Johns Hopkins All Children's Hospital

Congenital Diaphragmatic Hernia

Our highly skilled specialists in the Johns Hopkins All Children’s Hospital Congenital Diaphragmatic Hernia Program provide a unique approach to surgery and care.

The Congenital Diaphragmatic Hernia (CDH) Program at Johns Hopkins All Children’s Hospital provides hope during an incredibly stressful time that may otherwise feel hopeless. Our CDH diagnosis, evaluation and treatment team, led by CDH expert David Kays, M.D., is rewriting the book on CDH survival and outcomes. Whatever you may have been told about your child’s chances with CDH, we urge you to speak with our team and learn that you have options and hope for a healthy outcome with your baby. 
 
Our CDH program is built on Dr. Kays’ advanced experience from treating more than 360 CDH newborns. His expertise, combined with that of our team, is changing how we think about the survival and outcome potential of the fetus diagnosed with CDH. Our mutual belief that your baby can survive is a fundamental component of outstanding outcomes.

What is CDH?

Occurring with relatively high frequency for a birth defect, CDH affects about one in every 3,000 pregnancies.  CDH is a result from the incomplete formation of the diaphragm, that muscle that separates the abdomen from the chest. The resulting hole may be small and allow a limited amount of intestine into the chest or it may be large and allow a very large amount of abdominal contents to herniate from the abdomen into the chest, starting early in development. The resulting mass of abdominal organs in the chest, which can include intestine, stomach, spleen and even liver, takes necessary space away from the developing lungs. The result is small lungs, varying from moderately to severely small, and making it hard for the baby to breathe.  A larger hole creates a larger impact on the lung size, so CDH presents as a spectrum of severity from less severe to highly severe.  It’s not straightforward to compare one CDH fetus or newborn to another.

Our experience has shown that the vast majority of CDH infants do have enough lung to survive, but it requires expert management to help those with the very small lungs to survive. Parents with a fetus diagnosed with CDH have traveled from all over the United States (and beyond) to access the expertise at Johns Hopkins All Children’s Hospital.

Infant without Congenital Diaphragmatic HerniaIllustration showing the organ arrangement of a normal healthy infant
 
Infant with Congenital Diaphragmatic HerniaIllustration showing the organ arrangement of an infant with congenital diaphragmatic hernia
 

The success of CDH treatment often depends upon whether other anomalies are present. CDH by itself or with other non-life-threatening conditions, such as a single kidney instead of two, often can result in a successful outcome with proper management. In 10 to 12 percent of cases, we find bilateral CDH, meaning it is on both sides of the abdomen, or CDH in combination with other life-threating conditions such as severe heart defects or major genetic or chromosomal defects. These additional life-threatening defects profoundly influence the outcomes for those affected, and these cases are not included in trials of prenatal interventions at other centers.

We evaluate cases where additional life-threatening conditions are present on an individual basis, but the very low survival and high-risk outcomes in these patients is directly related to the additional anomalies, not the treatment provided. Therefore, the survival and outcome data referred to here includes only cases of CDH by itself or CDH with other non-life-threatening conditions unless otherwise noted.

Why Choose Johns Hopkins All Children’s Hospital?

It’s the people.
The care
The hope.
The equipment, innovative techniques, and technology

Our highly skilled specialists in the Johns Hopkins All Children’s Hospital Congenital Diaphragmatic Hernia Program provide a unique approach to surgery and care. Led by Dr. David Kays , a world-renowned expert in caring for infants with CDH.  The care begins at the fundamental basic level.

Hope. Our care team believes your baby can survive and our outcomes consistently reinforce what we’ve always believed: CDH infants can survive and thrive*.  The team’s belief and unwavering dedication to patient care is a fundamental to the overall care of your baby and our support of you, the parents.

Expert Ventilator Care: Babies with CDH are born with critically small and fragile lungs. Any lung injury, including from the life-saving equipment being used, can potentially convert a survivable baby to non-survivable. Dr. Kays and his team recognize this delicate balance and have pioneered “kinder-gentler ventilation” for CDH infants.  Dr. Kays has published his work and outcomes on this ventilator care for nearly 20 years.

Inclusive and Expert Use of ECMO when needed: Extracorporeal membrane oxygenation (ECMO) is an important method whereby heart-lung support is provided mechanically in order to let the baby's heart and lungs rest and develop. Many CDH programs across the country do not provide ECMO to the most severely affected patients.  And, the national survival rates for CDH infants who require ECMO is 50 percent. Our team’s experts are comfortable administering this innovative support to the most highly complex CDH patients and our outcomes continue to push us to do so.  Since the implementation of the CDH program at Johns Hopkins All Children's in 2016, our CDH infants who require ECMO survive and thrive at a rate of over 90 percent.

Every baby receives appropriate and best care: Repair of the CDH does not automatically make small lungs become big lungs, but the repair activates an important cascade of events that are better for small lungs. The repair allows the lungs to grow to the fullest extent possible. It can be frightening for the parents because babies with severe CDH often get worse for a period of time before they get better after CDH repair. To understand why this happens, Dr. Kays and his team have spent decades studying the “best time” to do the CDH repair.  Reviews and studies have been conducted on early repair versus late repair, and how the severity of the CDH (liver position) affects the outcome of those decisions. As noted earlier ECMO makes it more complex, as the most severe babies often require ECMO, and CDH repair on ECMO is difficult and can cause bleeding. But,  what we’ve learned is that the more severe the CDH, the more important it is to do the repair. This is counter to common thought nationally, but we are convinced this is the right direction.
 
Consistency of Care: Parents know their child better than anyone and for consistency across the continuum of a child’s care, the parent is an integral member of the care team. Because of this engagement, a parent will never wonder what the plan is, or who is in charge of their child's care. This diligent and consistent oversight for patient management makes a difference in outcome with quality of life and the day-to-day patient experience while in the hospital.

Leading Survival and Outcomes: Left liver-up CDH is the most severe anatomic subset of CDH.  It’s more severe on average than left liver-down or right CDH.  The national survival rate for left liver-up is 45 percent. Our published survival in 2015 was 75 percent. Today, it’s even higher. Because of our experts delivering the specialized, innovative, collaborative and compassionate care for some of the most severe CDH patients and achieving high outcomes for survival and productive healthy long lives, families in search of hope and help travel thousands of miles for care.

The care of CDH babies is about both survival and quality of outcome. We know that the number of babies who survive is about how well we take care of their lungs. The quality of that outcome is about how well we take care of their brains. That link is our number one priority every day.

In 2016 we cared for 17 infants, and in 2017 we cared for 33 infants.  So far in 2018, we have cared for 20 CDH infants in just 3 months, averaging over 6 newborns with CDH per month.  The majority have traveled here from around the US, and represent patients with very high severity.

*Infants with severe CDH plus a highly severe second anomaly such as uni-ventricular heart defect, severe chromosomal defects, or severe bilateral CDH, face complex physiologic challenges we have generally not been able to overcome. This statement relates to the spectrum of CDH excluding those with a highly severe or lethal second anomaly).
 

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