Fetoscopic Endoluminal Tracheal Occlusion (FETO)

Despite advances in prenatal diagnosis and postnatal therapies, including extracorporeal
membrane oxygenation (ECMO), inhaled nitric oxide therapy, and ventilator strategies that
minimize ventilator-induced lung injury, morbidity and mortality rates for babies with severe
CDH remain high. Data from Children's Hospital of Philadelphia (CHOP) between January 2006
and December 2010 for prenatal and postnatal care for 64 patients with isolated left CDH
showed overall survival was 63%. Survival was 33% in patients requiring the use ECMO.
Immediate morbidity/mortality is related to the severity of the pulmonary hypoplasia caused
by the mass effect of the herniated abdominal contents on the developing lungs. Quantifying
the severity of pulmonary hypoplasia has been performed using the observed/expected lung to
head circumference ratio (O/E LHR). It is a tool validated in 354 fetuses with unilateral
isolated CDH evaluated between 18 and 38 weeks gestation. For O/E LHR < 25%, survival was
dismal at 1/9 or 11% (CHOP experience, not published). Published data from an
interdisciplinary follow-up program at CHOP shows striking morbidities in neuromuscular tone
and neurodevelopmental status.

The rationale for fetal therapy in severe CDH is to improve fetal lung growth and therefore
neonatal survival. Prenatal tracheal occlusion obstructs the normal egress of lung fluid
during pulmonary development leading to increased lung tissue stretch, increased cell
proliferation, and accelerated lung growth. European colleagues have developed foregut
endoscopy and techniques to position and remove endoluminal tracheal balloons in utero.
Recently, the Belgium group published summary results of FETO showing an improved survival in
175 patients with isolated left CDH from 24% to 49%.

The investigators goal with this pilot study is to study the feasibility of implementing FETO
therapy in the most severe group of fetuses with left CDH (O/E < 30%) and right CDH (O/E <
45%).

Inclusion Criteria:

- Pregnant women age 18 years and older, who are able to consent

- Singleton pregnancy

Fetal

- Normal Karyotype

- Fetal Diagnosis of Isolated Left or Right CDH with liver up

- Gestation at enrollment prior to 29 wks plus 6 days

- SEVERE pulmonary hypoplasia with Ultra Sound L-sided O/E LHR < 25% or R-sided O/E LHR
<45%

Exclusion Criteria:

- Pregnant women <18 years of age.

- Maternal contraindication to fetoscopic surgery or severe maternal medical condition
in pregnancy

- Technical limitations precluding fetoscopic surgery

- Rubber latex allergy

- Preterm labor, cervix shortened (<15 mm at enrollment or within 24 hours of FETO
balloon insertion procedure) or uterine anomaly strongly predisposing to preterm
labor, placenta previa

- Psychosocial ineligibility, precluding consent

- Inability to remain at FETO site during time period of tracheal occlusion, delivery
and postnatal care