Regression of Chiari malformation type 2 following early postnatal meningomyelocele repair-a retrospective observation from an institutional series of patients.

IF 1.3 4区 医学 Q4 CLINICAL NEUROLOGY
Child's Nervous System Pub Date : 2024-11-01 Epub Date: 2024-08-29 DOI:10.1007/s00381-024-06586-3
Radek Frič, Mona Kristiansen Beyer, Bernt Johan Due-Tønnessen
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引用次数: 0

Abstract

Purpose: Spontaneous regression of Chiari malformation type 2 (CM2) is observed rarely, as CM2 is associated with meningomyelocele (MMC) that is surgically repaired either pre- or early postnatally. While the radiological regression of CM2 occurs frequently following prenatal repair of MMC, it has been reported in only a few studies after postnatal repair.

Methods: From the consecutive series of children with postnatally repaired MMC, we reviewed the clinical and radiological data regarding CM2, particularly its regression either spontaneously or following CSF diversion.

Results: Eighteen children underwent postnatal repair of MMC between February 2011 and April 2023. CM2 was present in 16 (89%), and hydrocephalus in 15 children (83%), requiring shunting in 14 of them. During the mean clinical observation time (from birth to April 2023) of 59 ± 51 months, three children with CM2 (19%) underwent 1-2 foramen magnum decompressions (FMD), five children (28%) 1-4 surgical untethering procedures and 13 children with shunted hydrocephalus (93%) 1-5 shunt revisions. Out of sixteen children with CM2, we observed regression of CM2 on MRI in only one case (6%) during the mean radiological follow-up (from birth to the last MRI taken) of 49 ± 51 months.

Conclusion: In our experience, spontaneous regression of CM2 in children with postnatally repaired MMC occurs quite rarely. Pathophysiological mechanisms behind the development of CM2 in children with MMC remain unclear, but our observation supports the hypothesis of an association between the downward displacement of the hindbrain and the low intraspinal pressure secondary to CSF leakage in children born with MMC.

Abstract Image

产后早期脑膜瘢痕修复术后Chiari畸形2型的消退--机构系列患者的回顾性观察。
目的:Chiari畸形2型(CM2)很少出现自发性消退,因为CM2与脑膜疝(MMC)有关,而MMC在出生前或出生后早期就已通过手术修复。虽然在产前对 MMC 进行修复后,CM2 的放射学退变经常发生,但在产后修复后,只有少数研究报告了这种情况:方法:我们从一系列连续的产后修复 MMC 患儿中回顾了有关 CM2 的临床和放射学数据,尤其是其自发或 CSF 转移后的消退情况:2011年2月至2023年4月期间,18名患儿接受了MMC产后修复手术。16名患儿(89%)出现CM2,15名患儿(83%)出现脑积水,其中14名患儿需要进行分流。在 59 ± 51 个月的平均临床观察时间内(从出生到 2023 年 4 月),3 名 CM2 患儿(19%)接受了 1-2 次枕骨大孔减压术(FMD),5 名患儿(28%)接受了 1-4 次手术松绑,13 名分流性脑积水患儿(93%)接受了 1-5 次分流手术。在 16 名患有 CM2 的儿童中,我们在平均 49 ± 51 个月的放射学随访期间(从出生到最后一次拍摄核磁共振成像),仅在一个病例(6%)的核磁共振成像中观察到 CM2 消退:根据我们的经验,产后修复的 MMC 患儿 CM2 自发消退的情况非常罕见。MMC 患儿出现 CM2 的病理生理学机制尚不清楚,但我们的观察结果支持后脑向下移位与先天性 MMC 患儿脑脊液渗漏导致椎管内压力过低之间存在关联的假设。
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来源期刊
Child's Nervous System
Child's Nervous System 医学-临床神经学
CiteScore
3.00
自引率
7.10%
发文量
322
审稿时长
3 months
期刊介绍: The journal has been expanded to encompass all aspects of pediatric neurosciences concerning the developmental and acquired abnormalities of the nervous system and its coverings, functional disorders, epilepsy, spasticity, basic and clinical neuro-oncology, rehabilitation and trauma. Global pediatric neurosurgery is an additional field of interest that will be considered for publication in the journal.
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