Congenital Cystic Adenomatoid Malformation
Also written as: CCAM — Congenital Cystic Adenomatoid Malformation
Congenital lung malformation characterized by abnormal development of terminal respiratory structures, now more commonly called CPAM.
Full Definition
Congenital Cystic Adenomatoid Malformation (CCAM), now preferentially termed Congenital Pulmonary Airway Malformation (CPAM), is a developmental anomaly affecting the lower respiratory tract. The condition involves hamartomatous overgrowth of terminal respiratory structures with formation of intercommunicating cysts that do not function in gas exchange. CCAM is classified into five types based on cyst size and histology, with Type I (large cysts >2cm) being most common. Presentation ranges from severe neonatal respiratory distress to asymptomatic lesions discovered incidentally. Surgical resection is typically recommended due to infection risk and rare malignant potential. Many cases are now detected prenatally on ultrasound.
Usage
Usage note: CPAM is the preferred current terminology over CCAM.
In Context
- "Prenatal ultrasound detected a large CCAM in the right lower lobe requiring immediate postnatal evaluation." — Prenatal consultation note
- "Lobectomy was performed for the symptomatic congenital cystic adenomatoid malformation." — Operative report