Congenital Pulmonary Airway Malformation (CPAM)

Learn symptoms, causes, management of pediatric CPAM, and when to contact a pediatric surgeon in Singapore.

What Is Congenital Pulmonary Airway Malformation (CPAM)?

Congenital Pulmonary Airway Malformation (CPAM), also called Congenital Cystic Adenomatoid Malformation, is a rare lung condition that develops before birth. In CPAM, abnormal lung tissue forms cysts or air-filled sacs in one or both lungs. These abnormal areas don't work like normal lung tissue and can affect your child's breathing.
Most cases of CPAM are found before birth during routine prenatal ultrasounds. This early detection allows for CPAM prenatal counseling and planning for specialized care after delivery. Some smaller CPAMs may not be discovered until childhood when symptoms develop, or during tests for other reasons.
CPAM can range from small areas affecting just part of one lung to larger areas affecting an entire lobe (section) of the lung. The size and location determine what symptoms your child may have and what treatment, such as minimally invasive CPAM resection or open lobectomy, will be needed.

Types of CPAM

Doctors classify CPAM into different types based on what the abnormal tissue looks like:

Type 1 (most common): Large cysts - this type usually has the best outcome
Type 2: Multiple small cysts - sometimes associated with other birth differences
Type 3: Solid-looking tissue with very tiny cysts - may cause more breathing problems
Type 4: Large cysts in the outer part of the lung

The type affects treatment decisions, including whether minimally invasive CPAM resection or open lobectomy is best.

What Is Congenital Pulmonary Airway Malformation (CPAM)?

Symptoms vary widely depending on the size and location of the abnormal lung tissue.

In Newborns

Babies with large CPAMs may have symptoms right after birth:

Trouble breathing with fast, labored breaths
Blue or purple skin (cyanosis) from low oxygen
Chest looks uneven - one side may appear larger
Grunting sounds when breathing
Needing oxygen or breathing support

Babies with smaller CPAMs may look completely normal at birth and develop no symptoms for months or years.

In Older Infants and Children

Children with CPAM that wasn't found at birth may develop:

Frequent lung infections (pneumonia) that keep coming back in the same area
Persistent cough that doesn't go away
Wheezing or noisy breathing
Chest pain in the affected area
Shortness of breath during activity
Fever with recurring chest infections
Slow growth if breathing problems are significant

No Symptoms

Many children with small CPAMs feel completely fine and have no symptoms. The CPAM is only found on a chest X-ray or CT scan done for another reason.

When to Seek Care

If your baby has trouble breathing after birth, contact medical care immediately. For older children, see us if your child gets lung infections frequently, has ongoing breathing problems or cough, or if CPAM was found on ultrasound and you need CPAM counseling. We'll evaluate whether CPAM thoracic surgery is needed.

What Causes CPAM in Children?

CPAM develops during pregnancy when the lungs are forming; the exact reason this happens isn't yet fully understood. It’s important to remember that CPAM is not caused by anything the mother did or didn't do during pregnancy. It appears to be a random occurrence during development.
During weeks 4-7 of pregnancy, the lungs begin as a small bud that branches out like a tree. Each branch continues dividing to create smaller and smaller airways. At the very end of these branches, tiny air sacs called “alveoli” form and do the actual work of breathing.
In CPAM, something goes wrong during this branching process. Instead of forming normal airways and air sacs, abnormal tissue develops. This abnormal tissue may form cysts (fluid or air-filled spaces), have too much tissue, or have airways that don't connect properly.
CPAM isn't inherited from parents. If you have one child with CPAM, your chance of having another child with it is not increased. However, CPAM prenatal counseling can help address family concerns.

Consequences of Untreated CPAM

While some small CPAMs cause no problems, leaving CPAM untreated can lead to serious complications.

Repeated Lung Infections

The most common problem with CPAM is recurrent pneumonia. The abnormal lung tissue doesn't clear mucus and bacteria well, making infection more likely. Each infection causes pain, fever, coughing, missed school, and antibiotic treatment. Over time, repeated infections can damage healthy lung tissue around the CPAM. Without CPAM thoracic surgery to remove the abnormal tissue, infections keep coming back.

Collapsed Lung (Pneumothorax)

Cysts in CPAM can rupture, allowing air to leak into the space around the lung. This causes the lung to collapse (pneumothorax). Symptoms include sudden chest pain, trouble breathing, and rapid heart rate. A collapsed lung is a medical emergency requiring immediate treatment. It's more likely to happen with certain types of CPAM.

Breathing Difficulties

Large CPAMs can press on normal lung tissue, preventing it from expanding fully. This causes shortness of breath, difficulty exercising, and fatigue. In babies, large CPAMs can cause severe breathing problems right after birth, which requires immediate medical intervention.

Risk of Cancer

While rare, there's a small long-term risk that CPAM tissue could develop into a type of lung cancer called pleuropulmonary blastoma or bronchioloalveolar carcinoma. This risk is another reason doctors often recommend removing CPAM tissue through minimally invasive CPAM resection or open lobectomy, even when it's not causing symptoms.

Heart Problems

Very large CPAMs can press on the heart and major blood vessels in the chest. Before birth, this can cause heart failure in the fetus. After birth, significant pressure can affect heart function.

Reduced Quality of Life

Children with untreated symptomatic CPAM may have limited ability to play and exercise, frequent doctor visits and hospitalizations, anxiety about breathing problems, and missed school and activities. CPAM thoracic surgery can resolve these issues and allow normal childhood activities.
Chronic breathing problems and repeated infections can slow a child's growth and development, affecting weight gain and overall health.

How to Treat CPAM in Children

Treatment depends on the size and type of CPAM, whether your child has symptoms, and other factors. At The Little Surgery, Dr. Ong Lin Yin provides comprehensive care including CPAM prenatal counseling for expecting parents, evaluation of children with CPAM, and expert CPAM thoracic surgery, including both minimally invasive CPAM resection and open lobectomy.

CPAM Prenatal Counseling

If CPAM is found on prenatal ultrasound, CPAM prenatal counseling helps prepare your family.
We meet with expecting parents to explain what CPAM is, discuss what to expect after delivery, review monitoring during the rest of pregnancy, plan for delivery at a hospital with specialized care, and answer all questions about the condition and treatment.
Throughout pregnancy, regular CPAM ultrasounds check its size, watch for signs if it's affecting the baby's heart, and help plan delivery timing and location. Most babies with CPAM do well, but some large lesions need intervention even before birth.
Based on CPAM size and any complications, we coordinate delivery at a hospital where specialized CPAM thoracic surgery expertise and neonatal intensive care are available if needed.

Treatment for CPAM Found after Birth

If CPAM wasn't found before birth, diagnosis after birth involves physical examination, chest X-ray showing the abnormal area, and CT scan providing detailed pictures to plan surgery.

Conservative Management

Very small CPAMs that cause no symptoms may be monitored with regular check-ups and periodic imaging to watch for growth or complications. However, most doctors recommend surgery even for asymptomatic CPAM to prevent future problems including infection and cancer risk.

CPAM Thoracic Surgery

CPAM thoracic surgery is recommended when your child has symptoms (breathing problems, infections), the CPAM is large, repeated lung infections occur in the same area, or to prevent future complications. Surgery is typically done after 6 months of age if possible, though babies with severe symptoms may need earlier intervention.The goal of CPAM thoracic surgery is to remove all the abnormal lung tissue while preserving as much healthy lung as possible. For many children, CPAM can be removed using minimally invasive techniques called thoracoscopy or VATS (video-assisted thoracoscopic surgery). Minimally invasive CPAM resection is best for smaller to medium-sized CPAMs, CPAMs located in accessible areas, and when there's no active infection or significant inflammation.For some cases, traditional open lobectomy is necessary. This involves removing the entire affected lobe of the lung. Open lobectomy is needed for very large CPAMs, complicated CPAM with significant inflammation or infection, abnormal blood vessels requiring careful dissection, or for babies with severe symptoms.CPAM thoracic surgery is done under general anesthesia, so your child is asleep and comfortable. The surgery typically takes 2-4 hours depending on the approach and complexity.

Recovery Post-Surgery
Most children stay 3-7 days in the hospital after minimally invasive CPAM resection or 5-10 days after open lobectomy. Care includes pain management, breathing exercises to help the lung expand, chest tube monitoring and removal when drainage decreases, and gradual increase in activity.
At home, recovery continues for several weeks. Your child should avoid strenuous activity and contact sports for 4-6 weeks, do breathing exercises as instructed, and attend follow-up appointments to monitor healing.
After successful CPAM thoracic surgery, most children recover completely. The remaining healthy lung tissue grows and compensates, leading to normal lung function for most children. They can participate in all regular activities including sports. Long-term follow-up with a paediatric surgeon ensures continued good health.

Why Choose The Little Surgery for CPAM Treatment

We provide comprehensive care for CPAM, including CPAM prenatal counseling for expecting families, thorough evaluation and diagnosis, expert CPAM thoracic surgery using both minimally invasive CPAM resection and open lobectomy, and complete follow-up care.

Experienced Pediatric Surgeon

Dr. Ong Lin Yin has over 20 years of experience in paediatric surgery, including specialized training in CPAM thoracic surgery. As the former Head of Paediatric Surgery at KK Women's and Children's Hospital, she has successfully performed numerous CPAM resections using minimally invasive and open techniques with excellent outcomes.

Comprehensive Care

Every CPAM is different. We tailor our approach based on the size, type, and location of the CPAM, your child's age and symptoms, presence of complications, and your family's circumstances. Our goal is optimal outcomes with the safest, most appropriate treatment options.
After CPAM thoracic surgery, we provide ongoing care to monitor lung healing and growth, ensure complete recovery, watch for any rare complications, and support your child's return to all normal activities.

Family Support

For families whose baby has been diagnosed with CPAM before birth, we offer detailed CPAM prenatal counseling to explain the condition, answer questions, coordinate with maternal-fetal medicine specialists, plan for delivery and postnatal care, and provide reassurance and support throughout pregnancy.
We understand that having a child with CPAM can be overwhelming. Dr. Ong provides clear explanations of the condition, treatment options, what to expect during recovery, and compassionate support throughout the treatment journey. As a mother herself, she understands family concerns and ensures you feel informed and comfortable with all decisions.

Contact The Little Surgery Today

If your child has been diagnosed with CPAM, whether before birth or later in childhood, we can help.
Contact The Little Surgery today and take the first step toward ensuring your child's health and happiness.