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Introduction

Congenital cystic adenomatoid malformation(CCAM), a benign lung lesion, develops before childbirth, visible as a mass or lesion in the chest region. It is an abnormal lung tissue that malfunctions and grows continuously. Congenital Cystic Adenomatoid Malformation is often known as pulmonary airway malformation.

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What are Congenital Cystic Adenomatoid Malformation?

Congenital Cystic Adenomatoid Malformation is a kind of fetal lung lesion that develops before the baby’s birth, varying in size. The lesions are generally fluid-filled or solid. The small cyst is known as a microcystic lesion, and the larger cysts are known as a macrocytic lesion.

If left untreated, it can be fatal, early and accurate diagnosis is essential. Most of the lesions are minute and do not harm the baby. Treatment of congenital adenomatoid formation is generally by removing it after birth. A few large lesions may require treatment before birth because they may cause severe and life-threatening complications known as fetal heart failure or maternal mirror syndrome.

What causes Congenital Cystic Adenomatoid Malformation ?

The disease is not due to genetic or chromosomal abnormality but because of the abnormal lung tissue growth, usually in one lobe of lung tissue.

What are the symptoms of Congenital Cystic Adenomatoid Malformation?

Prenatal ultrasound is a routine examination to detect lung lesions. During an ultrasound, the technician identifies a bright spot in your baby’s lung, showing a mass or cyst. The lesion grows in size until 26 to 28 weeks of pregnancy, increasing or decreasing in the cyst size.

What are the evaluation and diagnosis of Congenital Cystic Adenomatoid Malformation?

The radiologists use prenatal diagnosis and cutting-edge technologies for detailed information regarding the baby’s diagnosis.

  • ● You may have a high-resolution fetal ultrasound for evaluating fetal anatomy and lung lesion.
  • ● A pediatric cardiologist will evaluate the heart structure and function by performing an echocardiogram.
  • ● If necessary, the cardiologist may also perform an ultrafast fetal MRI for anatomical evaluation of lung lesions.

The following tests enable the physicians to detect the size, type, and location of the mass at the blood supply site and see any impact on the baby’s heart.

Ultrasound is performed twice weekly based on your baby’s age and size of the mass for monitoring the growth of lung lesions.

How to Manage Congenital Cystic Adenomatoid Malformation during pregnancy?

Microcystic lesions may not cause any breathing difficulties in your baby. Monitoring microcystic lesions to ensure they do not grow larger is essential.

In some instances, the cystic lesions are large and may need treatment before birth. The lung lesion growth occupies spaces in the chest region, restricting normal lung growth leading to malfunctioning of lungs at the time of birth.

Large lesions hamper blood flow shifting the position of the heart. These will cause fetal heart failure causing fluid build-up in the liver and placenta. Suppose your baby is diagnosed with congenital cystic adenomatoid malformation during your pregnancy; in that case, you should monitor blood pressure and other signs and symptoms of maternal mirror syndrome or preeclampsia. Urine tests and blood pressure help detect the signs of preeclampsia.

What Fetal intervention required for monitoring the congenital cystic adenomatoid formation?

There are several treatment options before birth to decrease the size of congenital cystic adenomatoid malformation; the following are some of the treatment options:

  • ● Steroids to prevent lesion growth:

The solid microcystic lesion is responsive to steroids; this may interrupt the lesion growth. Steroids stunt the growth of Congenital Cystic Adenomatoid Malformation help in preventing fetal surgery in some cases.

  • ● Fluid drainage from the cyst:

Prenatally microcystic lesions are drained for removing fluid that accumulates to decrease the mass size. There are chances for the fluid to accumulate again; in such cases shunt is placed. Ultrasound guidance is used for performing shunting procedures. The hollow needle (large trocar) is inserted through the mother’s abdomen, uterus, and further into the fetal chest. The shunt passes through the trocar and into the fluid-filled cysts. The objective of this procedure is to decrease the size of a mass warding off heart failure.

  • ● Open fetal surgery for removing congenital cystic adenomatoid malformation:

The advancement in the care of childbirth has ruled out open fetal surgery. In case your baby is not responsive to other prenatal treatments and early signs of heart failure, an experienced team in the hospital is ready to remove the congenital cystic adenomatoid malformation. Highly skilled fetal surgeons open the mother’s abdomen and uterus for removing mass from the baby’s lung. After surgery, the fetus is relocated to the womb, where a normal lung continues to grow.

  • ● EXIT Procedure:

If your baby has a macrocystic lesion in the lungs resulting in Congenital Cystic Adenomatoid Malformation and hydrops are not developed, a delivery technique is required ex utero intrapartum treatment (EXIT) procedure. This procedure is essential due to the lungs’ compression because it may not allow the baby to breathe independently after birth.

Babies delivered with Congenital Cystic Adenomatoid Malformation

You and your medical team plan for a 32-week gestation period; your delivery is based on the size of congenital cystic adenomatoid malformation and the prenatal treatment course.

In a vast majority, a mother delivers her baby at a local hospital without the requirement of high-risk neonatal care. Microcystic lesions (small lesions) are not visible during follow-up ultrasound imaging at birth; however, the lesion may still be present. Your fetal surgeon determines the removal of lesions after birth, and you must schedule a follow-up appointment. Babies with large and complex lesions have difficulty breathing and are shifted to a center for mother and baby’s care.

What surgery required for Congenital Cystic Adenomatoid Malformation?

A majority of the small lesions are surgically treated after birth. First, you will come along with your baby with an appointment for evaluation by the surgical team. A CT scan with contrast is performed to confirm the diagnosis and discover the exact location of your child’s lung lesion.

While the baby is young, performing surgery has many benefits, including promoting compensatory lung growth and preventing potential complications such as lung infection.

In rare cases, the mass may turn cancerous.

Treatment Outcome:

Most children with fetal lung lesions have normal lung function after removing their lesion and do well because of the rapid compensatory lung growth during their childhood. Early surgery maximizes their compensatory growth.

Result

The baby and mother are healthy after the lung lesions are removed.

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FAQ's

Can Congenital Cystic Adenomatoid Malformation be Fatal?

Babies with Congenital Cystic Adenomatoid Malformation with hydrops may not survive if they do not respond to medicines quickly or if the lesions are untreated or removed via surgery.

What is Meant by the Term Congenital Cystic Adenomatoid Malformation?

Congenital Cystic Adenomatoid Malformation is developing a cyst in one of the lung lobes, either fluid-filled or solid, further preventing the lungs’ normal functioning. It seldom occurs in both the lobes.

Can Congenital Cystic Adenomatoid Malformation be Genetic?

Congenital Cystic Adenomatoid Malformation occurs sporadically and has no known genetic cause. There are no chances of such recurrence for mothers with such babies as it is not a hereditary condition.

Does Congenital Cystic Adenomatoid Malformation Go Away on its Own?

Congenital Cystic Adenomatoid Malformation usually grows, and in a few of the cases, it can cause severe problems for you and your baby, further developing into hydrops. The majority of the lesions usually stay the same size or increase, decrease and eventually disappear following the pregnancy progression.

Is Congenital Cystic Adenomatoid Malformation Common?

The incidence of Congenital Cystic Adenomatoid Malformation is between 1 in 11,000 to 1 in 35000 live births. However, the incidence may be high in the mid-trimester because of spontaneous resolution.

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