Congenital Diaphragmatic Hernias (CDH): Improving Outcomes with Advanced Imaging & Nutrition

CDH occurs in approximately 1 in 3,000 births

Referral centers tend to see 20 to 30 CDH babies per year

CDH anatomy is variable, and variability greatly impacts patient survival

Initial ultrasound at roughly 20 weeks of pregnancy often identifies CDH and potentially other anomalies that can greatly impact outcome

Overall survival for infants born with CDH remains static at about 70% over the past two decades despite intensive efforts to improve care

Imaging findings such as fetal lung volumes, liver herniation, and lung to head ratio contribute to prognosis

Cincinnati Children's has an MRI in their NICU which allows easy postnatal imaging for CDH babies both before and after repair

Ultra-short echo T MRI technique allows determination of lung volumes, lung density, and lung mass postnatally

In a cohort of 13 babies at Cincinnati Children's with postnatal imaging compared to fetal MRI, the lungs do grow postnatally with increases in total lung volume, right lung volume, and left lung volume

The non-CDH or good lung (right lung) grew better than the left side lung

The left lung was growing at about 2 mL per week slower than the right lung

The relative growth of the left lung increased in babies that had better weight gain

Maximizing calories and protein intake is important for CDH patients to maximize growth, as growth is associated with better growth of the more severely hypoplastic lung

Babies who gained weight better had more growth of their left lung mass but did not impact the mass of the right lung

2D time-of-flight MRI sequence can detect blood vessels in the lungs down to a size of 0.7 millimeters

Vascular density is calculated by dividing the volume of blood vessels by the volume of the lungs

CDH pulmonary vascular density correlated very strongly with outcomes in babies with CDH

Lower vascular density correlates with longer time spent on the ventilator

Babies discharged on room air had significantly higher vascular density than those discharged on oxygen or mechanical ventilation