There is a wide range of severity and outcomes for CDH. In the best cases, some infants do very well with routine treatment after birth. In the worst cases, some will not survive no matter how hard we try. And in the middle, some will live normally while others will have a difficult time and have to deal with some handicaps ranging from mild learning problems to breathing and growth problems. How the baby does after birth is determined by how well the lung grows before birth.
Fetuses on the best end of the spectrum have an excellent chance to lead a perfectly normal life. They do not require special prenatal management in terms of the timing or type of delivery, but should be delivered in a perinatal center with a Level III intensive care nursery with good neonatal and pediatric surgery support. The place of delivery is very important because transporting these babies after birth can be dangerous for the infant. Many babies still have to have the defect repaired after birth and will be in the intensive care nursery for several weeks. Even though the lung isn’t of normal size at birth, it has the capacity to grow and adapt for many years, so these kids will lead normal active lives without restriction.
On the other end of the spectrum, babies with severe CDH and very small lungs are guaranteed to have a difficult struggle after birth, and some will not survive. These babies require very skilled intensive care to stay alive—things like high-frequency or oscillatory ventilation, inhaled nitric oxide and, in some cases, extracorporeal membrane oxygenation (ECMO). ECMO provides temporary support for lung failure by circulating the baby’s blood through a heart-lung type machine. It can be life-saving, but can be used for only limited time before complications become excessive.
These babies must be delivered in a very experienced tertiary perinatal center with ECMO capability. The surgery to repair diaphragmatic hernia after birth is not an extreme emergency and is usually performed when the baby has stabilized in the first week of life. After repair, these babies will need intensive support for many weeks or even months. Even when the CDH is severe, greater than 70% of affected babies can be saved with intensive support. However, there will be long term health issues related to breathing, feeding, growth and development problems.
Most fetuses with CDH fall between these extremes of severity.
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