Thursday, January 6, 2011

Congenital Cystic Adenomatoid Malformation of the Lung

Since the advent of the prenatal ultrasonography we have been able to pick-up a lot of diseases while the baby is in the womb so we can be better prepared to handle them when they are born. CCAM(Congenital Cystic Adenomatoid Malformation) is a rare intrathoracic tumor that is present in the lungs. This occurs in 1 - 25,000 to 35,000 pregnancies. This is usually found during the 20 week gestation and they hare followed closely with repeat ultrasounds. The wonderful thing about this is that 60% resolve at birth. The ones that do not resolve are classified into types. The presenting symptoms at birth depends on the size of the cyst if it is encroaching on the blood vessels or the other organs.
Type I - isolated cyst present in the lungs during the neonatal period
Type II - the cyst in the lung is usually associated with other malformations on the baby
Type III - this is the worse kind because the baby would be born as a fetal hydrops and they do not survive.

If the cyst is significant that baby will be in respiratory distress at birth. The ultimate cure for this is surgical excision. If the baby is asymptomatic and the cyst is small. The pediatric surgeon will follow-up in a few weeks and determine when they are going to operate. Even if the cyst is small this can be a focus of recurrent pneumonia so most surgeons operate on the lesion.