Congenital unilobar pulmonary lymphangiectasis

: A left upper lobectomy was performed on a 27-day-old male infant with clinically suspected congenital lobar emphysema. At operation a cranial pericardial hiatus was also noted. The infant died ten days postoperatively of a septicaemia of extrapulmonary origin. An autopsy was not performed. Histologic examination of the left upper lobe revealed the changes to be pulmonary lymphangiectasis, which is only rarely unilateral or unilobar. In this infant alveolar rupture is thought to have provided air entry into the primarily ectatic lymphatic vessels with further spread of air. The clinical symptoms were due to this complication. This interpretation is supported by the finding of numerous foreign-body giant cells in the lymph vessels, an extraordinary finding in congenital pulmonary lymphangiectasis.