Had our routine visit yesterday to check Isabell out and she is 1 pound 11 ounces and is growing great but unfortunately . . . so is the mass. The doctor said he is NOT alarmed by the growth because we knew that it could grow but we were hoping that it would just go away. There is still NO fluid build-up (hydrops) PRAISE THE LORD! They did change her diagnosis last week and it is NOT a CCAM 3 it is a Pulmonary Sequestration = (also known as bronchopulmonary sequestration) is a cystic piece of abnormal lung tissue that does not work like normal lung tissue. They usually appear next to the lung (extrapulmonary) or within one part of the lung (intrapulmonary). We do not know what causes one part of the lung to develop abnormally. The cystic tissue involved will never function as normal lung tissue. There are several types of cystic lung disease including congenital cystic adenomatoid malformation (CCAM), pulmonary sequestration, and defects that are a mixture of these two. The abnormal piece of lung can be microcystic (many small cysts) or macrocystic (several large cysts). Pulmonary sequestrations are distinguished from CCAMs by a blood vessel that comes directly from the main artery (aorta).
Pulmonary sequestrations vary in size at presentation and can change throughout the pregnancy. The diagnosis is made by prenatal ultrasonographic findings of an echogenic (bright) mass appearing in the chest of the fetus. The mass may displace the heart from its normal position or push the diaphragm downward, but the key feature of a sequestration is the finding of an artery leading from the cystic mass directly to the aorta. This is what distinguishes a pulmonary sequestration from a CCAM.
The majority of these fetuses have a very good outcome. Pulmonary sequestrations remain the same size or grow with the fetus, but usually do not cause severe problems, probably because there remains enough room for the normal part of the lung to grow. The mass may shrink in size before birth. In all these cases, the outlook for a normal life is excellent. These fetuses should be followed closely, delivered near term, and the pulmonary sequestration removed surgically after birth.
Those who do not have hydrops when the lesion is first detected must be closely followed by doing ultrasounds at least every week to look for the development of hydrops. If they do not develop hydrops, we continue a “wait-and-see” attitude with close follow-up. Many of these lesions will begin to decrease in size before 26 weeks of gestation and almost all can be safely dealt with after birth at a tertiary perinatal center. Some lesions will even take care of themselves entirely.
A small number of fetuses with pulmonary sequestrations may develop large pleural effusions (excess fluid in the chest cavity) and even signs of heart failure (hydrops). Unlike CCAM that cause trouble by virtue of their size, pulmonary sequestrations cause trouble by virtue of the high blood flow through the tumor. These are the only fetuses who require treatment before birth.
PLEASE CONTINUE TO PRAY!
* here are a few pictures of her. Our Doctor said that she was playing with her hair, but I think she likes the feeling of her bald head! The first one is her peace sign . . surprised that we could see her tiny little fingers because she's usually making a fist!