Omphalocele

Subject: Midwifery III (Theory)

Overview

An omphalocele is a rare abdominal wall defect in which the intestines, liver, and other organs remain outside of the abdomen in a sac due to a defect in the development of the abdominal wall muscles (exomphalos). Some omphalocele cases are thought to be caused by an underlying genetic disorder, such as Edward's syndrome or Patau syndrome. Omphaloceles are also linked to Beckwith-Wiedemann syndrome. It is normal for the intestines to protrude from the abdomen into the umbilical cord until about the tenth week of pregnancy when they return to the fetal abdomen. The omphalocele can be mild, with only a small loop of intestines visible outside the abdomen, or severe, with the majority of the abdominal organs visible. Surgical treatment is used in severe cases.

Omphalocele is a rare abdominal wall defect in which the intestines, liver, and occasionally other organs remain outside the abdomen in a sac due to a defect in the development of the abdominal wall muscles (exomphalos).

Causes:

During development, the bowels malrot while returning to the abdomen.
Some omphalocele cases are thought to be caused by an underlying genetic disorder, such as Edward's syndrome or Patau Syndrome.
Omphaloceles are also associated with Beckwith-Wiedemann syndrome.

Signs and symptoms:

The sac, which is formed by peritoneum outpouching, protrudes in the midline, through the umbilicus (navel).
The intestines are normal to protrude from the abdomen into the umbilical cord until about the tenth week of pregnancy when they return inside the fetal abdomen.
The omphalocele can be mild, with only a small loop of intestines visible outside the abdomen, or severe, with most of the abdominal organs visible. In severe cases, surgical treatment is made more difficult because the infant's abdomen is abnormally small due to the absence of the need for expansion to accommodate the developing organs.
A larger omphalocele is linked to a higher risk of cardiac arrest.

Diagnosis:

Prenatal ultrasounds are frequently used to identify infants with an omphalocele before they are born. Otherwise, your health care provider can diagnose this condition based on a physical examination of the infant. Typically, testing is not required.

Treatment:

Omphaloceles are surgically repaired, though not always immediately. A sac protects the abdominal contents while allowing time for other, more serious issues (such as heart defects) to be addressed first, if necessary.
To repair an omphalocele, the sac is wrapped in special man-made material and stitched in place. The abdominal contents are gradually pushed into the abdomen over time.
The man-made material is removed and the abdomen is closed once the omphalocele can fit comfortably within the abdominal cavity.
The omphalocele can sometimes be so large that it cannot be reinserted into the infant's abdomen. The skin surrounding the omphalocele grows and eventually covers it. When the child is older, the abdominal muscles and skin can be repaired.

Things to remember

An omphalocele is a rare abdominal wall defect in which the intestines, liver, and occasionally other organs remain outside of the abdomen in a sac due to a defect in the development of the abdominal wall muscles.
Some cases of omphalocele are thought to be caused by an underlying genetic disorder, such as Edward's syndrome or Patau Syndrome.
Beckwith-Wiedemann syndrome is also linked to omphaloceles.
It is normal for the intestines to protrude from the abdomen into the umbilical cord until about the tenth week of pregnancy when they return inside the fetal abdomen.
The omphalocele can be mild, with only a small loop of intestines visible outside the abdomen, or severe, containing the majority of the abdominal organs.
In severe cases, surgical treatment is made more difficult because the infant's abdomen is abnormally small due to the absence of the need for expansion to accommodate the developing organs.
Omphaloceles are surgically repaired, though not always immediately.
A sac protects the abdominal contents while allowing time for other, more serious issues (such as heart defects) to be addressed first, if necessary.

Questions and Answers

What are the sign and symptoms of omphalocele?

The sac, which is created when the peritoneum bulges, protrudes through the umbilicus in the middle (navel).
Up until the tenth week of pregnancy, when they return to being inside the fetal belly, it is typical for the intestines to protrude from the abdomen and into the umbilical cord.
Omphaloceles can range from minor, where just a small loop of intestine is visible outside the belly, to severe, when the majority of the abdominal organs are present. Surgery is more challenging in severe situations since the infant's abdomen is unusually small because it didn't need to grow to fit the developing organs.
The risk of cardiac arrest is increased with larger omphalocele.

What are the treatment of Omphaloceles?

Although not necessarily right away, surgery can fix omphaloceles. If necessary, a sac shields the abdominal contents and gives time to address other, more severe issues (such heart anomalies).
An omphalocele is treated by covering the sac with a unique synthetic material and stitching it in place. The abdominal contents are gradually pushed into the abdomen over time.
The artificial material is taken out, and the abdomen is closed, once the omphalocele can fit comfortably within the abdominal cavity.
The omphalocele can occasionally be too big to fit back into the baby's abdomen. The omphalocele is finally covered by the skin that has grown around it. When the child is older, the abdominal muscles and skin can be restored for a better aesthetic result.