Muthukumar Natarajan
Department of Neurosurgery, Madurai Medical College, Madurai, India.
J Neurosurg. 2007 Aug;107(2 Suppl):87-97. doi: 10.3171/PED-07/08/087.
The purpose of this study was to report the author's experience with 14 cases of terminal and nine cases of nonterminal myelocystoceles and to highlight the differences between these two groups in regard to the embryological origins, clinical presentation, operative findings, results on neuroimaging studies, and prognosis for these lesions.
This is a retrospective analysis of 14 cases of terminal and nine of nonterminal myelocystoceles treated between January 1998 and January 2006. All patients underwent neurological examination, plain x-ray films of the spine, computed tomography scans of the brain, and magnetic resonance (MR) imaging of the spine. In seven of these cases, MR imaging included three-dimensional constructive interference in steady-state sequences, and in four cases MR myelography was also done. Follow-up duration ranged from 3 months to 4 years.
All of the patients with terminal myelocystocele presented with swelling in the low back and varying degrees of neurological deficits, except four who had normal results on neurological tests. The MR images revealed classic features of terminal myelocystoceles in all patients. In each case, patients underwent excision of the meningocele sac and drainage of the syringocele with untethering of the spinal cord. During the last follow-up visit, there was no change in the neurological status of these children. In the nonterminal myelocystocele group, one lesion was cervical, six were thoracic, and two were lumbar lesions. All except one patient presented without neurological deficits; that patient had paraplegia with incontinence. Admission MR images revealed Rossi Type I nonterminal myelocystocele in six and Rossi Type II nonterminal myelocystocele in three patients. Children with Type I lesions underwent excision of the fibroneurovascular stalk and excision of the meningocele sac, whereas those with Type II lesions underwent drainage of the syringocele, untethering of the cord, and excision of the meningocele sac. There was no change in the neurological status postoperatively. During the follow-up period no patient in either group presented with retethering.
Myelocystoceles, both terminal and nonterminal, are different from other skin-covered masses in the back. A proper imaging evaluation is required to differentiate myelocystoceles from other skin-covered masses in this area, because the surgical treatment and prognosis are different for this subset of patients with occult spinal dysraphism. Terminal myelocystoceles are different from nonterminal ones embryologically, clinically, radiologically, surgically, and prognostically. These differences are discussed.
本研究旨在报告作者对14例终末型脊髓脊膜膨出和9例非终末型脊髓脊膜膨出的治疗经验，并着重阐述这两组病例在胚胎起源、临床表现、手术所见、神经影像学检查结果以及这些病变的预后方面的差异。
这是一项对1998年1月至2006年1月期间治疗的14例终末型和9例非终末型脊髓脊膜膨出病例的回顾性分析。所有患者均接受了神经系统检查、脊柱X线平片、脑部计算机断层扫描以及脊柱磁共振（MR）成像检查。其中7例患者的MR成像包括三维稳态构成干扰序列，4例患者还进行了MR脊髓造影检查。随访时间为3个月至4年。
所有终末型脊髓脊膜膨出患者均表现为下腰部肿胀及不同程度的神经功能缺损，但有4例患者神经系统检查结果正常。MR图像显示所有患者均具有终末型脊髓脊膜膨出的典型特征。每例患者均接受了脊膜膨出囊切除术及脊髓空洞引流术，并松解脊髓。在最后一次随访时，这些患儿的神经功能状态无变化。在非终末型脊髓脊膜膨出组中，1例病变位于颈部，6例位于胸部，2例位于腰部。除1例患者外，所有患者均无神经功能缺损表现；该患者患有截瘫伴尿失禁。入院时的MR图像显示，6例患者为Rossi I型非终末型脊髓脊膜膨出；3例患者为Rossi II型非终末型脊髓脊膜膨出。I型病变的患儿接受了纤维神经血管蒂切除术及脊膜膨出囊切除术，而II型病变的患儿则接受了脊髓空洞引流术、脊髓松解术及脊膜膨出囊切除术。术后神经功能状态无变化。在随访期间，两组均无患者出现脊髓再栓系。
终末型和非终末型脊髓脊膜膨出均不同于背部其他有皮肤覆盖的肿块。由于对于这一隐性脊柱裂患者亚组，手术治疗和预后有所不同，因此需要进行恰当的影像学评估，以将脊髓脊膜膨出与该区域其他有皮肤覆盖的肿块区分开来。终末型脊髓脊膜膨出在胚胎学、临床、放射学、手术及预后方面均与非终末型不同。文中对这些差异进行了讨论。
