Case Of Split Cord Malformation Research Articles

Prenatally diagnosed fetal thoraco-lumbar spine duplication associated with lipomyelomeningocele: An extremely rare case of split cord malformation.

Spine duplication is considered rare, a more serious form of split cord malformation. Ultrasonographic evaluation of the spine in the second trimester is central to the antenatal diagnosis of spinal malformations. Here, we report a case of thoraco-lumbar spine duplication associated with lipomyelomeningocele diagnosed by ultrasonography at 19 weeks of gestation. To the best of our knowledge, this is the first case report of spine duplication diagnosed by antenatal ultrasonography.

PEDIATRIC SPINAL DYSRAPHISM ON MAGNETIC RESONANCE IMAGING: TRENDS IN A TERTIARY CARE HOSPITAL IN NORTH INDIA

Background: Spine and spinal cord development is governed by intricate genetic mechanisms and any deviation from the normal embryological cascade during 2-6 gestational weeks causes imperfect fusion of midline mesenchymal, bony and neural structures causing spinal dysraphism. Imaging plays a pivotal role in their evaluation as many patients present with concurrent anomalies that need to be corrected simultaneously to avoid repeat surgeries. To demonstrate the spectrum of MRI ndings of spinal dysraphi Aim: Settings and design: sm. A cross sectional observational study was done in 36 pediatric patients with known or suspected spinal dysraphism on 3T MRI. MRI was Materials And Methods: performed using Siemens Skyra 3T MRI. In this study comprising of 36 patients, mean age Results: was 37.1months (3 years 1month) with minimum age of 4 days and maximum age of 18 years. Children of age less than 5 years were maximally affected. A female predominance was noted with male: female ratio of 0.9:1. Open spinal dysraphism (55.6%) was more common than closed dysraphism (44.4%). The most common dysraphism was myelomeningocele. Among open dysraphism, we also encountered 1 case each of hemimyelomeningocele and myelocele. Among closed defects, split cord malformation was most common (19.4%) as it was present with other dysraphic defects (called complex spina bida), followed by lipomyelocele (11.1%) and dermal sinus (11.1%). Among 7 cases of split cord malformation, type II (6 cases; 85.7%) was more common than type I (1 case; 14.3%). Other cases of closed dysraphism were lipomyelomeningocele, meningocele, terminal myelocystocele, intradural lipoma, lar lipoma and caudal regression syndrome. MRI is an excelle Conclusion: nt modality for dening the characteristic imaging features of each dysraphism to give a composite diagnosis.

Split Cord Malformation: Our Experience

Background: Split cord malformation (SCM) is a rare form of neural tube defects. It belongs to closed type of neural tube defects. SCM constitutes around one-third of cases of spinal dysraphism. In SCM, there is a division in the spinal cord into two halves which may be equal or unequal.Objective: To analyse the clinical, radiological, intraoperative findings and surgical outcome of patients with SCM.Materials and Methods: This was a retrospective analysis of cases of SCM operated during 3-year period (2016–2019) in our institute. The data for the study were obtained from case records, operation notes, and radiology investigations.Results: There were 20 patients of spinal dysraphism in total, among which 6 cases were of SCM. All 6 cases were symptomatic with swelling in the back. Four had distal foot weakness, two had low backache, two had difficulty in walking, two had hypertrichosis, and one patient had foot deformity with trophic ulceration in the foot. Magnetic resonance imaging (MRI) revealed tethered cord in all 6 cases. Three had syringomyelia, three had lipomeningocele, and one case had intramedullary epidermoid. Computed tomography scan revealed bony spur in 4 cases. All patients underwent surgery.Conclusion: MRI is the investigation of choice and surgery is the treatment of choice. We have observed detethering done at the same sitting as that of the primary surgery has improved outcome. It also avoids the dilemma in attributing the postoperative neurological deficits if any, either to surgical complication or that due to tethered cord.

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.

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.

Split cord malformation in children undergoing neurosurgical intervetion in India: a descriptive study

To describe the clinical parameters, radiological findings and surgical outcome of 49 cases of split cord malformation (SCM). Forty nine patients of SCM were operated between June 1989 and June 2002, at Sanjay Gandhi postgraduate institute of medical sciences, Lucknow, India out of total 155 cases of spinal dysraphism. Detailed clinical evaluation with magnetic resonance imaging (MRI), preferably craniospinal, was main tool of investigation. All patients underwent excision of bony spur or fibrous septum, repair of associated myelomeningocele sac, when present, excision of associated spinal lesions and detethering of cord. Seven patients needed shunt operation. Patients having minimum 11⁄2 month of follow up were included in this analysis. Mean follow-up period was 3.4 years. Female to male ratio was 1.04: 1, with mean age of 4.8 years. The commonest cutaneous manifestation was hypertrichosis in 16 (32.7%) cases and among the neuro-orthopedic syndromes, most frequent were scoliosis in 16 (32.7%) and congenital talipes equino varus in 11 (22.4%) cases. The commonest clinical indicators were lower limb weakness, graded sensory loss, sphincter involvement, and autoamputation of toes and trophic ulcer in 24, 20, 14, two and four patients, respectively. Common imaging findings were low lying cord in 28, neural placode in 19, and SCM with and without meningomyelocele (MMC) sac in 20 and 29 cases, respectively. Common sites of occurrence of SCM were dorsal region in 19 (38.8%) and lumbar in 14 (28.6%) patients. Postoperative complications were cerebrospinal fluid leak, pseudomeningocele, wound infection, and meningitis in 12, eight, four and six patients, respectively. Two patients died in the postoperative period. On average follow up of 3.4 years, motor weakness improved in 10 children and remained static in 14, whereas sensory dysfunction improved in 13 and remained static in seven, and sphincteric function improved in 10 and was static in four cases. Backache dramatically relieved in both children and in all four patients, trophic ulcer completely healed. SCM cases may have association with MMC in significant number of patients hence craniospinal MRI remains the choice, as this will not only delineate complex spina bifida and cord anatomy, but also screen the entire neuraxis for other associated cranial and spinal anomalies. (J Pediatr Neurol 2004; 2(1): 21-27).

UNUSUAL PRESENTATION OF SPLIT CORD MALFORMATION TYPE I ASSOCIATED WITH MYELOMENINGOCELA

Objective – We report an unusual case of split cord malformation (SCM) associated with open spinal dysraphism and other anomalies of the central nervous system. Case report – A male newborn was admitted to the Pediatric Intensive Care Unit immediately after birth by Caesarean delivery. Clinical examination and diagnostics (MRI) showed open spinal dysraphism in the distal part of the spine (level L5 and S1), split cord malformation type I which separated two hemicords, tethered cord and syringomyelia. Two days after birth microneurosurgery was performed. Cranial and cervical MRI showed Chiari I and corpus callosum hypoplasia. There was no hydrocephalus. Preoperatively the patient had paraparesis and discretely moved his left foot. MRI showed a dilated bladder so he may have had urinary retention. The postoperative recovery was satisfactory. The patient did not have any additional neurological deficit. The patient was followed up by a neurosurgeon, pediatric surgeon, pediatrician and physiatrist. Control MRI scans showed significant regression of the syrinx and some ascensus of the medulla. About 18 months after operation the patient had discrete paresis of the left foot. Conclusion – Complex spina bifida is an extremely rare condition. In this paper we describe this interesting case of complex spina bifida: a split cord malformation characterized by atypical bony morphology with a dural fold into the bone septum.

Interesting Case Reports of Neurenteric Cyst with Type 1 Spilt Cord Malformation at Conus Medullaris

Two rare cases of split cord malformation with neurenteric cyst are presented. The clinical, radiological and surgical findings are presented with brief review of literature. Co-existent neurenteric cysts along with SCM type 1 have been described very rarely in the literature.

Prenatal diagnosis of split cord malformation by ultrasound and fetal magnetic resonance imaging: case report and review of the literature

Split cord malformation (SCM) is rare congenital disorder of spine in which bony, fibrous, or cartilaginous septum subdivides partially or completely the vertebral canal. SCM can be associated with other spinal abnormalities such as spina bifida, Arnold-Chiari malformation, hemivertebra, butterfly vertebra, or kyphoscoliosis and also can be seen as part of Jarcho-Levin syndrome. Prenatal diagnosis of SCM is possible by ultrasonography (US). Fetal magnetic resonance imaging (MRI) and amniotic fluid acetylcholine esterase (AF-AChE) levels can be helpful to rule out additional anomalies. We present a case of fetal SCM diagnosed by US and fetal MRI. An 18-year-old woman with no obstetrics risk factor was referred for routine US screening. At sagittal section, fetal spine was seen to be disordered. A coronal view of the spinal canal showed evidence of widening at the lower thoracic and lumbar level. An echogenic mass was identified within the enlarged spinal canal at level of T6-L5. Fetal MRI and AF-AChE analysis confirmed diagnosis and ruled out other anomalies and spina bifida. Neurological examination of the baby at 16 months of age was normal.

Split cord malformations: A two years experience at AIIMS

Background: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.Materials and Methods: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.Observations: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.Conclusion: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.

Split cord malformation - A study of 300 cases at AIIMS 1990-2006.

Background:Split cord malformation (SCM) is a rare condition. With decreasing incidence of neural tube defect (NTD) in the West, the reports of SCM are getting lesser and lesser. However, in India, spinal dysraphism is still a major problem encountered by the neurosurgeons.Objective:Our aim was to analyze 300 patients of SCM for their clinical features, radiological findings and outcome of surgery, which can throw light on the subject to others, who have less scope of finding these cases frequently.Materials and Methods:Over a 16-year period, we encountered 300 cases of SCM at AIIMS. Over the same period, more than 1500 cases of NTD were managed. SCM was noticed in 20% of cases with NTD. Skin stigmata were noted in two-third of the cases, and scoliosis and foot deformity were observed in 50% and 48% cases, respectively. Motor and sensory deficits were observed in 80% and 70% cases, respectively. Commonest site affected was lumbar or dorsolumbar (55% and 23%, respectively). In 3% cases, it was cervical in location. Magnetic resonance imaging (MRI) scan revealed a large number of anomalies like lipoma, neuroenteric cyst, thick filum and dermoid or epidermoid cysts. All the patients were surgically treated. In type I, bony spurs were excised, and in type II, bands tethering the cord were released. Associated anomalies were managed in the same sitting. Patients were followed up from 3 months to 3 years.Results:Overall improvement was noticed in 50% and stabilization in 44% cases and deterioration of neurological status was recorded in 6% cases. However, 50% of those who deteriorated improved to preop status prior to discharge, 7-10 days following surgery.Conclusions:SCM is rare and not many large series are available. We operated 300 cases and noticed a large number of associated anomalies and also multilevel and multisite splits. Improvement or stabilization was noted in 94% and deterioration in 6% cases. We recommended prophylactic surgery for our asymptomatic patients.

A case of split cord malformation associated with myeloschisis

Split cord malformation (SCM) associated with myeloschisis is a very rare form of spinal dysraphism. We encountered a case of SCM associated with myeloschisis showing split neural placodes (hemicords) in the upper lumbar region. Radiological examinations, including prenatal MRI and postnatal CT scan, clearly demonstrated a bony spur between the two hemicords as well as myeloschisis, which facilitated a precise preoperative diagnosis of this complex anomaly. Resection of the spur, closure of the myeloschisis, and untethering of the thickened filum terminale were successfully accomplished in one stage. The presence of SCM associated with myeloschisis is consistent with the hypothesis of an ontogenic basis of neural development, and we emphasize the importance of early imaging including prenatal MRI for diagnosing this complex anomaly.

Split cord malformations: a clinical study of 254 patients and a proposal for a new clinical—imaging classification

Split cord malformations (SCMs) are rare anomalies of the spinal cord. A total of 254 cases of SCM were treated surgically at the authors' center during a period of 16 years. Patients' demographic profiles, imaging studies, operative details, complications, and surgical outcomes were evaluated retrospectively. A new classification based on intraoperative findings is proposed. The mean age of the patients was 7.3 years (female/male 1.5:1). Type I SCM was seen in 156 patients (61.4%) and 98 patients (38.6%) had Type II SCM. Skin stigmata were present in 153 cases (60%); hypertrichosis, being the most common, was seen in 82 cases (32.3%). Asymmetrical lower-limb weakness and sphincter disturbances were present in 173 (68.1%) and 73 (33%) cases, respectively. Of the symptomatic cases, 39% (68 of 173) showed improvement in motor power, 57.9% (33 of 57) experienced sensory improvement, and 27.3% (20 of 73) regained continence. None of the 38 patients in the asymptomatic group had postoperative neurological deterioration. The neurological status was unchanged in 63% of the cases. A new subclassification of Type I SCM is proposed, based on intraoperative location of a bone spur causing the split, which may have a bearing on surgical dissection and outcome. Based on the authors' experience with 25 cases of Type I SCM, they have classified the disorder into four subtypes: Type Ia, bone spur located in the center with duplicated cord above and below the spur (12 cases); Type Ib, bone spur at the superior pole with no space above it (four cases); Type Ic, bone spur at the lower pole with large duplicated cord above (three cases); and Type Id, bone spur straddling the bifurcation with no space above or below the spur (six cases). The risk of injury to the hemicords is highest in the Id subtype (four of six patients in this group deteriorated neurologically in the present series, whereas none with subtypes Ia-c worsened). The authors present the largest series on SCMs so far reported in the world literature The risk of neurological deficits developing increases with age; hence, all patients with SCM should be surgically treated prophylactically even if they are asymptomatic. This new classification is easy to use and remember and takes into account the use of intraoperative findings that may have a bearing on surgical outcome.

Composite type of split cord malformation: two different types at three different levels

The authors report on a child with a composite type of split cord malformation (SCM). The patient presented with symptoms of a common cold. The diagnosis of SCM was made based on computerized tomography and magnetic resonance (MR) imaging of whole spinal axis. The SCM was Type I at T-4 and T-5 and Type II at T-12, according to the classification developed by Pang. The child underwent resection of the splitting lesions and terminal filum release. No case of composite-type SCM reported to date contains documentation of such a malformation. Because of the possible neurological and urological problems, the authors recommend MR imaging of the whole spine be performed during/an evaluation for SCM.

Diastématomyélie révélée chez l’adulte : étude de deux cas et revue de la littérature

Split cord malformation (SCM) is an uncommon developmental anomaly characterized a cleft spinal cord. In type I, each of the hemicords is contained within an individual dural tube whereas in type II there is a common dural tube housing both hemicords. Commonly diagnosed in childhood, adult presentation is exceptional. We report the case of two women whose type II SCM was discovered at the age of 40 and 54 years. The first patient complained of chronic lombar and radicular chronic pain with dysuria. Physical examination revealed a radicular syndrome with abolition of the left Achille reflex and a lombar hair tuft. MRI showed a disc herniation at the L5-S1 level, with a partial SCM at the level of the L2 vertebra, spina bifida and tethered cord. The second patient complained of lombar pain with perineal irradiation for 6 years. Physical examination showed a lombar cutaneous angioma. MRI revealed a thoraco-lombar SCM at the T12 to L1 level, with spina bifida. No spur could not be identified in either patient. No further surgical treatment was given. Based on these two observations, we propose a review of literature reporting 90 cases of adult SCM.

A split cord malformation with paresis of the unilateral lower limb: case report

BACKGROUND We report a rare case of split cord malformation. CASE DESCRIPTION A female neonate presented with split cord malformation (SCM) manifesting as right lower limb paresis. Myelomeningocele and meningocele were found in the lumbosacral region at birth. Magnetic resonance imaging (MRI) demonstrated division of the spinal cord into two hemicords below the T5 level. The right hemicord formed a hemimyelomeningocele and the left hemicord terminated in the low-lying conus. Three-dimensional computed tomography (3D CT) showed extensive vertebral body abnormalities. The hemimyelomeningocele was repaired 1 day after birth, and septectomy and the repair of the meningocele were performed when the patient was 1 year old. The patient has been followed up as an outpatient, and has residual right lower limb paresis. CONCLUSION SCM can be associated with multiple spinal abnormalities. MRI and 3D-CT are useful for identifying such abnormalities and planning the surgical treatment.

Split cord malformation and neurenteric cyst‘;case report and a theory of embryogenesis

A rare case of split cord malformation with neurenteric cyst is presented. The clinical, radiological and surgical findings are presented, and the possible embryological basis is discussed.To our knowledge, only seven such cases have been reported in the literature.

3D spiral CT imaging of bone anomalies in a case of diastematomyelia

The case of a 48-year-old woman, suffering from a diastematomyelia, is presented. This case and the diagnostic findings are used to demonstrate the demands on imaging methods with respect to a new classification of split cord malformations (SCMs) recently published. Although MRI is the method of choice for imaging of the spinal cord generally, only X-ray methods and especially conventional computer tomography provide the information necessary for correct classification of SCMs. Additional 3D-reconstructions from suitable CT-data are helpful in visualizing complex anomalies of bony structures found in most cases of SCM.

Split Cord Malformation

MUCH CONFUSION STILL exists concerning the pathological definitions and clinical significance of double spinal cord malformations. Traditional terms used to describe the two main forms of these rare malformations, diaste-matomyelia and diplomyelia, add to the confusion by their inconsistent usage, ambiguities, and implications of their dissimilar embryogenesis. Based on the detailed radiographic and surgical findings of 39 cases of double cord malformations and the autopsy data on two other cases, this study endorses a new classification for double cord malformations and proposes a unified theory of embryogenesis for all their variant forms and features. The new classification recommends the term split cord malformation (SCM) for all double spinal cords. A Type I SCM consists of two hemicords, each contained within its own dural tube and separated by a dura-sheathed rigid osseocartilaginous median septum. A Type II SCM consists of two hemicords housed in a single dural tube separated by a nonrigid, fibrous median septum. These two essential features necessary for typing, the state of the dural tube and the nature of the median septum, do not ever overlap between the two main forms and can always be demonstrated by imaging studies so that accurate preoperative typing is always possible. All other associated structures in SCM such as paramedian nerve roots, myelomeningoceles manqué, and centromedian vascular structures frequently do overlap between types and are not reliable typing criteria. The unified theory of embryogenesis proposes that all variant types of SCMs have a common embryogenetic mechanism. Basic to this mechanism is the formation of adhesions between ecto- and endoderm, leading to an accessory neurenteric canal around which condenses an endomesenchymal tract that bisects the developing notochord and causes formation of two hemineural plates. The altered state of the emerging split neural tube and the subsequent ontogenetic fates of the constituent components of the endomesenchymal tract ultimately determine the configuration and orientation of the hemicords, the nature of the median septum, the coexistence of various vascular, lipomatous, neural, and fibrous oddities within the median cleft, the high association with open myelodysplastic and cutaneous lesions, and the seemingly unlikely relationship with fore and midgut anomalies. The multiple facets of this theory are presented in increasing complexity against the background of known embryological facts and theories; the validity of each facet is tested by comparing structures and phenomena predicted by the facet with actual radiographic, surgical, and histopathological findings of these 41 cases of SCM.