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A congenital pulmonary airway malformation CPAM is a mass of abnormal fetal lung tissue that forms during pregnancy. This mass, or lesion, is usually located in one lung and it does not function as normal lung tissue. Lesions vary in size and appearance and can change significantly during the pregnancy.
A baby with CPAM can have one lesion or many. These lesions can be solid or filled with fluid. The condition is relatively rare, affecting about 1 in 25, pregnancies. It is slightly more common in males than in females. CPAM is not hereditary, so it usually does not recur in families.
Our team has evaluated more than babies with CPAM since We know that a diagnosis of CPAM is an emotional experience for families, and in some cases difficult decisions about treatment must be made quickly. Show For most babies with a congenital pulmonary airway malformation, the condition is not life threatening.
Sometimes, the lesions decrease in size or can no longer be seen during the pregnancy. Other times, the lungs develop normally despite the presence of these lesions. In about 10 percent of cases, CPAM can cause serious problems for the baby and sometimes, for the mother as well. The most serious problems occur when babies develop hydrops, a prenatal form of heart failure characterized by fluid build-up in the skin, chest or abdomen. Babies who have CPAM and hydrops typically will not survive unless they can respond quickly to medicine or undergo fetal surgery to remove the lesions.
The mother can develop high blood pressure and fluid retention. Additional tests can confirm the diagnosis, evaluate how severe the CPAM is, and identify any other problems. The patient meets regularly with members of our team, who include a maternal-fetal medicine specialist, a surgeon and a nurse coordinator. Their goal is to explain all test results thoroughly and work with the patient to establish a comprehensive treatment plan.
Weekly ultrasound tests are very helpful because they can show whether the lesions are growing and whether hydrops is developing. A CVR of less than 1. Another indicator of serious problems is swelling in the placenta. In this case, our team will meet with the patient to discuss the option of immediate delivery. In most cases, the growth of the lesion stops at about the 26th week of pregnancy. Babies who reach this important milestone and have not developed hydrops are no longer at risk for this dangerous condition.
These include the size and number of lesions, whether the lesions are solid or filled with fluid, and whether hydrops or mirror syndrome has developed. Steroid Treatment When the lesions are solid or are growing too quickly, our team may recommend steroid treatment as a first step.
Steroids can slow the growth of the lesions, possibly allowing patients to avoid fetal surgery. Thoracentesis When large, fluid-filled cysts are present, the medical team may recommend draining them using thoracentesis. This involves inserting a needle through the womb into the lesion and draining the fluid. To prevent future fluid build-up, the surgeon may place a thoracoamniotic shunt, or catheter, to continue draining fluid from the lesion into the amniotic cavity.
Fetal Surgery If large, solid lesions are present and severe hydrops has developed, the team may recommend open fetal surgery to remove the lesions. Early Delivery If mirror syndrome has occurred, our team will recommend early delivery to protect the life of the mother. Treatment Options for Newborns with CPAMShow Babies born with congenital pulmonary airway malformation are at high risk for complications during and after delivery.
Complications can include breathing issues, underdeveloped lungs and high blood pressure, among others. Managing these problems is the first and most important step in caring for babies with CPAM after birth. Therefore, all babies with CPAM should be delivered at a center with a neonatal intensive care unit and pediatric surgical services. Lesions should be removed surgically since they can become infected or, in rare cases, become cancerous. If the baby has no breathing problems, he or she may be discharged home and scheduled for surgery sometime within the first year of life.
In some cases, the lesions are very large and interfere with normal breathing, so surgery must take place immediately after birth. This involves delivering the baby by Cesarean and immediately performing surgery while the baby is still connected to the placenta. Even if immediate surgery is not necessary, some babies with CPAM will experience breathing problems at birth. They may need to be placed on a ventilator or have extracorporeal membrane oxygenation ECMO , a type of heart-lung bypass machine.
In rare situations, patients who had large lesions may have small lungs and subsequent chronic lung disease. They provide long-term follow-up programs focused to help children reach their full potential. Since , physicians at the Cincinnati Fetal Center have evaluated more than patients for congenital pulmonary airway malformation. Our team offers a comprehensive, interdisciplinary strategy for diagnosing and treating this condition.
The same team of physicians cares for patients from diagnosis to delivery. Sophisticated treatment options. Our experience with CPAM means that we can provide the most effective therapies available. Family-centered care. The Cincinnati Fetal Center team works closely with referring obstetricians and maternal-fetal medicine specialists to facilitate prenatal management, and with pediatricians who will provide primary care once the baby has been discharged home.
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Nomenclatures et codage
Congenital Pulmonary Airway Malformation