|ECR 2019 / C-3313|
|Diffuse lung disease associated with neurofibromatosis type-1 in children|
Seventeen paediatric patients that had undergone spinal CT for scoliosis (mean age=11.6, range 3-17 years-old) were collected. The entire lung volumes were completely evaluable in CT scans in 8/17 patients, while in 9/17 patients it was possible to evaluate about 75% of total lung volume. In 3/17 subjects (17.6% - ages 4, 13 and 14 years old) a condition of diffuse lung disease involvement was found. In the 3 lung-affected patients the disease was characterized by subpleural air-filled lesions in the upper lobes (blebs in 2/3 and cysts in 1/3 patients) (Fig. 2). No basilar fibrosis was detected (Fig. 3). All the patients with lung disease were asymptomatic.