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Omphalocele

Fetal pregnancy complications


Video

Types of Abdominal Wall Defects : Explaining Gastroschisis and Omphalocele

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Description

Omphalocele is a birth defect characterized by the abdominal wall not closing correctly, causing what looks like a wide hernia at the base of the navel. This opening allows the externalization of certain organs such as the liver and intestines, which are surrounded by an amniotic and peritoneal membrane.

Omphalocele affects about 1 in 10,000 births.

In about 70% of cases, other malformations are associated with omphalocele. Among them, we find:

  • Cardiac abnormality (in 50% of cases)
  • Gastrointestinal atresia
  • Renal abnormalities
  • Brain abnormalities
  • Omphalocele is also associated, in 40% of cases, with chromosome abnormalities or genetic syndromes that require genetic investigation.

Causes

Omphalocele is the result of a defect in the abdominal wall that causes an opening near the belly button. The causes of omphalocele are unknown, although some risk factors such as advanced maternal age (over 35 years) and pregnancy by in vitro fertilization have been identified.


Symptoms

Depending on the clinical course of the condition, regular ultrasound monitoring is recommended to monitor fetal growth and the amniotic fluid level.

Regardless of the size of the omphalocele, there is a significant risk of complications or death in utero or in early infancy, which is why care needs to be in the hands of a tertiary hospital like CHU Sainte-Justine.


Tests and procedures

It is possible to see omphalocele on ultrasound in the first trimester. A reference to a tertiary centre is recommended in order to confirm the diagnosis.

Other diagnostic tests are also recommended such as a fetal heart ultrasound and amniocentesis.


Treatment and follow-up care

In order to ensure an optimal outcome, the monitoring of the pregnancy and delivery must be done in a tertiary hospital centre.

The care of the mother and the unborn baby is managed by a multidisciplinary team that includes an obstetrician specialized in fetal-maternal medicine, a geneticist, a pediatric surgeon, a neonatologist pediatrician, a radiologist, a radiology technician, and a clinical nurse. Several other professionals such as a psychologist or a lactation consultant can also be part of the team depending on individual needs.

A non-stress test to check the reactivity of the fetus is recommended twice a week from the 32nd week of pregnancy. A biophysical profile done by ultrasound might also be suggested according to the results of the non-stress test.

The mode of delivery is planned according to the clinical conditions of each case. A vaginal delivery is possible, but under certain obstetric conditions a caesarean section is recommended. Birth is usually expected around the 37th week of pregnancy. When the baby is born, immediate care is provided by the neonatology and pediatric surgery team.

There are two ways to replace the omphalocele to the newborn’s abdomen:

  • Surgical method – If the omphalocele is less than 5 cm in diameter, the organs are placed in the peritoneal cavity and the abdominal wall is closed. If the omphalocele is more than 5 cm in diameter, a staged approach is planned that involves gentle compression to gradually reduce the amount of herniated viscera before surgery.
     
  • Natural method – Small omphaloceles are coated with an antimicrobial agent until the opening on the abdomen heals over (epithelialization).

The choice of the most appropriate method is based on the size of the omphalocele.

Duration of hospitalization is dependent on short- and medium-term complications, including infections, respiratory failure, and abdominal compartment syndrome.


Resources and useful links

Robert K. Creasy, Robert Resnik et coll. Creasy & Resnik’s Maternal-Fetal Medecine : Principle and Practice. 7e ├ędition, Elsevier Canada, 2014.

Fiche par

Chantal Larcher, inf. clinicienne et Dre Lucie Morin

About this page
Updated on 12/19/2017
Created on 12/19/2017
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