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[隐性脊柱裂中膀胱排空的神经源性疾病]

[Neurogenic disorders of bladder emptying in closed spinal dysraphism].

作者信息

Madersbacher H, Ebner A

机构信息

Universitäts-Kliniken Innsbruck.

出版信息

Urologe A. 1992 Nov;31(6):347-53.

PMID:1462486
Abstract

Closed (occult) spinal dysraphism, e.g. lipomyelomeningocele, intraspinal lipoma, diastematomyelia, the tethered spinal cord in its various forms and dysgenesis of the sacrum, is often diagnosed late and only symptoms of neurogenic bladder dysfunction are present. A lipomyelomeningocele mostly causes detrusor and sphincter dysfunction, as was the case in five of six children among our patients. However, improvement of neurological and urological symptoms after the operation can only be achieved in about 40%. Four of eight children with diastematomyelia suffered from neurogenic bladder dysfunction; three have meanwhile undergone surgery with complete recovery in one, no relevant change in the second, and worsening in the third. Originally a specific term, the "tethered spinal cord" when associated with spinal dysraphism has taken on a more general meaning. Nowadays this term is not only used for a short, thickened and tight filum terminale, but comprises any pathology, which prevents the spinal cord from ascending. MRI examination of the craniovertebral junction and spinal cord of patients with treated myelomeningocele often reveals secondary pathologic changes: these may be areas of cord atrophy, hydromyelic cavitation or ventral compression from arachnoid cysts with clinical symptoms mostly after the age of 5 years. In these children a changing urodynamic pattern may therefore be caused by such a pathology and is an indication for a thorough neurological examination including MRI. Of all the dysrhaphic states mentioned above, sacral dysgenesis is the most frequent. The sacral osteological anomaly, as a numerical and as a structural anomaly, also determines the neuro-urological deficit.(ABSTRACT TRUNCATED AT 250 WORDS)

摘要

闭合性(隐匿性)脊柱裂,如脂肪瘤型脊髓脊膜膨出、脊髓内脂肪瘤、脊髓纵裂、各种形式的脊髓拴系以及骶骨发育不全,通常诊断较晚,且仅出现神经源性膀胱功能障碍的症状。脂肪瘤型脊髓脊膜膨出大多会导致逼尿肌和括约肌功能障碍,我们的患者中有六分之五的儿童是这种情况。然而,手术后神经和泌尿系统症状的改善率仅约为40%。八名脊髓纵裂患儿中有四名患有神经源性膀胱功能障碍;其中三名已接受手术,一名完全康复,一名无明显变化,一名病情恶化。“脊髓拴系”最初是一个特定术语,当与脊柱裂相关时,其含义已更为宽泛。如今,该术语不仅用于指短而增厚且紧绷的终丝,还包括任何阻止脊髓上升的病理情况。对接受过脊髓脊膜膨出治疗的患者进行颅颈交界区和脊髓的MRI检查,常常会发现继发性病理改变:这些可能是脊髓萎缩区域、积水性空洞形成或蛛网膜囊肿导致的腹侧压迫,临床症状大多出现在5岁以后。因此,在这些儿童中,这种病理情况可能会导致尿动力学模式发生变化,这是进行包括MRI在内的全面神经学检查的一个指征。在上述所有脊柱裂状态中,骶骨发育不全最为常见。骶骨的骨骼异常,无论是数量上还是结构上的异常,也决定了神经泌尿系统的缺陷。(摘要截选至250词)

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