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脊柱裂-Spinal Dysraphism-翻译的外文文章

董月青 副主任医师 好大夫工作室 神经外科
2009-07-05 2330人已读
董月青 副主任医师
好大夫工作室

International Pediatrics/Vol. 16/No. 1/2001

 

Spinal dysraphism refers to a group of congenital anormalies that is characterized by a failure of fusion of midline structures and it is frequently referred to as open or closed. Open lesion occur in early stage and are called spina bifida aperta or myelomeningocele. The closed defect, spinal bifida occulta, is covered by skin and may not be detected until later in life.好大夫工作室神经外科董月青

脊柱裂是脊柱中线结构融合障碍导致的先天异常,分为开放性和闭合性脊柱裂。开放性脊柱裂发生在胚胎发育的早期,又叫脊髓脊膜膨出。闭合性脊柱裂又称隐形脊柱裂表面有皮肤覆盖,一般发现较晚。

Neonates with a tethered spinal cord from spinal dysraphism may have a normal neurological exam. However, if the condition id undetected and untreated, these children, as they grow, may develop neurological deficits, orthopedic changes, urinary dysfunction or pain in the lower back or leg. It is postulated that the mechanical distortion of the spinal cord, as the child grows or during normal daily activities, contribute to the clinical picture.

存在脊柱裂伴脊髓拴系的婴儿可能神经系统检查正常,但是如果不治疗随着孩子的长大,就会表现为神经功能障碍的缺失,小便异常或后背下肢的疼痛。可能是随着孩子的成长和日常活动的增加,脊髓功能的紊乱导致患者出现临床症状。

occasionally the question arises that if the patient is neurologically intact and has a tethered spinal cord, is surgical intervention necessary? Mclone has shown that once motor deficits occur, opproximately 45% of patient return to normal following and untethering procedure. If the patient has developed urinary incontinence, then only 12% of those patients will become continent following untethering. It is accepted that once the diagnosis of a tethered spinal cord is made untethering is recommended prior to the development of the inevitable deterioration that is seen in the unoperaed patient.

有时候我们会问,一个存在脊髓拴系但是没有症状的患者需要手术吗?Mclone认为一旦出现运动功能的缺失,大约有45%的患者经拴系解除手术后恢复到正常。如果出现小便失禁只有12%的患者经拴系解除手术后恢复到正常。因此我们形成一个治疗规范,一旦诊断为脊髓拴系,建议在没有发生不可逆神经损害前接受手术治疗,这是从不接受手术的患者那里得到的教训。

Lipomyelomeningocele and diastematomyelia require more extensive surgical procedure. The goal of surgery in the former is to resect as much of the fatty mass as possible and free up the attachment of the fatty tissue, which has tethered the dura and the spinal cord. Technological advances, such as the CO2 laser and improved illumination and magnification, have increased surgical precision and reduced the surgical trauma.

脂肪脊髓脊膜膨出和脊髓纵裂则要求更加深入的手术治疗,脂肪脊髓脊膜膨出手术就是要尽可能的切除脂肪瘤,解除其对硬脊膜和脊髓的拴系。目前技术的进步,如CO2 激光,照明和放大设施的改善大大增加了手术的准确性同时减少了手术的损伤。

上图中红色虚线部分代表与脊髓粘连紧密的脂肪瘤,是导致病变的罪魁祸首

Because most children with myelomeningocele also have hydrocephalus, treatment may include the implantation of a shunt, a tube that allows the excess cerebrospinal fluid to drain to other parts of the body.

由于很多脊髓脊膜膨出的患儿同时存在脑积水,因此在行修补术的同时要用引流管分流过多的脑脊液到身体的其他部位。

Tethered cord syndrome is frequently seen in our neurological department as urological problems present as the initial signs or symptoms for this syndrome. Lumbosacral lipoma can be responsible for progressive neurological and urological defects. Surgery for untethering these structures may prevent late deterioration. Pierre-Kahn et al. routinely and systematically performed an operation even in the absence of neurological defects. Untethering surgery may help decompress and spare functional neural tissue and prevent retethering of the spinal cord. In our patients, the operation included untethering, removal of tumors, duroplasty, laminectomy. Although it delayed the release of our patients, effects reversed the loss of neurological function and thus prevented deterioration of renal function.

我们经常碰到脊髓拴系综合症导致尿失禁作为首发症状就诊的患者。腰骶部脂肪瘤导致了神经功能和小便功能的缺失。手术解除脊髓拴系能够阻止病情进一步恶化。Pierre-Kahn 等对存在脊髓拴系的患者常规手术治疗,即使没有症状的患者同样如此。解除拴系的手术能够起到减压,保护神经功能和防止发生再次拴系的可能。我们对这种患者实施的手术包括,解除拴系,切除脂肪瘤,硬脊膜成形术和椎板切除术。尽管延缓了患者的出院,但是手术的效果能够起到防止神经功能的缺失和泌尿功能的恶化。

上图是我们切除肿瘤的全过程,可见手术中在显微镜下分离神经粘连是非常重要的。另外缺损的脊柱裂面积可见很大,手术中用人工硬脊膜修补。图中蓝色虚线部分为膨出的脂肪瘤和缺损的脊柱裂骨质)

Tethered cord syndrome is a progressively worsening disorder. It may cause irreversible changes of neurological and urological complication if the diagnosis is delayed. Then early diagnosis and early operation of tethered cord syndrome is important. Since surgical intervention did not completely cure this condition during adulthood, it would be helpful to study ways to detect this disease early during childhood.

脊髓拴系综合症是一种进行性恶化的疾病如果延迟治疗就会引起神经和泌尿功能的不可逆病变。因此早期治疗和手术治疗是关键。虽然到成年手术治疗不能完全治愈该病,但是却有助于我们寻求早期诊断 该病的方法。

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董月青 副主任医师

好大夫工作室 神经外科

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