Congenital cystic adenomatoid malformation(CCAM): Symptoms,Diagnosis & Treatments
Congenital cystic adenomatoid malformation (CCAM) is the most common congenital lung disease and occurs sporadically regardless of race, age, and other factors. There are no known genetic polymorphisms and are known to cause 1-4 out of 100,000 people.
It is a hamartomatous lesion composed of cystic and adenomatous tissue. Unlike pulmonary sequestration, blood is supplied through the pulmonary circulation and mainly affects one lobe of the lung. Because it is abnormally proliferating, not only the lung at the affected site but also the lung at the opposite site is also depressed, making it difficult to function.
Diagnosis
Among 5 types, the most common type is type 1 (60-70%). Type 1 CCAM exists as a 2 to 10 cm thin walled sac, not multiple. In addition, Type 2 CCAM is associated with congenital diseases of various organs such as esophageal atresia, tracheoesophageal fistula, and renal agenesis, and Type 4 CCAM is capable of malignant transformation.
About 75% of children with preeclampsia diagnosed with CCAM have no symptoms. Symptoms appear as respiratory symptoms such as respiratory depression, puffiness, etc.
Diagnosis is made mainly through radiologic methods. Basically, the location and size of the lesion are confirmed by Chest X-ray. Ultrasonography is used to distinguish it from prenatal diagnosis and pulmoanry sequestration using color doppler. CT or MRI can more accurately identify the shape and location of the lesion and distinguish it from other congenital lung diseases. However, in the case of CT, radiation exposure is severe in children and MRI is limited in use because of low compliance.
Treatments
Surgical resection is the fundamental treatment. Complete resection can be expected through surgical resection if symptoms are present, and steady progression is observed if there is no symptom. If there is a risk factor such as the following, surgical treatment may be considered.
<High risk group (asymptomatic)>
- large lesiohn (occupying> 20% of the involved lobe)
- bilateral or muultifocal cysts
- pneumothorax
- family history of pleuropulmonary blastoma
Pulmonary sequestration is the most common disease to be diagnosed. This disease differs from CCAM in that it is supplied with blood through the systemic circulation and is not connected to lung bronchial tissues.
It is a hamartomatous lesion composed of cystic and adenomatous tissue. Unlike pulmonary sequestration, blood is supplied through the pulmonary circulation and mainly affects one lobe of the lung. Because it is abnormally proliferating, not only the lung at the affected site but also the lung at the opposite site is also depressed, making it difficult to function.
Diagnosis
Among 5 types, the most common type is type 1 (60-70%). Type 1 CCAM exists as a 2 to 10 cm thin walled sac, not multiple. In addition, Type 2 CCAM is associated with congenital diseases of various organs such as esophageal atresia, tracheoesophageal fistula, and renal agenesis, and Type 4 CCAM is capable of malignant transformation.
About 75% of children with preeclampsia diagnosed with CCAM have no symptoms. Symptoms appear as respiratory symptoms such as respiratory depression, puffiness, etc.
Diagnosis is made mainly through radiologic methods. Basically, the location and size of the lesion are confirmed by Chest X-ray. Ultrasonography is used to distinguish it from prenatal diagnosis and pulmoanry sequestration using color doppler. CT or MRI can more accurately identify the shape and location of the lesion and distinguish it from other congenital lung diseases. However, in the case of CT, radiation exposure is severe in children and MRI is limited in use because of low compliance.
Treatments
Surgical resection is the fundamental treatment. Complete resection can be expected through surgical resection if symptoms are present, and steady progression is observed if there is no symptom. If there is a risk factor such as the following, surgical treatment may be considered.
<High risk group (asymptomatic)>
- large lesiohn (occupying> 20% of the involved lobe)
- bilateral or muultifocal cysts
- pneumothorax
- family history of pleuropulmonary blastoma
Pulmonary sequestration is the most common disease to be diagnosed. This disease differs from CCAM in that it is supplied with blood through the systemic circulation and is not connected to lung bronchial tissues.
