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The mystery of CPAM: What is this rare lung malformation and why is it so fascinating?
Congenital pulmonary airway malformation (CPAM), formerly known as congenital cystic adenomatous malformation (CCAM), is a congenital disorder of the lung that resembles bronchopulmonary sequestration. In CPAM, often an entire lobe of the lung is replaced by a cystic mass of abnormal lung tissue that no longer functions properly. This abnormal tissue can never function as a normal lung. The cause of CPAM is still unknown, so this condition causes certain troubles for both pregnant women and medical staff.
According to statistics, CPAM occurs in approximately 1 in every 30,000 pregnancies.
While the prognosis for most fetuses with CPAM is good, in some cases the cystic mass grows too large and may restrict the growth of the surrounding lungs or even affect the heart, posing a life-threatening risk. This makes CPAM one of the research hotspots. Based on clinical and pathological characteristics, CPAM can be divided into five types, among which CPAM type 1 is the most common and has a relatively better prognosis.
Symptoms and Signs
About 75% of patients have no obvious symptoms, but 25% may experience cyanosis, pneumothorax, and difficulty breathing. On examination, they may demonstrate hyperresonance on percussion, decreased alveolar breath sounds, and chest asymmetry.
Diagnostic Process
CPAM is usually discovered during routine prenatal ultrasound examinations. Ultrasound features include an echogenic (bright) mass in the fetal chest, a displaced heart, a flat or everted diaphragm, or an absence of visible lung tissue. There are three types of CPAM, classified primarily based on their appearance. Type 1 has multilocular cysts larger than 2 cm, type 2 has smaller uniform cysts, and type 3 is not obviously cystic and is called "adenomatous".
CPAM grows faster during the last few weeks of pregnancy, especially between weeks 20 and 26.
Imaging Examination and Risk Assessment
CPAM can be detected early in pregnancy through prenatal ultrasound. The data showed that as the cyst filled with fluid, the mass gradually disappeared during subsequent ultrasound examinations. Rapidly growing CPAM, whether solid or predominantly cystic, carries a higher risk of venous obstruction, heart failure, and ultimately fetal hydrops.
CVR value (cystic adenoma volume ratio) is used to predict the risk of edema, and a CVR value higher than 1.6 is considered high risk.
Treatment methods
In most cases, CPAM in a fetus requires close monitoring during pregnancy and surgical removal after birth. Many people with CPAM are born without complications and are usually monitored during the first few months of life. Most patients undergo surgery before the age of one year because they are at risk for recurrent lung infections associated with CPAM.
Prognosis and complications
According to a 2023 review, the overall prognosis for prenatally diagnosed CPAM is good. However, if hydrops fetalis is present, the survival rate decreases. If edema does not develop by 26 weeks of gestation, the risk is reduced and the survival rate is improved.
While most people with CPAM live healthy lives, long-standing CPAM may be at risk for developing cancer and presents the possibility of infection and other complications. Future research will help to better understand the causes and effects of CPAM so that better medical interventions can be provided to patients.
When facing the congenital disease CPAM, we should think about: How can we more effectively provide early support and medical resources to fetuses that are diagnosed and need treatment?
