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Congenital Diaphragmatic Hernia Treatments

• What are my choices during this pregnancy?
• Who is a candidate for balloon tracheal occlusion?
  • Inclusion/Exclusion criteria
  • UCSF formal evaluation
  • The balloon tracheal occlusion operation
  • Insurance pre-authorization requirement
• What will happen after birth?

What are my choices during this pregnancy?

For those families who choose to continue the pregnancy the most important next step is the accurate prenatal diagnosis about the severity of the condition which determines the choices available for prenatal management. If your fetus is on the better end of the spectrum, the most important choice is where to deliver the baby. The timing and type of delivery will not greatly affect outcome, but the place of delivery certainly will. These families will want to work out a plan for delivery and postnatal care with their obstetrician/perinatologist, the neonatologist, and pediatric surgeons so the baby can be stabilized and treated in the same center.

For fetuses in the middle of the spectrum, the place of delivery becomes even more important. These babies will need very intensive support after birth and should be delivered in highly experienced centers with ECMO capability. ECMO is a heart-lung machine that provides oxygen to the baby when the lungs are not capable of doing this. It is a medical therapy that can be used for a limited time, usually up to two weeks. Careful planning and coordination of the timing of delivery is necessary to avoid high-risk situations such as delivering in one hospital and transporting a critically ill baby to another center.

Fetal Intervention

Fetuses on the most severe end of the spectrum with liver up and LHR less than 1.0 are candidates for prenatal intervention. For more than two decades, we have been working on ways to improve the outcome for these fetuses by getting the lung to grow before birth, so that it will be adequate at the time of birth. The most promising uses FETENDO fetal surgery, specifically a fetoscopic temporary tracheal occlusion to enlarge the lung.

While in the uterus, the fetal lung constantly makes fluid that escapes through its mouth and into the amniotic fluid. When the trachea is blocked, this fluid stays in the lungs. As it builds up, the lung fluid expands the lungs, stimulates their growth, and pushes the abdominal contents (liver, intestine) out of the chest and into the abdomen. The goal of this treatment is to have a baby born with lungs that are big enough that the child can breathe and provide itself enough oxygen to breath on its own.

Temporary tracheal occlusion of the fetal trachea can now be accomplished at 27-28 weeks gestation using a very small telescope (the size of a straw) placed through the mother’s skin. A tiny detachable balloon is placed in the fetal trachea. Once the fetal lung has grown, the balloon can be removed or deflated so the baby can be born normally. This deflation procedure is usually done before birth. Again, fetal intervention is offered only when the diaphragmatic hernia is on the bad end of the spectrum with the liver up and an LHR less than 1.0.

Who is a candidate for balloon tracheal occlusion?

Balloon tracheal occlusion is only offered to those patients whose fetus have the most severe form of congenital diaphragmatic hernia. We have defined this group as: 1) a part of the liver has moved into the fetal chest cavity, and 2) the measured lung on the right side (not the side of the hernia) is small and has a lung to head ratio (LHR) of less than or equal to 1.0

In order to be considered for fetal intervention, you must first undergo an amniocentesis to determine that there are no chromosome problems with the fetus. The results of this test usually takes 7-10 working days.

UCSF formal evaluation

If you meet all these criteria and wish to be considered for tracheal occlusion you will need to travel to San Francisco for a formal evaluation. A formal evaluation would include: an ultrasound (to determine liver position and LHR), and ECHO (ultrasound of the heart to rule out any abnormalities), and counseling by a number of the members of our Fetal Treatment Center team (surgeon, social worker, perinatologist, nurse, etc).

The balloon tracheal occlusion operation

If you are found to be a tracheal occlusion candidate, and you chose to undergo the procedure, the tracheal occlusion operation will be performed between the 26th and 28th week of your pregnancy. The procedure requires an overnight hospital stay. After discharge from the hospital you will need to stay in San Francisco while the balloon is in your fetuses' trachea. This is usually for a period of 4 weeks. During this time you would have weekly ultrasound and medical appointments. After 4 weeks you would again enter the hospital for an overnight stay to have the balloon removed from the fetal trachea. If the procedures are successful, you could return home to deliver.

Insurance pre-authorization requirement

Both the evaluation and the operations need to be authorized by your insurance company. Most insurance companies have paid for this procedure for their insured. You should contact your insurance company to determine whether pre-authorization is required. We can provide your with the diagnosis and procedures codes.

What will happen after birth?

All babies with CDH should be delivered at a tertiary perinatal center with a high-level intensive care nursery and pediatric surgery. Although some babies with a very favorable outlook (LHR > 1.4) will not need very high level intensive care, most should be cared for by neonatologists and surgeons in an ICN experienced in high frequency and oscillation ventilation, nitric oxide inhalation, and particularly extracorporeal membrane oxygenation (ECMO). It is not possible to tell before birth how much support any baby will need after birth. Babies with CDH known before birth should never be delivered in an institution incapable of providing all the support needed: transporting a sick baby is dangerous.

All babies with CDH should be delivered into a “set-up” where the neonatologists take the baby from the obstetricians and immediately provide life support—breathing, oxygen, IV fluids, etc., as needed. The baby’s response will determine the amount of breathing support is needed, ranging from a little oxygen to a breathing machine to ECMO.

The severity of the lung problem will also determine the course in the intensive care nursery—again, ranging from surgical repair in the first day and home in a few weeks, to full support including ECMO for weeks, surgical repair (sometimes done while the baby is on ECMO), and then months of very intensive support before going home.

The long-term outcome depends on this need for very intensive support.