Fact-sheet: Cystic adenomatoid malformation
  • Congenital cystic adenomatoid malformation


Updated on 06/09/2023 at 11:15 AM

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Definition

Cystic adenomatoid malformation (CPAM), formerly known as congenital pulmonary adenomatoid cystic malformation

Cyst = air-containing cavity surrounded by a wall without central vascularity

-25% of lung malformations

-Male predominance

-Predominantly involves the lower lobes

-Most often unilateral and unilobar

Clinical features

Prenatal diagnosis: on the routine second-trimester ultrasound.

Postnatal diagnosis: variable clinical presentation, ranging from absence of symptoms to acute respiratory distress.

Radiography

In type I and II CPAM, hyperlucency and multicystic lesion of the affected lung field.

Possible mediastinal shift and diaphragmatic displacement in the case of a large lesion.

In the early neonatal period, the lesion may appear as fluid-filled opacities or show an air-fluid level. Type III lesions may be fluid-filled or present as consolidation.

CT

Destruction of healthy lung parenchyma, replaced by cysts of variable appearance depending on CPAM type.

Type III CPAM may present as consolidation.

Management

Early surgical management: lobectomy or segmentectomy

Spontaneous course: superinfection-degeneration-regression (prenatal)-hydrops (postnatal)

Classification

Classification:

  • Type I=50-70%: macrocystic, 1 or several thin-walled cysts >2 cm, which may be surrounded by smaller cysts
  • Type II=10-40%: more numerous cysts <2 cm. Possible association with other anomalies: renal agenesis or renal dysgenesis, pulmonary sequestration, congenital heart malformations
  • Type III=10%: microcystic = solid-appearing structure composed of microcysts <0.5 cm
  • Type IV= cyst without a wall, indistinguishable from type I on imaging
  • Type 0= very rare, lethal postnatally = acinar dysgenesis or dysplasia

Differential diagnosis

  • Pulmonary sequestration: malformation sometimes associated with anomalous systemic arterial supply to the lesion.
  • Congenital diaphragmatic hernia
  • Bronchogenic cyst
  • Congenital lobar overinflation
  • Congenital cystic bronchiectasis
  • Thoracic teratoma
  • Thoracic cystic lymphangioma
  • Pleuropericardial cyst