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脊髓先天性皮样窦——10年期间5例的经验

Spinal congenital dermal sinus--experience of 5 cases over a period of 10 years.

作者信息

Wang K C, Yang H J, Oh C W, Kim H J, Cho B K

机构信息

Department of Neurosurgery, Seoul National University College of Medicine, Korea.

出版信息

J Korean Med Sci. 1993 Oct;8(5):341-7. doi: 10.3346/jkms.1993.8.5.341.

Abstract

Spinal congenital dermal sinus (CDS) is a rare entity which supposedly results from the failure of the neuroectoderm to separate from the cutaneous ectoderm during the process of neurulation. The lesions are most frequent at the lumbosacral followed by the occipital region. CDS of the thoracic region is very rare. The patients with spinal CDS present with meningitis and/or mass effect from the associated inclusion tumor. They are usually dermoid or epidermoid cysts. Teratoma is rarely associated. The authors experienced 5 cases of spinal CDS over a 10 year period. Of the 5 cases, 2 were at thoracic and 3 were at lumbosacral levels. Dermoid cyst, epidermoid cyst and teratoma were associated in one case each. Two cases presented with neurological deficit and meningitis while an additional case presented with neurological deficit and a history of probable meningitis. Pain was present in 2 cases. Magnetic resonance imaging played an important role in the diagnosis of the lesion and planning of surgery. All the cases showed a good response to surgery even though one patient had persistent neurological deficit.

摘要

脊柱先天性皮样窦(CDS)是一种罕见的疾病,据推测是由于神经外胚层在神经胚形成过程中未能与皮肤外胚层分离所致。病变最常见于腰骶部,其次是枕部。胸段CDS非常罕见。脊柱CDS患者表现为脑膜炎和/或相关包涵体肿瘤引起的占位效应。这些肿瘤通常是皮样或表皮样囊肿。畸胎瘤很少与之相关。作者在10年期间遇到了5例脊柱CDS。在这5例中,2例位于胸段,3例位于腰骶段。皮样囊肿、表皮样囊肿和畸胎瘤各有1例与之相关。2例出现神经功能缺损和脑膜炎,另外1例出现神经功能缺损并有可能患脑膜炎的病史。2例有疼痛。磁共振成像在病变诊断和手术规划中发挥了重要作用。尽管有1例患者存在持续性神经功能缺损,但所有病例对手术均有良好反应。

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