The following is courtesy of the Children's
Hospital of Philadelphia:
Diaphragmatic
Hernia
What is a diaphragmatic hernia?
A diaphragmatic hernia is a birth
defect,
which is an abnormality that occurs before birth as a fetus is forming
in its mother's uterus. An opening is present in the diaphragm (the
muscle
that separates the chest cavity from the abdominal cavity). With this
type
of birth defect, some of the organs that are normally found in the
abdomen
move up into the chest cavity through this abnormal opening.
There are two types of diaphragmatic
hernia:
Bochdalek hernia
Morgagni hernia
A Bochdalek hernia involves an opening
on
the left side of the diaphragm. The stomach and intestines usually move
up into the chest cavity. A Morgagni hernia involves an opening on the
right side of the diaphragm. The liver and intestines usually move up
into
the chest cavity.
What causes a diaphragmatic
hernia?
As a fetus is growing in its
mother's
uterus before birth, different organ systems are developing and
maturing.
The diaphragm forms between the seventh and 10th week of pregnancy. The
esophagus (the tube that leads from the throat to the stomach), the
stomach,
and the intestines are also developing at this time. In a Bochdalek
hernia,
the diaphragm may not develop properly, or the intestine may become
trapped
in the chest cavity as the diaphragm is forming. In a Morgagni hernia,
the tendon that should develop in the middle of the diaphragm does not
develop properly. In both cases, normal development of the diaphragm
and
the digestive tract does not occur. Diaphragmatic hernia is a
multifactorial
condition, which means that many factors, both genetic and
environmental,
are involved. It is thought that multiple genes from both parents, as
well
as a number of environmental factors that scientists do not yet fully
understand,
contribute to diaphragmatic hernia. In cases where it is the only
health
problem in a baby, the chance for diaphragmatic hernia to happen again
in a future pregnancy is 2 percent or 2 in 100 chances. This means that
there is a 98 percent chance that it would not be seen in a future
pregnancy.
How often does a diaphragmatic
hernia occur?
Bochdalek hernia:
Makes up about
ninety percent
of all
cases
Occurs in one out of
every
2,200 to
5,000 live births
Morgagni hernia makes up two
percent
of all cases.
Who is at risk for
developing
a diaphragmatic hernia?
Parents who have had one child
with
an isolated diaphragmatic hernia are at increased risk to have another
child with the same problem. The chance is 2 percent or two in 100.
Morgagni
hernia is more common in girls than boys, whereas Bochdalek hernia is
slightly
more common in boys than girls. Babies with the Bochdalek type of
diaphragmatic
hernia are more likely to have another birth defect.
Almost 20
percent have a
congenital
heart defect.
Between 5 to 16 percent
have
a chromosomal
abnormality.
Why is a diaphragmatic hernia
of concern?
The lungs are developing at the
same
time as the diaphragm and the digestive system. A diaphragmatic hernia
allows abdominal organs to move into the chest cavity, instead of
remaining
in the abdomen as they are developing. With the heart, lungs and
abdominal
organs all taking up space in the chest cavity, the lungs do not have
space
to develop properly. This underdevelopment of the lungs is called
pulmonary
hypoplasia. A diaphragmatic hernia is a life-threatening illness. When
the lungs do not develop properly during pregnancy, it can be difficult
for the baby to breathe after birth. Healthy lungs have millions of
small
air sacs (alveoli), which resemble a balloon filled with air. With
pulmonary
hypoplasia, the following conditions occur:
There are fewer air
sacs than normal.
The air sacs that are present
are
only able
to partially fill with air.
The air sacs deflate easily due
to a
lack
of a lubricating fluid called surfactant.
When these conditions are present,
the
baby is unable to take in enough oxygen to stay healthy. The intestines
also may not develop properly, especially if they are not receiving
enough
blood supply while they are developing. A good blood supply is
necessary
for the intestines to develop correctly, and to be healthy and function
properly.
What are the symptoms of a
diaphragmatic
hernia?
The symptoms of a Bochdalek
diaphragmatic
hernia are often observable soon after the baby is born. The following
are the most common symptoms of a Bochdalek diaphragmatic hernia.
However,
each child may experience symptoms differently.
Difficulty breathing
Fast breathing
Fast heart rate
Blue color of the skin
Abnormal chest development, with
one
side
being larger than the other
Abdomen that appears caved in
(concave)
A baby born with a Morgagni hernia
may
or may not show any symptoms. Symptoms of diaphragmatic hernia may
resemble
other conditions or medical problems. Please consult your child's
physician
for a diagnosis.
How is a diaphragmatic
hernia
diagnosed?
The problem can often be seen
on
a prenatal fetal ultrasound, before the baby is born. In some cases,
fetal
surgery may be performed. After birth, your child's physician will
perform
a physical examination. A chest X-ray is done to look at the
abnormalities
of the lungs, diaphragm and intestine. A blood test known as an
arterial
blood gas is often performed to evaluate the infant's breathing
ability.
Other tests may also be performed:
Blood test for
chromosomes
(to determine
if there is a genetic problem)
Ultrasound of the heart
(echocardiogram)
Ultrasound of the brain
(cranial ultrasound)
Treatment for a diaphragmatic
hernia
Your child's physician will
determine
a specific treatment based on the following factors:
When the
problem is diagnosed
(during
pregnancy or after birth)
Your child's overall
health
and medical
history
The severity of the
problem
Your child's tolerance
for
specific
medications, procedures or therapies
Your opinion or preference
Treatment may include:
Neonatal
intensive care - A
diaphragmatic
hernia is a life-threatening illness and requires care in a neonatal
intensive
care setting. Babies with diaphragmatic hernia are often unable to
breathe
effectively on their own because their lungs are underdeveloped. Most
babies
will need to be placed on a breathing machine called a ventilator to
help
their breathing.
ECMO - Some infants may
need
to be
placed on a temporary heart/lung bypass machine called ECMO if they
have
severe problems. ECMO does the job that the heart and lungs would be
doing:
putting oxygen in the bloodstream and pumping blood to the body. ECMO
may
be used temporarily while a baby's condition stabilizes and improves.
Medication-surfactant - A
medication
called surfactant is usually given to improve the health of the lungs.
Surfactant is a fluid that our body normally produces. It helps keep
the
air sacs in the lungs from deflating. Babies with a diaphragmatic
hernia
often do not have enough surfactant. This medication can help the air
sacs
to stay open and healthy.
Surgery - When the baby's
condition
has improved, the diaphragmatic hernia will be fixed with an operation.
This is usually done between the first and 10th days of life. An
incision
is made in the baby's left side, between the ribs. The stomach,
intestine
and other abdominal organs are moved from the chest cavity back to the
abdominal cavity. The hole in the diaphragm is repaired.
Can there be problems in the
future?
Children born with
diaphragmatic
hernia can have long-term problems and often need regular follow-up
after
going home from the hospital. Many babies will have chronic lung
disease
and may require oxygen or medications to help their breathing for
weeks,
months or years. Many babies will have gastroesophageal reflux (GERD).
Acid and fluids from the stomach move up into the esophagus (the tube
that
leads from the throat to the stomach), and can cause heartburn,
vomiting,
feeding problems or lung problems. GERD can often be controlled with
medications
prescribed by your child's physician. Some babies will have difficulty
growing. This is known as failure to thrive. The children with the most
serious lung problems are most likely to have growing problems. Because
of their illness, they often require more calories than a normal baby
in
order to grow and get healthier. GERD can also cause feeding problems,
preventing a baby from eating enough to grow. Some babies can have
developmental
problems. They may not roll over, sit, crawl, stand or walk at the same
time healthy babies do. Physical therapy, speech therapy and
occupational
therapy are often helpful for these children to gain muscle strength
and
coordination. Finally, some babies will have some degree of hearing
loss.
A hearing test should be performed prior to discharge from the
hospital.
Consult your child's physician regarding the prognosis for your child.
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