Collection
CPAM (congenital Pulmonary Airway Malformation)
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
Resources
4 items
Neonatal Lung Lesions with Dr. Steven Rothenberg
Listen →
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
Postnatal Management of Lung Lesions Part I: Pediatric Thoracic Surgery Part...
Watch →
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
Open Fetal Surgery Overview: Fetal Surgery 2012
Watch →
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
The Full Story on CPAMs
Listen →
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
Summaries and takeaways+ Show
Summary of this collection+ Show
Key points, with the moment each was said+ Show
Featured picks for families are being prepared — the summaries above are written for you.
Takeaways+ Show
CPAMs represent abnormally developed lung tissue arising from normal bronchial airways but lacking functional alveoli. The Stocker classification (types 0–4) correlates with bronchial tree location; type 1 (distal bronchi/proximal bronchioles) accounts for 60–70% of cases. Lesions typically grow until 20–25 weeks' gestation and plateau by week 28. The CPAM volume ratio (CVR = length×width×height×0.52 / head circumference) predicts hydrops risk: CVR >1.6 carries 75% hydrops risk,[e4463-c4,e4463-c5] and hydrops presence is the strongest mortality predictor. Maternal betamethasone (12 mg ×2, 24 hours apart) resolves hydrops and reduces lesion size in most microcystic cases,[e4463-c7,e1027-c22,e1027-c23] though macrocystic and bronchial atresia lesions respond poorly.[e1027-c26,e1027-c27] Open fetal surgery is now rare (<1 case/5 years at major centers) due to steroid efficacy; thoracoamniotic shunts (70–75% survival) or EXIT procedures address refractory cases.[e1027-c5,e1027-c10] Postnatal management is controversial: U.S. practice favors elective resection (typically by 3 months) to prevent infection (20–40% lifetime risk),[e307-c13,e307-c15] enable compensatory growth, and address malignancy risk—particularly pleuropulmonary blastoma, found in 10% of postnatally diagnosed lesions but 0% of prenatal diagnoses.[e4463-c21,e4463-c18] European centers often observe asymptomatic lesions. Thoracoscopic lobectomy via front-to-back fissure dissection with dual-seal vascular control is standard; median stay is 2.5 days (under 2 days in infants <5 kg).[e307-c62,e307-c63,e307-c33]
- CVR >1.6 predicts 75% hydrops risk; maternal betamethasone resolves hydrops in most microcystic CPAMs, reducing open fetal surgery to <1 case per 5 years at tertiary centers.
- Pleuropulmonary blastoma occurs in 10% of postnatally diagnosed lesions but 0% of prenatal diagnoses; feeding vessel and prenatal diagnosis confer very low malignancy risk.
- Elective resection by 3 months avoids infection (20–40% lifetime risk), exploits easier dissection in fresh anatomy, and maximizes compensatory lung growth; median stay 2.5 days.
- Thoracoscopic lobectomy demands dual-seal vascular control: seal proximally and distally, cut partway, confirm hemostasis—never take pulmonary vein near pericardium without distal length.
- Congenital lung lesions exist on a spectrum; hybrid pathology is common, and imaging cannot reliably differentiate CPAM from bronchial atresia or exclude CPAM histology in sequestrations.
For patients & families
CPAM is abnormally developed lung tissue that doesn't help with breathing. It typically grows during the first half of pregnancy and then levels off around week 28. Doctors follow these lesions with regular ultrasounds every few weeks, which are quick and noninvasive. In many cases—somewhere between 6% and 40%—the lesion gets smaller over time, and sometimes it seems to disappear completely. If the baby is doing well at birth with no breathing trouble, families can usually go home, and a CT scan is done at about 4 to 6 weeks to see what's there. A chest X-ray alone isn't enough to prove the lesion is gone; a CT scan is needed. Most doctors in the U.S. recommend removing the affected part of the lung even if the baby has no symptoms, because there is a small risk of infection later in childhood and a very small risk of a serious cancer called pleuropulmonary blastoma. [e4463-c16, e4463-c18] When surgery is done early—often around 3 months of age—babies typically go home within two days and recover quickly. The healthy lung tissue grows to fill the space over time.
CPAM is abnormally developed lung tissue that doesn't help with breathing. It typically grows during the first half of pregnancy and then levels off around week 28. Doctors follow these lesions with regular ultrasounds every few weeks, which are quick and noninvasive. In many cases—somewhere between 6% and 40%—the lesion gets smaller over time, and sometimes it seems to disappear completely. If the baby is doing well at birth with no breathing trouble, families can usually go home, and a CT scan is done at about 4 to 6 weeks to see what's there. A chest X-ray alone isn't enough to prove the lesion is gone; a CT scan is needed. Most doctors in the U.S. recommend removing the affected part of the lung even if the baby has no symptoms, because there is a small risk of infection later in childhood and a very small risk of a serious cancer called pleuropulmonary blastoma. [e4463-c16, e4463-c18] When surgery is done early—often around 3 months of age—babies typically go home within two days and recover quickly. The healthy lung tissue grows to fill the space over time.
The doctors in this collection+ Show
All expert statements+ Show
Every expert statement below comes from the recorded discussions, with its speaker and moment.
Neonatal Lung Lesions with Dr. Steven Rothenberg
Serial prenatal ultrasounds are the best way to follow cystic lung lesions; they are noninvasive, quick, and performed every couple of weeks.
clinicalSteven Rothenberg3:48 ↗
Fetal MRI for lung lesions provides little additional benefit and does not change the management plan.
opinionSteven Rothenberg4:11 ↗
Anywhere from 6 to 40% of prenatally detected lung lesions will regress over time, and in some cases appear to completely disappear.
epidemiologicalSteven Rothenberg5:43 ↗
Fetal intervention (open surgery) for lung lesions is extremely rare; CHOP performs less than one open fetal surgery every couple of years.
epidemiologicalSteven Rothenberg6:11 ↗
Fetal thoracentesis or thoracoamniotic shunt is done only if the fetus shows significant distress or evidence of hydrops, which significantly increases mortality.
clinicalSteven Rothenberg6:31 ↗
Steroids are given if larger cysts cause mediastinal shift, or to mature the lung if early delivery is anticipated.
clinicalSteven Rothenberg7:13 ↗
A cyst volume ratio (CVR) greater than 2 has an extremely bad prognosis and is more likely to require fetal intervention.
clinicalSteven Rothenberg7:54 ↗
Congenital lung lesions (CAMs, sequestrations, bronchogenic cysts) represent a spectrum; hybrid lesions are common on pathology.
clinicalSteven Rothenberg8:52 ↗
Sequestrations are classified by the presence of a systemic artery (usually from the aorta); intralobar sequestrations share pleura with the lobe, extralobar have their own pleural lining.
clinicalSteven Rothenberg9:37 ↗
If a baby is born doing well with no respiratory distress and a normal or mildly abnormal chest X-ray, the family can go home; CT scan is obtained at 4–6 weeks.
clinicalSteven Rothenberg11:43 ↗
A chest X-ray or ultrasound is not adequate to prove a prenatally detected lung lesion has completely resolved; CT scan is required.
clinicalSteven Rothenberg12:39 ↗
Some children with normal postnatal chest X-ray or ultrasound later present with pneumonia (9 months to 6–7 years) and are found to have an infected CPAM.
clinicalSteven Rothenberg14:44 ↗
Depending on the series, 20–40% of untreated congenital lung lesions will develop a significant infection at some point.
epidemiologicalSteven Rothenberg15:47 ↗
The incidence of malignancy in untreated congenital lung lesions is over 1% in Rothenberg's series, which he considers significant.
epidemiologicalSteven Rothenberg16:56 ↗
Rothenberg prefers to operate by 3 months of age to avoid infection, because surgery is technically easier with smaller vessels and fresh anatomy, and to allow compensatory lung growth.
opinionSteven Rothenberg17:49 ↗
In Rothenberg's series, hospital stay, chest tube duration, recovery, and operative time were shorter in patients under 5 kg compared to those under 10 kg.
clinicalSteven Rothenberg19:19 ↗
Most infants undergoing early lobectomy are discharged within 48 hours; by one month post-op, chest X-ray shows no evidence of surgery.
clinicalSteven Rothenberg19:46 ↗
Even in asymptomatic children, waiting until around one year of age often reveals significantly enlarged lymph nodes and inflammation in the fissure, suggesting low-grade infection.
clinicalSteven Rothenberg18:46 ↗
The key to success in small infants is proper setup and port placement; with the right approach, there is plenty of room to work.
clinicalSteven Rothenberg21:13 ↗
Blood is typed and crossed for all lobectomies; it is one of the few pediatric cases where this is routine, because bleeding can be significant.
clinicalSteven Rothenberg22:36 ↗
Most asymptomatic children on room air tolerate single-lung ventilation without problem.
clinicalSteven Rothenberg23:38 ↗
Single-lung ventilation is achieved by main-stem intubation of the contralateral bronchus; bronchial blockers are difficult to place and add time, so are avoided.
clinicalSteven Rothenberg24:06 ↗
After lung collapse, infants initially desaturate (low 90s to high 80s) due to shunting; saturations improve once shunting to the collapsed lung stops.
clinicalSteven Rothenberg26:02 ↗
Anesthesiologists should use lower peak ventilatory pressures and increase rate (not pressure) to improve ventilation, avoiding high-pressure bagging that re-inflates the operative lung.
clinicalSteven Rothenberg26:36 ↗
End-tidal CO₂ in the mid-40s is tolerable and does not cause significant acidosis during thoracoscopic lobectomy.
clinicalSteven Rothenberg27:41 ↗
The surgeon and assistant stand at the patient's front (nipple side) in lateral decubitus position; there is more room from the front of the chest to the hilum than from the back.
clinicalSteven Rothenberg28:59 ↗
The camera port is placed over the major fissure in the mid-axillary line (5th or 6th interspace for lower lobe), anterior to the scapula tip, to allow working from front to back without paradoxical instrument angles.
clinicalSteven Rothenberg30:39 ↗
A fourth port is almost never needed; gravity and lung collapse provide adequate retraction in small children.
clinicalSteven Rothenberg32:28 ↗
Rothenberg uses a 4 mm 30° short scope (20 cm) for wider angle view and to allow the assistant to stay close without interference.
clinicalSteven Rothenberg33:01 ↗
Low-profile reusable 3 mm ports are essential in small infants; large-headed 5 mm ports cause instruments to collide in the limited space.
clinicalSteven Rothenberg33:56 ↗
What's new+ Show