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[小儿颅内脂肪瘤:20例患者的回顾性研究]

[Intracranial lipomas in paediatrics: a retrospective study of 20 patients].

作者信息

Gómez-Gosálvez F A, Menor-Serrano F, Téllez de Meneses-Lorenzo M, Aleu Pérez-Gramunt M, Sala-Sánchez A G, Rubio-Soriano A, Carbonell-Nadal J, Mulas F

机构信息

Servicio de Pediatría, Hospital Virgen de los Lirios, Alcoy, Alicante, España.

出版信息

Rev Neurol. 2003;37(6):515-21.

Abstract

INTRODUCTION

Intracranial lipomas (ICL) are congenital malformations that are due to an anomalous differentiation of the primitive meninges.

AIMS

The purpose of this study is to determine the most frequent symptoms related to ICL in the paediatric age, as well as to evaluate whether they are directly related to their location and to their association with other brain malformations.

PATIENTS AND METHODS

A retrospective clinico-radiological study was conducted involving 20 paediatric patients diagnosed as suffering from ICL between 1985 and 2003 at three hospitals in the Valencian Community.

RESULTS

70% of the cases were females and the average age when ICL was diagnosed was 5.3 years (interval from 2 months to 13 years). The most common initial clinical justification for the neuroradiological study was psychomotor retardation in eight patients (40%) and persistent, predominantly migrainous, headaches in six of them (30%). Only one patient was examined because of epilepsy. The prevailing site was the interhemispheric fissure in 10 patients (50%), followed by the quadrigeminal cistern in five (25%) and the mamillary body infundibulum axis in three of them (15%). One case was located in the cerebellum and another was found in the lateral ventricle, with involvement of the choroid fissure. Associated anomalies were observed in eight patients (40%). Alterations were seen in the development of the corpus callosum in six patients (30%), five in an isolated way and in one case there was partial absence of the associated septum pellucidum. Another patient was found to have an isolated partial agenesis of the septum and Goldenhar syndrome was detected in another case. The radiological follow up, which varied according to the initial clinical features, did not show any differences in the morphology or the size of the lipoma.

CONCLUSIONS

  1. ICL are more frequently located in the interhemispheric fissure and in the quadrigeminal cistern; 2) In these locations they can be associated with other brain malformations, the most important being defects in the callosa and septa; 3) Most ICL were found incidentally during the course of a neuroimaging examination. Only in the case of three patients (15%) was there any presumed relation between the location of the ICL (pericallosal), the symptomatology (overall psychomotor retardation) and the associated malformation (hypoplasia of the corpus callosum); 4) The incidence of epilepsy was much lower than that claimed in earlier reports, and the predominant associated neurological symptomatology was found to be psychomotor retardation and headache (70%); 5) ICL is a developmental anomaly that does not require neurosurgical treatment and which does not usually change with the passage of time, although it is liable to increase because of adipose hypertrophy.
摘要

引言

颅内脂肪瘤(ICL)是先天性畸形,由原始脑膜的异常分化引起。

目的

本研究的目的是确定小儿年龄组中与ICL相关的最常见症状,以及评估这些症状是否与其位置以及与其他脑畸形的关联直接相关。

患者和方法

进行了一项回顾性临床放射学研究,涉及1985年至2003年期间在巴伦西亚自治区三家医院诊断为患有ICL的20例儿科患者。

结果

70%的病例为女性,诊断ICL时的平均年龄为5.3岁(范围从2个月至13岁)。神经放射学检查最常见的初始临床理由是8例患者(40%)存在精神运动发育迟缓,其中6例(30%)持续出现以偏头痛为主的头痛。仅1例患者因癫痫接受检查。最常见的部位是大脑镰旁10例患者(50%),其次是四叠体池5例(25%),其中3例(15%)位于乳头体漏斗轴。1例位于小脑,另1例位于侧脑室并累及脉络膜裂。8例患者(40%)观察到相关异常。6例患者(30%)胼胝体发育出现改变,5例为孤立性改变,1例伴有透明隔部分缺如。另1例患者发现孤立性透明隔部分发育不全,1例检测到Goldenhar综合征。根据初始临床特征进行的放射学随访,脂肪瘤的形态或大小未显示任何差异。

结论

1)ICL最常位于大脑镰旁和四叠体池;2)在这些部位它们可与其他脑畸形相关,最重要的是胼胝体和间隔的缺陷;3)大多数ICL是在神经影像学检查过程中偶然发现的。仅3例患者(15%)的ICL位置(胼胝体周围)、症状(整体精神运动发育迟缓)和相关畸形(胼胝体发育不全)之间存在推测关系;4)癫痫的发生率远低于早期报告中的声称,主要相关的神经症状是精神运动发育迟缓和头痛(70%);5)ICL是一种发育异常,不需要神经外科治疗,通常不会随时间变化,尽管可能因脂肪肥大而增大。

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