Importance 

Congenital omphalocele is among the most common antenatally diagnosed anomalies. As additional abnormalities are found in majority of cases, antenatal investigations target the search for additional structural anomalies and genetic disorders, including aneuploidy. Antenatal management focuses on the assessment of fetal well-being. Unfortunately, antenatal prediction of postnatal and long-term outcomes represents 2 less well-documented but crucial facets of this pathology. A large part of the prognosis relies on aspects that are difficult to predict such as quality of life, neurological development, and autonomy, which cause significant anxiety in expectant parents.

Objective 

This article offers a comprehensive review of antenatal management of omphalocele with a specific focus on predictive factors and long-term outcomes.

Evidence Acquisition, Results 

We conducted an extensive literature review targeting management of fetal omphalocele. We had a specific interest in factors predictive of fetal and neonatal outcome as well as long-term consequences of omphalocele. Fetuses with large defects and those containing the liver are at higher risk of having a complicated postnatal course. Neonates may experience pulmonary hypoplasia, pulmonary hypertension, and gastroesophageal reflux. In selected cases, motor and cognitive delay may be present, but the overall life-long prognosis and quality of life is good.

Conclusions and Relevance 

A multidisciplinary approach should be encouraged after the diagnosis of fetal omphalocele. In addition to clinical team experience, antenatal counseling should be based on objective and gestational age-dependent criteria and should include long-term outcomes.

Target Audience 

Obstetricians and gynecologists, family physicians.

Learning Objectives 

After completing this activity, the learner should be better able to propose management of antenatal investigations and fetal surveillance; identify antenatal factors predictive of fetal and neonatal outcomes; and explain the different aspects and currents limitations in knowledge regarding long-term outcomes of omphalocele.