Pulmonary veno-occlusive disease of presumably intrauterine origin

Abstract

A case is presented of a male infant aged 8 weeks at the time of his death with pulmonary veno-occlusive disease. Almost all pulmonary veins and venules were narrowed or obstructed by intimal fibrosis with such a pronounced deposition of collagen and elastic tissue that an intrauterine origin of these lesions appeared likely. Bronchial veins and anastomoses between pulmonary and bronchial veins were also affected.

The vascular changes were of a thrombotic nature. The presence of subacute myocarditis and chronic interstitial pneumonia suggested that an infection may have caused these alterations. An upper respiratory infection in the mother during her pregnancy may be significant, but several serological titres in the maternal blood were too low to give a clue to the nature of the infection.