# CPAM (congenital Pulmonary Airway Malformation) — GCMD Library living collection Updated: n/a · 4 episodes · 157 cited statements ## Episodes ### Resources - [Neonatal Lung Lesions with Dr. Steven Rothenberg](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307) — podcast · 1:06:58 · [machine version](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307.md) - [Postnatal Management of Lung Lesions Part I: Pediatric Thoracic Surgery Part...](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420) — video · 31:07 · [machine version](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420.md) - [Open Fetal Surgery Overview: Fetal Surgery 2012](https://library.globalcastmd.com/watch/open-fetal-surgery-overview-fetal-surgery-2012-1027) — video · 37:55 · [machine version](https://library.globalcastmd.com/watch/open-fetal-surgery-overview-fetal-surgery-2012-1027.md) - [The Full Story on CPAMs](https://library.globalcastmd.com/watch/the-full-story-on-cpams-4463) — podcast · 56:08 · [machine version](https://library.globalcastmd.com/watch/the-full-story-on-cpams-4463.md) ## Chapters - [0:00](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=0) Introduction and Dr. Rothenberg's Background (Ep 1) - [3:06](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=186) Prenatal Evaluation and Workup of Lung Lesions (Ep 1) - [8:52](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=532) Spectrum of Congenital Lung Lesions (Ep 1) - [11:43](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=703) Postnatal Management and Timing of Imaging (Ep 1) - [17:28](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=1048) Timing of Surgery and Rationale for Early Operation (Ep 1) - [21:52](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=1312) Preoperative Preparation and Anesthetic Considerations (Ep 1) - [27:59](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=1679) Patient Positioning, Port Placement, and Instrumentation (Ep 1) - [34:20](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=2060) Vascular Control Technique and Device Selection (Ep 1) - [41:10](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=2470) Left Lower Lobectomy: Step-by-Step Technique (Ep 1) - [51:03](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=3063) Right Lower Lobectomy (Ep 1) - [51:42](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=3102) Left Upper Lobectomy (Including Lingula) (Ep 1) - [55:40](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=3340) Right Middle Lobectomy (Ep 1) - [57:02](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=3422) Additional Technical Pearls for Lobectomy (Ep 1) - [58:27](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=3507) Management of Extralobar Sequestration (Ep 1) - [62:32](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=3752) Segmentectomy vs. Lobectomy (Ep 1) - [64:40](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=3880) Postoperative Course and Discharge (Ep 1) - [65:50](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=3950) Closing Remarks (Ep 1) - [0:00](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=0) Prenatal Assessment and Delivery Planning for Lung Lesions (Ep 2) - [7:04](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=424) Indications for Resection and Thoracoscopic Technique (Ep 2) - [10:42](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=642) Case Presentations: Asymptomatic CPAM and Middle Lobe Challenges (Ep 2) - [15:04](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=904) Bilobar CPAM and Diagnostic Uncertainty (Ep 2) - [19:17](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=1157) Hybrid Lesions and Vascular Management (Ep 2) - [23:31](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=1411) Extralobar BPS: Indications and Technique (Ep 2) - [25:50](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=1550) Complex BPS Variants and Esophageal Bronchus (Ep 2) - [0:00](https://library.globalcastmd.com/watch/open-fetal-surgery-overview-fetal-surgery-2012-1027?t=0) Introduction and Overview of Open Fetal Surgery (Ep 3) - [2:31](https://library.globalcastmd.com/watch/open-fetal-surgery-overview-fetal-surgery-2012-1027?t=151) Congenital Pulmonary Airway Malformations (CPAM) (Ep 3) - [10:49](https://library.globalcastmd.com/watch/open-fetal-surgery-overview-fetal-surgery-2012-1027?t=649) Bronchial Atresia Experience (Ep 3) - [11:57](https://library.globalcastmd.com/watch/open-fetal-surgery-overview-fetal-surgery-2012-1027?t=717) Sacrococcygeal Teratoma (SCT) (Ep 3) - [18:15](https://library.globalcastmd.com/watch/open-fetal-surgery-overview-fetal-surgery-2012-1027?t=1095) The EXIT Procedure (Ep 3) - [23:57](https://library.globalcastmd.com/watch/open-fetal-surgery-overview-fetal-surgery-2012-1027?t=1437) Future Directions and Acknowledgments (Ep 3) - [24:44](https://library.globalcastmd.com/watch/open-fetal-surgery-overview-fetal-surgery-2012-1027?t=1484) Panel Discussion on CPAM and Steroids (Ep 3) - [0:00](https://library.globalcastmd.com/watch/the-full-story-on-cpams-4463?t=0) Introduction and Differential Diagnosis of Prenatal Lung Masses (Ep 4) - [3:40](https://library.globalcastmd.com/watch/the-full-story-on-cpams-4463?t=220) CPAM Pathophysiology and Classification (Ep 4) - [6:44](https://library.globalcastmd.com/watch/the-full-story-on-cpams-4463?t=404) Prenatal Monitoring and CPAM Volume Ratio (Ep 4) - [11:21](https://library.globalcastmd.com/watch/the-full-story-on-cpams-4463?t=681) Advanced Fetal Interventions for High-Risk Cases (Ep 4) - [19:19](https://library.globalcastmd.com/watch/the-full-story-on-cpams-4463?t=1159) Postnatal Evaluation and Imaging (Ep 4) - [22:00](https://library.globalcastmd.com/watch/the-full-story-on-cpams-4463?t=1320) Surgical Decision-Making: Geographic Variation and Malignancy Risk (Ep 4) - [30:07](https://library.globalcastmd.com/watch/the-full-story-on-cpams-4463?t=1807) Observation Protocol and Infection Management (Ep 4) - [36:01](https://library.globalcastmd.com/watch/the-full-story-on-cpams-4463?t=2161) Thoracoscopic Lobectomy Technique (Ep 4) - [51:37](https://library.globalcastmd.com/watch/the-full-story-on-cpams-4463?t=3097) Postoperative Care and Summary (Ep 4) ## Statements Every statement is attributed, typed, and timestamped; the link is the citation. - Serial prenatal ultrasounds are the best way to follow cystic lung lesions; they are noninvasive, quick, and performed every couple of weeks. — Steven Rothenberg (clinical) [Ep 1 · 3:48](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=228) - Fetal MRI for lung lesions provides little additional benefit and does not change the management plan. — Steven Rothenberg (opinion) [Ep 1 · 4:11](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=251) - Anywhere from 6 to 40% of prenatally detected lung lesions will regress over time, and in some cases appear to completely disappear. — Steven Rothenberg (epidemiological) [Ep 1 · 5:43](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=343) - Fetal intervention (open surgery) for lung lesions is extremely rare; CHOP performs less than one open fetal surgery every couple of years. — Steven Rothenberg (epidemiological) [Ep 1 · 6:11](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=371) - Fetal thoracentesis or thoracoamniotic shunt is done only if the fetus shows significant distress or evidence of hydrops, which significantly increases mortality. — Steven Rothenberg (clinical) [Ep 1 · 6:31](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=391) - Steroids are given if larger cysts cause mediastinal shift, or to mature the lung if early delivery is anticipated. — Steven Rothenberg (clinical) [Ep 1 · 7:13](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=433) - A cyst volume ratio (CVR) greater than 2 has an extremely bad prognosis and is more likely to require fetal intervention. — Steven Rothenberg (clinical) [Ep 1 · 7:54](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=474) - Congenital lung lesions (CAMs, sequestrations, bronchogenic cysts) represent a spectrum; hybrid lesions are common on pathology. — Steven Rothenberg (clinical) [Ep 1 · 8:52](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=532) - 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. — Steven Rothenberg (clinical) [Ep 1 · 9:37](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=577) - 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. — Steven Rothenberg (clinical) [Ep 1 · 11:43](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=703) - A chest X-ray or ultrasound is not adequate to prove a prenatally detected lung lesion has completely resolved; CT scan is required. — Steven Rothenberg (clinical) [Ep 1 · 12:39](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=759) - 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. — Steven Rothenberg (clinical) [Ep 1 · 14:44](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=884) - Depending on the series, 20–40% of untreated congenital lung lesions will develop a significant infection at some point. — Steven Rothenberg (epidemiological) [Ep 1 · 15:47](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=947) - The incidence of malignancy in untreated congenital lung lesions is over 1% in Rothenberg's series, which he considers significant. — Steven Rothenberg (epidemiological) [Ep 1 · 16:56](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=1016) - 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. — Steven Rothenberg (opinion) [Ep 1 · 17:49](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=1069) - 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. — Steven Rothenberg (clinical) [Ep 1 · 19:19](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=1159) - Most infants undergoing early lobectomy are discharged within 48 hours; by one month post-op, chest X-ray shows no evidence of surgery. — Steven Rothenberg (clinical) [Ep 1 · 19:46](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=1186) - 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. — Steven Rothenberg (clinical) [Ep 1 · 18:46](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=1126) - The key to success in small infants is proper setup and port placement; with the right approach, there is plenty of room to work. — Steven Rothenberg (clinical) [Ep 1 · 21:13](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=1273) - 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. — Steven Rothenberg (clinical) [Ep 1 · 22:36](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=1356) - Most asymptomatic children on room air tolerate single-lung ventilation without problem. — Steven Rothenberg (clinical) [Ep 1 · 23:38](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=1418) - 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. — Steven Rothenberg (clinical) [Ep 1 · 24:06](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=1446) - After lung collapse, infants initially desaturate (low 90s to high 80s) due to shunting; saturations improve once shunting to the collapsed lung stops. — Steven Rothenberg (clinical) [Ep 1 · 26:02](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=1562) - 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. — Steven Rothenberg (clinical) [Ep 1 · 26:36](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=1596) - End-tidal CO₂ in the mid-40s is tolerable and does not cause significant acidosis during thoracoscopic lobectomy. — Steven Rothenberg (clinical) [Ep 1 · 27:41](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=1661) - 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. — Steven Rothenberg (clinical) [Ep 1 · 28:59](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=1739) - 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. — Steven Rothenberg (clinical) [Ep 1 · 30:39](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=1839) - A fourth port is almost never needed; gravity and lung collapse provide adequate retraction in small children. — Steven Rothenberg (clinical) [Ep 1 · 32:28](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=1948) - Rothenberg uses a 4 mm 30° short scope (20 cm) for wider angle view and to allow the assistant to stay close without interference. — Steven Rothenberg (clinical) [Ep 1 · 33:01](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=1981) - Low-profile reusable 3 mm ports are essential in small infants; large-headed 5 mm ports cause instruments to collide in the limited space. — Steven Rothenberg (clinical) [Ep 1 · 33:56](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=2036) - A 3 mm vessel sealer (bipolar technology) can seal vessels up to 5 mm in diameter. — Steven Rothenberg (clinical) [Ep 1 · 35:01](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=2101) - The dual-seal technique: make two separate seals on a vessel 4–5 mm apart, then cut partway between them to confirm hemostasis before full division. — Steven Rothenberg (clinical) [Ep 1 · 35:15](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=2115) - Vascular control is everything in thoracoscopic lobectomy; the dual-seal technique allows recovery if a seal fails, whereas full division without confirmation risks uncontrollable hemorrhage. — Steven Rothenberg (clinical) [Ep 1 · 35:52](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=2152) - Clips can be knocked off vessels; the dual-seal technique with vessel sealing is safer than routine clipping. — Steven Rothenberg (opinion) [Ep 1 · 36:59](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=2219) - Using energy devices that seal and cut simultaneously (e.g., Harmonic) on major vessels is a mistake and sets up the surgeon for unrecoverable bleeding. — Steven Rothenberg (opinion) [Ep 1 · 37:46](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=2266) - Rothenberg had one case using a seal-and-cut device that failed, resulting in bleeding and conversion to open. — Steven Rothenberg (clinical) [Ep 1 · 39:09](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=2349) - The first step in left lower lobectomy is to take down the inferior pulmonary ligament to check for a systemic vessel and to expose the inferior pulmonary vein. — Steven Rothenberg (clinical) [Ep 1 · 42:01](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=2521) - The ease of lobectomy depends on fissure completeness; incomplete fissures require layer-by-layer completion using the vessel sealer, similar to finger fracture in liver surgery. — Steven Rothenberg (clinical) [Ep 1 · 42:54](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=2574) - In a left lower lobectomy, the pulmonary artery trunk bifurcates into four basal segment branches; the superior segmental branch comes off higher and more posteriorly. — Steven Rothenberg (clinical) [Ep 1 · 44:06](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=2646) - The bronchus sits directly underneath the pulmonary artery and can be palpated to aid dissection behind the artery. — Steven Rothenberg (clinical) [Ep 1 · 45:23](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=2723) - If the main arterial trunk has good length, a 5 mm stapler can be used; otherwise, dissect and seal individual basal segmental branches for safer vascular control. — Steven Rothenberg (clinical) [Ep 1 · 45:35](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=2735) - When using a stapler on a major vessel, always have proximal control (clamp) in place before firing, in case the staple line bleeds. — Steven Rothenberg (clinical) [Ep 1 · 46:25](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=2785) - Rothenberg now works 'front to back' through the fissure (like turning pages of a book) rather than flipping the lung, because it is hard to change exposure thoracoscopically. — Steven Rothenberg (clinical) [Ep 1 · 47:35](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=2855) - After dividing the artery, the next step is the bronchus (superior segmental first, then main trunk); the pulmonary vein lies directly behind the bronchus. — Steven Rothenberg (clinical) [Ep 1 · 48:04](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=2884) - In children over 10 kg, a 12 mm stapler is needed for the bronchus; under 10 kg, a 5 mm stapler or clips suffice. — Steven Rothenberg (clinical) [Ep 1 · 49:11](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=2951) - After dividing the bronchus, dissect the inferior pulmonary vein to its first bifurcation, seal the smaller branch for length, then staple the main trunk—never take the vein near the pericardium. — Steven Rothenberg (clinical) [Ep 1 · 49:46](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=2986) - If a vascular device fails near the pericardium, the vessel retracts and the child will bleed to death before you can intervene; always ensure adequate length for proximal control. — Steven Rothenberg (clinical) [Ep 1 · 50:33](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=3033) - Right lower lobectomy is the mirror image of left lower lobe; the key caution is that middle lobe vessels branch off just above the lower lobe artery. — Steven Rothenberg (clinical) [Ep 1 · 51:03](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=3063) - For left upper lobectomy, retract the apex inferiorly to expose the apical/anterior arterial trunk at the apex of the chest; divide its branches first. — Steven Rothenberg (clinical) [Ep 1 · 52:02](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=3122) - After taking the upper lobe arteries, retract the lung posteriorly to expose and divide the superior pulmonary vein and lingular vein. — Steven Rothenberg (clinical) [Ep 1 · 53:23](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=3203) - In left upper lobectomy, the lingula is almost always taken with the upper lobe; on the right, the middle lobe is usually preserved. — Steven Rothenberg (clinical) [Ep 1 · 54:06](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=3246) - Working through the fissure in upper lobectomy, the lingular artery is encountered first, then a large posterior segmental branch, then the upper lobe bronchus. — Steven Rothenberg (clinical) [Ep 1 · 54:30](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=3270) - For right middle lobectomy, complete the minor fissure anteriorly; the pulmonary artery enters posteriorly and bifurcates into upper and lower branches. — Steven Rothenberg (clinical) [Ep 1 · 55:47](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=3347) - If the lung has large cysts limiting visualization, use the vessel sealer to pop the cysts at the start of the case to decompress and improve exposure. — Steven Rothenberg (clinical) [Ep 1 · 57:38](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=3458) - Extralobar sequestrations can have up to 6 systemic vessels; Rothenberg has seen vessels as large as 15 mm in diameter. — Steven Rothenberg (clinical) [Ep 1 · 58:40](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=3520) - For sequestration vessels, use clips or vessel sealer with dual-seal technique; a 5 mm stapler can be used for very large vessels (e.g., 15 mm). — Steven Rothenberg (clinical) [Ep 1 · 58:58](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=3538) - Never use both clips and energy sealing on the same vessel; energy changes the vessel wall, causing clips to lose purchase and leading to delayed bleeding. — Steven Rothenberg (clinical) [Ep 1 · 59:45](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=3585) - Systemic vessels to sequestrations come off the aorta under higher pressure than pulmonary vessels, so meticulous technique is critical. — Steven Rothenberg (clinical) [Ep 1 · 60:43](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=3643) - Extralobar sequestrations can become infected; Rothenberg removes them all thoracoscopically with no chest tube and next-day discharge, so embolization is not indicated. — Steven Rothenberg (opinion) [Ep 1 · 61:07](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=3667) - Rothenberg historically performed complete lobectomy but now considers segmentectomy (superior segment of lower lobe, lingula) if CT shows disease confined to one segment; approximately 20 cases with no recurrence on follow-up. — Steven Rothenberg (clinical) [Ep 1 · 62:42](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=3762) - A chest tube is left overnight after lobectomy; if no air leak or drainage, it is removed the morning of postoperative day 1, chest X-ray obtained 2 hours later, and patient discharged that afternoon. — Steven Rothenberg (clinical) [Ep 1 · 64:46](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=3886) - Average length of stay for lobectomy is 2.5 days; it is shorter (under 2 days) in infants under 5 kg. — Steven Rothenberg (clinical) [Ep 1 · 65:33](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=3933) - At 32 weeks gestation, ultrasound is performed to prognosticate whether the fetus will be asymptomatic or symptomatic at birth based on mediastinal shift. (clinical) [Ep 2 · 0:05](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=5) - 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. (clinical) [Ep 2 · 0:18](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=18) - 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. (clinical) [Ep 2 · 0:40](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=40) - Major mediastinal shift with lung or cardiac compression requires delivery at a tertiary center with EXIT procedure or ECMO availability. (clinical) [Ep 2 · 1:00](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=60) - All CPAMs, intralobar BPS, and hybrid lesions should be resected routinely. (opinion) [Ep 2 · 1:20](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=80) - Extralobar BPS in some cases do not need resection and can be observed. (opinion) [Ep 2 · 1:33](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=93) - CPAMs are prone to infection and routinely have mucoid stasis; many show inflammatory evidence at 2-3 months of age when resected. (clinical) [Ep 2 · 2:05](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=125) - CPAMs have established malignant potential, with reported cases of pleuropulmonary blastoma, bronchoalveolar carcinoma, and rhabdomyosarcoma. (clinical) [Ep 2 · 2:27](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=147) - Pleuropulmonary blastoma cannot be differentiated radiologically from CPAM and is highly malignant and lethal if allowed to progress beyond stage 1. (clinical) [Ep 2 · 2:31](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=151) - 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. (clinical) [Ep 2 · 2:51](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=171) - There is anatomical and histological overlap between congenital lung lesion types; they exist on a continuum rather than as discrete categories. (clinical) [Ep 2 · 3:15](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=195) - A sequestration lesion with small cysts cannot be assumed to lack CPAM histology based on imaging alone. (clinical) [Ep 2 · 3:30](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=210) - Intralobar BPS can have communications via the pores of Kohn leading to infection risk and potential for high-flow physiology. (clinical) [Ep 2 · 3:40](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=220) - Waiting until 4-5 months of age for resection can result in significant inflammation within fissures that changes the complexion of the dissection. (clinical) [Ep 2 · 4:15](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=255) - Earlier resection is surgically easier and less traumatic for the infant than later resection. (opinion) [Ep 2 · 6:36](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=396) - 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. (opinion) [Ep 2 · 7:04](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=424) - Segmental bronchial stenoses have a very confluent appearance, almost like emphysematous pulmonary parenchyma, and may not require routine resection, though this is controversial. (opinion) [Ep 2 · 7:46](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=466) - If any cysts are visible in a segmental bronchial stenosis, that usually indicates CPAM histology. (clinical) [Ep 2 · 7:58](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=478) - 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. (clinical) [Ep 2 · 8:12](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=492) - CPAMs often have abnormal fissures and globulated appearance related to developmental effects, so normal anatomy cannot be depended upon during lobectomy. (clinical) [Ep 2 · 10:28](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=628) - 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. (clinical) [Ep 2 · 11:09](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=669) - Feeding vessels in sequestrations can arise from essentially any systemic source, not just the thoracic or abdominal aorta. (clinical) [Ep 2 · 15:55](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=955) - Some sequestrations have abnormal pleural investments or pleural caps that must be dissected during resection. (clinical) [Ep 2 · 16:11](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=971) - The speaker had a pulmonary artery hemorrhage when a ligature seal broke down during lobectomy, leading to conversion to open thoracotomy. (clinical) [Ep 2 · 17:26](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=1046) - After the hemorrhage experience, the speaker tied every pulmonary artery and ligatured distally until the newer Force Triad energy source became available. (clinical) [Ep 2 · 17:52](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=1072) - Tying all vessels intracorporeally takes about twice as long as using energy devices but is a safe alternative. (clinical) [Ep 2 · 18:12](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=1092) - Many sequestrations are edematous due to restricted venous and lymphatic outflow, often with associated pleural effusions. (clinical) [Ep 2 · 19:17](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=1157) - Edematous extralobar BPS typically have small vascular pedicles and are very easy to resect. (clinical) [Ep 2 · 19:30](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=1170) - 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. (clinical) [Ep 2 · 20:32](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=1232) - 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. (clinical) [Ep 2 · 21:14](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=1274) - When taking the inferior pulmonary ligament during lobectomy, careful inspection is needed because blood flow through small systemic vessels can be phenomenal. (clinical) [Ep 2 · 22:21](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=1341) - If a systemic feeding vessel is missed and the pulmonary vein is taken first, tremendous congestion of the lobe will occur. (clinical) [Ep 2 · 22:32](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=1352) - In high-output sequestrations, the pulmonary vein dilates dramatically and can become huge. (clinical) [Ep 2 · 22:56](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=1376) - Extralobar BPS have separate pleural investment, no bronchial connection, systemic arterial supply from almost any source, and systemic or pulmonary venous drainage. (clinical) [Ep 2 · 23:09](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=1389) - Extralobar BPS can have CPAM histology, usually indicated by a visible cyst on imaging. (clinical) [Ep 2 · 23:31](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=1411) - Extralobar BPS have mucostasis but no airway communication, so there is no infection risk from that route. (clinical) [Ep 2 · 23:37](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=1417) - Extralobar BPS can get infected, likely via hematogenous route, and present as pneumonias; infected lesions are much more difficult to resect. (clinical) [Ep 2 · 24:24](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=1464) - A purely extralobar sequestration that is prenatally diagnosed is a relatively straightforward case and a great place to start when learning thoracoscopic resection. (opinion) [Ep 2 · 24:41](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=1481) - Extralobar BPS could potentially be done as outpatient procedures with two ports and no chest tube. (opinion) [Ep 2 · 24:59](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=1499) - 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. (clinical) [Ep 2 · 28:10](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=1690) - Some sequestrations are essentially pure AV fistulas—a small piece of lung tissue with large blood vessels going in and out. (clinical) [Ep 2 · 28:31](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=1711) - Arterialization of vasculature occurs in sequestrations, with even pulmonary veins becoming thickened like arteries due to high flow. (clinical) [Ep 2 · 21:19](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=1279) - 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. (clinical) [Ep 2 · 21:57](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=1317) - When a sequestration is against the esophagus and difficult to separate, an esophageal bronchus should be suspected. (clinical) [Ep 2 · 30:53](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=1853) - Esophageal bronchi can be small and may be missed if the surgeon is not aware they may be present. (clinical) [Ep 2 · 31:00](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=1860) - Sequestrations with esophageal bronchi are cesspools full of mucoid material. (clinical) [Ep 2 · 30:38](https://library.globalcastmd.com/watch/postnatal-management-of-lung-lesions-part-i-pediatric-thoracic-surgery-part-420?t=1838) - Open fetal surgery for CPAM is now rare due to maternal steroid therapy; CHOP and UCSF combined have performed one case in the past 5 years. (clinical) [Ep 3 · 28:44](https://library.globalcastmd.com/watch/open-fetal-surgery-overview-fetal-surgery-2012-1027?t=1724) - The CPAM volume ratio (CVR) is calculated by measuring the CPAM in 3 dimensions, applying an ellipse formula, and dividing by head circumference to standardize for gestational age. — Alan Flake (clinical) [Ep 3 · 26:38](https://library.globalcastmd.com/watch/open-fetal-surgery-overview-fetal-surgery-2012-1027?t=1598) - CVR >1.6 predicts high risk for CPAM evolving into hydrops, particularly for microcystic lesions. — Alan Flake (clinical) [Ep 3 · 6:11](https://library.globalcastmd.com/watch/open-fetal-surgery-overview-fetal-surgery-2012-1027?t=371) - Maternal steroids are first-line therapy for high-risk CPAM (CVR >1.6); the majority respond, but open fetal surgery is considered if hydrops persists despite steroids at an early gestational age. — Alan Flake (clinical) [Ep 3 · 6:23](https://library.globalcastmd.com/watch/open-fetal-surgery-overview-fetal-surgery-2012-1027?t=383) - CHOP's open fetal surgery for CPAM has approximately 60% survival; thoracoamniotic shunts have 70-75% survival. — Alan Flake (epidemiological) [Ep 3 · 10:34](https://library.globalcastmd.com/watch/open-fetal-surgery-overview-fetal-surgery-2012-1027?t=634) - Bronchial atresia is more difficult to treat by fetal intervention than CPAM; CHOP has had one intraoperative fetal death, one postnatal hepatic necrosis death, and one likely survivor out of three cases. — Alan Flake (clinical) [Ep 3 · 10:49](https://library.globalcastmd.com/watch/open-fetal-surgery-overview-fetal-surgery-2012-1027?t=649) - Sacrococcygeal teratoma fetal intervention targets tumor vascular steal leading to high-output cardiac failure; surgical debulking interrupts the arteriovenous steal. — Alan Flake (clinical) [Ep 3 · 12:18](https://library.globalcastmd.com/watch/open-fetal-surgery-overview-fetal-surgery-2012-1027?t=738) - CHOP's recent SCT algorithm emphasizes preemptive early delivery (≥27 weeks) at the first sign of maternal or fetal decompensation, with improved outcomes in 5 recent cases. — Alan Flake (clinical) [Ep 3 · 16:33](https://library.globalcastmd.com/watch/open-fetal-surgery-overview-fetal-surgery-2012-1027?t=993) - Watchful waiting for SCT between 27-32 weeks can be hazardous; the majority of patients who go into preterm labor or evolve significant high-output failure will die. — Alan Flake (clinical) [Ep 3 · 16:03](https://library.globalcastmd.com/watch/open-fetal-surgery-overview-fetal-surgery-2012-1027?t=963) - The EXIT procedure maintains uteroplacental blood flow using complete uterine relaxation with deep maternal gaseous anesthetic and intrauterine volume maintenance. — Alan Flake (clinical) [Ep 3 · 18:41](https://library.globalcastmd.com/watch/open-fetal-surgery-overview-fetal-surgery-2012-1027?t=1121) - The EXIT procedure was initially developed to remove tracheal clips after tracheal occlusion for CDH. — Alan Flake (clinical) [Ep 3 · 18:25](https://library.globalcastmd.com/watch/open-fetal-surgery-overview-fetal-surgery-2012-1027?t=1105) - A massive cervical teratoma case required 11.5 hours on placental support during EXIT, with retrograde and antegrade cannulation attempts, ultimately requiring tumor dissection to reach the trachea. — Alan Flake (clinical) [Ep 3 · 20:04](https://library.globalcastmd.com/watch/open-fetal-surgery-overview-fetal-surgery-2012-1027?t=1204) - CHAOS (congenital high airway obstruction syndrome) can result in marked diaphragm inversion, ascites, and thoracic abnormalities; the first survivor was delivered by EXIT. — Alan Flake (clinical) [Ep 3 · 21:05](https://library.globalcastmd.com/watch/open-fetal-surgery-overview-fetal-surgery-2012-1027?t=1265) - CHAOS has a strong rationale for prenatal treatment due to morbidity associated with treating at birth and the need for patients to grow into their ventilatory mechanics. — Alan Flake (opinion) [Ep 3 · 21:33](https://library.globalcastmd.com/watch/open-fetal-surgery-overview-fetal-surgery-2012-1027?t=1293) - Preterm labor in hydropic CPAM patients may be secondary to a maternal-fetal immune response, and steroids may ameliorate this response. (opinion) [Ep 3 · 30:24](https://library.globalcastmd.com/watch/open-fetal-surgery-overview-fetal-surgery-2012-1027?t=1824) - Echocardiographic assessment of cardiac function is a more specific indicator of fetal heart failure than fluid in body compartments alone. (clinical) [Ep 3 · 31:39](https://library.globalcastmd.com/watch/open-fetal-surgery-overview-fetal-surgery-2012-1027?t=1899) - Some fetuses with massive ascites and mild pleural effusion can be observed if cardiac function remains intact, even before steroids became popular. (clinical) [Ep 3 · 31:49](https://library.globalcastmd.com/watch/open-fetal-surgery-overview-fetal-surgery-2012-1027?t=1909) - The CVR has been a very good way to counsel families and determine the pace of follow-up; high CVR may warrant follow-up 2-3 times per week. (clinical) [Ep 3 · 33:34](https://library.globalcastmd.com/watch/open-fetal-surgery-overview-fetal-surgery-2012-1027?t=2014) - CPAM growth rate can be quite abrupt between 18 and 26-28 weeks gestation, then tends to plateau; this is useful for counseling. (clinical) [Ep 3 · 34:11](https://library.globalcastmd.com/watch/open-fetal-surgery-overview-fetal-surgery-2012-1027?t=2051) - Steroids may put the CPAM further along the growth curve in terms of gestational age, which might explain their effect. (opinion) [Ep 3 · 34:30](https://library.globalcastmd.com/watch/open-fetal-surgery-overview-fetal-surgery-2012-1027?t=2070) - In Cincinnati's experience with 56 CPAM cases, prophylactic steroids for CVR >1.6 resulted in literally 100% survival. (epidemiological) [Ep 3 · 36:28](https://library.globalcastmd.com/watch/open-fetal-surgery-overview-fetal-surgery-2012-1027?t=2188) - In Cincinnati's experience, once hydrops was established, steroids resulted in 49% survival and good response. (epidemiological) [Ep 3 · 36:42](https://library.globalcastmd.com/watch/open-fetal-surgery-overview-fetal-surgery-2012-1027?t=2202) - If one course of steroids fails, a second course salvages about 2 out of 6 patients; others go on to fetal surgery. (clinical) [Ep 3 · 36:51](https://library.globalcastmd.com/watch/open-fetal-surgery-overview-fetal-surgery-2012-1027?t=2211) - Steroids are most effective in microcystic (solid) CPAM forms, but are used even in type 1 and type 2 lesions to arrest growth of the solid component, though less efficacious. (clinical) [Ep 3 · 35:52](https://library.globalcastmd.com/watch/open-fetal-surgery-overview-fetal-surgery-2012-1027?t=2152) - Macrocystic CPAM and bronchial atresia do not respond as well to steroids as microcystic CPAM. — Alan Flake (clinical) [Ep 3 · 29:19](https://library.globalcastmd.com/watch/open-fetal-surgery-overview-fetal-surgery-2012-1027?t=1759) - Bronchial atresia can be differentiated from microcystic CPAM by the presence of a dilated central bronchus or mucocele and documentation of a contralateral lung to rule out lung agenesis. — Alan Flake (clinical) [Ep 3 · 3:45](https://library.globalcastmd.com/watch/open-fetal-surgery-overview-fetal-surgery-2012-1027?t=225) - Open fetal surgery for CPAM is indicated when hydrops persists despite steroids at an early gestational time point, if the capability exists. — Alan Flake (clinical) [Ep 3 · 6:31](https://library.globalcastmd.com/watch/open-fetal-surgery-overview-fetal-surgery-2012-1027?t=391) - Macrocystic CPAM can be treated by thoracoamniotic shunt rather than open fetal surgery. — Alan Flake (clinical) [Ep 3 · 9:40](https://library.globalcastmd.com/watch/open-fetal-surgery-overview-fetal-surgery-2012-1027?t=580) - RFA (radiofrequency ablation) technology for SCT has not identified a safe ablative technology that avoids collateral injury and bad outcomes; it is not recommended at present. — Alan Flake (clinical) [Ep 3 · 17:33](https://library.globalcastmd.com/watch/open-fetal-surgery-overview-fetal-surgery-2012-1027?t=1053) - Future directions in fetal surgery include clinical trials, reduction of maternal and fetal risk, better imaging for physiologic information, and tissue engineering approaches to make open fetal surgery obsolete. — Alan Flake (opinion) [Ep 3 · 23:57](https://library.globalcastmd.com/watch/open-fetal-surgery-overview-fetal-surgery-2012-1027?t=1437) - The EXIT procedure requires a multidisciplinary team with specific roles, and may require a second operating room team for definitive postnatal procedures and ECMO capability. — Alan Flake (clinical) [Ep 3 · 19:10](https://library.globalcastmd.com/watch/open-fetal-surgery-overview-fetal-surgery-2012-1027?t=1150) - CPAMs are abnormally developed lung tissue that doesn't participate in gas exchange, coming off normal bronchial airways but lacking normal alveoli. — Pam Choi and Dr. Beth Romesky (clinical) [Ep 4 · 3:47](https://library.globalcastmd.com/watch/the-full-story-on-cpams-4463?t=227) - Prenatal steroids (betamethasone 12mg given twice, 24 hours apart) have been shown to resolve hydrops and reduce the size of CPAM lesions such that the CVR can decrease. — Pam Choi and Dr. Beth Romesky (clinical) [Ep 4 · 10:42](https://library.globalcastmd.com/watch/the-full-story-on-cpams-4463?t=642) - A thoracoamniotic shunt is placed so that half is inside the fetal chest and half is in the amniotic space, allowing egress of fluid from the fetal chest into the amniotic space for continued decompression throughout pregnancy. — Pam Choi and Dr. Beth Romesky (clinical) [Ep 4 · 13:33](https://library.globalcastmd.com/watch/the-full-story-on-cpams-4463?t=813) - Fetal resection involves partially delivering the fetus, performing a thoracotomy, removing part of the lung, and placing the fetus back in the uterus to continue the pregnancy. — Pam Choi and Dr. Beth Romesky (clinical) [Ep 4 · 15:11](https://library.globalcastmd.com/watch/the-full-story-on-cpams-4463?t=911) - Mirror syndrome means the mother is showing evidence of what the baby has going on, with the mother developing significant edema, pleural effusion, and other symptoms very quickly. — Pam Choi and Dr. Beth Romesky (clinical) [Ep 4 · 16:15](https://library.globalcastmd.com/watch/the-full-story-on-cpams-4463?t=975) - During an EXIT procedure, the baby remains attached to mom through the umbilical cord while a thoracotomy and resection are performed, with the time available depending on how well mom tolerates it and uterine relaxation, typically ranging from a few minutes to rarely longer than an hour. — Pam Choi and Dr. Beth Romesky (clinical) [Ep 4 · 17:05](https://library.globalcastmd.com/watch/the-full-story-on-cpams-4463?t=1025) - Air trapping can occur where air enters the CPAM area as the infant breathes in but doesn't come out as easily, causing progressive hyperinflation of the non-functioning lobe and rapid progressive respiratory distress. — Pam Choi and Dr. Beth Romesky (clinical) [Ep 4 · 19:42](https://library.globalcastmd.com/watch/the-full-story-on-cpams-4463?t=1182) - Features suggesting higher risk of PPB include: lesion in more than one lobe, in more than one lung, associated pneumothorax, and DICER1 genetic mutation. — Jack Langer (clinical) [Ep 4 · 28:25](https://library.globalcastmd.com/watch/the-full-story-on-cpams-4463?t=1705) - Features suggesting very low risk of PPB are a feeding vessel and prenatal diagnosis. — Jack Langer (clinical) [Ep 4 · 28:59](https://library.globalcastmd.com/watch/the-full-story-on-cpams-4463?t=1739) - Dr. Langer follows observed CPAM patients with regular chest X-rays for the first 2 years, expecting to see changes on serial X-rays if a PPB is developing from type 1 to type 2, then follows clinically after that. — Jack Langer (clinical) [Ep 4 · 30:56](https://library.globalcastmd.com/watch/the-full-story-on-cpams-4463?t=1856) - If a CPAM becomes infected, it should be treated with IV antibiotics, allowed to settle down, then removed, as infected CPAMs make surgery more difficult with more blood loss and complications. — Jack Langer (clinical) [Ep 4 · 31:51](https://library.globalcastmd.com/watch/the-full-story-on-cpams-4463?t=1911) - Single lung ventilation for thoracoscopic lobectomy is obtained by mainstem intubation of the contralateral bronchus, preventing the lung from overinflating during dissection of key vessels or structures. — Steven Rothenberg (clinical) [Ep 4 · 36:43](https://library.globalcastmd.com/watch/the-full-story-on-cpams-4463?t=2203) - For thoracoscopic lobectomy, the scope port should be placed over the major fissure in the mid-axillary line, anterior to the tip of the scapula, to allow working from front to back and avoid working in paradox. — Steven Rothenberg (clinical) [Ep 4 · 39:02](https://library.globalcastmd.com/watch/the-full-story-on-cpams-4463?t=2342) - When completing an incomplete fissure during lobectomy, work through it layer at a time like finger fracturing during liver lobectomy, starting at the front and working posteriorly until exposing the pulmonary artery. — Steven Rothenberg (clinical) [Ep 4 · 41:39](https://library.globalcastmd.com/watch/the-full-story-on-cpams-4463?t=2499) - Vascular control in thoracoscopic lobectomy is everything; dissect out vessels to get enough length to make a seal proximally and distally, then cut partway between seals to check for bleeding before completing division. — Steven Rothenberg (clinical) [Ep 4 · 43:09](https://library.globalcastmd.com/watch/the-full-story-on-cpams-4463?t=2589) - The bronchus sits right underneath the pulmonary artery and can be felt to help dissect behind the artery during lobectomy. — Steven Rothenberg (clinical) [Ep 4 · 44:13](https://library.globalcastmd.com/watch/the-full-story-on-cpams-4463?t=2653) - When dissecting behind the bronchus during lobectomy, stay hard on the backside of the bronchus and be aware that the pulmonary vein is right behind it. — Steven Rothenberg (clinical) [Ep 4 · 46:57](https://library.globalcastmd.com/watch/the-full-story-on-cpams-4463?t=2817) - Do not take the pulmonary vein trunk near the pericardium during lobectomy, because if the device fails, the vessel will retract into the pericardium and the child will bleed to death before you can do anything; ensure enough length away from pericardium for proximal control. — Steven Rothenberg (clinical) [Ep 4 · 48:44](https://library.globalcastmd.com/watch/the-full-story-on-cpams-4463?t=2924) ## Host summaries Recaps by a host of what the experts said — not the host's own clinical position. - If using the LigaSure device, do not activate the cutting function; seal, remove the device, and cut with scissors to allow partial division and confirmation of hemostasis. — Todd Ponsky summarizes what Dr. Steven Rothenberg said [Ep 1 · 39:28](https://library.globalcastmd.com/watch/neonatal-lung-lesions-with-dr-steven-rothenberg-307?t=2368) - Steroids may drive lung development and maturation of microcystic CPAM tissue, though this is unproven. — The host summarizing the discussion [Ep 3 · 29:41](https://library.globalcastmd.com/watch/open-fetal-surgery-overview-fetal-surgery-2012-1027?t=1781) - Prophylactic steroid treatment for CVR >1.6 in the absence of hydrops is practiced, though there was equipoise for a randomized study because most centers give steroids in that group. — The host summarizing the discussion [Ep 3 · 37:17](https://library.globalcastmd.com/watch/open-fetal-surgery-overview-fetal-surgery-2012-1027?t=2237) - The Stocker classification for CPAMs ranges from type 0 to type 4, moving from proximal to distal in the bronchial tree, with type 1 (distal bronchi/proximal bronchioles) being most common at 60-70% of cases. — Rod Gerardo and Ellen Ancisco summarizing the discussion [Ep 4 · 5:12](https://library.globalcastmd.com/watch/the-full-story-on-cpams-4463?t=312) - CPAMs typically grow during the first 20-25 weeks of gestation and plateau around week 28. — Rod Gerardo and Ellen Ancisco summarizing the discussion [Ep 4 · 6:57](https://library.globalcastmd.com/watch/the-full-story-on-cpams-4463?t=417) - The CPAM volume ratio (CVR) is calculated as the CPAM's length times width times height times 0.52 divided by the head circumference. — Rod Gerardo and Ellen Ancisco summarizing the discussion [Ep 4 · 8:41](https://library.globalcastmd.com/watch/the-full-story-on-cpams-4463?t=521) - In patients with CVR over 1.6, it is estimated that 75% develop hydrops. — Rod Gerardo and Ellen Ancisco summarizing the discussion [Ep 4 · 9:14](https://library.globalcastmd.com/watch/the-full-story-on-cpams-4463?t=554) - The presence of hydrops is the strongest prognostic indicator of mortality in fetuses with CPAMs. — Rod Gerardo and Ellen Ancisco summarizing the discussion [Ep 4 · 8:02](https://library.globalcastmd.com/watch/the-full-story-on-cpams-4463?t=482) - CPAMs are differentiated as microcystic (less than 5 millimeters) or macrocystic (larger than 5 millimeters). — Rod Gerardo and Ellen Ancisco summarizing the discussion [Ep 4 · 10:14](https://library.globalcastmd.com/watch/the-full-story-on-cpams-4463?t=614) - CPAMs are not well-visualized on chest X-rays and can regress or get smaller over time. — Rod Gerardo and Ellen Ancisco summarizing the discussion [Ep 4 · 22:25](https://library.globalcastmd.com/watch/the-full-story-on-cpams-4463?t=1345) - In the United States, most surgeons resect CPAMs even if asymptomatic, while in Europe and Canada, surgeons sometimes choose to monitor asymptomatic lesions and avoid surgery. — Rod Gerardo and Ellen Ancisco summarizing the discussion [Ep 4 · 24:40](https://library.globalcastmd.com/watch/the-full-story-on-cpams-4463?t=1480) - The main reasons for operating on asymptomatic CPAMs are: risk of malignancy both at resection and from malignant transformation later, risk of infection that will make surgery more difficult, and better compensatory lung growth if operating sooner in life. — Rod Gerardo and Ellen Ancisco summarizing the discussion [Ep 4 · 25:29](https://library.globalcastmd.com/watch/the-full-story-on-cpams-4463?t=1529) - Thoracoscopic lobectomy risks include bleeding, prolonged post-op mechanical ventilation, infections, air leak, pneumothorax, nerve injury, and mortality. — Rod Gerardo and Ellen Ancisco summarizing the discussion [Ep 4 · 26:03](https://library.globalcastmd.com/watch/the-full-story-on-cpams-4463?t=1563) - The particular malignancy associated with CPAM is pleuropulmonary blastoma (PPB), with risk that the lesion at birth already has malignancy and risk of malignant transformation over a person's life. — Rod Gerardo and Ellen Ancisco summarizing the discussion [Ep 4 · 27:47](https://library.globalcastmd.com/watch/the-full-story-on-cpams-4463?t=1667) - A recent study published in April 2021 found that no prenatally diagnosed lesions had malignancy, but 10% of patients diagnosed postnatally had malignancy in their resected mass. — Rod Gerardo and Ellen Ancisco summarizing the discussion [Ep 4 · 30:17](https://library.globalcastmd.com/watch/the-full-story-on-cpams-4463?t=1817) - The incidence of CPAMs is estimated to be about 1 in every 8,000 to 35,000 births. — Rod Gerardo and Ellen Ancisco summarizing the discussion [Ep 4 · 52:39](https://library.globalcastmd.com/watch/the-full-story-on-cpams-4463?t=3159) - 60% of prenatally diagnosed lung lesions are CPAMs, making them the most common prenatal lung lesion. — Rod Gerardo and Ellen Ancisco summarizing the discussion [Ep 4 · 52:53](https://library.globalcastmd.com/watch/the-full-story-on-cpams-4463?t=3173) --- Educational content from recorded physician discussions — not medical advice. 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