Chiari Malformation Type 2, Congenital Abnormality, Fetal Disease, Hydrocephalus, Myelomeningocele, Neural Tube Defects, Spina Bifida, Open, Surgery; Maternal, Uterus or Pelvic Organs, Affecting Fetus
Conditions
Brief summary
This study aims to assess the feasibility to perform a fetoscopic prenatal repair of an open neural tube defect, and secondly to assess the maternal, fetal and neonatal outcomes.
Detailed description
Introduction: Spina bifida is a congenital malformation characterized by a failure in neural tube closure. Unprotected fetal neural tissue at the spine level undergoes not only progressive neurodegeneration in spinal cord, but also the impairment in brain development, hindbrain herniation or Chiari II malformation probably due to pressure disturbances resulting from a continuous leakage of cerebrospinal fluid through the defect. The neurological consequences at birth are irreversible and sometimes devastating, including paraplegia, sphincter urinary and fecal incontinence, hydrocephalus, cranial nerve disturbances, respiratory problems and death due to spinal cord injury and brain maldevelopment. Intrauterine fetal open repair of the defect has proved to improve hindbrain herniation and decrease the need of cerebrospinal shunting. Hypothesis: The investigators hypothesize that minimally invasive surgery of spina bifida is feasible and may minimize surgical aggression and obstetrical complications, with similar neonatal and neurological results. Intervention: In this study the investigators propose a fetoscopic skin closure technique. After a maternal laparotomy the uterus is exteriorized, the amniotic cavity is accessed by fetoscopic approach. The placode is dissected from the surrounding tissue and dropped into the opened spinal canal, untethered. Part of the cystic tissue is resected. The edges are then closed to the midline. Follow up: After surgery patients are followed up at the Fetal Medicine Unit every one or two weeks. The mode of delivery is decided according to obstetric criteria. Neonates will be examined at birth, and followed up regularly at least until 30 months. Patients are lifetime followed up by our Multidisciplinary myelomeningocele (MMC) Committee that comprises professionals from the Spina Bifida Unit, Neonatologists, Pediatric Surgeons, Pediatric Urologist, Pediatric Orthopedic Surgeons, Radiologists, Rehabilitation, Pathologists, Pediatric Neurosurgeons, Obstetricians and Fetal Therapists.
Interventions
PROCEDUREFetoscopic repair of spina bifida
Fetoscopic intrauterine repair of open spina bifida
Sponsors
Hospital Universitari Vall d'Hebron Research Institute
Study design
Allocation
NA
Intervention model
SINGLE_GROUP
Primary purpose
TREATMENT
Masking
NONE
Eligibility
Sex/Gender
FEMALE
Age
18 Years to 50 Years
Healthy volunteers
No
Inclusion criteria
* Maternal age \> 18 years old * Singleton pregnancy * Gestational age between 18 0/7 weeks and 26 6/7 weeks * Isolated neural tube defect between T1 and S1 * Cerebellar herniation (Chiari type II)
Exclusion criteria
* Fetal anomalies non related to neural tube defect (NTD) including chromosomal abnormalities * Body mass index \> 35 kg/m2 * Maternal disorders contraindicating surgery * Genetic anomalies with poor prognosis * Sever kyphosis (\>30º)
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Capability to achieve successful closure of the neural tube defect by fetoscopic surgery (yes/no) | Time of procedure (day 0) | Binary variable (yes/no) describing if the neural tube defect has been successfully closed, (placode dissected and dropped into the open spinal canal, cystic tissue resected and edges closed to the midline), by fetoscopic surgery, and without conversion to open surgery |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Operating time (minutes) | Time of procedure (day 0) | Time between the skin opening and skin closure |
| Fetoscopy time (minutes) | Time of procedure (day 0) | Time between the introduction of the first cannula and the removal of the last cannula |
| Hospital stay (days) | Between procedure and delivery, up to 21 weeks | Time between hospital admission and discharge |
Other
| Measure | Time frame | Description |
|---|---|---|
| Premature rupture of membranes (PROM) (yes/no) | Between the procedure and 37 weeks of pregnancy (18 weeks after procedure) | Binary variable describing amniotic fluid leakage before the onset of labor |
| Chorioamnionitis (yes/no) | Between the procedure and birth, up to 21 weeks after procedure | Binary variable describing the presence of a chorioamnionitis between the procedure and birth |
| Oligohydramnios without PROM (yes/no) | Between the procedure and birth, up to 21 weeks after procedure | Binary variable describing the presence of a oligohydramnios (deepest pool \< 2 cm) without evidence of vaginal amniotic fluid leakage |
| Placental abruption (yes/no) | Between the procedure and birth, up to 21 weeks after procedure | Binary variable describing the presence of a placental abruption |
| Mode of delivery (vaginal / cesarean) | At birth, up to 21 weeks after procedure | Binary variable describing the mode of delivery, vaginal or cesarean section |
| Post-operative hemoglobin (g/dL) | 24 hours after procedure | Hemoglobin level after the procedure |
| Acute Pulmonary edema (yes/no) | Between the procedure and birth, up to 21 weeks after procedure | Binary variable describing the presence of a maternal pulmonary edema, regardless of the severity, between the procedure and discharge. |
| Closure of the spinal defect (yes/no) | Between birth and 1 month of life | Binary variable describing the watertight closure of the defect |
| Maintenance of the neurological level (Better / same / worse) | 1 month of life | Categorical variable describing if the postnatally assessed neurological level is better / same or worse than the level assessed prenatally before the procedure. It will be assessed by physical examination |
| Reversal of Chiari malformation(completely reversed / mild improvement / no improvement) by MRI | 1 month of life | Categorical variable comparing the postnatal and prenatal MRI assessment of the Chiari malformation |
| Ventriculoperitoneal shunting (yes/no) | Between birth and 12 months | Binary variable describing the need of a ventriculoperitoneal shunting |
| Uterine dehiscence (yes/no) | At birth, up to 21 weeks after procedure | Binary variable describing the presence of a uterine dehiscence, regardless of whether it is symptomatic or it is diagnosed at the time of cesarean section |
| Blood transfusion (yes/no) | Between procedure and discharge, up to 21 weeks | Binary variable describing if a transfusion of blood derivates is carried out between the time of procedure and discharge |
| Gestational age at delivery (weeks and days as x/7) | At birth, up to 21 weeks after procedure | Gestational age at birth |
| Surgery to delivery interval (days) | At birth, up to 21 weeks after procedure | Interval between surgery and birth |
Countries
Spain
Contacts
Primary ContactElena Carreras, PhD
Backup ContactNerea Maiz, PhD
Outcome results
None listed