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Background: Congenital cystic adenomatoid malformation of the lungs (CCAM) is a rare congenital malformation of the respiratory tract. Authors present possibilities of the diagnostics of neonates with presumed CCAM based on radiological and morphological assessment of the respiratory tract. Clinical course of the CCAM may vary from uneventful to serious with different stage of the respiratory distress. The aim of the study was to establish diagnostic and clinical criteria of CCAM for neonates based on postnatal diagnostics in the reference centre. Material/Methods: We studied 27 cases of neonates with different types of CCAM (type I, II, III) who had been diagnosed and/or treated in the Neonatal Department of PMMHRI in 01.01.1991-31.03.2005. The diagnosis was established based on clinical course of the malformation, chest x-ray and CT of the neonate's lungs, autopsy and/or histopathology. Results: In the Neonatal Department of PMMHRI in 01.01.1991-31.03.2005 we observed 8 cases of CCAM type I (29,7%), 10 cases of CCAM type II (37%) and 9 cases of CCAM type III (33,3%). Diagnostic criteria were established based on radiological diagnostics of 17 cases and were confirmed by pathology. In 10 remaining cases of CCAM diagnosis was established by authopsy. Differential diagnosis included diaphragmatic hernia, bronchogenic cyst, enterogenic cyst, lung sequestrqtion, congenital lobar emphysema, hypoplasia or agenesis of the lungs, pneumonia/RDS. The guideline was established. Conclusions: 1. In case of CCAM suspicion monitoring in reference center is required 2. Surgical treatment should be applied based on the postnatal radiological diagnosis. 3. In case of CCAM in neonate complete differential diagnosis is required. 4. Asymptomatic course of CCAM or with a very few signs from respiratory tract can be difficult for identification both clinical and radiological.