Purpose: The purpose of this study was to investigate the extravascular thoracic multidetector computed tomography (MDCT) angiography findings of pediatric primary pulmonary vein stenosis (PVS) by comparing extravascular thoracic MDCT angiography findings in children with and without PVS.Materials and Methods: All pediatric patients (age 18 y and below) with a known diagnosis of primary PVS, confirmed by echocardiogram and/or conventional angiography, who underwent thoracic MDCT angiography studies from July 2006 to December 2020 were included. A comparison group, comprised of age-matched and sexmatched pediatric patients without PVS who underwent thoracic MDCT angiography studies during the same study period, was also generated. Two pediatric radiologists independently evaluated thoracic MDCT angiography studies for the presence of extravascular thoracic abnormalities in the lung (ground-glass opacity [GGO], consolidation, pulmonary nodule, mass, cyst, septal thickening, fibrosis, and bronchiectasis), pleura (pleural thickening, pleural effusion and pneumothorax), and mediastinum (lymphadenopathy and mass). When a thoracic abnormality was identified, the location and distribution of the abnormality (in relation to the location of PVS) were also evaluated. Extravascular thoracic MDCT angiography findings of pediatric patients with and without primary PVS were compared. Interobserver agreement between the 2 independent reviewers was evaluated with κ statistics. Results:The study group consisted of 15 thoracic MDCT angiography studies from 15 individual pediatric patients with primary PVS (8 males [53%] and 7 females [47%]; mean age: 10.9 mo; SD: 11.7 mo; range: 1 to 48 mo). The comparison group consisted of 15 thoracic MDCT angiography studies from 15 individual pediatric patients without PVS (8 males [53%] and 7 females [47%]; mean age: 10.2 mo; SD: 11.5 mo; range: 1 to 48 mo). In children with primary PVS, the characteristic extravascular thoracic MDCT angiography findings were GGO (14/15; 93%), septal thickening (5/15; 33%), pleural thickening (14/15; 93%), and ill-defined, mildly heterogeneously enhancing, noncalcified soft tissue mass (14/15; 93%) following the contours of PVS in the mediastinum. There was excellent interobserver κ agreement between 2 independent reviewers for detecting extravascular abnormalities on thoracic MDCT angiography studies (κ = 0.99 for the study group and κ = 0.98 for the comparison group).Conclusions: Children with primary PVS have characteristic extravascular thoracic MDCT angiography findings. In the lungs and pleura, GGO, septal thickening, and pleural thickening are common findings. Importantly, in the mediastinum, the presence of a mildly heterogeneously enhancing, noncalcified soft tissue mass in the distribution of PVS is a novel characteristic thoracic MDCT angiography finding unique to pediatric primary PVS. When this constellation of extravascular thoracic MDCT angiography findings is detected, although rare, primary PVS should be considered as a possible underlying dia...

Purpose: To investigate the characteristic thoracic multidetector computed tomography (MDCT) findings of pathologically proven combined congenital lung lesion consisting of bronchial atresia (BA) and congenital pulmonary airway malformation (CPAM) in children.Materials and Methods: All pediatric patients (age ≤ 18 years) with a known pathological diagnosis of a combined BA-CPAM congenital lung lesion, who underwent thoracic MDCT studies from January 2011 to January 2021 were included. Two pediatric radiologists independently evaluated thoracic MDCT studies for the presence of abnormalities in the lung, including nodule, mass, cyst, ground-glass opacity, and consolidation. When a lung abnormality was present, the number, size, composition (solid, cystic, or combination of both), borders (well-circumscribed vs. illdefined), contrast enhancement pattern (nonenhancement vs. enhancement), and location (laterality, and lobar distribution) were also evaluated. Interobserver agreement between two independent reviewers was evaluated with κ statistics.Results: Eighteen contrast-enhanced thoracic MDCT studies from 18 individual pediatric patients (8 males (44%) and 10 females (56%); mean age: 4.9 months; SD: 2.6; range: 1-10 months) with a pathological diagnosis of combined BA-CPAM congenital lung lesion comprised the final study population. The most frequent MDCT finding of combined BA-CPAM congenital lung lesion in children was a solitary (18/18; 100%), well-circumscribed (18/18; 100%), both solid and cystic (17/ 18; 94%) lesion with nonenhancing (17/17; 100%) nodule, reflecting the underlying BA component, adjacent to a well-circumscribed multicystic mass (18/18; 100%), representing the underlying CPAM component. This combined congenital lung lesion occurred in all lobes with similar frequency. There was almost perfect interobserver κ agreement between the two independent reviewers for detecting abnormalities on thoracic MDCT studies (k = 0.98). Conclusion:The characteristic thoracic MDCT findings of a combined BA-CPAM congenital lung lesion are a solitary, well-circumscribed solid and multicystic mass, with a nonenhancing nodule, reflecting the BA component, adjacent to a cystic mass,

To retrospectively investigate the extravascular thoracic MDCT angiography findings of pulmonary vein stenosis (PVS) in children with a cardiac septal defect. Materials and Methods: Pediatric patients (age ≤ 18 years) with cardiac septal defect and PVS, confirmed by echocardiogram and/or conventional angiography, who underwent thoracic MDCT angiography studies from April 2009 to April 2021 were included. Two pediatric radiologists independently evaluated thoracic MDCT angiography studies for the presence of extravascular thoracic abnormalities in: (1) lung and airway (ground-glass opacity (GGO), consolidation, pulmonary nodule, mass, cyst, septal thickening, fibrosis, and bronchiectasis); (2) pleura (pleural thickening, pleural effusion, and pneumothorax); and (3) mediastinum (mass and lymphadenopathy). Interobserver agreement between the two independent pediatric radiology reviewers was evaluated with kappa statistics. Results: The final study group consisted of 20 thoracic MDCT angiography studies from 20 consecutive individual pediatric patients (13 males (65%) and 7 females (35%); mean age: 7.5 months; SD: 12.7; range: 2 days to 7 months) with cardiac septal defect and PVS. The characteristic extravascular thoracic MDCT angiography findings were GGO (18/20; 90%), septal thickening (9/20; 45%), pleural thickening (16/20; 80%), and ill-defined, mildly heterogeneously enhancing, non-calcified soft tissue mass (9/20; 45%) following the contours of PVS in the mediastinum. There was a high interobserver kappa agreement between two independent reviewers for detecting extravascular abnormalities on thoracic MDCT angiography studies (k = 0.99). Conclusion: PVS in children with a cardiac septal defect has a characteristic extravascular thoracic MDCT angiography finding. In the lungs and pleura, GGO, septal thickening, and pleural thickening are frequently seen in children with cardiac septal defect and PVS. In the mediastinum, a mildly heterogeneously enhancing, non-calcified soft tissue mass in the distribution of PVS in the mediastinum is seen in close to half of the pediatric patients with cardiac septal defect and PVS.