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脊髓先天性皮样窦:7年23例的经验

Spinal congenital dermal sinus: an experience of 23 cases over 7 years.

作者信息

Jindal A, Mahapatra A K

机构信息

Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi--110 085, India.

出版信息

Neurol India. 2001 Sep;49(3):243-6.

Abstract

Spinal congenital dermal sinus is a rare entity, which supposedly results from the failure of neuroectoderm to separate from the cutaneous ectoderm during the process of neurulation. The present study was undertaken to know the clinical profile of these patients, to study associated anomalies and to assess the results of surgical intervention. We had 23 patients with male : female ratio of 9:16. Only 2 patients were below 2 years of age and most cases (16) were between 2-16 years (mean age =10.2 years). Lumbar region (17 cases) was most frequently involved, followed by lumbosacral and thoracic region in 3 patients each. Only three patients were asymptomatic at the time of presentation. Most of the cases presented with evidence of neural compression or tethered cord syndrome. Only one case presented with spinal abscess. The motor, sensory and autonomic deficits were seen in 20, 11 and 12 patients respectively. Scoliosis and CTEV (congenital talipus equino varus) were the common associated anomalies. MRI revealed associated dysraphic state of spinal cord in 21(>90%) cases. All patients underwent surgical exploration and repair of dysraphic state and excision of the sinus. None of the asymptomatic patients deteriorated. Overall 8 patients improved, 14 got their neurological status stabilized, including 3 asymptomatic cases. Only one patient deteriorated. Postoperative wound infection was seen in 2 cases. As age advances, the chance of developing neurological deficit increases. Associated dysraphic state should be looked for and treated simultaneously, using microsurgical technique, whenever possible. It is better to treat all these cases with aggressive surgical intervention before the neurological deficits appear.

摘要

脊髓先天性皮样窦是一种罕见的病症,据推测是由于在神经胚形成过程中神经外胚层未能与皮肤外胚层分离所致。本研究旨在了解这些患者的临床特征,研究相关异常情况,并评估手术干预的结果。我们有23例患者,男女比例为9:16。仅2例患者年龄在2岁以下,大多数病例(16例)年龄在2至16岁之间(平均年龄 = 10.2岁)。腰椎区域(17例)受累最为频繁,其次是腰骶部和胸部区域,各有3例。仅3例患者在就诊时无症状。大多数病例表现出神经受压或脊髓栓系综合征的证据。仅1例患者出现脊髓脓肿。分别有20例、11例和12例患者出现运动、感觉和自主神经功能障碍。脊柱侧弯和马蹄内翻足(先天性马蹄内翻足)是常见的相关异常情况。MRI显示21例(>90%)病例存在脊髓发育不全状态。所有患者均接受了手术探查、修复发育不全状态并切除窦道。无症状患者均未出现病情恶化。总体而言,8例患者病情改善,14例患者神经功能状态稳定,其中包括3例无症状病例。仅1例患者病情恶化。2例患者出现术后伤口感染。随着年龄增长,出现神经功能障碍的几率增加。只要有可能,应使用显微外科技术同时查找并治疗相关的发育不全状态。最好在神经功能障碍出现之前对所有这些病例进行积极的手术干预。

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