Congenital Pulmonary Airway Malformation​

Congenital pulmonary airway malformation (or CPAM) is a rare birth defect that causes a cyst or mass of abnormal tissue to develop in the lungs. A ‘cystic mass’ is a noncancerous tumour that is filled with fluid. CPAM can affect any of the lobes in the lungs. 

The symptoms of CPAM in children are as follows:

  • Respiratory distress: This is one of the most common symptoms of CPAM in newborns. It includes rapid breathing, grunting or severe respiratory failure.

  • Recurrent infection

  • Coughing up blood 

  • Shortness of breath and chest pain

  • Cough

  • Fever​​

If your child is experiencing any of the above symptoms at birth, consult with our expert doctors and nurses working with the Children's Hospital Services at The Aga Khan University Hospital.​

Your time with your doctor maybe limited, so make sure to prepare for your visit beforehand. Here are some tips to help get you started.

CPAM may be diagnosed using one of the following procedures:

With the use of prenatal ultrasound, doctors can now detect CPAM earlier during your pregnancy. This diagnosis must be confirmed because there are other birth defects that may appear similar to CPAM on ultrasound. CPAM will appear as a bright mass in the area of the chest where only lung tissue should be. The heart may be out of position, depending on the size and location of this cystic mass.

Your specialist will follow the growth of the mass by performing a series of ultrasounds. That means you will have targeted ultrasounds at regular intervals.

Your doctor also may recommend a foetal echocardiogram. This is an ultrasound of the heart performed by a paediatric cardiologist. Your doctor will recommend it to rule out structural heart defects and to assess heart function.

The treatment of CPAM in infants and children involves surgical removal of the affected area of the lung. The goals of the operation are to prevent infection and possible problems in the future.

The Aga Khan University Hospital offers various support services to help with managing or recovering from the disease or condition. These include but are not limited to nutrition, physiotherapy, rehabilitation, specialized clinics and some patient support groups. Your doctor or nurse will advise you accordingly.

The Aga Khan University Hospital offers financial assistance to those who are in need and fulfil the eligibility criteria. For further information, you can contact the Patient Welfare Department. You can find the contact number of the Patient Welfare Department in the 'Important Numbers' section on the website homepage.

The financial counselling staff is available during office hours, at the main PBSD (Patient Business Services Department), to answer your financial queries on treatments' costs and authorize admissions on partial deposit as per hospital policies allow. The financial counsellor in the emergency room is open 24/7. You can find the contact number of the Patient Business Services in the 'Important Numbers' section on the website homepage.

Your doctor and or nurse will give you specific instructions about the prescribed medication. Please ensure that you take or use the prescribed medicine as advised. It can be dangerous to your health if you self-prescribe. Please inform the doctor or nurse beforehand if you have experienced any adverse reactions to any medications in the past. If you experience any symptoms of drug poisoning, overdose or severe reaction please contact the Pharmacy Service at The Aga Khan University Hospital immediately. You can find the contact number of the Pharmacy Services in the ‘Important Numbers’ section on the website homepage.​

The information provided on our website is for educational purposes and not intended to be a substitute for medical advice, diagnosis or treatment. You should always seek the advice of your doctor or other healthcare professional provider.