Open Fetal Surgery Overview: Fetal Surgery 2012
With Dr. Alan Flake · StayCurrentMD
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Educational content from recorded physician discussions — not medical advice. Talk to your (or your child's) care team about your situation.
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What the experts said
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.
CVR >1.6 predicts high risk for CPAM evolving into hydrops, particularly for microcystic lesions.
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.
Open fetal surgery for CPAM is indicated when hydrops persists despite steroids at an early gestational time point, if the capability exists.
Macrocystic CPAM can be treated by thoracoamniotic shunt rather than open fetal surgery.
CHOP's open fetal surgery for CPAM has approximately 60% survival; thoracoamniotic shunts have 70-75% survival.
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.
Sacrococcygeal teratoma fetal intervention targets tumor vascular steal leading to high-output cardiac failure; surgical debulking interrupts the arteriovenous steal.
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.
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.
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.
The EXIT procedure was initially developed to remove tracheal clips after tracheal occlusion for CDH.
The EXIT procedure maintains uteroplacental blood flow using complete uterine relaxation with deep maternal gaseous anesthetic and intrauterine volume maintenance.
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.
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.
CHAOS (congenital high airway obstruction syndrome) can result in marked diaphragm inversion, ascites, and thoracic abnormalities; the first survivor was delivered by EXIT.
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.
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.
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.
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.
Macrocystic CPAM and bronchial atresia do not respond as well to steroids as microcystic CPAM.
Preterm labor in hydropic CPAM patients may be secondary to a maternal-fetal immune response, and steroids may ameliorate this response.
Echocardiographic assessment of cardiac function is a more specific indicator of fetal heart failure than fluid in body compartments alone.
Some fetuses with massive ascites and mild pleural effusion can be observed if cardiac function remains intact, even before steroids became popular.
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.
CPAM growth rate can be quite abrupt between 18 and 26-28 weeks gestation, then tends to plateau; this is useful for counseling.
Steroids may put the CPAM further along the growth curve in terms of gestational age, which might explain their effect.
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.
In Cincinnati's experience with 56 CPAM cases, prophylactic steroids for CVR >1.6 resulted in literally 100% survival.
In Cincinnati's experience, once hydrops was established, steroids resulted in 49% survival and good response.
If one course of steroids fails, a second course salvages about 2 out of 6 patients; others go on to fetal surgery.
Steroids may drive lung development and maturation of microcystic CPAM tissue, though this is unproven.
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.