Available via license: CC BY 3.0
Content may be subject to copyright.
Selection of our books indexed in the Book Citation Index
in Web of Science™ Core Collection (BKCI)
Interested in publishing with us?
Contact book.department@intechopen.com
Numbers displayed above are based on latest data collected.
For more information visit www.intechopen.com
Open access books available
Countries delivered to Contributors from top 500 universities
International authors and editor s
Our authors are among the
most cited scientists
Downloads
We are IntechOpen,
the first native scientific
publisher of Open Access books
12.2%
108,000
1.7 M
TOP 1%
151
3,350
Chapter 10
Fetal Abdominal Wall Defects
Roxana Cristina Drăgușin, Maria Șorop-Florea,
Ciprian Laurențiu Pătru, Lucian Zorilă,
Cristian Marinaș, Nicolae Cernea,
Cristian Neamțu and Dominic Gabriel Iliescu
Additional information is available at the end of the chapter
http://dx.doi.org/10.5772/intechopen.71936
© 2016 The Author(s). Licensee InTech. This chapter is distributed under the terms of the Creative Commons
Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution,
and reproduction in any medium, provided the original work is properly cited.
Roxana CristinaDrăgușin, MariaȘorop-Florea,
Ciprian LaurențiuPătru, LucianZorilă, CristianMarinaș,
NicolaeCernea, CristianNeamțu and
Dominic GabrielIliescu
Additional information is available at the end of the chapter
Abstract
Abdominal wall defects (AWDs) represent a group of congenital anomalies that can be
direct impact on pre- and postnatal fetal prognosis and management decisions. The most
frequent anomalies in this group are gastroschisis and omphalocele. The key method
-
-
-
Postnatal early interventions are usually required in specialized pediatric centers.
Keywords:
ultrasound
1. Introduction
herniation of abdominal organs through an unusual opening surrounding the umbilical cord.
The most common two types include omphalocele and gastroschisis. The omphalocele or
© 2018 The Author(s). Licensee IntechOpen. This chapter is distributed under the terms of the Creative
Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use,
distribution, and reproduction in any medium, provided the original work is properly cited.
1]. The correct
2. Embryology/pathophysiology and demographics
2
3
4
56]. It
is considered that an abnormal closure of the ventral body wall folds during the 4th week
7-
paracetamol and pseudoephedrine) may be important contributors to the development of gas-
89]. A combination of genetic and environmental factors was also thought to be
1012].
13
14
10]. The oldest embryologic
15]. Another theory pleads for the rupture of the amniotic membrane at the
16] or for the disruption of the omphalomesenteric (yolk-sac and
17]. As there
is no evidence that for the amniotic rupture almost exclusively on the right side and that the
185
19]. A physiological hernia seldom exceeds 7 mm in diameter or rarely
20].
15
Congenital Anomalies - From the Embryo to the Neonate208
The prevalence of the two most frequent entities of AWDs is reported to be for gastroschisis
21]. The prevalence of gastroschisis has increased in the last
22]. Regarding the prenatal diag-
2324 -
21].
3.1. Gastroschisis
Gastroschisis is an AWD characterized by the herniation of the abdominal viscera represented
by bowel loops and occasionally parts of other abdominal organs outside the abdominal wall
2526
27 28 -
29
30
-
31
32]. The key to an accurate diagnosis is
33]. Misdiagnosis of gas-
chromosomal anomalies and unnecessary amniocentesis may be needed with additional risks
34-
35]. After correctly
herniation of the bowel loops with no covering membrane (e.g. Figure 1a
defect is on the right side of the umbilical cord with a normal umbilical cord insertion. Beside
Fetal Abdominal Wall Defects
http://dx.doi.org/10.5772/intechopen.71936
209
bowel loops with a thickened and echogenic bowel wall (e.g. Figure 1b). Regarding the amni-
36-
-
37
38
-
-
39 40
41
42
43]. The proposed
37
the herniated bowel. Formulas that do not include abdominal circumference can be helpful in
44].
3.2. Omphalocele
into the amniotic cavity through the base of the umbilical cord. The herniated abdominal
Figure 1.
Congenital Anomalies - From the Embryo to the Neonate210
26 -
45
-
6]. The covering membrane of the omphalocele
46-
34 30]. The most frequent
-
mosaic tetrasomy 12p and Miller-Dieker lissencephaly syndrome with deletion of 17p13.3
47]. The risk of aneuploidy
-
14]. Non-chromosomal abnormalities include
48]. The diagnosis is possible even
Figure 2a and b-
-
14 49
(e.g. Figure 2a and b
50 51
-
52]. In
53
Fetal Abdominal Wall Defects
http://dx.doi.org/10.5772/intechopen.71936
211
53].
-
anomalies.
3.3. Ectopia cordis
Figure 2.
Congenital Anomalies - From the Embryo to the Neonate212
54] (e.g. Figure 3a). The most frequent
5556
57
58]. The
59
5760
55]. The
6162
63]. Three-dimensional (3D) scan can improve
64]. Invasive
65]. This rare malfor-
66
67
optimal parental counseling. The couple must decide whether they opt for termination of
63]. Prenatal care and accu-
health care system is lacking currently important physical and material resources.
Figure 3.
Fetal Abdominal Wall Defects
http://dx.doi.org/10.5772/intechopen.71936
213
3.4. Body stalk syndrome or limb: Body wall complex
68]. The anomaly
69] (e.g. Figure 3b). This is due to a large wall defect
26-
70
there is no association with chromosomal anomalies; still placental trisomy 16 or maternal
71]. The recurrence rate has been demonstrated
6872]. The reported
73] or even higher 1 in 7500
74
75 -
7677
natomic landmarks (Figure 4ab).
-
-
77
7874] and oligohydramnios can be present in the second
79].
80
80
an accurate diagnosis is often impossible. In the special situation represented by a twin preg-
80].
3.5. Cloacal and bladder exstrophy
Cloacal exstrophy (CE)-
nal tract that involves a low AWD with the exstrophy of all the structures that form the cloaca
-
such as abnormal overdevelopment of the lower cloaca that prevents mesenchymal tissue
81 82
Congenital Anomalies - From the Embryo to the Neonate214
83]. In
84
85]. Female
Doppler examination of both umbilical arteries can help in accurate localization of the blad-
86
87
-
-
88
89
90
91-
92-
Figure 4.
Fetal Abdominal Wall Defects
http://dx.doi.org/10.5772/intechopen.71936
215
-
vention with minimal damage to the exposed organs.
Bladder exstrophy represents an AWD with a failure of the anterior bladder wall to close
93]. The reported incidence is
94
95
9396]. The accuracy and
-
97]. Bladder exstrophy should be consid-
-
rect detection of the anomaly helps in parental counseling and recommendation for delivery
86]. Besides the bladder
-
98].
3.6. Urachal cyst
-
99]. It is diag-
with excellent prognosis.
complex (OIES complex)
Congenital Anomalies - From the Embryo to the Neonate216
100101]. The diagnosis is acces-
86]. As the prognosis is poor when multiple structural
100101].
3.8. Prune belly syndrome
consequence of severe bladder outlet obstruction and others consider an abdominal muscle
102
103104]. In the most severe form of Prune belly
-
105-
nancy. The postnatal management may include a single comprehensive surgical approach or
106].
3.9. Cantrell pentalogy
107
-
108]. The main event during embryo-
-
108
-
109]. The combination of omphalocele and ecto-
110
86
Fetal Abdominal Wall Defects
http://dx.doi.org/10.5772/intechopen.71936
217
-
vival is uncommon. Prenatal diagnosis is important as termination of pregnancy is the only
option for the couple.
The ultrasound features that best characterize fetal AWDs are presented in Figure 526].
4. Pregnancy surveillance
111
112]. Fetuses
with gastroschisis often tend to be small for gestational age and to develop oligohydramnios
113114
115
Figure 5.26].
Congenital Anomalies - From the Embryo to the Neonate218
intrauterine growth restriction and oligohydramnios seem not to worsen the prognosis of
116-
117 118
111]. Hospital admit-
-
112115].
delivery of fetuses with antenatal diagnosed abdominal wall defects remains a controversy.
119125
others consider a vaginal delivery more suitable in cases with diagnosed fetal abdominal
126130
131135
136
137]. The gestational
age for induced delivery or elective cesarean section is another controversy (preterm versus
138139
120124129140]. The
26
126141].
6. Postnatal prognosis and management
6.1. Gastroschisis
wrapped herniated loops in warm saline as there is an increased risk for water and heat losses
-
-
18
Fetal Abdominal Wall Defects
http://dx.doi.org/10.5772/intechopen.71936
219
1824].
6.2. Omphalocele
18].
-
of omphalocele that include serial reductions or closing the defect gradually after replacing
24].
7. Conclusion
pregnancy must be highlighted. This should be available even in under-developed health
systems.
Author details
1111
213 and Dominic Gabriel Iliescu1
*Address all correspondence to: roxy_dimieru@yahoo.com
Congenital Anomalies - From the Embryo to the Neonate220
Genetics. Part A. 2011;155A:2045-2059
88:201-234
26:1307-1321
19
2007;21:363-369
26:1135-1148
26:127-139
148C:199-212
America. 2000;10:297-316
99:261-269
-
143A:660-671
39:401-406
38:142-147
Fetal Abdominal Wall Defects
http://dx.doi.org/10.5772/intechopen.71936
221
10:235-244
1981;98:228-231
Williams and Wilkins; 1972. pp. 268-271
14(4):341-7
122:275-281
chromosomal abnormalities at 11-13 weeks. Prenatal Diagnosis. 2011;31:90-102
2013;122:1160-1167
57
28
-
146A:1280-1285
-
genital anomalies: How important is it? Prenatal Diagnosis. 2011;31:347-350
Gynecology. 2001;18
36:51-55
Congenital Anomalies - From the Embryo to the Neonate222
-
42:1520-1525
Radiology. 1992 Apr;65(772):298-301
Agarwal R. Prenatal diagnosis of anterior abdominal wall defects: Pictorial essay.
15(3):361-372
162
26
-
30:266-270
201:383e1-383e6
57
2016 Nov 22
27:50-55
30:551-572. viii
2013
death rate in gastroschisis following the introduction of an antenatal surveillance pro-
Research. 2017 Mar;43
-
29:1069-1074
1998;12(2):136
Fetal Abdominal Wall Defects
http://dx.doi.org/10.5772/intechopen.71936
223
-
146A(10):1280-1285
46(1):1-8
46:96-102
-
cency at 11-14 weeks on the likelihood of associated chromosomal abnormality. Prenatal
Diagnosis. 2012;32:1066-1070
-
2010;36:687-692
diagnosis of isolated omphalocele. Prenatal Diagnosis. 2009;29:668-673
45:718-723
190(1):135-141
14(3):287-290
23(3):426-428
-
1(6):
431-434
review. Fetal Diagnosis andTherapy. 2007;22(4):269-273
30(3):805-810
Congenital Anomalies - From the Embryo to the Neonate224
27(8):440-445
Anatomy. 2014;27
-
Gynecology. 2015;54
32(5):865-871.
17(1):64-66
17
28(3):529-548
the 2nd trimester. Fetal Diagnosis and Therapy. 2003;18:342-344
51:49-52
2000;94:284-286
India. 2004;60(1):77-80
Pediatrics. 1981;67:618-621
10(6):416-418
11
2009:6. Art #37
40(4):285-290
Fetal Abdominal Wall Defects
http://dx.doi.org/10.5772/intechopen.71936
225
25(1):67-70.
-
31(4):289-292
2016;2016
1962;88:766-796
23(2):166-170
34:1233-1257
the international clearinghouse for birth defects surveillance and research. American
157C(4):333-343
12:38993
21(5):498-500
-
2012;28(8):781
-
Feb;25
39
19-06-2013
Congenital Anomalies - From the Embryo to the Neonate226
Genetics. 2011;157C(4):321-332
and Gynecology. 1958;11:104-107
-
2013;32:1083-1101
85:961-964
8:140-144
repair of classic bladder exstrophy: A detailed postoperative management strategy for
8(5):549-555
Anatomical Record. 1941;80
-
31(1):13-24. doi:
-
84(7):458-461
23
Gynecology. 01-11-1998
-
11
Fetal Abdominal Wall Defects
http://dx.doi.org/10.5772/intechopen.71936
227
2(2):95-97
107(5):602-614
-
Gynecological Association. 2012;13(2):145-148
35(4):216-220
32(3):441-444
18:309-316
-
Aug;13(6):355-361
12:580-582
. Fetal abdominal wall defects--easy to diagnose--and then what?
18(4):301-304
Medicine. 2016 Dec;29
13:701-706
-
81:53-56
43
100(1):71-74.
Congenital Anomalies - From the Embryo to the Neonate228
-
25(1):12-1.5
169:1050-1053
34:1393-1395
infants with prenatally diagnosed gastroschisis and planned preterm delivery. Pediatric
31
31(6):553
174:1134-1138
-
43
2000;182
121(5):990-998.
2004;39(5):742-745
Gynecology. 2004;104
76:195-199
36:428-430
94:112-116
Fetal Abdominal Wall Defects
http://dx.doi.org/10.5772/intechopen.71936
229
16:164-172
-
diagnosed fetal omphalocele. Fetal Diagnosis and Therapy. 2011;30:60-69
48(11):2251-2255.
65
-
tive preterm delivery at 34 weeks and primary abdominal closure for the manage-
18
47
39:375-386
Congenital Anomalies - From the Embryo to the Neonate230