HomeHealth articlescongenital abnormalitiesWhat Is Congenital Cystic Adenomatoid Malformation?

Congenital Cystic Adenomatoid Malformation- An Overview

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The congenital cystic adenomatoid malformation is the formation of a cyst or mass in the chest before birth. Read this article to learn about this condition.

Written by

Dr. Sri Ramya M

Medically reviewed by

Dr. Veerabhadrudu Kuncham

Published At November 10, 2022
Reviewed AtMay 2, 2023

Inroduction:

Congenital cystic adenomatoid malformation (CCAM) refers to the formation of a non-cancerous cystic lesion or an abnormal mass in the chest. It is the most common fetal lung lesion, also known as congenital pulmonary airway malformation (CPAM). If left untreated, it can cause life-threatening complications.

What Is Congenital Cystic Adenomatoid Malformation?

The congenital cystic adenomatoid malformation is a common congenital lung lesion. It refers to the formation of a non-cancerous cystic lesion or an abnormal mass of tissue in the lungs. It is a non-functional mass but continues to grow in size. It can be a fluid-filled cyst or a solid mass that can vary in size.

What Are the Causes of CCAM?

The exact cause remains unknown. It is not due to gene or chromosomal abnormalities. It is due to the formation of an abnormal mass of tissue in the lungs.

What Are the Types of CCAM?

It is divided into five types.

  • Type 0 - Cysts are small in size. The severe and rarest form arises from the trachea (windpipe) or bronchus.

  • Type 1 - A single large cyst or a small number of large cysts are present. The large cysts may group to form a large mass. The most common form of CCAM arises from the distal bronchus or the proximal bronchiole.

  • Type 2 - consists of smaller cysts or a solid mass of tissue. It arises from the terminal bronchioles.

  • Type 3 - It is composed of very small cysts. It arises from acinar-like tissue.

  • Type 4 - It consists of very large cysts that may be associated with malignancy. They arise from the alveolus.

What Are the Signs and Symptoms of CCAM?

Congenital cystic adenomatoid malformations are identified during prenatal ultrasounds. It appears as a bright spot in the fetal lungs that indicates a cyst or a mass. It increases in size during 26 - 28 weeks of gestation. The baby develops symptoms of cyanosis (bluish skin discoloration), pneumothorax (collection of air in the pleural space between the lungs), and breathing difficulties after birth. It presents with hyper-resonance at percussion, diminished vesicular murmur, and an asymmetrical thorax.

How Is CCAM Diagnosed?

Congenital cystic adenomatoid malformations are diagnosed during routine prenatal ultrasounds.

The following tests are done to diagnose CCAM.

  • High-resolution ultrasound is used to evaluate the fetal anatomy and lung lesion.

  • A fetal echocardiogram is used to evaluate the structure and function of the heart.

  • A fetal MRI (magnetic resonance imaging) scan may be done to evaluate the anatomic details of the lung lesion.

These tests are done to determine the type, size, and location of the cyst and its blood supply and to check if there is any impact on the fetal heart.

How Is the Severity of CCAM Determined?

The severity of CCAM can be determined before birth using the CCAM volume ratio or CVR. The size of the lung lesion is measured in relation to the baby's size based on the head circumference. This measures the accurate growth of CCAM and aids in treatment. If the CVR is more than 1.6, the baby is at a higher risk for developing heart failure. Continuous monitoring is done to assess the growth of the lesion and the requirement for treatment before birth.

How Is CCAM Managed During Pregnancy?

CCAMs are small lesions and do not usually cause stress on the fetal heart or breathing problems after birth. It requires continuous monitoring before birth to ensure the size remains small. In rare cases, the lesion becomes large and requires treatment before birth. Large lesions can restrict the growth of the lungs and lead to underdeveloped lungs. It also displaces the heart and impairs blood flow. It can result in fluid buildup (hydrops) and heart failure. The mother should be continuously monitored for high blood pressure and signs and symptoms of preeclampsia.

How Is CCAM Managed in the Fetus?

CCAM can be managed with various treatment options before birth to reduce the lesion size and the chances of fetal heart failure. The treatment depends on the type of CCAM and the fetus's gestational age.

The following are the available treatment options:

  • Steroids - Steroids are given to prevent the growth of the lesion. It is given to interrupt the growth of solid microcystic lesions. Steroids reduce the growth of the lesion and prevent the need for surgery.

  • Draining Fluid From the Cyst - A trocar (hollow needle) is inserted into the mother's abdomen through a small incision to reach the uterus and the fetal chest under ultrasound guidance. The shunt is passed through the trocar to reach the fluid-filled cyst and divert the fluid from the cyst to the amniotic sac. This shunt remains till delivery to decrease the size of the lesion and prevent heart failure.

  • Fetal Surgery - Surgery is done if the fetus is not responding to other treatments and shows signs of heart failure. An incision is made in the mother's abdomen, and the fetus is reached to remove the abnormal mass from the fetus's lungs. The baby is placed in the womb after surgery for normal development. This procedure is rarely done.

How Is a Baby With CCAM Delivered?

The baby is delivered via specialized procedures.

  • C-Section - A baby with a large lesion can be safely delivered through C-section. The baby is then operated on, and the abnormal mass is removed from the lungs. Small lesions can be successfully removed after birth. After surgery, the baby is placed in the intensive care unit and continuously monitored.

  • EXIT Procedure - A specialized procedure called EXIT (ex utero intrapartum treatment) is performed if the lesion is large and continues to grow to obstruct the airway. The mother is anesthetized, and an incision is made in the abdomen. The baby is then partially delivered, remaining attached to the mother's placenta. The baby still receives oxygen through the umbilical cord, which provides time for the surgeon to remove the cystic lesion and establish airway support. The baby is then fully delivered, and it can breathe independently.

Conclusion:

The congenital cystic adenomatoid malformation is a common fetal lung condition that can be treated. Earlier diagnosis and timely intervention prevent complications. Early surgical removal promotes compensatory lung growth and prevents the risk of infections.

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Dr. Veerabhadrudu Kuncham
Dr. Veerabhadrudu Kuncham

Pediatrics

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