Adapa Arjun R, Teasley Damian E, Fogel Hart, Feldstein Neil
Department of Neurosurgery, Columbia University Irving Medical Center, New York, New York, USA.
Department of Neurosurgery, Columbia University Irving Medical Center, New York, New York, USA.
World Neurosurg. 2025 Jul;199:123984. doi: 10.1016/j.wneu.2025.123984.
Myelomeningocele is a common neural tube defect caused by failure of the closure of the posterior neuropore during neurulation. This defect, described as a neural placode (flattened neural tissue), manifests as an open spinal lesion most frequently found in the thoracolumbar region. Throughout history, clinicians have worked to better understand this condition, leading to a stepwise increase in understanding of the disease. Complications associated with myelomeningocele include cognitive and functional neurological deficits, hydrocephalus, Chiari II malformation, bladder dysfunction, meningitis, and often death. Historically, management of myelomeningocele evolved from a largely palliative approach to more aggressive surgical interventions aimed at improving quality of life and reducing morbidity. Early surgical repair within 24-48 hours after delivery remains the standard of care, although in utero fetal surgery is becoming increasingly commonplace. This paper reviews the origins of the early management of myelomeningocele and the advances that have shaped our modern day understanding of this disease.
脊髓脊膜膨出是一种常见的神经管缺陷，由神经胚形成过程中后神经孔闭合失败引起。这种缺陷被描述为神经基板（扁平的神经组织），表现为开放性脊柱病变，最常见于胸腰段。纵观历史，临床医生一直致力于更好地了解这种疾病，从而对该疾病的认识逐步提高。与脊髓脊膜膨出相关的并发症包括认知和功能性神经缺陷、脑积水、Chiari II 畸形、膀胱功能障碍、脑膜炎，并且常常导致死亡。历史上，脊髓脊膜膨出的治疗方法从主要的姑息治疗方法演变为旨在改善生活质量和降低发病率的更积极的手术干预措施。尽管宫内胎儿手术越来越普遍，但产后 24 - 48 小时内进行早期手术修复仍然是标准治疗方法。本文回顾了脊髓脊膜膨出早期治疗的起源以及塑造我们现代对该疾病认识的进展。
