CLINICAL PRACTICE

AbstractGastroschisis 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.

© 2003 Elsevier Inc. All rights

reserved.

Management ofthe Infant withGastroschisis: AComprehensiveReview of theLiteratureBy Tracey Williams, MSN, RN, NNP,Rachel Butler, MSN, RN, NNP, andTara Sundem, MSN, RN, NNP

Gastroschisis, formally thought to be a variant of omphalocele, was firstdescribed in the 1940s. It was not recognized as having a differentembryologic origin for almost 20 years after the first reported case.

Until the 1970s, survival of infants with gastroschisis was relatively poor.1 Withincreasing technology, mortality has been reduced, and survival is an expectedoutcome. Infants with gastroschisis present the health care team with numerouschallenges from delivery to postdischarge. This article reviews the embryology,incidence, diagnosis, clinical manifestations, associated anomalies, manage-ment, complications, and follow-up needs for newborns and infants withgastroschisis.

Gastroschisis is a defect in the abdominal wall causing evisceration ofabdominal contents.2 The defect usually occurs on the right side of the umbil-ical cord and is never enclosed in a peritoneal sac.2,3 The vertical opening isapproximately 2 to 5 cm in size, with the umbilicus normally developed andproperly positioned.4,5 The small and large intestines are usually the onlyorgans protruding outside the abdominal wall (Fig 1). The spleen and liver mayalso be involved, but with a much lower incidence.6 Malformations of othermajor organ systems are infrequently associated with gastroschisis; however, ifmalformations occur, they are commonly related to infarction or atresia of theherniated bowel.7

Etiology

The etiology of gastroschisis is uncertain, but it has been reported to beof nongenetic origin.8 Four hypotheses for the etiology of the defecthave been proposed. The first is that gastroschisis may result from a

vascular disruption of the right lateral fold allowing the abdominal contents toherniate outside the abdominal cavity.2 The second is that the defect resultsfrom occlusion of the omphalomesenteric artery in utero. This occlusion mayweaken the abdominal wall causing it to rupture.4 The third hypothesis is that

From Neonatology Associates Ltd., Phoenix,AZ; and Desert Samaritan Medical Center, Mesa,AZ.

Address reprint requests to Tracey L. Williams,RN, MSN, NNP, 411 N. Criss Street, Chandler,AZ 85226.

© 2003 Elsevier Inc. All rights reserved.1527-3369/03/0302-$30.00/0doi: 10.1053/nbin.2003.36106 Newborn and Infant Nursing Reviews, Vol 3, No 2 (June), 2003: pp 55–63 55

premature atrophy or abnormal persistence of the rightumbilical vein leads to mesenchymal damage and failureof the epidermis to differentiate.9 This damage or differ-entiation failure results in a defect of the abdominal wall.The fourth and last hypothesis is that a gastroschisis defectmay be the end result of an intrauterine rupture of a smallomphalocele with the absorption of the sac.7

Incidence

During the last two decades, the overall incidenceof gastroschisis is on the rise.10 Although theexact reason is unknown, the rise may be second-

ary to improved case ascertainment, the increased accu-racy of diagnosing gastroschisis from other abdominalwall defects,11 and environmental risk factors.10 The inci-dence of gastroschisis ranges from 1.4 to 2.5 per 10,000live births and has no gender predilection.7,9,10,12 Factorsassociated with an increased risk for gastroschisis includematernal age, parity, and maternal use of selected drugs.

The incidence of gastroschisis is higher in young moth-ers and declines markedly with increasing maternalage.2,13 Women less than 20 years of age are 11 timesmore likely to have an affected infant.5 Low parity has alsobeen shown to increase the risk for gastroschisis.2 A studyconducted by Sharp et al in 2000 found that 66% ofmothers who delivered an infant with gastroschisis were intheir first pregnancy, 26.4% were in their second, and3.8% were in their third or greater pregnancy.14 Drugstaken during the first trimester including nicotine, pseudo-ephedrine alone or in combination with acetaminophen,phenylpropanolamine, cocaine, aspirin, and acetamino-phen are associated with an increased incidence of gas-troschisis.5,15–17

Embryological Basis

The embryologic development of gastroschisis in-volves maldevelopment of the abdominal wall. Theabdominal wall is normally formed from the fusion

of the cephalic, caudal, and right and left lateral folds ofthe mesoderm. These four folds usually meet and fuse inthe center of the embryo to form the abdominal wall. Eachof the mesodermal folds creates different portions of theabdominal wall. The cephalic fold forms the upper abdo-men; the caudal fold forms the lower abdomen; and thelateral folds transform into both sides of the abdominalwall. As the folds are fusing, rotation occurs forming theumbilical ring. These folds are approaching the midline ofthe abdomen as the intestinal tract is beginning to form. Bythe 10th week of gestation, the anatomical positions of theabdominal wall contents should be complete. A defect ofthe abdominal wall can result if any of these four folds donot develop correctly.18

Differential Diagnoses

With advancements in prenatal testing and ultra-sonography, abdominal wall defects are com-monly diagnosed in utero as early as 12 to 14

weeks gestation. The differential diagnosis of abdominalwall defects includes gastroschisis and omphalocele. Gas-troschisis is a defect in the abdominal wall lateral to theumbilical cord, whereas omphalocele is a defect in whichthe intestines are enclosed within the umbilical cord. Itmay be difficult to distinguish between the two diagnosesif the protective sac of the omphalocele has been rup-tured.3,19 It is important to remember that gastroschisisdefects do not involve the umbilical cord. It is also essen-tial to distinguish between the two defects because there isa higher incidence of major congenital/chromosomalanomalies associated with omphalocele.7 The incidence ofchromosomal anomalies associated with gastroschisis isless than 5%.4

Clinical Manifestations

The infant with gastroschisis typically presents witha small, underdeveloped abdominal cavity causedby evisceration of the intestines. Although the dis-

tal portions of the colon, liver, and other solid organs havethe potential to protrude through the abdominal wall de-fect, these organs usually remain in the abdominal cav-ity.19 Malrotation occurs almost universally because of theprotrusion of the intestines outside the abdominal wall.20

Exposure to amniotic fluid can cause the uncovered bowel

Fig 1 Intestines and stomach protruding from gastroschisis defect.

56 Williams, Butler, and Sundem

to become inflamed, thickened, and edematous. The af-fected bowel can also appear as a matted mass with noidentifiable loops. A peel over the serosal surface of thebowel can occur as a result of amniotic fluid exposure.This, in conjunction with a chemical peritonitis, mayimpede reduction of the intestine into the abdominalcavity.4,19,21

Associated Anomalies

There is a lower incidence of associated anomalieswith gastroschisis compared with other abdominalwall defects. A 10-year review of infants with

gastroschisis found a 30% incidence of associated anom-alies with intestinal atresia and cryptorchidism or unde-scended testes being the most common.22 Intestinal atresiawas noted in 22% of affected infants, while cryptorchid-ism was noted in 55%.22 In a second 10-year review ofinfants with gastroschisis, ileal atresia occurred in 5.4% ofaffected infants and cryptorchidism occurred in 24%.14

Cryptorchidism in infants with gastroschisis has an esti-mated occurrence of 31%.23 Cryptorchidism is considereda minor anomaly that usually requires conservative man-agement.23

Management

Management of the newborn with gastroschisisoften occurs before birth, with the decision onthe mode of delivery. Postnatal management

includes presurgical stabilization and evaluation, surgicalrepair, and postsurgical care and follow-up. While numer-ous articles are available in the professional literature, norandomized clinical trials on any aspect of managementhave been published.

Mode of Delivery

While the goal of delivery of the newborn withgastroschisis is to optimize their outcome byminimizing trauma to the exposed gastrointes-

tinal contents, the best mode of delivery for these infantsremains controversial. From a theoretical standpoint, onemight assume delivery by cesarean section would be moreadvantageous than vaginal delivery for several reasons.The first reason is a cesarean delivery is thought to pro-duce less compromise to the mesenteric circulation be-cause there may be less compression and twisting of thebowel during uterine contractions and passage through thebirth canal. Another reason is that the risk of infection tothe exposed bowel is decreased by cesarean delivery with

intact membranes. The last theoretical disadvantage tovaginal delivery is if a large defect is present with possibleliver involvement, there may be an increased risk foravulsion injury.

While the rationale to promote cesarean delivery of thenewborn with gastroschisis makes sense from a theoreticalstandpoint, none of these assumptions have been con-firmed by clinical data.24 No significant differences inoutcomes between cesarean and vaginal delivery werenoted in several studies of morbidity associated with gas-troschisis and type of delivery.22,25,26 The measures ofmorbidity in these studies included time to full oral feed-ings, duration of parenteral nutrition, age at discharge,incidence of complications, and number of hospitaldays.22,25 A recent meta-analysis of 15 clinical studies alsoconcluded there is no significant relationship betweenmode of delivery and infant outcomes.27 In addition to theoutcomes previously listed, the meta-analysis also foundno significant relationship between mode of delivery andrate of primary repair, neonatal sepsis, and pediatric mor-tality.27

Presurgical Management

Stabilization and preoperative management of thenewborn with gastroschisis must take into consid-eration many factors, including thermoregulation,

fluid volume status, gastric distention and intestinal com-promise, infection, respiratory status, and preparation forsurgery. Stability of the aforementioned factors is neces-sary before the impending surgical repair to optimize theinfant’s outcome.

On delivery, the infant’s trunk and lower extremitiesshould be placed in a sterile plastic “bowel bag.” Theinfant should be placed under a preheated radiant warmerand dried to prevent heat loss.28 It is also important toensure 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 infantappears to be in distress, although decompression of thegastrointestinal tract may help reduce respiratory compro-mise.4 The infant must be monitored for signs of hypo-thermia, respiratory distress, and shock. A thorough phys-ical examination should also be performed to determinethe presence of other anomalies.3,7

Delivery room management of the infant with gastros-chisis has included the use of sterile bowel bags and/orsaline-soaked gauze dressings to prevent damage to theexposed intestines.29 Sterile, moist gauze dressings, cov-ered by a transparent plastic film have also been used tocover the exposed tissue. The use of moistened gauzedressings is associated with several problems including

Management of the Infant with Gastroschisis 57

hypothermia caused by increased insensible water loss andradiant heat loss from the larger surface area of the ex-posed bowel, tissue trauma, and infection. The bowel bagis the most appropriate alternative.28 Bowel bags provide asterile environment for the exposed intestine and reducethe risk for contamination and tissue trauma. In addition,the bowel bag helps to prevent evaporative heat and fluidlosses and enables pooling of fluid within the bag. Thispooling of fluid can be measured to provide a more accu-rate assessment of fluid loss.28

Once initial stabilization in the delivery room isachieved, the newborn is admitted to the neonatal inten-sive care unit (NICU) for further evaluation and stabiliza-tion before surgical repair. Because the newborn withgastroschisis is at an increased risk for fluid loss becauseof the large surface area of exposed bowel, the newbornmay present with symptoms of shock.30 Fluid resuscitationwith isotonic solutions such as normal saline or Ringer’slactate is recommended for the newborn in shock.31,32

Fluid resuscitation is usually continued until the infant’surine output normalizes and/or blood gases indicate nor-mal acid–base balance.31 The maintenance fluid require-ment for infants with gastroschisis is increased 2- to 3-foldbecause of the excessive losses through the exposedbowel.31,32 When fluid orders are written, it is important tokeep in mind the glucose load associated with the intra-venous fluids. Placing the newborn with a gastroschisis ona dextrose solution at two to three times the usual main-tenance rate increases the likelihood of hyperglycemia.

The newborn with gastroschisis is at constant risk forhypothermia because of the large surface area of exposedbowel. The use of warm, saline-soaked gauze to cover thedefect may increase the risk of hypothermia as the salinecools.28 Care must be taken to prevent cold stress bykeeping the infant on a radiant warmer or other externalheat device, and by minimizing fluid and heat loss fromthe exposed bowel.19

The infant must be continually assessed for signs ofgastrointestinal compression before surgical repair. Anaso/orogastric tube should be inserted and placed to in-termittent suction to keep the bowel and stomach decom-pressed.29,33 Decompression is important because it helpsto prevent partial or total obstruction of blood flow andoxygenation to the bowel. If decompression does not oc-cur, there is an increased risk for bowel necrosis secondaryto the constriction of the exteriorized intestine through thesmall visceral defect. Decompression will also reduce theinfant’s risk for emesis and thus aspiration.33

Bowel compromise can occur during positioning of theinfant. Infants with gastroschisis should be positioned ontheir right side in a lateral decubitus position to enhancevenous blood return from the gut.29 The right lateral de-cubitus position also decreases the risk of decreased per-

fusion caused by compression or kinking of mesentericvessels.20 It is important to make frequent assessments ofperfusion to the intestines with minimal handling of theexposed tissue.

Diagnostic testing and antibiotic prophylaxis are thelast two areas of presurgical management. While the spe-cific tests may vary from NICU to NICU, the most com-mon presurgical studies ordered include a baseline chestx-ray, complete blood count (CBC) with differential andplatelets, arterial blood gas, serum electrolytes, blood glu-cose level, total protein, and a blood type and crossmatch.3 Broad-spectrum antibiotics such as ampicillin andgentamicin are started to decrease the risk of infectionfrom bacterial contamination of the exposed bowel.33,34

Surgical Management

Surgical management of the infant with gastroschisisremains controversial. While primary closure of theabdominal defect is the preferred surgical approach,

each pediatric surgeon must subjectively assess the degreeof abdominal wall tension anticipated before deciding thenature of the repair.8,22 If primary closure cannot be ob-tained, the alternative management strategy is a staged silorepair.31

Because of the increased risk of sepsis and hypovole-mic shock, primary closure is considered in all caseswhere reduction does not cause hemodynamic or respira-tory compromise.7,33 Airway and intra-abdominal pres-sures should be kept less than 25 and 20 mm Hg, respec-tively, to prevent adverse hemodynamic consequences toother organs and tissues.7 Strategies to achieve primaryrepair include stretching of the abdominal wall, evacuatingthe contents of the stomach and small bowel, irrigatingmeconium from the intestines, and enlarging the defect byleaving a fascial hernia.20,35 If primary closure is at-tempted without sufficient space in the abdominal cavity,potential complications secondary to abdominal compart-ment syndrome may occur.36,37 These complications arelisted in Table 1.

If the surgeon is unable to achieve primary closure or ifa primary closure leads to hemodynamic and/or ventila-tory compromise, an alternative method of closure must beused. Currently, most surgeons use a silastic silo for grad-ual reduction of herniated abdominal contents (Fig 2).Secondary closure occurs at a later time when the intesti-nal contents fit within the abdominal cavity.7,20 Closure ofthe silo is usually performed in stages over 7 to 10 days,with reduction of the silo occurring one to two times daily.

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 ab-

58 Williams, Butler, and Sundem

dominal compartment syndrome, the nurse must remem-ber that the infant remains at risk for complications asso-ciated with abdominal compartment syndrome. The infantshould be carefully assessed during and immediately afterthe reduction for complications.

A major concern for nurses and family members is theissue of pain associated with the reduction process. Todate there is only one published study addressing thistopic.39 Kimble et al prospectively collected data on 35newborns with gastroschisis born between 1999 and 2001.They concluded analgesia for reduction is “safe if strictselection criteria are adhered to.”39 Infants with noticeableatresia, bowel perforation or ischemia, or clinical instabil-ity were excluded from the study.

The investigators of a pilot study involving 14 infantswith gastroschisis who underwent midgut reduction with-out anesthesia, concluded that the practice “appeared safe,carrying no additional morbidity or mortality.”40 Davies etal conducted a meta-analysis of the available literaturefrom 1966 to 2002 on the issue of reduction with orwithout general anesthesia.41 Although these investigatorswere unable to find any studies that meet their inclusioncriteria, they concluded “there is no evidence from ran-domized clinical trials to support or refute the practice ofward reduction....”41 The authors of both of these studiesare from England. Silo reduction in the NICU in theUnited States is seldom performed under anesthesia, so therelevance of these studies to American practice is ques-tionable. The issue of concern is whether the silo reductionis painful and warrants pain management. Based on clin-ical experience, the authors recommend that each infantundergoing a reduction procedure must be assessed forpain before, during, and after the reduction. Appropriatepharmacologic and nonpharmacologic pain therapiesshould be used for those infants who exhibit signs of painor distress with the reduction.

Postsurgical Management

Initial postsurgical management of the infant with gas-troschisis includes monitoring of vital signs, cardio-vascular and respiratory status, fluid and electrolyte

balance, and pain. After the repair, intra-abdominal pres-sure increases and can result in venous compression. Ve-nous compression may compromise renal blood flow andthe glomerular filtration rate, resulting in decreased urineoutput. A urinary catheter may be necessary to relievebladder distention and to allow for a more accurate mea-surement of urine output.4 Maintenance fluid requirementsmay need to be increased because of third spacing into thedistended bowel and abdominal cavity.29–31 Alterations inelectrolyte balance may ensue from this shift of fluids. Thepostoperative infant may require anywhere from 120 to170 mL/kg/d of a crystalloid solution that is adjusted toprovide for adequate tissue perfusion and urine output.20 Alarge-bore naso/orogastric tube placed to intermittent suc-tion is needed to prevent gastrointestinal distention causedby hypoperistalsis. Hypoperistalsis or adynamic ileus isfrequently seen in the postoperative period and may persistfor several weeks.29 The initial gastrointestinal drainage ischaracteristically green because of the back up of biliaryand pancreatic secretions in the immediate postoperativeperiod. As gut motility improves, the drainage becomesclear in appearance. Volume loss from the gastric tubemust be monitored, because it is possible for the infant tolose up to 100 mL/kg/d.3 Replacement of these losses isnecessary to maintain homeostasis.

Because of the increased intra-abdominal pressure,close monitoring of respiratory status is essential for thefirst 48 to 72 hours postsurgery. Respiratory support, asindicated, is provided to optimize oxygenation and venti-lation. The increase in abdominal pressure may interfere

Fig 2 Silo reduction.

Table 1. Complications of Abdominal CompartmentSyndrome3,36,37

Pulmonary ventilation restrictionNeed for increased peak inspiratory pressureHypercapniaCardiovascular collapse and shockVenous stasisTrauma and intraabdominal infectionsMesenteric ischemiaEnterocolitisAppendicitisBowel perforationDecrease in glomerular filtration rateOliguriaRenal vein thrombosisRenal failure

Management of the Infant with Gastroschisis 59

with optimal expansion of the diaphragm and venousreturn impeding both ventilation and oxygenation.42 Someinfants may benefit from mechanical ventilation or con-tinuous positive airway pressure (CPAP) to maximize lungexpansion, lung volume, and oxygenation.33 Central ve-nous pressure (CVP) must be monitored constantly duringCPAP because of its hindering effect on the CVP.33 Otherinfants may not tolerate CPAP because of increased ab-dominal distention from the increased airflow to the gas-trointestinal track. A properly functioning naso/orogastrictube will minimize this risk.

In the immediate postsurgical period, the infant withgastroschisis is usually returned to the NICU from theoperating room intubated and on mechanical ventilation.While most infants can be extubated within 24 to 48 hoursafter surgery, infants who are small for gestational age,preterm, and/or have significantly increased intra-abdom-inal pressure may require a longer period of ventilatorsupport.31 High peak inspiratory pressures (PIP), usuallygreater than 25 cm H2O, should be avoided if possible tominimize adverse effects to the renal and intestinal perfu-sion.4,29 High-frequency ventilation can be an acceptablealternative because its effects on intra-abdominal pressureare not as great.4

After the initial stabilization period, the main goal ofmanagement is to provide adequate nutrition and painmanagement. Initially, the infant will require parenteralnutrition. Gut motility is delayed because of the chemicalperitonitis that occurred when the intestinal contents wereexposed to amniotic fluid. Delayed gut motility may per-sist for weeks after surgical repair and is often influencedby the severity of the defect and other associated anoma-lies such as intestinal atresia.29,31 Because of the postop-erative ileus, total parenteral nutrition (TPN) is needed inall infants with gastroschisis and is usually initiated within24 to 48 hours after surgery.29,33 Because these infantsmay require TPN for weeks after surgery,31 a central lineis recommended. The minimal daily requirements for post-operative 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 main-tain euglycemia.43 Because of protein losses from thesurgical stress, wound healing, and/or third spacing, addi-tional protein in the TPN may be necessary.43

Once gut motility returns, it is important to be proactivewith the initiation of enteral feedings. A retrospectivestudy found the age of initial enteral feeding was posi-tively correlated with the time of discharge.14 These in-vestigators also noted that for every additional day enteralfeedings were delayed, hospital length of stay was in-creased by 1 day. Infants with gastroschisis have a ten-dency toward malabsorption of substrates and possibleallergies secondary to gut inflammation. The use of ele-mental formulas, expressed human milk, or preterm for-

mulas are indicated because they are more easily digest-ed.3,4,43 Typically, small volume feedings are initiated andadvanced by 10 to 20 mL/kg/d as tolerated.43 TPN isusually decreased as the feedings increase.

Manipulation of bowel and the increase in intra-abdom-inal pressure postrepair may increase the need for analge-sia in the first 48 to 72 hours after surgery.30 Infants shouldbe routinely assessed for pain using a validated pain as-sessment tool, and analgesia should be provided as neededaccording to established pain guidelines.44 Pain may becontrolled with analgesics such as morphine sulfate orfentanyl as a continuous intravenous drip or as a bolus atregularly scheduled intervals. The nurse should keep inmind that these medications may result in respiratorydepression and slow gut motility.3

Complications/Sequelae

Outcomes for the infant with gastroschisis are usu-ally affected by a number of complications, in-cluding cholestasis secondary to long term TPN,

malrotation, midgut volvulus, hypoperistalsis, gastroeso-phogeal reflux (GER), and aspiration pneumonia.3,7,14,22

The most common complications resulting in increasedmorbidity and mortality include intestinal atresia/stenosis,sepsis, and necrotizing enterocolitis (NEC).45–49

Intestinal atresia is seen in approximately 5% to 25% ofnewborns with gastroschisis.45,46 The development of in-testinal atresia/stenosis occurs secondary to torsion andvolvulus of the exteriorized bowel, causing a disruption ofmesenteric vessels and blood flow to the affected intestine.The size of the defect may also cause strangulation of thebowel, increasing the risk for an atretic/stenotic area toarise.50 Intestinal atresia is difficult to diagnose before thetime of closure because of the inflamed and matted ap-pearance of the bowel. In the postoperative period, intes-tinal atresia/stenosis should be considered in all infantswho present with poor feeding tolerance, abnormal stool-ing patterns, and/or abdominal distention with vomiting.Affected infants may need to be returned to the operatingroom for further evaluation and possible repair.20 Bowelresection and anastomosis are frequently required and thisplaces the infant at risk for short bowel syndrome. Intes-tinal atresia is considered a poor prognostic factor ininfants with gastroschisis, with mortality ranging from40% to 66%.45–47

Infection is another complication associated with gas-troschisis defects. Initially, the newborn is at risk forinfection because of the breach in skin integrity from theexteriorized bowel. The risk is then increased postopera-tively in infants with a staged repair because of delayedclosure of the defect. Other factors contributing to the risk

60 Williams, Butler, and Sundem

of sepsis include central venous access, prolonged TPN,and the immaturity or incompetence of the neonatal im-mune system. According to a recent retrospective studylooking at delayed closure of abdominal wall defects,sepsis was responsible for 71% of all mortality.48

Postoperative interventions to prevent and/or minimizethe risk of infection include continuation of broad-spec-trum antibiotic therapy for an additional 3 to 7 days20 anda high index of suspicion for infection on the part of healthcare providers. The infant must be assessed for signs andsymptoms of infection at the site of the repair, the site ofcentral vascular access, and systemically. Aseptic dressingchanges and constant monitoring of the wound site arenecessary measures to decrease the risk for opportunisticinfections.19

NEC is a common complication that occurs in approx-imately 20% of all infants with gastroschisis defects.51 Thedevelopment of NEC is facilitated by the diminished mes-enteric blood flow to the intestine.4 The presentation ofNEC in postoperative infants with gastroschisis is milderthan that seen in preterm infants with the disease andoccurs independent of the type of repair.52 Despite this,NEC is considered an independent predictor for increasedsepsis and mortality by some.49 The management of post-gastroschisis repair NEC is the same as nonsurgical relatedNEC.49 There is some data that expressed breast milk maydecrease the risk for NEC in the infant who has undergonesurgery for gastroschisis.51

Family Management

One of the most significant roles for the newbornand infant nurse is care of the family of thenewborn with gastroschisis. Throughout the

NICU stay, parents need basic information regarding theenvironment, equipment, treatment, prognosis, and pro-cess of recovery for their infant. Because of the anticipatedlengthy hospitalization and the involvement of multiplemedical services, consistency in care and communicationare essential. This consistency can be facilitated throughthe use of primary caregivers including bedside nurses,nurse practitioners, and physicians. Supportive resourcessuch as social services and hospital chaplains may help thefamily cope with the various stresses of the infant’s illnessand hospitalization. Parents should be encouraged to in-teract with their newborn before and after surgery. Theyneed to be encouraged to express concerns and feelings. Itis also essential that parents be involved in importantdecisions and participate in daily care activities as appro-priate.3

Before discharge, parents must be taught to recognizesigns and symptoms of possible late complications such as

strictures, bowel obstruction, and GER. Instruction shouldinclude the significance of bilious vomiting as it relates topossible bowel obstruction and/or midgut volvulus. Par-ents should also be aware that GER may require furthermedical treatment after discharge from the NICU. Becauseintestinal complications increase the risk for feeding in-tolerance and failure to thrive, follow-up visits must in-clude assessment of adequate growth and weight gain.4,43

In addition to the special needs associated with gastros-chisis, these infants also require routine infant care such asimmunizations and development evaluations. Parentsshould be informed about the number of follow-up ap-pointments and the importance of keeping their appoint-ments. Every effort should be made to coordinate appoint-ments on the same day to facilitate parental compliance.

Outcomes

Based on the available literature of short- and long-term outcomes of infants with gastroschisis, theoverall survival rate is high, and the majority of

infants experienced no significant sequelae impacting theirperceived quality of life. Interpretation of this data must bemade in light of variables such as the decade of treatment,the inclusion of infants with other abdominal wall disor-ders that have higher rates of associated anomalies, andglobal differences in management.

Recently, a number of prominent pediatric surgeonscalled for classifying infants with gastroschisis into tworisk categories based on differences in their clinicalcourses, length of stay, morbidity, and mortality.1 Infantswere classified as low risk if they had a “simple” defect.Infants classified as high risk included those infants withatresias, stenosis, volvulus, or other “complex” gastroin-testinal problems. According to a retrospective chart re-view of over 100 infants in a 5-year period, infants clas-sified as high risk, compared with those classified as lowrisk, were younger and smaller (mean gestational age 34weeks; mean birth weight 2.0 kg); had a lower rate ofprimary closure (65% vs. 71%); stayed on mechanicalventilation longer (22 vs. 7 days); and had a prolongedhospitalization (mean length of stay 85 vs. 26 days). Inaddition to experiencing more complications, infants in thehigh-risk category have more severe complications includ-ing short bowel syndrome, pneumatosis, pneumonia, andbowel obstruction.1 The survival rate of low-risk infantswas 100% compared with 72% for the high-risk infants.1

Published overall survival rates for infants with gas-troschisis in the United States range from 83% to97%.12,49,53 The reported mean gestational age at the timeof 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

Management of the Infant with Gastroschisis 61

adults ranging in age from 17 to 48 years of age who wereborn with an abdominal wall defect, 88% described theirhealth as good.54 The majority of subjects saw no signif-icant difference in their quality of life compared with thegeneral population. The most common long-term compli-cations noted were problems related to the abdominal scar(37% of subjects) and functional gastrointestinal problemssuch as GER (51% of subjects). According to an Austrianstudy of 19 children born between 1985 and 1996, 10 of 11children with gastroschisis were considered developmen-tally normal.55 An interesting side note in this study wasthe majority of mothers had a fear of giving birth toanother infant with a birth defect and subsequently de-cided against having further children. The mothers’ deci-sions were also supported by the fathers.55 In a similarstudy of 23 individuals ranging in age from 12 to 23 yearsconducted in England, 96% were growing normally andconsidered themselves to be in good health.56 Of thesubjects born with gastroschisis, 35% underwent addi-tional surgeries for small bowel adhesions and scar com-plications. Over one-half (57%) of the subjects stated thelack of an umbilicus caused significant distress duringchildhood.56 In a Swedish study of 61 patients born withan abdominal wall defect who were followed for 10 to 20years after their hospitalization, 80% of the subjects de-scribed their quality of life as good.53 Twenty-three per-cent of these individuals underwent additional surgery tocorrect abdominal wall hernias and/or sequelae of atresiasbefore school age.53 Late surgical problems noted in an-other Swedish study of 55 patients with abdominal walldefects included abdominal wall hernias, intestinal ob-struction, and revision of an intestinal stoma. Follow-upoccurred at a mean of 5 years after the initial hospitaliza-tion, and there was no significant difference in outcomesbetween those with a primary or staged repair.57

Financial outcomes were addressed in a University ofCalifornia at San Francisco study.58 Hospital costs andclinical outcomes associated with gastroschisis care in apopulation of 69 infants treated between 1990 to 2000provide interesting information.58 The survival rate was96%. The average length of stay was 47 days, and it took33 days to achieve full enteral feedings. Factors that neg-atively impacted the cost of hospitalization were the num-ber of surgical procedures, days on mechanical ventilation,length of stay, and male gender.

Conclusion

Gastroschisis is a rare, but complex, defect of theabdominal wall. There are numerous complica-tions 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 moreoptimal outcome for the infant with gastroschisis. With theadvances seen in neonatal medicine, including surgicaltechniques, parenteral nutrition, respiratory support, andcontrol of infection, these infants may go on to leadhealthy and productive lives.

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