Structural analysis of pulmonary vascular changes in a case of infantile calcifying arteriopathy

In a well documented case of ACI, the pulmonary vessels were subjected to light microscopy, radiography, electron microscopy and X-ray microanalysis. The analysis of calcified foci in the walls of large and medium pulmonary arteries demonstrated a marked correlation between the deposits of calcium and phosphorus and the number of elastic fibers. The interpretation of our observations is in favour of a congenital defect of elastic fiber formation in the vascular walls, followed by calcification. Massive accumulation of calcium in the vascular walls was associated with tissue destruction, fragmentation of fibers and formation of irregular calcified foci which provoked a foreign-body reaction.