Introduction
Congenital pulmonary airway malformation (CPAM) is a lung disorder that occurs from prenatal to childhood stages. This is the most common kind of congenital lung lesion, despite being a rare disorder. In order to effectively treat CPAM, an interdisciplinary healthcare team must be enlisted, particularly in cases of extreme severity.
What Is Congenital Pulmonary Airway Malformation (CPAM)?
Pregnancy-induced abnormal fetal lung tissue has the potential to develop into a mass referred to as a congenital pulmonary airway malformation (CPAM). This mass, also known as a tumor, is typically confined to one lung and does not function like normal, healthy lung tissue. Throughout the course of pregnancy, these masses can undergo significant changes in both size and appearance. A newborn with CPAM may have one or multiple masses, which can either be filled with fluid or solid in nature.
CPAM, previously known as congenital cystic adenomatoid malformation (CCAM), does not have a known cause. This rare condition affects approximately one in every 25,000 pregnancies. Although males are slightly more prone to experiencing CPAM than females, it is not inherited and, therefore, does not tend to recur within families.
In the majority of cases, congenital pulmonary airway malformation (CPAM) in babies is not a life-threatening condition. Occasionally, the lesions may decrease in size or become undetectable during pregnancy. Furthermore, there are instances where the lungs develop normally despite the presence of these lesions.
How Is Congenital Pulmonary Airway Malformation Diagnosed?
During a routine ultrasound in pregnancy, congenital pulmonary airway malformation (CPAM) can be detected. Additional tests can confirm the diagnosis, assess the severity of CPAM, and identify any other issues. These tests include magnetic resonance imaging (MRI) to determine the size and location of CPAM and its impact on lung development, as well as a fetal echocardiogram to evaluate the effect on the baby's heart structure and function. Weekly ultrasound tests are valuable in tracking the growth of the lesions and detecting the development of hydrops.
What Are the Treatment Options for Congenital Pulmonary Airway Malformation (CPAM)?
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Babies born with congenital pulmonary airway malformation (CPAM) are at a greater risk for complications both during and after delivery. These complications can manifest as breathing difficulties, underdeveloped lungs, and high blood pressure, among other issues.
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The primary and most crucial step in caring for babies with CPAM after birth is effectively managing these problems. As such, it is imperative that all babies with CPAM are delivered in a medical facility equipped with a neonatal intensive care unit and pediatric surgical services.
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Surgical removal of the lesions is necessary as they can potentially become infected or, in rare cases, cancerous. The timing of the surgery depends on the baby's symptoms following birth. If the baby does not experience any breathing problems, they may be discharged home and scheduled for surgery within the first year of life.
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When extensive lesions obstruct normal breathing, immediate surgery is required shortly after birth. Doctors may recommend a specialized birth plan that involves ex-utero intrapartum treatment (EXIT). This procedure entails delivering the baby via cesarean section and promptly performing surgery while the baby remains connected to the placenta.
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Even if immediate surgery is not necessary, some babies with CPAM may still encounter breathing difficulties at birth. They may require assistance from a ventilator or undergo extracorporeal membrane oxygenation (ECMO), a heart-lung bypass machine.
What Is Congenital Pulmonary Airway Malformation (CPAM) Resection?
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CPAM resection surgery is a medical procedure that eliminates a lung malformation in infants. A pediatric surgeon carries out this surgery, and the type of surgery performed depends on the size and location of the CPAM.
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In most cases, the surgery involves the removal of a lung lobe. However, in some instances, the surgeon may opt for minimally invasive surgery, also known as thoracoscopic lobectomy, which involves making three small incisions in the chest.
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In other cases, the surgeon may need to make a larger incision, also known as "thoracotomy," to access the malformation.
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After the surgery, infants will be closely monitored in the neonatal intensive care unit (NICU).
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If the surgery is performed using minimally invasive techniques, the hospital stay usually lasts only a few days. However, if open surgery is required or if the patient is unwell, the hospital stay will likely be longer.
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During this time, the child will be closely monitored for breathing issues and will be given pain medication as needed. The majority of infants who undergo CPAM removal surgery have a positive outcome, with most being able to go home from the hospital within a few days after surgery. Additionally, most infants will go on to have normal lung function and development.
What Is the Prognosis of Congenital Pulmonary Airway Malformation (CPAM)?
However, approximately 10 percent of CPAM cases can lead to serious problems for both the baby and sometimes the mother as well. The most severe complications arise when babies develop hydrops, a prenatal form of heart failure characterized by the accumulation of fluid in the skin, chest, or abdomen. Unless they receive prompt medical intervention or undergo fetal surgery to remove the lesions, babies with CPAM and hydrops typically do not survive.
Hydrops (fluid build up in tissues and organs) can also result in a condition known as "mirror syndrome," where the mother experiences symptoms mirroring the baby's illness. This includes the development of high blood pressure and fluid retention. In such cases, immediate delivery of the baby is necessary to safeguard the mother's life.
Conclusion
It is important to manage and monitor congenital pulmonary airway malformation from the prenatal period onwards, regardless of symptom severity. Communication and coordination between healthcare professionals are crucial, especially if the diagnosis is made before birth. If an infant with a known lesion is asymptomatic and observation is chosen over surgery, close follow-up during the first year of life is recommended to monitor for any respiratory symptoms.
