Long-term Outcomes of 8- to 13-Year-Old Patients in the Pulmonary Hypoplasia Program

Long-term outcomes of 8- to 13-year-old congenital diaphragmatic hernia, lung lesion and giant omphalocele patients in the Pulmonary Hypoplasia Program

Specialists within the multidisciplinary Pulmonary Hypoplasia Program team at The Children's Hospital of Philadelphia (CHOP) are studying the long-term outcomes of children ages 8 to 13 who have received care at CHOP for a diagnosis of congenital diaphragmatic hernia, lung lesion, or giant omphalocele.

Pulmonary hypoplasia is a condition characterized by small, underdeveloped lungs,. It often occurs in patients with congenital diaphragmatic hernia, lung lesion, and abdominal wall defects such as giant omphalocele. Advances in prenatal and neonatal treatment have significantly improved the outcomes of these patients. However, these patients are often left with neurodevelopmental, cardiopulmonary, gastrointestinal and surgical morbidities that require long-term follow-up.

While our team is actively gathering data on perinatal and follow-up outcomes through our Pulmonary Hypoplasia Program, little is known about the long-term outcomes of these patients as they grow.

Led by principal investigator Holly L. Hedrick, MD, attending pediatric and fetal surgeon and director of the Pulmonary Hypoplasia Program at CHOP, this study aims to assess the long-term neurodevelopmental, cardiac, pulmonary, and quality of life outcomes in patients aged 8 to 13 years. Participation in the study includes traveling back to CHOP for a two-day research visit that will include a general surgery appointment; comprehensive neurodevelopmental, autism and motor function assessments; a six-minute walk test; pulmonary function tests; a limited echocardiogram and blood draw; and a chest X-ray.

For more information about this study, please contact Samantha Adams, research coordinator, at 215-590-2732 or adamss3@email.chop.edu.

Learn more about the ongoing follow-up research underway through the Pulmonary Hypoplasia Program.