Pulmonary nodular opacities in children with hereditary hemorrhagic telangiectasia

Background Hereditary hemorrhagic telangiectasia (HHT) is a rare inherited disorder of vascular endothelium that can result in the formation of pulmonary arteriovenous malformations (pAVMs). We observed that small pulmonary nodules, possibly representing early vascular malformations, are more common in these children than in the general population. Objective To describe and characterize the small pulmonary nodules seen on chest CT examination in the lungs of children with HHT. Materials and methods We followed 19 children who had undergone chest CT as part of their initial diagnostic evaluation in the HHT clinic of a pediatric hospital. Chest CT scans were reviewed independently by two radiologists blinded to clinical data. Pulmonary nodules were analyzed according to lobar distribution, anatomic position in the lung (outer, mid, inner third), shape (round, linear, V- or Y-shaped, ill-defined), presence of overt AVMs, and nodule size (≤ or >5 mm). We also reviewed a control group of chest CT scans performed on 25 age-matched children. Results A total of 35 pulmonary nodules were identified on the CT scans in 15 of the 19 children. Multiple nodules were seen in eight children (42%), solitary nodules were seen in seven children (37%) and no nodules were seen in the remaining four children (21%). The nodules were found to be nonspecific with regard to multiple factors other than their anatomic position within the lung. The chest CT scans of 2 of the 25 age-matched controls revealed a total of only four nodules, a significantly lower total ( P = 0.0001 vs. children with HHT). Conclusion Nonspecific nodules are commonly seen in children with HHT.