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Open Fetal Surgery Overview: Fetal Surgery 2012

Video Published 2019-01-11 Updated 2022-08-22

Timestops (6)

Topic Overview

This discussion covers current applications of open fetal surgery, focusing on congenital pulmonary airway malformations (CPAM), sacrococcygeal teratoma (SCT), and the EXIT procedure. The speaker presents CHOP's experience showing that maternal steroid therapy has dramatically reduced the need for open fetal surgery in microcystic CPAM cases, though open surgery remains necessary for rare steroid-refractory cases and certain SCT presentations. The panel discusses the evolution from invasive open procedures toward less invasive approaches, with emphasis on careful patient selection, the role of the CPAM volume ratio (CVR) in risk stratification, and the importance of multidisciplinary coordination for EXIT procedures in airway-threatening lesions.

Key Takeaways

  • Maternal steroids dramatically reduced open fetal surgery for microcystic CPAM; CHOP+UCSF now ~1 case per 5 years combined. (28:44)
  • CVR >1.6 predicts hydrops risk in CPAM. Prophylactic steroids for high CVR achieved 100% survival in Cincinnati's 56-case series. (6:01)
  • SCT fetal deaths cluster 27-32 weeks. CHOP shifted to preemptive delivery at first decompensation sign with good outcomes. (15:51)
  • Steroids work best for microcystic CPAM (not macrocystic/bronchial atresia). Once hydrops present, survival drops to 49%. (29:23)
  • EXIT procedure demands multidisciplinary coordination: maintain uteroplacental flow, complete uterine relaxation, intrauterine volume. (18:41)

Inside this episode

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

AI-enriched

Who's speaking

  • Alan Flake — guest
  • Speaker 2 — host
  • Speaker 3 — guest
  • Doctor Farmer — guest
  • Speaker 5 — guest
  • Doctor Adzik — guest
  • Speaker 7

Chapters

  • 0:00Introduction and Overview of Open Fetal Surgery — Introduction to the session on open fetal surgery applications, moving from more invasive to less invasive approaches. Overview of congenital defects amenable to fetal intervention, categorized by acceptance level.
  • 3:00CPAM: Classification, Natural History, and Intervention — Discussion of CPAM prenatal behavior, classification (microcystic vs macrocystic), CVR risk stratification, and the role of maternal steroids. Presentation of a recent open fetal surgery case for steroid-refractory CPAM with hydrops.
  • 10:50Bronchial Atresia Experience — CHOP's challenging experience with three bronchial atresia cases treated by fetal intervention, including one intraoperative fetal death, one hepatic necrosis, and one ongoing NICU case.
  • 12:00Sacrococcygeal Teratoma: Pathophysiology and Surgical Approach — SCT fetal pathophysiology focusing on high-output cardiac failure from tumor vascular steal. Presentation of a successful case with 11-week post-operative gestation and discussion of early delivery protocols for decompensating cases.
  • 18:15EXIT Procedure: Technique and Applications — Overview of EXIT procedure components, team requirements, and applications including cervical teratoma, CHAOS, oropharyngeal teratoma, and mediastinal/pericardial teratomas. Emphasis on maintaining uteroplacental blood flow and multidisciplinary coordination.
  • 24:30Panel Discussion: Steroids, CVR, and Clinical Decision-Making — Panel discussion on steroid efficacy in CPAM, the rarity of open fetal surgery for CPAM in current practice, cardiac assessment in hydrops, CVR utility for counseling and follow-up tempo, and protocols for prophylactic steroid administration.

Key claims

  • 1:08Most congenital defects are still treated by waiting and treating the neonate, with fetal intervention reserved for a very few appropriately selected cases — Alan Flake
  • 1:41Accepted indications for fetal surgery include pulmonary airway malformations, bronchial atresia, CHAOS, sacrococcygeal teratoma, and myelomeningocele — Alan Flake
  • 2:53The majority of CPAM lesions regress late in gestation and do not require any fetal intervention — Alan Flake
  • 3:31Bronchial atresia can masquerade as microcystic CPAM but is distinguished by a dilated central bronchus or mucocele and requires documentation of contralateral lung presence — Alan Flake
  • 5:13Hydrops is the sole indication for consideration of open fetal surgery in CPAM — Alan Flake
  • 6:01CVR (CPAM volume ratio) greater than 1.6 indicates high risk for evolution to hydrops — Alan Flake
  • 6:15The majority of high-risk CPAMs respond to a trial of maternal steroids — Alan Flake
  • 28:44Open fetal surgery for CPAM is now extremely rare, with CHOP and UCSF reporting approximately one case in five years combined — Speaker 3
  • 29:11Steroids have had a dramatic impact on reducing the number of open fetal surgeries for microcystic CPAM — Alan Flake
  • 29:23Macrocystic CPAMs and bronchial atresia do not respond as well to steroids as microcystic CPAMs — Alan Flake
  • 10:34Open fetal surgery for CPAM has approximately 60% survival — Alan Flake
  • 10:34Thoracoamniotic shunts for macrocystic CPAM have 70-75% survival — Alan Flake
  • 10:52CHOP has treated three bronchial atresia cases by fetal intervention with one intraoperative fetal death, one hepatic necrosis death, and one ongoing NICU case — Alan Flake
  • 11:46Bronchial atresia is a much more difficult lesion to treat than CPAMs — Alan Flake
  • 12:18SCT fetal pathophysiology includes tumor vascular steal leading to high output cardiac failure — Alan Flake
  • 12:28Fetal SCT resection aims to interrupt arteriovenous steal from the low resistance tumor to prevent progression of high output failure — Alan Flake
  • 13:16Fetal SCT surgery is a quick debulking procedure to remove arteriovenous steal without coccyxectomy — Alan Flake
  • 15:51Watchful waiting in SCT can be hazardous, with majority of deaths occurring between 27-32 weeks gestation — Alan Flake
  • 16:33CHOP now uses a preemptive approach for SCT, delivering patients after 27 weeks at the slightest hint of fetal or maternal decompensation — Alan Flake
  • 16:59Five recent SCT cases managed with early delivery protocol had good outcomes with surprisingly little prematurity morbidity — Alan Flake
  • 17:36No safe ablative technology has been identified for SCT that does not induce significant collateral injury — Alan Flake
  • 18:41Essential components of EXIT procedure include maintenance of uteroplacental blood flow, complete uterine relaxation, maintenance of intrauterine volume, maternal homeostasis and hemostasis, control of membranes, and avoiding placenta and cord — Alan Flake
  • 19:10EXIT procedure requires a multidisciplinary team with specific roles for each member — Alan Flake
  • 21:05CHAOS (congenital high airway obstruction syndrome) can result in marked diaphragm inversion, ascites, and thoracic abnormalities — Alan Flake
  • 21:33CHAOS patients have to grow into their ventilatory mechanics and can have significant morbidity when treated at birth — Alan Flake
  • 26:38CVR is calculated by measuring CPAM in 3 dimensions, applying ellipse formula, and dividing by head circumference to standardize for gestational age — Alan Flake
  • 27:05CVR has been reliable for predicting hydrops risk, determining surveillance frequency, and counseling families — Alan Flake
  • 27:48Failure of steroid therapy is defined as progression of hydrops — Alan Flake
  • 28:05Patients on steroids for CPAM should be monitored with ultrasound every other day or 2-3 times per week because changes can occur rapidly — Alan Flake
  • 29:41One theory is that steroids drive lung development and make microcystic CPAM tissue mature more completely — Speaker 3
  • 30:19Steroids may ameliorate maternal immune response against the fetus in hydropic patients — Speaker 3
  • 30:49The evolution of fetal intervention for CPAM is a very happy story, demonstrating that solving one problem is not a reason to stop investigation — Doctor Farmer
  • 31:25Some fetuses with massive ascites and mild pleural effusion can be observed if cardiac function remains intact, rather than immediately intervening — Speaker 5
  • 33:34CVR has been useful for counseling families and determining follow-up tempo, with high CVR patients followed 2-3 times per week — Doctor Adzik
  • 34:11CPAM growth rate can be quite abrupt between 18 and 26-28 weeks gestation, then tends to plateau — Doctor Adzik
  • 35:52Steroids are most effective in microcystic/solid CPAM forms but are used even in type 1 and type 2 cases to arrest growth of solid components — Speaker 3
  • 36:28In Cincinnati experience with 56 CPAM cases, prophylactic treatment for CVR above 1.6 resulted in literally 100% survival — Speaker 3
  • 36:42Once hydrops is established in CPAM, steroids achieve 49% survival — Speaker 3
  • 36:51If first course of steroids fails in CPAM, second course salvages about 2 out of 6 patients, with others proceeding to fetal surgery — Speaker 3

Cases discussed

  • 6:4223-week gestation fetus with left lower lobe heterogeneous CPAM, CVR 2.7, treated with two courses of maternal steroids, progressed to hydrops with CVR 3.4, underwent open fetal surgery at 25.5 weeks
  • 9:40Macrocystic CPAM with CVR 3.6 treated successfully with thoracoamniotic shunt, CVR dropped to approximately 0.8
  • 10:52Right mainstem bronchial atresia case with intraoperative fetal death during attempted fetal intervention
  • 10:52Left mainstem bronchial atresia with hepatic necrosis following fetal surgery, resulting in neonatal death
  • 10:52Left upper lobe bronchial atresia progressing to hydrops, treated with left upper lobectomy, currently in NICU with stormy course but likely to survive
  • 13:3322-week gestation predominantly solid type 1 SCT with high blood flow, evolved high output cardiac failure between 22-23 weeks, underwent fetal debulking, remained in utero 11 weeks, delivered stable, underwent definitive resection, normal at 2 years
  • 19:55Massive cervical teratoma requiring 11.5-hour EXIT procedure, airway could not be achieved from above, required tumor dissection from thoracic inlet and retrograde attempts, ultimately successful with surgeon stabilizing ET tube during transfer to adjacent OR for tumor resection
  • 21:05CHAOS case with laryngeal cyst, first survivor of CHAOS delivered by EXIT procedure, required time to grow into ventilatory mechanics with significant morbidity
  • 21:51Massive oropharyngeal teratoma with tumor visible from nostrils, delivered by EXIT with tracheostomy, tumor debulked then ENT performed hypopharyngeal resection, child normal at 5-6 years
  • 22:51Mediastinal teratoma with cardiac compression, EXIT procedure with median sternotomy required before ET tube would advance, tumor easily resected after exposure
  • 23:28Pericardial teratoma compressing right atrium causing hydrops with large left pleural effusion, EXIT procedure with cardiac surgery, tumor attached to aortic base, resected without bypass, good outcome

Open questions

  • What is the mechanism by which maternal steroids prevent CPAM growth and hydrops evolution?
  • Is there a role for maternal fetal immune response in preterm labor in hydropic patients, and do steroids ameliorate this?
  • Should prophylactic steroids be given for all CPAM cases with CVR > 1.6, or should treatment wait for hydrops development?
  • How many courses of steroids should be attempted before proceeding to open fetal surgery?
  • Can a safer, more focused ablative technology be developed for SCT that avoids collateral injury?
  • Should CSF obstruction be reconsidered as an indication for fetal intervention?
  • What is the optimal timing and criteria for early delivery in SCT cases to balance prematurity risk against fetal decompensation?
This episode was analyzed and enriched by Kai, the Library's AI content creator. Every item links to the moment it comes from — click a timestamp to listen in context.
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