Pulmonary Hypoplasia Program Research

The Children’s Hospital of Philadelphia is the only institution studying the neurodevelopmental and other long-term outcomes of patients with diagnoses that cause pulmonary hypoplasia. Following children from birth throughout childhood ensures optimal outcomes and helps us to gain a better understanding of how this condition affects children over the long term.

The Pulmonary Hypoplasia Program (PHP) is designed to both utilize clinical data for research and to quickly and seamlessly integrate the expanded knowledge resulting from that research into improved care for children.

What we study

The PHP maintains a database of over 885 patients who have received or are currently receiving follow-up care. We have established a baseline for how these children do following treatment, and the program has already begun to yield insights that improve understanding of pulmonary hypoplasia and overall care. Our research has enabled the creation of clinical standards for treating pulmonary hypoplasia, better serving families.

The PHP study aims first to identify trends of outcomes for children with the various diagnoses leading to pulmonary hypoplasia. That information enables more extensive and useful counseling for parents who have just received a diagnosis for their child, and helps the team to identify topics for future research in areas that outcomes are less than optimal.

Studies of the neurodevelopmental outcomes for children with pulmonary hypoplasia is one example of the work being done to better understand what to expect for children with these diagnoses. Research published by CHOP's Pulmonary Hypoplasia Program in 2010 and 2011 shows neurodevelopmental outcomes are normal – the conditions and their repair do not appear to affect children’s neurocognitive development.

The team is currently engaged in numerous research projects to better understand long-term outcomes for patients with pulmonary hypoplasia and related conditions. Studies include:

  • Longitudinal neurodevelopmental outcome in children with CDH
  • Timing of CDH repair in patients on ECMO
  • Frequency of inguinal hernia in giant omphalocele patients
  • 8- to 13-year-old PHP follow-up

Volumes

Pulmonary Hypoplasia Program Enrollment

Total patients enrolled in PHP Research Study as of February 5, 2020: 885

 
Patients

 

 
Patients

Pulmonary Hypoplasia Program - Total Patients by Diagnosis

Total diagnoses: 923

 
Congenital Diaphragmatic Hernia (578 cases)
 
Lung Lesions (195 cases)
 
Omphalocele (138 cases)
 
Other (12 cases)

 

 

As of February 5, 2020

*Children with multiple diagnoses are counted in multiple categories.