Congenital Cystic Adenomatoid Malformation
Congenital cystic adenomatoid malformations (CCAMs, CCAM) are the most common genetic lung lesions. CCAM is a rare developmental anomaly of the lower respiratory system. It occurs when a baby's lung tissue grows more than average.
Babies may have only a single cyst or multiple. They cause cysts filled with fluid or solid masses in the lungs. Cysts can also prevent the alveoli (air sacs in the lungs) from developing normally. Cysts are usually present in one lobe of the lung. (CCAMs are also called congenital pulmonary airway malformations or CPAMs.)
Patients usually apply to the clinic with severe respiratory distress in the neonatal period. They may present as lung infection, growth retardation, or persistent pneumothorax.
Small cysts; can be diagnosed randomly or as infections months or years after birth.
How Are Congenital Cystic Adenomatoid Malformations Followed Up and Treated?
Lung CT is performed on these babies after birth to evaluate the cysts.
Treatment and follow-up
- Treatment depends on the cyst's size and the baby's symptoms.
- In the follow-up, we consider whether the cyst's growth is progressing or regressing.
- If babies have no severe problems in their follow-up, operating after one year of age is recommended.
If nutritional problems, growth retardation, infections, and respiratory distress occur, we perform surgery before age 1.
In babies, a new Thorax CT is taken after 12 months or six months; we compare it with the old one.
If there is a regression in the cysts or the diameter of the cyst was very small in the first tomography and did not grow in the new one, a repeat of the Thorax CT scan is recommended one year later.
Surgery is recommended if there is progression or growth compared to the first Thorax CT.
Surgery is recommended after one year for huge cysts, regardless of complaints or symptoms. Respiratory distress and infections disappear after surgery.
We generally prefer closed surgery methods (Thoracoscopic Surgery/VATS) in Pediatric lung diseases. With the development of technology, we prefer closed surgery methods even for children aged 8-12 months. The closed surgery method uses one or two incisions, as in open surgery, by placing an intercostal retractor. One of these incisions is 1 cm, and the other is a two or 3-cm incision. Surgical instruments are advanced through these incisions. The diseased area is removed. Closed surgery; has many advantages, such as less pain, shorter recovery time, and more aesthetics. Even a tiny incision looks very big in open surgery. Patients generally stay in the intensive care unit for one day. Patients are discharged in 3-4 days.
These closed surgeries in children require special high-tech devices and need a highly experienced center.