Postnatal Management of Lung Lesions Part I: Pediatric Thoracic Surgery Part...
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
At 32 weeks gestation, ultrasound is performed to prognosticate whether the fetus will be asymptomatic or symptomatic at birth based on mediastinal shift.
If there is no mediastinal shift and a relatively small lesion at 32 weeks, the child is considered likely to be asymptomatic at birth and can deliver close to home with elective resection after CT scan.
If mediastinal shift is present at 32 weeks without major cardiac or lung compression, delivery should occur at a surgical center with postnatal resection during the first few days of life.
Major mediastinal shift with lung or cardiac compression requires delivery at a tertiary center with EXIT procedure or ECMO availability.
All CPAMs, intralobar BPS, and hybrid lesions should be resected routinely.
Extralobar BPS in some cases do not need resection and can be observed.
CPAMs are prone to infection and routinely have mucoid stasis; many show inflammatory evidence at 2-3 months of age when resected.
CPAMs have established malignant potential, with reported cases of pleuropulmonary blastoma, bronchoalveolar carcinoma, and rhabdomyosarcoma.
Pleuropulmonary blastoma cannot be differentiated radiologically from CPAM and is highly malignant and lethal if allowed to progress beyond stage 1.
The speaker personally resected three CPAMs that turned out to be stage 1 pleuropulmonary blastomas, which could not be differentiated from relatively small asymptomatic CPAMs.
There is anatomical and histological overlap between congenital lung lesion types; they exist on a continuum rather than as discrete categories.
A sequestration lesion with small cysts cannot be assumed to lack CPAM histology based on imaging alone.
Intralobar BPS can have communications via the pores of Kohn leading to infection risk and potential for high-flow physiology.
Waiting until 4-5 months of age for resection can result in significant inflammation within fissures that changes the complexion of the dissection.
Earlier resection is surgically easier and less traumatic for the infant than later resection.
Thoracoscopic resection has a difficult learning curve but is superior to open thoracotomy with shorter hospital stay, less pain, no thoracotomy morbidity, and better cosmesis.
Segmental bronchial stenoses have a very confluent appearance, almost like emphysematous pulmonary parenchyma, and may not require routine resection, though this is controversial.
If any cysts are visible in a segmental bronchial stenosis, that usually indicates CPAM histology.
Imaging studies are not good enough to definitively differentiate bronchial atresia from CPAM; lesions thought to be bronchial atresia can have CPAM elements and vice versa.
CPAMs often have abnormal fissures and globulated appearance related to developmental effects, so normal anatomy cannot be depended upon during lobectomy.
Some prenatally large CPAMs regress predominantly by birth but can air-trap and enlarge in the first weeks of life, requiring resection when symptoms develop.
Feeding vessels in sequestrations can arise from essentially any systemic source, not just the thoracic or abdominal aorta.
Some sequestrations have abnormal pleural investments or pleural caps that must be dissected during resection.
The speaker had a pulmonary artery hemorrhage when a ligature seal broke down during lobectomy, leading to conversion to open thoracotomy.
After the hemorrhage experience, the speaker tied every pulmonary artery and ligatured distally until the newer Force Triad energy source became available.
Tying all vessels intracorporeally takes about twice as long as using energy devices but is a safe alternative.
Many sequestrations are edematous due to restricted venous and lymphatic outflow, often with associated pleural effusions.
Edematous extralobar BPS typically have small vascular pedicles and are very easy to resect.
Intralobar sequestrations can drain almost directly into a pulmonary vein, creating a very high-output potential shunt that can lead to cardiac failure by 3-4 years of age.
Feeding vessels in sequestrations are often abnormal, tortuous, and have abnormal integrity similar to PDAs; they can cut through if tied too tightly or clipped too firmly.
Arterialization of vasculature occurs in sequestrations, with even pulmonary veins becoming thickened like arteries due to high flow.
There are often multiple feeding vessels to sequestrations; what looks like one vessel on CT scan may be three or four vessels, or they may branch very early.
When taking the inferior pulmonary ligament during lobectomy, careful inspection is needed because blood flow through small systemic vessels can be phenomenal.
If a systemic feeding vessel is missed and the pulmonary vein is taken first, tremendous congestion of the lobe will occur.
In high-output sequestrations, the pulmonary vein dilates dramatically and can become huge.
Extralobar BPS have separate pleural investment, no bronchial connection, systemic arterial supply from almost any source, and systemic or pulmonary venous drainage.
Extralobar BPS can have CPAM histology, usually indicated by a visible cyst on imaging.
Extralobar BPS have mucostasis but no airway communication, so there is no infection risk from that route.
Extralobar BPS can get infected, likely via hematogenous route, and present as pneumonias; infected lesions are much more difficult to resect.
A purely extralobar sequestration that is prenatally diagnosed is a relatively straightforward case and a great place to start when learning thoracoscopic resection.
Extralobar BPS could potentially be done as outpatient procedures with two ports and no chest tube.
The speaker previously thought intralobar and extralobar BPS could be differentiated by pulmonary versus systemic venous drainage, but has encountered extralobar BPS with pulmonary venous drainage.
Some sequestrations are essentially pure AV fistulas—a small piece of lung tissue with large blood vessels going in and out.
Sequestrations with esophageal bronchi are cesspools full of mucoid material.
When a sequestration is against the esophagus and difficult to separate, an esophageal bronchus should be suspected.
Esophageal bronchi can be small and may be missed if the surgeon is not aware they may be present.