In Utero Fetoscopic Repair Program for Sacral Myelomeningoceles and Mye-LDM
试验速览
- 阶段
- 不适用
- 状态
- 进行中（未招募）
- 入组人数
- 10
- 试验地点
- 2
- 主要终点
- Ability to perform fetoscopic sacral MMC/MyeLDM repair without severe perinatal morbidity and mortality
研究概览
简要总结
The purpose of the study is to evaluate the feasibility and the maternal, fetal and postnatal outcomes of sacral myelomeningocele (MMC) and Myelic Limited Dorsal Myeloschisis (MyeLDM) fetoscopic repair at Trousseau Hospital (Paris, France).
详细描述
Myelomeningocele and Myelic limited dorsal myeloschisis (MyeLDM) correspond to neural tube defects which lead to lifelong disabilities including lower extremity paralysis, sphincters deficiency, and cerebral complications (Chiari 2 malformation and hydrocephalus). It is demonstrated that fetal surgery repair of MMC with upper limit between the first thoracic vertebra (T1) and the first sacral vertebra (S1) improves motor and cerebral prognosis. Nowadays, this fetal surgery is performed either after maternal laparotomy and hysterotomy (open fetal surgery) or using fetoscopy. the investigators, at Trousseau Hospital (APHP, Paris), initiated an open fetal surgery of MMC program called PRIUM1 and 16 fetuses has been currently operated.
Fetoscopic repair of MMC is proposed by several international groups in order to prevent from maternal and obstetric morbidity related to the hysterotomy and improve the mother's obstetrical prognosis by allowing vaginal delivery. Results of fetoscopic MMC repair are very satisfying, both in terms of repair surgery efficacy and in terms of obstetrical prognosis. The research team believe that it is justified to propose this minimally invasive repair technique using fetoscopy, for represented by sacral MMC (level S1 and lower) as well as for intermediate forms between open and closed dysgraphisms, represented by MyeLDM. Indeed, these dysraphism are associated with the same cerebral complications than MMC with upper limit between T1 and S1 which could be corrected with prenatal repair. In addition, the spinal cord protection offered by prenatal surgery could prevent from the neuroepithelium destruction observed during pregnancy, with a potential motor benefit for the children.
The main objective of PRIUM 2 is to evaluate the success of fetoscopic surgical repair of sacral MMC or MyeLDM with a birth after 32 weeks of gestation and without severe perinatal morbidity and mortality.
The secondary objectives of PRIUM 2 are to evaluate the complications of pregnancy related to fetoscopic surgery as well as to evaluate the prenatal and postnatal evolution of the cerebral complications (Chiari 2 malformation and hydrocephalus) after fetoscopic repair surgery of the dysraphism (up to 12 months of age).
In this protocol, fetal sacral MMC/ MyeLDM repair surgery will be performed using gas fetoscopy before 26 weeks. After an exteriorization of the uterus through a laparotomy, humidified and warmed gas will be insufflated with low pressure (6 to 8mmHg mmHg). Fetoscopic repair surgery will be performed by a multidisciplinary team (maternal fetal medicine specialists, pediatric neurosurgeons, pediatric surgeons).
研究设计
- 研究类型
- Interventional
- 分配方式
- Na
- 干预模型
- Single Group
- 主要目的
- Treatment
- 盲法
- None
入排标准
- 年龄范围
- 18 Years 至 —（Adult, Older Adult）
- 性别
- Female
- 接受健康志愿者
- 否
入选标准
- •Inclusion Criteria :
- •Pregnant women age 18 years and older who are able to consent
- •Singleton pregnancy before 26 weeks of gestation,
- •Sacral MMC (upper level S1 or below) or MyeLDM diagnosed on ultrasound and MRI,
- •Absence of associated malformation apart from the anomalies usually observed in cases of open dysraphisms (i.e. feet malpositions, associated cerebral signs) or chromosomal anomaly if verification of the karyotype was desired by the couple
- •Affiliated to health insurance, understanding and speaking French
- •Written consent of the patient for the surgery and representatives of the parental authority for the postnatal follow-up of the child
- •Patient who made the choice to continue the pregnancy
排除标准
- •Abnormal angulation of the fetal spine,
- •risk factors for prematurity: cervical length less < 15mm, history of late miscarriage before 22 weeks, pre-existing rupture of the membranes at inclusion,
- •Placenta praevia,
- •BMI greater than 35 kg / m2,
- •Abnormality of the uterus: large fibroid, uterine malformation, history of uterine body surgery
- •Maternal infection at risk of maternal-fetal transmission: HIV, HBV, HCV,
- •Surgical or anesthetic contraindication.
- •Participation in another interventional research protocol,
- •Patients under legal protection (guardianship, curatorship).
- •Allergies to drugs used in the research
结局指标
主要结局
Ability to perform fetoscopic sacral MMC/MyeLDM repair without severe perinatal morbidity and mortality
时间窗: From time of surgery to 8 weeks of life (up to 28 weeks)
Successful complete closure of the defect using the fetoscopic technique AND birth after 32 weeks without severe perinatal morbidity and mortality including grade III-IV intra ventricular hemorrhage, severe, cerebral parenchyma hemorrhage, periventricular leukomalacia, grade III ulcero-necrotizing enterocolitis, severe bronchodysplasia)
次要结局
- Maternal obstetric outcome as evidenced by antenatal betamethasone treatment(From time of surgery until 34 weeks of gestation)
- Proportion of postpartum hemorrhages(From time of surgery until delivery)
- Need for maternal transfusion (number of blood cells transfused)(From time of surgery until delivery)
- Adverse early childhood outcome as evidenced by need for a neurosurgical intervention(From the time of birth until 8 weeks of life)
- Maternal obstetric outcome as evidenced by gestational age at delivery (1)(From time of surgery until delivery)
- Adverse neonatal outcome at birth (1)(at birth)
- Maternal obstetric outcome as evidenced by preterm labor leading to delivery at less than 37 weeks of gestation(From time of surgery until 37 weeks of gestation)
- Maternal obstetric outcome as evidenced by chorioamnionitis(From time of surgery until 37 weeks of gestation)
- Maternal obstetric outcome as evidenced by the ability to deliver vaginally(From time of surgery until delivery)
- Postnatal evolution of brain abnormalities associated with open dysraphism (3)(From the time of birth until 12 months of life)
- Other Maternal obstetric outcome as evidenced by hypertensive disorders, preeclampsia, gestational diabetes(From time of surgery until 37 weeks of gestation)
- Adverse neonatal outcome at birth (2)(at birth)
- Postnatal evolution of brain abnormalities associated with open dysraphism(From the time of birth until 12 months of life)
- Adverse Maternal outcome(during the surgery)
- Maternal obstetric outcome as evidenced by preterm premature rupture of membranes(From time of surgery until 37 weeks of gestation)
- Prenatal evolution of brain abnormalities associated with open dysraphism, after fetoscopic repair(From time of surgery until birth)
- Prenatal evolution of brain abnormalities associated with open dysraphism, after fetoscopic repair (1)(From time of surgery until birth)
- Prenatal evolution of brain abnormalities associated with open dysraphism, after fetoscopic repair (2)(From time of surgery until birth)
- Prenatal evolution of brain abnormalities associated with open dysraphism, after fetoscopic repair (3)(From time of surgery until birth)
- Maternal obstetric outcome as evidenced by gestational age at delivery (2)(From time of surgery until delivery)
- Postnatal evolution of brain abnormalities associated with open dysraphism (1)(From the time of birth until 12 months of life)
- Postnatal evolution of brain abnormalities associated with open dysraphism (2)(From the time of birth until 12 months of life)