What Is Cystic Adenomatoid Malformation of the Lung?
Cystic adenomatoid malformation of the lung seen in the mother's womb is a mass or growth of the lung tissue that may not function well but can grow in size. It is also known as congenital pulmonary airway malformation. It is a rare congenital malformation. The mortality rate is much higher than around 30 % of children present with a cystic adenomatoid malformation in the lung. But these figures may not match the children who do not show any symptoms.
What Are the Causes of Cystic Adenomatoid Malformation of the Lung?
Congenital cystic adenomatoid malformation of the lungs is caused by the abnormal growth of lung tissue which continues to grow and does not function properly. These cystic adenomatoid are benign and not genetically inherited, so they do not pass to the upcoming generations. It is the abnormal growth of lung tissues.
When Does Cystic Adenomatoid Malformation of Lung Development Occur?
Cystic adenomatoid lung malformation develops at 18 to 26 weeks of gestation. But there will be rapid growth during the 20 and 25 th week of pregnancy. Any genetic defects do not cause it.
What Are the Signs and Symptoms of Cystic Adenomatoid Malformation?
The signs and symptoms of cystic adenomatoid malformation are diagnosed during the 18 to 26 weeks of pregnancy with the help of regular ultrasound that is performed during the pregnancy.
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The very first sign of congenital cystic adenomatoid is the pregnant mother who measures bigger than the usual size for her due date because of too much amniotic fluid. This is due to the congenital cystic adenoma pushing the heart and esophagus of the fetus, which prevents the swallowing of amniotic fluid by the fetus.
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A large cystic adenomatoid causes a condition called hydrops, in which there will be a severe accumulation of fluid in the skin, chest, and abdomen, which causes severe heart failure. As the cystic adenomatoid presses the heart, it reduces the blood supply to the heart and results in heart failure.
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About ten percent of all fetuses with cystic adenomatoid develop hydrops. Hydrops is the condition where there is excessive amniotic fluid in the mother's womb.
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Untreated fetuses with hydrops or congenital cystic adenomatoid will not survive.
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The mass or the abnormal tissue growth in congenital cystic adenomatoid will grow large and limit the growth of the lungs causing pulmonary hypoplasia, which is called small lungs.
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The mass or the tissue growth pushes the heart and esophagus of the fetus, which prevents swallowing amniotic fluid that results in polyhydramnios or too much amniotic fluid.
How to Diagnose Cystic Adenomatoid Malformation?
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The cystic adenomatoid malformation is generally diagnosed by a routine ultrasound done during pregnancy, in which an ultrasound shows a bright mass in the chest region.
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The size of the mass changes gradually throughout the pregnancy.
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The ultrasound also shows deviated position of the heart.
Can a Child With Cystic Adenomatoid Malformation of the Lung Survive?
If the size of the tissue is small, then the mass will shrink on its own. In most cases, the infant will survive. In very rare cases, there will be fetal death. Usually, the cyst will be harmless. But proper diagnosis and early intervention will cause further damage to the infant.
What Is the Treatment for Cystic Adenomatoid Malformation of the Lung?
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Most babies with cystic adenomatoid malformation of the lung are treated soon after birth.
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In very few cases, the treatment is done before the delivery through fetal intervention.
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Most infants with cystic adenomatoid will not have any symptoms, but in a few cases, the infants may develop difficulty breathing and require oxygen, and a breathing machine called a ventilator is put on.
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Infants without any symptoms can be discharged after a few days of observation.
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A CT scan is recommended after three months of age just to confirm if there is any mass or tissue growth left shrunk in the infant's lungs.
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The mass or the tissue growth should be removed surgically by the pediatric surgeon and a radiologist.
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Even though the child does not exhibit any symptoms, the tissue mass has to be surgically removed in order to prevent the mass from causing breathing problems in the future or may transform into a cancerous tumor.
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Fetal intervention is done only when there is heart failure in the fetus, and the surgery is performed on the mother; it is just like a C- section procedure where the surgeon removes the mass from the fetus.
How Is the Surgery Performed?
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The surgery is performed under anesthesia.
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The surgery is performed using many tiny telescopic instruments.
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Very small multiple incisions are given than giving a large chest incision to avoid scars.
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Very tiny scissors are used.
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And the child is discharged immediately after three to four days. When the child is breathing comfortably and able to drink formula milk or breast milk, and is comfortable on pain medications, the child will be discharged.
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The child should not be discharged if any of the following symptoms still persist, and post-discharge from the hospital also, the utmost care has to be taken.
Conclusion:
The congenital cystic adenomatoid malformation is a rare benign lung lesion that occurs in the fetus due to the abnormal tissue growth in the lung, causing difficulty in breathing and hindering lung development and also causing increased amniotic fluid in the pregnant mother which can be treated immediately after birth or any complications like heart failure arises then fetal intervention has to be performed which is similar to C-section. The procedure is done by a pediatric surgeon using tiny instruments with tiny incisions which cause minor scars. This mass will shrink on its own, but if it does not shrink, then only surgical intervention is required. Once the surgery is done and the child can breathe comfortably, the patient will be discharged by prescribing a few medications like painkillers.
