What is an omphalocele?
An omphalocele is a
congenital (present at birth) abnormality in which abdominal organs
protrude through an opening in muscles in the area of the umbilical
cord. These organs are covered by a transparent membrane called the
peritoneum. The omphalocele may be small, with only a portion of
the intestine protruding outside the abdominal cavity, or large,
with most of the abdominal organs
(including intestine, liver, and spleen) outside the abdominal
cavity. More than two-thirds of babies with omphalocele have
abnormalities of other organs or body parts, most commonly the
spine, digestive system, heart, urinary system and
limbs.
A small omphalocele occurs in one out of every 5,000 live births
while a large omphalocele occurs
in one out of every 10,000 live births. This abnormality affects
boys and girls equally.
Babies born with an omphalocele
frequently have other complications including:
- Poor lung
development
- Intestines that are slow
to handle food
- Heart malformations (20
percent)
- Beckwith-Wiedeman
Syndrome (a condition typified by a large tongue, high insulin and
low blood sugar)
- Chromosomal
abnormalities
What causes an
omphalocele?
It is not
known what causes an omphalocele or whether the mother can do
anything during pregnancy to prevent it. Between the 6th and the
10th weeks of pregnancy, the intestines actually project into the
umbilical cord as they are growing. By the 11th week of
development, the intestines should return to the abdomen. When this
fails to happen, an omphalocele occurs.
How is an omphalocele
diagnosed?
An omphalocele
is often be detected during the second and third trimesters of
pregnancy using an ultrasound. Once discovered, a fetal
echocardiogram (ultrasound of the heart) is frequently ordered to
check for heart abnormalities before the baby is born.
Once born, the doctor will note the
omphalocele and order x-rays to evaluate abnormalities of other
organs or body parts.
How is an omphalocele
treated?
Treatment for an
omphalocele will depend on many things including the extent of the
condition, the baby's age and overall health, the
baby's tolerance for medications and parental preferences for
treatment.
In the case of a small omphalocele, an
operation will be done immediately to return the organs to the
abdomen and to close up the opening in the abdominal wall to
prevent infection or any tissue damage.
For a large omphalocele involving
numerous organs, the surgery is often done in stages, moving the
organs back into the baby's body over a period of several
days. The need for the surgery to be done in stages is due to the
baby's abdomen being too small and underdeveloped to hold all
of the organs at once. During this time, the exposed organs are
protected by a sterile, protective sheeting to prevent the risk of
infection. Babies with omphalocele who have underdeveloped
abdominal cavities often have breathing difficulties and may
require the assistance of a ventilator until they can breathe on
their own.
What is the prognosis for an omphalocele? If
an omphalocele is the only obvious health problem that an infant
has, full recovery from it is frequently expected. However,
since an omphalocele is often associated with other birth defects,
the prognosis depends on these conditions. Consult your baby's
physician regarding the prognosis for your baby.