DOI: 10.1002/pd.4305

REVIEW

Gastroschisis – what should be told to parents? Karine Lepigeon1, Tim Van Mieghem2, Sabine Vasseur Maurer3, Eric Giannoni4 and David Baud1* 1

Materno-fetal & Obstetrics Research Unit, Department of Obstetrics and Gynecology, University Hospital, 1011 Lausanne, Switzerland Department of Obstetrics and Gynecology, University Hospitals Leuven, Leuven, Belgium 3 Department of Pediatric Surgery, University Hospital, 1011 Lausanne, Switzerland 4 Service of Neonatology, University Hospital, 1011 Lausanne, Switzerland *Correspondence to: David Baud. E-mail: [email protected] 2

ABSTRACT Gastroschisis is a common congenital abdominal wall defect. It is almost always diagnosed prenatally thanks to routine maternal serum screening and ultrasound screening programs. In the majority of cases, the condition is isolated (i.e. not associated with chromosomal or other anatomical anomalies). Prenatal diagnosis allows for planning the timing, mode and location of delivery. Controversies persist concerning the optimal antenatal monitoring strategy. Compelling evidence supports elective delivery at 37 weeks’ gestation in a tertiary pediatric center. Cesarean section should be reserved for routine obstetrical indications. Prognosis of infants with gastroschisis is primarily determined by the degree of bowel injury, which is difficult to assess antenatally. Prenatal counseling usually addresses gastroschisis issues. However, parental concerns are mainly focused on long-term postnatal outcomes including gastrointestinal function and neurodevelopment. Although infants born with gastroschisis often endure a difficult neonatal course, they experience few long-term complications. This manuscript, which is structured around common parental questions and concerns, reviews the evidence pertaining to the antenatal, neonatal and long-term implications of a fetal gastroschisis diagnosis and is aimed at helping healthcare professionals counsel expecting parents. © 2013 John Wiley & Sons, Ltd.

Funding sources: David Baud was supported by the ‘Société Académique Vaudoise’, the SICPA Foundation, an ‘Air Canada’ travel grant and the ‘Fondation Leenaards’ through the ‘Bourse pour la reléve académique’. Conflicts of interest: None declared

INTRODUCTION A diagnosis of a fetal anomaly is very distressing for expecting parents. The role of medical caregivers is to inform patients regarding the implications of the fetal anomaly and hopefully reduce parental anxiety and stress. In this manuscript, we review the essential medical information that counselors should provide to parents when a diagnosis of fetal gastroschisis has been made. Ideally, this counseling should be provided by a multidisciplinary team consisting of obstetricians, maternal–fetal medicine specialists, neonatologists and pediatric surgeons. This article has been written around questions commonly found on parent’s forum websites1–7 dealing with fetal anomalies, in order to make the available information applicable in clinical practice.

WHAT IS GASTROSCHISIS AND HOW COMMON IS IT?

the amniotic fluid. This defect, which is usually situated on the right side of the abdominal insertion site of the umbilical cord, occurs early in pregnancy (6–10 weeks after conception). Gastroschisis is the most common abdominal wall defect, affecting 1/2000 to 1/5000 pregnancies with an increasing prevalence over the past two decades.8–10 The contribution of young maternal age, as a consistent risk factor across epidemiological studies, has been thought to be linked to a number of potential confounding factors such as smoking and illicit drug use.11–15 Use of aspirin and nasal decongestants containing pseudoefedrin has also been linked to gastroschisis.13 Of note, mothers with a higher body mass index may have a lower risk of having an infant with gastroschisis (odds ratio = 0.35; 95% CI 0.12–0.80).16 However, the exact cause and physiopathology of gastroschisis have not been identified to date.

Parents: ‘What happened to my baby? Have I done something wrong?’1–7

WHEN AND HOW CAN GASTROSCHISIS BE DIAGNOSED?

The word ‘gastroschisis’ is derived from the Greek words ‘gaster’ and ‘schisis’, which mean abdomen and fissure, respectively. Gastroschisis is an abdominal wall defect allowing bowel loops to herniate outside of the abdominal cavity and to float freely in

Parents: ‘What was observed by my doctor?’1–7

Prenatal Diagnosis 2014, 34, 316–326

Gastroschisis can virtually always be diagnosed on an ultrasound as early as the first trimester of pregnancy17 when performed in expert centers.18 However, because the © 2013 John Wiley & Sons, Ltd.

Gastroschisis – what should be told to parents?

abdominal content develops outside the abdomen and the abdomen wall does not normally close until 11–12 weeks, it is sometimes not diagnosed until the second trimester. Elevated maternal serum alpha fetoprotein levels, which are used to screen for open spina bifida and trisomy 21, can be indirect indicators of gastroschisis.19,20 Typically, on ultrasound, free-floating loops of intestine are seen in the amniotic fluid. The lack of a surrounding tissue membrane distinguishes it from omphalocele, as well as a normal umbilical cord abdominal insertion merging from the left side of the fetus. Rarely, the stomach, bladder and internal genitalia can be exteriorized. Liver herniation is more frequently associated with omphalocele, but it can also be observed in 6% of gastroschisis.21 The presence of a normal umbilical cord adjacent to the abdominal wall defect and the absence of a remnant omphalocele sac accurately distinguish gastroschisis from ruptured omphalocele.21 Bladder, heart and sternum have to be visualized in order to be sure that a more complex anomaly is not present, such as total wall defect. Because in gastroschisis the bowel develops outside of the abdomen, rotation of the bowel is abnormal. The bowel is shorter than normal with compromised vascular blood supply, which may induce bowel atresia, ischemia and perforations.

WHAT IS THE RISK OF THE FETUS HAVING AN ASSOCIATED ANATOMIC, GENETIC OR SYNDROMIC ANOMALY? Parents: ‘Should I undergo an amniocentesis?’1–7 Gastroschisis is usually isolated and is not normally associated with chromosomal or genetic anomalies.22 Mastroiacovo et al. reported that 1.2% of infants with gastroschisis had an abnormal karyotype.23 Genetic testing is therefore generally not recommended in the absence of other markers. However, an amniocentesis may be discussed in cases of associated anomalies, suspicion of omphalocele’s rupture and positive first or second trimester serum screening. In 0.5% to 3.2% of cases, gastroschisis has been associated with amyoplasia.23 Up to 15% will have some form of associated anatomic malformation,24 and this incidence is even higher in atypical, left-sided, gastroschisis.25 Familial recurrence of gastroschisis has been reported, and the estimated sibling recurrence risk is 3.5%.26 Kohl et al. reported a recurrence risk of 2.4%.27

WHICH COMPLICATIONS CAN BE EXPECTED DURING PREGNANCY? Parents: ‘Is my baby at risk during pregnancy?’1–7 On the basis of a meta-analysis published by South et al. in 2013, the risk of intra-uterine death in fetuses with gastroschisis is reported to be 3% to 6% (mean 4.48%; 95% CI 3.48–5.76).28 The exact cause for this is unknown, but midgut volvulus or acute compression of the umbilical cord by eviscerated bowel29,30 and stomach31 has been implicated. Alternatively, intra-uterine growth restriction (IUGR), which occurs in 30% to 50% of cases, may play a role. The etiology of IUGR in gastroschisis remains unknown. Socioeconomic factors (young age, primigravida and smoking),13,32–34 protein Prenatal Diagnosis 2014, 34, 316–326

317

loss as a consequence of externalization of intestines in amniotic fluid35 and placenta chorangiosis36 have been implicated as potential causes. In general, the risk of IUGR is high for fetuses affected by gastroschisis (30–50% of cases). As described in epidemiologic studies,30 IUGR increases the rate of stillbirth by a factor of four, independently of congenital anomalies. The high rate of IUGR in gastroschisis may play a role in the increased risk of stillbirth. Gastroschisis pregnancies are known to be at increased risk of preterm labor. Lausman et al. found that 28% of 152 pregnant women with gastroschisis fetuses delivered spontaneously before 37 weeks’ gestation, compared with 6% in the general population.37 In another study from the same group, premature delivery before 34 weeks’ gestation was associated with a 23% outcomes of neonatal death.38 Educating expectant mothers about the signs of preterm labor will enable the women to be admitted in a timely fashion to a maternity unit.

WHICH ANTENATAL SURVEILLANCE STRATEGIES ARE RECOMMENDED? Parents: ‘Can I do something to help my baby during pregnancy?’1–7 Given the increased risk of IUGR and intra-uterine fetal death (IUFD), most authors concur that close assessment of fetal well-being is warranted in the third trimester of pregnancy. Although surveillance protocols vary, serial ultrasound examinations to assess fetal growth, amniotic fluid volume, gastrointestinal appearance and stomach location/position as well as fetal heart rate monitoring are recommended (reviewed by Kuleva et al.39). It is important however to understand the limitations of this surveillance. First, close surveillance does not always obviate IUFD.12,39 Second, the diagnosis of IUGR is difficult because of unreliable abdominal circumference measurements. Chaudhury et al.40 determined that birth weight was best predicted by the formulas of Shepard et al.41 and Siemer et al.42 Despite the use of adequate formulas, high false positive and false negative rates for diagnosing IUGR have been reported.38,43–45 Accurate detection of IUGR would nevertheless be useful, as it could help give parents more individualized counseling. Indeed, IUGR has been associated with longer neonatal intensive care unit (NICU) and hospital stay, delayed closure, more surgical complications and less body weight gain when compared with normally grown gastroschisis neonates.46,47 Fetal heart rate anomalies are common in gastroschisis fetuses during the third trimester of pregnancy (43%).39 This induces a considerable amount of iatrogenic prematurity. However, it remains uncertain whether earlier iatrogenic delivery of these fetuses improves perinatal outcomes.

WHAT HAPPENS TO THE EXPOSED BOWEL IN UTERO? Parents: ‘Can my baby hurt his bowel?’1–7 Similar to what is seen in spina bifida,48 amniotic fluid is a toxic environment for the fetal bowel. The outcome of infants with gastroschisis is primarily determined by the amount of intestinal damage, which occurs toward the end of pregnancy. © 2013 John Wiley & Sons, Ltd.

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The etiology of this injury is likely due to a combination of constriction of the bowel and the intestinal vasculature at the level of the abdominal wall defect and exposure of the fragile bowel to amniotic fluid.48 The bowel-toxic amniotic fluid induces a thickening of the bowel loops, which end up covered with a fibrinous coating that impairs bowel movements and mucosal absorptive function. There is no relation between the thickness of this coating and the quality of the bowel measured as the time required to achieve a normal feeding. Compromise of the vascular supply may lead to intestinal damage resulting in atresia, perforation and necrosis. All these adverse bowel events lead to a prolonged need for total parenteral nutrition and, in some cases, severe irreversible intestinal failure. A number of ultrasonographic prognostic factors including gastric dilatation,49,50 extra-abdominal or intra-abdominal bowel dilatation,23,51–58 bowel wall thickness53,55,58–60 and mesenteric artery Doppler61 have been investigated in an attempt to predict bowel integrity at delivery and/or neonatal outcomes. One of the most investigated markers antenatally is small bowel dilatation. However, controversies persist regarding its usefulness. Ghionzoli et al.54 investigated the predictive value of an antenatal diagnosis of bowel dilatation (>18 mm) and found that fetal bowel dilatation had 97% sensitivity and 37% specificity to detect intestinal atresia. The high negative predictive value of this test (99%) leads the authors to conclude that the absence of fetal bowel dilatation virtually excludes intestinal atresia. Long et al.51 on the other hand documented a 30% atresia rate in the group of gastroschisis fetuses without antenatal bowel dilatation (6 mm) was predictive of complex gastroschisis (necrosis, atresia, volvulus and perforation) with an odds ratio of 4.13 (95% CI 1.32–12.9). Moreover, each additional millimeter of dilatation was associated with a 10% increased risk of complex gastroschisis. Huh et al.62 reported that intra-abdominal bowel dilatation was associated with a higher rate of postnatal complications, but only when multiple loops were dilated. On the other hand, Badillo et al.63 did not find any correlation between the ultrasound finding of gastrointestinal abnormalities and adverse postnatal outcomes. Finally, Wilson et al.64 concluded that ultrasound-detected bowel dilation (>10 mm) was not predictive of important intestinal complications. The aforementioned studies are difficult to compare, because of the difference in definition of ‘bowel dilatation’. It nevertheless seems clear that significant internal or external bowel dilatation is probably not a favorable sign. Its exact implication however, and how this should guide management, is still unclear. To date, no single marker has shown good sensitivity or specificity for these adverse outcomes. Some investigators have suggested that fetal gastric dilation is associated with fetal heart rate anomalies, volvulus, meconiumstained amniotic fluid at delivery, increased length of stay in the NICU and neonatal death.47,49 However, these results were not confirmed by others.50 Finally, there is no evidence that timing of delivery should be guided by bowel findings. Prenatal Diagnosis 2014, 34, 316–326

IS IN UTERO THERAPY AVAILABLE? Parents: ‘Can we cure my baby before birth?’1–7 Although successful in utero repair of gastroschisis has been reported in an animal model,65 no cases have been performed in humans. Intestinal damage of neonates with gastroschisis is attributed to prolonged exposure to the toxic environment of amniotic fluid.66,67 Amnioexchange/infusion has been proposed to improve bowel quality by increasing amniotic fluid and diluting/removing inflammatory components. Despite 68 controversies, experimental studies on animal models69 and preliminary data in humans70 indicate that amnioexchange might improve the neonatal outcomes in cases of gastroschisis. A randomized controlled clinical trial is currently ongoing (http://clinicaltrials.gov/show/NCT00127946). Amnioinfusion could be proposed in case of severe early preterm oligoamnios.71 Oligohydramnios, which complicates 25% of pregnancies with gastroschisis,72 results from decreased umbilical blood supply and IUGR. Severe oligohydramnios may predispose to irreversible damage to the exteriorized bowel, and increased risk of fetal distress or death due to compression of the umbilical vessels leads to increased risk of fetal distress or life-threatening compression of umbilical vessels. In these cases, amnioinfusion could be proposed, because the risks of the procedure (preterm birth and chorioamnionitis) are lower than the adverse outcomes mentioned earlier.

WHERE AND WHEN SHOULD FETUSES WITH GASTROSCHISIS BE DELIVERED? Parents: ‘Can I have a home birth? When should I deliver?’1–7 There is compelling evidence from the Canadian Pediatric Surgery Network that infants with gastroschisis delivered in a tertiary perinatal center have fewer postnatal complications than infants born in smaller, less experienced centers.73 However, data regarding time of delivery are more confusing. The rationale for delivering gastroschisis fetuses early is that it shortens the duration of bowel exposure to the toxic amniotic fluid. Moreover, the risk of IUFD may be decreased by early delivery. A recent systematic review of the Cochrane database74 was able to include only one small trial,75 and the authors were unable to draw any conclusions regarding preterm birth for infants with gastroschisis, although there was a trend toward a shorter neonatal hospital stay and a shorter length of time on total parenteral nutrition.75 A recent meta-analysis did not demonstrate an increase rate of IUFD after 35 weeks’ gestation,28 and the authors concluded that the risk of IUFD should not be the primary indication for routine elective preterm delivery in these pregnancies.28 However, there is growing evidence from observational studies that late preterm (35–37 weeks’ gestation) delivery may improve postnatal outcomes. A large study of 296 pregnancies with gastroschisis showed that induction of labor at 37 weeks’ gestation was associated with a reduced risk of sepsis, bowel damage and neonatal death when compared with a historical cohort of pregnancies managed expectantly.38 © 2013 John Wiley & Sons, Ltd.

Gastroschisis – what should be told to parents?

Similar findings have been reported by other groups, who looked at two cohorts with different interventions, but were in contrast with studies that evaluated the relationship between gestational age at delivery and outcomes. This is because, when evaluating the linear relationship between age at delivery and outcome, the reason for delivery is not taken into account. As such, infants with severe spontaneous preterm births or iatrogenic preterm births due to in utero distress will bias the preterm group toward worse outcomes (reviewed by Baud et al.38). Of note, 8 of the 12 studies that failed to show any significant benefit to early delivery also failed to show any significant benefit to expectant management.38

WHAT IS THE OPTIMAL MODE OF DELIVERY? Parents: ‘Should I deliver by cesarean section?’1–7 There are conflicting recommendations in the literature concerning the mode of delivery in pregnancies with gastroschisis. This is partially because many studies mixed elective and emergency cesarean sections in the same report, as well as different gestational ages at intervention. In 2001, Segel et al. performed a systematic review of the mode of delivery in women carrying a fetus with an abdominal wall defect.76 There was no significant association between mode of delivery and the rate of primary repair, neonatal sepsis, pediatric mortality and time until enteral feeding or length of hospital stay.76 Since 2000, 13 additional studies evaluated the mode of delivery on postnatal outcomes (Table 1). There were no randomized trials, and all studies were retrospective. Apgar scores 10 years old104,107,111 and 4/14 studies compared their outcomes with a control group.102,104,110,113 None of these studies showed any significant difference between gastroschisis and controls in terms of neurodevelopmental outcomes or school achievements. Of note, response or participation rate varied between 37% and 69%.102,109,113,118 Parents of infants with adverse neurodevelopmental outcomes were probably more likely to participate than those without handicaps. Expecting parents should be informed that long-term neurodevelopmental outcomes or school achievements do not differ from other children, with 30% to 35% of these infants reaching a high level of education.104,118

Table 2 summarizes all studies investigating long-term gastrointestinal outcomes in patients born with gastroschisis since 1990. There is heterogeneity in intensity and frequency of reported symptoms. For example, gastro-esophageal reflux might be self-reported or deducted from anti-acid prescription. Moreover, patients involved in long-term studies were born in the 1990s or even earlier, and significant improvements have occurred in neonatal management since these studies were performed. Symptoms most frequently reported are chronic constipation, gastro-esophageal reflux, food allergy or intolerance and chronic abdominal pain. Their rates decrease in late childhood and adolescence. Hospital readmission is frequent (23.5–50%), mainly for gastroschisis related surgery (25–35%). One of the most severe long-term intestinal complications is short bowel syndrome, a malabsorption disorder that occurs when a large part of the small intestine has been surgically removed or antenatally vanished.97 Short bowel syndrome complicates 4.1% to 9.8% of gastroschisis cases.50,80 The impaired absorptive capacity may cause diarrhea, fatigue and weight loss.119 Some infants remain dependent on parenteral nutrition, with significant morbidity and mortality.120

In 2003, Zaccara et al. extensively studied cardiopulmonary performance in 18 infants aged 7 to 18 years, who were born with large (>4 cm) abdominal wall defects.121 Half of them regularly participated in sports activities, whereas reasons for sport abstinence were parental or medical advice for the others. All of them had normal heart rate, blood pressure, forced vital respiratory capacity and electrocardiogram during physical activity. Maximal oxygen consumption (VO2max) however was significantly lower than controls. The authors concluded that this was likely due to a lack of physical activity. There is no medical reason to limit physical activities in these children. In another study, limited sport activity was only reported in 1 of 22 children born with gastroschisis.112 Despite the fact that 40% of these children were reported not to be involved in sports activities,112,118,119 their physical health may be greatly improved by regular exercise.117,121,129,130 Expecting parents should be informed that there is no medical reason to limit physical activity in these children.

3. Infant growth

6. Abdominal scarring

Parents: ‘Will my child grow normally?’1–7 In children with gastroschisis, childhood growth is within the normal range. Henrich et al. reviewed the outcomes of 40 children with a median follow-up of 6.3 years (range 1–10).112 The weight of only two children (9%) and the height of only three children (14%) were below the third percentile. In a longer follow-up study of 11.5 years in 47 children with gastroschisis, Dickinson et al. described weight and height percentiles of 56th and 59th, respectively.111 When these children reached puberty (follow-up of 16 years), none of the 27 children with gastroschisis were below the third percentile for weight and only one was below his standard for height.107 Similar results were reported by others.102,103,113,115,118,121 4. Neurodevelopment

Parents: ‘Will my child have difficulties learning?’1–7 We identified 14 studies investigating long-term neurodevelopmental outcomes or school achievements (Table 3). Only 2/14 studies enrolled more than 50 infants born with Prenatal Diagnosis 2014, 34, 316–326

5. Physical capacity

Parents: ‘Will my child be able to play football with others?’1–7

Parents: ‘Will my child suffer from his scar?’1–7 Personal attitudes toward cosmetic abdominal scarring were described as excellent or good in 82% of cases.112 Five of 22 children (23%) have had umbilical reconstruction, and the other 17 children (77%) had no umbilicus. Four of them (24%) were troubled by the lack of an umbilicus, seven were unconcerned (41%) and the remaining were too young to assess. Tunell et al. showed that 25% of the children were disturbed by the absence of an umbilicus, but none had chosen plastic reconstruction.105 In a longer follow-up (16 years)108 where all 23 patients born with gastroschisis were left without an umbilicus, 57% of them reported distress during childhood and teasing during sport activities at school. Similar results were described by others.103,106,115,116,118 Interestingly, 9% of children reported being proud of their scar.115 7. Future fertility

Parents: ‘Will my child be able to have children?’

1–7

Involvement of the gonads in the gastroschisis is frequent, and this may affect future fertility. Brantberg et al. reported © 2013 John Wiley & Sons, Ltd.

Prenatal Diagnosis 2014, 34, 316–326

58

58

Mostafavi et al. (2012)103

South et al. (2011)122

57

127

16

Larson et al. (1990)128

5.4 [1–17]

8

Not mentioned

[2–12]

1 [0–11]

[1–28]

7.3 [4–13]

16 [12–23]

6.2 [3–10]

26.5 [17–48]

[1–8]

7.3 [0–33]

0.7

6.3 [1–10]

Not mentioned

5.5 [3–12]

11.5

1.7 [1.4–3.7]

ASBO, adhesive small bowel obstruction; GS, gastroschisis. a Patient born with GS and long-term follow-up.

31

Fashing et al. (1996)118

Cusik et al. (1997)

33

Koivusalo et al. (1999)

Boyd et al. (1998)117

19

20

Kaiser et al. (2000)116

126

11

115

Lunzer et al. (2001)

23

42

11

73

Davies et al. (1997)108

Saxena et al. (2002)

125

Koivusalo et al. (2002)104

Kirana et al. (2006)

59

Van Eijck et al. (2008)123

124

17

South et al. (2008)113

22

47

Dickinson et al. (2012)111

Henrich et al. (2008)

39

Van Manen et al. (2013)109

112

na

References

Age at assessment years, [range]

Table 2 Gastrointestinal long-term outcomes

3.8%

8.7%

20%

59%

5.2%

Chronic constipation

22.6%

1.8%

16%

18.2%

0%

0%

81% severe in 1.7%

89.7%

Gastroesophageal reflux

0%

9.1%

12–21%

Food allergy/ intolerance

52% rarely 6% often

26% mild

7.1% frequent 77.4% rarely

5.2% daily

50%

Chronic abdominal pain

37.5%

3.2%

7.7%

15%

13.6%

Abdominal hernia

1.8%

23.5%

50%

29.3%

Recurrent hospitalization related to GS

25%

34.8%

35%

25.9%

28.2%

Recurrent surgery related to GS

6.3%

8.7%

27%

25%

8.6%

7.8%

Surgery for ASBO

322 K. Lepigeon et al.

© 2013 John Wiley & Sons, Ltd.

Gastroschisis – what should be told to parents?

323

Table 3 Neurodevelopmental long-term outcomes n GS patients with long-term follow-up

References Minutillo et al. (2013)

102

Van Manen et al. (2013)109

89/128

a

39/63a

Age at assessment years, [range]

School/employment achievement

Learning disability/mental delay MIQ = 99 (100.2, NS)c, 1% MIQ < 75, 9% suboptimal neurodevelopmental outcomes

1 1.7 [1.4–3.7]

0% mental delay, 16% minor mental delay 0% cerebral palsy 5% visual impairment in a syndromic context 10% hearing loss (due to prematurity or syndrome)

Mostafavi et al. (2012)103

48

5.5 [3–12]

65% no school assistance

22% learning disabilities

22% assistance 1–3 h/week 8% assistance 4–6 h/week Gorra et al. (2012)110 Dickinson et al. (2012)111 Henrich et al. (2008)

112

46b

2

47/110a

11.5

22

Not mentioned

15.2% special needs (13%, NS)b MIQ 98.2 77% started school at expected age

32% delayed sitting or walking 4% developmental delay and amblyopia

South et al. (2008)113

17/24a

MDI = 101 (108, NS),c PDI = 101 (109, NS)c

1.7

6% MDI < 70, none PDI < 70 Lodha et al. (2004)

114

21

1.3 [0.4–2.3]

14% minor motor delay 5% minor cognitive delay

Koivusalo et al. (2002)104

57

26.5 [17–48]

Lunzer et al. (2001)115

10

[0.5–10]

Kaiser et al. (2000)116

20

[1–28]

26

Not mentioned

23

16 [12–23]

31% students

35% high level of education (30%)c

56% employed

37% medium level education (43%)c

14% unemployed

28% low level education (27%)c

80% in regular school

10% slight learning disabilities 10% marked mental retardation

Boyd et al. (1998)

117

Davies et al. (1997)107

10% moderate trouble with coordination 12% developmental delay 52% still at school 22% full-time employment 5% part-time employment 13% unemployed (not due to GS) 9% unknown

Fasching et al. (1996)

118

20/54

a

8 [3.6–12.4]

65% satisfactory school performances 30% at the top of their class

GS, gastroschisis; MIQ, mean intellectual quotient; NS, non-significant; MDI, Mental Developmental Index of Bayley Scales of Infant development (BSID-II); PDI, Psychomotor Developmental Index of Bayley Scales of Infant development (BSID-II). a Number of patients contacted in the study. b Compared with 46 controls (nonsurgical, nonsyndromic infants discharged from the NICU matched for gestational age, weight and date of birth). c Compared with healthy controls or general population.

thatgonads were exteriorized in 7% and 50% of the male and female fetuses, respectively.131 Undescended testes are frequently observed (8–39%).117,132,133 Conservative management with later orchidopexy, if necessary, is usually recommended.132,133 In case of severe testicular atrophy (6% of the cases), orchiectomy is an option.132 In the study conducted by Koivusalo et al., 2 of 11 adult patients born with gastroschisis had infertility investigations (one male and one female)104. Davies et al. reported three pregnancies in two women, resulting in a healthy baby, a miscarriage and a termination of pregnancy.107 Prenatal Diagnosis 2014, 34, 316–326

CONCLUSIONS We describe the most frequent questions and concerns of parents expecting a fetus with gastroschisis and provide answers supported by an extensive literature review. In the third trimester of pregnancy, close monitoring of fetal well-being is recommended (non-stress testing every 2 weeks from 30 weeks, weekly from 34 weeks and twice weekly after 35 weeks gestation until delivery). Presently, no specific ultrasound marker has been identified to predict postnatal outcomes, but severe bowel dilatation is probably not a favorable sign. Referral to and delivery in a © 2013 John Wiley & Sons, Ltd.

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324

tertiary pediatric center should be encouraged. Timing and mode of delivery remains controversial, yet growing evidences suggest that elective delivery by 37 weeks’ gestation decreases the risk of adverse neonatal outcomes. Cesarean section should be reserved for routine obstetrical indications. Longterm outcomes are good to excellent, with 95% postnatal survival, normal physical and neurodevlopmental capacities. Gastro-esophageal reflux and constipation are however common and should be actively monitored.

ACKNOWLEDGEMENTS We warmly thank Olivier Reinberg, Sam Vasilevsky, Cécile Guenot and Jérome Mathis for their critical review of this manuscript.

WHAT’S ALREADY KNOWN ABOUT THIS TOPIC? • Gastroschisis is a common anterior abdominal wall defect that is diagnosed antenatally in >90% of cases.

WHAT DOES THIS STUDY ADD? • In this manuscript, we reviewed antenatal, neonatal and long-term implications of a diagnosis of fetal gastroschisis, with special attention to parental concerns and questions, in order to make it applicable for clinical practice. • As survival of infants with gastroschisis has improved, our research focus should now move toward improving long-term outcomes.

REFERENCES 1. 2. 3. 4. 5. 6. 7. 8. 9.

10.

11.

12.

13. 14.

15.

16. 17.

18.

19.

20.

21. 22.

www.i-am-pregnant.com/. Cruddace D. www.gastroschisis.co.uk. www.netmums.com. The Parenting Group I. http://www.gastroschisis.co.uk/. www.forums.parenting.com. www.baby-gaga.com. www.babycenter.com. Fillingham A, Rankin J. Prevalence, prenatal diagnosis and survival of gastroschisis. Prenat Diagn 2008;28:1232–7. Chabra S, Gleason CA, Seidel K, et al. Rising prevalence of gastroschisis in Washington State. J Toxicol Environ Health A 2011;74:336–45. Kirby RS, Marshall J, Tanner JP, et al. Prevalence and correlates of gastroschisis in 15 states, 1995 to 2005. Obstet Gynecol 2013;122:275–81. Loane M, Dolk H, Bradbury I, et al. Increasing prevalence of gastroschisis in Europe 1980–2002: a phenomenon restricted to younger mothers? Paediatr Perinat Epidemiol 2007;21:363–9. Reid KP, Dickinson JE, Doherty DA. The epidemiologic incidence of congenital gastroschisis in Western Australia. Am J Obstet Gynecol 2003;189:764–8. Rasmussen SA, Frias JL. Non-genetic risk factors for gastroschisis. Am J Med Genet C Semin Med Genet 2008;15:199–212. Richardson S, Browne ML, Rasmussen SA, et al. Associations between periconceptional alcohol consumption and craniosynostosis, omphalocele, and gastroschisis. Birth Defects Res A Clin Mol Teratol 2011;91:623–30. Werler MM, Mitchell AA, Moore CA, et al. Is there epidemiologic evidence to support vascular disruption as a pathogenesis of gastroschisis? Am J Med Genet A 2009;7:32897. Marengo L, Farag NH and Canfield M. Body mass index and birth defects: Texas, 2005–2008. Matern Child Health J 2013;31:31. Syngelaki A, Chelemen T, Dagklis T, et al. Challenges in the diagnosis of fetal non-chromosomal abnormalities at 11–13 weeks. Prenat Diagn 2011;31:90–102. Barisic I, Clementi M, Hausler M, et al. Evaluation of prenatal ultrasound diagnosis of fetal abdominal wall defects by 19 European registries. Ultrasound Obstet Gynecol 2001;18:309–16. Palomaki GE, Hill LE, Knight GJ, et al. Second-trimester maternal serum alpha-fetoprotein levels in pregnancies associated with gastroschisis and omphalocele. Obstet Gynecol 1988;71:906–9. Saller DN, Jr., Canick JA, Palomaki GE, et al. Second-trimester maternal serum alpha-fetoprotein, unconjugated estriol, and hCG levels in pregnancies with ventral wall defects. Obstet Gynecol 1994;84:852–5. McClellan EB, Shew SB, Lee SS, et al. Liver herniation in gastroschisis: incidence and prognosis. J Pediatr Surg 2011;46:2115–8. Williams CA, Hauser KW, Correia JA, et al. Ascertainment of gastroschisis using the ICD-9-CM surgical procedure code. Birth Defects Res A Clin Mol Teratol 2005;73:646–8.

Prenatal Diagnosis 2014, 34, 316–326

23. Mastroiacovo P, Lisi A, Castilla EE, et al. Gastroschisis and associated defects: an international study. Am J Med Genet A 2007;143:660–71. 24. Stoll C, Alembik Y, Dott B, et al. Omphalocele and gastroschisis and associated malformations. Am J Med Genet A 2008;146A:1280–5. 25. Suver D, Lee SL, Shekherdimian S, et al. Left-sided gastroschisis: higher incidence of extraintestinal congenital anomalies. Am J Surg 2008;195:663–6;discussion 6. 26. Torfs CP, Curry CJ. Familial cases of gastroschisis in a populationbased registry. Am J Med Genet 1993;45:465–7. 27. Kohl M, Wiesel A, Schier F. Familial recurrence of gastroschisis: literature review and data from the population-based birth registry “Mainz Model”. J Pediatr Surg 2010;45:1907–12. 28. South AP, Stutey KM, Meinzen-Derr J. Meta-analysis of the prevalence of intrauterine fetal demise in gastroschisis. Am J Obstet Gynecol 2013, DOI: 10.1016/j.ajog.2013. 29. Kalache KD, Bierlich A, Hammer H, et al. Is unexplained third trimester intrauterine death of fetuses with gastroschisis caused by umbilical cord compression due to acute extra-abdominal bowel dilatation? Prenat Diagn 2002;22:715–7. 30. Gardosi J, Madurasinghe V, Williams M, et al. Maternal and fetal risk factors for stillbirth: population based study. BMJ 2013;346:f108, DOI: 10.1136/bmj.f108. 31. Kanda E, Ogawa K, Sugibayashi R, et al. Stomach herniation predicts fetal death or non-reassuring fetal status in gastroschisis at late pregnancy. Prenat Diagn 2012;33:1302–4. 32. Weinsheimer RL, Yanchar NL, Canadian Pediatric Surgical N. Impact of maternal substance abuse and smoking on children with gastroschisis. J Pediatr Surg 2008;43:879–83. 33. Curry JI, McKinney P, Thornton JG, et al. The aetiology of gastroschisis. Bjog 2000;107:1339–46. 34. Castilla EE, Mastroiacovo P, Orioli IM. Gastroschisis: international epidemiology and public health perspectives. Am J Med Genet C Semin Med Genet 2008;148C:162–79. 35. Carroll SG, Kuo PY, Kyle PM, et al. Fetal protein loss in gastroschisis as an explanation of associated morbidity. Am J Obstet Gynecol 2001;184:1297–301. 36. Payne NR, Simonton SC, Olsen S, et al. Growth restriction in gastroschisis: quantification of its severity and exploration of a placental cause. BMC Pediatr 2011;11:1471–2431. 37. Lausman AY, Langer JC, Tai M, et al. Gastroschisis: what is the average gestational age of spontaneous delivery? J Pediatr Surg 2007; 42:1816–21. 38. Baud D, Lausman A, Alfaraj MA, et al. Expectant management compared with elective delivery at 37 weeks for gastroschisis. Obstet Gynecol 2013;121:990–8. 39. Kuleva M, Salomon LJ, Benoist G, et al. The value of daily fetal heart rate home monitoring in addition to serial ultrasound examinations in pregnancies complicated by fetal gastroschisis. Prenat Diagn 2012;32:789–96.

© 2013 John Wiley & Sons, Ltd.

Gastroschisis – what should be told to parents?

40. Chaudhury P, Haeri S, Horton AL, et al. Ultrasound prediction of birthweight and growth restriction in fetal gastroschisis. Am J Obstet Gynecol 2010;203:17. 41. Shepard MJ, Richards VA, Berkowitz RL, et al. An evaluation of two equations for predicting fetal weight by ultrasound. Am J Obstet Gynecol 1982;142:47–54. 42. Siemer J, Hilbert A, Hart N, et al. Specific weight formula for fetuses with abdominal wall defects. Ultrasound Obstet Gynecol 2008;31:397–400. 43. Netta DA, Wilson RD, Visintainer P, et al. Gastroschisis: growth patterns and a proposed prenatal surveillance protocol. Fetal Diagn Ther 2007;22:352–7. 44. Horton AL, Powell MS, Wolfe HM. Intrauterine growth patterns in fetal gastroschisis. Am J Perinatol 2010;27:211–7. 45. Emusu D, Salihu HM, Aliyu ZY, et al. Gastroschisis, low maternal age, and fetal morbidity outcomes. Birth Defects Res A Clin Mol Teratol 2005;73:649–54. 46. Chen IL, Lee SY, Ou-Yang MC, et al. Clinical presentation of children with gastroschisis and small for gestational age. Pediatr Neonatol 2011;52:219–22. 47. Santiago-Munoz PC, McIntire DD, Barber RG, et al. Outcomes of pregnancies with fetal gastroschisis. Obstet Gynecol 2007;110:663–8. 48. Adzick NS. Fetal myelomeningocele: natural history, pathophysiology, and in-utero intervention. Semin Fetal Neonatal Med 2010;15:9–14. 49. Aina-Mumuney AJ, Fischer AC, Blakemore KJ, et al. A dilated fetal stomach predicts a complicated postnatal course in cases of prenatally diagnosed gastroschisis. Am J Obstet Gynecol 2004;190:1326–30. 50. Alfaraj MA, Ryan G, Langer JC, et al. Does gastric dilation predict adverse perinatal or surgical outcome in fetuses with gastroschisis? Ultrasound Obstet Gynecol 2011;37:202–6. 51. Long AM, Court J, Morabito A, et al. Antenatal diagnosis of bowel dilatation in gastroschisis is predictive of poor postnatal outcome. J Pediatr Surg 2011;46:1070–5. 52. Nick AM, Bruner JP, Moses R, et al. Second-trimester intra-abdominal bowel dilation in fetuses with gastroschisis predicts neonatal bowel atresia. Ultrasound Obstet Gynecol 2006;28:821–5. 53. Heinig J, Muller V, Schmitz R, et al. Sonographic assessment of the extra-abdominal fetal small bowel in gastroschisis: a retrospective longitudinal study in relation to prenatal complications. Prenat Diagn 2008;28:109–14. 54. Ghionzoli M, James CP, David AL, et al. Gastroschisis with intestinal atresia – predictive value of antenatal diagnosis and outcome of postnatal treatment. J Pediatr Surg 2012;47:322–8. 55. Kuleva M, Khen-Dunlop N, Dumez Y, et al. Is complex gastroschisis predictable by prenatal ultrasound? Bjog 2012;119:102–9. 56. Piper HG and Jaksic T. The impact of prenatal bowel dilation on clinical outcomes in neonates with gastroschisis. J Pediatr Surg 2006;41:897–900. 57. Heinig J, Steinhard J, Witteler R, et al. Is there a seasonal variation in the frequency of gastroschisis? Ultraschall Med 2007;28:584–6. 58. Goetzinger KR, Tuuli MG, Longman RE, et al. Sonographic predictors of postnatal bowel atresia in fetal gastroschisis. Ultrasound Obstet Gynecol 2013, DOI: 10.1002/uog.12568. 59. David AL, Tan A, Curry J. Gastroschisis: sonographic diagnosis, associations, management and outcome. Prenat Diagn 2008;28:633–44. 60. Durfee SM, Benson CB, Adams SR, et al. Postnatal outcome of fetuses with the prenatal diagnosis of gastroschisis. J Ultrasound Med 2013;32:407–12. 61. Volumenie JL, de Lagausie P, Guibourdenche J, et al. Improvement of mesenteric superior artery Doppler velocimetry by amnio-infusion in fetal gastroschisis. Prenat Diagn 2001;21:1171–4. 62. Huh NG, Hirose S and Goldstein RB. Prenatal intraabdominal bowel dilation is associated with postnatal gastrointestinal complications in fetuses with gastroschisis. Am J Obstet Gynecol 2010;202:396 e1-6. 63. Badillo AT, Hedrick HL, Wilson RD, et al. Prenatal ultrasonographic gastrointestinal abnormalities in fetuses with gastroschisis do not correlate with postnatal outcomes. J Pediatr Surg 2008;43:647–53. 64. Wilson MS, Carroll MA, Braun SA, et al. Is preterm delivery indicated in fetuses with gastroschisis and antenatally detected bowel dilation? Fetal Diagn Ther 2012;32:262–6.

Prenatal Diagnosis 2014, 34, 316–326

325

65. Stephenson JT, Pichakron KO, Vu L, et al. In utero repair of gastroschisis in the sheep (Ovis aries) model. J Pediatr Surg 2010;45:65–9. 66. Burc L, Volumenie JL, de Lagausie P, et al. Amniotic fluid inflammatory proteins and digestive compounds profile in fetuses with gastroschisis undergoing amnioexchange. Bjog 2004;111:292–7. 67. Guibourdenche J, Berrebi D, Vuillard E, et al. Biochemical investigations of bowel inflammation in gastroschisis. Pediatr Res 2006;60:565–8. 68. Midrio P, Stefanutti G, Mussap M, et al. Amnioexchange for fetuses with gastroschisis: is it effective? J Pediatr Surg 2007;42:777–82. 69. Luton D, de Lagausie P, Guibourdenche J, et al. Influence of amnioinfusion in a model of in utero created gastroschisis in the pregnant ewe. Fetal Diagn Ther 2000;15:224–8. 70. Luton D, de Lagausie P, Guibourdenche J, et al. Effect of amnioinfusion on the outcome of prenatally diagnosed gastroschisis. Fetal Diagn Ther 1999;14:152–5. 71. Sapin E, Mahieu D, Borgnon J, et al. Transabdominal amnioinfusion to avoid fetal demise and intestinal damage in fetuses with gastroschisis and severe oligohydramnios. J Pediatr Surg 2000;35:598–600. 72. Towers CV, Carr MH. Antenatal fetal surveillance in pregnancies complicated by fetal gastroschisis. Am J Obstet Gynecol 2008;198:686 e1-5;discussion e5. 73. Nasr A, Langer JC. Influence of location of delivery on outcome in neonates with gastroschisis. J Pediatr Surg 2012;47:2022–5. 74. Grant NH, Dorling J, Thornton JG. Elective preterm birth for fetal gastroschisis. Cochrane Database Syst Rev 2013;6:CD009394. 75. Logghe HL, Mason GC, Thornton JG, et al. A randomized controlled trial of elective preterm delivery of fetuses with gastroschisis. J Pediatr Surg 2005;40:1726–31. 76. Segel SY, Marder SJ, Parry S, et al. Fetal abdominal wall defects and mode of delivery: a systematic review. Obstet Gynecol 2001;98:867–73. 77. Snyder CW, Biggio JR, Brinson P, et al. Effects of multidisciplinary prenatal care and delivery mode on gastroschisis outcomes. J Pediatr Surg 2011;46:86–9. 78. Boutros J, Regier M, Skarsgard ED, et al. Is timing everything? The influence of gestational age, birth weight, route, and intent of delivery on outcome in gastroschisis. J Pediatr Surg 2009;44:912–7. 79. Abdel-Latif ME, Bolisetty S, Abeywardana S, et al. Mode of delivery and neonatal survival of infants with gastroschisis in Australia and New Zealand. J Pediatr Surg 2008;43:1685–90. 80. Hadidi A, Subotic U, Goeppl M, et al. Early elective cesarean delivery before 36 weeks vs late spontaneous delivery in infants with gastroschisis. J Pediatr Surg 2008;43:1342–6. 81. Snyder CL, St Peter SD. Trends in mode of delivery for gastroschisis infants. Am J Perinatol 2005;22:391–6. 82. Puligandla PS, Janvier A, Flageole H, et al. Routine cesarean delivery does not improve the outcome of infants with gastroschisis. J Pediatr Surg 2004;39:742–5. 83. Salihu HM, Emusu D, Aliyu ZY, et al. Mode of delivery and neonatal survival of infants with isolated gastroschisis. Obstet Gynecol 2004;104:678–83. 84. Singh SJ, Fraser A, Leditschke JF, et al. Gastroschisis: determinants of neonatal outcome. Pediatr Surg Int 2003;19:260–5. 85. Strauss RA, Balu R, Kuller JA, et al. Gastroschisis: the effect of labor and ruptured membranes on neonatal outcome. Am J Obstet Gynecol 2003;189:1672–8. 86. Vilela PC, Ramos De Amorim MM, Falbo GH, et al. Risk factors for adverse outcome of newborns with gastroschisis in a Brazilian hospital. J Pediatr Surg 2001;36:559–64. 87. Driver CP, Bruce J, Bianchi A, et al. The contemporary outcome of gastroschisis. J Pediatr Surg 2000;35:1719–23. 88. How HY, Harris BJ, Pietrantoni M, et al. Is vaginal delivery preferable to elective cesarean delivery in fetuses with a known ventral wall defect? Am J Obstet Gynecol 2000;182:1527–34. 89. Christison-Lagay ER, Kelleher CM, Langer JC. Neonatal abdominal wall defects. Semin Fetal Neonatal Med 2011;16:164–72. 90. Islam S Advances in surgery for abdominal wall defects: gastroschisis and omphalocele. Clin Perinatol 2012;39:375–86. 91. Mortellaro VE, St Peter SD, Fike FB, et al. Review of the evidence on the closure of abdominal wall defects. Pediatr Surg Int 2011;27:391–7.

© 2013 John Wiley & Sons, Ltd.

326

92. Arnold MA, Chang DC, Nabaweesi R, et al. Risk stratification of 4344 patients with gastroschisis into simple and complex categories. J Pediatr Surg 2007;42:1520–5. 93. Bradnock TJ, Marven S, Owen A, et al. Gastroschisis: one year outcomes from national cohort study. Bmj 2011;343:d6749. 94. Abdullah F, Arnold MA, Nabaweesi R, et al. Gastroschisis in the United States 1988–2003: analysis and risk categorization of 4344 patients. J Perinatol 2007;27:50–5. 95. Snyder CL, Miller KA, Sharp RJ, et al. Management of intestinal atresia in patients with gastroschisis. J Pediatr Surg 2001;36:1542–5. 96. Phillips JD, Raval MV, Redden C, et al. Gastroschisis, atresia, dysmotility: surgical treatment strategies for a distinct clinical entity. J Pediatr Surg 2008;43:2208–12. 97. Kronfli R, Bradnock TJ, Sabharwal A. Intestinal atresia in association with gastroschisis: a 26-year review. Pediatr Surg Int 2010;26:891–4. 98. Hoehner JC, Ein SH, Kim PC. Management of gastroschisis with concomitant jejuno-ileal atresia. J Pediatr Surg 1998;33:885–8. 99. Guida E, Pini-Prato A, Mattioli G, et al. Abdominal wall defects: a 33year unicentric experience. Minerva Pediatr 2013;65:179–85. 100. Emil S, Canvasser N, Chen T, et al. Contemporary 2-year outcomes of complex gastroschisis. J Pediatr Surg 2012;47:1521–8. 101. Kassa AM, Lilja HE. Predictors of postnatal outcome in neonates with gastroschisis. J Pediatr Surg 2011;46:2108–14. 102. Minutillo C, Rao SC, Pirie S, et al. Growth and developmental outcomes of infants with gastroschisis at one year of age: a retrospective study. J Pediatr Surg 2013;48:1688–96. 103. Mostafavi RM. Long term follow-up of morbidity and quality of life assiociated with isolated gastroschisis. UT GSBS Dissertations and Theses (open Access). 2012. 104. Koivusalo A, Lindahl H, Rintala RJ. Morbidity and quality of life in adult patients with a congenital abdominal wall defect: a questionnaire survey. J Pediatr Surg 2002;37:1594–601. 105. Tunnel WP, Puffinbarger NK, Tuggle DW, et al. Abdominal wall defects in infants, survival and implication for adult life. Ann Surg 1995;221: 525–30. 106. Lindham S. Long-term results in children with omphalocele and gastroschisis – a follow-up study. Zeitschrift fur Kinderchirurgie : organ der Deutschen, der Schweizerischen und der Osterreichischen Gesellschaft fur Kinderchirurgie = Surgery in infancy and childhood. 1984;39:164–7. 107. Davies BW, Stringer MD. The survivors of gastroschisis. Arch Dis Child 1997;77:158–60. 108. Davies MW, Kimble RM, Woodgate PG. Ward reduction without general anaesthesia versus reduction and repair under general anaesthesia for gastroschisis in newborn infants. Cochrane Database Syst Rev 2002; Issue 3 Art. No.: CD003671. 109. van Manen M, Hendson L, Wiley M, et al. Early childhood outcomes of infants born with gastroschisis. J Pediatr Surg 2013;48:1682–7. 110. Gorra AS, Needelman H, Azarow KS, et al. Long-term neurodevelopmental outcomes in children born with gastroschisis: the tiebreaker. J Pediatr Surg 2012;47:125–9. 111. Dickinson JE, Harris E, Hart S, et al. Long-term outcomes of children with congenital gastroschisis: a regional cohort. Am J Obstet Gynecol 2012;26(S1):S263–4. 112. Henrich K, Huemmer HP, Reingruber B, et al. Gastroschisis and omphalocele: treatments and long-term outcomes. Pediatr Surg Int 2008;24:167–73.

Prenatal Diagnosis 2014, 34, 316–326

K. Lepigeon et al.

113. South AP, Marshall DD, Bose CL, et al. Growth and neurodevelopment at 16 to 24 months of age for infants born with gastroschisis. J Perinatol 2008;28:702–6. 114. Lodha A, Green P, Smissart K, et al. Growth and neurodevelopmental outcome of infants with abdominal walll defects. The Hospital for sick Children: Toronto, Ontario, 2004. 115. Lunzer H, Menardi G, Brezinka C. Long-term follow-up of children with prenatally diagnosed omphalocele and gastroschisis. J Matern Fetal Med 2001;10:385–92. 116. Kaiser MM, Kahl F, von Schwabe C, et al. Omphalocele and gastroschisis. Outcome–complications–follow-up–quality of life. Der Chirurg; Zeitschrift fur alle Gebiete der operativen Medizen. 2000;71:1256–62. 117. Boyd PA, Bhattacharjee A, Gould S, et al. Outcome of prenatally diagnosed anterior abdominal wall defects. Arch Dis Child Fetal Neonatal Ed 1998;78:F209-13. 118. Fasching G, Huber A, Uray E, et al. Late follow-up in patients with gastroschisis. Pediatr Surg Int 1996;11:103–6. 119. Sala D, Chomto S, Hill S. Long-term outcomes of short bowel syndrome requiring long-term/home intravenous nutrition compared in children with gastroschisis and those with volvulus. Transplant Proc 2010;42:5–8. 120. Thakur A, Chiu C, Quiros-Tejeira RE, et al. Morbidity and mortality of short-bowel syndrome in infants with abdominal wall defects. Am Surg 2002;68:75–9. 121. Zaccara A, Iacobelli BD, Calzolari A, et al. Cardiopulmonary performances in young children and adolescents born with large abdominal wall defects. J Pediatr Surg 2003;38:478–81;discussion 81. 122. South AP, Wessel JJ, Sberna A, et al. Hospital readmission among infants with gastroschisis. J Perinatol 2011;31:546–50. 123. van Eijck FC, Wijnen RM, van Goor H. The incidence and morbidity of adhesions after treatment of neonates with gastroschisis and omphalocele: a 30-year review. J Pediatr Surg 2008;43:479–83. 124. Kirana E, Sprot A, Hussein H, Hill S. Long term outcome of infants born with gastroschisis. J Pediatr Gastroenterol Nutr 2006;42(5):E108. 125. Saxena AK, Hulskamp G, Schleef J, et al. Gastroschisis: a 15-year, single-center experience. Pediatr Surg Int 2002;18:420–4. 126. Koivusalo A, Rintala R, Lindahl H. Gastroesophageal reflux in children with a congenital abdominal wall defect. J Pediatr Surg 1999;34:1127–9. 127. Cusick E, Spicer RD, Beck JM. Small-bowel continuity: a crucial factor in determining survival in gastroschisis. Pediatr Surg Int 1997;12:34–7. 128. Larsson LT, Kullendorff CM. Late surgical problems in children born with abdominal wall defects. Ann Chir Gynaecol 1990;79:23–5. 129. Thompson PJ, Greenough A, Dykes E, et al. Impaired respiratory function in infants with anterior abdominal wall defects. J Pediatr Surg 1993;28:664–6. 130. Tunell WP, Puffinbarger NK, Tuggle DW, et al. Abdominal wall defects in infants. Survival and implications for adult life. Ann Surg 1995;221:525–8;discussion 8–30. 131. Brantberg A, Blaas HG, Salvesen KA, et al. Surveillance and outcome of fetuses with gastroschisis. Ultrasound Obstet Gynecol 2004;23:4–13. 132. Hill SJ, Durham MM. Management of cryptorchidism and gastroschisis. J Pediatr Surg 2011;46:1798–803. 133. Yardley IE, Bostock E, Jones MO, et al. Congenital abdominal wall defects and testicular maldescent – a 10-year single-center experience. J Pediatr Surg 2012;47:1118–22.

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Gastroschisis--what should be told to parents?

Gastroschisis is a common congenital abdominal wall defect. It is almost always diagnosed prenatally thanks to routine maternal serum screening and ul...
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