page_title: Congenital pulmonary airway malformation
section_title: Diagnosis
hierarchical_section_title: Congenital pulmonary airway malformation / Diagnosis
caption_attribution_description: Deutsch: Zystisch adenomatoide Malformation (CCAM) bei einem Neugeborenem in der Röntgenaufnahme des Thorax. Man erkennt die großen zystischen Veränderungen der linken Lunge, die durch ihre raumfordernde Wirkung zu einem Mediastinalshift nach rechts geführt haben. English: CCAM on chest radiograph in a newborn. Large cystic changes in the left lung, leading to a mediastinal shift to the right due to their mass effect.
context_page_description: Congenital pulmonary airway malformation, formerly known as congenital cystic adenomatoid malformation, is a congenital disorder of the lung similar to bronchopulmonary sequestration. In CPAM, usually an entire lobe of lung is replaced by a non-working cystic piece of abnormal lung tissue. This abnormal tissue will never function as normal lung tissue. The underlying cause for CPAM is unknown. It occurs in approximately 1 in every 30,000 pregnancies.
  In most cases the outcome of a fetus with CPAM is very good. In rare cases, the cystic mass grows so large as to limit the growth of the surrounding lung and cause pressure against the heart. In these situations, the CPAM can be life-threatening for the fetus. CPAM can be separated into five types, based on clinical and pathologic features. CPAM type 1 is the most common, with large cysts and a good prognosis. CPAM type 2 often has a poor prognosis, owing to its frequent association with other significant anomalies. Other types are rare.
context_section_description: CPAMs are often identified during routine prenatal ultrasonography. Identifying characteristics on the sonogram include: an echogenic (bright) mass appearing in the chest of the fetus, displacement of the heart from its normal position, a flat or everted (pushed downward) diaphragm, or the absence of visible lung tissue.
  CPAMs are classified into three different types based largely on their gross appearance.  Type I has a large (>2 cm) multiloculated cysts.  Type II has smaller uniform cysts.  Type III is not grossly cystic, referred to as the "adenomatoid" type.  Microscopically, the lesions are not true cysts, but communicate with the surrounding parenchyma. Some lesions have an abnormal connection to a blood vessel from an aorta and are referred to as "hybrid lesions."
caption: CPAM on chest radiograph in a newborn. Large cystic changes in the left lung, leading to a mediastinal shift to the right due to their mass effect.
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