Heuer Gregory G, Moldenhauer Julie S, Scott Adzick N
Division of Neurosurgery, Children's Hospital of Philadelphia, Wood Center, 6th Floor, 34th and Civic Center Blvd, Philadelphia, PA, 19104, USA.
The Center for Fetal Diagnosis and Treatment, Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Childs Nerv Syst. 2017 Jul;33(7):1149-1155. doi: 10.1007/s00381-017-3440-z. Epub 2017 May 17.
Open spina bifida or myelomeningocele (MMC) is one of the most common serious congenital malformations. Historically, this condition has been treated with closure of the MMC defect shortly after birth. The goal of postnatal closure is to cover the exposed spinal cord and prevent infection. However, postnatal surgery does not reverse or prevent the neurologic injury seen in MMC, reverse hindbrain herniation, or prevent hydrocephalus. The neurologic defects result from primary incomplete neurulation and secondary chronic prenatal damage to the exposed neural elements through mechanical and chemical trauma. In a hope to reduce the numerous surgical procedures, medical complications, and lifelong disabilities associated with MMC, the proposal of prenatal closure was put forth more than two decades ago. After promising results in animal models and some clinical series, a randomized controlled trial, the Management of Myelomeningocele Study (MOMS), was conducted. The MOMS trial demonstrated that closure during the prenatal period could be performed relatively safely and can result in significant benefit to the child. Specifically, prenatal closure results in improved motor function, reduced hindbrain herniation, and reduced need for a cerebral spinal fluid diversion. Long-term outcomes of the patients in the MOMS trial continues in the MOMS 2 study as these children grow. Additionally, investigations are underway on modifications to the open fetal MMC closure techniques.
开放性脊柱裂或脊髓脊膜膨出(MMC)是最常见的严重先天性畸形之一。从历史上看,这种疾病在出生后不久就通过闭合MMC缺损来治疗。出生后闭合的目的是覆盖暴露的脊髓并预防感染。然而,出生后手术并不能逆转或预防MMC中出现的神经损伤、后脑疝或预防脑积水。神经缺陷是由原发性神经管形成不完全以及继发性慢性产前对暴露的神经元件通过机械和化学创伤造成的损害引起的。为了减少与MMC相关的众多外科手术、医疗并发症和终身残疾,二十多年前就提出了产前闭合的建议。在动物模型和一些临床系列研究取得有希望的结果后,进行了一项随机对照试验,即脊髓脊膜膨出管理研究(MOMS)。MOMS试验表明,产前闭合可以相对安全地进行,并且可以给儿童带来显著益处。具体而言,产前闭合可改善运动功能、减少后脑疝并减少脑脊液分流的需求。随着这些儿童的成长,MOMS试验患者的长期结局在MOMS 2研究中继续进行。此外,正在对开放性胎儿MMC闭合技术的改进进行研究。