Syringomyelia Research Papers - Academia.edu (original) (raw)

BACKGROUND To study the clinico-radiological determinants of outcome in patients with Chiari I malformation (CIM). MATERIALS AND METHODS The disability assessment of 48 patients with C I M who underwent posterior decompression was done by... more

BACKGROUND To study the clinico-radiological determinants of outcome in patients with Chiari I malformation (CIM). MATERIALS AND METHODS The disability assessment of 48 patients with C I M who underwent posterior decompression was done by modified Klekamp and Samii scoring system. The outcome was regarded as good when the patient was ambulant without any aid with an improvement in the disability score; and, poor when (a) there was postoperative deterioration or lack of improvement; (b) the patient was non-ambulant without aid, irrespective of the improvement in the clinical score; or, (c) there was a perioperative mortality. Patients with hydrocephalus; those who underwent syringo-subarachnoid or syringo-peritoneal shunt as the primary procedure; and, patients with atlanto-axial dislocation were excluded from the study. STATISTICAL METHODS USED: Categorical data were expressed in proportions and analyzed with Chi square test. Analysis of factors predicting clinical outcome at 6 mont...

Currarino syndrome is a type of caudal regression syndrome characterized by the association of hemisacrum, anorectal malformation and presacral mass. Only few studies on small series report the incidence of urinary dysfunction in... more

Currarino syndrome is a type of caudal regression syndrome characterized by the association of hemisacrum, anorectal malformation and presacral mass. Only few studies on small series report the incidence of urinary dysfunction in Currarino syndrome. Our aim was to evaluate the urological outcome in patients with Currarino syndrome. We retrospectively reviewed all Currarino syndrome patients treated in our institution. Of 20 patients, we could evaluate the urological outcome in 16. This group of patients underwent clinical, radiological and urodynamic evaluation. All 16 patients had a sacral defect, fourteen of them presenting a presacral mass (87.5%), eight a tethered cord (50%), and 7 anorectal malformations (43.7%). Eight patients underwent neurosurgical treatment for neural tube defects. In 14 patients, the presacral mass was resected. One case presented detrusor overactivity, 2 recurrent urinary tract infections and 2 vesicoureteral refluxes. Both patients with lipomyeloschisis ...

We recorded electrically stimulated somatosensory evoked potentials (electric SEPs) and pain-related SEPs following CO2 laser stimulation (CO2 laser SEPs) from a 17-year-old patient affected by myotonic dystrophy whose MRI disclosed a... more

We recorded electrically stimulated somatosensory evoked potentials (electric SEPs) and pain-related SEPs following CO2 laser stimulation (CO2 laser SEPs) from a 17-year-old patient affected by myotonic dystrophy whose MRI disclosed a large syrinx extending from spinal level C2 to S3. Careful clinical and electromyographic examinations revealed no motor or sensory disturbances, apart from myotonia. The only abnormality noted in median and ulnar nerve short-latency electric SEPs (recorded with a non-cephalic reference electrode) was the absence of cervical component N13, the other SEP responses (N9, N10, N11, P14, N20) being normal. The cutaneous pain threshold and CO2 laser SEPs (both obtained by a CO2 laser beam applied to the back of the hand) were normal. Thus cervical component N13 appears to be highly sensitive to the effects of central cord lesions, even when these are asymptomatic.

Background. This prospective study, conducted in patients with Chiari I malformation (C I) related syringomyelia who underwent posterior decompression and duroplasty, utilizes radionuclide cisternography in order to study the... more

Background. This prospective study, conducted in patients with Chiari I malformation (C I) related syringomyelia who underwent posterior decompression and duroplasty, utilizes radionuclide cisternography in order to study the cerebrospinal fluid (CSF) dynamics at the foramen magnum and to predict the clinical outcome following surgery. Methods. 17 consecutive patients of C I with syringomyelia (but without hydrocephalus or fixed atlanto-axial dislocation), underwent a detailed neurological examination and were assigned a clinical disability score based on the modified Klekamp and Samii score. A radionuclide cisternography (using Tc99m-DTPA) was performed via the lumbar route and the ascent of the tracer was followed utilizing a gamma camera immediately after injection and then sequentially after 1, 2, 4, 6 and 24 hours. After posterior decompression and duroplasty, the modified Klekamp and Samii score was repeated at follow-up visits (range: 3 months to one year) along with radionuclide cisternography at 3 months, and MR imaging at 6 months. Findings. Three patterns of tracer flow were observed: a) rapid flow (n=7); b) supratentorial subarachnoid delay (n=7); and, c) foramen magnum block (n=3). The patients having foramen magnum block had the poorest clinical scores on admission. At follow up, there was an improvement in the clinical scores so that the mean scores in all three categories reached nearly the same level. Following posterior decompression, the radionuclide cisternography performed in 10 patients showed a rapid flow of the tracer without any obstruction. The syrinx resolved in 4 of the 11 patients in whom an MRI was done. Interpretation. The patients with C I with syringomyelia may often have a free flow of tracer across the FM. Posterior decompression and duroplasty provides maximum clinical relief in patients with a demonstrable foramen magnum block on radionuclide cisternography while those with a normal flow have less relief. The symptomatology related to brain-stem compression immediately responds to the surgical procedure but the syrinx-induced signs and symptoms of spinal cord dysfunction persist.

Purpose With the frequent use of magnetic resonance imaging (MRI), patients with subtle and diffuse symptoms due to small syrinx cavities increasingly present to neurosurgical care. In this respect, a dilated central canal, hydromyelia,... more

Purpose With the frequent use of magnetic resonance imaging (MRI), patients with subtle and diffuse symptoms due to small syrinx cavities increasingly present to neurosurgical care. In this respect, a dilated central canal, hydromyelia, must be separated from patients with true syringomyelia with an underlying disorder, as they do not share clinical and radiological features. We hypothesize that a differentiation of these two entities with distinct diagnostic tools is possible. Methods To describe the entity of hydromyelia, we excluded all patients from the syringomyelia database (n = 142) with any obvious cause of a syringomyelia, any objective neurological deficits on clinical examination, pathological results on electrophysiological monitoring (SSEP, MEP, silent periods) or a widening of the spinal cord cavity of more than 6 mm on MRI [routine acquisitions with FLAIR, T1/T2-weighted images, Cine and CISS (constructive interference in steady-state) studies]. Life quality was assessed through SF-36 questionnaires and an individualized questionnaire for the clinical history, pain and alternative therapies. Results Forty patients (15 males/25 females) matched the criteria of a hydromyelia. With a mean age of 36.7 years (range 11–62), they almost all presented with pain (79%) or dysaesthesia of the limbs, with some having been an incidental finding (10%). Over a follow-up time of 36.9 months (range 6–93) there was no neurological or radiological deterioration. Conclusions Patients with a hydromyelia do not share clinical or radiological characteristics with patients harbouring a true syringomyelia. As hydromyelia does not represent a disease with an underlying pathology, no clinical or radiological progression has been seen. With sophisticated diagnostic tools to rule out any pathology this subset of patients can be identified.

Objective: Surgery for syringomyelia generally aims to treat the underlying cause, if it is known. Optimal management is unclear for idiopathic syringomyelia, or where treatment of the putative cause has failed or is high risk. Syrinx to... more

Posterior craniocervical decompression is the procedure most currently used for treating Chiari I malformation (alone or in association with syringomyelia in the absence of hydrocephalus). We reviewed the various technical modalities... more

Posterior craniocervical decompression is the procedure most currently used for treating Chiari I malformation (alone or in association with syringomyelia in the absence of hydrocephalus). We reviewed the various technical modalities reported in the literature. We present a personal series of 44 patients harboring Chiari type I malformation (CM-I) operated with a suboccipital craniectomy and a C1 (or C1/C2) laminectomy, plus an extreme lateral Foramen Magnum opening, a "Y" shaped dural incision with preservation of the arachnoid membrane, and an expansile duraplasty employing autogenous periosteum. Outcomes were analyzed with follow-up ranging from 1 to 10 years (4 years on average). The presented technique was compared with the other surgical modalities reported in the literature. This comparative study shows that this type of craniocervical decompression achieved the best results with minimal complications and side-effects. Syringomyelia associated with CM-I must be trea...

Spinal spondylosis is an extremely common condition that has only rarely been described as a cause of syringomyelia. We describe a case of syringomyelia associated with cervical spondylosis admitted at our division and treated by our... more

Spinal spondylosis is an extremely common condition that has only rarely been described as a cause of syringomyelia. We describe a case of syringomyelia associated with cervical spondylosis admitted at our division and treated by our institute. It is the case of a 66-year-old woman. At our observation she was affected by moderate-severe spastic tetraparesis. T2-weighted magnetic resonance imaging (MRI) showed an hyperintense signal within spinal cord from C3 to T1 with a more sharply defined process in the inferior cervical spinal cord. At the same level bulging discs, facets and ligamenta flava hypertrophy determined a compression towards subarachnoid space and spinal cord. Spinal cord compression was more evident in hyperextension rather than flexion. A 4-level laminectomy and subsequent posterior stabilization with intra-articular screws was executed. At 3-mo follow up there was a regression of tetraparesis but motor deficits of the lower limbs residuated. At the same follow up postoperative MRI was executed. It suggested enlargement of the syrinx. Perhaps hyperintensity within spinal cord appeared "bounded" from C3 to C7 with clearer margins. At the level of surgical decompression, subarachnoid space and spinal cord enlargement were also evident. A review of the literature was executed using PubMed database. The objective of the research was to find an etiopathological theory able to relate syringomyelia with cervical spondylosis. Only 6 articles have been found. At the origin of syringomyelia the mechanisms of compression and instability are proposed. Perhaps other studies assert the importance of subarachnoid space regard cerebrospinal fluid (CSF) dynamic. We postulate that cervical spine instability may be the cause of multiple microtrauma towards spinal cord and consequently may damage spinal cord parenchyma generating myelomalacia and consequently syrinx. Otherwise the hemorrhage within spinal cord central canal can cause an obstruction of CSF outflow, finally generating the syrinx. On the other hand in cervical spondylosis the stenotic elements can affect subarachnoid space. These elements rubbing towards spinal cord during movements of the neck can generate arachnoiditis, subarachnoid hemorrhages and arachnoid adhesions. Analyzing the literature these "complications" of cervical spondylosis are described at the origin of syringomyelia. So surgical decompression, enlarging medullary canal prevents rubbings and contacts between the bone-ligament structures of the spine towards spinal cord and subarachnoid space therefore syringomyelia. Perhaps stabilization is also necessary to prevent instability of the cervical spine at the base of central cord syndrome or syringomyelia. Finally although patients affected by central cord syndrome are usually managed conservatively we advocate, also for them, surgical treatment in cases affected by advanced state of the symptoms and MRI.

Goldenhar syndrome is a multifocal developmental disorder consisting of ocular, auricular and vertebral anomalies. A case of Goldenhar syndrome is presented with a previously undescribed association with syringohydromelia. The pertinent... more

Goldenhar syndrome is a multifocal developmental disorder consisting of ocular, auricular and vertebral anomalies. A case of Goldenhar syndrome is presented with a previously undescribed association with syringohydromelia. The pertinent literature is reviewed and possible mechanisms of the pathogenesis of syringohydromyelia in this syndrome are discussed.

A 16.5-year-old boy underwent concomitant posterior fossa decompression (PFD) for CM-I and spinal curve correction. Preop MRI evaluation revealed a CM-I, cervicothoracic syringomyelia. A thoracic kyphosis (62°) with a double thoracic... more

A 16.5-year-old boy underwent concomitant posterior fossa decompression (PFD) for CM-I and spinal curve correction. Preop MRI evaluation revealed a CM-I, cervicothoracic syringomyelia. A thoracic kyphosis (62°) with a double thoracic scoliotic curve (40°, 42°) diagnosed. A preoperative neurological examination revealed numbness in the left arm and a slight loss of motor function. Under intraoperative neurophysiological monitoring (IONM), a PFD and resection of C1 vertebra were performed before the surgical correction of the spinal deformity. Posterior pedicle screws were applied to vertebrae between T2 and L1. IONM revealed a clear improvement in the left arm following PFD. A postoperative magnetic rezonans imaging revealed a satisfactory decompression at the craniocervical junction and a significant improvement of the syrinx. Follow-up study after 3 years later showed that proximal thoracic angle was 10°, main thoracic angle was 10°, and lateral Cobb angle was 42°. Deformity correction surgery and treatment for CM-I performed in one session seems relatively new. IONM is vital in assessing a patient’s condition prior to surgical operation and changes, which occur during surgery, as well as evaluation the follow-up period. Our study showed that PFD and spinal curve correction can be conducted in a single surgical session.

Background. This prospective study, conducted in patients with Chiari I malformation (C I) related syringomyelia who underwent posterior decompression and duroplasty, utilizes radionuclide cisternography in order to study the... more

Background. This prospective study, conducted in patients with Chiari I malformation (C I) related syringomyelia who underwent posterior decompression and duroplasty, utilizes radionuclide cisternography in order to study the cerebrospinal fluid (CSF) dynamics at the foramen magnum and to predict the clinical outcome following surgery. Methods. 17 consecutive patients of C I with syringomyelia (but without hydrocephalus or fixed atlanto-axial dislocation), underwent a detailed neurological examination and were assigned a clinical disability score based on the modified Klekamp and Samii score. A radionuclide cisternography (using Tc99m-DTPA) was performed via the lumbar route and the ascent of the tracer was followed utilizing a gamma camera immediately after injection and then sequentially after 1, 2, 4, 6 and 24 hours. After posterior decompression and duroplasty, the modified Klekamp and Samii score was repeated at follow-up visits (range: 3 months to one year) along with radionuclide cisternography at 3 months, and MR imaging at 6 months. Findings. Three patterns of tracer flow were observed: a) rapid flow (n=7); b) supratentorial subarachnoid delay (n=7); and, c) foramen magnum block (n=3). The patients having foramen magnum block had the poorest clinical scores on admission. At follow up, there was an improvement in the clinical scores so that the mean scores in all three categories reached nearly the same level. Following posterior decompression, the radionuclide cisternography performed in 10 patients showed a rapid flow of the tracer without any obstruction. The syrinx resolved in 4 of the 11 patients in whom an MRI was done. Interpretation. The patients with C I with syringomyelia may often have a free flow of tracer across the FM. Posterior decompression and duroplasty provides maximum clinical relief in patients with a demonstrable foramen magnum block on radionuclide cisternography while those with a normal flow have less relief. The symptomatology related to brain-stem compression immediately responds to the surgical procedure but the syrinx-induced signs and symptoms of spinal cord dysfunction persist.

Chiari malformations (CM) include a series of congenital anomalies involving the descent of the cerebellar tonsils below the foramen magnum, which may be associated with compression in the brainstem, upper spinal cord, and cranial nerves,... more

Chiari malformations (CM) include a series of congenital anomalies involving the descent of the cerebellar tonsils below the foramen magnum, which may be associated with compression in the brainstem, upper spinal cord, and cranial nerves, consequently altering the responses of brainstem auditory evoked potentials (BAEP) and somatosensory evoked potentials (SSEP). However, only a small number of authors have described the indications of these tests in CM, and study groups to date have been small and heterogeneous. To review the results of BAEPs and SSEPs in published studies of patients with Chiari type 1 malformation (CM-1) or Chiari type 2 malformation (CM-2) as well as the indications of both tests in the diagnosis, treatment, and monitoring of both diseases, especially CM-1. We present a review article analyzing data from all published studies in Medline starting in 1966, located through PubMed, using combinations of the following keywords: 'Chiari malformation', 'Arn...

In 1998 the authors identified 5 patients with syringomyelia and no evidence of Chiari malformation Type I (CM-I). Magnetic resonance imaging of the entire neuraxis ruled out other causes of a syrinx. Ultimately, abnormal CSF flow at the... more

In 1998 the authors identified 5 patients with syringomyelia and no evidence of Chiari malformation Type I (CM-I). Magnetic resonance imaging of the entire neuraxis ruled out other causes of a syrinx. Ultimately, abnormal CSF flow at the foramen magnum was the suspected cause. The label "Chiari 0" was used to categorize these unique cases with no tonsillar ectopia. All of the patients underwent posterior fossa decompression and duraplasty identical to the technique used to treat patients with CM-I. Significant syrinx and symptom resolution occurred in these patients. Herein, the authors report on a follow-up study of patients with CM-0 who were derived from over 400 operative cases of pediatric CM-I decompression. The authors present their 12-year experience with this group of patients. Fifteen patients (3.7%) were identified. At surgery, many were found to have physical barriers to CSF flow near the foramen magnum. In most of them, the syringomyelia was greatly diminished...