POLSKI

PRZEGLD CHIRURGICZNY
2012, 84, 2, 8285

10.2478/v10035-012-0013-4

Omphalocele, gastroschisis:
epidemiology, survival, and mortality in Imam Khomeini
Hospital, Ahvaz- Iran
Shahnam Askarpour1, Nasrollah Ostadian1, Hazhir Javaherizadeh2,
Shahrzad Chabi1
Department of Surgery, Imam Khoemini Hospital, Ahvaz Jundishapur University of Medical
Sciences, Ahvaz, Iran1
Arvand International Division, Ahvaz Jundishapur University of Medical Sciences, Abadan, Iran2
Gastroschisis and omphalocele are the most common malformation of the anterior abdominal wall.
The aim of the study was to determine the abdominal wall defect frequencies, survival, and mortalities in Ahvaz, Khuzestan province of Iran.
Materia and methods. All cases born with omphalocele or gastroschisis whom born in Imam
Khomeini hospital, were included in this study. Duration of study was 3 years from April 2005. All
patients treated at Imam Khomeini hospital in Ahwaz Iran.
Results. Among 15321 consecutive births 42 patients had abdominal wall deformity. Overall incidence was 27.41 per 10000 live births. Of all cases, 18 (42.9%) of cases were male and 24 (57.1%)
were female. Of all cases, 21.7% of patients with omphalocele and 10% patients with gastroschisis
had other anomalies. Of all cases, 71.8% of patients with omphalocele and 60% with gastroschisis
underwent surgery. The type of anomaly (omphalocele and gastroschisis) had correlation with post
operation prognosis significantly (p<0.001). Of 66.7% of patients under went surgery 46.4% with
mesh and 53.6% without mesh performed. 80% of patients with omphalocele and 20% with gastroschisis were lived.
Conclusions: In our study, mortality was significantly higher in cases with gastroschisis than cases
with omphalocele
Key words: omphalocele, gastroschisis, mortality, abdominal wall defect

Gastroschisis occurs in one of 10/000 live

births and omphalocele in one of 4/000 to 7/000
live birth (1). The Centers for Disease Control
and prevention have estimated the national
prevalence for these defects to be 3.73 and 2.09
per 10,000 live birth for gastroschisis and
omphalocele, respectively (2). Association with
young maternal age ( < 20 yr) and environmental factors have been proposed (3, 4). Khuzestan province have many oil and petrochemical complex. Also, 8 year war was occurred in
this province. So, Khuzestan may had more
possible risk factor than other province in
Iran.
The aim of the study was to evaluate epidemiology, outcome, and mortality of cases with
abdominal wall defect in our hospital.

Material and methods

This retrospective study was carried out in
Imam Khomeini hospital from 2005, 1st April
till 2007, 30th March. During 3 year of study,
all of neonates born with abdominal wall defect
included in this study. Sex, gestational age,
mothers age, parental consanguinity, type of
defect, associated abnormality, and size of
defect were recorded. Data was analyzed by
SPSS version 13.0 (SPSS Inc, Chicago, IL,
USA). Chi-square was used for analysis.
Results
Of 15321 live newborn, 42 cases had abdominal wall defect. Of 42 cases, 32 (76.2%) had

Omphalocele, gastroschisis: epidemiology, survival, and mortality in Imam Khomeini Hospital

omphalocele and 10 (23.8%) had gastroschisis.

Incidence was 27.41/10,000 live birth. Incidence
for omhalocele was 20.88/ 10,000 live newborn
and for gastroschisis was 6.52/ 10,000 live newborn. Omphalocele was significantly more common than gastroschisis (p<0.0001, Chi-square).
Associate anomalies were shown in tab. 1.
Of these cases, 14 (33.3%) were seen in 1st
year of study; 16 (38.1%) in the 2nd; and 12
(28.57%) in the 3rd year of study.
Of all cases, 18 (42.9%) were male and 24
(56.1%) were female (p=0.19, Chi-square).
Of these cases; 69% of cases were the 1st
newborn, 19% of cases were the 2nd newborn,
4.8% were the 3rd newborn, and 7.2% of cases
were the 6th or 7th newborns of their family.
Of all cases, 78.6% were term infants and
21.4% were preterm neonates (p<0.0001, Chisquare). Mean of birth weight was 2971697g
with range 1470 to 4800 g. Parental consanguinity was detected in 28.2% of cases. Cesarean section was done for 59.5% of cases. One
twin pregnancy was seen with one affected
twin.
Associated anomalies were seen in 27.1% of
omphalocele cases and 10% of gastroschisis
cases (p=0.5). Of these cases, 28 cases underwent surgery. Of surgical procedure, mesh was
used in 46.4% of cases. Single staged repair
was done in 82.1% of cases and two staged
repair was used for 17.9% of cases.
Eighty percent of cases with omphalocele
survived. Only, twenty percent of cases with
gastroschisis survived. There was significant
correlation between type of anomaly and outcome (p<0.0001).
Of cases underwent surgery, 65% were discharged with good condition and 35% were
expired.
Gestational age had no significant correlation with post operative prognosis (p=0.176).
Birth weight had no significant correlation
with post operative prognosis (p=0.126).
Mothers age had no significant correlation
with type of diagnosed anomaly (p=0.176).
There was no significant difference between
mother aged 25 yr with mother aged > 25 year
(p<0.295).
Of all cases, 81% had not prenatal ultrasonography and 19% had prenatal diagnosis.
Minimum size of defect was 2 cm and maximum size was 12 cm (5.222.6). There was no
correlation between size of defect and post
surgical prognosis (p=0.31).

83

Table 1. Associated abnormalities in patients with

omphalocele and gastroschisis
Omphalocele
Without anomaly (78%)
Clubfoot (22%)
Cyanotic heart disease
(2,4%)
Macroglossia (2,4%)
Toe deformity (2,4%)
Peripheral cyanosis (2,4%)
Hypospadiasis (2,4%)
Thoracic mass (2,4%)

Gastroschisis
Without anomaly (73,8%)
Heart murmur (9,5%)
Respiratory distress (7,1%)
Gastric rupture (2,4%)
Intestinal atresia (2,4%)

Discussion
In our study, incidence of omphalocele and
gastroschisis was 20.88 and 6.52 /10,000 live
newborn respectively. In our study, associated
anomalies were detected in 21.8% of cases with
omphalocele and 10% of cases with gastroschisis.
Stoll et al, studied 86 cases with omphalocele and 86 cases with gastroschisis. They
found associated anomalies in 74.4% of cases
with omphalocele and 16.6% of gastroschisis.
However, in two studies rate of associated
anomalies were higher in omphalocele, but the
rate of anomalies in Stoll et al. study was much
higher than our study (6). In other study by
Stoll et al, on 265,858 consecutive births, mean
prevalence rate for omphalocele was 2.18/10,000
and for gastroschisis was 1.76/10,000 (6). However this difference may be due to failure in
registration of associated anomalies in chart
of patients.
In a study by Tan et al., incidence of gastroschisis and omphalocele were 0.63/10000
live birth and 2.17/10000 live birth respectively (7). In the current study, incidence of
gastroschisis and ompahlocele was 6.52 and
20.8 /10000 live birth respectively.
In Williams et al. study, between 19681998, incidence of gastroschisis was reported
0.78-4.64 (8). In Rankin et al., study, from
1986-1996, incidence of gastroschisis was reported from 1.48-5.29 (9).
In the study by Golalipour et al, on 10,000
birth (live and still birth) in Gorgan at north
of Iran, omphalocele was detected in 2 case
(10). In our study, we found 24 cases with
omphalocele in live born. However, still born
cases were not included in our study, it seems
that rate of omphalocele was significantly
higher in our province than Gorgan (9).

84

S. Askarpour et al.

In another study by Golalipour et al, on

37951 live newborn from 1 January 1998 till
31 December 2003, omphalocele and gastroschisis were found in 7 (1.84/10,000 live birth)
and 4 (1.05/10,000 live birth) respectively (11).
In our study, rate of both condition was higher than Golalipour et al. study.
As shown above, incidence of gastroschisis
and omphalocele was more common than previous study. Although, it is possible that environmental exposure play a role in the development of gastroschisis (12, 13, 14).
In our previous study, we compared anomalies in two period. First period was 1993-1996
and the second was 2002-2005. In the first
period, 88 cases were included and in the 2nd
period 452 cases were included. In the 1st period of study omphalocele was detected in 6.8%
of cases with anomalies and in the 2nd period
was present in 4.9% of cases. Gastroschisis was
present in 2.72% of cases with anomalies and
1.54% of cases with anomalies in the 2nd period. In the 1st and 2nd periods of study, mortality rate of gastroschisis was 100% and 85.7%
respectively. Mortality rate of omphalocele was
66.7% and 22.7% in the 1st and 2nd period of
study (15). In the current study, period of study
was 2005-2007. Results showed that there is
no difference between mortality rate in the 2nd
period (2002-2005) of previous study and the
current study (2005-2007). However there is
overlap between two study.
In other study gestational age was 38 (32-40)
and mean age was 2900 g with range of 1000
to 3100 g (16). In our study gestational age was
37 was (32-40) and mean age was 2971 g (14704000 g). Findings of two study were similar.
In our study, we found no correlation between age of mother and frequency of omphalocele or gastroschisis. In study done by Materna-Kiruluk et al, there was no significant

correlation between parental age and omphalocele. Risk of gastroschisis is inversely related to both maternal and parental age (17).
In our study, there was 35% mortality in
cases underwent surgery. In Alavrez and Burd
study (18), mortality rate among gastroschisis
cases was 3.5% and was less than our study.
This higher mortality may be due to less equipments available in our hospital compared to
US hospital.
In our study, rate of abdominal wall defect
was higher than other studies conducted in
US. Previous studies, showed that younger
maternal age had association with abdominal
wall defect. In Iran, especially in Khuzestan,
pregnant women is generally younger than
women in Western countries.
Although, Khuzestan had suffered from 8
year war and also other wars that occurred in
Iraq, neighbor of Kuzestan province. Effect of
released chemical material during war may be
the cause of this high prevalence of abdominal
wall defect.
Environmental exposure may play a role in
the development of gastroschisis (13). Khuzestan had many oil and petrochemical company. This high number of chemical company
may be another possible factor that associated
with high rate of abdominal wall defect.

Conclusions
In our hospital, rate of gastroschisis and
omphalocele was higher than other country.
Mortality rate among cases with gastroschisis
was significantly higher than cases with
omphalocele. Due to high rate of abdominal
wall defect in our study, we recommend careful observation of pregnant mother and early
detection of this anomalies.

references
1. Paidas MJ, Crombleholme TM, Robertson FM:
Prenatal diagnosis and management of the fetus
with an abdominal wall defect. Semin Perinatol
1994; 18: 196-214.
2. Grosfeld JL, ONeil JA, Colon AG et al.: editors.
Pediatric surgery. 6th ed St Louis (Mo): Mosby 2006;
p. 1167-71.
3. 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.
4. Goldkrand JW, Causey TN, Hull EE: The challenging face of gastroschisis and omphalocele in
southeast Georgia. J Matern Fetal Neonatal Med
2004; 15: 331-35.
5. Stoll C,Alembik K, Dott B et al.: Omphalocele
And gastroschisis and associated malformation.
Am J Med Gene A2008; 40: 30-37.

Omphalocele, gastroschisis: epidemiology, survival, and mortality in Imam Khomeini Hospital

6. Stoll C, Alembik Y, Dott B et al.: Risk factors in

congenital abdominal wall defects (omphalocele
and gastroschisi): a study in a series of 265858
consecutive births. Ann de Genet 2001; 44(4): 20108.
7. Tan KB, Tan KH, Chew SK et al.: Gstroschisis and
Omplalocele in Singapore: aten-year series from19932002.Singapore Med J 2008; 49: 31-36.
8. Williams LJ, Kucik JE, Alverson CJ et al.: Epidemiology of gastroschisis in metropolitan Atlanta,
1968 through 2000. Birth Defects Res AClin Mol
Teratol 2005; 73: 177-73.
9. Rankin J, Dillon E, Wright C: Congenital anterior abdominal wall defects in the north of England,
1986-1996: occurrence and outcome. Prenat Diagn
1999; 19: 662-68.
10. Golalipour MJ, Ahmadpour-Kacho M, Vakili
MA: Congenital malformations at areferral hospital in Gorgan, Islamic Republic of Iran.
Eastern Mediterranean Health Journal 2005; 11:
707-15.
11. Golalipour MJ, Mobasheri E, Hoseinpour KR
et al.: Gastrointestinal malformations in Gorgan,
North of Iran: epidemiology and associated malformations. Pediatr Surg Int 2007; 23: 75-79.
12. Werler MM, Sheehan JE, Mitchel AA: Maternal
medication use and risk of gastroschisis and small

85

intestinal atresia. Am J Epidemiol 2002; 155: 2631.

13. Dolk H, Vrijheid M, Armstrong B et al.: Risk of
congenital anomalies near hazardous-waste landfill
sites in Europe: the EUROHAZCON study. Lancet
1998; 352: 423-27.
14. Torfs CP, Verlie EM, Oeschsli FW et al.: Apopulation- based study of gastroschisis: demographic, pregnancy, and life style risk factors. Teratology 1994; 50: 44-53.
15. Peyvasteh M, Askarpour S, Javaherizadeh H et
al.: Evaluation of epidemiologic indices of neonates
diseases in the pediatric surgery ward of the Ahvaz
Jundishapur University hospitals during the period
1993-1996 and 2002-2005. Ann Pediatr Surg 2011;
7-9.
16. Pacilli M,Spitz L,Kiely EM et al.: Staged repair of giant omphalocele in the neonatal period. J
Pediatr Surg 2005; 40: 785-88.
17. Materna-Kiryluk A, Winiewska K, BaduraStronka M et al.: Parental age as risk factor for
isolated congenital malformations in aPolish population. Paediatr Perinat Epidemiol 2009 Jan; 23:
29-40
18. Alvarez SM, Burd RS: Increasing prevalence of
gastroschis repairs in the United States: 1996-2003.
J Pediatr Surg 2007; 42: 943-46.

Received: 19.12.2011r.
Adress correspondence: Shahnam Askarpour; Dept. of Surgery, Imam Khomeini Hospital,

Ahraz Jundishapur University of Medical Sciences, Ahraz, Iran