Teasley Damian E, Adapa Arjun R, Fogel Hart, Tugend Margaret, Yevudza Wisdom E, Simpson Lynn, Duron Vincent, Feldstein Neil A
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;199:123985. doi: 10.1016/j.wneu.2025.123985.
Myelomeningocele is a neural tube defect associated with high rates of morbidity and mortality. This review traces the evolution of its management, beginning with the Sheffield protocol in the mid-20th century, which promoted early postnatal surgical intervention. Ethical, logistical, and surgical challenges at the time sparked debate among clinicians regarding which patients might truly benefit from surgery. While postnatal intervention did improve survival for some, morbidity and mortality rates remained significant, with many patients facing severe physical and cognitive disabilities. Advances in our understanding of myelomeningocele physiology, surgical techniques, and management of multiorgan complications have shaped modern treatment. Preclinical studies underscored the detrimental impact of open neural tube defects exposed to the amniotic environment, giving rationale to prenatal intervention. Several animal studies and select pilot cases of human patients with prenatal intervention led to the pivotal Management of Myelomeningocele Study (MOMS), which confirmed the advantages of in utero repair over postnatal surgery. While the MOMS trial marked a shift in the standard of care, its implementation remained limited by disparities in access and the strict eligibility criteria of the trial. Ongoing studies have aimed to validate the generalizability of MOMS findings, with hopes of enabling more widespread prenatal intervention.
脊髓脊膜膨出是一种与高发病率和死亡率相关的神经管缺陷。本综述追溯了其治疗方法的演变，始于20世纪中叶的谢菲尔德方案，该方案提倡出生后早期进行手术干预。当时的伦理、后勤和手术挑战引发了临床医生关于哪些患者可能真正从手术中受益的争论。虽然出生后干预确实提高了一些患者的生存率，但发病率和死亡率仍然很高，许多患者面临严重的身体和认知残疾。我们对脊髓脊膜膨出生理学、手术技术和多器官并发症管理的认识进展塑造了现代治疗方法。临床前研究强调了暴露于羊膜环境的开放性神经管缺陷的有害影响，为产前干预提供了理论依据。几项动物研究以及对接受产前干预的人类患者的部分试点病例导致了关键的脊髓脊膜膨出管理研究（MOMS），该研究证实了宫内修复相对于出生后手术的优势。虽然MOMS试验标志着护理标准的转变，但其实施仍然受到获得治疗机会的差异和试验严格的纳入标准的限制。正在进行的研究旨在验证MOMS研究结果的普遍性，希望能够实现更广泛的产前干预。
