1/38. arnold-chiari malformation with syringomyelia in an elderly woman.PRESENTATION: A 76-year-old woman, complaining of leg pain and unsteady gait for 3 years, presented with a spastic paraparetic gait, severe spasticity and touch, thermal and pain sensory loss limited to arms, lower thorax and upper abdomen. Brain and spinal cord magnetic resonance imaging showed a large loculated syrinx. Cerebellar tonsillar herniation into the foramen magnum was also seen (arnold-chiari malformation, type I). OUTCOME: The patient had successful cervico-spinal surgical decompression which resulted in marked reduction in hypertonia and weakness, normal gait and normal joint movement at 6 months. CONCLUSION: This unusual, late clinical presentation of a congenital disease underlines the importance of a comprehensive diagnostic work-up in the elderly patients with complex neurological signs.- - - - - - - - - - ranking = 1keywords = herniation (Clic here for more details about this article) |
2/38. Occipital fibrous dysplasia tonsillar herniation and cervical syringomyelia.A small posterior fossa volume has been thought to lead to hind brain herniation. The resultant obstruction to the CSF pathways at the level of the foraman magnum has been implicated in the development and subsequent progression of syringomyelia. This report highlights the gradual development of a small posterior fossa due to fibrous dysplasia of the occipital bone with consequent tonsillar herniation resulting in syringomyelia.- - - - - - - - - - ranking = 6keywords = herniation (Clic here for more details about this article) |
3/38. Resolution of tonsillar herniation and syringomyelia after supratentorial tumor resection: case report and review of the literature.OBJECTIVE AND IMPORTANCE: The pathophysiological features of syringomyelia are not yet entirely understood. We present a case of a supratentorial mass causing tonsillar herniation and syringomyelia. CLINICAL PRESENTATION: A 51-year-old woman underwent magnetic resonance imaging for evaluation of progressive headaches. A large parieto-occipital mass was revealed. Herniation of the cerebellar tonsils and a cervical syrinx were also noted. INTERVENTION: A craniotomy was performed without incident. After tumor resection, the tonsils ascended and the syrinx resolved in a 1-year period. CONCLUSION: This case highlights the importance of tonsillar herniation in the pathogenesis of syringomyelia. "Acquired" Chiari malformations and syringomyelia attributable to supratentorial masses may be treated by mass resection alone, without the need for foramen magnum decompression.- - - - - - - - - - ranking = 6keywords = herniation (Clic here for more details about this article) |
4/38. Expansion of Chiari I-associated syringomyelia after posterior-fossa decompression.Chiari I malformation (CMI) is an abnormality that involves caudal herniation of the cerebellar tonsils into the foramen magnum. CMI has been shown to be closely associated with the development of syringomyelia (SM). OBJECTS: Several theories have emerged to explain the apparent correlation between the existence of CMI with subsequent development of SM. However, the exact mechanism of the evolution of SM is still subject to controversy. We report here the case of a 12-year-old girl admitted to hospital with headache, vomiting, ataxia, and moderate pyramidal signs. methods: Radiological evaluation revealed the presence of CMI, accompanied by a small SM. The patient underwent posterior fossa decompression and improved significantly. She was re-admitted 6 months later with clinical evidence of progressive spinal cord dysfunction. MR revealed gross expansion of the syrinx. CONCLUSIONS: This case raises questions regarding the pathophysiology of CMI and its association with SM. The case indicates the need for neurological and radiological follow-up for patients undergoing posterior fossa decompression due to CMI, even for those without an initial syrinx. This is the first report known to us of expansion of a syrinx following decompression of an associated CMI.- - - - - - - - - - ranking = 1keywords = herniation (Clic here for more details about this article) |
5/38. Comparison of different operative modalities in post-traumatic syringomyelia: preliminary report.Post-traumatic syringomyelia (PTS) is a relatively rare, but potentially disastrous, complication of spinal cord injury. Operative treatment by shunting procedures often shows only a short-term improvement, and the rate of recurrence of syringomyelia is high, so different treatment modalities have been used in the last years. The various results are discussed in this analysis. A prospective clinical study was conducted of 30 patients with PTS treated by shunting procedures or with pseudomeningocele over a period of 9 years, and followed with regular clinical and magnetic resonance imaging examinations. Shunting procedures like syringosubarachnoid and syringopleural or -peritoneal shunting showed good results only at the first follow-ups. In our department, we perform an artificial liquor reservoir at the level of the lesion after opening the spinal pathways and arachnoid adhesions at that level. This procedure was performed in 12 patients. Five of these had been previously operated by shunting procedures; all of them had suffered a recurrence of syringomyelia because of internal occlusion. In the group of patients treated by shunting procedures, a neurological improvement was be recorded in five, and a steady state in eight. Five patients showed a further deterioration. The performance of an artificial liquor reservoir to guarantee a free flow of cerebrospinal fluid around the lesion resulted in a neurological improvement in ten patients, with two maintaining a steady state. Our experience is that shunting procedures often show a neurological improvement only in the short term; the rate of recurrence of typical shunting complications is high. The performance of a pseudomeningocele is an encouraging new step in the treatment of PTS. Further long-term follow-up studies are necessary to assess the benefits of this new method.- - - - - - - - - - ranking = 1.0437501065656keywords = meningocele (Clic here for more details about this article) |
6/38. 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 = 0.52187505328281keywords = meningocele (Clic here for more details about this article) |
7/38. A craniocervical injury-induced syringomyelia caused by central canal dilation secondary to acquired tonsillar herniation. Case report.The authors report on a 19-year-old man with an acquired tonsillar herniation caused by a craniocervical junction injury in which serial magnetic resonance (MR) images demonstrated patent and isolated segments of the central canal participating in the dilation and then formation of a cervical syrinx. The patient was involved in a motor vehicle accident; he developed tonsillar herniation as a complication of subarachnoid and epidural hemorrhage, predominantly observed around the cisterna magna and upper cervical canal. Repeated MR images obtained over an 11-month period indicated the for mation and acute enlargement of the syrinx. Ten months after the accident, the patient presented with sensory disturbance in both upper extremities and spasticity due to syringomyelia. He underwent craniocervical decompressive surgery and doraplasty, which reduced the size of syringomyelia. The authors postulate that the patent central canal may play a role in determining the location of a syrinx remote from a focus of cerebrospinal fluid obstruction.- - - - - - - - - - ranking = 6keywords = herniation (Clic here for more details about this article) |
8/38. A novel fibroblast growth factor receptor 2 mutation in Crouzon syndrome associated with Chiari type I malformation and syringomyelia.OBJECT: It has been reported that due to premature synostosis of the lambdoid suture in the first 24 months of life, more than 70% of patients with Crouzon syndrome concurrently suffer from chronic tonsillar herniation (Chiari Type I malformation) and some (20%) associated syringomyelia. The goal of the present study was to examine mutations in the fibroblast growth factor receptor (FGFR) genes in Crouzon syndrome and its related conditions. methods: Five patients were studied: three with Crouzon syndrome (one sporadic and two familial), one with sporadic Chiari I with syringomyelia, and one with unilateral lambdoid synostosis. Deoxyribonucleic acid was screened for FGFR1-3 mutations by using single-strand conformational polymorphism and subsequent direct sequencing. Two types of missense mutations were detected in the FGFR2 gene, Cys342Trp (1205, TGC --> TGG) in a patient with sporadic Crouzon syndrome and Tyr281Cys (1021, TAC --> TGC) in two siblings (brother and sister) with familial Crouzon syndrome, respectively. The former has been reported only in sporadic cases but the latter has not previously been identified. A polymorphism in the FGFR3 gene, Asn294Asn (882, AAT --> AAC), was also detected in three patients. No mutation was found in the patient with sporadic Chiari I with syringomyelia. CONCLUSIONS: The FGFR2 missense mutation was detected in Crouzon syndrome but not in sporadic Chiari I with syringomyelia or lambdoid synostosis. A novel FGFR2 mutation, Tyr281Cys, was found in familial Crouzon syndrome with Chiari I and syringomyelia. It may be informative to look for this in patients with Crouzon syndrome and associated syringomyelia.- - - - - - - - - - ranking = 1keywords = herniation (Clic here for more details about this article) |
9/38. syringomyelia without hindbrain herniation: tight cisterna magna. Report of four cases and a review of the literature.Idiopathic syringomyelia, which is not associated with any definite pathogenic lesions, has been treated mainly by shunting of the syrinx and rarely by craniocervical decompression. The authors report four cases of syringomyelia thought to be idiopathic syringomyelia but treated by craniocervical decompression with favorable results. syringomyelia was present without hindbrain herniation. In such cases, the subarachnoid space anterior to the brainstem at the level of the foramen magnum is usually open but the cisterna magna is impacted by the tonsils, a condition the authors term "tight cisterna magna." All patients underwent foramen magnum decompression and C-1 laminectomy, and the outer layer of the dura was peeled off. Further intradural exploration was performed when outflow of cerebrospinal fluid (CSF) from the fourth ventricle was deemed to be insufficient. Postoperatively, improvement in symptoms and a reduction in syrinx size were demonstrated in three patients, and a reduction in ventricle size was shown in two. syringomyelia associated with tight cisterna magna should not be classified as idiopathic syringomyelia; rather, it belongs to the category of organic syringomyelia such as Chiari malformation. A possible pathogenesis of cavitation is obstruction of the CSF outflow from the foramen of Magendie, and the cavity may be a communicating dilation of the central canal. Ventricular dilation may depend on the extent to which CSF drainage is impaired from the foramina of Luschka. These cavities may respond to craniocervical decompression if it results in sufficient CSF outflow from the foramen of Magendie, even in cases with concomitant hydrocephalus.- - - - - - - - - - ranking = 5keywords = herniation (Clic here for more details about this article) |
10/38. Maternal Arnold-Chiari type I malformation and syringomyelia: a labor management dilemma.Arnold-Chiari type I malformations consists of elongation of the cerebellar tonsils with their displacement below the foramen magnum. syringomyelia is an associated cyst that accumulates cerebrospinal fluid in the cord that can impinge on local nerve fibers. pregnant women with either of these disorders are of special concern due to the potential risk of brain stem herniation and or spinal column compression from physiological changes that occur during labor. We present two cases. The first case is a patient with syringomyelia who was admitted in labor with worsening peripheral neurological symptoms. Epidural anesthesia was placed and she underwent an uncomplicated cesarean delivery with resolution of her symptoms postpartum. The second case is a patient with an Arnold-Chiari type I malformation and syringomyelia who presented in labor. The patient had an epidural placed and was allowed to progress to complete dilation and effacement at 2 station. She underwent a successful operative vaginal delivery without voluntary maternal expulsive efforts. Both patients had uncomplicated postpartum courses. Although these are rare disorders with significant potential morbidity, labor can be managed by either mode of delivery with careful patient selection. We caution that this review has insufficient numbers of patients to address the safety and efficacy of either delivery mode but rather focuses on alternatives for delivery. This report is the first to document a case of a patient with an arnold-chiari malformation and syringomyelia successfully managed in labor with a vaginal delivery.- - - - - - - - - - ranking = 1keywords = herniation (Clic here for more details about this article) |
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