Postnatal Management of Lung Lesions Part III: Pediatric Thoracic Surgery...
With Dr. Todd Ponsky · StayCurrentMD
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
There are several cases of prenatally diagnosed lesions that turned out to be PPB after birth.
If you do thoracoscopic resection, put the specimen in a bag before extraction, because if it turns out to be PPB and you mush it up, there is a risk of recurrence.
Extralobar sequestrations with no communication and no air on CT scan do not have a high infection rate; hematogenous infection is possible but rare.
Malignant transformation of extralobar sequestration is extremely rare, with maybe one or two cases of squamous cell carcinoma in world literature.
Infection and cancer are not good arguments to resect a non-communicating extralobar sequestration.
The incidence of infection and malignancy in extralobar sequestration is very low, but it has occurred.
Imaging is not perfect and we cannot always be absolutely sure of the diagnosis or whether it is a hybrid lesion.
The morbidity of resecting extralobar sequestration is so low that removal is favored.
There is no role for embolization of extralobar sequestrations; it is much easier to resect them using minimally invasive techniques.
The differential diagnosis for infradiaphragmatic lesions prenatally is adrenal hemorrhage or neuroblastoma (cystic neuroblastoma).
Small infradiaphragmatic lesions can be followed by ultrasound; if they stay the same or get smaller, they do not require resection.
There is no good way to follow pulmonary lesions; CT scan is the best method but induces a certain incidence of malignancy.
You cannot differentiate CCAM from PPB on CT scan until you have a stage two or three occurrence.
You cannot counsel a family, say the word cancer, and have them watch; that is almost universally unacceptable.
When deciding to operate or not, we must weigh the risks of not doing the operation versus the risks of doing the operation.
There are children who die from pulmonary lobectomy, particularly done thoracoscopically.
Jack knows of two cases of death from thoracoscopic lobectomy that he was asked to review.
Thoracoscopic lobectomy should not be done unless the surgeon is experienced; in experienced hands it should have no more morbidity than open.
The mortality for thoracoscopic lobectomy in experienced hands should be zero.
If you do enough of any operation, you will have significant complications, whether open or thoracoscopic.
If you have massive bleeding when already open, your chance of salvaging the situation is probably better than if you have that bleeding in a thoracoscopic case.
PPB can be indistinguishable radiologically from CCAM, but the incidence of PPB remains extremely rare.
In a high-volume center (Toronto), de novo PPB is seen extremely rarely, like once every 3 or 4 years, while 20-25 new CCAM cases are seen every year.
The estimate of lifelong risk of infection for CCAM is somewhere around 20 or 30%.
Most infections in CCAM can be treated and lobectomy can be done afterwards.
Jack's data showed 10% infection rate with a mean follow-up of 4 years, which was tripled to estimate 30% lifetime risk.
Before prenatal diagnosis, it was not common for people to present in teenage years or adulthood with symptomatic infected CCAMs, though it happened from time to time.
If 25 new prenatally diagnosed asymptomatic CCAM cases are seen per year in Toronto and none were operated on, we should be seeing 25 infected cases per year if the infection rate were 100%.
The majority of CCAMs do become symptomatic; it is not a normal variant.
Toronto follows all prenatally diagnosed lesions with CT postnatally, and those not operated on are followed with chest X-ray and another CT.
Jack is not advocating non-operative management of all CCAMs; he counsels families about risks including cancer, and many choose surgery.
Jack advocates a balanced approach to families, allowing them to decide, rather than operating on every single lesion.
There is a hidden mortality in pediatric surgery because people do not report bad results; the only way to know is through lawsuits or future unbiased registry data like NSQIP.
We do not have the data to make truly informed consent, so we must give families the whole picture and let them decide.
Centers that do thoracoscopic lobectomy routinely and see high volumes of CCAMs can treat them with extremely low morbidity and no mortality.
In a review of the last 100 thoracoscopic lobectomies (not sequestrations), there were 2 transfusions and 2 latent pneumothoraces as the only complications.
For infected CCAM with abscess, 6 weeks of IV antibiotics is reasonable, but duration depends on symptoms (fever, ongoing symptoms).
Large abscesses in CCAM can be drained percutaneously and treated with antibiotics for a couple of weeks, then re-evaluated for residual mass.
It is very hard to clear infection from a macrocystic CCAM.
MRI is not used as primary study because children require more anesthesia for MRI than for CT.
MRI has been tried for follow-up of non-operated patients, but it does not give as clear a picture as CT.
Bronchial blocker is not needed in infants for single-lung ventilation and is potentially harmful (risk of bronchial stenosis).
The incidence of extralobar sequestration in diaphragmatic hernia is probably 15%; they are often small and can be ignored.
Bilateral lung lesions are managed sequentially, not at the same time.
Prophylactic antibiotics are given as one preoperative dose only; patients are not sent home on antibiotics if waiting a month for surgery.
Macrocystic lesions can always be reduced thoracoscopically by chipping away at them with a ligature.
Most CCAM specimens can be removed through a 5 millimeter incision, though hybrid lesions with big arteries are tougher.
You never lose anything by putting a scope in; you can evaluate thoracoscopically and convert to open if the fissure is obliterated or anatomy is difficult.
Most people believe now that CCAMs do not become PPB, but PPB is a de novo tumor that is cystic and cannot be differentiated on imaging.
One series out of Toronto estimates that cystic lesions that look like CCAM, about 4% of them will actually turn out to be PPB.
There is about a 1% risk of bronchioalveolar carcinoma in the teenage years or early adulthood.
The COG study showed that it was safe to observe adrenal masses (in the context of neuroblastoma concern).
The paper by Peter Kim used the Stalker classification in which one classification has been termed PPB, but the natural history of that histological finding is unknown.
The only prospective study that followed patients long-term showed 18 of 21 asymptomatic patients developed symptomatology during an interval averaging 2 years up to 13 years.
Autopsy series show that small asymptomatic extralobar sequestration is a relatively known finding, but asymptomatic CCAM on autopsy is nonexistent.