The wide, flat muscle that separates the chest and abdominal cavities is called the diaphragm. The diaphragm forms over the course of the 6th through 12th weeks'' gestation in the fetus. When it does not form completely, a defect, called congenital diaphragmatic hernia (CDH), is d. This defect or hole in the diaphragm muscle.

Prenatal diagnosis & fetal treatment of CDH

CDH is typically diagnosed before birth during routine screening tests. Once this initial diagnosis is made it is important to get an expert assessment of your fetus''s condition in order to determine the severity. The severity of your fetus''s condition is an important guideline for determining the best treatment options.

Most fetuses with CDH require some level of prenatal monitoring. Care must be taken when choosing where to deliver the baby. 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.

Fetuses on the most severe end of the spectrum with liver up into the chest and lung-to-heart ratio (LHR) less than 1.0 may be candidates for prenatal intervention, including fetoscopic balloon tracheal occlusion. More detailed information about prenatal testing, diagnosis, and fetal treatment for CDH can be found at Fetal Treatment Center: Congenital Diaphragmatic Hernia.

What is the outcome for a child with CDH?

There is a wide range of severity and outcomes for CDH. In the best cases, some infants do very well with routine neontal and surgical treatment after birth. Even though the lung isn''t of normal size at birth, it has the capacity to grow and adapt for many years, so these children can 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. Even when the CDH is severe, greater than 70% of affected babies can be saved with intensive support. However, there are often long term health issues related to breathing, feeding, growth, hearing, and development.

Most fetuses with CDH fall somewhere in-between these extremes of severity. For more information, read our page on CDH Long-Term Complications.

Delivery management of CDH

Babies on the better 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 experienced neonatal and pediatric surgery providers. The place of delivery is very important because transporting these babies after birth can be dangerous for the infant. Most babies will be in the intensive care nursery for several weeks.

Babies diagnosised with severe CDH require very skilled intensive care to stay alive: high-frequency 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 of 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.

After-birth surgical repair of CDH

The surgery to repair diaphragmatic hernia after birth is not an emergency and is usually performed after the baby has stabilized in the first week of life. After repair, these babies will need intensive support for many weeks or even months.

The severity and exact location of the hernia are evaluated by the surgeon at the time of the operation. Based on these findings, the hernia will be repaired in one of three ways. If the opening in the diaphragm is small and the amount of diaphragmatic muscle is adequate, the hole can be sewn closed. This is called a primary repair.

If the opening in the diaphragm is large or a significant portion of the diaphragm is absent, the surgeon will require additional tissue or material to close the defect. Often, a synthetic biocompatible material will be used to patch the opening-called a patch repair. In some patients, the surgeon will close the hole in the diaphragm with a flap of muscle-the internal oblique or latissimus dorsi from the infant''s abdomen/back. This is called a muscle flap repair. In circumstances, infants may be a candidate for a minimally invasive thoracoscopic repair. The surgeon will determine the type of repair required and the material to be used, at the time of the operation.

Follow up medical appointments & long-term care

A discharge summary of your child''s hospital stay will be sent to your pediatrician. We recommend that you see your pediatrician within one week of leaving the hospital. We will make an appointment for your child to have their first visit in our long-term Multidisciplinary CDH Clinic soon after discharge. For questions before and in between appointments you may call the pediatric surgical office.

Long Term CDH Complications

How a baby with congenital diaphragmatic hernia recovers after birth is determined by many factors. Babies who experienced more severe CDH may face challenges which can include mild learning problems, breathing illnesses, hearing loss, and growth problems.

CDH recurrence

Some children require subsequent operation(s) to repair a recurrence of the CDH. Often this is related to the severity of the CDH at birth, and whether or not a synthetic patch was required to close the hole in the diaphragm. In our experience recurrence is less common for children with a primary repair and more common in children that require a patch repair. The patch may pull away from the chest wall, as the child grows, causing a recurrence.

The symptoms of a recurrence include retching, gagging and vomiting feedings, pain, or rapid breathing. Some children will have no symptoms. If you suspect a recurrence, take your child to the emergency room and explain your child''s history and your suspicions to the emergency room team. You and the emergency room physicians can contact our surgeons at anytime.