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Case 26 

Case 26
Chapter:
Case 26
Author(s):

Sanjeev Bhalla

, Cylen Javidan-Nejad

, Kristopher W. Cummings

, and Andrew J. Bierhals

DOI:
10.1093/med/9780195394535.003.0026
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Diagnosis

Case 26: Congenital Cystic Adenomatoid Malformation

Findings

  • CT demonstrates localized conglomeration of medium-sized and large cysts (arrows in Figs. 26.526.7) in the apical and posterior segments of the right upper lobe without adjacent airspace disease or pleural effusion.

Differential Diagnosis

  • Based on the CT features, the differential consists of cystic adenomatoid malformation, cystic intralobar sequestration, or postinfectious pneumatoceles.

Teaching Points

  • Cystic adenomatoid malformation is a congenital lesion with abnormal bronchiolar proliferation. The “cysts” are actually massively dilated bronchioles.

  • The Stocker classification system divides these lesions into type I (large cysts >2 cm), type II (multiple small cysts <2 cm), and type III (solid, microcystic lesions). Type II and III lesions have been associated with a poor prognosis, probably from their frequent association with other congenital anomalies. Type I lesions do have a low likelihood of developing malignancies, such as bronchoalveolar carcinoma.

  • The majority of lesions are detected in childhood as masses on chest radiographs in infants with respiratory distress. Initially, the lesion may appear solid, but over the first 24 to 48 hours of life fluid clears from the lesion, leaving cystic airspaces. In older children or adults, these lesions can be encountered incidentally or come to attention due to mass effect or superinfection.

Management

  • In children, these lesions are routinely resected. In adults, lesions are resected if symptomatic, but do not necessarily require radiographic follow-up if asymptomatic.

Further Reading

Bush A. Congenital lung disease: a plea for clear thinking and clear nomenclature. Pediatr Pulmonol. 2001 Oct;32(4):328-337.Find this resource:

Epelman M, Kreiger PA, Servaes S, et al. Current imaging of prenatally diagnosed congenital lung lesions. Semin Ultrasound CT MR. 2010 Apr;31(2):141-157.Find this resource: