The management of giant omphaloceles remains a challenge for pediatric
surgeons. Although the mortality rate is still high (up to 20%) in case of
multiple congenital anomalies, surviving patients with omphalocele achieve
a state of health and quality of life comparable to that of general
population peers.
Results from our study confirm the hypothesis that neonates with a
congenital abdominal wall defect have a high risk for adhesive small bowel
obstruction and could benefit from adhesion prevention. Awaiting complete
epithelialisation before operation of giant omphalocele might reduce
serosal injury and limit adhesiogenic areas.
The liver was partly unprotected in all giant omphaloceles evaluated in
this thesis. In case of an incisional hernia, the liver was located
underneath the abdominal defect. A pre-operative ultrasound study is
recommended, therefore. Furthermore, the parents should receive good
documentation and information. The question remains whether contact sports
and other risk behaviour should be advised against, as there is no
indication of more blunt trauma in these patients in the literature.
The results of the questionnaire sent to the authors do not show a
consensus for a generally accepted treatment method after more than thirty
years of innovations in the management of patients with a giant
omphalocele.
The newly introduced Component Separation Technique seems to have a good
outcome. The herniation rate is low, and prosthetic materials are not
needed. However, the question remains if delayed closure with this
technique is better than immediate staged closure. There is not yet an
evidence base; we shall have to await the long-term results of the
published techniques. Based on these outcomes, a randomized multicenter
trial comparing the staged and delayed techniques is recommended. Until
then, we remain dependent on expert opinion.http://repub.eur.nl/res/pub/22906/110406_Eijck%2C%20Floortje%20Clemens%20van.pdf
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