Congenital cystic adenomatoid malformation: the experience of a level III Neonatal Intensive Care Unit

Nuno Medeiros, Gustavo Rocha, Tiago Henriques-Coelho, Carla Ramalho, Hercília Guimarães

Abstract


Background: Congenital cystic adenomatoid malformation (CCAM) is a rare disease. Because few cases per center are reported each year, there is still uncertainty concerning pathophysiology, natural history of the disease and the best treatment approach.

The aim of this paper is to report the experience of a level III Neonatal Intensive Care Unit (NICU) in CCAM cases.

Methods: We evaluated all cases diagnosed with CCAM admitted to our hospital between January 2000 and December 2014.

Results: Twenty-four neonates with prenatal diagnosis of CCAM were identified. The majority of lesions (75%) had microcystic features. Thirteen newborns were admitted to NICU for observation and evaluation. Half of the prenatally diagnosed lesions were confirmed cases of CCAM. The remaining corresponded to 5 cases (20.8%) of in utero spontaneous regression of the lesion, 5 (20.8%) bronchopulmonary sequestration, 1 (4.2%) bronchial atresia and 1 (4.2%) pleuro-pericardial cyst. Two (8.3%) neonates became symptomatic during the neonatal period and surgery was performed in the first week of life. Ten cases remained asymptomatic. Six of these cases (40%) underwent surgery (thoracotomy in 3 cases and thoracoscopy in 3 cases). Respiratory morbidity was reported in 1 symptomatic case and in 1 asymptomatic case that was managed conservatively. No mortality was reported. Histological examination showed definitive features of CCAM (Stocker classification: type I = 1; type II = 4; type III = 2).

Conclusion: Management of CCAM in a NICU does not seem necessary in asymptomatic newborns. Concordance of ante-natal and post-natal findings is very variable. Thoracoscopy is a less invasive surgical procedure in the management of these patients. The outcome is good in most patients.


Keywords


congenital cystic adenomatoid malformation of lung; neonatal intensive care unit; thoracoscopy

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