Congenital Diaphragmatic Hernia
Treatment

There is a wide range of severity and outcomes for CDH. In the best cases, some infants do very well with routine neonatal care 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, however, more than 70 percent 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 babies with CDH fall somewhere in between these extreme ends of the spectrum.

Delivery

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 or perinatologist, the neonatologist and pediatric surgeons, so the baby can be stabilized and treated in the same center.

Babies with less severe CDH 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 neonatologists and pediatric surgeons. 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 with severe CDH need very skilled intensive care: 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 machine. It can be life-saving, but can be used for only limited amounts of time before complications become excessive. These babies must be delivered in a very experienced tertiary perinatal center with ECMO capability.

Surgical Repair After Birth

The surgery to repair CDH after birth 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 surgeon will evaluate the severity and exact location of the hernia during 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 there is enough diaphragmatic muscle, the hole can be sewn closed. This is called a primary repair. If the opening is large or a significant portion of the diaphragm is absent, the surgeon will use additional tissue or material to close the defect. Often, a synthetic biocompatible material will be used to patch the opening. This is called a patch repair. In some patients, the surgeon will close the hole in the diaphragm with a flap of muscle from the infant's abdomen or back. This is called a muscle flap repair.

In select 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 Care

We recommend seeing your child's pediatrician within one week of leaving the hospital.

Once your child is home, we wish to closely monitor your child's progress in our outpatient follow-up program for children with CDH, called the LIFE Program. Our specialists have specific expertise in providing continued long-term care for children with CDH. Team members include experts in pediatric surgery, neonatology, pulmonology, nutrition, developmental pediatrics, gastroenterology, social services and nursing. Before your child is discharged from the hospital, we will make an appointment for your child's first visit to the LIFE Program.

CDH Recurrence

Some children will need another operation or operations later on, 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 needed 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 who had 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 any time.

Reviewed by health care specialists at UCSF Benioff Children's Hospital.

Related Information

UCSF Clinics & Centers

Fetal Treatment Center
1855 Fourth St., Second Floor, Room A-2432
San Francisco, CA 94158
Phone: (800) 793-3887
Fax: (415) 502-0660
Appointment information

Intensive Care Nursery
1975 Fourth St., Third Floor
San Francisco, CA 94158
Phone: (415) 353-1565
Fax: (415) 353-1202

Patient Experiences

  • Luke Marton
    After Three Surgeries, Boy with Congenital Diaphragmatic Hernia Thrives
  • Sloane Rilliet
    After Specialized Prenatal and Newborn Care, a Healthy Girl