Bochdalek Hernia

What is Bochdalek hernia?

This is a congenital defect that could be potentially life threatening. It is a condition in which the baby’s diaphragm does not close correctly, which could possibly cause abdominal organs and other tissues to push upward into their chest cavity. There is usually a hole on the left side of their diaphragm, which is where approximately eighty-five percent of them occur. Only fifteen percent occur on the right side. It is more common in babies and is rarely seen in adults but they can have it.Bochdalek hernias occur in approximately one in every two thousand two hundred to twelve thousand five hundred births yearly. Approximately twenty percent of those with a Bochdalek hernia also have a congenital heart defect. From five to sixteen percent will have some type of chromosomal abnormality along with a Bochdalek hernia. These hernias are more common in males than in females. Because it is a life-threatening condition between twenty-five and sixty percent die from a Bochdalek hernia.

Symptoms at birth

When a baby is born with a Bochdalek hernia, they may show signs of breathing difficulties due to the pressure on their lungs from the other organs that are pressing upwards. They may also have a rapid heart rate. Because of the lack of oxygen in their bloodstream, their skin may have a blue tint. The baby may also fail to cry, open their eyes, have irregular chest movements, breathe rapidly, and be limp. One side of their chest cavity may appear larger than the other side. Their abdomen may also appear to look as if it is caved in.

Causes of Bochdalek Hernia

The exact causes for a baby to have a Bochdalek hernia are not completely understood but physicians believe that there are different factors that could contribute to these diaphragm deformities. Some of these factors include:

• A random genetic mutation that affects organ development
• Vitamin A deficiency
• Inherited metabolic disorder

Normally the bottom of their diaphragm will close around the eighth week of gestation. It is thought that the problems that results in the baby having a Bochdalek hernia occurs about two weeks earlier, around the sixth week of gestation. During the time of diaphragm development the baby’s intestines, stomach, and esophagus are also forming. In this period of development their intestines either become locked into the chest cavity while the diaphragm is developing or there is a malformation in their diaphragm. In addition to being born with a Bochdalek hernia, some are also born with other problems like lungs that are significantly underdeveloped or heart defects.

Diagnosis

It is sometimes possible for a physician to detect this type of hernia using ultrasounds along with other imaging tests before the baby is born and is still in the womb but in most cases, they do not know that the newborn has a major problem until after they are born.After the baby is born, a team of physicians can quickly diagnose this type of hernia by doing a physical exam. During the physical exam, the baby’s abdomen is typically easy to compress and feels empty when their organs are too far up in their chest. Bowel noises may be heard in the chest by using a stethoscope. If the baby is stable enough they may do a CT scan or chest x-rays to confirm the diaphragm deformity. To evaluate the health of the baby’s heart they may do an echocardiogram, which is an ultrasound of their heart. They can also do a chromosomal test to see if the cause is genetic.

Treatment

When the baby has a Bochdalek hernia emergency surgery is often required to move the abdominal contents into the correct alignment and close the opening in their diaphragm permanently. In treating a Bochdalek hernia the first goal is to stabilize the baby’s breathing with cardiopulmonary resuscitation (CPR) or oxygen therapy. If their heart is not functioning correctly a bypass machine may be necessary in order to preserve blood circulation. If the baby’s lungs are not fully developed they may put the baby on a life-support machine until their lungs have developed more.

Surgery

This surgery is a delicate procedure and involves cutting the chest cavity open and then fixing the kidneys, stomach, bowels, and any other organs back into the proper place manually. Once the organs are back in their proper place the hole in their diaphragm is sutured followed by the closing of the surgical wound. After the surgery the baby will need monitoring around-the-clock and treatment to make sure that there are no complications. This could be for several days, even weeks. It depends on how severe the Bochdalek hernia was and any problems the baby had after birth. Normally after surgery the baby will stay in the hospital almost thirty days.

Long-term complications

Most babies who get immediate treatment are able to recover and develop as normal but for some there could be long-term complications that can include:

• Chronic lung disease because of the initially undeveloped lungs
• Have gastroesophageal reflux disease (GERD), which is when their stomach acids come back up into their esophagus. It is a painful condition and one that could cause feeding problems.
• Have developmental delays
• Hearing loss
• Not grow properly, which is also referred to as failure to thrive.

It may be necessary for the baby to have medical intervention for years but it all depends on the extent of the complications.