Fetal Endotracheal Occlusion (FETO) in Severe and Extremely Severe Congenital Diaphragmatic Hernia
Condition: Congenital Diaphragmatic Hernia
MeSH terms: Hernias, Diaphragmatic, Congenital; Hernia; Hernia, Diaphragmatic
Study type: Interventional

Summary: 
      Congenital diaphragmatic hernia (CDH) occurs when the diaphragm fails to fully fuse and
      leaves a portal through which abdominal structures can migrate into the thorax. In the more
      severe cases, the abdominal structures remain in the thoracic cavity and compromise the
      development of the lungs. Infants born with this defect have a decreased capacity for gas
      exchange; mortality rates after birth have been reported between 40-60%. Now that CDH can be
      accurately diagnosed by mid-gestation, a number of strategies have been developed to repair
      the hernia and promote lung tissue development.

      Fetal tracheal occlusion is one technique that temporarily closes the herniated area with the
      Goldvalve balloon to allow the lungs to develop and increase survival at birth. This is a
      pilot study of a cohort of fetuses affected by severe CDH that will undergo FETO to
      demonstrate the feasibility of performing the procedure, managing the pregnancy during the
      period of tracheal occlusion, and removal of the device prior to delivery at BCM/Texas
      Children's Hospital (TCH). It is anticipated that fetal tracheal occlusion plug-unplug
      procedure will improve mortality and morbidity outcomes as compared with current management,
      but this is not a primary endpoint of the feasibility study. We will perform 20 FETO
      procedures on fetuses diagnosed prenatally with severe and extremely severe CDH.
    

Detailed description: 
      Enrollment

      Women carrying fetuses with severe or extremely severe CDH and a normal karyotype will
      undergo routine clinical evaluation. The fetuses will be 27+0/7 to 29+6/7 weeks of
      gestational age for severe CDH and can be as early as 22+0/7 weeks gestational age for those
      deemed as "extremely severe" cases of CDH. They will have ultrasound and/or MRI evaluation to
      rule out other anomalies, calculation of the LHR from ultrasound measurements,
      echocardiography, and detailed obstetric/perinatal consultation. Patients who meet the
      eligibility criteria will be extensively counseled, and those who wish to participate will
      provide written, informed consent for the study.

      Procedure

      The procedure will be performed under spinal anesthesia or local anesthesia with intravenous
      sedation. The technique of fetal endoscopic tracheal occlusion has been described. Using
      standard technique, a cannula loaded with a pyramidal trocar will be inserted into the
      amniotic cavity and a fetoscope or flexible operating endoscope will be passed through the
      cannula into the amniotic fluid. If, upon evaluation, the baby cannot be accessed through the
      way just described above, the uterus will be accessed through an incision in the belly
      (called a laparotomy). A laparotomy is a surgical technique that makes an incision in the
      abdomen. After the incision has been made, the uterus will be temporarily repositioned
      externally. The baby will then be accessed using the fetoscope and ultrasound, as described
      above. The laparotomy will only be done if the baby cannot be reached and repositioned to a
      more favorable one by doing external maneuvers (called external version) for the FETO
      procedure.

      The scope will be guided into the fetal larynx either through a nostril and then via the
      nasal passage or through the fetal mouth, and then through the fetal vocal cords with the aid
      of both direct vision through the scope and cross-sectional ultrasonographic visualization. A
      detachable latex balloon will be placed in the fetal trachea midway between the carina and
      the vocal cords. The balloon will be inflated with isosmotic contrast material so that it
      fills the fetal trachea.

      Postoperative

      The mothers will be discharged once stable. Serial measurements of sonographic lung volume
      and LHR will begin within 24-48 hours following surgery and continue weekly by targeted
      ultrasound evaluation. Amniotic fluid level and membrane status will also be monitored at
      weekly intervals. Comprehensive ultrasonography for fetal growth will be performed every four
      weeks (+/- 1 wk). All discharged patients will stay within 30 minutes of TCH to permit
      standardized postoperative management and emergent retrieval of the balloon in the event of
      preterm labor or premature rupture of membranes prior to the scheduled removal.

      After the FETO surgery, prior to leaving the hospital, the mother will be given a medical
      alert bracelet identifying her as a patient with a baby with blocked airways. She will be
      encouraged to wear the bracelet at all times so that in case of emergency, she and others
      will know who to contact. She will also be given a pamphlet with instructions for medical
      personnel describing how to remove the balloon in case of an emergency. She should carry it
      with her at all times.

      Balloon retrieval will be planned at between 32+0/7 and 34+6/7 weeks or no longer than 10 wks
      after placement, at the discretion of the FETO center. The patient will need to commit to
      remaining in 30 minutes of Texas Children's Hospital Pavilion for Women until the balloon is
      retrieved. In the event of a patient relocating after having the balloon placed, despite
      having committed to remain in the area during consent process, she will be asked to return
      for the removal. Every effort to make arrangements for her to be managed by the nearest
      center capable of an EXIT procedure or balloon retrieval (San Francisco or Philadelphia) will
      be made.

      After removal of the balloon, patients will have the choice of delivering at Texas Children's
      Hospital- Women's Pavilion with the CDH managed and repaired at TCH, or returning to their
      obstetrician for delivery with subsequent repair of the CDH by the pediatric surgeons at
      their referring facility. Given the severity of the CDH, the baby will need to be delivered
      in a facility that has the capability of immediate pediatric surgery services.

      We will need to monitor the baby at regular intervals (at 6 weeks, 3 months, 6 months, 1
      year, and 2 years) after delivery to see how well the baby is breathing and how well the baby
      is developing. These check- ups may be at Texas Children's Hospital- Women's Pavilion or can
      be coordinated with other doctors of the participant's choosing.

      If the child continues care at another institution, we will attempt to follow up with a
      review of the child's medical records.
    

Intervention name(s): Goldballoon Detachable Balloon and delivery microcatheter
Intervention type(s): Device
Intervention description(s): Between 27+0/7 - 29+ 6/7 weeks gestation for severe CDH and can be as early as 22+0/7 weeks gestational age for those deemed as "extremely severe" cases of CDH, placement of the Goldvalve detachable balloon. Balloon retrieval will be planned for between 32+0/7 and 34+6/7 weeks or no longer than 10 weeks after balloon placement at the discretion of the FETO center.
Other intervention name(s): GOLDBAL4 or GOLDBAL2 Goldballoon; BALTACCIBDPE100 delivery microcatheter
Intervention model: Single Group Assignment
Masking: None (Open Label)
Primary purpose: Device Feasibility
Keywords: Fetal Tracheal Occlusion; congenital diaphragmatic hernia; FETO plug-unplug; Goldvalve balloon; Severe Congenital Diaphragmatic Hernia; Extremely Severe Congenital Diaphragmatic Hernia; Goldbal balloon; FETO