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Prenatal Management of CPAMs: Lung Lesions
Educational content from recorded physician discussions — not medical advice. Talk to your (or your child's) care team about your situation.
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What the experts said
Prenatal diagnosis has had a tremendous impact on the understanding and management of lung lesions.
Right CDHs are frequently misdiagnosed as CPAMs and vice versa because of the similar echogenicity of the liver and the right chest.
Steroids have reduced the need for surgical fetal intervention to almost zero in most fetal centers over the past 4-7 years for microcystic CPAMs when hydrops is threatened.
The CCAM volume ratio (CVR) is the volume of an ellipse (three dimensional volume of the CPAM) over the head circumference to standardize for gestational age.
CVR has proven to be probably the most valuable prognostic indicator for CPAMs both retrospectively and prospectively.
CPAMs typically grow up until about 25 to 28 weeks when they tend to plateau, and then they'll actually regress in size very often.
If the CVR is less than 1.6 with a solid lesion at presentation, there's less than a 3% chance of that lesion progressing to hydrops.
CVR of greater than 1.6 has a very high likelihood of developing hydrops, as high as 75%.
At the speaker's center, lesions with CVR greater than 1.6 are treated with steroids prophylactically to try to avoid heart failure.
The presented case is probably the only case in the last 5 years of a microcystic CPAM that didn't respond to steroids at the speaker's center.
Complete uterine relaxation with deep inhalational anesthetic is required before touching the uterus in fetal surgery.
If there is an anterior placenta, a posterior uterine incision is required, which necessitates dividing the rectus muscle and doing a bigger abdominal incision.
IV access is extremely important in fetal surgery to allow resuscitation, transfusions, and other interventions.
Continuous echocardiographic monitoring of the fetal heart by a scrubbed cardiologist is an invaluable component of fetal surgery.
When tumors are decompressed out of the chest during fetal surgery, the fetus can become bradycardic due to loss of preload on the heart.
Fetuses are usually preloaded via IV before chest decompression to prevent bradycardia.
Amniotic fluid is replaced with warm lactated Ringer solution during fetal surgery.
A three-layer closure of the uterus is performed after fetal surgery to prevent amniotic fluid leakage and control the membranes.
Open fetal surgery is required very rarely for lung lesions.
The fetus in the presented case was delivered at 35 weeks without ventilation required and showed good compensatory lung growth at 3.5 weeks post-op.
At CHOP, overall survival for open fetal surgery for lung lesions is around 60%, consistent over all years.
Survivors of open fetal surgery have good quality of life outcomes with no major neurologic sequelae or other bad quality of life impacts.
Losses in open fetal surgery are usually kids that have gone too far with their hydrops and can't tolerate the procedure or have early preterm labor.
Macrocystic CPAMs are less predictable, and even patients with CVR less than 1.6 will occasionally grow rapidly and induce hydrops.
In the presented macrocystic CPAM case, a thoracoamniotic shunt converted CVR from 3.6 to 0.8 and reversed the associated hydrops.
Many CPAMs have a large solid component, and the shunt can't entirely decompress that, but can still reverse the hydrops.
The shunt experience has been about 70% survival.
Losses after shunt placement are usually due to early delivery and associated prematurity, inadequate compensatory lung growth, or sometimes residual mass effect requiring invasive treatment.
Marked chest wall deformity can occur if shunts are placed for giant macrocystic CPAMs early in gestation, most prominent when shunts are done at 18 to 20 weeks.
Chest wall deformity from early shunt placement is related to collapse of the chest wall as opposed to the harpoon placement of the shunt.
Chest wall deformity from shunt placement becomes less pronounced over time, and thus far affected children haven't required chest reconstructive procedures.
CPAMs with multiple macrocysts generally communicate, so placement of a shunt can decompress those lesions dramatically.
Ascites alone is not considered hydrops.
The EXIT procedure is designed to maintain uteroplacental blood flow during delivery and resection of the mass.
Infants can be maintained with normal blood gases for an hour and a half by a well-performed EXIT procedure.
CHOP has performed 16 EXIT procedures for lung lesions, with 4 requiring ECMO and 15 survivors.
ECMO is a very unusual requirement for lung lesions because of their late enlargement, so they don't have the same effect on lung hypoplasia as CDH.
Fetal intervention with twins is generally considered a contraindication.
EXIT procedures are much trickier when you have twin gestations.
The majority of kids with large CPAMs at birth don't need EXIT procedures and can be managed with C-section and immediate resection.
EXIT procedures are reserved for kids where you see mass effect with diaphragmatic aversion, can't visualize the opposite lung easily, or have dramatic mediastinal shift.