Congenital cystic adenomatoid malformation of lung

Congenital Cystic Adenomatoid Malformation of the Lung
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Synonyms	Congenital Pulmonary Airway Malformation (CPAM)
Pronounce	N/A
Specialty	N/A
Symptoms	Respiratory distress, recurrent infections
Complications	Pulmonary hypoplasia, infection
Onset	Prenatal
Duration	Lifelong
Types	N/A
Causes	Developmental anomaly
Risks
Diagnosis	Ultrasound, CT scan, MRI
Differential diagnosis	N/A
Prevention	N/A
Treatment	Surgical resection
Medication	N/A
Prognosis	Generally good with treatment
Frequency	Rare
Deaths	N/A

Congenital Cystic Adenomatoid Malformation of the Lung (CCAM), also known as Congenital Pulmonary Airway Malformation (CPAM), is a rare developmental anomaly of the lung characterized by the presence of cystic masses in the lung tissue. These masses are due to an abnormality in the development of the bronchial tree during fetal growth.

Classification

CCAM is classified into five types based on the Stocker classification system:

• Type 0: Acinar dysplasia, incompatible with life.
• Type 1: Large cysts (2-10 cm), most common type.
• Type 2: Multiple small cysts (<2 cm), associated with other congenital anomalies.
• Type 3: Solid mass, microcystic, often causing mediastinal shift.
• Type 4: Large cysts, similar to type 1 but with different histology.

Pathophysiology

CCAM results from an abnormality in the branching morphogenesis of the lung during fetal development. This leads to the formation of cystic and adenomatous structures instead of normal alveoli. The exact cause of this developmental anomaly is not well understood, but it is believed to occur during the pseudoglandular stage of lung development.

Clinical Presentation

The clinical presentation of CCAM can vary depending on the size and location of the cystic lesions. Some infants may present with respiratory distress at birth, while others may remain asymptomatic and be diagnosed incidentally during imaging for other reasons. Recurrent respiratory infections are common in symptomatic cases.

Diagnosis

CCAM is often diagnosed prenatally through ultrasound imaging, which can reveal cystic lung lesions. Postnatal diagnosis may involve chest X-ray, CT scan, or MRI to assess the extent and nature of the malformation.

Treatment

The primary treatment for CCAM is surgical resection of the affected lung tissue. This is typically performed in symptomatic cases or when there is a risk of complications such as infection or malignancy. In asymptomatic cases, the decision to operate may depend on the size and growth of the lesion.

Prognosis

The prognosis for infants with CCAM is generally good, especially when the condition is diagnosed early and treated appropriately. Surgical resection usually results in excellent outcomes, with most children leading normal lives post-surgery.

Also see

• Congenital diaphragmatic hernia
• Bronchopulmonary sequestration
• Pulmonary hypoplasia
• Lung development