From
StayCurrentMD
Severe CDH case with FETO: unexpected anatomic findings and clinical outcome
With Dr. Beth Romeske · hosted by Dr. Jill Knepprath
Chapter 1 of 4
Initial diagnosis
Case presentation and initial prenatal diagnosis
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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
The patient was a 33-year-old G3P2 diagnosed with CDH on routine 20-week ultrasound
The left lung was so small they couldn't measure it (less than 1 mL)
The O/E (observed to expected lung to head ratio) by ultrasound was 14%
While the balloon was in place, the lungs showed some growth but it was shy of 20%, making this a non-responder
Post-FETO imaging showed left lung volume just over 2 mL (increased from less than 1 mL) and some interval growth in the right lung, but overall still really poor numbers
The baby was immediately put on ECMO in the delivery room
The baby had only 6 pairs of ribs at birth, missing a lot of ribs
The baby had spinal kyphosis
The baby had no anus (anorectal malformation)
The cardiac anatomy was determined to be a right dominant double aortic arch with narrowing on the right and atresia on the left, after considerable discussion about what the anatomy actually was
The baby remained on ECMO for several weeks
Multiple attempts were made to wean off ECMO, achieving partial weaning but never getting below a certain threshold
After discussion with the family, they decided to withdraw support and the baby passed at around 3 weeks of life, never making it off ECMO
Radiology reviewed the prenatal imaging retrospectively knowing the postnatal findings and still could not identify the abnormalities that were found after birth
FETO uses a fetoscope to insert a balloon into the fetal trachea to temporarily block it, trapping fluid in the lungs to allow expansion and growth
The PPLV (percent predicted lung volume) on MRI was 7%
The balloon was placed early at 27 weeks and 4 days gestation
Dr. Romeski stated this patient was the ideal case for FETO, with not a single hospitalization stay during the procedure period
The balloon was removed at 34 weeks gestation
A non-responder is a fetus with CDH who received FETO but did not show any improvement in lung growth
The remainder of the pregnancy was uneventful
The patient went into labor at 37 weeks and 3 days and had a vaginal delivery
The Apgar scores for the baby were 2 and 1
The baby had several unexpected findings after delivery despite extensive prenatal imaging
Anorectal malformations are rarely diagnosed prenatally
Genetic testing showed only a few variations of unclear significance
The team was not confident they could repair the cardiac defect, especially for a baby who couldn't come off ECMO due to limited lung volume