Pediatric Omphalocele and Gastroschisis Follow-up

  • Author: James G Glasser, MD; Chief Editor: Ted Rosenkrantz, MD   more...
 
Updated: Dec 1, 2011
 

Further Inpatient Care

Omphalocele

An intact omphalocele sac protects the intestine from contact with the amnionic fluid. Even if an atresia is present, babies can be fed early following closure of the abdominal wall defect because no inflammation of the intestine occurs.

Babies with giant omphaloceles have prolonged hospitalizations because of delay in wound closure and respiratory failure, which may require tracheostomy and mechanical ventilation.

Gastroschisis

Primary closure of the abdominal wall defect is possible only if inflammation of the intestine is minimal; even so, it is usually several weeks before the baby tolerates enteral feedings.

If closure of the abdominal wall defect necessitates use of a silo to contain the eviscerated intestine, the silo should be removed within a week because of the risk of wound infection (see the image below).

Silo closure of a baby with gastroschisis. Silo closure of a baby with gastroschisis.

Parenteral nutrition is used until the baby passes "starvation stools." If this has not occurred within 3-4 weeks, a mechanical obstruction, rather than an ileus, should be suspected; Upper GI/small bowel radiography should be performed to assess the transit of contrast through the intestinal tract. If this study demonstrates an intestinal obstruction, laparotomy is indicated.

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Further Outpatient Care

Following discharge from the hospital, these infants require close follow-up to assess their growth, weight gain, and chronolog their developmental milestones.

They frequently have symptoms of gastroesophageal reflux, which are notoriously variable and occasionally life-threatening. Practitioners must maintain a high index of suspicion for this diagnosis. Treatment follows the same protocol as for any other infant with this problem.

Hirschsprung disease may also occur. Physicians should consider this diagnosis in a child with refractory constipation and failure to thrive.

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Transfer

Placing a warm, moist laparotomy pad over the exposed intestine intuitively makes sense. The problem is that a warm, moist lap pad rapidly becomes a cold, wet lap pad; consequently, the author prefers covering the intestine with a dry lap pad and wrapping the baby's torso in such a way as to avoid pulling or kinking the bowel mesentery.

If intravenous (IV) access can be obtained, IV fluids and antibiotics should be administered, and the stomach should be decompressed with a nasogastric tube.

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Complications

Nutritional depletion can occur in a baby with an omphalocele treated conservatively, because of the large open wound. Positive nitrogen balance occurs following skin closure.

Prolonged parenteral nutrition can cause hepatotoxicity, manifested by cholestasis and hepatomegaly, which may complicate staged closure of a giant omphalocele.

Pulmonary insufficiency/hypoplasia often requires tracheostomy and prolonged ventilatory support in infants with giant omphaloceles. Staged abdominal closure sequentially increases intra-abdominal pressure, which elevates the diaphragm making ventilation more difficult.

Infants with giant omphaloceles have an increased risk of sepsis because of their poor nutritional status, chronic ventilatory requirements, and presence of a central venous catheter.

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Prognosis

Omphalocele

Infants with omphaloceles are complex, with multisystem organ involvement. Their prognosis depends on the severity of the associated problems.

Giant omphaloceles can be closed; however, multiple surgical procedures are usually necessary. In addition, these infants may have additional medical problems that make caring for them quite challenging.

The critical factor affecting the survival of a baby with a giant omphalocele is the size of the thoracic cavities, and whether associated pulmonary hypoplasia and chronic respiratory failure is present. Even when the baby's thorax is diminutive in size, the reality of pulmonary growth and development encourages optimism regarding the ultimate prognosis of children with this anomaly.

Gastroschisis

The patient's prognosis depends on the severity of associated problems, such as prematurity, intestinal atresia, short gut, and intestinal inflammatory dysfunction. A population-based cohort study analyzed the 1-year outcomes of infants with gastroschisis from all 28 pediatric surgical centers in the United Kingdom and Ireland. The research found that those with complex gastroschisis realized longer hospital stays and greater complications than did those with simple gastroschisis. Classifying infants with gastroschisis into 1 of these 2 groups may be a reliable predictor of outcome.[38]

Many pediatric surgeons believe that the prognosis has improved because of maternal sonographic diagnosis and monitoring, which allows expeditious delivery of these babies at tertiary centers.

Obtaining primary abdominal wall closure in a baby with gastroschisis rarely occurred in the past; it was usually necessary to use a silo. Primary closure is commonplace today, and people attribute this progress to improvements in prenatal and obstetrical care.[11, 8, 9]

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Patient Education

Parents should be instructed regarding the importance of bilious (green) vomitus because this may indicate that adhesive small-bowel obstruction or midgut volvulus has occurred.

They should be informed that their child's appendix is located in an unusual location, and that CT scanning is the most reliable way to diagnose acute appendicitis.

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Contributor Information and Disclosures
Author

James G Glasser, MD  Clinical Associate Professor, Department of Surgery and Pediatrics, Department of Pediatric Surgery, University of South Carolina Medical School; Consulting Staff, Palmetto Health Alliance Children's Hospital

James G Glasser, MD is a member of the following medical societies: American Pediatric Surgical Association, Christian Medical & Dental Society, and South Carolina Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

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

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

Disclosure: Nothing to disclose.

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 (Neonatology), Vanderbilt University School of Medicine; Director, Neonatal Follow-up Program, Monroe Carell Jr Children's Hospital at Vanderbilt

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 Society for Bioethics and Humanities, American Society of Law, Medicine & Ethics, National Hospice and Palliative Care Organization, Society for Pediatric Research, and Southern Society for Pediatric Research

Disclosure: Nothing to disclose.

Carol L Wagner, MD  Professor of Pediatrics, Medical University of South Carolina

Carol L Wagner, MD is a member of the following medical societies: American Academy of Pediatrics, American Chemical Society, American Medical Women's Association, American Public Health Association, American Society for Bone and Mineral Research, American Society for Clinical Nutrition, Massachusetts Medical Society, National Perinatal Association, and Society for Pediatric Research

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 Medical Association, American Pediatric Society, Connecticut State Medical Society, Eastern Society for Pediatric Research, and Society for Pediatric Research

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. (Gastrostomy tubes are no longer routinely used.)
Note the enlarged tongue in this baby with Beckwith-Wiedemann syndrome.
Baby with pentalogy of Cantrell.
Silo closure of a baby with gastroschisis.
Completed reduction of the bowel contained within the silo; the silo is about to be removed and the abdominal wall closed.
Case A. Baby with a giant omphalocele.
Case A. Closure of the giant omphalocele using a synthetic patch.
Case A. Tightening the abdominal wall closure
Case A. Flank flaps were used to close the giant omphalocele in the baby whose patch became infected.
Case A. The flank wounds were skin grafted and closure of the giant omphalocele obtained.
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 ileum 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.
An operative photo from the repair of a draining umbilicus.
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.
Persistent drainage from the umbilicus.
Baby with an omphalocele.
Complicated gastroschisis.
Following reduction of eviscerated viscera (and lysis of adhesions, tubularization of the viable, mesenteric portion of the proximal jejunum).
Recent radiograph showing the intestine following multiple "STEP" procedures.
 
 
 
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