Anterior Basal Encephalocele of the Neonatal and Infantile Period
Anterior Basal Encephalocele of the Neonatal and Infantile Period
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新生儿和婴儿期前基底脑膨出
DOI：
10.1227/00006123-198609000-00025
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发表时间：
1986
期刊：
影响因子：
4.8
通讯作者：
S. Nagahiro
中科院分区：
文献类型：
--
作者：
A. Yokota;Y. Matsukado;I. Fuwa;Kooichi Moroki;S. Nagahiro
&NA;如果考虑到这种异常的特殊临床和放射学体征，隐匿性基底脑膨出的诊断并不困难。最近的经蝶脑膨出手术的效果比想象的要好。然而，婴儿早期经蝶脑膨出仍可能面临较高的手术风险，因为该年龄段的脑膨出通常合并垂体-下丘脑结构。作者根据经颅修复失败导致术后早期因下丘脑功能障碍死亡的情况，讨论了婴儿期经蝶脑膨出的手术指征和手术方法。回顾前基底脑膨出的病例报告，发现经蝶脑膨出（特别是在儿科年龄组）与面部、眼睛和大脑的相关畸形之间存在高度相关性。这种特征性的畸形复合体可以通过在胚胎期大约前神经孔闭合阶段运行并发生在神经管头端腹侧表面的常见发病机制来解释。还报告了 3 例新生儿期诊断的经蝶脑膨出并伴有鼻咽气道进行性阻塞的病例。两例患者存在特征性畸形，包括正中裂面综合征、视神经发育不良和胼胝体发育不全；另一名患者患有极为罕见的鼻中隔视神经发育不良。两名患者分别在术前和术后死亡；另一名患者因多重异常严重而未接受手术。 (神经外科 19:468‐478, 1986)
&NA; Diagnosis of occult basal encephalocele is not difficult if the peculiar clinical and radiological signs of this anomaly are borne in mind. Recent surgery for transsphenoidal encephalocele has had better results than realized. However, high surgical risks may still be encountered in transsphenoidal encephalocele of the early infantile period, because the pituitary‐hypothalamic structures are usually incorporated in the herniated encephalocele of this age group. Surgical indications for and operative approaches to transsphenoidal encephalocele in the infantile period are discussed on the basis of the authors' failure in transcranial repair, which resulted in early postoperative death due to hypothalamic dysfunction. Reviewing the reported cases of anterior basal encephalocele, a high correlation between transsphenoidal encephalocele, particularly in the pediatric age group, and allied malformations of the face, eye, and brain was disclosed. This characteristic malformation complex may be explained by a common pathogenetic mechanism operating in the embryonal period at about the stage of the anterior neuropore closure and occurring in the ventral surface of the cephalic end of the neural tube. Three cases of transsphenoidal encephalocele diagnosed in the neonatal period with progressive obstruction in the nasopharyngeal airway are also reported. A characteristic malformation complex consisting of median cleft face syndrome, optic nerve dysplasia, and agenesis of the corpus callosum was associated in two cases; the other patient had an extremely rare combination of septooptic dysplasia. Two patients died pre‐ and postoperatively, respectively; the other patient did not undergo operation because of grave multiple anomalies. (Neurosurgery 19:468‐478, 1986)