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Congenital cystic adenomatoid lung malformation (CCAM) is a rare disorder characterized by replacement of normal pulmonary tissue with cysts of variable size and distribution. Only sporadic cases of CCAM type III have been described that were stillborn or died in the early neonatal period. To our best knowledge we report the first infant with bilateral CCAM type III that was not ventilator-dependent for the first 10 weeks of life and survived until the age of 5 months. Postnatally the newborn presented with signs of only mild respiratory distress and chest radiograph did not show evidence of cystic lesions. In the following weeks the major clinical symptom was persistent pulmonary hypertension with severe tachypnea, O 2  requirement and CO 2  retention. Pulmonary hypertension did not respond to HFOV ventilation, NO ventilation or prostacyclin inhalations.

Bilateral cystic adenomatoid lung malformation type III – a rare differential diagnosis of pulmonary hypertension in neonates