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Joseph Junewick, MD FACR
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Congenital Pulmonary Airway Malformation

Case Detail

Anatomy: Chest
Junewick
Joseph Junewick, MD FACR
Diagnostic Category: Neoplasia Benign
Created: over 6 years ago
Updated: over 6 years ago
Tags: PEDS
Modality/Study Types: US MR CT
Activities:
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History

“Suprarenal” mass on prenatal ultrasound.


Case Images


Diagnosis

Congenital Pulmonary Airway Malformation

Clinical Notes

Pathology – Congenital pulmonary airway malformation, adenomatoid type.

Findings

Prenatal MR – T2 hyperintense lesion bounded inferiorly by the diaphragm.

US – Immediate postnatal scan demonstrates an echogenic intrathoracic mass with few small cysts; systemic arterial supply demonstrated on Doppler.

CT – Examination performed at 6 months of age shows the hypodense left lower lobe lesion to be slightly smaller.

Discussion

Congenital pulmonary airway malformation, formerly referred to as congenital cystic adenomatoid malformation, is classified by cyst size and histologic resemblance to segments of the developing bronchial tree and airspaces. There are 5 types: Type 0 has a tracheal or bronchial origin and is really acinar dysgenesis or dysplasia, Type 1 has a bronchial or bronchiolar origin and has large cysts, Type 2 has a bronchiolar origin and small cysts, Type 3 has a bronchiolar–alveolar duct origin (adenomatoid type), and Type 4 has a distal acinar origin (the “unlined” cyst lesion). A CPAM may communicate with the proximal airways, although this communication is abnormal. Most CPAMs derive their blood supply from the pulmonary artery and drain via the pulmonary veins; systemic arterial supply can be seen in macrocystic CPAMs, especially small cyst CPAMs (these lesions are often referred to as hybrid lesions since they demonstrate histologic and imaging features of both a CPAM and bronchopulmonary sequestration). A fast-growing CPAM may cause mediastinal shift and subsequent development of polyhydramnios and hydrops.

Reference

Biyyam DR, Chapman T, Ferguson MR, et al. Congenital Lung Abnormalities: Embryologic Features, Prenatal Diagnosis, and Postnatal Radiologic-Pathologic Correlation. RadioGraphics (2010); 30: 1721-1738.



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