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.

 

Click the image below to view an informative video on CDH
 









Text, graphics and video above courtesy of the Children's Hospital of Philadelphia








How well do you know the facts about Congenital Diaphragmatic Hernia?


1.
What does CDH stand for?
* Cat Dog Horse
* Congenital Diaphragmatic Hernia
* Congenital Diaphragmatic Hernia

2.
What is CDH?
* A severe and often deadly birth defect that occurs when the diaphragm fails to fully form and allows organs into the chest cavity.
* A severe and often deadly birth defect that occurs when the diaphragm fails to fully form and allows organs into the chest cavity.
* A medical disease

3.
What is the survival rate for CDH?
* 50% - 60%
* 25%
* 70%

4.
Which is more common, Left-sided CDH or Right-sided CDH?
* Left-Sided
* Neither
* Right-Sided

5.
What organs are / can be herniated in CDH?
* Diaphragm, Lungs, Stomach, Liver, Intestines, Spleen, and more
* Any organ in the body
* Diaphragm, Lungs, Stomach
6.
What are the odds of a baby having CDH?
* 1 in 2000
* 1 in 5000
* 1 in 2500

7.
What complication is most lethal to CDH babies?
* Small Lungs
* Infection & Pneumonia
* Poor Lung Function

8.
Approximately how many babies are born with CDH every year in the U.S.?
* 500
* 1600
* 1000

9.
Approximately how often is a CDH baby born in the world?
* Every day
* Every hour
* Every 10 minutes

10.
Which is NOT a form of CDH?
* Morgani
* Reynaldi
* Bochdalek

11.
Which gender has a slightly higher survival rate of CDH?
* Female
* Male
* Neither

12.
When was CDH first described in medical books?
* 1575
* 1887
* 1903

13.
In what year was the first successful CDH repair on an infant?
* 1946
* 1893
* 1973

14.
In what year was ECMO (Extracorporeal Membrane Oxygenation) first used on a CDH patient?
* 1988
* 1976
* 1991

15.
In what year was the world's first CDH Research Group (The CDH Stud Group) and the world's first CDH Support Group (CHERUBS) both founded?
* 1995
* 1999
* 2002

16.
What causes Congenital Diaphragmatic Hernia in babies?
* No one yet knows.
* Pesticide exposure during pregnancy.
* Drug exposure during pregnancy.

17.
Which of these complications are most common with many CDH survivors?
* Pulmonary Hypertension, Allergies, Feeding Issues
* Pneumonia, Allergies, Asthma, Developmental Delay
* Pneumonia, Pulmonary Hypertension, Allergies, Asthma, Feeding Issues, Developmental Delay

18.
Can you tell what a CDH survivor looks like if you saw one in public?
* They have a funny look to them
* They look like everyone else.
* Only if they had their shirt off and you saw a scar.

19.
Which one of these procedures is more common than the other 2 in 2008 for these children?
* Trachel Occlusion
* Tracheal Ligation
* In Utero Repair

20.
What does ACDHO stand for?
* Alliance of Congenital Diaphragmatic Hernia Organizations
* American Congenital Diaphragmatic Hernia Organization
* American Congenital Diaphragmatic Hernia Office


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Answers Below (don't cheat!):









1. What does CDH stand for?
Congenital Diaphragmatic Hernia

2. What is CDH?
A severe and often deadly birth defect that occurs when the diaphragm fails to fully form and allows organs into the chest cavity.

3. What is the survival rate for CDH?
50 - 60%

4. Which is more common, Left-sided CDH or Right-sided CDH?
Left-Sided CDH

5. What organs are / can be herniated in CDH?
Diaphragm, Lungs, Stomach, Liver, Intestines, Spleen, and more

6. What are the odds of a baby having CDH?
1 in 2500

7. What complication is most lethal to CDH babies?
Infections & Pneumonia

8. Approximately how many babies are born with CDH every year in the U.S.?
1600

9. Approximately how often is a CDH baby born in the world?
Every 10 minutes

10. Which is NOT a form of CDH?
Reynaldi

11. Which gender has a slightly higher survival rate of CDH?
Female

12. When was CDH first described in medical books?
1575

13. In what year was the first successful CDH repair on an infant?
1946

14. In what year was ECMO (Extracorporeal Membrane Oxygenation) first used on a CDH patient?
1976

15. In what year was the world's first CDH Research Group (The CDH Study Group) and the world's first CDH Support Group (CHERUBS) both founded?
1995

16. What causes Congenital Diaphragmatic Hernia in babies?
No one yet knows.

17. Which of these complications are most common with many CDH survivors?
Pneumonia, Pulmonary Hypertension, Allergies, Asthma, Feeding Issues, Developmental Delay

18. Can you tell what a CDH survivor looks like if you saw one in public?
Only if they had their shirt off and you saw a scar.

19. Which one of these procedures is more common than the other 2 in 2008 for these children?
Trachel Occlusion

20. What does ACDHO stand for?
Alliance of Congenital Diaphragmatic Hernia Organizations

DISCLAIMER:The information on all pages of this web site is for education only. It is not meant to be used in place of proper medical care and advice. CHERUBS does not encourage or discourage any medical treatments or procedures. Our purpose is to educate families and medical care providers so that they may make the best decisions for the patients' interests. You can not compare your child to other children born with CDH, they are all different. The opinions aired by members are not necessarily the views of all members, staff, or of CHERUBS.


 
 

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CHERUBS is proud to a member of The Alliance of Congenital Diaphragmatic Hernia Organizations.

CDH Organizations from around the world, working together on one goal.  Helping families affected by Congenital Diaphragmatic Hernia.

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