Advances in Care of Congenital Diaphragmatic Hernia

By Holly L. Hedrick, MD, Attending Surgeon; Surgical Director, Extracorporeal Membrane Oxygenation (ECMO) Center; Co-director, Neonatal Surgical Team; Director, Pulmonary Hypoplasia Program; Louise Schnaufer Endowed Chair in Pediatric Surgery

Congenital diaphragmatic hernia (CDH) is a birth defect that occurs around the 11th week of gestation. The diaphragm muscle has a defect in it that allows organs in the abdomen to move into the chest and push into the developing lungs. This condition can cause profound problems with lung development and, in turn, heart development.

Over the past three decades, Children’s Hospital of Philadelphia (CHOP) has established one of the best programs in the world to treat CDH. Our Richard D. Wood Jr. Center for Fetal Diagnosis and Treatment is the first program to combine comprehensive fetal diagnosis, expert antenatal care, delivery planning and delivery in a children’s hospital to prevent transportation, which we have found reduces the need for extracorporeal membrane oxygenation (ECMO) and improves survival. We deliver 50 patients with CDH every year in our Garbose Family Special Delivery Unit, the most of any program, and what we learn allows us to make breakthroughs.

Specialized Surgical Care from the Start

The Neonatal Surgical Team cares for babies who need surgery or other complex care immediately or soon after birth.

Our unique Neonatal Surgical Team is made up of a dedicated group of clinicians devoted to the care of babies with CDH in the crucial perioperative time period — when they may need treatment that includes specialized equipment such as the oscillator ventilator, ECMO or nitric oxide — and throughout their time in our Harriet and Ronald Lassin Newborn/Infant Intensive Care Unit (N/IICU). Our Pulmonary Hypoplasia Program (PHP), created in 2004, provides a lifetime of care for patients born with CDH, whose experiences and care needs are unique.

Translational discoveries

Our dedicated CDH team translates our findings into advances that have pushed care for this unsolved problem to new frontiers and improved the life course for children. We continue to study the molecular and genetic factors that cause CDH, and we are on the cusp of revolutionary advances.

ECMO is a way to rest the heart and lungs to get the sickest babies with CDH to the next stage of care. But it is fraught with complications. My colleague, Attending Fetal Surgeon Emily Partridge, MD, PhD, has been working to find a way to make ECMO safer and more eff ective for CDH patients. She has been leading pre-clinical studies of a postnatal pumpless arterio-venous (AV) ECMO circuit, and she is preparing to initiate a clinical safety trial.

We are also collaborating with our hospital’s Chronic Lung Disease Program to see if partial liquid ventilation offers another way of ventilating babies with CDH that is less harmful to their lungs. Fetal imaging radiologists complement these studies with in utero contrast-enhanced ultrasound and ultrasound elastography techniques.

Optimizing patient discharge

Our program emphasizes caring for families from “gestation to graduation.” This means supporting patients from their prenatal diagnosis, through any necessary fetal therapy, planned delivery and specialized newborn resuscitation (more on this in the article that follows), all the way through neonatal intensive care, convalescence, discharge and long-term follow-up.

To provide more consistent and cohesive care and a more positive patient/ family experience, we have developed an Infant Transitional Care Unit (ITCU) that cares exclusively for babies following neonatal critical care in our N/IICU. Having a dedicated space to care for this patient population increases their ability to thrive, providing expert clinical care in one location and cohorting patients to enhance family support systems.

Nurses specializing in education and follow-up meet with families to train them on homecare equipment prior to discharge. This is a crucial step to empowering families to care for medically at-risk babies.

"It’s a long and winding road, and sometimes what you think is going to be the answer turns out to be very different but it leads you to something that is the answer. That’s really the joy of science.” — Holly Hedrick, MD, on the team’s CDH research efforts"

Follow-up and the PHP

Our most groundbreaking achievement has been our ability to bring multiple disciplines including neonatology, pulmonary medicine, cardiology, general surgery, nutrition, audiology, behavioral health, social services and more, together to work on a single problem with multiple perspectives. Nowhere is the power of this collaborative approach more visible than in our PHP.

About the PHP

The PHP provides comprehensive care for children with conditions that cause underdeveloped lungs.

Our PHP provides the expert, interdisciplinary follow-up care children born with CDH often require into adolescence to manage pulmonary hypoplasia and other health issues. Our comprehensive pulmonary hypoplasia treatment involves coordination between many specialists that begins before a child is born and is focused on improving long-term outcomes related to pulmonary health, neurodevelopmental growth, surgical histories, nutritional needs, and late onset hearing loss. More than 600 patients from across the world are actively receiving follow-up care through the program.

Other advances we’ve made over the past three years include:

• Built a robust way to collect clinical outcomes data that has changed how we think about our patients in real time
• Achieved a milestone of more than 1,000 patients recruited from around the country to enroll in our outcomes registry
• Improved clinical practice by developing and adhering to an evidence-based feeding advance algorithm to improve the nutritional status of infants upon discharge

Our delivery room management of CDH includes:

• Videorecording of complicated newborn resuscitations to study human factors and performance
• Implemented use of a respiratory function monitor (RFM) during delivery room (DR) resuscitations to provide real-time monitoring of tidal volumes and exhaled CO2 to troubleshoot and improve respiratory support for infants
• Display of CDH-specifi c newborn resuscitation guidelines in the DR