Abnormal arterial vascular development/remodeling in congenital lung lesions. In contrast to a healthy arteriole (A–D), hypertrophied and often tortuous arteries could be seen in nearly all BPS and hybrid lesions, as well as a subset of macrocystic lesions (M). (E–L) These arteries exhibited variable degrees of hypertrophy of the vascular smooth muscle (TAGLN+), and outer layer (tunica adventitia) consisting of VIM+ fibroblasts invested in a thick layer of extracellular matrix. Percentages in M refer to N = 58 patients. Ar = artery; BPS = bronchopulmonary sequestration; H&E = hematoxylin and eosin; Ta = tunica adventitia; TAGLN = transgelin; Ti = tunica intima; Tm = tunica media; VIM = vimentin. Scale bars: (A, E, and I) 500 μm; all others, 100 μm.