New Study Establishes Benefits of Tracheal Occlusion for Most Severe CDH

Published on in CHOP News

Newly published results from the Tracheal Occlusion to Accelerate Lung Growth (TOTAL) Trial have established that tracheal occlusion offers benefits in severe cases of prenatally diagnosed congenital diaphragmatic hernia (CDH).

CDH is a birth defect characterized by the development, very early in gestation, of a hole in the diaphragm, the breathing muscle that separates the chest from the abdomen. As a result, the intestines, liver and other organs in the abdomen can move into the chest and press on the developing lungs. This prevents the lungs from growing and developing normally.

In severe cases, CDH can lead to serious disease and death at birth. For these babies, treatment before birth may allow the lungs to grow enough before birth so these children are capable of surviving and thriving. Fetoscopic endoluminal tracheal occlusion (FETO) is a minimally invasive procedure that involves placing a balloon in the unborn baby's airway for several weeks, which distends the lungs with fluid. This stretch in turn signals the lungs to grow. Lungs stimulated to grow before birth are more capable of sustaining life after birth.

Before FETO can be offered as a treatment option, it is critical to obtain an accurate prenatal diagnosis confirming the severity of the condition. A comprehensive evaluation of each pregnancy will determine if patients meet the strict criteria required to consider FETO.

At Children’s Hospital of Philadelphia’s Richard D. Wood Jr. Center for Fetal Diagnosis and Treatment, we care for more than 50 newly diagnosed patients per year with CDH. We provide a rare continuity of care for patients spanning the spectrum of severity.

Since the early ’90s, our team has investigated fetal tracheal occlusion as a viable treatment. Our fetal medicine specialists have extensive experience in fetoscopy, an endoscopic procedure performed during pregnancy to allow surgical access to the fetus, the amniotic cavity, the umbilical cord, and the fetal side of the placenta. In 2015, we began offering FETO as a treatment option to select fetuses with severe CDH. Throughout this time, we have consulted with our colleagues in Europe leading the TOTAL Trial, who are the authors of this promising study.

There is no one-size-fits-all approach to treating CDH, which is why it’s so important to develop a custom treatment plan for each baby's and family's unique needs. We have unparalleled experience providing the most detailed prenatal diagnosis and using that information to predict a baby’s prognosis after birth in order to determine the best options.

Advancements in CDH care

Our team has made many advances that have improved the outcome for all children with CDH.

  • We built the Garbose Family Special Delivery Unit (SDU), the world’s first special delivery unit in a freestanding children’s hospital for healthy mothers carrying babies with known birth defects. The SDU allows us to get to work immediately, providing the complex care CDH babies need.
  • When a baby with CDH is born in our SDU, they have immediate access to our expert infant stabilization team and CDH neonatal team. By delivering right there in the same hospital where they’ll be cared for, we eliminate the need for neonatal transport, which is associated with increased mortality and need for extracorporeal membrane oxygenation (ECMO) in these patients.
  • We created the first dedicated Neonatal Surgical Team to co-manage the care of babies born with conditions that require surgery after birth, particularly CDH.
  • Members of our team also helped develop the ex utero intrapartum treatment, also known as the EXIT procedure, a highly technical delivery intervention. The procedure was originally developed to deliver babies with occluded tracheas, and it was subsequently expanded for babies with large neck/lung masses. We have experience with more than 120 ex utero intrapartum treatment (EXIT) deliveries, and we have the personnel and protocols in place for emergently attending to the airway 24/7/365. These teams can be mobilized at a moment’s notice.

Continuing CDH research to improve care

Inspired by our patients and families, we have built the most robust research program dedicated to improving our understanding and care of CDH. Our Pulmonary Hypoplasia Program — created in 2004 — is the only program in the world to provide long-term multidisciplinary care to over 1,000 children. We use all we learn to improve both prenatal and postnatal care for CDH.

FETO is an innovative care option and one of many treatment options we are able to utilize based on a baby's unique needs. We offer it cautiously and with many layers of protection to ensure the well-being of both mother and baby, and we continue to seek a better understanding of the fetal response to tracheal occlusion.

More information on our care of CDH and the FETO procedure.

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