1/30. Pediatric syringomyelia with chiari malformation: its clinical characteristics and surgical outcomes.BACKGROUND: Most reports regarding pediatric syringomyelia have focused not only on Chiari malformation, but also on spinal dysraphism. However, the clinical characteristics of syringomyelia with spinal dysraphism are quite different from those of syringomyelia due to Chiari Type I malformation. The objectives of this study were to identify clinical characteristics of pediatric syringomyelia and to determine whether surgery prevents or corrects the scoliosis associated with syringomyelia. methods: We reviewed the records of 16 pediatric patients with syringomyelia and Chiari Type I malformation. The patients' ages ranged from 3 to 15 years, with mean age of 9.8 years. None of the patients had spinal dysraphism. RESULTS: Nystagmus was observed in 2 of the 16 patients, motor weakness in 8 patients, sensory disturbance in 10 patients, and scoliosis in 13 patients. As the initial surgical procedure, foramen magnum decompression (FMD) was performed in seven patients and syringo-subarachnoid (S-S) shunting in nine patients. The motor function improved in 7 of the 8 affected patients, and the sensory disturbance improved in 9 of the 10 affected patients. The magnetic resonance images obtained after the surgery revealed marked decrease of the syrinx size in all patients. Of the 13 patients with scoliosis, 5 showed improvement, 5 stabilization, and 3 deterioration. CONCLUSIONS: Compared with adolescent and adult syringomyelia, pediatric syringomyelia shows a much lower incidence of sensory disturbance and pain, but quite a high incidence of scoliosis. Surgery is effective in improving or stabilizing scoliosis in these patients.- - - - - - - - - - ranking = 1keywords = syringomyelia (Clic here for more details about this article) |
2/30. syringomyelia in children with primary scoliosis.The clinical notes of 35 children presenting with scoliosis were reviewed; all 35 had been investigated with MRI. Seven were found to have syringomyelia, and six of these had Chiari malformation. Correction of the syrinx resulted in improvement or stabilisation of the spinal curvature. We recommend that all cases presenting with primary scoliosis should have MRI and should be treated if a syrinx is found.- - - - - - - - - - ranking = 0.083333333333333keywords = syringomyelia (Clic here for more details about this article) |
3/30. Evaluating congenital spine deformities for intraspinal anomalies with magnetic resonance imaging.SUMMARY: The incidence of intraspinal abnormalities associated with congenital spinal anomalies as detected by magnetic resonance imaging (MRI) is becoming better defined. In this study, 41 nonrandomized children with congenital spinal deformities (excluding myelomeningocele) who underwent complete MR evaluation were reviewed. Of the 41 congenital spinal deformities, 37 demonstrated congenital scoliosis, with failure of formation in 19, failure of segmentation in 4, and mixed defects in 14. The remaining four deformities were cases of congenital kyphosis. Thirteen patients with congenital spine anomalies were noted to have intraspinal abnormalities identified by MRI: tethered cord in 12 patients, syringomyelia in 3 patients, and diastematomyelia in 5 patients. Of the 12 patients with tethered cord, 2 patients had neurologic deficits. Urorectal anomaly was one of the most common associated findings (15%). Considering an incidence of intraspinal anomalies of 31% and as clinical manifestations may not be initially detectable, MRI is recommended in patients with congenital spinal deformity as part of the initial evaluation even in the absence of clinical findings.- - - - - - - - - - ranking = 0.083333333333333keywords = syringomyelia (Clic here for more details about this article) |
4/30. Chiari I malformation associated with syringomyelia and scoliosis: a twenty-year review of surgical and nonsurgical treatment in a pediatric population.STUDY DESIGN: Retrospective review of patients with Chiari I malformation with or without associated scoliosis. OBJECTIVES: Determine the effect of decompression of Chiari I malformation with syringomyelia on stabilization or improvement of associated scoliosis. SUMMARY OF BACKGROUND DATA: Chiari malformations are often associated with spinal deformities, including scoliosis. Studies have suggested a causal relation between syringomyelia and scoliosis. methods: patients with Chiari I malformation and syringomyelia with or without scoliosis treated over the last 20 years were reviewed. patients with any other anomalies were excluded. Scoliotic curves were classified by magnitude and curve type. All patients were treated with surgical decompression of the Chiari malformation with or without drainage of the syringomyelia. RESULTS: Twenty-five patients were identified, ranging in age from 19 months to 16.5 years. Nineteen patients (76%) had associated scoliosis. The majority of the patients with scoliosis (13 of 19) sought treatment for spinal deformity, and only 6 had for pain or neurologic symptoms. Eleven of 19 patients with scoliosis (58%) underwent fusion. Eight of 19 (42%) patients have not undergone fusion: 3 have experienced progress, 1 remains in a stable condition, and 4 have experienced improvement of curvature since undergoing decompression. The mean age of patients who experienced progress after decompression was 14.5 years, compared to 6 years for patients who experienced improvement. CONCLUSION: Early decompression of Chiari I malformation with syringomyelia and scoliosis resulted in improvement or stabilization of the spinal deformity in 5 cases. Each of these patients underwent decompression before 8 years of age and before the curve was severe. However, this series represents a few patients demonstrating this trend, and further follow-up and investigation are warranted.- - - - - - - - - - ranking = 0.75keywords = syringomyelia (Clic here for more details about this article) |
5/30. Idiopathic scoliosis as a presenting sign of familial neurologic abnormalities.STUDY DESIGN: Familial cases of "idiopathic" scoliosis associated with neurologic abnormalities are reported with a review of the literature. OBJECTIVE: To investigate the prevalence of neurologic abnormalities such as syringomyelia, Chiari 1 malformation, and tonsillar ectopia in patients with genetically determined "idiopathic" scoliosis. SUMMARY OF BACKGROUND DATA: Idiopathic scoliosis is widely considered to be a genetic disorder of unknown etiology. magnetic resonance imaging (MRI) studies have shown that several cases of "idiopathic" scoliosis show neurologic abnormalities including syringomyelia and Chiari 1 malformation. Recently, several familial cases of either syringomyelia or Chiari malformation were reported, and it is suspected that genetic factors may influence the development of the craniovertebral malformation. It was hypothesized that some cases of "idiopathic" scoliosis include a craniovertebral malformation that is genetically determined. methods: This study, using clinical examinations and MRI, investigated 71 patients with scoliosis and a family history of "idiopathic" scoliosis in third-degree relatives for the presence of neurologic abnormalities. If neurologic abnormalities were confirmed with MRI, the relatives affected with scoliosis were also examined. RESULTS: Nine (13%) patients showed neurologic abnormalities on MRI. magnetic resonance imaging showed syringomyelia with Chiari 1 malformation in four patients, Chiari 1 malformation in three patients, and tonsillar ectopia in two patients. Among the relatives of these patients, 4 of 15 individuals affected with scoliosis also showed neurologic abnormalities on MRI. CONCLUSIONS: It is suggested that familial neurologic abnormalities may have a wide range of expression, and that some patients with "idiopathic" scoliosis present with genetically determined craniovertebral malformations such as syringomyelia, Chiari 1 malformation, and tonsillar ectopia.- - - - - - - - - - ranking = 0.41666666666667keywords = syringomyelia (Clic here for more details about this article) |
6/30. Hyperkyphosis as an indicator of syringomyelia in idiopathic scoliosis: a case report.STUDY DESIGN: A case of thoracic hyperkyphosis is reported as an atypical presentation of otherwise typical adolescent idiopathic scoliosis that showed a large syringomyelia on magnetic resonance imaging. OBJECTIVE To review atypical curve patterns that indicate the need for more intensive preoperative workup including a magnetic resonance imaging scan. SUMMARY OF BACKGROUND DATA: The literature on atypical curve patterns of adolescent "non-idiopathic" scoliosis is reviewed. No report in the literature describes patients with an idiopathic coronal plane and thoracic hyperkyphosis as an atypical feature found to have a large syringomyelia and Chiari I malformation on magnetic resonance imaging examination. methods: The clinical and radiographic features associated with thoracic hyperkyphosis and juvenile and adolescent scoliosis with syringomyelia are presented. RESULTS: The neurosurgical treatment of the syringomyelia subsequently led to a potentially safer spinal fusion. The patient tolerated the procedures well and at this writing has remained asymptomatic during 3 years of follow-up evaluation. CONCLUSIONS: The reported case adds to the hyperkyphosis literature category of unusual spinal deformities seen in scoliosis associated with syringomyelia and should further help to categorize adolescent idiopathic scoliosis as a diagnosis of exclusion.- - - - - - - - - - ranking = 0.75keywords = syringomyelia (Clic here for more details about this article) |
7/30. A patient with optic pathway glioma, scoliosis, Chiari type I malformation and syringomyelia: is it Neurofibromatosis type 1?A 22 years old girl had features of optic pathway glioma, scoliosis, Chiari type 1 malformation and cervical syringomyelia. She had no cutaneous lesions. We considered this combination to be more than coincidental and argue in favour of considering the case as a variant form of Neurofibromatosis type 1. The relevent literature in favour of our contention has been reviewed.- - - - - - - - - - ranking = 0.41666666666667keywords = syringomyelia (Clic here for more details about this article) |
8/30. Preoperative radiological and electrophysiological evaluation in 100 adolescent idiopathic scoliosis patients.This is a prospective study of spinal magnetic resonance imaging (MRI), electrophysiological recordings, and neurological examinations of 100 patients admitted for surgery for adolescent idiopathic scoliosis (AIS), which was conducted to assess the prevalence of structural and functional abnormalities within the spinal cord in patients with clinically normal neurologic condition. In all patients the clinical diagnosis and intact neurological condition was ascertained by a spinal orthopedic surgeon. Full-length spinal axis MRI studies (T1/T2 sequences) and somato-sensory evoked potentials of the tibial nerves (tSSEPs) were preoperatively assessed by independent evaluators blinded to the patients' medical histories. Structural spinal cord abnormalities were found in three of 100 AIS patients on MR imaging. In one patient a Chiari malformation type 1 with an accompanying syringomyelia was diagnosed, which required a suboccipital decompression. In the other two patients small thoracic syringomyelias were diagnosed. Abnormalities of spinal cord function were detected in 68% of the 100 patients: tSSEP latencies corrected for body height were increased in 56% of the patients; pathological differences between tSSEPs on the left and right sides were present in 17% (12% in combination with a prolongation of the latency). The findings of this study indicate that MRI and electrophysiological examinations are essential to assess spinal cord abnormalities that are clinically not detectable in AIS patients. Even in patients with intact neurologic condition and clinically typical right-curved thoracic scoliosis, the possibility of intraspinal pathologies should be ruled out by MRI. It is especially important to detect structural pathologies like syringomyelia and Chiari malformation before proceeding with scoliosis surgery, as these conditions are associated with a higher neurological risk during scoliosis surgery. The electrophysiological recordings made in the present study, with the high number of pathological tSSEPs, are indicative of functional abnormalities with a subclinical involvement of the recorded neuronal pathways. The relevance of the latter findings is not yet clear, but pre-operative tSSEP examinations offer the possibility of assessing alterations in spinal cord function that are undetectable by clinical examination.- - - - - - - - - - ranking = 0.25keywords = syringomyelia (Clic here for more details about this article) |
9/30. Primary spinal oligoastrocytoma: a case report.BACKGROUND: Primary oligoastrocytomas of the spinal cord are rare, and the treatment options for low-grade intramedullary tumors are controversial. DESCRIPTION: A 10-year-old girl presented with thoracic scoliosis. magnetic resonance imaging (MRI) revealed an enhancing intramedullary mass lesion extending from C-5 to T-5, associated with whole spine syringomyelia and syringobulbia. (18)F-fluorodeoxyglucose-positron emission tomography (FDG-PET) showed high uptake in the lesion. The patient underwent C-5 to T-5 laminoplastic laminotomy and subtotal removal of the tumor. Histologic examination revealed that the tumor was an oligoastrocytoma without anaplastic findings. Genetic analysis revealed loss of heterozygosity of 1p and 19q but not 10q, which is characteristic of oligodendroglial tumors. CONCLUSIONS:To our knowledge, this is only the second report describing primary oligoastrocytoma of the spinal cord. Genetic analysis may provide a clue in selecting optimal adjuvant therapies.- - - - - - - - - - ranking = 0.083333333333333keywords = syringomyelia (Clic here for more details about this article) |
10/30. Spontaneous resolution of a Chiari I malformation associated syringomyelia in one child.A child with complete spontaneous resolution of a Chiari I malformation associated syringomyelia without surgical intervention is presented. The child was followed clinically by serial magnetic resonance imaging (MRI) and remains neurologically stable after 8-years of follow-up. To our knowledge, only 6 pediatric cases with spontaneous resolution of a spinal cord syrinx documented by MRI without surgical intervention have been reported. This case is of interest in the light of the postulated theories to explain spontaneous resolution of syringomyelia.- - - - - - - - - - ranking = 0.41666666666667keywords = syringomyelia (Clic here for more details about this article) |
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