Congenital Pulmonary Airway Malformation

Also known as: CPAM, cystic adenomatoid malformation (CAM).

What is congenital pulmonary airway malformation?

CPAM is a congenital abnormality usually of one lung where a whole lobe of the lung is replaced by a non-working cystic (non- cancerous tumor filled with fluid) piece of abnormal lung tissue which will never work.

There are a number of types, however the most common one has large cysts.
 

What causes congenital pulmonary airway malformation?

Researchers don’t know what causes CPAM. It does not appear to be hereditary. It occurs among more boys than girls. 
 

What are the symptoms of congenital pulmonary airway malformation?

Symptoms depend on the size of the cysts. Usually babies will have breathing problems after birth, however in some cases, the cysts are small enough that breathing is hardly affected.

In very rare cases the cysts cause problems to the baby while still in the uterus.
 

What are congenital pulmonary airway malformation care options?

A mother with a fetus with CPAM needs to be closely monitored during pregnancy. In most cases the CPAM is large enough to need to be surgically removed shortly after birth; however if the baby has no problem breathing or other complication, then surgery can be delayed for some months.

In very rare cases where the fetal heart or swallowing is affected by large cysts while in the uterus, medication or in utero surgery may be suggested.


Reviewed by: Jack Wolfsdorf, MD, FAAP

This page was last updated on: 3/23/2018 2:17:16 PM

From the Newsdesk

The Fetal Care Center at Nicklaus Children's Hospital
03/30/2018 — A look into the Fetal Care Center at Nicklaus Children's Hospital. For more information and to contact a nurse navigator visit nicklauschhildrens.org/FetalCare
 
March Patient of the Month: Theodore
03/15/2018 — Meet our March Patient of the Month, Theodore. Theodore was diagnosed with cleft palate, cleft lip and a heart problem when he was only 18 weeks old. After he was born, Theodore had to be admitted into the NICU to be able to perform the necessary surgeries for him to live a healthy life.