Collection
Congenital Lung Lesions (CPAM)
Also covers: CPAM · congenital pulmonary airway malformation · hydrops · CPAM (congenital pulmonary airway malformation) · bronchial atresia · pleuropulmonary blastoma · bronchogenic cyst · pneumonia
Educational content from recorded physician discussions — not medical advice. Always talk to your child's care team about your child's situation.
Content of this collection
Using energy devices that seal and cut simultaneously (like Harmonic) is a mistake and sets the surgeon up for trouble; if the device fails, recovery is not possible. — Steven Rothenberg, Neonatal Lung Lesions with Dr. Steven Rothenberg · 37:46
Neonatal Lung Lesions with Dr. Steven Rothenberg
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Dr. Steven Rothenberg discusses Newborn Lung Lesions with Dr Todd Ponsky. Edited by Ian C. Glenn, MD Nicholas E. Bruns, MD
podcast1:06:58 · Jan 2019
Chest wall deformity from early shunt placement is related to collapse of the chest wall as opposed to the harpoon placement of the shunt. Prenatal Management of CPAMs: Lung Lesions · 12:34
Prenatal Management of CPAMs: Lung Lesions
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Dr. Alan Flake discusses prenatal management of congenital pulmonary airway malformations (CPAMs). It includes two videos of examples of fetal surgery.
video18:16 · Jan 2019
Maternal expertise must be available at the hospital performing EXIT procedures, with protection of the mother being the first priority. Fetal Interventions Part I: Lung Lesions · 5:13
Fetal Interventions Part II: Lung Lesions
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Dr. Alan Flake leads a discussion in fetal interventions for prenatally-diagnosed lung lesions.
video16:30 · Jan 2019
Open Fetal Surgery Overview: Fetal Surgery 2012
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Dr. Flake, attending surgeon and director of the General Surgery Fellowship Program at The Children's Hospital of Philadelphia,presents a brief overview of open fetal surgery. Topics discussed include the prenatal diagnosis of an abnormal f
video37:55 · Jan 2019
Fetal Interventions Part I: Lung Lesions
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Dr. Alan Flake leads a discussion in fetal interventions for prenatally-diagnosed lung lesions.
video14:27 · Jan 2019
Lung Lesions: Fetal Interventions Parts I+II
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Dr. Alan Flake leads a discussion in fetal interventions for prenatally-diagnosed lung lesions.
video30:56 · Jan 2019
Segmental bronchial stenoses have a very confluent appearance, almost like emphysematous pulmonary parenchyma, and may not require routine resection, though this is controversial. Postnatal Management of Lung Lesions Part I: Pediatric Thoracic Surgery Part... · 7:46
Postnatal Management of Lung Lesions Part III: Pediatric Thoracic Surgery...
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Course directors Drs Alan Flake, Steve Rothenberg and Todd Ponsky, along with additional faculty including Drs Jean Martin Laberge, Ramesh Santhanakrishnan, Giovanna Riccipetitoni, Mark Wulkan, Alexander Razumovskiy, Starla Martinez, Marcel
video27:56 · Sep 2018
Postnatal Management of Lung Lesions Part II: Pediatric Thoracic Surgery...
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Course directors Drs Alan Flake, Steve Rothenberg and Todd Ponsky, along with additional faculty including Drs Jean Martin Laberge, Ramesh Santhanakrishnan, Giovanna Riccipetitoni, Mark Wulkan, Alexander Razumovskiy, Starla Martinez, Marcel
video25:07 · Sep 2018
Postnatal Management of Lung Lesions Part I: Pediatric Thoracic Surgery Part...
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Course directors Drs Alan Flake, Steve Rothenberg and Todd Ponsky, along with additional faculty including Drs Jean Martin Laberge, Ramesh Santhanakrishnan, Giovanna Riccipetitoni, Mark Wulkan, Alexander Razumovskiy, Starla Martinez, Marcel
video31:07 · Sep 2018
Technique: Blinded Left Upper Lobectomy
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Dr. Steven Rothenberg performs a thoracoscopic blinded left upper lobectomy for a prenatally-diagnosed CPAM.
video4:28 · Jan 2019
Thoracoscopic Right Lower Lobe Cystic Lesion Excision: Update Course 2014
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Dr. Steve Rothenberg, Chief of Pediatric Surgery at Rocky Mountain Hospital for Children, Denver, Colorado, presents the topic of congenital cystic lung lesions. Topics discussed include asymptomatic congenital lung lesions, thoracoscopic l
video11:24 · Jan 2019
Thoracoscopic Upper Lobectomies for Symptomatic Congenital Pulmonary Airway...
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This video appears in a new pediatric surgery textbook, Clinical Pediatric Surgery: A Case-Based Interactive Approach," by Dr. Sherif Emil. The book is an innovative educational resource that focuses on judgment and decision-making in pedia
video5:58 · Feb 2020
Thoracoscopic Left Lower Lobectomy for Congenital Pulmonary Airway Malformation
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This video appears in a new pediatric surgery textbook, Clinical Pediatric Surgery: A Case-Based Interactive Approach," by Dr. Sherif Emil. The book is an innovative educational resource that focuses on judgment and decision-making in pedia
video9:59 · Feb 2020
Thoracoscopic Upper Lobectomies for Symptomatic Congenital Pulmonary Airway...
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This video appears in a new pediatric surgery textbook, Clinical Pediatric Surgery: A Case-Based Interactive Approach," by Dr. Sherif Emil. The book is an innovative educational resource that focuses on judgment and decision-making in pedia
video5:58 · Feb 2020
Neonatal Lung Lesions with Dr. Steven Rothenberg
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Dr. Steven Rothenberg discusses Newborn Lung Lesions with Dr Todd Ponsky. Edited by Ian C. Glenn, MD & Nicholas E. Bruns, MDPrenatal evaluationPatients with prenatal diagnosis of cystic lung lesions are referred for prenatal evaluation and
podcast1:06:58 · Dec 2020
Kansas City group has eliminated posterior crural sutures in fundoplication using minimal dissection technique, creating retroesophageal window but leaving posterior crura alone — Sean Barnhart, Complications and Beyond · 41:51
Complications and Beyond
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This clip from the 2020 Pediatric Surgery Update Course features an entertaining debate from the full panel surrounding unique and complicated cases presented by Jason Fraser, MD; and Miguel Guelfand, MD.
Highlighted Topics Include:
- M
video1:06:36 · Sep 2020
Cross-sectional imaging should not be performed on children with spontaneous pneumothorax to help make clinical decisions; the data support this conclusion — Elizabeth Speck, Journal of Pediatric Surgery Article Review: October 2023 · 6:22
Management of Asymptomatic Lung Lesions: Pediatric Thoracic Surgery Part...
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Course directors Drs Alan Flake, Steve Rothenberg and Todd Ponsky, along with additional faculty including Drs Jean Martin Laberge, Ramesh Santhanakrishnan, Giovanna Riccipetitoni, Mark Wulkan, Alexander Razumovskiy, Starla Martinez, Marcel
video20:11 · Jan 2019
Should We Resect Asymptomatic CPAM Flake vs Langer
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Great Debate from 2017! Watch Alan Flake and Steve Rothenberg debate Jack Langer and Jean Matin Laberge on wether or not asymptomatic CPAM's need to be resected. Originally aired March 23, 2017
video14:03 · May 2019
Journal of Pediatric Surgery Article Review: November 2021
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We're back with the November issue of JPS article highlights. This time we're talking to Dr. Pablo Laje and authors Dr. Frederick Rescorla and Dr. Niloufar Hafezi.Hosts: Ellen Encisco & Rod GerardoArticles: Rubalcava NS, Overman RE, Hirschl
podcast11:48 · Jan 2022
Case Based Journal Review - CPAM in 2022
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Dr. José Campos is back, this time helping us review some of the latest literature on the diagnosis and management of CPAM in children. In this podcast, we're reviewing a typical case with Dr. Todd Ponsky and incorporating literature from t
podcast12:23 · Apr 2022
Update Course 2022 - APSA PDC UPDATES - Mary Edwards, Paul Jeziorczak, Craig Lillehei, and Charles Snyder,
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In this session, members of the APSA Professional Development Committee (PDC) discussed the latest top articles and practice updates. We were joined by Drs. Mary Edwards, Paul Jeziorczak, Craig Lillehei, and Charles Snyder.
Topics discus
video1:04:30 · Sep 2022
Update Course Rewind: Congenital Lung Lesions 2022
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We are back with another Update Course Rewind video! This time we are presenting you "Congenital Lung Lesions" with Dr. Charles Snyder from APSA Professional Development Committee.
Host: Brittany Levy
Register for #UpdateCourse2023 (A
video4:33 · Aug 2023
Clinical Symptoms Affect Treatment and Prognosis in Pediatric Patients with Congenital Pulmonary Airway Malformation
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New article you should know by Dr. Cecilia Gigena
"Clinical Symptoms Affect Treatment and Prognosis in Pediatric Patients with Congenital Pulmonary Airway Malformation: A Propensity Score Matching Retrospective Cohort Study"
Authors: Guow
video0:55 · Dec 2023
Journal of Pediatric Surgery Article Review: October 2023
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We’re featuring Journal of Pediatric surgery articles to bring you some of the latest news!
This week we are discussing 3 articles from October 2023 issue, with editor Dr. Pablo Laje and authors Thomas Wyllie, and Drs. Mallinath Chakribo
podcast12:59 · Apr 2024
Quick Literature Updates Episode 18
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We’re back with eighteenth episode of "Quick Literature Updates" the podcast series that delivers the latest updates in pediatric surgery literature in a quick and digestible format. In each episode, we review articles covering the most int
video4:21 · Mar 2025
Yama would perform bronchoscopy to identify the involved segment, then thoracotomy (not thoracoscopy) in conjunction with intraoperative bronchoscopy for a patient with multiple prior operations. — Marco, Difficult Cases of Lung Lesions: Pediatric Thoracic Surgery Part 1-Lung... · 8:36
Panel Discussion: Pediatric Thoracic Surgery Part 1-Lung Lesions 2012
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The internationalfaculty debate and discuss management of difficult cases, includinglower lobe cystic lesion, spontaneous pneumothorax and unilateral bleb disease, and right pulmonary cyst.
video1:01:56 · Jan 2019
Difficult Cases of Lung Lesions: Pediatric Thoracic Surgery Part 1-Lung...
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During this session, the faculty discuss a patient with right pulmonary cyst, reviewingthe thoracoscopic to thoracotomy approach, and discuss varying ways of managing this particular case.
video21:56 · Jan 2019
Spontaneous Pneumothorax: Lung Lesions
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Dr. Mark McCollum leads a discussion in the management of spontaneous pneumothorax, including the type of anesthesia for chest tube insertion, indications for CT scans, number of ports in VATS, recurrence of bilateral blebs, and different t
video22:21 · Jan 2019
In Germany, the standard practice is to operate on any cystic lung lesion at 3-6 months of age because of potential malignancy risk later in life. — Benno, Congenital Cystic Lung Lesions: Update Course 2014 · 17:46
Congenital Cystic Lung Lesions: Update Course 2014
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Dr. Steve Rothenberg, Chief of Pediatric Surgery at Rocky Mountain Hospital for Children, Denver, Colorado, presents the topic of congenital cystic lung lesions. His discussion includes various case presentations and comprehensive panel dis
video24:39 · Nov 2018
The Full Story on CPAMs
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Here we cover Congenital Pulmonary Airway Malformations (CPAMs) through a story with the help of Dr. Todd Ponsky, Dr. Pam Choi, Dr. Beth Rymeski, Dr. Jacob Langer, and Dr. Steven Rothenberg. Hosts: Ellen Encisco & Rod GerardoChapter 1: Pren
podcast56:08 · Sep 2021
Featured picks for families are being prepared — the summaries above are written for you.
Natural history and prenatal management. 6–40% of prenatally diagnosed lesions regress and may appear to disappear, though chest X-ray or ultrasound are inadequate to confirm absence [e307-c1, e307-c5]. CVR >1.6 predicts hydrops risk; CVR >2 carries extremely poor prognosis [e307-c3, e1027-c6]. Steroids (betamethasone) dramatically reduce hydrops in microcystic CPAM, achieving ~100% survival when given prophylactically for CVR >1.6 but only 49% once hydrops is established [e1027-c7, e1027-c9, e1027-c37, e1027-c38]. Open fetal surgery is now extremely rare (<1 case/5 years at major centers); thoracoamniotic shunts for macrocystic lesions achieve 70–75% survival [e1027-c8, e1027-c12].
Postnatal timing and technique. 20–40% of untreated lesions develop significant infection; malignancy incidence exceeds 1% [e307-c6, e307-c7]. Surgery is technically easier in smaller infants: vessels are smaller, anatomy is fresh, and inflammation is minimal [e307-c8, e307-c10, e419-c16, e419-c17]. Waiting beyond 3–4 months produces significant fissural inflammation that complicates dissection [e307-c9, e420-c13, e420-c14]. Thoracoscopic lobectomy is feasible in infants >2 kg; median age at high-volume centers is 8–10 weeks [e419-c22, e4911-c13, e4911-c14]. Vascular control demands two separate seals 4–5 mm apart; never take the pulmonary vein trunk near the pericardium—device failure causes fatal retraction [e307-c14, e4463-c27]. Hybrid lesions (CPAM + sequestration) are common on pathology and may harbor large systemic vessels [e307-c4, e420-c21, e420-c26].
Malignancy risk and observation. Pleuropulmonary blastoma (PPB) is radiologically indistinguishable from CPAM until stage 2–3; ~4% of cystic lesions prove to be PPB [e883-c1, e883-c3]. Prenatally diagnosed lesions carry near-zero PPB risk; postnatally diagnosed lesions carry ~10% malignancy risk [e5319-c9, e5319-c10, e6996-c4, e6996-c5]. DICER1 mutation, bilateral disease, and multi-lobar involvement raise PPB suspicion; feeding vessels and prenatal diagnosis lower it [e4463-c19, e4463-c20]. Bronchioalveolar carcinoma risk is ~1%, occurring in teenage years . Extralobar sequestrations without airway communication have minimal infection and near-zero malignancy risk; observation is reasonable [e883-c6, e883-c7, e883-c8].
- CVR >1.6 predicts hydrops; prophylactic steroids achieve ~100% survival vs. 49% once hydrops is established. Open fetal surgery is now rare (<1/5 years).
- Surgery at 8–10 weeks is optimal: vessels smaller, anatomy fresh, minimal inflammation. Waiting beyond 3–4 months produces fissural inflammation complicating dissection.
- Vascular control: two seals 4–5 mm apart, cut partway to verify. Never take pulmonary vein trunk near pericardium—device failure causes fatal retraction.
- PPB is radiologically indistinguishable from CPAM; ~4% of cystic lesions are PPB. Prenatally diagnosed lesions carry near-zero PPB risk; postnatal diagnosis ~10% malignancy.
- Extralobar sequestrations without airway communication have minimal infection/malignancy risk; observation is reasonable. Intralobar lesions carry 20–40% infection risk.
For patients & families
Congenital lung lesions, including CPAM (congenital pulmonary airway malformation), are abnormal areas of lung tissue that develop before birth [e4463-c1, e4463-c2]. Most babies with these lesions are born healthy and have no breathing problems [e5319-c1, e5319-c5]. The lesions typically grow during the first half of pregnancy and then stop growing around week 28 . Doctors can see them on prenatal ultrasounds, and most do not require any treatment before birth [e1027-c3, e1088-c18]. A small number of babies with very large lesions may develop fluid buildup (hydrops), which is serious, but this can sometimes be treated with steroids given to the mother [e1027-c5, e1027-c7, e4463-c9]. After birth, doctors usually recommend surgery to remove the affected part of the lung, typically around 2–3 months of age, because waiting longer can lead to infections that make surgery harder [e419-c15, e420-c14, e4911-c10]. The surgery is often done with small incisions (thoracoscopy), and most babies go home within a few days [e1006-c20, e2227-c27]. Doctors recommend surgery partly because untreated lesions can become infected and, very rarely, may be associated with unusual tumors, though the overall risk is low [e307-c6, e307-c7, e883-c17, e5319-c10].
Congenital lung lesions, including CPAM (congenital pulmonary airway malformation), are abnormal areas of lung tissue that develop before birth [e4463-c1, e4463-c2]. Most babies with these lesions are born healthy and have no breathing problems [e5319-c1, e5319-c5]. The lesions typically grow during the first half of pregnancy and then stop growing around week 28 . Doctors can see them on prenatal ultrasounds, and most do not require any treatment before birth [e1027-c3, e1088-c18]. A small number of babies with very large lesions may develop fluid buildup (hydrops), which is serious, but this can sometimes be treated with steroids given to the mother [e1027-c5, e1027-c7, e4463-c9]. After birth, doctors usually recommend surgery to remove the affected part of the lung, typically around 2–3 months of age, because waiting longer can lead to infections that make surgery harder [e419-c15, e420-c14, e4911-c10]. The surgery is often done with small incisions (thoracoscopy), and most babies go home within a few days [e1006-c20, e2227-c27]. Doctors recommend surgery partly because untreated lesions can become infected and, very rarely, may be associated with unusual tumors, though the overall risk is low [e307-c6, e307-c7, e883-c17, e5319-c10].
Expert statements from the recordings in this collection.
Thoracoscopic Left Lower Lobectomy for Congenital Pulmonary Airway Malformation
The malformation was a hybrid congenital pulmonary airway malformation diagnosed prenatally.
The patient was asymptomatic at birth and remained asymptomatic through 9 months of age.
CT scan at 4 months showed a left lower lobe CPAM with systemic blood supply arising from the sub-diaphragmatic aorta.
The operation was performed at 9 months of age.
The left lung is isolated by right main stem intubation.
The patient is placed in the right lateral decubitus position with surgeon and assistant operating facing the patient.
A large arterial vessel coming through the diaphragm medial to the inferior pulmonary ligament and entering the left lower lobe confirms a hybrid lesion.
The inferior pulmonary ligament is divided all the way to the border of the inferior pulmonary vein to mobilize the left lower lobe off the diaphragm.
Division of the inferior pulmonary ligament allows stretching of the systemic arterial vessel for skeletonization with hook cautery.
The systemic arterial vessel is double-clipped proximally and divided distally with a ligature device.
The approach of proximal clipping and distal ligature division is used repeatedly for control of several major vessels.
The fissure is completed using ligature and sharp dissection.
Division of pulmonary parenchyma in the fissure allows visualization of pulmonary artery branches to the lower lobe.
The dissection should be kept as bloodless as possible, with any bleeding from divided parenchyma controlled early with ligature.
Expert opinion2:50
Excellent visualization is essential for adequate vascular control.
Expert opinion3:13
A right angle dissector is extremely useful in skeletonizing vessels and gaining adequate distance for safe ligation.
Expert opinion3:41
Dissection in the fissure always proceeds from medial to lateral.
While ligature can be used as the sole method of vessel control, the surgeon prefers to apply clips proximally if adequate vessel length has been achieved.
Expert opinion4:54
Once the pulmonary artery and all pulmonary parenchyma in the fissure is divided, the bronchus comes into view.
The inferior pulmonary vein is skeletonized using a right angle dissector.
The junction of the inferior pulmonary vein and the left atrium can be clearly seen after adequate dissection.
It is quite common for the inferior pulmonary vein to consist of two major tributaries that join as they approach the left atrium, in addition to one or more small tributaries.
When the inferior pulmonary vein has two major tributaries, it is best to dissect each tributary separately.
Expert opinion8:31
Each pulmonary vein tributary is controlled by clipping on the cardiac side and applying ligature on the pulmonary side.
Hook cautery is used to clean the bronchus of surrounding lymphatic and adventitial tissue.
An endo-GIA device is introduced directly through the chest wall and used to staple and divide the bronchus.
The specimen is extracted by slightly enlarging the posteriormost port site.
A chest tube is placed at the end of the operation.
The patient was extubated at the end of the procedure.
The patient was discharged on the 2nd postoperative day with excellent recovery.