Division of Pediatric Surgery - Department of Surgery and Anatomy, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, São Paulo, Brazil.
Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology, University of Texas, Houston, TX, USA.
Childs Nerv Syst. 2021 Sep;37(9):2769-2785. doi: 10.1007/s00381-021-05299-1. Epub 2021 Jul 31.
Myelomeningocele (MMC), the commonest type of spina bifida (SB), occurs due to abnormal development of the neural tube and manifest as failure of the complete fusion of posterior arches of the spinal column, leading to dysplastic growth of the spinal cord and meninges. It is associated with several degrees of motor and sensory deficits below the level of the lesion, as well as skeletal deformities, bladder and bowel incontinence, and sexual dysfunction. These children might develop varying degrees of neuropsychomotor delay, partly due to the severity of the injuries that affect the nervous system before birth, partly due to the related cerebral malformations (notably hydrocephalus-which may also lead to an increase in intracranial pressure-and Chiari II deformity). Traditionally, MMC was repaired surgically just after birth; however, intrauterine correction of MMC has been shown to have several potential benefits, including better sensorimotor outcomes (since exposure to amniotic fluid and its consequent deleterious effects is shortened) and reduced rates of hydrocephalus, among others. Fetal surgery for myelomeningocele, nevertheless, would not have been made possible without the development of experimental models of this pathological condition. Hence, the aim of the current article is to provide an overview of the animal models of MMC that were used over the years and describe how this knowledge has been translated into the fetal treatment of MMC in humans.
脊髓脊膜膨出(MMC)是最常见的神经管缺陷(SB)类型,由于神经管发育异常而导致,表现为脊柱后弓完全融合失败,导致脊髓和脑膜发育不良。它与损伤水平以下的多种程度的运动和感觉功能缺陷、骨骼畸形、膀胱和肠道失禁以及性功能障碍有关。这些儿童可能会出现不同程度的神经运动发育迟缓,部分原因是出生前神经系统损伤的严重程度,部分原因是相关的脑畸形(尤其是脑积水,可能导致颅内压升高和 Chiari II 畸形)。传统上,MMC 是在出生后立即进行手术修复的;然而,已经证明,MMC 的宫内矫正具有多种潜在益处,包括更好的感觉运动结果(因为暴露于羊水及其随后的有害影响缩短)和降低脑积水的发生率等。然而,如果没有对这种病理状况的实验模型的发展,胎儿脊髓脊膜膨出的手术就不可能实现。因此,本文的目的是概述多年来用于研究 MMC 的动物模型,并描述这些知识如何转化为人类胎儿 MMC 的治疗。