1/182. 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 = malformation (Clic here for more details about this article) |
2/182. Spontaneous resolution of a cervicothoracic syrinx in a child. Case report and review of the literature.A child with near complete spontaneous resolution of a cervicothoracic syrinx and improvement in a Chiari type I malformation without surgical intervention is presented. The child was followed clinically with serial magnetic resonance (MR) imaging and has remained neurologically stable over an 11-year period. To our knowledge, only 3 pediatric cases of spontaneous resolution of a spinal cord syrinx as documented by MR imaging without surgical intervention have been reported. This case contributes to the literature on the natural history of syringes.- - - - - - - - - - ranking = 0.14285714285714keywords = malformation (Clic here for more details about this article) |
3/182. Syringohydromyelia or HTLV-I-associated myelopathy/tropical spastic paraparesis. A diagnostic challenge. Case report.Human T-cell lymphotropic virus type I (HTLV-I) associated myelopathy/tropical spastic paraparesis (HAM/TSP) is the most common chronic myelopathy in brazil. We present the case of a 53 year old man that fulfilled the diagnostic criteria for HAM/TSP but had at the magnetic resonance imaging (MRI) of the spinal cord evidences of syringohydromyelia at the C6-C7 and D2-D7 levels along with Chiari type I malformation. The clinical picture was more typical of HAM/TSP than of syringohydromyelia, which was probably asymptomatic. The present case clearly demonstrates that serology and neuroimaging should be always used together. We conclude that, specially in places where HTLV-I is endemic, every patient with a spastic paraparesis, even with a radiological picture suggestive of a structural spinal cord lesion, should have a screening test for HTLV-I. The clinical picture must dictate the final direction of the diagnosis.- - - - - - - - - - ranking = 0.14285714285714keywords = malformation (Clic here for more details about this article) |
4/182. 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 = 0.71428571428571keywords = malformation (Clic here for more details about this article) |
5/182. Syringobulbia caused by delayed postoperative tethering of the cervical spinal cord - delayed complication of foramen magnum decompression for Chiari malformation.Postoperative tethering of the high cervical spinal cord is a rare cause of neurological deterioration after foramen magnum decompression (FMD) with duraplasty for Chiari type I malformation. A review of the literature revealed that only 5 cases have been reported. This entity is not widely known to occur as a complication of the common surgical procedure for Chiari type I malformation. A 17-year-old boy experienced rapidly progressive neurological deterioration over a 3-month period. FMD and duraplasty with lyophilized cadaver dura had been performed 8 years previously. Follow-up MR images showed that the cerebrospinal fluid (CSF) space dorsal to the cord was gradually disappearing and that syringobulbia had developed. Opening the dura mater of the posterior fossa revealed dense fibrous scarring, arachnoid thickening over the cervicomedullary area, and tethering the cord to the dura from the medulla to C2. The adhesions were dissected free, and the tethering was released. A syringosubarachnoid (SS) shunt was inserted and duraplasty was performed with an expended polytetrafluoroethylene sheet (Gore-Tex). Postoperative MR images demonstrated that the syringobulbia had completely collapsed and that a dorsal CSF space was present. Follow-up MR images provided significant information on the cervical spinal cord tethering after FMD with duraplasty for Chiari malformation. We encourage sharp surgical detethering and duraplasty with Gore-Tex to avoid retethering. Early recognition and treatment of this unusual but important complication are emphasized.- - - - - - - - - - ranking = 1keywords = malformation (Clic here for more details about this article) |
6/182. Diabetic neuropathic pain in a leg amputated 44 years previously.The mechanism of neuropathic pain in the diabetic limb is far from clear. phantom limb pain likewise is of obscure aetiology. The development of typical pain in an absent leg in a patient with diabetes many years after the amputation stimulates thought as to the mechanism, not only of neuropathic pain, but also of phantom limb pain. A 58-year-old man was diagnosed with type 2 diabetes 44 years after having undergone left below knee amputation for congenital AV malformation, at the age of 13. Eight months before the diagnosis of diabetes he began to complain of pain in the leg on the amputated side-pain very similar to that described in typical diabetic neuropathy. This was followed by similar pain in the right leg. MR scan of the spine revealed a small syringohydromyelia of the thoracic cord in addition to a prolapse of disc at L(5)/S(1) level on the left side, which was first noted 5 years previously. There were no other features of S(1) compression. The typical neuropathic character of the pain involving both the amputated and the intact limbs that developed with the diagnosis of type 2 diabetes suggest that the neuropathic pain may originate from centres higher than peripheral nerves.- - - - - - - - - - ranking = 0.14285714285714keywords = malformation (Clic here for more details about this article) |
7/182. spinal cord swelling preceding syrinx development. Case report.The pathophysiology of syrinx development is controversial. The authors report on a patient with progressive cervical myelopathy and a Chiari I malformation in whom spinal cord swelling preceded, by a few months, the development of a syrinx in the same location. The patient underwent a craniocervical decompressive procedure and duraplasty, and complete resolution of cord swelling and syringomyelia was achieved. This report is consistent with the theory that patients with Chiari I malformation have increased transmural flow of cerebrospinal fluid, which causes spinal cord swelling that later coalesces into a syrinx. The pathophysiology of syrinx development from spinal cord edema and the success of surgical decompressive treatments that do not invade the central nervous system support the prompt treatment of patients with spinal cord edema who are at risk for the development of a syrinx.- - - - - - - - - - ranking = 0.28710439326151keywords = malformation, nervous system (Clic here for more details about this article) |
8/182. Management of Chiari I malformations with holocord syringohydromyelia.The management of patients with Type I Chiari malformations (CM 1) with or without syringohydromyelia (SHM) has remained quite controversial, and many different surgical procedures have been advocated. Over the past several years, the authors have treated 7 children presenting with CM 1 and holocord syringohydromyelia with suboccipital decompression and duraplasty alone without intradural procedures. All children received MRI imaging at 2-4 months and 1 year postoperatively. On the early postoperative MRI examination, marked reduction in the syringohydromyelia was seen in 6 children, with minimal change in syrinx size in 1 child who was clinically improving after the operation. At 1 year, all children with early collapse remained collapsed, and the child with minimal early collapse demonstrated an approximately 50% reduction in syrinx size. Clinical follow-up (mean 30 months, range 21-50 months) showed good results in all patients: none of the children have required further neurosurgical intervention, and all have shown improvement in their preoperative function. One child with a 46 degrees scoliosis had a complete collapse of her SHM, but ultimately required spinal fusion. The presenting clinical findings, operative technique, MRI imaging and clinical outcomes will be discussed. The results from these 7 patients with CM 1 and holocord syringomyelia suggest that posterior fossa decompression alone (without intradural procedures) can provide excellent radiographic and clinical outcome.- - - - - - - - - - ranking = 0.71428571428571keywords = malformation (Clic here for more details about this article) |
9/182. Spontaneous resolution and recurrence of a Chiari I malformation and associated syringomyelia. Case report.The spontaneous resolution of syringomyelia has been reported infrequently. In patients with Chiari I malformations, resolution of the syringomyelia has sometimes been associated with improvement of their malformation. The authors present a case of spontaneous resolution followed by recurrence of syringomyelia and a corresponding change in the Chiari malformation. This case is of interest in light of the theories postulated to explain spontaneous resolution of syringomyelia.- - - - - - - - - - ranking = 1keywords = malformation (Clic here for more details about this article) |
10/182. Chiari malformation and syringomyelia in monozygotic twins: birth injury as a possible cause of syringomyelia--case report.A 26-year-old female, the elder of monozygotic twins, presented with slow progressive numbness and pain in her left arm. Magnetic resonance (MR) imaging showed syringomyelia with Chiari malformation. The patient's birth had been difficult with prolonged delivery time, breech delivery, and neonatal asphyxia. MR imaging of the patient's twin sister showed mild tonsillar ectopia, but absence of syringomyelia. This younger sister was born without problems. The patient underwent syringosubarachnoid shunt at the C5-6 level. The syrinx was collapsed promptly, and her symptoms disappeared. This case of syringomyelia with Chiari malformation in one of twins suggests that birth injury is likely to be a cause of the pathogenesis of syringomyelia.- - - - - - - - - - ranking = 0.85714285714286keywords = malformation (Clic here for more details about this article) |
| | Next -> |