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先天性包涵瘤在脊柱发育不良中的表现。

Congenital inclusion tumours in spinal dysraphism.

机构信息

Department of Pediatric Surgery, St. John's National Academy of Health Sciences, Bangalore, India.

出版信息

Indian J Pediatr. 2010 Feb;77(2):167-70. doi: 10.1007/s12098-009-0290-z. Epub 2010 Jan 20.

Abstract

OBJECTIVE

To review the presentation, diagnosis and management of children with spinal dysraphism and CIT.

METHODS

It is a retrospective review of 146 children of spina bifida over 9 years (2000-2008) and details the clinical course and outcome of seven with associated congenital inclusion tumors.

RESULTS

7/146 (4.7%) had spina bifida with CIT, 5 dermoid cysts and 2 mature teratoma. The diagnosis was missed by the primary physician even in the presence of a neurocutaneous marker. Spinal imaging with MRI was conclusive. All were managed with multilevel laminectomy, near total/total excision of the CIT and detethering of cord. Intramedullary involvement and established neurological deficits at presentation were associated with persistent deficits.

CONCLUSION

Early detection and comprehensive management of CIT with spinal dysraphism ensures social fecourinary continence, preserves renal function, achieves ambulation and enables patients to lead an acceptable quality of life.

摘要

目的

回顾儿童脊髓脊膜膨出合并先天性包涵瘤(CIT)的表现、诊断和治疗。

方法

回顾性分析 9 年来(2000-2008 年)收治的 146 例脊髓脊膜膨出患儿,详细描述 7 例伴有先天性包涵瘤患儿的临床过程和结局。

结果

7/146(4.7%)例患儿存在脊髓脊膜膨出合并 CIT,包括 5 例皮样囊肿和 2 例成熟畸胎瘤。尽管存在神经皮肤标记物,但初级保健医生仍漏诊。脊髓 MRI 检查明确诊断。所有患儿均行多节段椎板切除术,尽可能全切/次全切除 CIT,并松解脊髓。髓内受累和就诊时存在神经功能缺损与持续性缺损相关。

结论

早期发现和全面治疗脊髓脊膜膨出合并 CIT 可确保社交和泌尿功能的控尿,保护肾功能,实现行走能力,并使患者过上可接受的生活质量。

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