Should We Resect Asymptomatic CPAM Flake vs Langer
With Dr. Jean Martin · StayCurrentMD
Cued at 9:30 · stops at 10:15 · press play
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
CT scan is the best method to follow pulmonary lesions but induces a certain incidence of malignancy.
CPAM cannot be differentiated from pleuropulmonary blastoma (PPB) on imaging until stage 2 or 3 disease occurs.
Surveillance cannot prevent malignancy or allow earlier detection than would otherwise occur.
Families cannot be counseled to watch a lesion once the word cancer is mentioned.
Children have died from pulmonary lobectomy, particularly done thoracoscopically.
Jack (spk_3) has reviewed two cases of mortality from thoracoscopic lobectomy, not at his own institution.
In experienced hands, thoracoscopic lobectomy should have no more morbidity than open lobectomy.
Thoracoscopic lobectomy should only be performed by surgeons with advanced thoracic and minimally invasive skills, and mortality should be zero.
If massive bleeding occurs during thoracoscopic lobectomy, the chance of salvage is probably lower than if the case is already open.
Pleuropulmonary blastoma (PPB) can be indistinguishable radiologically from CPAM.
The incidence of CPAM is markedly higher than the incidence of PPB; PPBs remain extremely rare.
In a high-volume center, de novo PPB is seen extremely rarely, approximately once every 3 to 4 years, while 20 to 25 new cases of CPAM are seen annually.
The incidence of cancer in CPAM is extremely low and must be balanced against the risk of lobectomy.
The estimated lifelong risk of infection for untreated CPAM is approximately 20 to 30%, based on institutional data showing 10% infection at a mean follow-up of 4 years.
Most infections in CPAM can be treated, and lobectomy can be performed afterwards rather than prophylactically.
Before prenatal diagnosis, it was not common for teenagers or adults to present with symptomatic infected CPAMs, despite the lesions being common prenatally.
In early career in San Francisco, infected CPAMs were not a rare event.
If 25 new prenatally diagnosed asymptomatic CPAMs are seen per year in the Toronto area and none are operated on, one would expect to see 25 infected cases per year if the infection rate is 100%.
The majority of CPAMs become symptomatic; CPAM is not a normal variant.
Jack's institution follows all prenatally diagnosed cystic lung lesions with postnatal CT, and those not operated on are followed with chest X-ray and repeat CT.
Jack does not advocate non-operative management of all CPAMs; he counsels families about risks including cancer and infection, and many choose surgery after hearing the risks.
A balanced approach should be given to families, allowing them to decide, rather than a dogmatic approach that every CPAM needs operation.
In an audience poll, 23-25% of respondents do not advocate routine resection of asymptomatic CPAM.
There is a hidden mortality in pediatric surgery because people do not report bad results; the only way to know about them is through lawsuits.
In 10 years, participation in the American College of Surgeons Pediatric NSQIP will provide unbiased large-scale data on thoracoscopic and open surgery complications and mortality.
Currently, there is insufficient data to make fully informed consent regarding CPAM management.
Thoracoscopic lobectomy requires a major investment in time and learning to become proficient.
Centers that routinely perform thoracoscopic lobectomy and see high volumes of CPAMs can treat them with extremely low morbidity and no mortality.
Infants undergoing thoracoscopic lobectomy are generally in the hospital for 2 days.
In a review of the last 100 thoracoscopic lobectomies (not sequestrations), there were 2 transfusions and 2 prolonged air leaks as the only complications.
Peter Kim published a paper on histological evaluation of pulmonary lesions using the Stocker classification, in which one classification has been termed PPB, but the natural history of that histological finding is unknown.
A prospective study (Wong, Pediatric Surgery International) followed 21 asymptomatic patients (8 prenatally diagnosed, rest serendipitous) and found 18 of 21 developed infections or symptoms requiring resection, with mean follow-up of 2 years and range up to 13 years.
Small asymptomatic extralobar sequestrations are a relatively known finding at autopsy, but asymptomatic CPAMs are nonexistent in autopsy series.