Immediate management
Maintain temperature.
Protect the sac from:
- Trauma
- Rupture
- Evaporation
- Infection
Use sterile nonadherent dressings and an appropriate protective covering.
Keep nil orally initially.
Insert an orogastric tube for decompression when significant intestinal distension is present.
Establish IV fluids according to neonatal requirements.
Fluid losses are generally lower than in gastroschisis because an intact sac limits evaporation.
Do not clamp or divide a broad umbilical base blindly because bowel or other viscera may lie within the cord.
Primary closure
A small omphalocele with adequate abdominal domain can undergo early primary closure.
Reduce the viscera gently.
Close the fascia only when the infant tolerates reduction without significant:
- Ventilatory deterioration
- Reduction in venous return
- Renal hypoperfusion
- Lower limb venous congestion
Do not force the liver into a small abdominal cavity simply to achieve neonatal primary closure.
Giant omphalocele
Management must be individualised according to:
- Pulmonary function
- Liver herniation
- Abdominal domain
- Associated cardiac anomalies
- Skin availability
Options include:
Staged operative reduction
A silo, mesh or staged closure gradually returns abdominal contents.
Delayed nonoperative epithelialisation
The sac is treated to encourage eschar formation and epithelialisation, converting the defect into a large ventral hernia that can be repaired months or years later after the abdominal cavity has enlarged.
No single closure strategy is clearly superior for every giant omphalocele, and recent reviews continue to demonstrate substantial variation in practice.
Monitoring during reduction
Watch for intra abdominal hypertension.
Concerning findings include:
- Rising ventilator pressures
- Hypercapnia
- Hypotension
- Reduced urine output
- Lower limb oedema or venous congestion
- Increasing lactate
If these occur, stop or reverse the reduction.
Feeding
Enteral feeding can begin once:
- Gut function is adequate
- No associated intestinal obstruction exists
- Respiratory status permits safe feeding
Giant defects and associated anomalies can significantly delay full nutrition.
Long term problems
Important issues include:
- Ventral hernia
- Adhesive obstruction
- Gastroesophageal reflux
- Feeding difficulties
- Pulmonary disease
- Cosmetic abdominal wall deformity
- Complications from associated syndromes
