Congenital cystic adenomatoid malformation

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[edit] Discussion of Congenital cystic adenomatoid malformation

  • Congenital cystic adenomatoid malformation (CCAM) of the lung is an uncommon cause of respiratory distress in neonates and infants.
  • CCAM is characterized by a multicystic mass of pulmonary tissue with an abnormal proliferation of bronchial structures.
  • CCAM is classified into three histologic types.
    • Type I is composed of variable-size cysts, with at least one dominant cyst (>2 cm in diameter). This is the most common (75%) form.
    • Type II congenital cystic adenomatoid malformation is composed of smaller, more uniform cysts less than 1 cm in diameter (10% to 15% of all congenital cystic adenomatoid malformations).
    • Type III congenital cystic adenomatoid malformation is a solid mass composed of bronchoalveolar microcysts.

  • Among infants in whom the malformation is diagnosed postnatally, one-half to two-thirds will have some form of respiratory distress or compromise.
  • The presentation in older patients is usually recurrent pulmonary infections.
  • CCAM usually involves a single lobe, multilobar involvement being infrequent.

[edit] Imaging Findings for Congenital cystic adenomatoid malformation

  • The postnatal diagnosis is made with plain chest radiography, which demonstrates multiple air-filled thin-walled cysts that vary in size.
  • Unlike in diaphragmatic hernia, the distribution of abdominal bowel gas is normal.
  • CT can be useful for characterizing congenital cystic adenomatoid malformation by showing its location and extent, and for differentiating it from congenital lobar emphysema and bronchogenic cysts.

[edit] Images

Patient #1

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[edit] References for Congenital cystic adenomatoid malformation