Supratentorial Intracranial Anomalies in Patients with Myelomeningocele.

Larisa Andrada Ay, Ibrahim Alataş, Bahattin Özkül, Revna Çetiner, Orkhan Alizada, Doğa Uğurlar
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Abstract

Aim: The aim of this study is to assess associated cerebral supratentorial anomalies in patients who underwent myelomeningocele repair in hopes of developing a better morphological apprehension of the forebrain's anomalies in this category of patients.

Material and methods: This retrospective observational study assessed 426 pediatric patients who underwent myelomeningocele repair between January 2013 and December 2020. Cranial MRIs with T1- and T2-weighted sequences were obtained as part of the postoperative assessment to determine the presence of associated supratentorial anomalies in pediatric patients following myelomeningocele repair.

Results: The most common supratentorial anomalies identified in patients who underwent myelomeningocele repair are associated with the configuration of the corpus callosum (CC). Moreover, the complete agenesis of the CC was noticed in 9 (2.1%) subjects, whereas partial agenesis was identified in 148 (34.7%) subjects. Hypoplasia of the CC was observed in three (0.7%) patients. Gray matter heterotopia were the second most commonly observed supratentorial anomalies in 110 (25.8%) patients. Furthermore, the absence of the septum pellucidum was observed in two (0.47%) of the total patients. The widening of the interhemispheric fissure and abnormal maturation of the white matter were observed in 10 (2.34%) patients and 11 (2.58%) patients, respectively. Polymicrogyria, a consequence of abnormal cortical organization, was identified in 22.53% of the patients (96 patients) included in our series.

Conclusion: This study confirms that, except for hydrocephalus and Chiari malformation, other associated cerebral supratentorial anomalies may be observed in patients with myelomeningocele. However, only limited research has confirmed the interconnection between the cerebral supratentorial anomalies and cognitive function. Therefore, this study emphasizes the necessity for further supplementary studies, in conjunction with accurate postnatal followups, in order to assess the real significance and repercussions of these anomalies on neurological development and also to establish how these structural changes in brain anatomy translate clinically.

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脊髓脊膜膨出患者的幕上颅内异常。
目的:本研究的目的是评估接受脊髓脊膜膨出修复的患者的相关脑幕上异常,希望对这类患者的前脑异常有更好的形态学认识。材料和方法:这项回顾性观察性研究评估了2013年1月至2020年12月期间接受脊髓脊膜膨出修复术的426例儿童患者。获得T1和t2加权序列的颅mri作为术后评估的一部分,以确定脊髓脊膜疝修复后儿科患者是否存在相关的幕上异常。结果:在接受髓系脑膜膨出修复的患者中,最常见的幕上异常与胼胝体(CC)的结构有关。此外,9例(2.1%)患者的CC完全发育不全,148例(34.7%)患者的CC部分发育不全。3例(0.7%)患者CC发育不全。在110例(25.8%)患者中,灰质异位是第二常见的幕上异常。此外,2例(0.47%)患者出现透明隔缺失。10例(2.34%)和11例(2.58%)患者出现脑间裂增宽和白质异常成熟。在我们的研究中,有22.53%的患者(96名患者)出现了皮层组织异常导致的多小回症。结论:本研究证实,除了脑积水和Chiari畸形外,脊髓脊膜膨出患者还可观察到其他相关的脑幕上异常。然而,只有有限的研究证实了大脑幕上异常与认知功能之间的联系。因此,本研究强调了进一步补充研究的必要性,并结合准确的产后随访,以评估这些异常对神经发育的真正意义和影响,并确定这些脑解剖结构变化如何在临床上转化。
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