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An omphalocele is a rare abdominal wall defect in which a baby's intestines, and occasionally the liver or other organs, remain outside of the abdomen. This is caused by a defect in the development of the muscles of the abdominal wall. It can vary in size, from a few centimeters to most of the abdominal wall.
Receiving the news that your baby has an omphalocele can feel scary and overwhelming. Yet the Colorado Fetal Care Center is at the forefront of diagnosing and treating this condition. We also perform state-of-the-art surgeries that enable babies to live healthy, full lives.
An omphalocele is a birth defect where abdominal organs protrude from the belly and lie exposed outside the abdomen. The organs, usually the intestines and liver, are covered in a thin sac. Associated abnormalities can include a smaller-than-normal abdominal cavity or lungs, organ damage or infection (especially if the sac holding the intestines ruptures).
The abdominal wall with an omphalocele fails to develop properly, leaving the abdominal contents covered by a thin membrane. The size and severity of this condition ranges from small, with only part of the intestines protruding, to large, with entire and multiple organs remaining outside of the abdomen.
The incidence of omphalocele ranges from approximately 1 in 4,000 to 1 in 7,000 live births. There is an increased risk of stillbirth in babies with this condition, which is why we strive for early diagnosis and observation.
The defect is thought to be caused by an abnormality that occurs during the process of body infolding in the embryo at 3 to 4 weeks of pregnancy. While no specific cause is known, omphalocele has been associated with advanced maternal age.
Studies also show that several factors can increase the likelihood of having a baby with omphalocele. Women who are obese or overweight prior to pregnancy are more likely to have a baby with omphalocele, as are women who drink alcohol, smoke cigarettes or take certain kinds of anti-depressants during pregnancy.
Omphalocele can present as part of a syndrome (meaning multiple organ structures are involved) or as an isolated defect (meaning without other abnormalities). Small abdominal wall defects that contain only bowel are associated with an increased risk of chromosomal abnormalities (considered to be the baby's "blueprint").
Other organ abnormalities vary greatly, ranging from single minor abnormalities that are not life-threatening for the baby to multiple complex life-threatening abnormalities that influence long-term prognosis more than the omphalocele itself. Genetic testing is strongly recommended due to the multiple studies that have documented a high rate of chromosomal abnormalities.
Omphalocele is often detected from a routine prenatal ultrasound. The condition can also trigger abnormal results on prenatal blood screening tests. If not discovered during pregnancy, it becomes obvious when the baby is born.
Babies born with omphalocele can be treated in a number of ways depending on the size of the defect, associated abnormalities and gestational age at delivery. We have found that a team approach is best for families who’ve received this diagnosis. We offer comprehensive counseling and advice for parents with a fetus diagnosed with this anomaly. In addition to maternal and fetal medicine specialists, the parents will be able to meet with specialists in pediatric surgery, genetics, neonatology and pediatric cardiology.
After receiving an omphalocele diagnosis, some patients might decide to terminate their pregnancy. After a decision has been reached regarding the continuation of the pregnancy, our team will then focus on detecting preterm labor and intrauterine growth restriction (a condition where the baby is not growing enough while in the uterus). Both of these complications are frequently associated with omphalocele.
There will be many more ultrasounds performed in a pregnancy complicated by omphalocele to assess the baby’s growth and the amount of amniotic fluid. In addition, during ultrasound assessment, we observe for occasional rupture of the omphalocele membrane which can expose the herniated intestines to amniotic fluid. The goal with omphalocele is to deliver the baby as close to term as possible.
C-sections are only recommended for specific cases, usually when the defect in the fetal abdomen measures 5 cm or greater or if the liver is entirely outside the body.
Omphalocele treatment plans depend on the number of organs involved and how much of those organs remain outside of the belly at birth. If only parts of the intestines are protruding, surgeons return them to the abdomen soon after birth and close the opening in the abdominal wall. If the omphalocele is large with multiple organs exposed, or there are associated respiratory problems, physicians usually take a phased approach. In the most severe cases, doctors will take time to allow the body to grow skin over the membrane. Ace wraps can then be used to develop space to accommodate the organs, with repair of the defect occurring at 1-2 years of age. Currently, there are no fetal (in utero) interventions offered to treat omphalocele.
Modern surgical advances have made life not only possible, but also probable, for children born with this birth defect. The omphalocele survival rate for babies with no additional abnormalities is 90 percent. Those with other defects have a survival rate of 70 percent.
Babies with multiple organs exposed, as well as related abnormalities such as smaller-than-average lungs, can experience ongoing breathing and heart problems. These children require long-term care and monitoring from a multidisciplinary team of specialists.
The diagnosis of omphalocele has been made as early as 10 to 12 weeks of gestation by trans-vaginal sonography, when an echogenic mass nearly equal to the size of the diameter of the fetal abdomen was found anterior to the fetal abdomen (Brown et al., 1989). The use of three-dimensional transvaginal ultrasound may facilitate this early gestation diagnosis (Anandakumer et al., 2002; Tonni et al., 2006). The ultrasonographic appearance of omphalocele varies depending on the size and location of the defect, the presence of ascites and the organs contained within the defect. However, a principal diagnostic feature of omphalocele is the umbilical cord insertion into the membrane covering the abdominal wall defect. This contrasts with gastroschisis, in which the defect is immediately to the right of the normal umbilical cord insertion into the abdominal wall.
The cord insertion site at the caudal apical portion of the omphalocele membrane can be visualized with color flow Doppler studies on sagittal or oblique images. An additional diagnostic feature is the presence of the intrahepatic portion of the umbilical vein coursing through the central portion of the defect. Omphaloceles are characterized in utero by the presence of a membrane; however, occasionally this membrane will rupture. In cases of ruptured omphalocele, the abdominal contents are floating free in the amniotic cavity, similar to gastroschisis. However, unlike gastroschisis, in ruptured omphaloceles, the defects are usually large and have at least exposed liver if not extracorporeal liver.
Elevated maternal serum α-fetoprotein (MSAFP) levels have traditionally been associated with open neural tube defects, but they are also associated with ventral abdominal wall defects (Brooke et al., 1979; Killam et al., 1991; Stiller et al., 1990). The sensitivity of MSAFP screening for the detection of abdominal wall defects will vary depending on whether it is omphalocele or gastroschisis and on the cutoff value of MSAFP used (Paidas et al., 1994). MSAFP screening has a much higher sensitivity for detecting gastroschisis than for detecting omphalocele. Palomaki et al. (1988) found that at each cutoff value of MSAFP, detection rates were higher for gastroschisis than for omphalocele. For example, at a cutoff value of >2.5 multiples of the median (MoM) and >3.0 MoM, the detection rates were more than 98 percent and 71 percent, and 96 percent and 65 percent for gastroschisis and omphalocele, respectively. The median MSAFP values for cases of omphalocele in this study were 4.1 MoM (Palomaki et al., 1988). The poorer detection rate for omphalocele is thought to be due to the presence of the intact amnioperitoneal membrane covering the abdominal cavities in unruptured omphalocele, as opposed to direct exposure of bowel to the amniotic fluid in gastroschisis (Paidas et al., 1994).
Once identified, a sonographic estimation of the size of the omphalocele, contents of the omphalocele sac, location of the umbilical cord insertion relative to the herniation and the presence of an amnioperitoneal membrane should be documented. A careful sonographic search for other fetal anomalies should also be performed, including fetal echocardiography. Chromosomal analysis is strongly recommended. We have found that a team approach provides comprehensive counseling and advice for parents with a fetus diagnosed with this anomaly. In addition to maternal and fetal medicine specialists, the parents should meet with specialists in pediatric surgery, genetics, neonatology and pediatric cardiology. This type of approach, coordinated by the maternal and fetal medicine specialists, affords the parents the opportunity to ask questions regarding postnatal surgery, postoperative care and long-term outcomes. If chromosomal abnormalities, associated anomalies or a particular syndrome is suspected, these issues can be further discussed in detail. After a decision has been reached regarding continuation of the pregnancy, attention is then focused on antepartum surveillance for the development of preterm labor and intrauterine growth restriction. Both of these complications are frequently associated with omphalocele. Rates for preterm delivery range from 26 to 65 percent and for intrauterine growth restriction from 6 to 35 percent (Carpenter et al., 1984; Lafferty et al., 1989; Sermer et al., 1987; Sipes et al., 1990a, 1990b). There is also a high rate of emergency cesarean delivery because of fetal distress (Molenaar and Tibboel, 1993; Moretti et al., 1990). Because of the high incidence of intrauterine growth restriction, we perform serial ultrasound examinations to assess fetal growth and amniotic fluid volume. In addition, during ultrasound assessment we observe for occasional rupture of the omphalocele membrane, which exposes the herniated intestines to amniotic fluid.
In up to 50 percent of cases, significant pulmonary hypoplasia and pulmonary hypertension may complicate the neonatal course, particularly in giant omphaloceles (Lee et al., 2006; Tsakayannis et al., 1996). We routinely recommend MRI total lung volume assessment at 32 to 34 weeks’ gestation to help identify fetuses at risk for these complications which, if present, become the overriding determinant of management in omphalocele. The site and mode of delivery have been debated in the obstetric literature (Lewis et al., 1990; Segel et al., 2001; Lurie et al., 1999). The goal of the management of fetuses with omphalocele is to deliver the fetus as close to term as possible. Delivery at a tertiary care center provides optimal immediate care for the newborn (Geijn et al., 1991; Hsieh et al., 1989; Lafferty et al., 1989; Lewis et al., 1990). In addition, transporting the pregnant woman before delivery, rather than transporting the neonate after delivery, provides immediate neonatal surgical care and eliminates the risk of transporting a critically ill newborn.
Mode of delivery — vaginally or by cesarean — has been the subject of several retrospective reviews. No results from available prospective randomized trials have settled this issue. Older literature advocated the use of cesarean section (Cameron et al., 1978). However, the most recent retrospective reviews do not support the idea that cesarean delivery is associated with an improved survival rate (Kirk et al., 1995; Lurie et al., 1999; Segel et al., 2001; Sermer et al., 1987; Sipes et al., 1990; Moretti et al., 1990). None of the six reported series show any benefit to cesarean delivery. The outcome of giant omphaloceles was not specifically addressed in these studies. Several other authors do not support routine cesarean delivery for fetuses with omphalocele (Carpenter et al., 1984; Lewis et al., 1990; Hasan and Hermansen, 1986; How et al., 2000; Hsieh et al., 1989; Lafferty et al., 1989). Labor itself does not seem to adversely affect outcomes, based on the study by Lewis et al. (1990), who compared outcome data from infants delivered via elective cesarean section with those whose delivery was preceded by labor. In cases of small omphaloceles, we currently recommend vaginal delivery and reserve cesarean delivery for routine obstetric indications. However, in isolated cases of giant omphalocele with a defect in the fetal abdomen measuring 5 cm or greater by ultrasound examination, cesarean delivery may be necessary to avoid dystocia. Particularly in cases of extracorporeal liver, we recommend delivering by cesarean section. This approach underscores the need for reevaluation of the defect as pregnancy progresses.
Omphalocele treatment for newborns is a staged approach. Delivery should occur in a tertiary care center, with neonatologists available for immediate resuscitation. Initial treatment consists of airway stabilization and sterile wrapping of the abdominal defect to preserve heat and minimize insensible fluid loss.
A complete physical examination should be performed to rule out a syndromic diagnosis. Peripheral vascular access should be established and intravenous fluids given. Mechanical ventilation is frequently necessary, especially postoperatively, when abdominal content replaced into a small abdominal cavity impede diaphragmatic occlusion and lung expansion. Antibiotics are generally given postoperatively. Initial treatment of the newborn is directed toward preoperative stabilization. Significant pulmonary hypoplasia and associated pulmonary hypertension may complicate the neonatal management of omphalocele from the delivery room on. This may be the most challenging management feature of up to 50 percent of neonates with giant omphaloceles.
Omphalocele can present as part of a syndrome or as an isolated defect. The most important prognostic variable is the presence of associated malformations or chromosomal abnormalities. Visceral malformations can accompany omphalocele in 50 to 70 percent of the cases and chromosomal abnormalities can be seen in 30 to 69 percent (Paidas et al., 1994; Brantberg et al., 2005; Lakasing et al., 2006). Interestingly, the absence of the liver in the omphalocele has been correlated with fetal karyotypic abnormalities and perinatal mortality. Nyberg and colleagues (1989) were the first to report an association between omphalocele contents and fetal chromosomal abnormalities. Other investigators have validated the finding that small defects in omphalocele that contain only bowel are associated with an increased risk of chromosomal abnormalities (Benacerraf et al., 1990; Getachew et al., 1991). In one study, chromosomal abnormalities were present in all 8 fetuses with intracorporeal liver, as opposed to 2 of the 18 fetuses with an extracorporeal liver. They also found a significant association between advanced maternal age (33 years and older) and abnormal karyotype. Gilbert and Nicolaides (1987) found that in a series of 35 fetuses, there was a high rate of chromosomal abnormalities (54 percent) with a predominance of trisomy 18 (17 of 19 cases of chromosomal abnormalities). They also demonstrated a high male:female ratio (3:1). This is in contrast to gastroschisis, in which the male:female ratio is 1:1 in the majority of studies (Salinas et al., 1979). Brantberg et al. found a higher incidence of karyotypic abnormalities when the omphalocele was central (69 percent) as opposed to epigastric (12.5 percent) in location (Brantberg et al., 2005).
The constellation of other associated malformations varies greatly, ranging from single, minor, nonlethal abnormalities to multiple complex life-threatening abnormalities that influence long-term prognosis more than the omphalocele itself. The pediatric literature (as opposed to the obstetric literature) has reported a better prognosis for neonates with omphalocele, due to the fact that many of the fetuses with multiple associated anomalies die in utero or during the immediate perinatal period. The report from Rijhwani et al. from King’s College Hospital is illustrative of this point with survival of 34 of 35 neonates undergoing primary or staged closure (Rijhwani et al., 2005). The same institution reported that fewer than 10 percent of the 445 prenatally diagnosed cases of omphalocele survived to repair (Lakasing et al., 2006).
Several investigators have described the impact of associated anomalies on survival in cases of omphalocele. Hughes et al. (1989) reviewed a series of 46 cases detected by prenatal ultrasound examination from three high-risk obstetric referral centers. In 43 of 46 cases, adequate follow-up information was available. Twenty-nine of the 43 cases (67 percent) had additional malformations, with 23 (79 percent) considered major and 6 (21 percent) considered minor. Three of the 29 pregnancies were terminated. There was a total of 58 individual anomalies in the 26 fetuses in which the pregnancy was continued. Cardiac anomalies were the most common (14 cases), including ectopia cordis (4). The other systems involved were skeletal (9), gastrointestinal (6), genitourinary (6) and central nervous (7). Fetal mortality was most strongly associated with the presence of concurrent malformations. Twelve of the 15 fetuses (80 percent) with concurrent malformations died, and the 3 that survived had isolated minor abnormalities. This was in contrast to 7 fetuses without additional anomalies who survived. In the series from Hughes et al., the size of the omphalocele was not associated with fetal mortality. Six of the 10 survivors had a transverse omphalocele:abdomen ratio of >0.6 and 2 omphaloceles measured more than 10 cm. Abnormal amniotic fluid volume was present in 9 of the 12 fetuses that died spontaneously and 3 of these had no abnormalities detected on sonographic examination.
Tucci and Bard (1990) reviewed a 5-year Canadian experience consisting of 28 cases of omphalocele. They initially divided their cases into 2 groups on the basis of the size of the defect, small (<5 cm) and giant (>5 cm). Of the 12 fetuses with small omphaloceles, only 1 died whereas 10 of the 16 infants with giant omphalocele died and all except 1 had severe associated anomalies. There were 5 cases of congenital heart disease, 3 diaphragmatic hernias and 2 central nervous system malformations. Of note, none of the 6 surviving infants had associated severe malformations. In this series, 4 of the survivors had liver herniation, which suggests that giant omphaloceles can have a favorable prognosis if other severe anomalies are not present.
Nicolaides et al. (1992) compiled their 8-year experience with omphalocele and reviewed both the obstetric and pediatric literature regarding the presence of chromosomal abnormalities and associated malformations. Of the 116 cases of omphaloceles, 87 (75 percent) had associated malformations. They also found a higher incidence of chromosomal abnormalities when the omphalocele contained only bowel as compared with omphaloceles that contained liver and bowel (25 of 44 vs. 17 of 72). In their summary, of 349 cases detected antenatally, 229 (65.6 percent) had associated malformations. Summarizing 13 studies with postnatal follow-up, an overall incidence of associated anomalies is 50 percent. They also noted an association with neural tube defects in chromosomally normal fetuses (Ardinger et al., 1987).
Nicolaides’ group reported an 11-year experience from the Harris Birthright Centre for Fetal Research at King’s College Hospital with 445 cases of omphalocele (Lakasing et al., 2006). In 250 cases (56 percent) the karyotype was found to be abnormal and in 130 cases (30 percent) the karyotype was normal with the remainder declining karyotype analysis. In the group with karyotype abnormalities, 248 (99 percent) underwent termination of pregnancy or died in utero. Among the 130 cases with normal karyotype, 74 (56 percent) were found to have associated structural anomalies. Because of the high incidence of associated congenital cardiac disease (19 to 32 percent), we recommend fetal echocardiography when an omphalocele is diagnosed (Carpenter et al., 1984; Copel et al., 1986; Crawford et al., 1985; Greenwood et al., 1974). The incidence of congenital heart disease is related to the embryology of the body-fold defect. Ten percent of neonates with lateral-fold defects have congenital heart disease, whereas the incidence approaches 100 percent if the cephalic fold is affected. Alternatively, if the caudal fold is involved, the incidence of associated congenital heart disease is low (Carpenter et al., 1984; Copel et al., 1986; Crawford et al., 1985; Greenwood et al., 1974).
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Anesthesiology, Anesthesiology - Pediatric
Cardiology - Pediatric, Pediatrics
Surgery - Pediatric, Surgery
Cardiology - Pediatric, Pediatrics