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[脊髓脊膜膨出合并中枢神经系统畸形的意义]

[The significance of associated malformations of the central nervous system in myelomeningocele].

作者信息

Christensen B, Rand-Hendriksen S

机构信息

Avdeling for medisinsk genetikk, Ullevål sykehus, Oslo.

出版信息

Tidsskr Nor Laegeforen. 1998 Nov 10;118(27):4232-4.

PMID:9857808
Abstract

Neural tube defects are the most frequent congenital structural malformations in Norway. Approximately half of these are myelomeningocele. Infants with myelomeningocele frequently have hydrocephalus at birth. Problems with intracranial pressure may as well develop later. There are several reports on pathological corpus callosum and an increasing number of reports on cognitive problems in patients with myelomeningocele. Most patients with myelomeningocele have a tethered spinal cord, and some have syringomyelia. Chiari malformation type II is a malformation of the skull and brainstem which is frequently observed in individuals with myelomeningocele. Chiari malformation may cause severe respiratory problems in infants. Chiari malformation, tethered cord as well as syringomyelia are associated with a range of neurological problems which may progress in adulthood. Surgical intervention may improve the situation. Anaesthesia may induce neurological complications in individuals with Chiari malformation or syringomyelia. Since pregnancy and childbirth are associated with complications, women with myelomeningocele should be examined before they become pregnant. Children and adults with myelomeningocele should routinely undergo MRI examinations of caput and the spinal cord to clarify their anatomical situation.

摘要

神经管缺陷是挪威最常见的先天性结构畸形。其中约一半是脊髓脊膜膨出。患有脊髓脊膜膨出的婴儿出生时经常患有脑积水。颅内压问题也可能在以后出现。有几篇关于胼胝体病理的报告,并且脊髓脊膜膨出患者认知问题的报告数量也在增加。大多数脊髓脊膜膨出患者有脊髓栓系,有些还患有脊髓空洞症。II型Chiari畸形是一种颅骨和脑干畸形,在脊髓脊膜膨出患者中经常观察到。Chiari畸形可能导致婴儿出现严重的呼吸问题。Chiari畸形、脊髓栓系以及脊髓空洞症都与一系列可能在成年期进展的神经问题有关。手术干预可能会改善这种情况。麻醉可能会在患有Chiari畸形或脊髓空洞症的个体中引发神经并发症。由于怀孕和分娩会带来并发症,患有脊髓脊膜膨出的女性在怀孕前应接受检查。患有脊髓脊膜膨出的儿童和成人应定期进行头部和脊髓的MRI检查,以明确其解剖情况。

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