Split Cord Malformation Research Articles

Is It Better to Resect a Bony Spur Before Corrective Surgery for Congenital Scoliosis with Type I Split Cord Malformation?

To compare bony spur resection (BR) with bony spur nonresection (BN) treatment in patients with congenital scoliosis and type I split cord malformation. Patients with congenital scoliosis and type I split cord malformation who underwent corrective surgery in a single institution from 2008 to 2015 were retrospectively evaluated. Patients were divided into 2 groups according to whether BR was performed or not: the BR group included 12 patients, and the BN group included 20 patients. Complications and perioperative and radiographic data were recorded and compared. The study included 32 patients. Mean age at surgery was 14.3 years, and average follow-up period was 2.8 years. There were no statistically significant differences in correction rate of the major curve or other radiographic parameters at final follow-up. Preoperative concerns (6.8 vs. 5.8, P= 0.006), operative times (508.5 minutes vs. 311.3 minutes, P= 0.009), blood loss (1433.3 mL vs. 1015.8 mL, P= 0.064), and hospital charges ($22,387.80 vs. $15,706.8, P= 0.032) were lower in the BN group than in the BR group. Total and major complication rates were higher in the BR group than in the BN group, even though there were no statistically significant differences (58.3% vs. 30.0% and 8.3% vs. 5.0%, respectively). In patients with congenital scoliosis and type I split cord malformation, not resecting the bony spur can achieve satisfactory radiographic and clinical outcomes with potentially fewer complications.

Cervicothoracic Spinal Dysraphism: Unravelling the Pandora's Box.

Introduction:Cervicothoracic spinal dysraphism (CTSD) is relatively rare with reported incidence of 1%–6.5%. The entity has a separate spectrum of associations with other anomaly such as split cord malformation, Chiari malformation, and corpus callosum agenesis as compared with its lumbosacral counterpart. In this study, we have highlighted the associated anomalies (AAs). To the best of our knowledge, this study is one of the largest series reported before.Materials and Methods:In this study, we included the patients with spinal dysraphism operated between December 2007 and December 2017 at the Department of Neurosurgery. All patients underwent neurological and radiological examinations followed by surgical excision of the sac and exploration of the intradural sac. Neurological, orthopedic, and urological abnormalities were analyzed in our retrospective description. The last available follow-up in hospital records was taken for outcome assessment.Results:Of 34 cases, 18 were women and 16 were men. Seven of nine patients in cervical group had AAs, whereas 22 of 25 patients in dorsal group had AA. In this study, we taken 6 patients with Chiari malformation, 4 with corpus callosum agenesis, 7 with hydrocephalus, and 16 with split cord malformation. The follow-up ranges from 1 year to 11 years with a mean of 59.45 months.Conclusion:The management strategy and association with other congenital anomalies separate CTSD as different clinical entity as compared with their lumbosacral counterparts. These patients show relatively favorable outcome with regard to neurological, orthopedic, and urological symptoms. Early surgical intervention after proper radiological and clinical evaluation is recommended.

Microsurgical efficacy in 326 children with tethered cord syndrome: a retrospective analysis.

Tethered cord syndrome is a progressive disease with a typically insidious onset in infants and children, and which can lead to persistent progress of neurological deficits and a high rate of disability without timely intervention. The purpose of this study was to investigate the curative effect of microsurgery in children with different types of tethered cord syndrome. In this study, we analyzed 326 patients with tethered cord syndrome, aged from 2 months to 14 years old, who were followed for 3-36 months after microscopic surgery. Based on clinical manifestations and imaging findings, these patients were classified into five types: tight filum terminale (53 cases), lipomyelomeningocele (55 cases), lipomatous malformation (124 cases), postoperative adhesions (56 cases), and split cord malformation (38 cases). All patients underwent microsurgery. Curative effects were measured before and 3 months after surgery by Spina Bifida Neurological Scale based on sensory and motor functions, reflexes, and bladder and bowel function. The results showed that Spina Bifida Neurological Scale scores improved in all five types after surgery. Overall effective rates in these patients were 75%. Effective rates were 91% in tight filum terminale, 84% in lipomyelomeningocele, 65% in lipomatous malformation, 75% in postoperative adhesion, and 79% in split cord malformation. Binary logistic regression analysis revealed that types of tethered cord syndrome (lipoma-type or not) and symptom duration before surgery were independent influencing factors of surgical outcome. These results show that therapeutic effect is markedly different in patients with different types of tethered cord syndrome. Suitable clinical classification for tethered cord syndrome will be helpful in predicting prognosis and guiding treatment. This trial has been registered in the Chinese Clinical Trial Registry (registration number: ChiCTR1800016464).

Split cord malformations: from the surgical viewpoint

Split cord malformations: from the surgical viewpoint

Split-Cord Malformation of the Cervical Spinal Cord Following Dorsal Root Entry Zone Lesioning Surgery

The authors report a rare case of a 54-year-old man who developed an asymptomatic cleft within the cervical spinal cord at C7-T1 following Dorsal Root Entry Zone (DREZ) lesioning surgery. The patient presented to the authors’ institution with neuropathic pain from a left brachial plexopathy resulting from a lower trunk avulsion. The initial MRI at 6 months post-injury confirmed preganglionic avulsions of the left C7, C8 and T1 nerve roots. Of note, there was a prominent anterior median fissure at the C7-T1 spinal level. He underwent a DREZ lesion 8 months after the injury. Subsequent MRI studies showed a progressive near complete split within the cervical cord at C7- T1. The patient did not develop any new neurological deficits. It remains unclear whether this observation was a sequela of the root avulsion or the DREZ surgery.

Spinal Imaging Findings of Open Spinal Dysraphisms on Fetal and Postnatal MRI.

Fetal MRI has become a valuable tool in the evaluation of open spinal dysraphisms making studies comparing prenatal and postnatal MRI findings increasingly important. Our aim was to determine the accuracy of predicting the level of the spinal dysraphic defect of open spinal dysraphisms on fetal MR imaging and to report additional findings observed when comparing fetal and postnatal MR imaging of the spine in this population. A single-center retrospective analysis was performed of fetal MRIs with open spinal dysraphisms from 2004 through 2016 with available diagnostic postnatal spine MR imaging. Images were reviewed by 2 board-certified fellowship-trained pediatric neuroradiologists. Corresponding clinical/operative reports were reviewed. One hundred nineteen fetal MRIs of open spinal dysraphisms were included. The level of the osseous defect between fetal and postnatal MR imaging was concordant in 42.9% (51/119) of cases and was 1 level different in 39% (47/119) of cases. On postnatal MR imaging, type II split cord malformation was seen in 8.4% (10/119) of cases, with only 50% (5/10) of these cases identified prospectively on fetal MR imaging. Syrinx was noted in 3% (4/119) of prenatal studies, all cervical, all confirmed on postnatal MR imaging. Fetal MR imaging is accurate in detecting the level of the spinal dysraphic defect, which has an impact on prenatal counseling, neurologic outcomes, and eligibility for fetal surgery. In addition, fetal MR imaging is limited in its ability to detect split cord malformations in patients with open spinal dysraphisms. Although rare, fetal MR imaging has a high specificity for detection of cervical spinal cord syrinx.

A Case of Split Cord Malformation with Triple Bony Spur in a Single Dural Sleeve—A Classification Dilemma

AbstractSplit cord malformation-I (SCM-I) is characterized by the presence of double dural sacs, with rigid extradural bony/cartilaginous spur leading to symmetrical or asymmetrical division of the cord. In split cord malformation-II (SCM-II), there is a single dural sac with a nonrigid fibrous spur and symmetrical division of the cord. SCM-II are slightly more common than SCM-I, constituting around 50 to 60% of SCMs. The authors report a unique case of SCM with triple bony spurs lying both intra- and extradurally in a single dural sleeve. At the time of submission of this report, to the best of authors’ knowledge, no case of SCM with triple bony spurs in single dural sleeve has been reported anywhere in the world literature.

Adult Versus Pediatric Tethered Cord Syndrome: Clinicoradiological Differences and its Management.

Background:Dysraphic lesions in adults, presenting clinically as tethered cord syndrome (TCS), are relatively rare, and their optimal management remains controversial.Patients and Methods:We performed a retrospective analysis of our pediatric database over a period of last 7 years to focus on the adult TCS. Our aim was to determine the clinicoradiological and etiopathological differences between adult and pediatric patients as well as to determine the results of surgery in adult TCS.Results:Adult spinal dysraphisms constituted 15.4% of our patients (20 out of 130). Motor weakness, sphincteric dysfunction, and backache (n = 13, 65.0% each) predominated in adults unlike children who presented with subcutaneous swellings (n = 74, 67.6%) followed by motor weakness (n = 40, 46.4%), backache being reported by only three patients. The different pathologic substrates underlying adult dysraphisms were lipomeningocele (n = 8), split cord malformation (total = 7; Type 1: n = 5; Type 2: n = 2), dermal sinus (n = 2), and fatty filum (n = 3). On the other hand, meningomyelocele/meningocele (61, 54.9%) followed by split cord malformation Type 1 and 2 (n = 29, 26.1%) predominated in children. The radiological differences between the two groups were a higher incidence of vertebral body defects (hemivertebrae and butterfly vertebrae) and lack of intracranial anomalies in adults. At a mean follow-up of 20.5 months, the most common symptoms to improve following detethering were pain (11 out of 13, 84.6%) followed by motor weakness (six out of 13, 56.2%) and sphincteric control (7 out of 13, 53.8%).Conclusion:Most common symptoms to improve following detethering in adult TCS were pain followed by motor weakness. The major radiological differences between these two groups were a higher incidence of vertebral body defects (hemivertebrae and butterfly vertebrae) and lack of intracranial anomalies in adults.

Correction to: Management of split cordmalformation in children: the Lyon experience.

The original version of this article unfortunately contained an error. The authors have been published with inverted given and family names. Given in this article are the corrected author names.

Surgical management of adult type 1 split cord malformation. Report of two cases with literature review

Split cord malformation (SCM) is a rare form of spinal dysraphism wherein the spinal cord is divided longitudinally into two distinct hemicords. Surgery is usually performed in children while management in adults, who rarely manifest symptoms, remains controversial. Both expectant management and prophylactic surgery have been variously advocated. The present article describes our experience in two adult patients with predominant pain-related complaints who underwent surgical excision of type 1 SCM lesions. A comprehensive review of the literature on SCM in adults is also provided. While pain, disability, and quality of life scores improved in these two patients, further larger studies will be required to define the role of surgery in adults with type 1 SCM and a pain-dominant presentation.

Management of split cord malformation in children: the Lyon experience.

Split cord malformation (SCM) is a rare congenital spinal abnormality. Clinical presentation varies. Other congenital defects can be associated. Management is surgical. We retrospectively reviewed all our SCM cases and reported our experience for its management. From 1990 to 2014, 37 patients were operated. Five situations lead to the diagnosis (orthopedic disorders (n = 8), orthopedic and neurological disorders (n = 16), pure neurological disorders (n = 5), no symptoms except cutaneous signs (n = 7), antenatal diagnosis (n = 1)). Scoliosis was the most common associated condition. The level of the spur was always under T7 except in one case. There were more type I (n = 22) than type II (n = 15) SCM. Patients with preoperative neurological symptoms (n = 21) were improved in 71.4%. Five out of nine patients that had preoperative bladder dysfunction were improved. Eleven patients needed surgical correction of the scoliosis. For us, the surgical procedure is mandatory even in case of asymptomatic discovery in order to avoid late clinical deterioration. In any case, the filum terminale need to be cut in order to untether completely the spinal cord. In case a surgical correction of a spinal deformity is needed, we recommend a two-stage surgery, for both SCM type. The SCM surgery can stop the evolution of the scoliosis and it may just need an orthopedic treatment with a brace.

Missing Links Between Genetically Inherited Molecules in Split Cord Malformation and Other Anomaly: A Bench to Bedside Approach.

Aim:Split cord malformation (SCM) is associated with extensive vertebral fusions (Klippel–Feil anomaly). In light of previous embryological theories and recent research findings, we attempt to document the origin of split cord, and vertebral fusions involvement of spectrum of genes is necessary to know better the etiopathogenesis of SCM and its associated diseases.Materials and Methods:We used the various databases such as PubMed/MEDLINE, Cochrane Review, Hinari, and Google Scholar for the recently published medical literature. The women had been living and still born infants had SCM. The relative risk (RR) and possible molecular mechanism are described details of major genes and its variants in details. Although molecular genetics involvement including with recent advances of study add an evidence of both Mendelian and Non-Mendelian fashion is discussed with all genetic components. We mentioned our earlier experience and responsibility of SCM and its associated diseases.Results:Although different mechanisms are suggested for the development of SCM observed in our experience, there is a midline lesion bisecting the neuroepithelium and the notochordal plate, which is responsible for complete splitting of the cervical cord with anterior bony defect. The localized disturbance of cervical neural tube closure accounts for SCM with partial dorsal splitting of the cord with posterior vertebral defect and associated diseases.Conclusions:According to the best of our knowledge, this report is the first one to be documented by wider spectrum of variants from (experimental studies to human subject). This add a complex interaction of mutant variants drive toward an additional second-hit alterations for the SCM. The up-to-date information, documented in proper order, derived the bench-to-bedside approach to overcome this burden of SCM, which is globally noticed with other additional diseases.

Type 1 Split Cord Malformation: The Significance of the 3D Orientation of the Bony Spur, Its Clinical Relevance, and Surgical Nuances.

Background:Split cord malformation (SCM) remains an important cause of tethered cord syndrome. SCM type 1, wherein a bony spur separates the two dural sacs, represents a challenging condition to treat. The management can be further complicated by the other associated dysraphic states in the same patient. The bony spur at the level of split cord has been variably classified in the literature. There is, however, a paucity of literature dealing with the three-dimensional disposition of the bony spur and its clinical and operative implications.Materials and Methods:Our study included 50 patients (male/female = 24:26) of SCM type 1. The orientation of bony spur was categorized into ventral complete, ventral incomplete, and dorsal types along with simultaneous assessment of their direction in sagittal and axial planes in multiplanar computed tomography (CT) scan. Surgical plan was individualized according to each patient’s morbid anatomy.Results:The most common site of bony spur was lumbar region (n = 24). Twenty-four (48%) patients had ventral complete type spur, 19 (38%) had ventral incomplete type, and 7 (14%) had dorsal bony spur. Dorsal bony spurs presented with a greater incidence of motor symptoms (71.4%) and bladder disturbances (28.5%), whereas the ventral incomplete type of bony spur was predominantly associated with sensory symptoms (42.1%). Patients with dorsal bony spurs showed better postoperative outcomes when compared to their ventral counterparts.Conclusion:Multiplanar assessment of the bony spur using CT scan helps in surgical drilling and improves the outcome. The dorsal SCM variety showed better clinical outcomes when compared to their ventral bony spurs.

Cystic Cervical Dysraphism: Experience of 12 Cases.

Introduction:Cystic spinal dysraphism of the cervical region is a relatively rare entity, which is more frequently associated with congenital anomalies such as split cord malformation, Chiari malformation, and corpus callosum agenesis, when compared to their lumbosacral counterpart. In our study, we have highlighted the clinical spectrum, associated anomalies (both neural and extra-neural), and surgical nuances of these.Materials and Methods:This study is a retrospective analysis of 225 patients from June 2010 to April 2017. Twelve patients who were between the age of 1 month and 16 years were included in our study. Average age was 32.6 months, and there were five female patients and seven male patients. All patients underwent neurological and radiological examinations followed by surgical excision of the sac and exploration of the intradural sac using the standard microsurgical technique. Neurological, Orthopedic and urological outcomes were studied in our description.Results:Of the 12 cases, 9 patients (75%) had some associated anomaly. Four of the 12 patients (25%) had split cord malformation, 3 had corpus callosum agenesis, and 5 had Chiari malformation. Patients with cervical spina bifida cystica (SBC) present with less neurologic deficits and greater association with CCA.Conclusion:The management strategy and association with other congenital anomalies separates cervical dysraphism as a different clinical entity rather than just group. These patients rather show favorable outcome with regard to neurologic, orthopedic, and urologic problems as compared to their caudal counterpart. Early surgical intervention even before the onset of symptoms is recommended. A proper radiological and urological evaluation is warranted.

Fatty filum terminale (FFT) as a secondary tethering element in children with closed spinal dysraphism.

The purpose of this study was to assess the prevalence of FFT as an additional tethering element in children operated for closed spinal dysraphism, where FFT was not the primary tethering pathology. This is a retrospective study of 195 children (< 18years of age) who underwent surgery for closed spinal dysraphism and did not have FFT as the primary diagnosis. All patients were operated during the period 2005-2017 by a single surgeon. The commonest diagnosis was a lipomyelomeningocele (LMMC, n = 81, 41.5%), followed by split cord malformations (SCM, types I and II, n = 61, 31.3%), dermal sinus (n = 28, 14.4%), and dermoid cyst (n = 10, 5.1%). Factors such as age and sex, presenting symptoms, intraoperative findings, and radiological presence of a FFT on a magnetic resonance imaging (MRI) were documented, and the relationship between the primary diagnoses and presence of FFT was analyzed. FFT as a secondary finding was seen in 63 patients (32.3%). The mean age of the cohort was 54months (4.5years) and the sex distribution was relatively even (51.8% girls). The commonest symptom at presentation was a swelling in the back, followed by lower limb weakness. The mean duration of symptoms was nearly 30months. FFT was seen on the MRI and confirmed intraoperatively in 55 patients (28.2%). There were 8 patients (4.1%) where a FFT was seen intraoperatively, but was not diagnosed on the preoperative MRI. In 16 patients, FFT was seen > 2 segments away from the primary tethering pathology, 8 of which mandated a second skin incision for sectioning of the FFT. Secondary FFT was most commonly associated with a SCM (types I and II combined) and was seen in 42.6% of those patients. It was least commonly associated with intradural dermoid cysts. The presence of a secondary FFT should be considered and actively sought on preoperative thin-slice T1W axial MR images in the sacral region in all patients with spinal dysraphism. Even if a FFT is not seen on preoperative MR images, the filum should be explored and sectioned if it is in the vicinity of the primary surgical field, especially in patients with SCM.

Radiographic characteristics in congenital scoliosis associated with split cord malformation: a retrospective study of 266 surgical cases

BackgroundVertebrae, ribs, and spinal cord are anatomically adjacent structures, and their close relationships are clinically important for planning better corrective surgical approach. The objective is to identify the radiographic characteristics in surgical patients with congenital scoliosis (CS) and coexisting split cord malformation (SCM).MethodsA total of 266 patients with CS and SCM underwent surgical treatment at our hospital between May 2000 and December 2015 was retrospectively identified. The demographic distribution and radiographic data were collected to investigate the characteristics of spine curve, vertebral, rib, and intraspinal anomalies. According to Pang’s classification, all patients were divided into two groups: type I group is defined as two hemicords, each within a separate dural tube separated by a bony or cartilaginous medial spur, while type II group is defined as two hemicords within a single dural tube separated by a nonrigid fibrous septum.ResultsThere were 104 patients (39.1%) in Type I group and 162 patients (60.9%) in Type II group. SCM was most commonly found in the lower thoracic and lumbar regions. The mean length of the septum in Type I SCM was significantly shorter than Type II SCM (2.7 vs. 5.2 segments). Patients in Type I group had a higher proportion of kyphotic deformity (22.1%). The vertebral deformities were simple in only 16.5% and multiple in 83.5% of 266 cases. Patients in Type I group presented higher prevalence of multiple (90.4%) and extensive (5.1 segments) malformation of vertebrae. In addition, hypertrophic lamina and bulbous spinous processes were more frequent in Type I group (29.7%), even developing into the “volcano-shape” deformities. Rib anomalies occurred in 62.8% of all patients and 46.1% of them were complex anomalies. The overall prevalence of other intraspinal anomalies was 42.9%. The most common coexisting intraspinal anomalies was syringomyelia (30.5%).ConclusionThe current study, with the largest cohort to date, demonstrated that patients with CS and coexisting SCM presented high prevalence of multiple vertebral deformities, rib and intraspinal anomalies. The length of the split segment in Type I SCM was shorter than that in Type II SCM. Compared with Type II SCM, patients with Type I SCM presented with higher incidence of kyphotic deformity, more extensive and complicated vertebral anomalies, and more complex rib anomalies.

Benefit of Intraoperative Neurophysiological Monitoring in a Pediatric Patient with Spinal Dysmorphism, Split Cord Malformation, and Scoliosis

ABSTRACTIntraoperative neurophysiological monitoring (IONM) consists of a group of neurodiagnostic techniques that assess the nervous system’s functional integrity during surgical operations. A retrospective analysis of a pediatric female patient was conducted who underwent 12 operations for the correction of scoliosis, tethered cord, and split spinal cord wherein IONM played an important role. From age 3 to 6, she underwent six procedures including a release of the tethered cord, resection of the filum terminale, removal of a T11–T12 bony spur, release of L3 adhesions, repair of subcutaneous meningocele, and correction of scoliosis with a vertical expandable prosthetic titanium rod (VEPTR) technique without the use of IONM. However, a multimodality IONM protocol with somatosensory evoked potentials, transcranial electrical motor evoked potentials (TCeMEP), and an electromyogram was utilized during the later procedures. At age 6 (the seventh procedure), a VEPTR expansion was performed, with loss and recovery of the lower extremity motor evoked potentials. The postoperative magnetic resonance imaging (MRI) showed a partial split cord malformation with retethering of the spinal cord. We repaired her split cord malformation and tethered cord while employing IONM. Using IONM for her operation was crucial because a sudden significant loss of TCeMEP resulted in a cancellation of the procedure; the MRI showed a thick remnant attached to the spinal cord. If the procedure was performed without IONM, we could have missed the underlying pathology, an error that may have resulted in paraplegia. We strongly recommend using IONM during high-risk surgical procedures to help significantly reduce the risk of permanent postoperative complications.

P116 - Safety and Efficacy of Osteotomy for Congenital Spinal Deformity Associated with Split Spinal Cord Malformation

PURPOSE: The aim of this study is to retrospectively evaluate the safety and efficacy of osteotomy for 56 consecutive patients, who had progressive congenital spinal deformity (CSD) associated with split spinal cord malformation (SSCM).

Split Cord Malformation - A Diagnosis Missed

In this report, we describe the case of an 8 year old female child who presented with complaints of swelling in sacral region since birth and left leg weakness since 2 years of age. She presented with left clubbed foot to an orthopaedic surgeon and was treated at 1 year of age with serial plasters. The child was presented for club foot, rather than identifying the cause of deformity. When child was presented to our tertiary care hospital her workup was done and an incidental finding of split cord malformation on Magnetic Resonance Imaging (MRI) and Computed Tomography (CT) myelogram was revealed along with left gluteal sebaceous cyst. The child underwent surgery and tethering of cord by the fibrous septa was relieved along with excision of left gluteal sebaceous cyst. The post-operative course was uneventful. Spinal cord malformations usually present with number of presentations for example hypertrichosis, lipoma, dermal sinus or tail. Therefore, a thorough examination can result in early diagnosis of the malformation and the complications can be prevented. In our case hypertrichosis at the back was missed, prior to child being referred to us. Hence, this case is being reported as a diagnosis missed of split cord malformation due to lack of complete head to toe evaluation of child by a subspecialty expert.

Surgical management of 142 cases of split cord malformations associated with osseous divide

ObjectivesTo investigate the key surgical points in treating split cord malformations associated with osseous divide and scoliosis (SCM-OD-S). Materials and methodsThe surgical options and methods of a total of 142 SCM-OD-S cases were retrospectively analyzed, and the surgical precautions and imaging diagnosis were also discussed. ResultsThe 142 patients were performed osseous divide resection plus dural sac molding, which achieved good results and no serious complication such as spinal cord and nerve injury occurred; certain symptoms such as urination-defecation disorders, muscle strength subsidence, Pes Cavus, and toe movement disorder in partial patients achieved various degrees of relief, and it also created good conditions for next-step treatment against scoliosis. ConclusionsThe diagnosis of SCM-OD mainly depended on imaging inspection, routine magnetic resonance imaging (MRI) combined with computed tomography (CT) 3D reconstruction, which can comprehensively evaluate the types and features of diastematomyelia as well as other concomitant diseases. SCM alone needed no treatment, but surgery will be the only means of treating SCM-OD. Intraoperatively removing osseous divide step-by-step, as well as carefully freeing the spinal cord and remodeling the dural sac, can lay good foundations for relieving tethered cord, improving neurological symptoms, and further scoliosis orthomorphia, thus particularly exhibiting importance for the growth and development of adolescents.