[44] Pain may be controlled with analgesics such as morphine sulfate or fentanyl as a continuous intravenous drip or as a bolus at regularly scheduled intervals. The nurse should keep in mind that these medications may result in respiratory depression and slow gut motility.

Complications/Sequelae
Outcomes for the infant with gastroschisis are usually affected by a number of complications, including cholestasis secondary to long term TPN, malrotation, midgut volvulus, hypoperistalsis, gastroesophogeal reflux (GER), and aspiration pneumonia.[3,7,14,22]The most common complications resulting in increased morbidity and mortality include intestinal atresia/stenosis, sepsis, and necrotizing enterocolitis (NEC).[45-49] Intestinal atresia is seen in approximately 5% to 25% of newborns with gastroschisis.[45,46] The development of intestinal atresia/stenosis occurs secondary to torsion and volvulus of the exteriorized bowel, causing a disruption of mesenteric

vessels and blood flow to the affected intestine. The size of the defect may also cause strangulation of the bowel, increasing the risk for an atretic/stenotic area to arise.[50] Intestinal atresia is difficult to diagnose before the time of closure because of the inflamed and matted appearance of the bowel. In the postoperative period, intestinal atresia/stenosis should be considered in all infants who present with poor feeding tolerance, abnormal stooling patterns, and/or abdominal distention with vomiting. Affected infants may need to be returned to the operating room for further evaluation and possible repair.[20] Bowel resection and anastomosis are frequently required and this places the infant at risk for short bowel syndrome. Intestinal atresia is considered a poor prognostic factor in infants with gastroschisis, with mortality ranging from 40% to 66%. [4547]

Infection is another complication associated with gastroschisis defects. Initially, the newborn is at risk for infection because of the breach in skin integrity from the exteriorized bowel. The risk is then increased postoperatively in infants with a staged repair because of delayed closure of the defect. Other factors contributing to the risk of sepsis include central venous access, prolonged TPN, and the immaturity or incompetence of the neonatal immune system. According to a recent retrospective study looking at delayed closure of abdominal wall defects, sepsis was responsible for 71% of all mortality.[48] Postoperative interventions to prevent and/or minimize the risk of infection include continuation of broad-spectrum antibiotic therapy for an additional 3 to 7 days[20] and a high index of suspicion for infection on the part of health care providers. The infant must be assessed for signs and symptoms of infection at the site of the repair, the site of central vascular access, and systemically. Aseptic dressing changes and constant monitoring of the wound site are necessary measures to decrease the risk for opportunistic infections.[19] NEC is a common complication that occurs in approximately 20% of all infants with gastroschisis defects.[51] The development of NEC is facilitated by the diminished mesenteric blood flow to the intestine.[4] The presentation of NEC in postoperative infants with gastroschisis is milder than that seen in preterm infants with the disease and occurs independent of the type of repair.[52] Despite this, NEC is considered an independent predictor for increased sepsis and mortality by some.[49] The management of post-gastroschisis repair NEC is the same as nonsurgical related NEC.[49] There is some data that expressed breast milk may decrease the risk for NEC in the infant who has undergone surgery for gastroschisis.

Family Management
One of the most significant roles for the newborn and infant nurse is care of the family of the newborn with gastroschisis. Throughout the NICU stay, parents need basic information regarding the environment, equipment, treatment, prognosis, and process of recovery for their infant. Because of the anticipated lengthy hospitalization and the involvement of multiple medical services, consistency in care and communication are essential. This consistency can be facilitated through the use of primary caregivers including bedside nurses, nurse practitioners, and physicians. Supportive resources such as social services and hospital chaplains may help the family cope with the various stresses of the infant's illness and hospitalization. Parents should be encouraged to interact with their newborn before and after surgery. They need to be encouraged to express concerns and feelings. It is also essential that parents be involved in important decisions and participate in daily care activities as appropriate.[3] Before discharge, parents must be taught to recognize signs and symptoms of possible late complications such as strictures, bowel obstruction, and GER. Instruction should include the significance of bilious vomiting as it relates to possible bowel obstruction and/or midgut volvulus. Parents should also be aware that GER may require further medical treatment after discharge from the NICU. Because intestinal complications increase the risk for feeding intolerance and failure to thrive, follow-up visits must include assessment of adequate growth and weight gain. [4,43] In addition to the special needs associated with gastroschisis, these infants also require routine infant care such as immunizations and development evaluations. Parents should be informed about the number of follow-up appointments and the importance of keeping their appointments. Every effort should be made to coordinate appointments on the same day to facilitate parental compliance.

Outcomes
Based on the available literature of short- and long-term outcomes of infants with gastroschisis, the overall survival rate is high, and the majority of infants experienced no significant sequelae impacting their perceived quality of life. Interpretation of this data must be made in light of variables such as the decade of treatment, the inclusion of infants with other abdominal wall disorders that have higher rates of associated anomalies, and global differences in management.

Recently, a number of prominent pediatric surgeons called for classifying infants with gastroschisis into two risk categories based on differences in their clinical courses, length of stay, morbidity, and mortality.[1] Infants were classified as low risk if they had a "simple" defect. Infants classified as high risk included those infants with atresias, stenosis, volvulus, or other "complex" gastrointestinal problems. According to a retrospective chart review of over 100 infants in a 5-year period, infants classified as high risk, compared with those classified as low risk, were younger and smaller (mean gestational age 34 weeks; mean birth weight 2.0 kg); had a lower rate of primary closure (65% vs. 71%); stayed on mechanical ventilation longer (22 vs. 7 days); and had a prolonged hospitalization (mean length of stay 85 vs. 26 days). In addition to experiencing more complications, infants in the high-risk category have more severe complications including short bowel syndrome, pneumatosis, pneumonia, and bowel obstruction.[1] The survival rate of low-risk infants was 100% compared with 72% for the high-risk infants.[1] Published overall survival rates for infants with gastroschisis in the United States range from 83% to 97%.[12,49,53] The reported mean gestational age at the time of birth is 36 weeks gestation (range, 35.9 to 36.6 weeks) with a birth weight of 2.5 kg. [12,49] In a Finnish study of 57 adults ranging in age from 17 to 48 years of age who were born with an abdominal wall defect, 88% described their health as good. [54] The majority of subjects saw no significant difference in their quality of life compared with the general population. The most common long-term complications noted were problems related to the abdominal scar (37% of subjects) and functional gastrointestinal problems such as GER (51% of subjects). According to an Austrian study of 19 children born between 1985 and 1996, 10 of 11 children with gastroschisis were considered developmentally normal.[55] An interesting side note in this study was the majority of mothers had a fear of giving birth to another infant with a birth defect and subsequently decided against having further children. The mothers' decisions were also supported by the fathers. [55] In a similar study of 23 individuals ranging in age from 12 to 23 years conducted in England, 96% were growing normally and considered themselves to be in good health.[56] Of the subjects born with gastroschisis, 35% underwent additional surgeries for small bowel adhesions and scar complications. Over one-half (57%) of the subjects stated the lack of an umbilicus caused significant distress during childhood. [56] In a Swedish study of 61 patients born with an abdominal wall defect who were followed for 10 to 20 years after their hospitalization, 80% of the subjects described their quality of life as good.[53] Twenty-three percent of these individuals underwent additional surgery to correct abdominal wall hernias and/or sequelae of atresias before school age. [53] Late surgical problems noted in another Swedish study of 55 patients with abdominal wall

defects included abdominal wall hernias, intestinal obstruction, and revision of an intestinal stoma. Follow-up occurred at a mean of 5 years after the initial hospitalization, and there was no significant difference in outcomes between those with a primary or staged repair.[57] Financial outcomes were addressed in a University of California at San Francisco study.[58] Hospital costs and clinical outcomes associated with gastroschisis care in a population of 69 infants treated between 1990 to 2000 provide interesting information.[58] The survival rate was 96%. The average length of stay was 47 days, and it took 33 days to achieve full enteral feedings. Factors that negatively impacted the cost of hospitalization were the number of surgical procedures, days on mechanical ventilation, length of stay, and male gender.

Conclusion
Gastroschisis is a rare, but complex, defect of the abdominal wall. There are numerous complications that may occur secondary to the evisceration of the intestines, requiring long-term follow-up. The use of a multidisciplinary team is necessary to generate a more optimal outcome for the infant with gastroschisis. With the advances seen in neonatal medicine, including surgical techniques, parenteral nutrition, respiratory support, and control of infection, these infants may go on to lead healthy and productive lives.

Gastroschisis
Gastroschisis represents a congenital defect characterized by a defect in the anterior abdominal wall through which the abdominal contents freely protrude. There is no overlying sac and the size of the defect is usually less than 4 cm. The abdominal wall defect is located at the junction of the umbilicus and normal skin, and is almost always to the right of the umbilicus[1]. Omphalocele is another congenital birth defect, but it involves the umbilical cord itself, and the organs remain enclosed in visceral peritoneum, and the defect is much larger than in gastroschisis.

[edit]Causes

High-risk pregnancies such as those complicated by infection, young maternal age, smoking, drug abuse, or anything that contributes to low birth weight can increase the incidence of gastroschisis, which is more frequent in newborns who are small for gestational age. Whether the intrauterine growth retardation can facilitate the apparition of gastroschisis or the abdominal wall defect impairs fetal growth is not clear yet. A change in paternity (childbearing with different fathers) has been implicated as a risk factor in a recent study, suggesting that the immune system of the mother may play a role in the development of gastroschisis.[2] [edit]Genetics Gastroschisis as a stand-alone congenital defect is usually inherited in an autosomal recessive manner. It may begin as a sporadic mutation, can be associated with non-genetic congenital disorders, but has also been observed to be autosomal dominant.[3]

Gastroschisis has an autosomal recessive pattern of inheritance.

Genetic counseling and further genetic testing, such as amniocentesis, may be offered during the pregnancy, as this and other abdominal wall defects are associated with genetic disorders. If there are no additional genetic problems or birth defects, surgery soon after birth can often repair the opening. [edit]Pathophysiology At least six hypotheses have been proposed:

1. Failure of mesoderm to form in the body wall[4] 2. Rupture of the amnion around the umbilical ring with subsequent herniation of bowel[5] 3. Abnormal involution of the right umbilical vein leading to weakening of the body wall and gut herniation[6] 4. Disruption of the right vitelline (yolk sac) artery with subsequent body wall damage and gut herniation[7] 5. Abnormal folding of the body wall results in a ventral body wall defect through which the gut herniates[8] 6. Failure to incorporate the yolk sac and related vitelline structures into the yolk sac[9]

The first hypothesis does not explain why the mesoderm defect would occur in such a specific small area. The second hypothesis does not explain the low percentage of associated abnormality compared with omphalocele. The third hypothesis was criticized due to no vascular supplement of anterior abdominal wall by umbilical vein. The fourth hypothesis was commonly accepted, but it was later shown that the right vitelline artery (right omphalomesenteric artery) did not supply the anterior abdominal wall in this area.[10] More evidence is needed to support the fifth hypothesis.[8] [edit]Embryology During the fourth week of development, the lateral body folds move ventrally and fuse in the midline to form the anterior body wall. Incomplete fusion results in a defect that allows abdominal viscera to protrude through the abdominal wall. The bowel typically herniates through the rectus muscle, lying to the right of the umbilicus. [edit]Prognosis Current advances in surgical techniques and intensive care management for neonates have increased the survival rate to 90%, in adequate settings. The possibility of prenatal diagnosis either through echosonogram or any other method available allows the mother to be referred to an adequate center where a caesarean section or induced natural birth can be performed before term (as natural birth is recommended and just as safe as with a normal baby), preferably within 2 weeks of term, and allow the immediate surgery to be performed on the newborn. The general procedure for gastroschisis is to simply tuck the protruding organs back into the opening and apply a belly band pressure until the wound heals itself. New advances have been pioneered in repairing the protruding bowel by placing a protective "silo" around the intestine outside the abdomen, then slowly pressuring the herniated

intestine into the abdominal cavity. This new procedure allows for the bowel to return to its intended shape and location without further traumatizing the infant's viscera with undue internal pressure. The main cause for lengthy recovery periods in patients is the time taken for the infants' bowel function to return to normal. The morbidity is closely related to the presence of other malformations and complications of the wound or the intestine. Patients frequently require more than one surgery. [edit]Epidemiology The malformation is slightly more frequent in males than females. The frequency of gastroschisis is associated with young maternal age, and low number of gestations. This abnormality is seen in ratio of 1:10,000 and is usually detected before birth. It has been reported that the incidence of gastroschisis has increased in recent years. [8

Gastroschisis Causes
High-risk pregnancies such as those complicated by infection, young maternal age, smoking, drug abuse, or anything that contributes to low birth weight can increase the incidence of gastroschisis, which is more frequent in newborns who are small for gestational age. Whether the intrauterine growth retardation can facilitate the apparition of gastroschisis or the abdominal wall defect impairs fetal growth is not clear yet. A change in paternity (childbearing with different fathers) has been implicated as a risk factor in a recent study, suggesting that the immune system of the mother may play a role in the development ofgastroschisis. Research shows that in women who had both a sexually transmitted disease and a urinary tract infection just before or during early pregnancy, the infant may be 4 times more likely to havegastroschisis. There may be a relationship between the use of oral decongestants in early pregnancy andgastroschisis. However, the absolute risk in exposed fetuses is still extremely small.