What is
Congenital Diaphragmatic Hernia??
CDH occurs when the diaphragm fails to close or form properly around the 12th week of pregnancy. When the diaphragm fails to form properly the abdominal contents migrate or even grow up into the chest cavity. When this happens the lungs and heart are moved over and squashed. The lung are unable to develop properly causing pulmonary hypoplasia and the heart is strained. This can lead to heart problems.
What is a diaphragm?
The diaphragm is a very important muscle. The diaphragm is a skeletal muscle that extends the bottom of the rib cage and seperates the chest cavity from the abdomen. As the diaphragm contracts the space or volume in the chest cavity increases and air is drawn into the lungs. When the diaphragm relaxes the air in exhaled. The diaphragm plays other roles in the body other than breathing: helps to expel vomit, feces and urine from the body by increasing intra-abdominal pressure and preventing acid-reflux by exerting pressure on the esophagus as it passes through the esophageal hiatus.
What is the leading cause of death for CDHers?
CDH causes many medical complications in those affected but the leading cause of deaths in those born with CDH is Pulmonary Hypertension.
PH is the increase in blood pressure in the pulmonary artery, pulmonary vein or pulmonary capillaries. This is also known as the lung vasculature. This leads to dizziness, shortness of breath, fainting, leg sweating and more. PH is a severe problem that can lead to heart failure. In pregnancy the baby does not use it's lungs, instead oxygen is transferred from mother to child until birth. After birth the babies lungs should take over and the blood flow should switch so that it passes through the lungs instead of the ambilical cord for oxygen. In babies born with PH that switch doesn't happen. All babies born with CDH has PH to some degree.
What causes CDH?
Research is a really important part of finding out what causes CDH as well finding a way to prevent it.
To date research shows that CDH isn't caused by any one thing.
There are genetic factors in some cases. Once you have a baby born with CDH, your chances of having another baby with CDH are exponetially increased. There are a few known families that have given birth to multiple CDHer's and a few that only have CDHer's. Geneticist are still looking for the connection between your genes and the failure of the diaphragm.
In some cases CDH accompanies another birth defect and in others CDH presents alone.
There are several different types of CDH:
Left CDH is when the defect is on the left side of the diaphragm. Left is also the most common.
Right CDH is when the defect is on the right side. This is less common and more dangerous.
Bi-lateral CDH is when both sides of the diaphragm are affected or even completely missing.
What are the statistics?
*1 in every 2500 live births are affected by CDH
*That means every 10 minutes a baby is born with CDH
*50% of babies born with CDH do NOT survive
*Most survivors have life long medical problems but some go on to have nothing worse than mild asthma.
How is CDH treated?
The recommended treatment for CDH can vary on which hospital your baby is being treated at. What specialist can agree on is that babies with CDH should be intubated within minutes after birth so that the baby do not have a chance to breath on its own. Allowing the baby to try and breath on its own causes the lungs to expand when there is no room for them too and this causes more stress on both the lungs and the heart.
Some babies need to be put on ECMO.
ECMO (Extracorporeal membrane oxygenation) provides respitory and cardiac support oxygen to patients whose lungs are heart are to damaged or diseased to function on their own. ECMO canulas are surgically inserted into the patients main artery in the neck. ECMO patients require 24/7 observation by 2 ECMO trained and certified nurses at all times. ECMO is basically like a bypass machine that cleans out the toxic gas in the babies body. This is used in babies born with CDH because it also gives the lungs and heart time to rest and heal and become stable for repair surgery.
When the baby is stable, a surgery is required to bring the abdominal contents back into the proper cavity and to repair the diaphragm defect. Depending on the severity of the defect is how the diaphragm will be closed.
*diaphragm completely missing
*diaphragm flap is there but the tissue is too weak to use
*the remaining tissue can be used
If the diaphragm is completely missing or the remaining tissue is too weak to use than the surgeons use a patch. When the surgeons explained the patch to be they said you could think of it as a stretchy rain coat material. For those with a patch the chances of re-herniation are greater. Patch patients outgrow their patch so new ones need to be put in as the child grows. There is always a chance weather you have a patch or not that your will re-herniate.
