Alan Flake

130 statements · 5 topics

Congenital Lung Lesions (CPAM) · guest expert Fetal Surgery · guest expert

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At the time of birth, the lungs that had grown the most perhaps functioned the worst. Those kids were born relatively premature, but they didn't respond to surfactant. They had poor compliance of their lungs. It was not a biologically functioning gas exchanging lung.

quote · Congenital Diaphragmatic Hernia

I don't think as a rule at this point there's overwhelming data that we improve lung function with tracheal occlusion.

quote · Congenital Diaphragmatic Hernia

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.

clinical · Congenital Lung Lesions (CPAM)

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.

clinical · Congenital Lung Lesions (CPAM)

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.

clinical · Congenital Pulmonary Airway Malformation

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.

clinical · Congenital Pulmonary Airway Malformation

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Alan's statements about Congenital Diaphragmatic Hernia 5 statements

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Fetoscopic endoluminal tracheal occlusion and twin-twin transfusion: Fetal...

Quotation One major difference between Your trial and any trial on uh tracheal occlusion versus the myelomeningocele trial is that you have popularized this over the past 10 years. Open

Quotation So, has this impacted your effort to do a trial? Have you been able to recruit the numbers of patients that you anticipated? Open

Quotation I mean, I would argue, you know, when we did our tracheal occlusion trial, we got tremendous lung growth in some of the kids. We got no lung growth in other kids, and this is with complete tracheal occlusion. Open

Quotation At the time of birth, the lungs that had grown the most perhaps functioned the worst. Those kids were born relatively premature, but they didn't respond to surfactant. They had poor compliance of their lungs. It was not a biologically functioning gas exchanging lung. Open

Quotation I don't think as a rule at this point there's overwhelming data that we improve lung function with tracheal occlusion. Open

Alan's statements about Congenital Lung Lesions (CPAM) 46 statements

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Open Fetal Surgery Overview: Fetal Surgery 2012

Quotation you got to give Mike credit for having, uh, real cojones to, uh, start this field. Open

Quotation most of the time what we do is to wait and treat the neonate still, but we do have a whole armamentarium of options for timing and mode of delivery, and still in a very few cases to with appropriate selection treat patients in utero. Open

Statement 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. Open

Statement CVR >1.6 predicts high risk for CPAM evolving into hydrops, particularly for microcystic lesions. Open

Statement 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

Statement Open fetal surgery for CPAM is indicated when hydrops persists despite steroids at an early gestational time point, if the capability exists. Open

Quotation The key to fetal surgery is putting the kid back in the uterus and keeping them there for an adequate length of time for lung growth to occur. Open

Statement Macrocystic CPAM can be treated by thoracoamniotic shunt rather than open fetal surgery. Open

Statement CHOP's open fetal surgery for CPAM has approximately 60% survival; thoracoamniotic shunts have 70-75% survival. Open

Statement 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. Open

Statement Sacrococcygeal teratoma fetal intervention targets tumor vascular steal leading to high-output cardiac failure; surgical debulking interrupts the arteriovenous steal. Open

Statement 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. Open

Statement 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. Open

Statement 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. Open

Statement The EXIT procedure was initially developed to remove tracheal clips after tracheal occlusion for CDH. Open

Statement The EXIT procedure maintains uteroplacental blood flow using complete uterine relaxation with deep maternal gaseous anesthetic and intrauterine volume maintenance. Open

Statement 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. Open

Statement 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. Open

Statement CHAOS (congenital high airway obstruction syndrome) can result in marked diaphragm inversion, ascites, and thoracic abnormalities; the first survivor was delivered by EXIT. Open

Statement 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. Open

Statement 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. Open

Statement 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

Statement Macrocystic CPAM and bronchial atresia do not respond as well to steroids as microcystic CPAM. Open

Alan's statements about Congenital Pulmonary Airway Malformation 23 statements

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