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Journal of Neurorestoratology  2020, Vol. 8 Issue (2): 83-92    doi: 10.26599/JNR.2020.9040010
Review Article     
Chinese expert consensus on diagnosis and management of split cord malformation
Bo Xiu1,*, Fuyun Liu2, Aijia Shang3, Rui Zhang4
1 Department of Neurosurgery, The 7th Medical Center of PLA General Hospital, Beijing 100700, China
2 Department of Pediatric Orthopaedics, Zhengzhou University The 3rd Hospital, Zhengzhou 450052, Henan, China
3 Department of Neurosurgery, The 1st Medical Center of PLA General Hospital, Beijing 100853, China
4 Department of Neurosurgery, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Ji'nan 250021, Shandong, China
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Split cord malformation (SCM) is a neural tube defect that the spinal cord is longitudinally separated into two hemicords with individual functions, which causes severe spinal cord impairment and sensorimotor deficit. As a kind of myelodysplasia and a special type of tethered cord syndrome, SCM is not widely understood, and common issues in the diagnosis and treatment of SCM should be clarified. In this paper, the Chinese Split Cord Malformation Working Group made a consensus for SCM on embryopathogenesis and types, clinical presentations, neuroimaging assessment, indications and principle of the surgery, surgical techniques and nuances, and prognosis and follow up.

Key wordssplit cord malformation      tethered cord syndrome      neural tube defect      diagnosis      surgery      consensus     
Received: 26 April 2020      Published: 11 August 2020
Corresponding Authors: Bo Xiu   
Cite this article:

Bo Xiu, Fuyun Liu, Aijia Shang, Rui Zhang. Chinese expert consensus on diagnosis and management of split cord malformation. Journal of Neurorestoratology, 2020, 8: 83-92.

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1Nan BoShanghai Children's Medical Center
2Qian ChenShenzhen Children's Hospital
3Ruoping ChenXinhua Hospital Affiliated to School of Medicine, Shanghai Jiaotong University
4Shuo GuHainan Women and Children's Medical Center
5Xiaosheng HeXijing Hospital of Air-force Military Medical University
6Wen JinShanxi Children's Hospital
7Cuicui LiThe 7th Medical Center of PLA General Hospital
8Fangcheng LiGuangzhou Women and Children's Medical Center
9Hao LiFudan University Children's Hospital
10Fuyun LiuZhengzhou University the Third Hospital
11Wei LiuBeijing Tiantan Hospital, Capital Medical University
12Lin QiChildren's Hospital Affiliated to Zhengzhou University
13Xiang QiBeijing Children's Hospital, Capital Medical University
14Aijia ShangThe First Medical Center of PLA General Hospital
15Zhipeng ShenThe Children's Hospital , Zhejiang University
16Hangyu ShiXi'an Children's Hospital
17Benzhang TaoThe First Medical Center of PLA General Hospital
18Feng WanTongji Hospital Affiliated to Huazhong University of Science & Technology
19Guangyu WangQilu Children's Hospital of Shandong University
20Jvlei WangTangdu Hospital of Air-force Military Medical University
21Linlin WangInstitute of Reproductive and Child Health, Peking University Health Science Center
22Shuihua WuHunan Children's Hospital
23Bo XiuThe 7th Medical Center of PLA General Hospital
24Zhi YuanThe Second Hospital of Lanzhou University
25Xuan ZhaiThe Children's Hospital, Chongqing Medical University
26Rui ZhangShandong Provincial Hospital Affiliated to Shandong First Medical University
[1]   Saker E, Loukas M, Fisahn C, et al. Historical perspective of split cord malformations: A tale of two cords. Pediatr Neurosurg. 2017, 52(1): 1-5.
[2]   Babu R, Reynolds R, Moreno JR, et al. Concurrent split cord malformation and teratoma: Dysembryology, presentation, and treatment. J Clin Neurosci. 2014, 21(2): 212-6.
[3]   Bentley JF, Smith JR. Developmental posterior enteric remnants and spinal malformations: the split notochord syndrome. Arch Dis Child. 1960, 35: 76-86.
[4]   Pang D, Dias MS, Ahab-Barmada M. Split cord malformation: Part I: a unified theory of embryogenesis for double spinal cord malformations. Neurosurgery. 1992, 31(3): 451-480.
[5]   Luo YG, Li W, Dong CQ, et al. The clinical characteristics and surgical treatment of split cord malformation in children (in Chinese). J Clin Ped Sur. 2013, 12(1): 31-35.
[6]   Sinha S, Agarwal D, Mahapatra AK. Split cord malformations: an experience of 203 cases. Childs Nerv Syst. 2006, 22(1): 3-7.
[7]   Feng F, Tan HN, Li XY, et al. Radiographic characteristics in congenital scoliosis associated with split cord malformation: a retrospective study of 266 surgical cases. BMC Musculoskelet Disord. 2017, 18(1): 420.
[8]   Xiu B. Common problems and countermeasures in the diagnoses and treatment of split cord malformations (in Chinese). J Clin Pediatr Surg. 2019, 18(2): 84-87.
[9]   Lan BS, Wang KZ, Liu AQ, et al. Pathological factors and treatment of nerve injury of diastenatomyelia (in Chinese). J Clin Orth. 2002, 5(2): 94-97.
[10]   Meena RK, Doddamani RS, Gurjar HK, et al. Type 1.5 split cord malformations: An uncommon entity. World Neurosurg. 2020, 133: 142-149.
[11]   Salunke P, Kovai P, Malik V, et al. Mixed split cord malformation: are we missing something? Clin Neurol Neurosurg. 2011, 113(9): 774-778.
[12]   Mahapatra AK. Split cord malformation - A study of 300 cases at AIIMS 1990-2006. J Pediatr Neurosci. 2011, 6(): S41-S45.
[13]   Hu WM, Liu P, Liu FY,et al. Clinical analysis of 225 cases of split cord malformation in children (in Chinese). Chin J Pediatr Surg. 2018, 39(3): 204-208.
[14]   Xiu B. A practical classification of spinal neural tube defects and its clinical significance (in Chinese). Chin J Neurosurg Dis Res. 2017, 16(5): 393-396.
[15]   Pang D. Split cord malformation: Part II: Clinical syndrome. Neurosurgery. 1992, 31(3): 481-500.
[16]   Er?ahin Y. Split cord malformation types I and II: a personal series of 131 patients. Childs Nerv Syst. 2013, 29(9): 1515-1526.
[17]   Rokos J. Pathogenesis of diastematomyelia and spina bifida. J Pathol. 1975, 117(3): 155-161.
[18]   Guthkelch AN. Diastematomyelia with median septum. Brain. 1974, 97: 729-742.
[19]   Feng F, Shen JX, Zhang JG, et al. Characteristics and clinical relevance of the osseous spur in patients with congenital scoliosis and split spinal cord malformation. J Bone Jo Surg. 2016, 98(24): 2096-2102.
[20]   Heemskerk JL, Kruyt MC, Colo D, et al. Prevalence and risk factors for neural axis anomalies in idiopathic scoliosis: a systematic review. Spine J. 2018, 18(7): 1261-1271.
[21]   Cheng B, Li Y, Wang KZ, et al. Single tubular type of dia stematomyelia a retrospective study of 23 cases (in Chinese). Chin J Orthop. 2003, 23(5): 275-278.
[22]   Liu FY, Chen XL. Diagnosis and treatment of split spinal cord malformations combined with congenital spinal deformity (in Chinese). Chin J Appl Clin Pediatr. 2016, 31(11): 805-809.
[23]   Kline-Fath BM, Calvo-Garcia MA, O'Hara SM, et al. Water imaging (hydrography) in the fetus: The value of a heavily T2-weighted sequence. Pediatr Radiol. 2007, 37(2): 133-140.
[24]   Xiu B. Attention should be paid to standardizing the classification of spinal canal malformation (in Chinese). Natl Med J China. 2017, 97(48): 3761-3762.
[25]   Liu FY, Luo XF, Xia B, et al. Clinical study of congenital scoliosis with intraspinal deformity in children (in Chinese). Orthopedic J Chin. 2012, 20(9): 789-791.
[26]   Jin HM, Sun LP, Bao N, et al. Diagnosis and treatment of diastematomyeliain children (in Chinese). Chin J Pediastr Surg. 2003, 24(5): 430-432.
[27]   Shang AJ, Zhang YZ, Qiao GY, et al. Surgical treatment of split cord malformation (in Chinese). J Chin PLA Postgrad Med Sch. 2010, 31(4): 307-308, 311.
[28]   Pang D. Ventral tethering in split cord malformation. Neurosurg Focus. 2001, 10(1): e6.
[29]   Liu FY, Xia B, Si PC, et al. Management of split cord malformations associated with other deformities in children (in Chinese). Orthopedic J China. 2006. 14(11): 808-811.
[30]   Barutcuoglu M, Selcuki M, Selcuki D, et al. Cutting filum terminale is very important in split cord malformation cases to achieve total release. Childs Nerv Syst. 2015, 31(3): 425-432.
[31]   Barut?uo?lu M, Umur AS, ?zdemir S, et al. Double split cord malformations in a child: Types I and II at 2 different levels in the same route: Report of a rare case. Neurosurg Q. 2015, 25(4): 495-498.
[32]   Lee GYF, Paradiso G, Tator CH, et al. Surgical management of tethered cord syndrome in adults: Indications, techniques, and long-term outcomes in 60 patients. J Neurosurg Spine. 2006, 4: 123-131.
[33]   Ozturk E, Sonmez G, Mutlu H, et al. Split-cord malformation and accompanying anomalies. J De Neuroradiol. 2008, 35(3): 150-156.
[34]   Shen JX, Zhang JG, Feng F, et al. Corrective surgery for congenital scoliosis associated with split cord malformation: It may be safe to leave diastematomyelia untreated in patients with intact or stable neurological status. J Bone Joint Surg Am. 2016, 98(11): 926-936.
[35]   Hui H, Tao HR, Jiang XF, et al. Safety and efficacy of 1-stage surgical treatment of congenital spinal deformity associated with split spinal cord malformation. Spine. 2012, 37(25): 2104-2113.
[36]   Ayvaz M, Akalan N, Yazici M, et al. Is it necessary to operate all split cord malformations before corrective surgery for patients with congenital spinal deformities? Spine. 2009, 34(22): 2413-2418.
[37]   Cui ZQ, Xiu B, Sun ZX, et al. Microsurgical treatment of type I diastematomyelia in 75 children (in Chinese). Chin J Neurosurg. 2012, 28(7): 710-713.
[38]   Pediatric Neurosurgery Expert Committee of Neurosurgeon Branch of Chinese Medical Doctor Association. Common types of congenital spina bifida and expert consensus on surgical strategies (in Chinese). Chin J Neurosurg. 2016, 32(4): 331-335.
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