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肛门直肠畸形患者的脊髓栓系:初步结果。

Tethered cord in patients with anorectal malformation: preliminary results.

作者信息

Suppiej A, Dal Zotto L, Cappellari A, Traverso A, Castagnetti M, Drigo P, Midrio P

机构信息

Child Neurology and Clinical Neurophysiology Unit, University of Padua, Padua, Italy.

出版信息

Pediatr Surg Int. 2009 Oct;25(10):851-5. doi: 10.1007/s00383-009-2435-6.

Abstract

BACKGROUND

A tethered cord (TC) has been reported in as much as 50% of the patients affected by anorectal malformation (ARM). No guidelines for timing and modality of diagnosis and treatment have been established. We present the preliminary results of a multidisciplinary protocol carried out at our center.

METHODS

Seventy-four ARM patients underwent spinal magnetic resonance imaging (MRI). All TC patients underwent videourodynamic (UD), somatosensory-evoked potentials (SEPs), and neurological examination at baseline and, if normal, at 5 and 10 years of age. Conversely, when UD or SEP abnormalities were detected the follow-up was individually tailored at shorter time.

RESULTS

25/74 patients had a neuroradiological TC (33.7%). Based on the results of UD, SEP, and neurological status, four patients were untethered, eight are possible candidates, nine are stable, and four were excluded because of incomplete data.

DISCUSSION

Tethered cord is frequent in ARM patients. Because neurological deficits secondary to TC can contribute to neurological disability, we recommend routine MRI examination and a multidisciplinary program of follow-up in cases of TC. Preliminary results suggest the combined use of SEPs and UD could represent a useful adjunct to clinical examination in patients in whom a "wait and see" approach is preferred to the prophylactic surgery.

摘要

背景

据报道,在患有肛门直肠畸形(ARM)的患者中,多达50%存在脊髓栓系(TC)。目前尚未制定关于诊断和治疗时机及方式的指南。我们展示了在我们中心实施的多学科方案的初步结果。

方法

74例ARM患者接受了脊柱磁共振成像(MRI)检查。所有TC患者在基线时以及(若结果正常)在5岁和10岁时接受了视频尿动力学(UD)、体感诱发电位(SEP)和神经学检查。相反,当检测到UD或SEP异常时,随访时间则根据个体情况缩短。

结果

74例患者中有25例(33.7%)存在神经放射学上的TC。根据UD、SEP和神经学状况的结果,4例患者不存在脊髓栓系,8例可能是手术候选者,9例情况稳定,4例因数据不完整被排除。

讨论

脊髓栓系在ARM患者中很常见。由于TC继发的神经功能缺损可能导致神经残疾,我们建议对TC患者进行常规MRI检查和多学科随访方案。初步结果表明,对于倾向于采取“观察等待”而非预防性手术的患者,SEP和UD的联合应用可能是临床检查的有用辅助手段。

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