[Unilateral congenital pulmonary lymphangiectasis]

The authors report the anatomo-clinical description of a case of a complex malformation of the Ivemark type with asplenism. They draw attention to the presence of a left-sided unilateral congenital pulmonary lymphangectasia due to the absence of the left pulmonary venous trunk. This observation confirms the role of total blockage of pulmonary venous circulation in the aetiology of these very rare cases of congenital pulmonary lymphangectasia.