Written by ◆ Yang Qianni and compiled by ◆ Liang Yingxiu
With the advancement of ultrasound technology, congenital pulmonary respiratory malformations can be detected in abnormal scans at 20 weeks of pregnancy.
Otherwise, it’s usually because the child has breathing problems, recurring infections, or a pneumothorax, and it’s not discovered until a doctor visits him with an X-ray or computed tomography scan.
KUALA LUMPUR: During the fetal period, all lung lesions are evaluated and managed similarly. Although most of these lesions stop growing around the 26th week of pregnancy, some continue to grow during pregnancy and require close monitoring. More than 90% of fetuses with lung lesions diagnosed prenatally will not have problems compressing other normal lungs or hearts.
Consultant Pediatric Surgery
(Dr Nada Sudhakaran)
Disease monitoring to avoid polyhydramnios
Dr Nada Sudhakaran, Consultant Pediatric Surgeon, said that observation and monitoring can help avoid problems such as polyhydramnios, mediastinal shift and hydrops fetalis, such as skin, pleural effusion or ascites. The presence of edema may have the most serious prognosis for the fetus and pregnancy.
“Another frequently used prenatal assessment of lung masses is the Congenital Pulmonary Airway Malformation (CPAM) Volume Ratio (CVR). CPAM volume is calculated from the lung mass in 3 vertical planes, The CVR is calculated by dividing the CPAM volume by the head circumference in centimeters.”
CVR can assess the severity of lung malformations, and then the following interventional methods can be used to treat complex lung lesions.
In the past, mothers whose fetuses were diagnosed with CPAM were informed of the poor prognosis and advised to terminate the pregnancy. However, with good prenatal monitoring and timely intervention, termination of pregnancy is no longer appropriate (except for very rare complex malformations). In addition, there are also cases of lesions disappearing during serial ultrasound scans.
Prenatal treatment options for complex lesions include:
1. Steroids for pregnant women (most common): If the fetus has large microcystic lesions or hydrops in the first trimester, steroid injections can be given. Steroids appear to reduce the growth rate of these lesions and improve edema. Steroids are usually used in fetuses with larger lesions or any risk of edema.
2. Thoracoamniotic shunts: If there is a single large cyst of CPAM causing hydrops fetalis, or if there is obvious pleural effusion, it can be treated with a shunt. The shunt is a thin tube that drains the contents of the fetal chest into the amniotic fluid in the uterus. Once the shunt is implanted, it remains in place until the baby is born.
3. Fetal lobectomy and laser ablation of single feeding vessels are intervention options in other countries or regions, and relevant treatments are not implemented in my country.
Removal of abnormal tissue to prevent malignant transformation
Asked whether lung malformations require surgical treatment?
“Let’s talk about CPAM first. If symptoms or lung infection develop at this time, it is highly recommended to remove the affected lobe. Remember, every lung infection (pneumonia) causes scarring internally, which will make the surgery more difficult. difficulty.”
He said that removing abnormal lung tissue can reduce the risk of infection and malignant transformation. If the lesions are large, they may compress functioning lung tissue or other structures such as the heart.
In addition, if surgery can be performed as early as possible, compensatory lung growth can occur. Basically, lung tissue continues to mature and grow until about 2 years of age. Before this period is up, removing the lung allows the lung to regenerate as if it had never been removed.
Although the risks of surgery are minimal, pre-evaluation is important because the benefits of surgical treatment should far outweigh the risks of leaving abnormal lung tissue behind.
Infected and malignant large cysts should be removed
He said that in the previous article we mentioned 5 types of CPAM. Current medical evidence shows that type I is a large cyst with a risk of infection and malignant transformation, so it is best to undergo surgical removal.
“Type II cysts are smaller and have a lower risk of infection or malignant transformation, so removal is recommended when the cyst is large or causes symptoms. Type III CPAMs are solid structures and have a low risk of infection or malignant transformation, but they are usually Large, so excision is recommended if it is large or causes symptoms.”
“Type IV CPAM is best treated surgically because there are large cysts in the periphery of the lungs, which carry a high risk of infection, malignancy and pneumothorax. Type 0 is very rare and usually affects the entire lung, so individual evaluation and management is required. “
He reminded that congenital lobar emphysema (CLE) can gradually worsen over time, so it is best to monitor closely. A simple chest X-ray is sufficient at your follow-up visit, as changes can usually be seen this way. Patients often require a lobectomy to prevent lung infection and worsening emphysema.
Avoid affecting heart function
Minimally invasive surgery suitable for BPS
Bronchopulmonary sequestration (BPS) may cause pressure effects on nearby organs or, more commonly, vascular problems.
Resection is often recommended based on the long-term effects on heart function.
In recent years, embolization to block the feeding vessels and aorta has been tried in BPS. However, the long-term results of this technique are unclear, so most surgeons are reluctant to use this treatment without actual resection. method is worrying.
Surgery is recommended between 8 and 10 months old
Nada said it is generally recommended that babies with lung malformations undergo surgery when they are 8 to 10 months old. The timing is good because the baby is strong enough to undergo elective surgery and there is enough time for new lung tissue to regenerate after the lobe is removed.
Lobectomy or BPS resection can be performed by traditional open surgery, known as thoracotomy, or minimally invasive surgery, known as thoracoscopic approach.
Thoracotomy is a 4cm wide incision made on the side of the chest parallel to the ribs; thoracoscopic surgery involves 3 or 4 incisions, but most are 3mm wide except for one which will be ‘stretched’ to 1cm length to remove the lung lobes.
Here, the surgeon uses a small “telescope” in the chest to visualize the area involved, and a few (2 or 3) instruments are required to finely separate and dissect the affected lobe or free lung (sequestration). The tissue will be gently removed from the chest and the inside of the incision will be closed with some sutures and the skin will be glued.
Children do not experience much pain after surgery
He said the advantage of minimally invasive or keyhole surgery is that the child will not experience much pain after the operation. If nothing comes out of the chest, the drain will be removed the next day. The average postoperative hospital stay was 3 days, and surprisingly the children were back to normal functions by this time.
The child needs a follow-up visit 1 week after the operation. The doctor will examine the wound and explain the histological report (laboratory analysis) of the removed lung tissue. After 3 months, another evaluation will be performed by a respiratory physician. Most children will have new lung tissue growing by age 2, and medications usually are not needed.
However, a small number of people may need inhalers to help them breathe. Therefore, it is important to see a pediatric respiratory medicine physician, and the doctor should confirm that the child’s lung function is normal before leaving the hospital. All in all, the entire process of the lobectomy and subsequent recovery was very good.
Abnormal lung development is not hereditary
Question 1. Are lung malformations hereditary? Is there still a risk for the next pregnancy?
Answer 1.Lung malformations are not hereditary, but are caused by abnormalities in the development of the lungs. This is just a by chance event of the pregnancy. Therefore, the chances of it happening again are extremely slim.
Although most pulmonary abnormalities detected prenatally are asymptomatic after birth, especially for smaller lesions (CVR less than 1), it is generally recommended that good neonatal care be provided or a pediatric surgeon is available. Supported medical center delivery.
Fetuses with large lesions may need MRI
Question 2. Ultrasonic testing can measure CVR and is used to diagnose CPAM and BPS. Under what circumstances are other prenatal tests needed?
Answer 2.For some patients with larger lesions, fetal MRI may be required to further understand the shape, size and impact of the lesions on other structures. A fetal echocardiogram is used to evaluate how well the heart is working when there is a problem.
Fetuses with lung abnormalities usually have normal chromosomes, and amniocentesis is recommended only if other problems are present.