Pediatrics

Omphalocele/Gastroschisis

OVERVIEW: What every practitioner needs to know:

Omphalocele and gastroschisis are the two most common but also most challenging congenital malformations of the anterior abdominal wall.

Omphalocele is a midline abdominal wall defect with extrusion of abdominal viscera, covered by a membranous sac, into the base of the umbilical cord.

Gastroschisis is a defect in the anterior abdominal wall typically located to the right of the umbilical ring and resulting in the herniation of the intestine without overlying peritoneum or amniotic membrane. The stomach and / or ovaries or testes, which are often undescended, may rarely herniate as well.

Are you sure your patient has omphalocele/gastroschisis? What are the typical findings for this disease?

Omphalocele: Omphalocele is a large (usually >4 cm) midline abdominal wall defect in which rectus muscles insert widely on the costal margins and do not meet in the midline at the xiphoid. Herniated intra-abdominal organs protrude through the base of the umbilicus into the membrane sac made of peritoneum, Wharton’s jelly, and amnion. The umbilical cord inserts into the membrane covering the omphalocele at a location far from the abdominal wall ( Figure 1). It normally contains the small intestine and liver. The defect may be classified as “giant” due to the loss of abdominal domain and considerable size of the defect (Figure 2). At times, the omphalocele sac may be ruptured and the protective effect of an intact membrane is lost (Figure 3).

Figure 1.

Omphalocele with intact membrane. Herniated liver is absent.

Figure 2.

Giant omphalocele with herniated liver. No abdominal domain.

Figure 3.

Omphalocele with ruptured membrane.

Gastroschisis: Gastroschisis is anatomically characterized by evisceration of the abdominal organs through a small opening usually present to the right of the umbilicus ( Figure 4). Gastroschisis always includes the small intestine and may also include the stomach, colon, and gonads. A membrane covering the herniated viscera is always absent. The umbilical cord is present and found in the normal location. Associated organ system malformations are uncommon, although intestinal atresia occurs in approximately 10% due to an ischemic insult either due to constriction of the defect or torsion of the herniated intestine (Figure 5).

Figure 4.

Gastroschisis. Note herniation to the right of the umbilicus and absent peritoneal membrane.

Figure 5.

Intestinal atresia with gastroschisis

Gastroschisis is classified as:

Simple: Absence of associated bowel pathology

  1. Gastroschisis minor: Small abdominal wall defect to the right of and adjacent to the umbilical cord, with a small omental protrusion only.

  2. Closed gastroschisis: Presence of significant closure of the abdominal wall defect around the prolapsed bowel.

  3. Complex: Presence of bowel pathology including malrotation, volvulus, infarction, atresia, perforation, or stenosis.

A ruptured omphalocele may be misclassified as gastroschisis due to lack of membranes, although it can be distinguished from gastroschisis based on the size of the defect, location of the defect, and the presence of exposed liver.

What other disease/condition shares some of these symptoms?

The differential diagnosis of prenatally diagnosed anterior abdominal wall defects includes bladder exstrophy, cloacal exstrophy, cystic cord lesion, exomphalos (congenital umbilical hernia), urachal cyst, omphalocele and gastroschisis. These are easily differentiated on postnatal physical examination and selected imaging studies.

What caused this disease to develop at this time?

  • The flat disc of the embryo folds in on itself in the fourth gestational week in both the craniocaudal and mediolateral directions. Two lateral folds then meet at the anterior midline, creating the pleuroperitoneal space which is then divided into pleural and peritoneal cavities by the septum transversum, carried by the cephalic fold. The caudal fold rises cranially as well, and later the midline constricts around the sac, forming the umbilical celom and eventually the cord. Failure of the lateral folds to meet together at the midline and failure of the intestines to return into the abdominal cavity from the umbilical stalk results in omphalocele. Moreover, because an insult leading to this failure of fusion occurs in embryogenesis, other organ systems are at risk for maldevelopment; thus, the association of omphalocele with other significant associated anomalies.

    From weeks 6-10, there is transient herniation of the intestines into the umbilical cord due to rapid intestinal growth. At 10-12 weeks of gestation, the midgut returns to the abdominal cavity, where it rotates and fixes to the posterior abdominal wall. The specific etiology of gastroschisis is unknown, but multiple theories have been proposed. It may be a malformation caused by abnormal involution of the right umbilical vein at approximately 28-32 days of gestation, disruption of the right omphalomesentric artery within the extraembryonic celom, or the failure of one or more folds responsible for wall closure. Alternatively, gastroschisis may be the result of disruption of the abdominal wall in development caused by teratogens including radiation, aspirin, pseudoephedrine, acetaminophen, or maternal smoking.

What laboratory studies should you request to help confirm the diagnosis? How should you interpret the results?

  1. Serum alpha-fetoprotein (AFP): Prenatal maternal serum AFP is recommended in high-risk cases at 15-20 weeks of gestation. Elevated AFP is observed in up to 42% of fetuses with omphalocele.

  2. Amniocentesis: AFP and acetylcholinesterase elevations in the amniotic fluid in the absence of a myelomeningocele have been correlated with both omphalocele and gastroschisis. It is also recommended for chromosomal analysis, fetal karyotyping in cases of omphalocele. In the event of planned early delivery, fetal lung maturity can be determined.

Would imaging studies be helpful? If so, which ones?

  1. Ultrasound: Diagnosis of omphalocele and gastroschisis is can be made by the 3-D transvaginal ultrasound at a gestational age between 18 and 22 weeks in 80% to 90% of cases. The sensitivity of prenatal ultrasound is 75% for omphalocele and 83% for gastroschisis. Advanced diagnosis provides an opportunity to choose the location, date and time of delivery, as well as optimal delivery room and surgical team preparation. Moreover, the appearance of the fetal intestine can be characterized with specific attention to progressive dilation suggesting threatened bowel. A serial USG every 4 weeks is recommended to assess fetal growth and intestinal development or dilation.

  2. Fetal echocardiogram and fetal MRI: When significant or life threatening anomalies may be present, all infants should undergo echocardiography. Fetal MRI is rarely indicated but may be necessary if ultrasound findings are inconclusive or intracranial abnormality is suspected.

Confirming the diagnosis

  • Please see Figure 6 and Figure 7.

Figure 6.

Treatment algorithm for omphalocele defects

Figure 7.

Treatment algorithm for gastroschisis.

If you are able to confirm that the patient has omphalocele/gastroschisis, what treatment should be initiated?

PREGNANCY AND DELIVERY MANAGEMENT:

Parental counseling is advised soon after the diagnosis is made, including emphasis on associated anomalies, genetic syndromes, morbidity, mortality, and expected results following surgical intervention. The family must be advised that surgical correction may be staged or repeated interventions necessary based on the severity of the defect. Education materials should be provided to the family.

The significant risk of intrauterine growth restriction or stillbirth warrants close fetal monitoring in the third trimester. Early intervention may be necessary if potential serious abnormalities are identified. Delivery should be planned at a tertiary care center to better coordinate the obstetrical, neonatal, and pediatric surgery care.

The preferred optimal mode of delivery of infants with abdominal wall defects has not been determined. Contemporary studies indicate that 53% of children with gastroschisis and up to 80% of the children with omphalocele are delivered by cesarean section. Yet, there does not appear to be a consistent difference in the neonatal outcome between infants delivered vaginally or those delivered by cesarean section. At present, most clinicians advocate delivery at term or at least the gestational age when lungs are mature.

POSTNATAL MANAGEMENT:

Key considerations in the management of gastroschisis and omphalocele are:

1. Stabilize the patient, administer IV fluids, and protect the herniated bowel or omphalocele sac.

Place an orogastric tube to prevent further distention of the bowel.

Place the infant in a bowel bag to protect the bowel and reduce insensible fluid losses.

2. Reduce the evisceration:

Management is dictated by the severity of the defect, the degree of herniated viscera, and the effect of infant physiology. Successful reduction must not negatively impact end organ perfusion or ventilation. Thus, ventilatory pressures, oxygenation, and urine output are all carefully monitored and reduction is abandoned when compromise occurs with attempts at full reduction. In this situation, staged reduction is indicated. While staged reduction is generally accomplished in 5 to 7 days in gastroschisis, staged reduction in a “giant” omphalocele may take months. In the event of severe or lethal anomalies, the infant may undergo palliative therapy alone.

3. Identify and treat the associated anomalies.

4. Recognize and treat complications.

INITIAL MANAGEMENT:

Early neonatal management is directed to limit fluid and protein loss and prevent hypothermia. The exposed viscera is immediately covered by enveloping the infant partially in a plastic bowel bag to minimize handling of the bowel and reduce insensible fluid losses. The clear bag provides close observation of the bowel or omphalocele. Avoid saline-soaked dressings applied directly to the bowel or omphalocele and the use of plastic wrap to secure the dressing. The bowel is examined for vascular compromise and, if present, the bowel should be carefully untwisted to restore perfusion. If this is unsuccessful, it may be necessary to enlarge the abdominal wall defect emergently with a lateral extension of the fascial opening. Orogastric tube placement, IV fluid administration, and broad-spectrum antibiotics follow. After initial stabilization in the neonatal ICU, the infant is transported to the OR for surgical reduction.

Fluid requirements in neonates with these abdominal wall defects within the first 24 hours of life–especially in cases of gastroschisis–exceed the usually accepted maintenance requirements for a normal newborn. These infants may require as much as 150-300 cc/kg in the first day of life to support tissue perfusion. The end points of adequate fluid resuscitation are an appropriate heart rate, blood pressure, urine output of 1-2 cc/kg/hr, and normal base deficit. An appropriate combination of crystalloids and colloids should be used, and naso-gastric losses should be replaced. This increasing requirement has been attributed to antenatal peritonitis, increased insensible loss due to exposed viscera, and excessive fluid transudation as third space loss from inflamed bowel wall. This can be further aggravated in the presence of lymphatic and venous obstruction secondary to incarceration of the bowel within the defect.

SURGICAL MANAGEMENT:

After initial stabilization in the neonatal ICU, the infant is transported urgently to the operating room. The type of repair performed is related to the degree of bowel inflammation, and the relative size discrepancy between available abdominal cavity and the amount of herniated viscera.

Primary abdominal wall closure is the preferred technique; a staged repair is reserved for when primary closure is not possible. The newborn is given general anesthesia, and the herniated viscera is carefully reduced into the abdominal cavity. Attempts should be made to determine if additional intestinal anomalies are present. This may be difficult due to the thickened, inflamed bowel. The feasibility of primary closure is assessed by estimates of intraoperative intra-abdominal pressures, ventilatory pressures, and lower body perfusion to avoid the development of abdominal compartment syndrome. Intra-abdominal pressure can be determined from gastric or bladder pressure measurements. A value of more than 20 mmHg indicates that further reduction be discontinued. Infants suffering intrauterine or peri-natal bowel damage may require bowel resection with temporary intestinal stoma. Alternatively, if free perforation or necrosis is absent, the atretic segments are reduced with planned re-exploration at 4-6 weeks once the inflammatory peel has diminished and permits re-anastomosis.

In the presence of a large defect causing significant visceral-abdominal disproportion or evidence of abdominal compartment syndrome with primary reduction, an alternative approach to primary reduction must be taken. Reduction is abandoned. The herniated viscera are placed into a silastic silo, which in turn is secured either to the fascia with suture or below the fascia with a spring-loaded ring. Over the ensuing days the bowel gradually returns to the abdomen by gravity and gentle reduction of the bowel within the silo (Figure 8). Once fully reduced, the fascial defect and skin are closed (Figure 9). At times, the fascia cannot be closed, primarily due to excessive tension. When primary fascial closure cannot be accomplished, prosthetic mesh is utilized. Non-absorbable material, e.g., Gore-Tex, or absorbable, biologic matrix, e.g., AlloDerm or Surgisis, is placed and skin is used to cover the prosthetic. Rarely, there is insufficient skin to cover the defect and the prosthetic remains exposed, requiring either delayed excision or replacement with a biologic substitute.

Figure 8.

Sequential reduction using silo technique

Figure 9.

Final result after closure

In special circumstances, for example, infants with structural cardiac anomalies, lethal chromosomal anomalies, or extreme prematurity, bedside reduction and the use of prosthetic pre-formed silo may be preferable. Alternatively, elevation of skin flaps to close the skin defect, leaving a fascial defect, or the use of sclerosing agents like silver sulfadiazine to paint the external omphalocele membrane to promote fibrosis and eventual epithelialization, have been utilized. These techniques result in large ventral hernias that become quite challenging to close due to the loss of abdominal domain (Figure 10). Often, multiple operations are needed to achieve final closure. Again, these methods should only be utilized in patients with severe congenital associated anomalies that limit the possibility of using other strategies for closure.

Figure 10.

Skin closure of omphalocele without fascial closure. Note large ventral hernia.

What are the adverse effects associated with each treatment option?

Both in gastroschisis and in omphalocele, the best results are achieved with primary closure with or without the use of prosthetic mesh. There is no survival difference between the appropriately chosen primary or staged closure. Although in some studies, immediate repair has been shown to increase the number of ventilator days required, randomized controlled trials failed to demonstrate any difference in the length of stay, time to return to full enteral feeds, parenteral nutrition days, and overall morbidity and mortality. Available biomaterials further enhance our ability to achieve closure for all abdominal wall defects without intra-abdominal hypertension and with good cosmetic outcomes. The choice between primary or staged reduction should be guided by the changes in the infant's physiology and appearance of the viscera.

The use of skin flaps or sclerosing agents for delayed repair technique is associated with development of large ventral hernia defects. Furthermore, the loss of abdominal domain continues with growth, and results in increasing difficulty to restore the viscera to the abdominal cavity. The result is a series of operations to restore the abdominal domain and correct the herniation of the viscera; this is often associated with significant morbidity and poor cosmetic result when compared with primary or early staged repair. Not surprisingly, delayed closure is associated with prolonged hospital stay, and wound and infectious complications.

Aggressive reduction of the viscera and closure is ill advised. Increased intra-abdominal pressures and abdominal compartment syndrome may ensue with resultant systemic complications, bowel complications such as necrotizing enterocolitis, ischemia or perforation, and even death.

What are the possible outcomes of omphalocele/gastroschisis?

Mortality: Postnatal survival in cases of gastroschisis and omphalocele largely depends on the presence or absence of associated anomalies, the remaining intestinal length, and the degree of visceral-abdominal disproportion. Mortality has markedly decreased in recent years due to improved prenatal diagnosis, in concert with advances in surgical and medical management.

Overall mortality has been found to be approximately 8% to 28% in cases of gastroschisis. Survival for simple gastroschisis is nearly 100%. Mortality for complex gastroschisis is increased and reported up to 28%. Death usually results from cholestatic liver disease secondary to intestinal failure and the need for prolonged total parenteral nutrition.

Mortality in omphalocele has been reported to be only approximately 15% without associated anomalies, but increases, up to 61% if other anomalies are present. In patients with omphalocele and a major cardiac anomaly, mortality approaches 80%. No difference in the survival outcome between giant and minor onphalocele has been reported.

Gastrointestinal function: Long-term gastrointestinal problems are seen in only 7% to 10% of children with abdominal wall defects and are generally related to the degree of intestinal loss from intestinal atresia or injury. Late small bowel obstruction has been reported in approximately 25% of infants with gastroschisis and approximately 13% of those with omphalocele. Eighty-five percent occur in the first year of life. Gastro-esophageal reflux disease affects nearly one half of patients with large defects, and may be complicated by the development of esophagitis and esophageal stricture. Cryptorchidism, inguinal hernia, and recurrent abdominal wall hernia are more frequent in children with abdominal wall defects.

Cosmetic outcome: Cosmetic results are described as excellent or good in the majority of patients. Few patients require umbilical reconstruction due to lack of umbilicus or scar revision later in life.

Physical and intellectual development and quality of life: Long-term follow-up reveals normal growth and development of these children, in the absence of additional congenital anomalies. More than two thirds of these patients achieve their milestones normally, but up to one third demonstrate delayed development in growth and in achieving their developmental milestones. The most pronounced retardation in motor development is observed in young children. Of children under the third percentile for height or weight, most are also young, but developmental delays appear to improve during the first 2 years of life.

Children with isolated defects typically participate in normal activities and education without any compromise in their quality of life. Most patients start kindergarten at the usual age. Restrictions in physical exercise and sports are reported in fewer than 10% of children.

What causes this disease and how frequent is it?

How frequent is it?

  • The incidence of gastroschisis is 3-4/10,000 live births. Its overall incidence has been globally increasing over the past few decades, independent of maternal age.

  • The incidence of omphalocele is 1/110 fetuses by prenatal ultrasound at a gestational age of 18 weeks; however, this decreases to 1/4,000 live births with a male preponderance, due to fetal demise.

What causes the disease?

  • Predisposing factors/Clinical risk factors: The exact etiology of omphalocele and gastroschisis and the associated risk factors remain controversial. It appears that development of these abdominal wall defects involves multiple environmental, lifestyle and/or genetic factors that most likely act in combination with maternal pre-pregnancy lifestyle choices or genetic predisposition.

How do these pathogens/genes/exposures cause the disease?

Omphalocele: Omphalocele is more likely to be associated with multiple anomalies and likely has a chromosomal or genetic etiology. Genetic variations, and especially chromosomal abnormalities, are by far the most recurrently identified factors associated with omphalocele. Chromosomal abnormalities and omphalocele include trisomy 18, trisomy 13, trisomy 21, partial deletions and mutations, and sex chromosome abnormalities such as Turner syndrome (45,X), Klinefelter syndrome, (47,XXY) and triploidy (69,XXX). The recognized association of omphalocele and chromosomal abnormalities strongly implicates a genetic origin.

Additional known risk factors associated with omphalocele include both advanced and very young maternal age, African-American heritage, maternal obesity, preconception use of multivitamins, maternal febrile illness, in vitro fertilization and consanguinity.

Gastroschisis: In contrast to omphalocele, environmental teratogens combined with maternal factors appear responsible for the development of gastroschisis. Little association with genetic factors has been found. Risk factors associated with gastroschisis include young maternal age, young paternal age, Hispanic population, low socio-economic status, less maternal education, poor maternal nutrition, maternal smoking, medications (vasoconstrictors, aspirin, acetaminophen, pseudoephedrine, oral contraceptives), and recreational drugs. Interestingly, an increased incidence of gastroschisis has been observed in communities near toxic waste sites.

Other clinical manifestations that might help with diagnosis and management

Associated malformations: The presence of associated systemic malformations or physical findings characteristic of chromosomal abnormalities suggest omphalocele.

Omphalocele: In descending order of frequency, anomalies include cardiac (septal defects, tetralogy of Fallot and ectopia cordis gastrointestinal tract (diaphragmatic hernia, intestinal duplications, and atresia), musculoskeletal system (Figure 11), genitourinary system, and central nervous system. Chromosomal abnormalities are observed in 40% of omphaloceles, including trisomy 18, trisomy 13, trisomy 21, Turner syndrome, Klinefelter syndrome, and triploidy. Genetic syndromes associated with omphalocele include Beckwith-Weidemann Syndrome (omphalocele, macroglossia, hemihypertrophy, ventricular septal defect (VSD)), pentalogy of Cantrell (omphalocele, anterior diaphragmatic hernia, sternal cleft, ectopia cordis, cloacal exstrophy) (Figure 12), Goltz syndrome, Marshall-Smith Syndrome, Meckel-Gruber syndrome, and CHARGE syndrome (coloboma of the eye, heart defects, atresia of the nasal choanae, retardation of growth and/or development, genital and/or urinary abnormalities, and ear abnormalities and deafness). Due to co-existence of the high prevalence of secondary defects, newborns should undergo comphrehensive evaluation that includes a genetic profile and karyotyping.

Figure 11.

Limb hypoplasia associated with omphalocele- a rare anomaly

Figure 12.

Pentalogy of Cantrell

Gastroschisis: Gastroschisis is rarely associated with a chromosomal abnormality. Typically, infants born with gastroschisis are small for gestational age. Intestinal atresia ( Figure 5) is the most common associated anomaly and is among the leading causes of congenital short gut. Skeletal dysplasias, arthrogryposis, craniosynostosis, and unspecified dwarfism have been observed.

What complications might you expect from the disease or treatment of the disease?

Complications in abdominal wall defects are influenced by birth weight, prematurity, and the presence of associated anomalies. Immediate postoperative complications can be seen in up to 70% to 80% cases of gastroschisis and omphalocele and include postoperative ileus with associated reoperation rate of up to 28%, sepsis, delay in achieving oral feedings, catheter-related infections, and acute renal failure. Two main causes of postoperative morbidity in newborns with an abdominal wall defect are sepsis and acute renal failure.

Additional complications of necrotizing enterocolitis (NEC), intestinal atresias, subsequent short bowel syndrome, prolonged parenteral nutrition, cholestasis, and intestinal failure-associated liver disease are seen with gastroschisis. The incidence of NEC may be reduced by the introduction of early enteral feeding and the use of breast milk. The use of a prosthetic silo can result in silo detachment, an increased rate of infection, and subsequent loss of abdominal domain, especially in large defects associated with giant omphaloceles. Again, the presence of associated anomalies in omphalocele further complicates the postoperative course and, if severe, may delay definitive repair of the abdominal wall defect.

Are additional laboratory studies available; even some that are not widely available?

Please see suggested laboratory studies and imaging sections.

How can omphalocele/gastroschisis be prevented?

There are no known preventive interventions to date for these defects.

What is the evidence?

Frolov, P, Alali, J, Klein, MD. "Clinical risk factors for gastroschisis and omphalocele in humans: a review of the literature". Pediatr Surg Int. vol. 26. 2010. pp. 1135-48.

(In a review of the literature, the authors conclude that there appears to be little evidence to support a genetic cause in the development of gastroschisis, but there is evidence to support environmental teratogens as important contributors to the development of this defect. In contrast, they suggest that in omphalocele, the evidence supports a genetic rather than an environmental etiology.)

Stoll, C, Alembik, Y, Dott, B, Roth, MP. "Omphalocele and gastroschisis and associated malformations". Am J Med Genet A. vol. 146A. 2008. pp. 1280-5.

(In a study of 334,262 consecutive births between 1979 and 2003, the authors observed a striking difference in the prevalence of total malformations between infants with omphalocele (74.4%) and gastroschisis (16.6%). In addition, specific patterns of malformations were associated with each defect with a recognizable malformation syndrome or pattern in 44.2% of infants born with omphalocele.)

Sadler, TW. "The embryologic origin of ventral body wall defects". Semin Pediatr Surg. vol. 19. 2010. pp. 209-14.

(An excellent review of the embryology of ventral body wall defects.)

Wilson, RD, Johnson, MP. "Congenital abdominal wall defects: an update". Fetal Diagn Ther. vol. 19. 2004. pp. 385-98.

(A review of abdominal wall defects including incidence and epidemiology, prenatal evaluation, pregnancy and delivery management, and postnatal outcome.)

Marven, S, Owen, A. "Contemporary postnatal surgical management strategies for congenital abdominal wall defects". Semin Pediatr Surg. vol. 17. 2008. pp. 222-35.

(A review of current techniques and management of abdominal wall defects.)

Weber, TR, Au-Fliegner, M, Downard, CD, Fishman, SJ. "Abdominal wall defects". Curr Opin Pediatr. vol. 14. 2002. pp. 491-7.

(A review of abdominal wall defects, including areas of controversy.)

Rahn, S, Bahr, M, Schalamon, J, Saxena, AK. "Single-center 10-year experience in the management of anterior abdominal wall defects". Hernia. vol. 12. 2008. pp. 345-50.

(A single-center experience of surgical techniques in the management of abdominal wall defects. The authors report that primary skin closure of the defect was achieved in 67%, and a prosthetic patch used in the remaining 33%. Twelve percent required an inner patch. A synthetic patch was used most commonly, but in several instances a biologic prosthetic was employed.)

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. vol. 43. 2008. pp. 479-83.

(In a series of infants undergoing treatment of an abdominal wall defect, nearly a fifth developed a small bowel obstruction requiring laparotomy in 88% of cases. Most cases occured within the first year of life. Infants at greatest risk were those suffering fascial dehiscence or sepsis. Mortality was reported to be 15%.)

Danzer, E, Gerdes, M, D'Agostino, JA. "Prospective, interdisciplinary follow-up of children with prenatally diagnosed giant omphalocele: short-term neurodevelopmental outcome". J Pediatr Surg. vol. 45. 2010. pp. 718-23.

(In a prospective study of infants with giant omphalocele at one year of age, the authors demonstrated a high incidence of significant cognitive, language, and motor delay using the Bayley Scales of Infant Development neuromuscular assessment tool.)

Mollitt, DL, Ballantine, TV, Grosfeld, JL, Quinter, P. "A critical assessment of fluid requirements in gastroschisis". J Pediatr Surg. vol. 13. 1978. pp. 217-9.

(In a seminal study of infants with gastroschisis, the authors demonstrated that the fluid requirements of the newborn with gastroschisis are significantly increased over those of the normal neonate, necessitating aggressive fluid resuscitation in the first 24 hours of life.)

Mortellaro, VE, St Peter, SD, Fike, FB, Islam, S. "Review of the evidence on the closure of abdominal wall defects". Pediatr Surg Int. vol. 27. 2011. pp. 391-7.

(The authors demonstrate that the literature is indeterminate with regards to the optimal management of either omphalocele or gastroschisis. Moreover, it remains indeterminate if immediate or delayed closure is superior.)

Henrich, K, Huemmer, HP, Reingruber, B, Weber, PG. "Gastroschisis and omphalocele: treatments and long-term outcomes". Pediatr Surg Int. vol. 24. 2008. pp. 167-73.

(In a long-term follow-up study of children with abdominal wall defects, the authors found that most patients enjoy good quality of life. For children suffering early development delay and/or gastrointestinal difficulties, most resolve by two years except patients who suffer severe defects.)

Ongoing controversies regarding etiology, diagnosis, treatment

Many areas of controversy exist in the management of omphalocele and gastroschisis:

  • Timing and mode of delivery: Although full-term vaginal delivery has been proposed and practiced by most, few authors have supported caesarean section with reported advantage being decreased exudative fibrinous reaction on bowel surface and theoretical advantage of protecting the sac in omphalocele.

  • Closure technique: There has been continued controversy between primary and staged closure with use of silo. Although primary repair is the method of choice, no difference in survival has been noted between the two. There are data showing the impact of associated anomalies, presence of intestinal atresia, or ischemia playing a larger role in overall outcome than type of abdominal wall defect repair.

  • Bedside vs operating room closure: For infants without significant fibrous peel over the bowel and no obvious associated intestinal anomalies requiring urgent intervention, bedside reduction without general anesthesia can be safely done but has not yet been universally accepted.

  • Repair of atresias: The presence of atresias poses a challenging clinical problem. There has been ongoing debate about optimal timing of atresia repair. Recent studies suggest repair at the first operation only if the atresia is distal, associated with necrosis, and/or perforation and is recognized. Proximal or uncomplicated atresias can be safely addressed and repaired at a later time.

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