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Concept: Tethered spinal cord syndrome

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The clinical case is presented on a patient with an extensive sacral dysraphism, a history of myelomeningocele surgical repair in her childhood, as well as tethered cord syndrome. The patient was also diagnosed with pseudomeningocele and a cerebrospinal fluid cutaneous fístula. A surgical approach was used, with encouraging results being obtained in the clinical outcome of the patient. A review of the literature was performed to support the surgical decision in this case.

Concepts: Spinal cord, Medical terms, Diagnosis, Scoliosis, Physician, Cerebrospinal fluid, Spina bifida, Tethered spinal cord syndrome

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As awareness and frequency of tethered spinal cord (TSC) related to occult spinal dysraphism (OSD) has increased with magnetic resonance imaging (MRI), variability exists in its evaluation and management. Due to no published level I data, we summarize the current International Children’s Continence Society (ICCS) recommendations for diagnosis and treatment of OSD.

Concepts: Spinal cord, Medical imaging, Nuclear magnetic resonance, Scoliosis, Magnetic resonance imaging, Ultrasound, Spina bifida, Tethered spinal cord syndrome

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The Ehlers-Danlos syndromes (EDS) are a heterogeneous group of heritable connective tissue disorders characterized by joint hypermobility, skin extensibility, and tissue fragility. This communication briefly reports upon the neurological manifestations that arise including the weakness of the ligaments of the craniocervical junction and spine, early disc degeneration, and the weakness of the epineurium and perineurium surrounding peripheral nerves. Entrapment, deformation, and biophysical deformative stresses exerted upon the nervous system may alter gene expression, neuronal function and phenotypic expression. This report also discusses increased prevalence of migraine, idiopathic intracranial hypertension, Tarlov cysts, tethered cord syndrome, and dystonia, where associations with EDS have been anecdotally reported, but where epidemiological evidence is not yet available. Chiari Malformation Type I (CMI) has been reported to be a comorbid condition to EDS, and may be complicated by craniocervical instability or basilar invagination. Motor delay, headache, and quadriparesis have been attributed to ligamentous laxity and instability at the atlanto-occipital and atlantoaxial joints, which may complicate all forms of EDS. Discopathy and early degenerative spondylotic disease manifest by spinal segmental instability and kyphosis, rendering EDS patients prone to mechanical pain, and myelopathy. Musculoskeletal pain starts early, is chronic and debilitating, and the neuromuscular disease of EDS manifests symptomatically with weakness, myalgia, easy fatigability, limited walking, reduction of vibration sense, and mild impairment of mobility and daily activities. Consensus criteria and clinical practice guidelines, based upon stronger epidemiological and pathophysiological evidence, are needed to refine diagnosis and treatment of the various neurological and spinal manifestations of EDS. © 2017 Wiley Periodicals, Inc.

Concepts: Central nervous system, Nervous system, Intracranial pressure, Muscle, Neurology, Ehlers-Danlos syndrome, Arnold-Chiari malformation, Tethered spinal cord syndrome

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Tethered cord syndrome (TCS) is an increasingly recognized clinical condition, with untethering surgery as the classically effective treatment. Yet, as evidence aggregated, the surgical outcome untethering, remains controversial. This study aimed to systematically evaluate the clinical outcomes after untethering surgery in primary and re-tethering patients.

Concepts: Medicine, The Canon of Medicine, Scoliosis, Anesthesia, Spina bifida, Tethered spinal cord syndrome

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Purpose Tethered cord syndrome (TCS) is the clinical manifestation of an abnormal stretch on the spinal cord, caused by several pathological conditions. Tethered cord release is the gold standard treatment for TCS. However, direct untethering carries potential risks of spinal cord injury, post-operative retethering, and CSF-related complications. Spine-shortening osteotomy (SSO) has recently been performed as an alternative technique to avoid these risks. We aimed to systematically review the literature on indications and outcome of SSO in TCS patients. Methods We searched PubMed, Embase, Google Scholar, and the Cochrane Library to identify all studies on SSO in TCS patients. We used random or fixed-effects models to calculate rates and 95% confidence intervals to establish the rates of clinical improvement in TCS patients performed with SSO. Sensitive analysis and metaregression were made to explore potential sources of heterogeneit. Results We identified six eligible surveys with a total population of 57. Rates ranged from 62 to 88% for neurological deficits improvement, 80-100% for motor function improvement, 60-96% for pain or numbness scores improvement, 13-67% for sensory function improvement, and 79-100% for urinary and bowel dysfunction improvement. We noted substantial heterogeneity in rate estimates for motor function and urinary and bowel dysfunction improvement (all Cochran’s χ² significant at P < 0.001; I² = 78.11%, 95%CI 61-94%; 84.28%, 18-100%; respectively). Conclusion SSO is a safe and effective technique for TCS patients, especially in more challenging cases, such as complex malformations or revision surgery. However, future cohort studies and randomized studies with large numbers and the power to provide illumination for the surgical decision-making of TCS are warranted.

Concepts: Spinal cord, Clinical trial, Evidence-based medicine, Systematic review, Randomized controlled trial, MEDLINE, Arnold-Chiari malformation, Tethered spinal cord syndrome

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Adult tethered cord syndrome is a rare neurological disorder that classically presents with back or leg pain, weakness, and urinary dysfunction. Spinal cord tethering has been associated with acquired Chiari malformations. While the effects of tethered cord release on Chiari malformation symptoms have been previously described, we report an unusual case of acquired tethered cord syndrome following Chiari decompression.

Concepts: Spinal cord, Neurology, Congenital disorder, Ehlers-Danlos syndrome, Neurological disorders, Arnold-Chiari malformation, Tethered spinal cord syndrome

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Pediatric regional anesthesia is widely used to relieve postoperative pain after abdominal surgery. Commonly used techniques of regional anesthesia include lumbar epidural and caudal block. However, the use of central neuraxial blockade has limitations. It is contraindicated in patients with clotting abnormalities, spinal dysraphism with tethered cord syndrome, meningomyelocele, and following spinal surgery with instrumentation. Ultrasound guided transversus abdominis plane block is a new method of regional anesthesia that can be used in settings where central neuraxial blockade is contraindicated. In this study, we present 5 pediatric cases in which major abdominal surgery was performed but central neuraxial blockade could not be carried out due to spinal abnormalities.

Concepts: Scoliosis, Physician, Transversus abdominis muscle, Opioid, Epidural, Spina bifida, Tethered spinal cord syndrome

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The Chiari Malformation I (CM I) and the tethered cord syndrome (TCS) are both congenital abnormalities whose mechanisms are still not fully understood. The association of CM I and TCS has been reported only a few times previously.

Concepts: Congenital disorder, Congenital disorders, Congenital, Arnold-Chiari malformation, Congenital abnormality, Tethered spinal cord syndrome

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OBJECTIVE Previous reports have addressed the short-term response of patients with Chiari-related scoliosis (CRS) to suboccipital decompression and duraplasty (SODD); however, the long-term behavior of the curve has not been well defined. The authors undertook a longitudinal study of a cohort of patients who underwent SODD for CRS to determine whether there are factors related to Chiari malformation (CM) that predict long-term scoliotic curve behavior and need for deformity correction. METHODS The authors retrospectively reviewed cases in which patients underwent SODD for CRS during a 14-year period at a single center. Clinical (age, sex, and associated disorders/syndromes) and radiographic (CM type, tonsillar descent, pBC2 line, clival-axial angle [CXA], syrinx length and level, and initial Cobb angle) information was evaluated to identify associations with the primary outcome: delayed thoracolumbar fusion for progressive scoliosis. RESULTS Twenty-eight patients were identified, but 4 were lost to follow-up and 1 underwent fusion within a year. Among the remaining 23 patients, 11 required fusion surgery at an average of 88.3 ± 15.4 months after SODD, including 7 (30%) who needed fusion more than 5 years after SODD. On univariate analysis, a lower CXA (131.5° ± 4.8° vs 146.5° ± 4.6°, p = 0.034), pBC2 > 9 mm (64% vs 25%, p = 0.06), and higher initial Cobb angle (35.1° ± 3.6° vs 22.8° ± 4.0°, p = 0.035) were associated with the need for thoracolumbar fusion. Multivariable modeling revealed that lower CXA was independently associated with a need for delayed thoracolumbar fusion (OR 1.12, p = 0.0128). CONCLUSIONS This investigation demonstrates the long-term outcome and natural history of CRS after SODD. The durability of the effect of SODD on CRS and curve behavior is poor, with late curve progression occurring in 30% of patients. Factors associated with CRS progression include an initial pBC2 > 9 mm, lower CXA, and higher Cobb angle. Lower CXA was an independent predictor of delayed thoracolumbar fusion. Further study is necessary on a larger cohort of patients to fully elucidate this relationship.

Concepts: Cohort study, Longitudinal study, Scoliosis, Term, Univariate, Arnold-Chiari malformation, Syringomyelia, Tethered spinal cord syndrome

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OBJECTIVE Patients with myelomeningocele are often affected by scoliosis and tethered cord syndrome, and frequently require spine surgery. Intradural spine surgeries may carry an inherently higher risk of inducing shunt malfunction due to entry into the subarachnoid space. In this study, the authors sought to compare rates of shunt malfunction after intradural and extradural spine surgeries among pediatric patients with myelomeningocele. METHODS The authors reviewed records of the National Spina Bifida Program Registry for Children’s Hospital of Alabama. The Exago reporting function was used to identify patients who had received at least one of the following procedures: shunt revision, tethered cord release (TCR), or spinal fusion for deformity. The registry records were reviewed for all identified patients to determine if a shunt revision was performed within the 1st year after TCR or spinal fusion. RESULTS Final analyses included 117 patients, of whom 39 underwent spinal fusion and 78 underwent TCR. Among patients who underwent spinal fusion, shunt revision was performed within 30 days in 2 patients (5.1%), within 60 days in 2 (5.1%), within 90 days in 4 (10.3%), and within 1 year in 5 (12.8%). Among patients who underwent TCR, shunt revision was performed within 30 days in 7 patients (9.0%), within 60 days in 10 (12.8%), within 90 days in 11 (14.1%), and within 1 year in 17 (21.8%). Using the log-rank test, there was no significant difference in Kaplan-Meier curves between intradural and extradural groups (p = 0.59). CONCLUSIONS In a review of single-institution registry data, the authors found no statistically significant difference in the risk of shunt malfunction after intradural and extradural spine surgeries.

Concepts: Spinal cord, Statistical significance, Scoliosis, Physician, Neurosurgery, Windows Registry, Spina bifida, Tethered spinal cord syndrome