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Pediatric Omphalocele and Gastroschisis Treatment & Management

  • Author: James G Glasser, MD, MA, FACS; Chief Editor: Ted Rosenkrantz, MD  more...
 
Updated: Apr 28, 2015
 

Medical Care

Intestinal inflammation

Intestinal inflammation may occur with either gastroschisis or rupture of an omphalocele.

The eviscerated intestine may appear entirely normal, as if a surgeon had made a fresh laparotomy incision. Alternatively, the intestines may be grossly abnormal, matted together, congealed, and covered with a thick inflammatory membrane or “peel”. Function follows appearance; in the first instance, babies may tolerate feedings immediately. In the second, they may require prolonged parenteral nutrition because of intestinal dysmotility and malabsorption. The extent of intestinal dysfunction depends on the magnitude of the inflammatory and ischemic injury caused by exposure to the amniotic fluid and compression of the herniated intestinal mesentery by the abdominal wall defect.

Histology reveals atrophy of the myenteric ganglion cells.

The inflamed intestine is dysmotile, with prolonged transit time and decreased absorption of carbohydrate, fat, and protein. These deleterious effects remit as the inflammation resolves, usually in 4-6 weeks. During this time, total parenteral nutrition (TPN) is required.

Intact omphalocele

Neonates with intact omphaloceles are usually in no distress unless associated pulmonary hypoplasia is present.

The baby should be carefully examined to detect any associated problems, such as Beckwith-Wiedemann syndrome, chromosomal abnormalities, congenital heart disease, or any other associated malformations.

Maintenance intravenous (IV) fluids are administered, and the omphalocele sac is covered with a nonadherent dressing, such as Xeroform and Kerlix, and covered with Saran wrap to preserve body heat and moisture.

Prophylactic antibiotics may be given preoperatively if an associated intestinal anomaly is suspected.

Closure of a small or moderate-sized omphalocele is accomplished without difficulty.

A baby with a ruptured omphalocele is treated the same way as a baby with gastroschisis.[16] See the image below.

Baby with a ruptured omphalocele. Baby with a ruptured omphalocele.

Closure of giant omphaloceles containing the liver is always challenging.[26, 27, 28] See the images below.

Giant omphalocele treated with topical agents for Giant omphalocele treated with topical agents for several weeks. The omphalocele sac will absorb, leaving granulation tissue that gradually epithelializes.
The omphalocele sac was adherent to the protuberan The omphalocele sac was adherent to the protuberant liver. It was covered with Gore-Tex so that gradual reduction could be effected.
The Gore-Tex sheet is imbricated, gradually reduci The Gore-Tex sheet is imbricated, gradually reducing the liver into the abdominal cavity: the rectus muscles are pulled over the liver.
Final skin closure of the giant omphalocele was de Final skin closure of the giant omphalocele was delayed because the baby developed respiratory distress. Unfortunately, the patch became infected and was removed. Later, bipedicled flank flaps were used to close the giant omphalocele, but reduction was lost.
Split-thickness skin grafts were applied to the fl Split-thickness skin grafts were applied to the flank wounds resulting from mobilization of the bipedicle flaps.

Gastroschisis

Respiratory distress in a neonate with gastroschisis may respond to gastric decompression, although endotracheal intubation may still be needed.

Fluid, electrolyte, and heat losses must be minimized and corrected. Administer an intravenous fluid bolus (20 mL/kg lactated Ringer solution or normal saline), followed by 5% dextrose/0.45 normal saline with potassium chloride (once a urine output is established) at 2-3 times the baby's maintenance fluid rate. This also helps compensate for third space (within the lumen of the gut and tissue edema) loses.

The baby should be placed under a radiant heater. The eviscerated intestine should be situated on top of the baby's abdomen and wrapped with Kerlix in a way that avoids applying traction upon the bowel mesentery.

A urinary catheter should be inserted to monitor urine output and asses the efficacy of fluid resuscitation. A rectal examination should be performed to dilate the anal canal. Reduction of the herniated viscera is facilitated by evacuating meconium from the sigmoid colon; this can be easily accomplished during the operative procedure.

Broad-spectrum antibiotics are administered to prevent contamination of the peritoneal cavity.

A central venous line is placed to provide parenteral nutrition and minimize catabolic protein loss during the period of GI dysfunction.[20, 21, 22]

Consultations

Neonatologists and pediatric surgeons share the responsibility for the treatment of these babies.

Consultation with a cardiologist, pulmonologist, gastroenterologist, and geneticist may be indicated.

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Surgical Care

Omphalocele

Ambroise Pare, a 17th-century French surgeon, accurately described the dire consequences of opening the omphalocele sac to obtain closure of the abdominal wall. His experience encouraged conservative treatment such as squeezing the sac to reduce the herniated viscera or painting the sac with escharotic agents to promote contraction and epithelization. This approach prolongs healing, and the sac may rupture, and a wound infection may result. Even if complications do not occur, the healing of such a large wound exacts a significant metabolic and nutritional toll.

Healing may be hastened by mobilizing skin flaps to cover the omphalocele sac (Gross technique); however, this results in the creation of a large ventral hernia.

In 1967, Schuster developed a technique that more expeditiously treats babies with giant omphaloceles. It may also be used to correct ventral hernias created by skin flap closure.

A circumferential incision is made along the skin-omphalocele junction; the omphalocele membrane is left intact. The incision is extended in the midline and the rectus fascia is exposed from xiphoid to pubis. Teflon sheets are sutured along the edge of the fascia and approximated over the omphalocele sac.

Reduction is effected by gradually pulling the Teflon sheets and attached rectus muscles over the liver and suturing them in the midline. At an appropriate time, the Teflon sheets are removed, the omphalocele sac is excised, and a Dual Mesh patch (Gore-Tex) is sutured circumferentially to the fascia. See the image below.

Silon sheets are pulled over the omphalocele sac, Silon sheets are pulled over the omphalocele sac, elevating the rectus muscles, and, because of their attachment to the costal arch, expanding the thoracic cavity. The Silon sheets are removed and replaced by a permanent Gore-Tex patch that is covered by skin flaps.

The patch is made larger than the defect so that the anterior abdominal wall has a concave appearance, lessening pressure on the diaphragm. Skin flaps are mobilized laterally and approximated over the patch.

The patch is attached to the margins of the defect, superiorly to the costal arch, inferiorly to the pubis, and laterally to the rectus fascia. Expansion of the abdominal cavity is stimulated by the increased intra-abdominal pressure, which elevates the costal arch and expands the thorax, providing space for lung inflation.

A Gore-Tex patch requires skin coverage, whereas an AlloDerm (acellular human dermis) does not. It is vascularized by the underlying liver and its interstices will be filled by ingrowth of epithelium. Because it is vascularized, the patch is no longer a foreign body that may become infected and require removal; however, AlloDerm is not rigid and a ventral hernia will develop that ultimately will require repair.[29, 30] Ssee the images below.

Closure of a giant omphalocele with an AlloDerm pa Closure of a giant omphalocele with an AlloDerm patch.
Two months after implantation: epithelialization o Two months after implantation: epithelialization of the AlloDerm patch.
Eight months after implantation: epithelization is Eight months after implantation: epithelization is nearly complete, but a huge ventral hernia has developed.

Gastroschisis

In 1969, Allen and Wrenn adapted Schuster's technique to treat gastroschisis.

Silastic sheets are sutured to the full thickness of the extended abdominal wall defect and closed over the eviscerated intestine, whose reduction is facilitated by stretching the abdominal musculature, emptying the stomach and bladder, and manually evacuating the colon.

The major factor permitting reduction of the extruded viscera is resolution of the intestinal inflammation; in time the rigid, congealed mass is transformed into multiple soft, pliable loops of intestine, which can fit into the nooks and crannies of the abdominal cavity.[31, 32]

Too tight a closure of the abdominal wall must be avoided, for this limits excursion of the diaphragm and necessitates increased inspiratory pressure to compensate for the increase in ventilatory resistance. In general, peak inspiratory pressures (PIP) higher than 25 mm Hg should be avoided. High-frequency oscillatory ventilation may be an alternative to conventional ventilation if intra-abdominal pressures are markedly increased.[33]

In addition, tight closure of the abdominal cavity impedes venous return to the heart, which compromises cardiac output and decreases renal blood flow and glomerular filtration rate. Renal vein thrombosis and renal failure may ensue. Diminished mesenteric blood flow compounds the risk of necrotizing enterocolitis (NEC) because the intestine of these infants is immature immunologically and the dysmotility leads to stagnation and bacterial overgrowth.[34]

The intra-abdominal pressure can be measured by connecting a manometer to a Foley catheter or a nasogastric tube. The central venous pressure, intravesical pressure, and the intragastric pressure should not exceed 20 cm H2 O to avoid development of the abdominal compartment syndrome.[11]

The alternative ways of managing babies with abdominal wall defects generate lively discussion among colleagues. This happens whenever disparate techniques yield equivalent results. When no one technique is demonstrably superior, personal preference is determinative.

The main dilemma is balancing the safety of the reduction of the viscera and closure of the defect against the risk of complications. The safety of reduction and closure techniques is related to the level of intra-abdominal pressure and, therefore, the degree of viscera-abdominal disproportion. This, in turn, influences the risk of complications and outcome. Minimizing intra-abdominal hypertension (IAH) and avoiding abdominal compartment syndrome is important.

An intra-abdominal pressure of more than 15 mm Hg influences IAH and more than 20 mm Hg influences abdominal compartment syndrome. The effects of IAH are both hemodynamic and ventilatory. Reduced cardiac output and reduced splanchnic perfusion pressure lead to oliguria and gut mucosal acidosis; hypoventilation compounds the hemodynamic effects. In turn, this leads to abdominal compartment syndrome and potentially devastating physiological complications, including renal failure, sepsis, bowel ischemia, and wound complications.

Bowel ischemia may develop into NEC, which may lead to loss of bowel, resulting in short bowel syndrome. Wound complications include dehiscence, sepsis, and enterocutaneous fistula and can lead to negative cosmetic outcomes. Tight closure of gastroschisis may be involved in as many as 75% of patients who have short bowel syndrome who are referred for transplantation.[35]

Questions include the following:

  • Should these babies be delivered by cesarean delivery or vaginally?
  • Should a prefabricated silo be placed in the NICU with sedation or in the OR under general anesthesia? Does this matter?
  • Can the intestines be more thoroughly cleansed and the anatomy assessed in the OR rather than in the NICU?
  • Are "off the shelf" silos preferable to those fabricated by the surgeon from silon sheets?
  • Does stretching the flaccid (paralyzed) abdominal wall enlarge the abdominal cavity?
  • Is enterolysis of the inflamed intestine, cutting through the inflammatory peel and separating the congealed loops of intestine feasible, or is the risk of damaging the intestine prohibitive?
  • Should an attempt be made to evacuate meconium from the intestine?
  • Does squeezing the intestine injure the serosa and promote formation of adhesions?
  • Should the appendix be removed, and the opening in the cecum used to evacuate the meconium?
  • Should the surgeon dilate the anus and milk the meconium through the colon into the rectum and out the anal canal?
  • If a silo is placed, should pressure be applied to the eviscerated intestine, and does this pressure cause enlargement of the abdominal cavity?
  • Is resolution of inflammation the determinative factor effecting reduction? In which case, simply allowing the intestine to sink by gravity into the abdominal cavity should be sufficient.
  • When should a silo be removed to minimize the risk of infection?
  • Which antibiotics should be used?
  • Should the antibiotic cover gut or skin flora?

Success with placement of a prefabricated silo in the nursery and applying pressure to the extruded intestine may be appropriate in some situations. Criteria that preclude this technique include poor bowel perfusion, bowel/mesentery attached to the abdominal wall defect, gross viscera-abdominal disproportion, narrow defect diameter, and deteriorating metabolic acidosis.[35, 36]

The use of a preformed silo is an efficacious treatment modality; however, reduction of gastroschisis against a tight silo ring may result in venous congestion, leading to bowel ischemia and necrosis. Close inspection of the reduction process is necessary to ensure the bowel at the base of the silo is actually reducing into the abdomen with manual reduction of the silo. Otherwise, it may simply be compressing the bowel against the loops at the bottom of the silo or against the ring of the silo or the fascia, causing congestion and possible ischemia. Any sign of venous congestion mandates immediate removal of the silo and inspection of the intestines. Necrosis of the anterior wall of the duodenum from the ring of the silo abutting the duodenum has also been reported.[36] See the image below.

Gastroschisis complicated by jejunal atresia and l Gastroschisis complicated by jejunal atresia and loss of the entire distal small bowel = the grey tissue.

The baby in the image above is truly remarkable: she had jejunal atresia with necrosis of the distal small intestine; there was even necrosis along the antimesenteric aspect of the atretic jejunum, which was excised, and the remaining rectangular plaque of intestine was tubularized. The tubularized segment ultimately dilated, and, later, a serial transverse enteroplasty (STEP) procedure[37] was performed and the remaining proximal intestine was joined to the unused microcolon. The anastomosis strictured and required several revisions and additional STEP procedures. She was referred for an intestinal transplantation, but this was not necessary.

See the images below.

Following lysis of adhesions, tubularization of th Following lysis of adhesions, tubularization of the viable, mesenteric portion of the proximal jejunum, the eviscerated viscera are reduced and the gastroschisis abdominal wall defect closed.
Recent radiograph showing the intestine following Recent radiograph showing the intestine following multiple serial transverse enteroplasty (STEP) procedures.
Successful repair; the child, approximately age 30 Successful repair; the child, approximately age 30 months in the photo, whose clinical course is described above.
Appearance of the dilated bowel prior to performin Appearance of the dilated bowel prior to performing the serial transverse enteroplasty (STEP) procedure.
The intestine as it appeared after the serial tran The intestine as it appeared after the serial transverse enteroplasty (STEP) procedure.
The intestine as it appeared after the serial tran The intestine as it appeared after the serial transverse enteroplasty (STEP) procedure.

Bladder exstrophy

Surgical repair of bladder exstrophy aims to preserve kidney function. Surgical reconstruction involves the following:

  • The bladder is dissected away from its attachments to the lower abdominal wall and folded upon itself.
  • Closure of the lower abdominal wall defect necessitates creating a space for the bladder and other pelvic organs.
  • The shape of the pelvis must be altered, from flat and shallow to concave.
  • The intersymphyseal band between the splayed pubic symphysis is divided, and the anterolateral pelvic bones are rotated medially. If left untreated, these children have a waddling gait because of the "down-and-out" rotation of the anterior pelvic ring and diastasis of the pubic symphysis.
  • Urinary continence and voluntary micturition is obtained by early closure of the bladder and reconstruction of the bladder neck and urethra.
  • Vesicoureteral reflux is usually present and requires antibiotics and perhaps reimplantation.
  • Finally, epispadias is corrected, to achieve adequate genitourinary function.

See the images below.

Baby with bladder exstrophy and epispadias; note t Baby with bladder exstrophy and epispadias; note the appearance of the bladder mucosa, indicating chronic inflammation.
Another view demonstrating the epispadias shown in Another view demonstrating the epispadias shown in the previous image.

Prune-belly syndrome

Operative procedures to correct prune-belly syndrome include the following:

  • Reconstruction of the abdominal wall
  • Repair of the urinary collecting system
  • Performance of bilateral orchiopexies

Cloacal exstrophy

Repair of cloacal exstrophy includes the following:

  • The prolapsed ileum (the "elephant trunk") is reduced.
  • The flayed-open cecum is separated from the central portion of the bifid bladder, and this placode of cecum is tubularized.
  • The bladder halves are approximated and repaired as in bladder exstrophy.
  • Later, the colostomy may be mobilized and anastomosed to the rudimentary hind gut and a stoma created; or an anus reconstructed by posterior sagittal anorectoplasty. [38]

See the images below.

Baby with cloacal exstrophy. Baby with cloacal exstrophy.
Note the bifid genitalia in this baby with cloacal Note the bifid genitalia in this baby with cloacal exstrophy.
In the repair of cloacal exstrophy, the cecal plat In the repair of cloacal exstrophy, the cecal plate in the middle of the bifid bladder is excised and used to create an ostomy, and the bladder halves are approximated.
Closure of the bladder exstrophy. Closure of the bladder exstrophy.
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Diet

Babies with omphaloceles have normal intestine and do not require special formulas. The occasional intestinal atresia, perhaps associated with a patent omphalomesenteric duct, is not usually associated with short gut.

Babies with gastroschisis, on the other hand, may have sustained injury to the intestine and require elemental or protein hydrolysate formulas, lactose-free carbohydrates, and medium-chain triglycerides.

Babies with short-gut syndrome absorb medium-chain triglycerides more readily than long-chain triglycerides; however, the latter are more valuable as regards gut adaptation.

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Activity

The liver of a child with a repaired giant omphalocele is located in the epigastrium, where it is more vulnerable to trauma; therefore, contact sports should be avoided.

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Contributor Information and Disclosures
Author

James G Glasser, MD, MA, FACS Associate Professor of Surgery and Pediatrics, University of South Alabama College of Medicine; Attending Staff, USA Children's and Women's Hospital

James G Glasser, MD, MA, FACS is a member of the following medical societies: Christian Medical and Dental Associations, American Pediatric Surgical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Brian S Carter, MD, FAAP Professor of Pediatrics, University of Missouri-Kansas City School of Medicine; Attending Physician, Division of Neonatology, Children's Mercy Hospital and Clinics; Faculty, Children's Mercy Bioethics Center

Brian S Carter, MD, FAAP is a member of the following medical societies: Alpha Omega Alpha, American Academy of Hospice and Palliative Medicine, American Academy of Pediatrics, American Pediatric Society, American Society for Bioethics and Humanities, American Society of Law, Medicine & Ethics, Society for Pediatric Research, National Hospice and Palliative Care Organization

Disclosure: Nothing to disclose.

Chief Editor

Ted Rosenkrantz, MD Professor, Departments of Pediatrics and Obstetrics/Gynecology, Division of Neonatal-Perinatal Medicine, University of Connecticut School of Medicine

Ted Rosenkrantz, MD is a member of the following medical societies: American Academy of Pediatrics, American Pediatric Society, Eastern Society for Pediatric Research, American Medical Association, Connecticut State Medical Society, Society for Pediatric Research

Disclosure: Nothing to disclose.

Additional Contributors

David N Sheftel, MD, MD Assistant Professor of Pediatrics, Chicago Medical School at Rosalind Franklin University of Medicine and Science

David N Sheftel, MD, MD is a member of the following medical societies: American Academy for Cerebral Palsy and Developmental Medicine, American Academy of Pediatrics

Disclosure: Nothing to disclose.

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Baby with an intact omphalocele.
Baby with an umbilical cord hernia.
Baby with gastroschisis.
Baby with a ruptured omphalocele.
Baby with gastroschisis and associated intestinal atresia.
Baby with gastroschisis and colon atresia. Bulbous proximal end of the atretic colon is excised, and a colostomy is created at the abdominal wall defect. An anastomosis of the proximal, dilated colon to the distal microcolon (in view of its small caliber) would not function properly. The colostomy can be closed 4-6 weeks later.
Note the enlarged tongue in this baby with Beckwith-Wiedemann syndrome.
Baby with pentalogy of Cantrell.
Silo closure of a baby with gastroschisis.
Silon sheets are pulled over the omphalocele sac, elevating the rectus muscles, and, because of their attachment to the costal arch, expanding the thoracic cavity. The Silon sheets are removed and replaced by a permanent Gore-Tex patch that is covered by skin flaps.
Giant omphalocele treated with topical agents for several weeks. The omphalocele sac will absorb, leaving granulation tissue that gradually epithelializes.
The omphalocele sac was adherent to the protuberant liver. It was covered with Gore-Tex so that gradual reduction could be effected.
The Gore-Tex sheet is imbricated, gradually reducing the liver into the abdominal cavity: the rectus muscles are pulled over the liver.
Final skin closure of the giant omphalocele was delayed because the baby developed respiratory distress. Unfortunately, the patch became infected and was removed. Later, bipedicled flank flaps were used to close the giant omphalocele, but reduction was lost.
Split-thickness skin grafts were applied to the flank wounds resulting from mobilization of the bipedicle flaps.
Baby with prune-belly syndrome.
Note the laxity of the abdominal wall in this baby with prune-belly syndrome.
Baby with cloacal exstrophy.
Note the bifid genitalia in this baby with cloacal exstrophy.
In the repair of cloacal exstrophy, the cecal plate in the middle of the bifid bladder is excised and used to create an ostomy, and the bladder halves are approximated.
Closure of the bladder exstrophy.
Baby with bladder exstrophy and epispadias; note the appearance of the bladder mucosa, indicating chronic inflammation.
Another view demonstrating the epispadias shown in the previous image.
Baby with isolated epispadias.
Operative finding: patent omphalomesenteric duct, which is being excised.
Closure of a giant omphalocele with an AlloDerm patch.
Two months after implantation: epithelialization of the AlloDerm patch.
Eight months after implantation: epithelization is nearly complete, but a huge ventral hernia has developed.
Baby with a giant omphalocele, in which the liver assumes an ectopic position in the epigastrium.
Gastroschisis complicated by jejunal atresia and loss of the entire distal small bowel = the grey tissue.
Following lysis of adhesions, tubularization of the viable, mesenteric portion of the proximal jejunum, the eviscerated viscera are reduced and the gastroschisis abdominal wall defect closed.
Recent radiograph showing the intestine following multiple serial transverse enteroplasty (STEP) procedures.
Baby with gastroschisis and colon atresia, with the proximal end open. An ostomy is brought out of the silo.
Inflammatory distortion of the extruded intestine. There appears to be an associated atresia (the dilated intestine), but this resolved in concert with resolution of the inflammation.
A silo is fashioned from Silon sheets, in case reduction of the extruded intestine cannot be achieved without unduly elevating the intra-abdominal pressure.
The sac is removed and the abdominal wall defect is closed around the in utero colostomy.
Baby with a small omphalocele sac whose contents were liver and gall bladder.
The omphalocele sac contains liver and gall bladder.
Completed repair; simulating an umbilicus.
Successful repair; the child, approximately age 30 months in the photo, whose clinical course is described above.
Appearance of the dilated bowel prior to performing the serial transverse enteroplasty (STEP) procedure.
The intestine as it appeared after the serial transverse enteroplasty (STEP) procedure.
The intestine as it appeared after the serial transverse enteroplasty (STEP) procedure.
Omphalomesenteric duct remnant presenting as an "umbilical granuloma."
Table 1. Incidence rates for gastroschisis and/or omphaloceles in various regions and time periods. [1, 6, 7, 8]
Country Time Period / Incidence Time Period / Incidence
Japan 1975-1980 1996-1997
Gastroschisis 1/77,000 1/20,000
Omphalocele 1/30,000 1/27,000
     
England and Wales 1987 1991
Gastroschisis 1/10,000 2/10,000
Omphalocele 1/10,000 1/12,500
     
Galveston, Texas 1983 2002
Gastroschisis 1/4000 1/900
     
England and Wales 1995 2005
Gastroschisis 1/7500 1/2500
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