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Dr. Todd Ponsky

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Postnatal Management of Lung Lesions Part II: Pediatric Thoracic Surgery...

Video Published 2018-09-16 Updated 2022-08-22

Timestops (8)

Pulmonary Venous Anatomy and Sequestrations

Infradiaphragmatic Sequestration Case

Bronchial Atresia and Cystic Lesions

Surgical Technique and Instrumentation

Clips Versus Energy Devices

Fetal Intervention and Pathology Considerations

Timing of Resection and Outcomes

Pneumothorax Pressures and Small Infants

Topic Overview

A surgical discussion on postnatal management of congenital lung lesions, focusing on bronchopulmonary sequestrations (BPS), congenital pulmonary airway malformations (CPAM/CCAM), and bronchogenic cysts. The speakers review anatomic variants including infra-diaphragmatic and intra-diaphragmatic sequestrations, discuss optimal timing for resection (typically before 3 months of age), and address technical considerations for thoracoscopic lobectomy in small infants. Key clinical points include the importance of identifying abnormal vascular anatomy on CT, the risk of misdiagnosis when lesions are located near the diaphragm, and the finding that delayed resection (up to 18 months) does not significantly impact long-term pulmonary function but increases risk of complications like infection.

Key Takeaways

Resect CPAMs before 3 months: delayed surgery increases infection risk without improving long-term pulmonary function. (15:33)
Infra-diaphragmatic sequestrations often extend through esophageal hiatus and can cause esophageal obstruction. (0:51)
Thoracoscopic lobectomy is easier in younger infants but requires >2kg weight and stable single-lung ventilation. (17:44)
Bronchogenic cysts with upper lobe obstruction require lobectomy, not just cyst resection, due to bronchial destruction. (8:32)
Use vessel sealing over clips in thoracoscopic resection: clips can dislodge during dissection, sealed vessels cannot. (11:50)

Inside this episode

Kai, the Library's AI content creator, listened to this episode and mapped who's speaking, the chapters, key claims, and cases. Every item links to the exact moment in the recording.

AI-enriched

Who's speaking

Alan — guest
Steve — guest
Speaker 3 — host
Speaker 4 — guest
Shari — guest
Liam — guest

Chapters

0:00Anatomic Variants of Pulmonary Sequestrations — Review of abnormal vascular patterns in sequestrations, including pulmonary venous drainage variants, extra-lobar sequestrations with systemic arterial inflow and azygous outflow, and infra-diaphragmatic lesions causing esophageal obstruction.
3:50Infra-diaphragmatic BPS Case Demonstration — Laparoscopic resection of an infra-diaphragmatic BPS with CPAM histology causing esophageal obstruction. Discussion of technical challenges including adherence to surrounding structures, vascular pedicles from celiac axis, and extension through esophageal hiatus.
7:20Bronchogenic Cysts and Bronchial Atresia — Overview of bronchogenic cysts associated with bronchial obstruction, hyperplastic distal lobe growth, and technical approach to upper lobe lobectomy when cyst obstructs lobar bronchus. Discussion of energy device technique and vessel sealing.
11:00Q&A: Clips, Ligatures, and Referral Centers — Panel discussion on vessel management techniques (clips vs. ligatures), risk of clip dislodgement during dissection, and US referral centers for advanced fetal lung lesion care (UCSF, Cincinnati, Houston).
15:00Timing of CPAM Resection — Discussion of optimal timing for CPAM resection, citing recent literature showing no significant difference in long-term pulmonary function between early (3 months) and later (18 months) resection, but increased complication risk with delay. Compensatory lung growth continues until age 4-6 years.
18:10Technical Considerations for Small Infants — Discussion of lower size limits for thoracoscopic resection (approximately 2 kg for lobectomy, 800g for PDA ligation), limitations imposed by current 5mm instrument technology, and importance of trocar positioning in small spaces.
22:00Management of Complex Anatomy — Discussion of bilobar involvement, abnormal fissure formation, and approach to creating appropriate resection planes while preserving lung parenchyma. Rare need for re-resection of residual cystic lesions.

Key claims

0:00Sequestrations can have abnormal pulmonary venous drainage patterns visible on CT — Alan
0:19Lung lesions are frequently misdiagnosed as intra-diaphragmatic or sub-diaphragmatic when they are actually in the chest — Steve
0:30Some infra-diaphragmatic lesions are approached laparoscopically — Steve
0:39A new class of lesions exists within the leaves of the diaphragm muscle requiring opening of the diaphragm for access — Steve
0:51Infra-diaphragmatic sequestrations are often located at the GE junction or esophageal hiatus — Alan
0:56Infra-diaphragmatic sequestrations can cause esophageal obstructive symptomatology — Alan
1:18Extra-lobar sequestrations can have systemic arterial inflow and distal azygous outflow — Alan
1:52Extra-lobar sequestrations are completely separately invested by pleura with no parenchymal connection — Alan
4:45The majority of infra-diaphragmatic lesions approached through the abdomen extend up through the esophageal hiatus — Steve
7:20Bronchogenic cysts can be alone or associated with bronchial obstruction — Alan
7:29Hyperplastic growth of the distal lobe is often seen when bronchogenic cysts are associated with bronchial atresia — Alan
16:27Alveolarization phase of lung development continues until 4 to 6 years of age — Alan
15:33Recent study showed increased risk of complications like infection with delayed resection but no difference in long-term pulmonary function — Alan
16:43Resection timing probably doesn't matter from a lung growth perspective if done in the first 3 months — Alan
17:38Most CPAM lesions are removed before 3 months of age at Steve's center — Steve
17:44Thoracoscopic resection is technically easier in younger infants, though requires comfort working in small spaces — Steve
17:51Children recover quicker from thoracoscopic resection at younger ages with shorter hospitalization — Steve
18:15Setup and trocar positioning is critical for thoracoscopic surgery in small neonates — Steve
19:12CO2 pressure of 7 centimeters of water is typically used for thoracoscopic lung resection — Alan
19:27Starting CO2 pressure of 4 is adequate if good single lung ventilation is achieved — Steve
19:55Mild tension pneumothorax from CO2 insufflation is well tolerated in infants — Steve
20:49Current technology limits thoracoscopic lobectomy to infants over approximately 2 kg — Steve
20:49PDA ligations are now routinely done thoracoscopically down to 800g — Steve
21:325mm clip applicators take up 2/3 of the chest in very small infants limiting maneuverability — Steve
22:08Thoracoscopic lobectomy requires a healthy stable baby with adequate contralateral lung for single lung ventilation — Alan
22:44Stable babies with small lesions can be sent home to grow for 1-2 months before returning for resection — Steve
24:13True bilobar involvement of CPAM is relatively rare — Alan
24:18Most apparent bilobar involvement is actually abnormalities of lobulation or fissure formation — Alan
11:50Clips on vessels can be dislodged during subsequent dissection — Steve
12:02Sealed vessels cannot be dislodged during dissection unlike clips — Steve
14:15Good histologic diagnoses can be obtained from thoracoscopic resections despite pathologist concerns about specimen quality — Alan
8:32When bronchogenic cyst obstructs upper lobe bronchus, the bronchus is destroyed enough that lobectomy is required, not just cyst resection — Alan

Cases discussed

4:10Infra-diaphragmatic BPS with CPAM histology causing esophageal obstruction in 5-week-old infant
1:18Extra-lobar sequestration with systemic arterial inflow and azygous venous drainage
8:20Bronchial atresia with bronchogenic cyst obstructing right upper lobe
16:522-month-old with CPAM and mediastinal shift, CVR 1.6 antenatally

Open questions

What is the optimal lower size limit for thoracoscopic lobectomy as technology improves?
How should surgeons approach lesions that appear to have bilobar involvement versus abnormal fissure formation?
What is the role of re-resection for residual cystic lesions after initial surgery?

This episode was analyzed and enriched by Kai, the Library's AI content creator. Every item links to the moment it comes from — click a timestamp to listen in context.

Written for:

Congenital Lung Lesions in Infants: When and How Pediatric Thoracic Surgeons Intervene

The episode's main topic retold as a plain-language walkthrough — what it is, why it matters, and what the speakers concluded. Written by Kai from the episode transcript and reviewed before publishing.

For the care team · Explainer · AI-written, human-reviewed

Why This Subspecialty Exists

Congenital pulmonary malformations — sequestrations, congenital pulmonary airway malformations (CPAMs), bronchogenic cysts — are developmental anomalies that create abnormal lung tissue or vascular connections during fetal life. Most are detected on prenatal ultrasound. Some resolve spontaneously; others cause respiratory distress at birth or harbor long-term infection and malignancy risk. Pediatric thoracic surgery exists because these lesions require precise anatomic resection in infants whose lungs are still developing, whose chest cavities measure centimeters, and whose compensatory growth potential is maximal if you operate early and minimal if you wait too long.

The Core Clinical Problem

The central question is not whether to resect — most congenital lung lesions that persist postnatally will eventually require removal — but when, and by what approach. A CPAM occupying the right lower lobe in a thriving two-month-old is not an emergency, but delaying resection beyond infancy increases infection risk without improving long-term pulmonary function 15:33. An infra-diaphragmatic sequestration compressing the esophagus is an emergency 0:56. A bronchogenic cyst obstructing an upper lobe bronchus has already destroyed the bronchus; you cannot simply shell out the cyst and preserve the lobe 8:32.

The technical challenge is operating in a space where a 5mm instrument occupies two-thirds of the hemithorax 21:32. The physiologic challenge is achieving single-lung ventilation in a neonate whose contralateral lung may not tolerate it. The anatomic challenge is that fetal lung development does not respect lobar boundaries — sequestrations can drain into pulmonary veins or azygous veins 0:00 1:18, lesions can sit between the leaves of the diaphragm 0:39, and what looks like bilobar disease on imaging is often abnormal fissure formation rather than true involvement of two lobes 24:18.

How the Approach Works

Most congenital lung lesions in stable infants are now resected thoracoscopically, typically before three months of age 17:38. The operation is technically easier in younger infants — tissue planes are cleaner, dissection is faster, recovery is quicker 17:44 17:51 — but the workspace is unforgiving. Setup is everything: trocar position determines whether the operation is straightforward or impossible 18:15. CO₂ insufflation at 4–7 cm H₂O creates mild tension pneumothorax, which infants tolerate well and which aids lung collapse 19:12 19:27 19:55.

Single-lung ventilation is achieved by mainstem intubation of the contralateral bronchus, which always leaves some overflow ventilation 19:27. This is not the controlled apnea of adult thoracic surgery; it is managed hypoxia in a patient whose oxygen reserve is minimal. The operation requires a stable baby with adequate contralateral lung 22:08.

Vessel management is debated. Clips are fast but can dislodge during subsequent dissection 11:50. Energy-sealing devices (Enseal, LigaSure) are operator-dependent — the blade advances as the vessel seals, and advancing too quickly divides the vessel before sealing is complete 12:02. One panelist uses clips only as a final maneuver after all dissection is complete; the other prefers sealed vessels and reports never having dislodged a seal 11:50.

Infra-diaphragmatic lesions — sequestrations that sit below the diaphragm, often at the gastroesophageal junction — are frequently misdiagnosed as intra-abdominal masses 0:19[q1]. Most extend through the esophageal hiatus into the chest 4:45. These are approached laparoscopically, but the dissection is difficult: the lesion adheres to stomach, esophagus, and diaphragmatic crura, and the vascular supply comes from the celiac axis rather than the aorta.

Bronchogenic cysts associated with bronchial atresia cause hyperplastic overgrowth of the obstructed lobe 7:29. By the time the cyst is large enough to image, the bronchus is destroyed; lobectomy is required 8:32. Extra-lobar sequestrations are invested in their own pleural envelope with no parenchymal connection to normal lung 1:52; these can sometimes be shelled out without formal lobectomy.

Where Practice is Contested

Timing of resection is the most contested question. Recent literature shows no difference in long-term pulmonary function between resection at three months versus eighteen months, but delayed resection increases complication risk 15:33. Compensatory alveolarization continues until age four to six years 16:27, so theoretically earlier is better, but one discussant notes that timing within the first few months may not significantly affect lung growth outcomes 16:43. The practical answer is that stable babies with small lesions can go home and return in one to two months 22:44, but there is no reason to wait beyond early infancy.

The lower size limit for thoracoscopic lobectomy is around 2 kg, constrained not by surgical skill but by instrument size 20:49. PDA ligations are now routine at 800 g 20:49, but lobectomy requires vessel sealing and specimen extraction, and current 5mm devices are too large for effective use below 2 kg 21:32.

Histologic diagnosis from thoracoscopic specimens is reliable despite pathologist complaints about specimen handling 14:15[q3]. This matters because distinguishing CPAM from pleuropulmonary blastoma — a rare malignancy that can mimic benign cystic disease — requires intact tissue architecture.

When to Involve This Team

Any infant with a prenatally diagnosed lung lesion that persists on postnatal imaging should be referred to pediatric thoracic surgery, ideally before discharge from the newborn nursery. Infants with respiratory distress, feeding difficulty, or failure to thrive in the setting of a known lung lesion require urgent evaluation. Lesions discovered incidentally on chest imaging obtained for other reasons — pneumonia that doesn't resolve, recurrent wheeze — warrant referral even if the child is otherwise well.

Centers with expertise in fetal intervention and neonatal thoracic surgery include UCSF, Cincinnati Children's, and Texas Children's Hospital in Houston. For stable lesions, delivery at a tertiary center is not required [q4]; the infant can be evaluated in the first weeks of life and surgery scheduled electively.

Takeaways from this story

Thoracoscopic resection is easier in younger infants but requires comfort working in small spaces with precise trocar positioning.
Infra-diaphragmatic sequestrations are often misdiagnosed as abdominal masses; most extend through the esophageal hiatus.
Resection timing between 3-18 months doesn't affect long-term lung function, but delayed resection increases infection risk.
Current 5mm instruments limit thoracoscopic lobectomy to infants over ~2kg, though PDA ligations are routine at 800g.
Bronchogenic cysts obstructing lobar bronchi require lobectomy, not just cyst resection, because the bronchus is destroyed.

Bronchogenic Cyst Obstructing Right Upper Lobe: When Cyst Resection Isn't Enough

The patient case from this episode, retold from presentation to outcome with the decisions made along the way. Written by Kai from the episode transcript and reviewed before publishing.

For specialists · Case narrative · AI-written, human-reviewed

Presentation

A bronchogenic cyst was identified obstructing the upper lobe bronchus on the right side 8:32. CT demonstrated the cyst positioned between the pulmonary artery branches supplying the upper lobe, sitting directly on top of the upper lobe bronchus 8:32. The distal lobe showed hyperplastic enlargement — the characteristic response to bronchial atresia with a bronchogenic cyst 7:29.

The Decision Point

The question was whether to resect only the cyst or proceed with upper lobectomy 8:32. In bronchial atresia, the obstructing cyst can theoretically be removed while preserving the lobe if the bronchus remains viable 8:32. But when a bronchogenic cyst obstructs an upper lobe bronchus, the bronchus is destroyed enough that cyst resection alone is insufficient — lobectomy becomes necessary 8:32. The chronic obstruction had rendered the bronchus nonviable 8:32.

The team chose thoracoscopic upper lobectomy 8:32.

Technical Challenge

The usual approach through the fissure was not available 8:32. The incomplete fissure and anatomic distortion from the cyst forced an approach from above 8:32. The cyst's position between the upper lobe pulmonary artery branches and directly on the bronchus meant dissection had to proceed carefully around critical hilar structures in a field where normal planes were obliterated 8:32.

This is where the choice of vessel control matters 11:50. During dissection, clips on vessels can be dislodged 11:50. In a tight space with distorted anatomy, clips projecting beyond the vessel surface become hazards during subsequent dissection 11:50. Sealed vessels cannot be dislodged 12:02. One discussant reserves clips for the final maneuver — the systemic arterial supply to a sequestration, for example, where nothing remains to be dissected afterward 12:02.

What This Case Teaches

Bronchogenic cysts associated with bronchial atresia present differently than isolated cysts 7:29. The hyperplastic distal lobe 7:29 signals chronic obstruction and should prompt careful assessment of bronchial viability 8:32. By the time the lobe is hyperplastic enough to be visible on imaging, the bronchus is typically destroyed 8:32.

The pathology concern about thoracoscopic specimens — that they are too traumatized for diagnosis — proves unfounded in practice 14:15. One discussant noted that thoracoscopic resections yield histologic diagnoses just as good as open procedures 14:15. Pathologists complain about specimen quality but consistently arrive at the correct diagnosis 14:15.

The case also demonstrates the technical ceiling for neonatal thoracoscopy 20:49 21:32. Current vessel sealers occupy a large portion of the chest in very small infants, limiting maneuverability 21:32. While PDA ligations are now routine at low birth weights 20:49, lobectomy remains difficult in smaller infants 20:49. The limitation is technological, not physiologic — as instruments shrink, the weight threshold will drop 21:32.

For bronchogenic cysts obstructing lobar bronchi, the transferable judgment is this: hyperplastic enlargement of the distal lobe means the bronchus is gone 7:29 8:32. Plan for lobectomy, not cyst resection 8:32. The anatomy will be distorted, the fissure may be incomplete, and the usual planes will not exist 8:32. Approach from wherever the anatomy permits 8:32, control vessels with a method that won't complicate subsequent dissection 11:50 12:02, and accept that preserving the lobe is not an option when the bronchus has been destroyed by chronic obstruction 8:32.

Takeaways from this story

Hyperplastic lobe distal to bronchogenic cyst means bronchus is destroyed — plan lobectomy, not cyst resection alone
Clips on vessels can dislodge during dissection in tight spaces; sealed vessels cannot
Thoracoscopic specimens yield diagnostic histology despite pathologist concerns about specimen quality
Current technology limits thoracoscopic lobectomy in smaller infants; vessel sealers occupy large portion of chest

Bronchogenic Cyst With Bronchial Atresia: When Cyst Resection Alone Is Not Enough

The patient case from this episode, retold from presentation to outcome with the decisions made along the way. Written by Kai from the episode transcript and reviewed before publishing.

For trainees · Case narrative · AI-written, human-reviewed

Presentation

A bronchogenic cyst was identified obstructing the right upper lobe bronchus, with characteristic hyperplastic enlargement of the affected lobe 7:29. CT imaging showed the cyst positioned between the pulmonary artery branches supplying the upper lobe, sitting directly on top of the upper lobe bronchus [case3]. The anatomic distortion was significant — the cyst's location and the associated bronchial obstruction had altered normal lobar architecture 7:29.

The Decision Point

The surgical team faced a fundamental question: could they preserve the upper lobe by resecting only the cyst, or had the chronic obstruction destroyed the bronchus beyond salvage?

Bronchogenic cysts can occur in isolation or in association with bronchial obstruction 7:20. When associated with bronchial atresia, the distal lobe undergoes hyperplastic growth 7:29 — a compensatory response that can make the affected lobe appear deceptively viable on imaging 7:29. But viability of lung parenchyma means nothing if the bronchus supplying it is destroyed 8:32.

The team approached this thoracoscopically [case3]. The usual dissection plane through the fissure was not available — the fissure was incomplete and the cyst had distorted the normal anatomy [case3]. They worked from above instead, exposing the bronchus and its relationship to the obstructing cyst [case3].

What they found at operation resolved the question: the upper lobe bronchus was destroyed enough that cyst resection alone would leave behind non-functional lung 8:32. The chronic obstruction had done irreversible damage 8:32. Lobectomy was required 8:32.

What the Team Did

They proceeded with thoracoscopic upper lobectomy [case3]. The approach from above — rather than through the fissure — gave them the exposure they needed despite the anatomic distortion [case3]. The destroyed bronchus was divided along with the vascular supply, and the hyperplastic upper lobe was removed [case3].

This is the pattern with bronchial atresia and bronchogenic cysts: by the time the obstruction is identified, the bronchus has typically been compromised long enough that preservation is not realistic 8:32. The hyperplastic lobe is not functional lung — it is the downstream consequence of chronic obstruction 7:29 8:32.

The Transferable Judgment

When you see a bronchogenic cyst obstructing a lobar bronchus with hyperplastic enlargement of the distal lung, do not assume the lobe is salvageable 8:32. The hyperplasia is a marker of chronic obstruction, and chronic obstruction destroys bronchi 7:29 8:32. Plan for lobectomy, not cyst resection 8:32.

The imaging will show you the cyst and the enlarged lobe 7:29. It will not tell you whether the bronchus is viable 8:32. That determination happens at operation, but the preoperative imaging pattern — cyst plus obstruction plus hyperplasia — should set your expectation that you are removing a lobe, not shelling out a cyst 8:32.

Incomplete fissures and anatomic distortion are common in these cases [case3]. The standard dissection planes may not be available [case3]. Approach flexibility matters — if you cannot work through the fissure, work from above or below [case3]. The goal is safe vascular and bronchial control, not adherence to a preferred dissection sequence [case3].

Finally: thoracoscopic resection is feasible even in distorted anatomy, provided you have the space to work and the infant is stable enough for single-lung ventilation 22:08. The specimen quality will be adequate for histologic diagnosis despite what the pathologists write in their reports 14:15. The maceration they complain about does not prevent them from making the diagnosis 14:15.

Takeaways from this story

Bronchogenic cysts obstructing lobar bronchi typically destroy the bronchus beyond salvage, requiring lobectomy rather than cyst resection alone.
Hyperplastic enlargement of lung distal to a bronchogenic cyst indicates chronic obstruction, not viable lung tissue.
Incomplete fissures in bronchial atresia cases may require approaching the hilum from above rather than through standard fissure planes.
Thoracoscopic specimens yield adequate histologic diagnosis despite pathologist concerns about maceration and trauma.

Keywords

bronchopulmonary sequestration BPS CPAM CCAM congenital pulmonary airway malformation bronchogenic cyst bronchial atresia pleuropulmonary blastoma PPB arteriovenous fistula esophageal obstruction hydrops lobectomy thoracoscopic resection laparoscopic resection

Hashtags

#PulmonaryAnomalies #CTImaging #SurgicalTechniques #PediatricSurgery #BronchialAtresia #FetalSurgery #EsophagealObstruction #VascularAnomalies #LungSequestration #CysticLesions #MedicalEducation #SurgicalAnatomy #CPAM #BPS #Lobectomy #Hyperplasia #VascularReconstruction #SurgicalApproach #PediatricPulmonology #SurgicalInstruments #ThoracicSurgery #CongenitalLungLesions #PediatricThoracicSurgery #PulmonarySequestration #MinimallyInvasiveSurgery

Transcript

And this is another pulmonary venous drain. And I'll show this one just to show some of the abnormal CT patterns. That'll be a tip off to you of what you're dealing with. So, you can see this sequestration there. You can see this very abnormal, uh, vessel that goes through the pulmonary parenchyma up into a pulmonary vein. Think the other thing is, you mentioned you didn't initially see anything. A number of these lesions, I'm amazed at how often they're misdiagnosed. They're either intra diaphragmatic or sub diaphragmatic. Um-hum. And they're thought to be in the chest. And so, it's really worth looking over these very well because there are times where certainly some of the infratic ones will approach laparoscopically. And then there are this new class that I've seen 3 or 4 of in the last year that are actually sort of in the lead, The muscle of the diaphragm and that you have to open the diaphragm in order to get at them. Yeah, between the leaves of the diaphragm. Right. And they're often in the GE junction as well. Right. Uh, or at the, uh, esophageal hiatus. And, and you can get oesophageal obstructive symptomatology. Right. Related to these. So again, here's a sequestration. And here's this big venous channel that you saw on the CT that's coming out of it and, uh, ascending up to the pulmonary vein. Again, just an arteriovenous fistula. This is an interesting one. This is a, uh, anatomic, Uh, extra pleural, or, uh, uh, extra lobar sequestration that has huge systemic arterial inflow, as you can see on the, uh, CT image there. And distal azygous outflow. Now, this is a left sided lesion and the, uh, the venous outflow actually goes across the chest and into the azygous vein. Hopefully it's, Present here. You can see this is completely, Separately invested by pleura. It has completely CCA histology and macrocystic abnormality on the CT scan. And, uh, there's, there's no bronchus whatsoever there. And you can see this, uh, There's no parenchymal connection. And here's this uh massive venous drainage that actually goes up. And then takes a right turn. Um, Out of the field and across the chest into the distal lazygu vein. You can see the, the big arterial inflow here. Coming off the aorta. So anyway, just a whole variety of different manifestations. And here's what Steve was referring to. Here's an infra diaphragmatic BPS. And you can see this thing, it's actually down under the diaphragm there. You can see the feeding vessels, which were, there were a bunch of them, and coming off the celiac axis, you can see the blush in the vascular reconstruction there. That is the BPS. And, uh, this was causing, uh, uh, obstruction to swallowing. So, the kid had a lot of secretions, um, And regurgitation of feedings and slow feeding. You can see the cysts in the middle of this, and this turned out to have CPAM histology again. So, an infra diaphragmatic BPS with CCAM, Uh, CPAM histology and, uh, esophageal obstruction. And I think that's video 14. See that I gave you in the new, in the new folder. Like, uh, it's, this is my fault cause I made a bunch of last minute changes. It's worth taking. It's worth it though. To see these. So it's, it's, yeah. Just wanted to give you an idea of the variety of these things. And this is, this is not it. This is a bronchial atresia. Um, a bronchial atresia with a, he said bronchogenic cysts that was 14. Did we try 13? Uh, let me see 13. Yeah, I think I want 13, actually. Sorry. Uh, yeah, this is it. OK, so, that's the spleen on the right. So, I'm taking a laparoscopic approach, cause I could see this was under the diaphragm. Although it clearly went up into the esophageal hiatus and some of it extended up to the chest. And you can see this is a big mass. It's not small. And it's very adherent to surrounding structures. So, this didn't come off the stomach easily. It didn't come off the GE junction easily. Actually went up into the esophageal hiatus there. And, uh, there's the pancreas underneath it. Actually, I found that the majority of these I approached through the abdomen do go up through the hiatus. Um-hum. There's some component, even if it doesn't look like it on CT. And often you can get a dumbbell lesion, which has a significant component on both sides. Yep. This had some fairly, uh, difficult vasculature coming off the celiac axis. You can see a big vascular pedicle there. Um, here's a, uh, a big vessel coming right out from underneath. You've been able to complete the procedure from the abdomen, not have to enter the chest? Yeah. Yeah, you can just sort of the hiatus. Follow it up and tease it up. And this, I'll show you the hiatus at the end. And I actually ended up putting some sutures to close the hiatus after I finished, um, Some of these vessels are. They're pretty substantial, as you can see. And then this had a, a cystic thing. I actually thought it, I had gone into the stomach or there was a connection to the stomach, but, um, There were mucoid cysts within this thing that were, Very adherent to the stomach. And then we're going up in the hiatus here. I try to fish it out. Opening the expanded fronto esophageal ligament here. But you can see how these can obstruct swallowing and uh. As Steve said, when, when you see them in this position, they're almost always in the hiatus and abutting the esophagus. I found the intra intra diaphragmatic ones, the ones with a leaf on each side are also frequently associated with the hiatus. So, there's the, uh, left cruise you just saw. There's another big vascular pedicle that goes up to it. So this was, it was a pretty tedious lesion to remove. It was amazingly adherent to everything around it, even in this, uh, 5 week old kid. And there's sort of the blown out hiatus. You can see there with the, The left cruise. Yeah, and then I, I think it's just a brief thing on, uh, bronchogenic cysts. They can be, uh, alone or they can be associated with a bronchial obstruction. You often see hyperplastic, uh, growth of the distal lobe, when they're associated with the bronchial atresia. And these can be, uh, huge prenatally, and actually, in the, the PowerPoint, we didn't show on the prenatal, I actually showed a fetal case where we had to, uh, resect a lobe that was causing eye drops, didn't respond to steroids, and it was related to, uh, bronchial atresia. So, they, you can have massive growth of even a lobe, um, lobar, uh, bronchial atresia. And then postnatally, you often just see a hyperplastic distal lobe, uh, with the, either the bronchial cyst, uh, at the origin of the, uh, lobar bronchus or no cyst present. And uh. This is an example of that. Here you can see a, uh, bronchial, or bronchogenic cyst that's obstructing the upper lobe bronchus on the right side, and you can see the hyperplastic enlargement of the upper lobe. And unfortunately, uh, when you see this, the bronchus is, uh, destroyed enough that you can't just resect the cyst, but you need to actually do the lobectomy as well. Yes. This isn't too informative. I just wanted to show you what a cyst looks like. Um, I'm, there's an incomplete fissure here that I'm working in, but I couldn't do the usual approach to the upper lobe vessels through the fissure, so, uh, because of the distortion from the cyst. So, I ended up going up, uh, over the top like I used to with open lobectomies. But you can see where the cyst is located. It's sitting there right on top of the upper lobe bronchus between the pulmonary artery branches that go to the upper lobe. And the, uh, bronchus and obstructing the bronchus in that position. You can see the pulmonary arteries are actually slung over the cyst. On each side. This is when I was still Testing the seal and you can see it's pretty tight. Fit in there, um, because of its blunt ends. I didn't really like this, the NCal on this particular operation. The, the thing you need to be careful about with this particular energy source is that it's very operator dependent cause it's sealing as. Um-hum. The knife advances. And if you advance the knife, so you really have to have good technique, which I'm showing here, but if you advance the knife too fast, you'll divide the vessel before you seal it. Yeah, and they now have, they now, they didn't used to have it, but now they have a tone. Right. Frequency. That, uh, tells you when the, the vessel's sealed and it's safe to advance the, the blade. But, Yeah, it's not if you just get in there and squeeze, but it's not a good thing. The general point that I, that I, I want, and I'm sure that Steve will focus on in the second half is the whole idea of sealing and dividing as a second, as a separate step, and this looks to be very safe, but is a different approach to that. And again, I think it's, uh, this is a, a much better instrument for larger kids and larger. Right. Um, Dissection planes. I love it in the abdomen for doing, uh, you know, Coker maneuvers and things like that where you're going through a lot of mesentery or. Attachment. So I think that's all I have for, for various lung lesions. That was, that was great. And we have, let's, um, Alan, thanks for that. We have the, uh, Stefan, do we still have the, the panelists on the phone? OK, let's get, uh, let's get the panelists back on the phone and then we have many, many questions to go through and, um, I heard there was someone on the phone calling him, but I guess he left. Is that right, Jesse? We had a caller from India. Um, is that gone, Amanda? OK, so we'll just go with the text questions, and if you wanna call in, your questions will get prioritized. I can tell you we're not gonna get to all of these. Uh, we'll get to the ones that we can, um. Ramesh's question about the clip we're gonna get to, uh, later on in the second half about clips versus ligature versus suture, all the different techniques, um, we did touch upon it already. I don't know if there's any additional things you want to say, but we can probably focus on that in the second half of the show. The biggest thing I'll say about clips in terms of vessels, the reason I don't like to use clips on vessels is because during your dissection you can dislodge them, right? And so I think that to depend. And, and you could, and I've never. Dislodged the seal. So, uh, that's what I worry about clips. They're just, they stick out past the vessels. They're relatively big in a small space and I just worry about inadvertently knocking that clip off while I'm dissecting. Yeah, I use clips for my last maneuver. So, if I'm. Yeah. For the sequestration. Right. You know, it's the last thing you put on there, you can do that. Right. I usually reinforce it with a ligacele, or a ligature anyway, distal to the clip. Um, or, you know, when you're doing the pulmonary vein, you can clip it and then, Uh, Sealed this story. Uh, Jean-Martin, the, uh, is not seeing his webcam icon. I, it's in the top right corner on my screen, Jean-Martin, just above Jack's picture. Uh, uh, Stefan's gonna work with you. Um, and, and the comments that were made, uh, about the clips from a lot of people which a whole discussion ensued about using the weck clips or the liga clips, uh, which, which won't have the risk of, of falling off nearly as much, although there is the risk of perforating the vessel if you don't get all around it, um. There there was a question, I think in relation to your first talk, uh, from Fawn Lewis about can you please list the US referral centers which should be considered for advanced care of early unfavorable fetal lung lesions. It's chop the, no, no. Yeah, it's the best, but it's not. Um, no, certainly, uh, there are a number of, uh, places that are currently, uh, doing open fetal surgery. And those include UCSF, which has a long history, obviously, of fetal, uh, uh, intervention, uh, Cincinnati, although Tim Crummelholm recently went to Denver. Um, but they still have a program, uh, in fetal surgery. Uh, Houston, uh, has done a number of open procedures. Um, Texas Children's in Houston, um, so there are, there are a number of other possibilities. Great. Um, Doctor Gabbra uh as um. I guess it was a case you were talking about. How did you detect that it was a, uh, PPB if it was done thoracoscopically's a, that's a great question. Um, I think there's a misconception that you can't, uh, do good histologic diagnoses with thoracoscopic resections. Um, I've learned through my experience that in fact you get just about as good a histology as you do, uh, with open procedures. The pathologists complain a lot more, you know, there's always a lot of verbiage in the report about this is a macerated, uh, traumatized specimen, but then they come up with the diagnosis. And in fact, this case, uh, these cases were all sent to, um, another center with expertise in PPB. I've forgotten, I think it's in Saint Louis, um. Where they, uh, where they analyzed it as well and were able to definitively say it was PPB. So, uh, we have a, a question, a phone call question from India. Uh, Shari, are you there? Yes, I'm here. Welcome. Thank you for calling in. Uh, where are you in India? I'm an angler. Great, great. OK. Uh, go ahead with your question. Yeah, thanks for allowing me to ask the question. Uh, uh, I just wanted to know, uh, how, how long can you delay the treatment postnatally of a, a CCA? Would it affect the lung development later in life? That's a great question too. There's actually a recent study, I think published in uh JPS where they compared early versus slightly later, uh resection, and, and the interval was something like 18 months as opposed to 3 months. I don't remember the exact intervals, but they concluded, um. One that uh. There is an increased risk of complications occurring like infection prior to your resection. And 2, that it doesn't really make a difference in terms of outcome or long term. Uh, pulmonary function. I think there's another study as well looking at pulmonary function at slightly later resection times that showed fairly normal pulmonary function, Once the child had grown up a little bit, so I don't think it matters a great deal. I think theoretically recession in infancy has more potential for compensatory lung growth and alveolarization, but that phase of lung development continues until you're 4 to 6 years of age. And so I think. You know, probably it doesn't matter if you do it in the 1st 3 months from a lung growth perspective. Uh, Cherish, did that answer your question? Yes, uh, um, thank you for your answer. I have a patient, uh, whose, um, uh, mother was diagnosed antenatally with, uh, C cam with a CVR of 1.6 and was given steroids at the age of 30 weeks and, uh, fortunately was born with a normal baby but still has a C cam, uh, with the medicinal shift. So I was wondering when to take that out. Uh, it's about 2 months now. Well, I would take it out now. Yeah. Uh, but, um, you know, I, I think, particularly in the international sense, you have to look at your capabilities in your center. And if you have, uh, very good anesthesia and you're comfortable with doing an early lung resection, then it should be done sooner rather than later. There's no reason to wait. I would, I would probably in Allen in my center, most of these lesions are removed before 3 months of age. And, and, and honestly, it's technically easier, although you have to be comfortable working in a small space. The children recover quicker, and we'll talk about this. And so the hospitalization and everything else is less, and the, um, so, in general, that's, that's the timeframe. But part of it depends on what your center's able to do, the comfort of your anesthesiologist, um, doing single lung ventilation in a small infant, um, and also your technical, uh, capabilities. So, even though we say it's easier, It is a much smaller space and so you have to get comfortable. The setup becomes everything in these cases because if you set up wrong, if your trochars are in the wrong position, assuming you're doing it thoracoscopically, now if you're doing it open. It doesn't matter as much, but the setup is critical when you do these operations, especially in a small neonate, because if your trocars are in a bad position, it will be very, very difficult. All right, uh, Shari, thank you so much for your phone call and your question. We are enjoying it. Thank you very much. Thank you. Uh, we have another question, uh, from, uh, Vietnam. Professor Liam, are you there, Liam? Yes, welcome, Liam. Good to hear your voice again. Um, yes, do you have a question for Alan or Steve? Yeah, thank you very much for your lecture. I would like to know how high CO2 pressure because I watched the video, you seem to have very big room to perform operation. Thank you. Um, I, I usually use a pressure of 7 centimeters of water. Um, I may go up or down a little bit depending on the child and, uh, whether, uh, they're having any difficulty. But in general, uh, it's very well tolerated and it gives you adequate exposure. Right. I, I would agree. I start at a pressure of 4. Part of it depends on how good of a single lung ventilation you have and how much overflow ventilation. We'll talk about this a little bit, but if you, the majority of times, we just do a, a main stem innovation of the contralateral, um, bronchus. So, that you always have a little bit of overflow ventilation. But usually a pressure of 4 is adequate. Um, but sometimes you have to go up to 7 or 8 to get the lung collapsed, and then you can back off a little bit. But the kids actually tolerate that quite well. That mild tension pneumothorax is not usually a significant issue. All right. Uh, thank you, Liam, for the question. And I think, uh, we'll be hearing from Liam later on in the show. I know he's got a great experience. Um, we have a question from Emery. Uh, what is the lower limit for infant size in which you will perform a thoracoscopic resection? Steve, Steve probably has no limit. I don't know. The smallest of what I've done was uh 2400, approximately 22 2400 g. I don't, you know. It didn't seem to be that much different than doing a term baby. So, uh, I think you could probably go lower still. Um, Uh, but just have to try it and see. Yeah. I mean, uh, we're limited, honestly right now by our technology. Um, I think that once you get, for instance, uh, something like a PDA ligation, which you're still holding vessels, we're now routinely doing those thoracoscopically down to about 800 g. Um, certainly, and now suddenly, 1000 g, which a few years ago seemed extremely small, now seems like a huge space. The problem is we're limited by the technology and, and honestly, getting a clip, you have to get a 5 millimeter clip into that tiny chest, and so you have to get through the rib spaces. When you start doing a lobectomy in a kid under 2 kg or TF, and the smallest TF we've done has been 1300 g. But we don't rely necessarily on a clip. We just rely on small instruments. But when you start relying on technology to get in and seal vessels and things like that, the current, the current sealers that we have are 5 millimeter instruments that take up 2/3 of the chest, and so you can't get in there very effectively. And I think as your experience grows and hopefully as the technology gets better, we'll be able to go smaller and smaller. But I think under 2. Kilos for a lobectomy is difficult. Yeah, and I think there are physiologic and, and physical limitations. Right. So, when you're talking about CCAs, normally the only reason you would be resecting something in a premature infant is if it was a large mass that was impacting ventilation. And so, in that circumstance, you know, you're not going to be able to do it thoracoscopically. And you also have, Issues with, uh, the contralateral lung for single lung ventilation. So, you really need a healthy. Right. Stable baby to do a thoracoscopic lobectomy and you can't have a big mass in the chest, um, unless it's cystic when you, where you can, you know, break it down. But if you have a big solid mass in the chest, uh, it's not going to work. So, I mean, just a management issue. Most of these patients who come, delivered, uh, many of them, if we know it's small, we'll tell them they can deliver at home and they don't need to come to our center and deliver. There's some concern that the child might have some, um, Immediate postnatal issues, then we'll have them deliver in our high risk maternal center. But if the baby's stable and doing well and there aren't really issues, we'll often let mom and baby go home. Even they can live in a, you know, anywhere in the country. They'll go home for a month or two, have a chance to kind of grow and have some normal development and then come back for the resection, um, so that the, one, the operation's easier, and two, I think it's a little easier on the parents. So, the smallest you've done is 800 g, is that what you said? No. PDA, not a lobectomy. Lobectomy is a, is a, well, extra, we've done some where there, it's ended up being an extra lobar mass around 2 kg, but 2 kg is about as small as we've got. I mean, I saw Starla shaking her head, you know, you have to understand Steve, Steve likes going down the double black diamonds in his tele, what is your tele scheme. He, he likes those kind of challenges. Don't expect that we're gonna be doing, uh, you know, grammar machine. Yeah, we, we, yeah, again, we've tried to let most of these kids go home and grow for a time and. And do it. But, you know, if it was an extreme case. Uh, the patients I've had clinically that have been problematic have, uh, congenital pulmonary malformations that cross the lines into another lobe. So, they'll have a main lobe, a lower lobe, and then you have just a little involvement of the middle lobe without a fissure that's well formed. How do you manage that? I mean, you talked a lot about lobectomy. Are you doing segmentectomies? Are you doing double lobectomies? Well, well, I think what you're really referring to is abnormal, uh, lobulation and fissure formation. And, uh, true bilobar involvement is relatively rare. You see it, like the case I showed, but most of it is abnormalities of globulation or fissure formation. And so, uh, all you can do is try to, uh, you know, make a fissure. In an appropriate position where you preserve as much lung, um, parenchyma as you can. And we'll show some cases of that. And, and where you don't leave devascularized lung or, or lung without an airway. So, so that if there's some residual cystic lesion seen post resection, do you follow that? Do you go back and reresect? I, I go back and, Resect that. But I, I, it shouldn't really happen if you try to avoid, uh, or if you try to follow, you know, the anatomic boundaries that are there. It's pretty rare that I've had one case where I've had to go back and remove further sein. Um,

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