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Surgery - management of abdominal wall defects in the neonate

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Definitions

Gastroschisis and omphalocoele are congenital defects of abdominal wall that result in a portion of the intestinal contents remaining outside the abdominal cavity. These defects occur with an incidence of 1:6000 births. An omphalocoele is a defect in the umbilicus, with herniation of intestine as well as other viscera. The viscera are covered with a peritoneal sac, unless the sac has ruptured. If the defect is <5cm in diameter a primary repair is usually done. If the defect is >10cm then a staged repair is done.

In contrast, gastroschisis occurs as a defect in the abdominal wall lateral and to the right of the umbilicus, and no peritoneal sac covers the exposed bowel.

Other intestinal abnormalities are common (malrotation is nearly universal in omphalocoele; intestinal atresias are found with both defects but occur in up to 10% of gastroschisis). Extra-intestinal anomalies are common with omphalocoele, with congenital cardiac disease occurring in up to 20% of babies. Trisomy 13 and 18 occur in up to 10% of fetuses with omphalocoele. Other conditions associated with omphalocoele include Beckwith-Wiedemann syndrome (omphalocoele, macroglossia, and hypoglycaemia) and the pentalogy of Cantrell (epigastric omphalocoele, defects of the diaphragm, heart, pericardium, and sternum).

Initial management of the neonate born with Gastroschisis/ Ruptured Omphalocoele

Preoperative Care

  1. Follow standard pre-operative care guidelines.
  2. The bowel needs to be protected from injury. 
  3. Wrap the exposed bowel in Gladwrap (ordinary supermarket Gladwrap - does not need to be sterile).
    Slide large piece of Gladwrap under the baby's buttocks and back. 
    Place exposed organs on baby's abdomen (using sterile latex-free gloves) and wrap Gladwrap around the abdomen and exposed organs.
    1. This is preferred because the bowel can be covered, without enclosing the genital area and lower limbs. This keeps urine and meconium from coming in contact with the exposed bowel. Indwelling urinary catheter is also helpful to decompress bladder. 
    2. The Gladwrap is clear and enables the bowel to be continually observed for colour changes indicating obstruction to the blood supply by kinking or other causes. 
    3. The Gladwrap is easily removed if bowel requires closer inspection. 
    4. Use only latex free gloves and equipment to reduce risk of latex allergies developing. 
  4. Support the intestines to prevent occlusion of the blood supply by kinking.
    1. If necessary support the exposed intestines with your hands. 
    2. Where possible nurse the neonate on its right side so that the eviscerated bowel is supported and does not flop over the side of the abdomen potentially occluding the blood supply by kinking where the bowel exits the defect in the abdominal wall. 
  5. Fluids management: The eviscerated bowel loses large volumes of fluid.
    1. Fluid resuscitation. 
    2. 20mls/kg boluses to manage fluid losses. 
    3. Replace nasogastric losses with 0.9% NaCl + 10 mmol KCl/ per 500ml bag. 
    4. Intravenous fluids as prescribed - usually 10% Glucose. Peripheral IVs should be sited in an upper limb. 
    5. The baby may develop rapid and severe hypovolaemia, requiring resuscitation with 0.9% NaCl and / or Dopamine infusion. 
  6. Observe peripheral perfusion, warmth and colour. 
  7. Record urine output. Renal failure can easily occur due to dehydration or if the bowel to be replaced into the abdominal cavity causes raised intra-abdominal pressure, resulting in circulation to the kidneys being compromised. 
  8. Monitor blood glucose levels
    There is potential for hypoglycaemia (babies with omphalocoele may have Beckwith-Weidemann Syndrome). 
  9. Nasogastric Tube:
    Large bore (8 Fr minimum, preferably 10Fr) nasogastric tube to decompress stomach and bowel.
    1. Helps to prevent respiratory distress secondary to a compressed diaphragm.
    2. Protects airway by preventing vomiting and subsequent aspiration.
    3. Aids in reduction of eviscerated bowel back into abdominal cavity.
    4. Aspirate nasogastric tube regularly - do not rely on free drainage alone.
    5. Record colour and quantity of drainage.
  10. Medical staff will inform the surgeons when the baby is on its way or as soon as baby is delivered. The earlier they are informed the better.

Postoperative care

  1. Follow standard steps for postoperative care
  2. Ensure baby is nursed in a supine position.
  3. Fluid balance is maintained.
    1. IVN support is usually commenced on day 1
    2. Monitor IV fluids, electrolyte levels, nutritional balance (large amounts of fluid may be lost from bowel into the pouch causing fluid retention).
  4. The orogastric tube remains on free drainage, and is also aspirated 2-4 hourly. Aspirate losses should be replaced intravenously as prescribed (0.9% NaCl with 10mmol KCl/500ml).
  5. Administer volume expander as prescribed.
  6. Accurately measure and record urine output - 6 hourly total urinalysis with every nappy change
  7. Observe for metabolic acidosis (low pH <7.3, normal pCO2, raised base deficit >-5).
  8. Observe for respiratory compromise due to increased bowel returned into peritoneal cavity causing increased pressure on the diaphragm.
  9. Observe for signs of inferior vena cava compression.
    1. mottling
    2. cyanosis
    3. oedema of lower extremities
    4. systemic hypotension
    5. If possible do not use legs for intravenous cannulation as inferior vena cava flow is reduced.
  10. Observe wound/suture line for:
    1. colour, discharge.
    2. signs of infection or breakdown.
  11. Introduce oral feeding on surgeon's orders (usually when gastric aspirates are clear and minimal amount).

Gastroschisis/Ruptured Omphalocoele, Postoperative Care of the Pouch (Staged Repair)

  1. Support the silastic pouch by cotton tape tied to the top of the bag and tied to top of radiant heat table, keeping tension firm.
  2. Ensure that 2 nurses are present when the baby is weighed or x-rayed. One nurse holds the silastic bag in place (upright and correct tension); the other nurse holds the baby.
  3. The surgeon reduces the pouch daily, lowering the bowel into the abdominal cavity and gradually reducing the size of the defect. The length of time for complete closure will depend on the defect.

Care of the non-surgical Omphalocoele

  1. Swab the omphalocoele 4 hourly with 70% ethanol alcohol or as per the surgeons instructions.
  2. Observe for Eschar (scab) formation which then peels off, leaving granulation tissue forming over the defect (it takes 2-6 months for skin to grow over).
  3. Observe for any signs of infection.
  4. Administer antibiotics only when baby has an infection, as prescribed. Prophylactic antibiotics are sometimes used
  5. Ensure comfort of the baby is maintained. Carefully support omphalocoele with folded up napkins when baby is lying on their side

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Document Control

  • Date last published: 01 April 2005
  • Document type: Clinical Guideline
  • Services responsible: Neonatology, Paediatric Surgery
  • Owner: Newborn Services Clinical Practice Committee
  • Editor: Sarah Bellhouse
  • Review frequency: 2 years