Prenatal period to adolescence: the variable presentations of congenital cystic adenomatoid malformation.

BACKGROUND

Congenital cystic adenomatoid malformation (CCAM) of the lung is a rare but potentially life-threatening pulmonary anomaly.

METHODS

Seven patients operated on with the diagnosis of CCAM were reviewed to determine the clinical presentation, age at diagnosis, histopathologic types and the postoperative course.

RESULTS

Cough and respiratory distress were common clinical findings in the patients in the present study. Two of the patients had congenital respiratory distress. The patient who was diagnosed at 15 years had cough, hemoptysis and anorexia for the last 4 months. One patient who received the diagnosis of CCAM prenatally was asymptomatic until she was operated on, on the 45th postnatal day. All patients but one were operated on and histopathological diagnosis was made for each of them between the ages of 4 days to 12 months. Four patients were diagnosed as having CCAM type I, and three patients had CCAM type II histopathologically. Duration of postoperative follow up was between 3 months and 15 years. Neither patient had complaints during that period.

CONCLUSION

CCAM can present at different ages and in various clinical presentations. There can even be asymptomatic patients who receive their diagnosis during the prenatal period. The possibility of an underlying CCAM should be considered in infants with recurrent chest infections or persistent abnormalities on chest X-ray following an acute infection. CCAM should also be considered in cases with lung abscess, even in adolescents with no past history of pulmonary complaints.