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Fetopathology and developmental pathology of the embryo and fetus
Marta Ježová, Josef Feit et al.
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Congenital cystic adenomatoid malformation (CCAM)
Etiology

Unclear, perhaps abnormal blood supply or abnormal proliferation of bronchioli.

Clinical signs
  • more common in males
  • clinical presentation:
    • prenatally often hydrops of the fetus and intrauterine death
    • respiratory distress syndrome perinatally
    • usually one lobe is affected
    • the finding may mimic congenital lobar emphysema
  • 20% of affected (usualy CCAM of type 2) have other congenital malformations, e.g. agenesis of the kindeys

Cystic adenomatoid malformation (72698)

Cystic adenomatoid malformation (72699)

Pictures

Cystic adenomatoid malformation of the right lung, newborn: Cystic adenomatoid malformation of the lung, Macro, autopsy (72050)

Another case, CCAM, lower lobe of the right lung, 21-week fetus, compared with normal lung: CCAM, lower left lobe, fetus 21th week, Macro, autopsy (72502) CCAM, lower left lobe, fetus 21th week, Macro, autopsy (72503)

Histology

Cystic malformation of the pulmonary tissue; according to the size and histological structure of the wall and lining of the cysts (thickness, presence of the cartilage and striated muscle, mucin producing cells) 3 types of CCAM can be recognized: CCAM I – III.

Case study
Congenital cystic adenomatoid malformation of lung
Marta Ježová
History

Abortus imminens in 1st trimester. Ultrasound examination in 20 w.g. reveals bilateral macrocystic malformation of lungs with hydrops, ascites.

Macroscopic appearance
  • 21-week fetus
  • congenital cystic adenomatoid malformation of inferior and middle lobe of the right lung
  • mediastinal shift
  • hypoplastic left lung
  • ascites
Pictures

Hydrops, large abdomen (ascites): Hydrops, large abdomen (ascites), Macro, autopsy (72696)

Cystic malformation of the right lung: Cystic malformation of right lung, Macro, autopsy (72697)

Cystic malformation of two lobes of the right lung, detail: Cystic malformation of right lung, Macro, autopsy (72698)

Cystic malformation of the right lung, cross section: Cystic malformation of right lung, section, Macro, autopsy (72699)

CCAM, histology: Congenital cystic adenomatoid malformation of the lung (CCAM), HE 40x (72615)

CCAM, US video: Cystic malformation of right lung, Ultrasound, video (72730)