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脊柱裂

Spinal dysraphism.

作者信息

Jindal A, Mahapatra A K, Kamal R

机构信息

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

出版信息

Indian J Pediatr. 1999 Sep-Oct;66(5):697-705. doi: 10.1007/BF02726258.

Abstract

Spinal dysraphism (SD) is characterized by maldevelopment of neural tube, notochord, mesoderm and cutaneous ectoderm. Incidence of SD is 2-4/1000 live births. One hundred and nineteen patients operated from January 1991-June 1996 at Department of Neurosurgery, All India Institute of Medical Sciences, were studied. Only 21 patients (17.6%) presented when they were less than one year old and 17 patients came in adult age group (> 16 years). Lumbar and lumbosacral region was the commonly involved site in 81 patients (74.7%). Weakness of lower limbs (74%), difficulty in walking (54%), muscle atrophy (41.2%) were the commonest indicators of motor system involvement. Loss of sensation, trophic ulcer, backache were seen in 45, 14, 10 patients respectively. Cutaneous lipoma (26%), hypertrichiosis (20%), dermal sinus (13.4%), midline dimples (7%) were the important cutaneous markers. Foot and limb deformity was seen in 25% cases. Tethering of cord, syringomyelia & split cord malformation were the most common radiological findings. Only 10% of our patients had hydrocephalus that required shunt. Out on 119 cases operated, 43 improved, Twenty had sensory improvement and 18 showed motor improvement. Fifteen patients regained continence. Twelve patients were lost to follow-up. Sixty-seven patients had no change in neurological status, post-operatively. Six cases deteriorated in terms of motor or sensory deficit and one patient lost continence. CSF leak (8%) and wound infection (6%) were the common complications. Six patients required second surgery as T.P. Shunt (4), rotation flap (1), reexploration and duraplasty (1).

摘要

脊柱裂(SD)的特征是神经管、脊索、中胚层和皮肤外胚层发育异常。脊柱裂的发病率为每1000例活产中有2 - 4例。对1991年1月至1996年6月在全印度医学科学研究所神经外科接受手术的119例患者进行了研究。只有21例患者(17.6%)在1岁以下就诊,17例患者为成年年龄组(>16岁)。81例患者(74.7%)的病变部位常见于腰骶部。下肢无力(74%)、行走困难(54%)、肌肉萎缩(41.2%)是运动系统受累最常见的指标。分别有45例、14例、10例患者出现感觉丧失、营养性溃疡、背痛。皮肤脂肪瘤(26%)毛过多(20%)、皮样窦(13.4%)、中线酒窝(7%)是重要的皮肤标志。25%的病例出现足部和肢体畸形。脊髓栓系、脊髓空洞症和脊髓纵裂畸形是最常见的影像学表现。我们的患者中只有10%患有需要分流的脑积水。在接受手术的119例病例中,43例病情好转,20例感觉功能改善,18例运动功能改善。15例患者恢复了大小便自控能力。12例患者失访。67例患者术后神经功能状态无变化。6例患者在运动或感觉功能方面出现恶化,1例患者失去了大小便自控能力。脑脊液漏(8%)和伤口感染(6%)是常见的并发症。6例患者需要二次手术,包括脑室腹腔分流术(4例)、旋转皮瓣术(1例)、再次探查及硬脑膜成形术(1例)。

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