Spinal development and spinal dysraphism

2019 ◽  
pp. 983-992
Author(s):  
Dominic Thompson
Keyword(s):  
Spinal Cord ◽  
Spinal Dysraphism ◽  
Radical Resection ◽  
Management Plan ◽  
Congenital Disorders ◽  
Spinal Cord Development ◽  
Precise Diagnosis ◽  
Over Time ◽  
Spinal Cord Tethering

The term spinal dysraphism encompasses a group of congenital disorders of spinal cord development. This potentially confusing array of conditions is best understood from an embryological perspective, and a unifying method of classification is presented. Spinal dysraphism is associated with neurological, urological, and orthopaedic deficits, these may be present at birth or may evolve over time due to the effects of spinal cord tethering. Precise diagnosis is essential to formulating an appropriate surgical management plan in order to optimize long-term neurological outcome. Contemporary and controversial surgical advances in the field are discussed including electrophysiology directed radical resection for spinal lipomas and antenatal surgery for myelomeningocele.

scholarly journals Long term outcome of functional independence and quality of life after traumatic SCI in Germany

Spinal Cord ◽  
2021 ◽  
Author(s):  
Florian Möller ◽  
Rüdiger Rupp ◽  
Norbert Weidner ◽  
Christoph Gutenbrunner ◽  
Yorck B. Kalke ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Spinal Cord ◽  
Spinal Cord Injury ◽  
Functional Independence ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Cord Injury ◽  
Over Time

Abstract Study design Multicenter observational study. Objective To describe the long-term outcome of functional independence and quality of life (QoL) for individuals with traumatic and ischemic SCI beyond the first year after injury. Setting A multicenter study in Germany. Methods Participants of the European multicenter study about spinal cord injury (EMSCI) of three German SCI centers were included and followed over time by the German spinal cord injury cohort study (GerSCI). Individuals’ most recent spinal cord independence measure (SCIM) scores assessed by a clinician were followed up by a self-report (SCIM-SR) and correlated to selected items of the WHO short survey of quality of life (WHO-QoL-BREF). Results Data for 359 individuals were obtained. The average time passed the last clinical SCIM examination was 81.47 (SD 51.70) months. In total, 187 of the 359 received questionnaires contained a completely evaluable SCIM-SR. SCIM scores remained stable with the exception of reported management of bladder and bowel resulting in a slight decrease of SCIM-SR of −2.45 points (SD 16.81). SCIM-SR scores showed a significant correlation with the selected items of the WHO-QoL-BREF (p < 0.01) with moderate to strong influence. Conclusion SCIM score stability over time suggests a successful transfer of acquired independence skills obtained during primary rehabilitation into the community setting paralleled by positively related QoL measurements but bladder and bowel management may need special attention.

Posttraumatic spinal cord tethering and syringomyelia: surgical treatment and long-term outcome

2009 ◽  
Vol 11 (4) ◽  
pp. 445-460 ◽  
Author(s):  
Scott P. Falci ◽  
Charlotte Indeck ◽  
Daniel P. Lammertse
Keyword(s):  
Spinal Cord ◽  
Spinal Injury ◽  
Treatment Center ◽  
Term Outcome ◽  
Repeat Surgery ◽  
Long Term Outcome ◽  
Progressive Myelopathy ◽  
Cord Injury ◽  
Spinal Cord Tethering

Object Permanent neurological loss after spinal cord injury (SCI) is a well-known phenomenon. There has also been a growing recognition and improved understanding of the pathophysiological mechanisms of late progressive neurological loss, which may occur after SCI as a result of posttraumatic spinal cord tethering (SCT), myelomalacia, and syringomyelia. A clinical study of 404 patients sustaining traumatic SCIs and undergoing surgery to arrest a progressive myelopathy caused by SCT, with or without progressive myelomalacia and cystic cavitation (syringomyelia) was undertaken. Both objective and subjective long-term outcomes were evaluated. To the authors' knowledge, this is the first series of this size correlating long-term patient perception of outcome with long-term objective outcome analyses. Methods During the period from January 1993 to November 2003, 404 patients who had previously sustained traumatic SCIs underwent 468 surgeries for progressive myelopathies attributed to tethering of the spinal cord to the surrounding spinal canal, with or without myelomalacia and syrinx formation. Forty-two patients were excluded because of additional pathological entities that were known to contribute to a progressive myelopathy. All surgeries were performed by the same neurosurgeon at a single SCI treatment center and by using a consistent surgical technique of spinal cord detethering, expansion duraplasty, and when indicated, cyst shunting. Results Outcome data were collected up to 12 years postoperatively. Comparisons of pre- and postoperative American Spinal Injury Association sensory and motor index scores showed no significant change when only a single surgery was required (86% of patients). An outcome questionnaire and phone interview resulted in > 90% of patients self-assessing arrest of functional loss; > 50% of patients self-assessing improvement of function; 17 and 18% self-assessing improvement of motor and sensory functions to a point greater than that achieved at any time postinjury, respectively; 59% reporting improvement of spasticity; and 77% reporting improvement of hyperhidrosis. Conclusions Surgery for spinal cord detethering, expansion duraplasty, and when indicated, cyst shunting, is a successful treatment strategy for arresting a progressive myelopathy related to posttraumatic SCT and syringomyelia. Results suggest that surgery leads to functional return in ~ 50% of patients, and that in some patients posttraumatic SCT limits maximal recovery of spinal cord function postinjury. A patient's perception of surgery's failure to arrest the progressive myelopathy corresponds closely with the need for repeat surgery because of retethering, cyst reexpansion, and pseudomeningocele formation.

Long-term spinal cord injury: Functional changes over time

1993 ◽  
Vol 74 (10) ◽  
pp. 1030-1034 ◽  
Author(s):  
Kenneth A. Gerhart ◽  
Ebba Bergstrom ◽  
Susan W. Charlifue ◽  
Robert R. Menter ◽  
Gale G. Whiteneck
Keyword(s):  
Spinal Cord ◽  
Spinal Cord Injury ◽  
Functional Changes ◽  
Cord Injury ◽  
Changes Over Time ◽  
Over Time

scholarly journals Infected congenital cervical dermal sinuses leading to spinal cord abscess: two case reports and a review of the literature

2020 ◽  
Vol 37 (1) ◽  
pp. 225-228
Author(s):  
Rhian Bevan ◽  
Paul Leach
Keyword(s):  
Spinal Cord ◽  
Case Reports ◽  
Spinal Dysraphism ◽  
Febrile Illness ◽  
Surgical Excision ◽  
Late Recurrence ◽  
Cervical Region ◽  
Developmental Defects ◽  
Complete Surgical Excision

Abstract Purpose Congenital dermal sinuses are a rare form of spinal dysraphism. The developmental defects are located along the midline neuroaxis, with sinuses in the cervical region being the least common. Congenital dermal sinuses can be associated with intraspinal infection as they act as a direct route from the skin and subcutaneous tissues into the spinal cord. Methods The authors present two cases of cervical dermal sinuses complicated by intramedullary abscess. Both children presented with neurological decline and febrile illness. MRI showed intraspinal abscess. Both underwent prompt surgical excision of the sinus tract, exploration of the cord and intravenous antibiotics. Results Both patients demonstrated excellent neurological recovery. Conclusions Complete surgical excision of the sinus and tract in addition to long-term antimicrobials can yield excellent neurological outcomes. At surgery, do not expect to find pus when exploring the intramedullary component. Long-term follow-up is advocated due to potential late recurrence.

Diffusion tensor imaging in the evaluation of the long-term efficacy of HBO2 therapy in rats after traumatic spinal cord injury

2020 ◽  
pp. 435-443
Author(s):  
Yijing Zhao ◽  
◽  
Yingyan Zheng ◽  
Zebin Xiao ◽  
Jianyi iu ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Spinal Cord Injury ◽  
Diffusion Tensor Imaging ◽  
Diffusion Tensor ◽  
Traumatic Spinal Cord Injury ◽  
Term Efficacy ◽  
Cord Injury ◽  
Hbo2 Therapy ◽  
Over Time

Objective: This study explored the feasibility of diffusion tensor imaging (DTI) in the evaluation of the long-term efficacy of hyperbaric oxygen (HBO2) therapy in rats after traumatic spinal cord injury (TSCI) with different degrees of injury. Method: Adult Sprague-Dawley rats (total n = 60) were randomly separated into three groups of mild, moderate and severe TSCI (20 rats per group). Each group was then randomly divided into TSCI and TSCI+HBO2 subgroups (10 rats per subgroup). Basso Beattie and Bresnahan (BBB) scores and DTI parameters including fractional anisotropy (FA), mean apparent diffusivity (MD), radial diffusivity (RD) and axial diffusivity (AD) were collected at pre-TSCI and at 0, six and 24 hours, and three, seven, 14, 21, 28 and 56 days post-TSCI. Two-way repeated measures analysis of variance was used for comparison between the TSCI and TSCI+HBO2 subgroups over time in the mild, moderate and severe TSCI groups. Pearson correlation analysis was applied to analyze the correlations between BBB scores and DTI parameters. Results: BBB scores, FA, MD and RD values showed significant differences between the TSCI and TSCI+HBO2 subgroups over time in the mild, moderate and severe TSCI groups (all p<0.01). FA, MD and RD values were positively correlated with BBB scores in all TSCI and TSCI+HBO2 subgroups (all p<0.05). Conclusions: DTI parameters, especially MD, could quantifiably assess the long-term efficacy of HBO2 therapy and reflect the functional recovery in rats after TSCI with different degrees of injury.

scholarly journals Syringomyelia in children with closed spinal dysraphism: long-term outcomes after surgical intervention

2020 ◽  
Vol 25 (3) ◽  
pp. 319-325 ◽  
Author(s):  
Amy K. Bruzek ◽  
Jordan Starr ◽  
Hugh J. L. Garton ◽  
Karin M. Muraszko ◽  
Cormac O. Maher ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Clinical Symptoms ◽  
Spinal Dysraphism ◽  
Tethered Cord ◽  
Presenting Symptoms ◽  
Age At Surgery ◽  
Intradural Lipoma ◽  
The Relationship

OBJECTIVEThe nature of the relationship between spinal cord syrinx and tethered cord is not well known. It is unclear if surgical cord untethering results in resolution or improvement of an associated syrinx. The objective of this study was to report the response of spinal cord syrinx to surgical cord untethering.METHODSThe authors retrospectively reviewed all patients with a syrinx and tethered cord who presented to a single institution over an 11-year interval. Patients with open neural tube defects were excluded. Thirty-one patients were identified, 25 of whom had both clinical and imaging follow-up after surgery. Patients were grouped according to etiology of the tethered cord. Clinical outcomes and syrinx characteristics were recorded.RESULTSOf the 25 patients with tethered cord, 68% (n = 17) were male. The average age at presentation was 2.5 years (0–10.1 years) and age at surgery was 3.7 years (range 1 day to 17 years). Etiologies of tethered cord were lipomyelomeningocele (n = 8), thickened/fatty filum (n = 7), intradural lipoma (n = 5), myelocystocele (n = 2), meningocele (n = 2), and diastematomyelia (n = 1). Twenty-three of the patients underwent primary untethering, whereas 2 patients had received untethering previously at another institution. The average syrinx length and width prior to surgery were 4.81 vertebral levels (SD 4.35) and 5.19 mm (SD 2.55 mm), respectively. Conus level ranged from L1 to S3. Patients were followed for an average of 8.4 years (1.35–15.85 years). Overall there was no significant change in syrinx length or width postoperatively; the average syrinx length increased by 0.86 vertebral levels (SD 4.36) and width decreased by 0.72 mm (SD 2.94 mm). Seven of 25 patients had improvement in at least one presenting symptom, including scoliosis, weakness, bowel/bladder dysfunction, and pain. Eight patients had stable presenting symptoms. Six patients were asymptomatic and 5 patients had new or worsening symptoms, which included scoliosis, pain, or sensory changes.CONCLUSIONSAlthough some syrinxes improved after surgery for tethered cord, radiological improvement was not consistent and did not appear to be associated with change in clinical symptoms. The decision to surgically untether a cord should be focused on the clinical symptoms and not the presence of a syrinx alone. Further studies are needed to confirm this finding.

scholarly journals Delayed variations in the diagnostic accuracy of intraoperative neuromonitoring in the resection of intramedullary spinal cord tumors

2021 ◽  
Vol 50 (5) ◽  
pp. E21
Author(s):  
Gil Kimchi ◽  
Nachshon Knoller ◽  
Akiva Korn ◽  
Yahel Eyal-Mazuz ◽  
Yechiam Sapir ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Diagnostic Accuracy ◽  
Postoperative Period ◽  
Intraoperative Neuromonitoring ◽  
Spinal Cord Tumors ◽  
Intramedullary Spinal Cord ◽  
Over Time ◽  
D Wave

OBJECTIVE The use of intraoperative neuromonitoring (IONM) has become an imperative adjunct to the resection of intramedullary spinal cord tumors (IMSCTs). While the diagnostic utility of IONM during the immediate postoperative period has been previously studied, its long-term diagnostic accuracy has seldom been thoroughly assessed. The aim of this study was to evaluate long-term variations in the diagnostic accuracy of transcranial motor evoked potentials (tcMEPs), somatosensory evoked potentials (SSEPs), and D-wave recordings during IMSCT excision. METHODS The authors performed a retrospective evaluation of imaging studies, patient charts, operative reports, and IONM recordings of patients who were operated on for gross-total or subtotal resection of IMSCTs at a single institution between 2012 and 2018. Variations in the specificity, sensitivity, positive predictive value (PPV), and negative predictive value (NPV) for postoperative functional outcome (McCormick Scale) were analyzed at postoperative day 1 (POD1), 6 weeks postoperatively (PO-6 weeks), and at the latest follow-up. RESULTS Overall, 28 patients were included. The mean length of follow-up was 19 ± 23.4 months. Persistent motor attenuations occurred in 71.4% of the cohort. MEP was the most sensitive modality (78.6%, 87.5%, and 85.7% sensitivity at POD1, PO-6 weeks, and last follow-up, respectively). The specificity of the D-wave was the most consistent over time (100%, 83.35%, and 90% specificity at the aforementioned time points). The PPV of motor recordings decreased over time (58% vs 33% and 100% vs 0 for tcMEP and D-wave at POD1 and last follow-up, respectively), while their NPV consistently increased (67% vs 89% and 70% vs 100% for tcMEP and D-wave at POD1 and last follow-up, respectively). CONCLUSIONS The diagnostic accuracy of IONM in the resection of IMSCTs varies during the postoperative period. The decrease in the PPV of motor recordings over time suggests that this method is more predictive of short-term rather than long-term neurological deficits. The increasing NPV of motor recordings indicates a higher diagnostic accuracy in the identification of patients who preserve neurological function, albeit with an increased proportion of false-negative alarms for the immediate postoperative period. These variations should be considered in the surgical decision-making process when weighing the risk of resection-associated neurological injury against the implications of incomplete tumor resection.

Tethered cord release: a long-term study in 114 patients

2009 ◽  
Vol 3 (3) ◽  
pp. 181-187 ◽  
Author(s):  
Robin M. Bowman ◽  
Avinash Mohan ◽  
Joy Ito ◽  
Jason M. Seibly ◽  
David G. McLone
Keyword(s):  
Spinal Cord ◽  
Tethered Cord ◽  
Rehabilitation Medicine ◽  
Memorial Hospital ◽  
Control Group ◽  
Common Cause ◽  
Surgical Interventions ◽  
Long Term Study ◽  
Spinal Cord Tethering

Object All children born with a myelomeningocele at the authors' institution undergo aggressive treatment to maintain or improve functional outcome. Consequently, when any neurological, orthopedic, and/or urological changes are noted, a search for the cause is initiated. The most common cause of decline in a child born with a myelomeningocele is shunt malfunction. The second most common cause is tethering of the distal spinal cord at the site of the original back closure. In this report, the authors review the indicators of symptomatic spinal cord tethering and discuss the surgical interventions and outcomes in the children with myelomeningocele who underwent treatment at Children's Memorial Hospital from 1975 to 2008. Methods Among the 502 children who underwent original closure at Children's Memorial Hospital, a symptomatic tethered spinal cord developed in 114 (23%). Eighty-one patients (71%) have undergone 1 untethering procedure, and 33 patients (29%) have undergone multiple untetherings, for a total of 163 total surgeries. The indicators of symptomatic spinal cord tethering include scoliosis, decline in lower-extremity (LE) motor strength, LE contractures, LE spasticity, gait change, urinary changes, and pain. Results Pain has shown the best response to surgical untethering, with 100% of children experiencing postoperative improvement. The results of long-term follow-up (average 12 years, range 1 month–23.3 years) in this cohort demonstrated scoliosis progression after surgical untethering in 52% of patients, with 28% requiring spinal fusion. On the 3-month postoperative manual muscle test, 70% of patients showed improved LE muscle strength compared to preoperatively. Gait was also similarly improved after untethering as evaluated by an orthopedic surgeon. Spasticity improved in two-thirds of the cohort, and as expected, LE contractures were stable (78%) postoperatively, as assessed by orthopedic and rehabilitation medicine specialists. Urologically, 64% of patients showed improvements on postoperative bladder evaluation. Conclusions Although this is a clinical outcome study with no control group, the authors' experience has been that tethered cord release is beneficial in maintaining neurological, urological, and orthopedic functioning in children born with a myelomeningocele.

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