Congenital Cystic Adenomatoid Malformation (CPAM/CCAM) and Bronchopulmonary Sequestration (BPS)

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What are Congenital Cystic Adenomatoid Malformation (CPAM/CCAM) & Bronchopulmonary Sequestration (BPS):

CPAM/CCAM and BPS are both benign lung tumors. CPAM/CCAM is a cystic overgrowth of part of the lung.  It can appear with large “macro” cysts (type I), small “micro” cysts (type lll) or a mixture of small and large cysts (type II). . In BPS, part of the lung tissue, has a blood supply but is not connected to any airway.  We do not know what causes one part of the lung to develop abnormally, but the cystic tissue will never function as normal lung

Both CPAM/CCAM and BPS can be identified during a routine prenatal ultrasound (at ~ 18-20 weeks gestation) and will appear either as a bright mass or a dark cystic mass within the fetal chest. A BPS may occasionally be accompanied by a small amount of fluid in the chest. In most BPS’s, a feeding blood vessel can be seen coming from the aorta.

The cause of CPAM/CCAM and BPS is unknown. They appear to be random events that can happen to anyone.

What is the outcome for a fetus with CPAM/CCAM or BPS?

Most commonly, CPAM/CCAM’s and sequestrations are small. As such, they will not compromise the function and development of the fetal heart or lungs. In the third trimester of pregnancy, 50% of CPAM/CCAM’s decrease in size and some may even disappear before birth. When the lung mass is small or moderate in size, the outlook for a normal life is excellent.

Rarely, the fetal lung mass can be very large and may compromise fetal heart function or lung development. This may become evident by the accumulation of fluid in the fetal skin, abdomen or chest (hydrops). Fetuses with this condition are very sick and, without in utero treatment, may not survive. However, the vast majority of fetuses with either BPS or CPAM/CCAM do very well.

What other tests should be considered?

Every fetus with a suspected lung mass should have a detailed ultrasound to determine the exact nature and size of the mass and to examine the other fetal organs. Sometimes, a fetal heart scan (echocardiogram) may be ordered. Ongoing ultrasounds are used to monitor the growth of the lung mass and to look for early signs of heart failure.

What therapy is available?

In fetuses with small or moderate sized lung masses, no therapy is required before birth. The fetal medicine specialist will be able to determine whether the baby can be born in a local hospital or needs delivery in a level III hospital, such as Mount Sinai. After birth, the baby will need to have a chest X-Ray soon after birth and, usually, a CT scan at approximately 3 months of age, at which point the baby will be seen in the paediatric surgery clinic at Sick Kids hospital.  Depending on your baby’s symptoms, the lung mass may or may not need to be surgically removed after birth. The long-term outcome of these children is normal.

In large fluid filled CPAM/CCAM’s, doctors may suggest draining the cysts with a fine needle or inserting a shunt  to drain away the extra fluid. In a sick baby with a BPS, a special procedure to close off the feeding blood vessel may need to be considered.

Babies with a large CPAM/CCAM or BPS should be born at a hospital with a neonatologist (a paediatrician with expertise in newborn babies) and have follow-up with a paediatric surgeon. Babies with large lung masses may require care in a neonatal intensive care unit (NICU).

Special Pregnancy Program

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Fetal Medicine: 416-586-4800 x 7756
Fax: 416-586-3216

Maternal Medicine: 416-586-4800 x 7000
Fax: 416-586-5109

Main clinic hours: Monday to Friday, 8am to 4pm

Prenatal Diagnosis & Medical Genetics

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phone: 416-586-4800 x 4523
fax numbers:
416-586-4723 or 416-586-8384

Perinatal Mental Health

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Phone: 416-586-4800 x 8325
Fax: 416-586-8596