Fetal Endotracheal Occlusion (FETO) in Severe Congenital Diaphragmatic Hernia
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 prospective observational study will determine the survival rate and 90 day outcomes of 20 babies diagnosed prenatally with severe CDH and who undergo fetal endotracheal occlusion (FETO). It is anticipated that fetal tracheal occlusion plug-unplug procedure will improve mortality and morbidity outcomes as compared with current management.
The study will prospectively enroll twenty women carrying fetuses with left-sided congenital diaphragmatic hernia and a normal karyotype to undergo evaluation. The babies will be 24 to 28 weeks of gestational age. They will have ultrasound evaluation to rule out other anomalies, calculation of the lung-to-head ratio, echocardiography, and detailed obstetric/perinatal consultation. The eligibility criteria will be the presence of left-sided congenital diaphragmatic hernia, a normal fetal echocardiogram, no ultrasonographic evidence of other anomalies, a normal karyotype, liver herniation into the left hemithorax, a lung-to-head ratio below 1.0 or an O/E LHR of < 25% as measured between 22 and 28 weeks of gestation, a fetal age between 24 and 28 weeks of gestation, a singleton pregnancy, no preterm labor, and no medical contraindication to general anesthesia. Patients who meet the eligibility criteria will be extensively counseled, and those who wish to participate will provide written, informed consent for the study.
The technique of fetal endoscopic tracheal occlusion has been described. (2,30) Betamethasone will be given to the mother preoperatively to improve fetal lung compliance.(36) The mother will preferably receive spinal anesthesia for analgesia but will be given the option of local anesthesia and sedation if she prefers. The fetus will receive an intramuscular injection of narcotic and muscle relaxant into its buttock or thigh given using a 22G needle that is passed through the maternal abdominal and uterine walls in order to provide muscle relaxation, sedation and analgesia. Depending on the amniotic fluid volume in the uterus an amnioinfusion may or may not be employed to optimize access to the fetal nose and mouth. Using standard technique a 9F Terumo vascular sheath will be placed in the amniotic cavity and a 1.2mm Storz fetoscope or flexible operating ureteroscope will be passed through the vascular sheath into the amniotic fluid. 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 silicone balloon (Goldvalve, NfocusNeuro, Palo Alto, CA) 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 (diameter, 0.5 mm) for a length of at least 2 cm. Studies have shown that this maneuver effectively occludes the fetal trachea.(35,37)
Antenatal steroids will be administered prior to surgery in all patients. Patients will be treated with a preoperative indomethacin suppository (50 mg), spinal anesthesia (or local anesthesia and sedation), intraoperative sedation as needed, and postoperative tocolysis with nifedipine and indomethacin (for a maximum of two days). The mothers will be discharged to the nearby Ronald McDonald House, where they will rest in bed and undergo ultrasonography twice weekly with nonstress testing until removal of the balloon or delivery. The patients will commit to remaining in Denver until removal of the balloon. After that, they will have the choice of delivering at PSL and having the CDH repair done at PSL by Dr.Rothenberg and his team, 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.
The FETO balloon will be left in place for 6 weeks and then removed ("Balloon Retrieval"). Since we believe that we will be the only US site offering this service (placement and retrieval of the FETO balloon) the patient will need to commit to remaining in Denver (or within a reasonable distance) and to being accessible to PSL until the balloon is retrieved. If there are any sites other than the PSL site in Denver that are offering this procedure, and the patient lives closer to one of those sites, and they agree to remove the balloon, arrangements may be made to have the balloon retrieval at another site. Anesthesia, sedation and tocolysis for the balloon retrieval will be as described above for the balloon placement. A 9F vascular sheath and fetoscope will be placed in the uterus and the fetoscope will be maneuvered into the fetal larynx using the same fetoscopic techniques previously described. The balloon will be visualized and removed after being punctured. The removal technique is to flush the deflated balloon out of the larynx into the amniotic fluid with irrigation fluid (saline). The fetoscope will then be removed in the routine fashion. The patient will then recover in the usual way and be discharged once she is stable. If necessary she will be given tocolytics drugs until 34 weeks.
Delivery will be scheduled at term and the baby will be handed off to the pediatric surgery team for further management after delivery. Delivery will be elective because of the need to have pediatric subspecialists at the delivery and this will be managed according to obstetric principles. If spontaneous labor does not occur, labor will be induced. Delivery will be vaginal unless cesarean section is indicated.
In the event of progressive and unremitting preterm labor in a patient who has a FETO balloon in-situ, the balloon will be removed emergently using the same procedure described above, time permitting. If this is not possible the FETO balloon will be punctured with the fetus still in-utero, using a 22G needle placed transabdominally under ultrasound guidance by one of the MFM team. If this is not possible the baby will be delivered by cesarean section using the EXIT (ex utero intrapartum therapy) procedure (39) In this procedure, only the fetal head will be delivered initially through a cesarean section incision while the mother is under general anesthesia. Once the head is delivered a neonatologist or pediatric surgeon will perform bronchoscopy and the balloon occluding the trachea will be deflated and removed. The airway will be suctioned, an endotracheal tube inserted, and exogenous surfactant (Exosurf Neonatal, Glaxo Wellcome) will be administered at a dose of 3 ml per kilogram of body weight, and assisted ventilation will be started before the umbilical cord is divided. Once the baby is being ventilated the body of the child will be delivered and the umbilical cord will be cut. This procedure allows the baby to be oxygenated while the balloon is removed and the endotracheal tube is placed. In the event that the baby delivers precipitously without the EXIT procedure being possible, a pediatric surgeon will be in the operating room to puncture the balloon and remove it as soon as possible after thee delivery. Patients will be counseled as to the importance of returning for care if there is any possibility of progressive preterm labor and delivery so that the necessary preparations can be made.
The survival, adverse event, and complication outcome data will be compared with historical control groups from the literature as well as with data from the Eurofetus consortium using Chi Square 2 x 2 tables and parametric and non-parametric statistics as necessary. P < 0.05 will be used to set significance.
Endpoint Classification: Safety/Efficacy Study, Intervention Model: Single Group Assignment, Masking: Open Label, Primary Purpose: Treatment
Congenital Diaphragmatic Hernia
Goldvalve Detachable Balloon
Presbyterian St. Luke's Hospital
Not yet recruiting
Utah HealthCare Institute
Results (where available)
- Source: http://clinicaltrials.gov/show/NCT00881660
- Information obtained from ClinicalTrials.gov on July 15, 2010
Medical and Biotech [MESH] Definitions
The type of DIAPHRAGMATIC HERNIA caused by TRAUMA or injury, usually to the ABDOMEN.
Protrusion of abdominal structures into the THORAX as a result of congenital or traumatic defects in the respiratory DIAPHRAGM.
STOMACH herniation located at or near the diaphragmatic opening for the ESOPHAGUS, esophageal hiatus. When the ESOPHAGOGASTRIC JUNCTION is above the DIAPHRAGM, it is called a SLIDING HIATAL HERNIA. When the ESOPHAGOGASTRIC JUNCTION is below the DIAPHRAGM, it is called a PARAESOPHAGEAL HIATAL HERNIA.
A HERNIA due to an imperfect closure or weakness of the umbilical ring. It appears as a skin-covered protrusion at the UMBILICUS during crying, coughing, or straining. The hernia generally consists of OMENTUM or SMALL INTESTINE. The vast majority of umbilical hernias are congenital but can be acquired due to severe abdominal distention.
A congenital abnormality characterized by the elevation of the DIAPHRAGM dome. It is the result of a thinned diaphragmatic muscle and injured PHRENIC NERVE, allowing the intra-abdominal viscera to push the diaphragm upward against the LUNG.
The purpose of this phase 2 limited study is to examine whether prenatal intervention correct the lung underdevelopment associated with severe diaphragmatic hernia.
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