CTICasesAtaxia-Telangiectasia with Progressive Bronchiectasis and Dendriform Pulmonary Ossification
Imaging findings
Initial chest radiograph and CT demonstrate peribronchial consolidation, bronchial wall thickening, and bronchiectasis in the upper lobes, along with extensive mosaic attenuation and air trapping in the lung bases. Over a multi-year follow-up, the disease progresses to severe upper lobe scarring, traction bronchiectasis, volume loss pulling the hilum superiorly, and the development of juxtaphrenic diaphragmatic peaks due to traction on accessory fissures. CT also reveals multiple tiny, calcified, and ossified parenchymal densities within the scarred areas.
Key takeaways
Ataxia-telangiectasia is a rare, autosomal recessive chromosomal abnormality characterized by progressive cerebellar ataxia, cutaneous telangiectasias, and a combined humoral and cellular immunodeficiency. Recurrent sinopulmonary infections lead to progressive bronchiectasis, airway remodeling, and lung scarring. Chronic lung injury and fibrosis can lead to secondary dystrophic bone formation, known as dendriform pulmonary ossification, as fibroblasts differentiate into osteoblasts in the setting of chronic inflammation.
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