Borkar Sachin A, Mahapatra A K
Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi, India.
Asian J Neurosurg. 2012 Apr;7(2):56-60. doi: 10.4103/1793-5482.98643.
Over a 2-year period, 2008-2009, a total of 53 cases of split cord malformation (SCM) were treated at the All India Institute of Medical Sciences (AIIMS). This study is a retrospective analysis of clinical features, radiological findings, and surgical outcome of these patients.
During this period, 53 cases of SCM were treated at AIIMS. They constitute around 27% of all spinal dysraphism surgeries performed at the department of Neurosurgery, AIIMS; as 200 cases of spinal dysraphism were operated during the study period. The data was obtained from case files, operation notes, discharge summaries, and follow-up files.
There were 30 cases of SCM type I and 23 cases of type II SCM. Seven patients were adult above 18 years of age. Except 7 patients, remaining 46 were symptomatic. Bony deformity of spine was recorded in 24 patients; of them, 19 had scoliosis and 4 had kyphosis. Deformity of foot was recorded in 10 patients. Thirteen patients had hypertrichosis, while four had dermal sinus. Magnetic resonance imaging (MRI) was performed in all patients. MRI revealed syringomyelia in 14 patients; however, only one patient had associated Chiari malformation. Six patients had meningomyelocele. Intra-operative; thick filum was noticed in 10 cases and in another 9 cases, there was filum lipoma. Dermoid was encountered in 4 patients, one patient had epidermoid tumor. Site of split was thoracic in 22, followed by lumbar region in 21 patients. Only 3 patients had split in cervical spinal cord. Seven patients had two separate splits at two different levels. Two patients had posteriorly located bony spur. All patients underwent surgery. Seven patients, those who had no neurological deficits pre-op, remained unchanged post-op. Amongst the 46 patients who had preoperative neurological deficits, eight had neurological deterioration post-op; five had deterioration in motor power and three had urinary problem. Five of these patients had type Id split, 2 had type Ic split, and one had type Ib split. However, among 8 patients who deteriorated post-op, four improved to preoperative status by the time of discharge. Thus, 4/53(7%) patients had long-term deficits, all with type Id split. Follow-up data was available for 36 patients (68%) and mean follow-up period was 12 months (range 6-24 months). Follow-up MRI revealed decrease in syringomyelic cavity in 6 of the 14 patients (44%) who had syringomyelia on preoperative MRI scans.
Overall, SCM is an uncommon condition. In all cases of progressive scoliosis, MRI must be carried out. We subjected all asymptomatic patients to surgery and none developed post-op deterioration. Overall post-op neurological deterioration was noticed in 15% patients, of which 8% had transient post-operative deterioration. The new Type I SCM subclassification system proposed by Mahapatra and Gupta is found to have a significant prognostic value in assessing post-operative neurological deterioration in patients with type I SCM.
在2008 - 2009年的两年期间，全印度医学科学研究所（AIIMS）共治疗了53例脊髓纵裂畸形（SCM）患者。本研究是对这些患者的临床特征、影像学表现及手术结果的回顾性分析。
在此期间，AIIMS共治疗了53例SCM患者。他们约占AIIMS神经外科所进行的所有脊柱裂手术的27%；因为在研究期间共进行了200例脊柱裂手术。数据来自病例档案、手术记录、出院小结及随访档案。
有30例I型SCM和23例II型SCM。7例患者为18岁以上成年人。除7例患者外，其余46例有症状。24例患者记录有脊柱骨性畸形；其中，19例有脊柱侧弯，4例有脊柱后凸。10例患者记录有足部畸形。13例患者有多毛症，4例有皮样窦。所有患者均行磁共振成像（MRI）检查。MRI显示14例患者有脊髓空洞症；然而，只有1例患者合并Chiari畸形。6例患者有脊膜脊髓膨出。术中；10例患者发现终丝增厚，另外9例患者有终丝脂肪瘤。4例患者发现皮样囊肿，1例患者有表皮样肿瘤。脊髓分裂部位在胸段的有22例，其次腰段有21例。只有3例患者脊髓分裂发生在颈段。7例患者在两个不同节段有两处独立的分裂。2例患者有位于后方的骨赘。所有患者均接受了手术。7例术前无神经功能缺损的患者术后情况无变化。在46例术前有神经功能缺损的患者中，8例术后出现神经功能恶化；5例运动功能恶化，3例有排尿问题。这些患者中5例为Id型分裂，2例为Ic型分裂，1例为Ib型分裂。然而，在8例术后恶化的患者中，4例在出院时恢复到术前状态。因此，4/53（7%）的患者有长期神经功能缺损，均为Id型分裂。36例患者（68%）有随访数据，平均随访期为12个月（范围6 - 24个月）。随访MRI显示，术前MRI扫描有脊髓空洞症的14例患者中，6例（44%）脊髓空洞腔缩小。
总体而言，SCM是一种罕见疾病。在所有进行性脊柱侧弯病例中，均必须进行MRI检查。我们对所有无症状患者进行了手术，且无一例术后出现恶化。总体术后神经功能恶化见于15%的患者，其中8%有短暂的术后恶化。发现Mahapatra和Gupta提出了新的I型SCM亚分类系统在评估I型SCM患者术后神经功能恶化方面具有重要的预后价值。
