Published on in In Utero Insights
Congenital diaphragmatic hernia (CDH) is a complex and challenging anomaly. From before birth through the first few days of life, the health of a baby born with CDH can change unexpectedly, making it critically important to diagnose the condition before birth, deliver within the hospital where postnatal care will be received and have all aspects of specialized care immediately available in one location.
The Children’s Hospital of Philadelphia (CHOP) is one of only a few U.S. hospitals offering comprehensive care of this condition for both mother and baby. Families faced with the possibility of this diagnosis are referred to our Center for Fetal Diagnosis and Treatment, where they’re cared for by a multidisciplinary team of maternal-fetal medicine specialists, obstetricians, neonatologists, surgeons, anesthesiologists, radiologists, geneticists, cardiologists, pulmonologists and advanced practice nurses.
Seeing an exceptionally high volume of babies with CDH — nearly 1,000 since 1995 — has enabled our team to identify key factors that influence prognosis so we can better predict where an unborn baby will fall on the spectrum of severity. A comprehensive, one-day evaluation at the Center for Fetal Diagnosis and Treatment, includes high-resolution level II ultrasound to thoroughly evaluate the fetus and determine liver position, the lung-to-head circumference ratio (LHR) and observed over expected (O/E) lung volumes; and ultrafast fetal MRI for more detailed imaging. Experts from our Fetal Heart Program, one of the largest of its kind, also perform a fetal echocardiogram to rule out structural heart disease, which can occur simultaneously with CDH and pose additional challenges. This exam can be difficult due to compression of the heart by herniated organs in the chest. (See Employing Expert, Thorough Analysis to Better Predict Outcomes.)
Based on findings from this imaging, we partner with referring physicians and families to map out the best plan for delivery, and closely monitor the pregnancy, with increased surveillance in the third trimester due to an increased risk of intrauterine fetal demise.
“Every little detail matters,” says Holly L. Hedrick, MD, surgical director of CHOP’s ECMO Program and director of the Pulmonary Hypoplasia Program. “If tubes don’t go in right during stabilization, if there’s too much noise in the N/IICU, it can impact the outcome. Everything needs to be perfect.”
Teams of experts
This need for specialized care led CHOP to create the Neonatal Surgical Team, the only one of its kind in the world, in 2004. The instant babies are born in CHOP’s Garbose Family Special Delivery Unit (SDU), the first-ever birth facility designed exclusively for mothers carrying babies with known birth defects, the Neonatal Surgical Team provides stabilization and, often, specialized ventilation techniques.
Babies then receive care in the nearby Newborn/Infant Intensive Care Unit (N/IICU), where they are monitored and cared for until discharge following standardized protocols our team developed. The section of Pulmonary Hypertension in the Division of Cardiology also monitors babies for cardiac issues, performs postnatal echocardiography and provides any necessary treatment.
“We’ve got continuity of care, a plan, and everybody’s on the same page,” says Hedrick. “It’s not reinventing the wheel every time. There’s a set protocol and process.”
Babies with severely compromised or fragile lungs may require extracorporeal membrane oxygenation (ECMO). CHOP’s ECMO Program, founded in 1991, has been designated a Center of Excellence by the Extracorporeal Life Support Organization (ELSO). The program’s first patient was a child born with CDH. Since then, the program has expanded to 10 beds throughout the Hospital for neonates as well as pediatric and cardiac patients.
Our ECMO team is one of the most experienced in the country. An ECMO specialist and a N/IICU surgical registered nurse are present at the bedside at all times. ECMO Core Team specialists are trained to assemble and prepare the circuit for each patient use, and function as an expert technical resource for N/IICU staff. The team also includes an ECMO program manager (a respiratory therapist with additional certification as a neonatal and pediatric specialist), a clinical nurse specialist and a surgical director.
Care during and after surgery
As babies receive this comprehensive medical care, social workers and psychologists help support family members through all the ups and downs they experience. Once a baby is stable enough for diaphragmatic repair, a board-certified pediatric anesthesiologist provides general anesthesia for the operation. The surgical team comes to the N/IICU to perform the surgery. The surgeon makes an abdominal incision on the left or right side, depending upon the CDH location, moves the abdominal organs from the chest to the abdominal cavity, and closes the incision using a plastic surgery closure technique. For babies with larger defects or completely lacking a diaphragm on one side, the hole is closed with a soft tissue patch. Our patch rate is 53.4 percent with a recurrence rate of 5.4 percent (Tsai, et al).
Care continues well after discharge. Our Pulmonary Hypoplasia Program provides comprehensive, interdisciplinary follow-up specifically focused on this challenging condition. The program currently follows more than 335 children, beginning before discharge and continuing well into school age. By studying children’s outcomes over time, our team hopes to improve care.
“Just getting babies out of the N/IICU isn’t enough,” says Hedrick. “We want to take care of the whole baby well into childhood to know how they’re really doing.”
Our team is currently researching alternative treatments focused on helping the lungs grow before birth, which have the potential to offer better outcomes. And CHOP’s Center for Applied Genomics (CAG), one of the world’s largest genotyping programs and the only center at a pediatric hospital to have access to state-of-the-art, high-throughput genotyping technology, is working closely with the Center for Fetal Diagnosis and Treatment to examine the genetic causes of CDH. CAG has collected and biobanked DNA samples and performed high-density genotyping in search of genes that either predispose to or cause CDH, to enable the development of new and more effective therapies tailored towards preventing the condition in the future.
ECMO Program facts 1991–December 2012
- Designated Center of Excellence by Extracorporeal Life Support Organization (ELSO) since 2008
- More than 1,000 ECMO runs for over 940 patients
- Has provided ECMO support to more than 130 patients with CDH
- Ten complete ECMO systems Hospital-wide
- Core team of eight specially trained RNs and RTs, and an additional 20 RNs and RTs trained to maintain the ECMO system
Categories: In Utero Insights Winter 2013