Congenital Pulmonary Cystic Lymphangiectasis

CONGENITAL pulmonary cystic lymphangiectasis (CPCL) is an uncommon disease, the first case of which was reported by Virchow¹over 100 years ago. Laurence²has suggested that this condition is not rare but rather is infrequently recognized by both the clinician and pathologist. Many of the features of this entity mimic those of the Wilson-Mikity syndrome, another uncommon type of respiratory distress of the newborn. The following is a case report of congenital pulmonary cystic lymphangiectasis, with a review of 32 cases and a discussion of the clinical and pathologic features of these two entities. Report of a Case A 4.9 kg (11 lb) white girl was born after a 40-week uncomplicated gestation. The child was cyanotic at birth, but there was no immediate respiratory distress. Cyanosis progressed despite intubation and oxygen therapy. Respiratory distress and signs of congestive heart failure developed shortly thereafter and persisted until death, 40