Chest CT scoring for evaluation of lung sequelae in congenital diaphragmatic hernia survivors

Objectives Data on long-term structural lung abnormalities in survivors of congenital diaphragmatic hernia (CDH) is scarce. The purpose of this study was to develop a chest computed tomography (CT) score to assess the structural lung sequelae in CDH survivors and to study the correlation between the CT scoring and clinical parameters in the neonatal period and at 1 year of follow-up. Methods A prospective, clinical follow-up program is organised for CDH survivors at the University Hospital of Leuven including a chest CT at the age of 1 year. The CT scoring used and evaluated, named CDH-CT score, was adapted from the revised Aukland score for chronic lung disease of prematurity. Results Thirty-five patients were included. All CT scans showed some pulmonary abnormalities, ranging from very mild to severe. The mean total CT score was 16 (IQR: 9-23), with the greatest contribution from the subscores for decreased attenuation (5; IQR: 2-8), subpleural linear and triangular opacities (4; IQR: 3-5), and atelectasis/consolidation (2; IQR: 1-3). Interobserver and intraobserver agreement was very good for the total score (ICC coefficient > 0.9). Total CT score correlated with number of neonatal days ventilated/on oxygen as well as with respiratory symptoms and feeding problems at 1 year of age. Conclusion The CDH-CT scoring tool has a good intraobserver and interobserver repeatability and correlates with relevant clinical parameters. This holds promise for its use in clinical follow-up and as outcome parameter in clinical interventional studies.