Resolving hypoplastic dilemma: Lung perfusion scintigraphy in a case of congenital cystic adenomatoid malformation (CCAM)

ABSTRACT

Congenital cystic adenomatoid malformation is a rare bronchopulmonary malformation resulting from an arrest in lung development between 4th and 7th week of fetal life. It typically presents as respiratory distress in the neonatal period and recurrent pulmonary infections in adults; and is sometimes associated with other congenital abnormalities [especially CCAM Type II]. Chest roentgenograms and CT scan are the usual modalities of choice in its initial evaluation and histopathology done post-operatively establishes the diagnosis. Ventilation-Perfusion [VQ] scintigraphy is of paramount importance in the routine assessment of CCAM patients but is often disregarded. This paper presents the incremental value of doing a lung perfusion scintigraphy and aims to emphasize the significance of performing a VQ scan in such cases.