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Each lung consists of two or three smaller sections known as lobes. CPAMs are normally restricted to one lobe of the lung, with the CPAM receiving the same blood flow as the remainder of the normal lung. CPAMs are somewhat more prevalent in male foetuses than female foetuses. The numerous forms of CPAMs are characterised based on how they show on ultrasound.
CPAMs are classified as
- Type I (macrocystic), which accounts for 50% of cases and comprises one or many large cysts (2-10 cm in diameter)
- Type II (microcystic lesions) account for 40% of cases and consist of many tiny cysts (less than 2 cm in diameter)
- Type III (solid-appearing lesions) account for the remaining 10% of instances and present as solid masses
Other issues that might arise in the foetal lungs include
- Bronchopulmonary sequestration (BPS) occurs when an aberrant component of lung tissue, usually a solid section, fails to function normally. This region gets blood flow from the foetal body's major artery (the aorta), allowing the bulk to increase in size.
- Hybrid lung lesions, like BPS, may be cystic and solid, with blood supply from the aorta.
- Bronchial atresia occurs when the main air tube (bronchus) fails to develop across the lung, making one or both foetal lungs look white on ultrasound.
Symptoms Of Congenital Pulmonary Airway Malformation
Some children born with CPAM will be unable to breathe properly owing to the massive size of the lump in their chest. Babies who are unable to breathe adequately on their own may have impaired cardiac function. These newborns need urgent critical care to stabilise them before the bulk can be removed surgically. Causes Of Congenital Pulmonary Airway Malformation
The cause of a CPAM is uncertain.Complications Of Congenital Pulmonary Airway Malformation
If a CPAM is big enough to cause the heart to move to the other side of the chest, the foetus may have further issues. One method for predicting the result is to use ultrasonography to quantify the CPAM and compare it to the size of the foetal head (cephalic to volume ratio, or CVR-Congenital Pulmonary Airway Malformation Volume Ratio ). A number higher than 1.6 indicates a high CPAM. CPAMs that move the heart around in the chest might cause swallowing issues in the foetus. This may result in excess fluid around the exterior of the foetus in the amniotic sac (polyhydramnios), which happens in about one-fourth of all instances. In around 10% of instances, the foetus may develop a more dangerous condition known as hydrops. In this case, fluid may accumulate in the belly (ascites), lungs (pleural effusions), heart (pericardial effusion), and skin (subcutaneous oedema). The placenta may also thicken with fluid (placentomegaly), and excess fluid can accumulate in the sac around the foetus (polyhydramnios). If left untreated, hydrops might indicate the imminent demise of the foetus.
Type II CPAMs are often connected with organ problems, such as those affecting the heart. Other foetal malformations are uncommon and more often linked with Type I and Type III CPAMs. As a result, an expert physician should do a thorough examination of the foetal anatomy using ultrasonography. A foetal MRI and a foetal echocardiogram (a special cardiac ultrasound) may also be beneficial in evaluating foetal anatomy. Chromosome abnormalities in foetuses with CPAM are quite infrequent. Long-term investigations of children with CPAMs that were not removed after birth found an increased risk of infection in the CPAM as well as a moderate increase in the development of cancer.
For these reasons, your infant will be evaluated by a paediatric surgeon during the first few weeks after birth, who may request a CT scan at roughly 2 to 3 months of age. A decision will be made about removing the CPAM, which happens between the ages of 3 and 6 months, using a tiny telescope (thoracoscopy) put in the baby's chest.
Diagnosing Congenital Pulmonary Airway Malformation
A CPAM is often discovered during an anatomy ultrasound at around 20 weeks (5 months) gestation. The mass might look like a cluster of fluid-filled cysts in one of the foetal lungs, or it can be more solid and whiter (echogenic) than the surrounding lung. If the CPAM is big enough, it may push the foetal heart to the other side of the chest.Treating Congenital Pulmonary Airway Malformation
In certain circumstances, your doctor may recommend inserting a shunt (a short tube) into the cyst to drain it into the fluid region outside the foetal chest. If your baby's CPAM has a CVR of more than 1.6, your doctor may recommend steroids.
If the CPAM remains big despite the use of steroids, you may be booked for a C-section so that paediatric surgeons in a nearby operating theatre are prepared to remove the CPAM immediately after delivery to address your baby's breathing issues.
Open foetal surgery has previously been performed when the foetus was very preterm and hydrops were developing.
FAQs on What You Need to Know About Congenital Pulmonary Airway Malformation (CPAM)?
- What is the usual treatment for CPAM?
This treatment often includes surgery to remove the aberrant section of the lung 6-9 months after birth. Foetuses with a high CPAM on prenatal ultrasonography are first intensively monitored by our centre, generally once a week but occasionally twice a week. - What abnormalities are related to CPAM?
This kind is often linked with additional abnormalities (renal agenesis, cardiovascular problems, diaphragmatic hernia, skeletal malformations, oesophageal atresia). Typically, it manifests as many tiny cysts with little bulk impact. The prognosis is favourable, with no malignant potential.
