The Pulmonary Hypoplasia Program (PHP) at CHOP provides long-term follow-up and the most accurate information to families of children with the most severe birth defects in our Newborn/Infant Intensive Care Unit: congenital diaphragmatic hernia (CDH), abdominal wall defects and lung lesions.

“There’s no other program quite like ours,” says Lisa Herkert, program coordinator. “All of our specialists work together to formulate the best treatment plan for each and every patient.”

Through the PHP, Children’s Hospital is one of few institutions studying the long-term outcomes of these patients. The program maintains an IRB-approved database of patients who have received or are currently receiving follow-up care and has already begun to yield insights that improve understanding of pulmonary hypoplasia and overall care. More than 16 publications describing surgical and neurodevelopmental outcomes have resulted.

Our investigators continue to study long-term neurodevelopmental outcomes of children born with CDH. At preschool age, the majority of our CDH patients have normal neurodevelopmental and functional scores.

CHOP’s Center for Fetal Diagnosis and Treatment recently won a national research grant for $10,000 from CHERUBS, the Association of Congenital Diaphragmatic Hernia Research Awareness and Support, which will help further our understanding of this condition.

Pulmonary Hypoplasia Program Enrollment

Total patients enrolled in PHP Research Study as of May 21, 2018: 740

 
Patients

 

 
Patients

Pulmonary Hypoplasia Program - Total Patients by Diagnosis

Total diagnoses: 774

 
Congenital Diaphragmatic Hernia (489 cases)
 
Lung Lesions (163 cases)
 
Omphalocele (115 cases)
 
Other (7 cases)

 

 

As of May 21, 2018

*Children with multiple diagnoses are counted in multiple categories.

 

In 2010 we published early neurodevelopmental outcomes of CDH survivors initially enrolled in our program. All patients underwent prenatal and postnatal care at CHOP with standardized methods of prenatal assessment and postnatal management.*

Patient characteristics

  • LCDH: 31/41 (76%)
  • RCDH: 10/41 (24%)
  • Liver up: 22/41 (54%)
  • Need for ECMO : 11/41 (27%)

Results of 36 children evaluated under 3 years of age

Overall neurodevelopmental categories (cognitive, language and psychomotor)

  • 31% of CDH children scored within the average range
  • 16% of patients demonstrated significant delays

Cognitive and language skills

  • Average: 49%
  • Mixed†: 19%
  • Mildly delayed: 17%
  • Severely delayed: 15%

†Average and mildly delayed in either cognitive or language scores

Psychomotor scores

  • Normal: 46%
  • Mildly delayed: 23%
  • Severely delayed: 31%

*Danzer E, Gerdes M, Bernbaum J, D’Agostino J, Bebbington MW, Siegle J, Hoffman C, Rintoul NE, Flake AW, Adzick NS, Hedrick HL. Neurodevelopmental outcome of infants with congenital diaphragmatic hernia prospectively enrolled in an interdisciplinary follow-up program. J Pediatr Surg. 2010 Sep;45(9): 1759–66.