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PubMed2000

Mechanisms underlying the formation and enlargement of noncommunicating syringomyelia: experimental studies.

The pathogenesis of noncommunicating syringomyelia is unknown, and none of the existing theories adequately explains the production of cysts that occur in association with conditions other than Chiari malformation. The authors' hypothesis is that an arterial pulsation-driven perivascular flow of cerebrospinal fluid (CSF) is responsible for syrinx formation and enlargement. They investigated normal CSF flow patterns in 20 rats and five sheep by using the tracer horseradish peroxidase; the effect of reducing arterial pulse pressure was examined in four sheep by partially ligating the brachiocephalic trunk; CSF flow was examined in 78 rats with the intraparenchymal kaolin model of noncommunicating syringomyelia; and extracanalicular cysts were examined using the excitotoxic model in 38 rats. In the normal animals there was a rapid flow of CSF from the spinal subarachnoid space into the spinal cord perivascular spaces and then into the central canal. This flow ceased when arterial pulsations were diminished. In animals with noncommunicating syringomyelia, there was rapid CSF flow into isolated and enlarged segments of central canal, even when these cysts were causing pressure damage to the surrounding spinal cord. Exitotoxic injury of the spinal cord caused the formation of extracanalicular cysts, and larger cysts were produced when this injury was combined with arachnoiditis, which impaired subarachnoid CSF flow. The results of these experiments support the hypothesis that arterial pulsation-driven perivascular fluid flow is responsible for syrinx formation and enlargement.

Animals↗
PubMed2000

The "presyrinx" state: is there a reversible myelopathic condition that may precede syringomyelia?

OBJECT: Alteration of cerebrospinal fluid (CSF) flow has been proposed as an important mechanism leading to the development of syringomyelia. We hypothesize that a "presyrinx" condition due to potentially reversible alteration in normal CSF flow exists and that its appearance may be due to variations in the competence of the central canal of the spinal cord. METHODS: Five patients with clinical evidence of myelopathy, no history of spinal cord trauma, enlargement of the cervical spinal cord with T1 and T2 prolongation but no cavitation, evidence for altered or obstructed CSF flow, and no evidence of intramedullary tumor or a spinal vascular event underwent MR imaging before and after intervention that alleviated obstruction to CSF flow. RESULTS: Preoperatively, all patients demonstrated enlarged spinal cords and parenchymal T1 and T2 prolongation without cavitation. Results of magnetic resonance (MR) imaging examinations following intervention in all patients showed resolution of cord enlargement and normalization or improvement of cord signal abnormalities. In one patient with severe arachnoid adhesions who initially improved following decompression, late evolution into syringomyelia occurred in association with continued CSF obstruction. CONCLUSION: Nontraumatic obstruction of the CSF pathways in the spine may result in spinal cord parenchymal T2 prolongation that is reversible following restoration of patency of CSF pathways. We refer to this MR appearance as the "presyrinx" state and stress the importance of timely intervention to limit progression to syringomyelia.

Adult↗
PubMed2000

Treatment of terminal syringomyelia in association with tethered cord syndrome: clinical outcomes with and without syrinx drainage.

OBJECT: Current use of magnetic resonance (MR) imaging has led to increased awareness of the frequency of terminal syringomyelia in patients with tethered cord syndrome. However, that the surgical treatment of terminal syringomyelia is necessary remains unclear. In this study the authors attempted to assess the clinical impact, if any, brought after syrinx decompression on the clinical outcome of tethered cord syndrome. METHODS: They randomly assigned 30 cases of pediatric tethered cord into two treatment groups: those in whom an untethering procedure was performed (Group I) and those in whom this procedure was combined with syrinx decompression (Group II). The 1-year follow-up clinical results obtained in the two groups, in correlation with MR imaging findings, were compared to evaluate the benefit of added syrinx drainage. Clinical follow-up evaluation revealed that surgical drainage of the syrinx, when combined with spinal cord untethering, resulted in better outcomes in terms of resolution of sensory deficits (p = 0.036) and bladder dysfunction (p = 0.05). The improvement in clinical outcome correlated with the radiologically documented resolution of the syrinx cavity; however, response rates of symptoms differed for each tethering subgroup. CONCLUSIONS: Preliminary results of this study indicated that terminal syringomyelia should be considered as a comorbidity that contributes to the clinical outcome of patients with tethered cord syndrome. A better clinical outcome is achieved following successful decompression of the syrinx in addition to untethering the spinal cord. These findings emphasize the importance of recognizing, evaluating, and treating this pathological entity.

Adolescent↗
PubMed2005

Prospective analysis of self-perceived quality of life before and after posterior fossa decompression in 112 patients with Chiari malformation with or without syringomyelia.

OBJECT: The purpose of this prospective study was to determine if there was a difference in the self-perceived quality of life (QOL) before and after surgery among patients with Chiari I malformations with or without syringomyelia. Most patients with Chiari I malformations report improvement in their QOL after decompression surgery; however, specific outcome data have not been empirically studied in this patient population. METHODS: One hundred seventy-two consecutive patients who underwent posterior fossa decompression based on neuroimaging evidence of a Chiari I malformation with or without syringomyelia were prospectively offered participation in the study. The Sickness Impact Profile (SIP) was chosen as the instrument for data collection. Completed questionnaires were returned by 112 patients. A statistically significant improvement (p < 0.0001) in SIP scores (self-reported QOL) was noted in 84% of participants after decompression surgery. Patient age, amount of tonsillar herniation, and evidence of syringomyelia before surgery did not correlate with or adversely affect outcomes. Among 16 participants who reported worsening in their QOL, anecdotal information revealed extraneous factors unrelated to the Chiari I malformation that they perceived as negatively influencing their outcome. Among the extraneous variables noted by the participants were general health status, unrelated injury, other illnesses, and significant stress. CONCLUSIONS: The majority of participants who underwent posterior fossa surgery for a Chiari I malformation reported significant improvement in their QOL after surgery.

Adolescent↗
PubMed1977

Terminal ventriculostomy for syringomyelia.

The clinical course of 12 patients who underwent terminal ventriculostomy for syringomyelia is presented. Opening the central canal at the tip of the conus medullaris is a relatively benign procedure that improves the symptoms of syringomyelia and syringobulbia. This canal normally terminates at the tip of the conus, but in each of the 12 surgical specimens it continued into the filum terminale for distances up to 8 cm. In most cases the tip of the conus was located more caudally than normal, indicating some degree of tethering in fetal life. This belief is supported by the fact that the newborn, whose conus is tethered to a lipoma at the sacral level, may develop syringomyelia in adult life.

Adult↗
PubMed1983

A critical appraisal of "terminal ventriculostomy" for the treatment of syringomyelia.

The clinical course of 31 patients who underwent attempted excision of the filum terminale and tip of the conus for syringomyelia is summarized. Of these patients, 17 had had some previous surgical intervention. Eleven patients were continuing to deteriorate at the time of the operation. In three cases, marked postoperative improvement was reported, and objective improvement was thought to result from the conus excision. Eighteen more patients claimed improvement in their preoperative symptoms of loss of pain sensibility, deafness, or reduced motor function, although most of such claims were unverified by objective clinical assessment. Sixteen of the patients who improved have since proceeded to deteriorate. In five of the patients, there was a long-standing improvement of at least subjective phenomena. Thirteen patients were not improved even subjectively and some of these have continued to grow worse. Proof that the greater part of the syrinx was in communication with the conus or filum was difficult to obtain even when the conus was dilated. No correlations have been found to suggest that the operation might be more effective if the central canal was patent at the conus or the filum, nor was there a correlation between a good clinical result and either the age of the patient or the age of the syrinx as judged by the history. The operation did not seem to be more or less beneficial if previous surgery had been performed. The suggestion is made that for syringomyelia with hindbrain abnormalities, other than dense arachnoiditis (particularly if there is evidence of pressure dissociation at the foramen magnum), craniovertebral decompression remains the procedure of choice. In syringomyelia with marked hydrocephalus, drainage by a valved shunt may be the preferred first procedure. If myelotomy is planned it should probably be done where the syrinx is wide, and it is more likely to succeed if the syrinx is drained to a low-pressure area outside the theca, such as the peritoneum or pleura, rather than the subarachnoid space.

Adolescent↗
PubMed2004

Retrospective study of surgery-related outcomes in patients with syringomyelia associated with Chiari I malformation: clinical significance of changes in the size and localization of syrinx on pain relief.

OBJECT: Pain is one of the major symptoms in patients with syringomyelia; however, its origin is not fully understood, and postoperative improvement of pain is difficult to predict. The objectives of this study were to assess the surgery-related results obtained in patients who underwent treatment for syringomyelia associated with Chiari I malformation, particularly related to pain status, and to identify factors that may influence improvement in postoperative pain by comparing pre- and postoperative magnetic resonance (MR) imaging findings. METHODS: The correlation between pre- and postoperative changes in the size and the location of the syrinx and pain improvement was investigated in 25 patients. The shapes of the syringes were classified into three types: central, enlarged, and deviated. In most cases in which the syrinx deviated toward the posterolateral aspect of the spinal cord at the level corresponding to dermatomal distribution of preoperative pain, the lesion remained at the same position postoperatively, and improvement in pain was poor. On the other hand, enlarged-type syringes were the most frequently observed prior to surgery, exhibited diverse changes postoperatively, and improvement in pain status was difficult to predict. When postoperative MR imaging revealed a transformation to the deviated type, poor pain improvement was noted. CONCLUSIONS: Neurons in the dorsal horn were thought to be involved in the development of pain as a result of the deafferentiation mechanism in cases of syringomyelia.

Adolescent↗
PubMed2005

Subarachnoid pressure-dependent change in syrinx size in a patient with syringomyelia associated with adhesive arachnoiditis. Case report.

The pathophysiology of syringomyelia is still not well understood. Current prevailing theories involve the assumption that cerebrospinal fluid (CSF) flows into the syrinx from the subarachnoid space through the perivascular space of Virchow-Robin. Reported here is the case of a patient with syringomyelia in which this course is clearly contradicted. This patient with a holocord syrinx associated with adhesive arachnoiditis was treated 3 years previously with insertion of a subarachnoid-peritoneal shunt and had recently experienced worsening myelopathy. On surgical exploration, the shunt system was functioning normally. The medium-pressure shunt valve was replaced with an adjustable valve with a higher closing pressure setting, thus increasing the CSF pressure in the subarachnoid space. Contrary to prevailing theories, this procedure markedly reduced the size of the syrinx. This case provides direct evidence that the syrinx size is inversely related to subarachnoid CSF pressure and supports the hypothesis that the pressure gradient across the spinal cord parenchyma is the force that generates syringes in syringomyelia.

Arachnoiditis↗
PubMed2005

Primary spinal syringomyelia. Invited submission from the joint section meeting on disorders of the spine and peripheral nerves, March 2005.

In the present review the author describes the different types of syringomyelia that originate from abnormalities at the level of the spinal cord rather than at the craniovertebral junction. These include posttraumatic and postinflammatory syringomyelia, as well as syringomyelia associated with arachnoid cysts and spinal cord tumors. The diagnosis and the principles of managing these lesions are discussed, notably resection of the entity restricting cerebrospinal fluid flow. Placement of a shunt into the syrinx cavity is reserved for patients in whom other procedures have failed or who are not candidates for other procedures.

Cerebrospinal Fluid Shunts↗
PubMedDate not supplied

[Syringomyelia: four clinical cases are reported as opportunity to reexamine the pathogenetic hypotheses (author's transl)].

In this paper four clinical cases of syringomyelia are presented as an opportunity to reexamine more discussed pathogenetic hypotheses. The authors are referring in particular to Gardner's theory since he has the merit of explaining the anomalies often associated with syringomyelia. In Gardner's opinion syringomyelia might be due to the non-perforation of the rhombic roof.

Arnold-Chiari Malformation↗
PubMed1999

[Syringomyelia in children].

From 1985 to 1997, 442 children were treated for syringomyelia. One hundred and eighty eight had syringomyelia in association with Chiari I malformation. In 65% of the case scoliosis was the initial symptom. All these children were treated by posterior fossa decompression, 95% were clinically improved or stabilized. The scoliosis remained unchanged or improved in only 44% of the cases. Many other causes of craniovertebral junction anomalies have been found (Chiari II, achondroplasia, mucopolysaccharidosis, craniosynostosis, Dandy-Walker malformation, posterior fossa cysts, birth injuries, or other causes of raised intracranial pressure). All these children were treated by posterior fossa decompression or when possible treatment of the cause. Features and results are presented. Spinal causes (diastematomyelias, lumbar lipomas, ventricule terminalis, spinal tumors ...) are presented. Whe discuss the treatment of scoliosis with specific attention to scoliosis associated to isolated syringomyelia.

Adolescent↗
PubMed1999

[Characterization of sensation disorders and neuropathic pain related to syringomyelia. A prospective study].

The present prospective study aimed to perform quantitative sensory testing (QST) in patients with painful or painless syringomyelia before and after surgical treatment of their syrinx (at 3 and 9 months). Eighteen consecutive patients with cervical or dorso-lumbar syringomyelia completed the study and 9 underwent surgery. Twelve patients had central neuropathic pain (of whom 6 were followed up). Spontaneous pain and brush-evoked allodynia were assessed. Von Frey hairs, vibrameter and a thermotest device were used to determine the mechanical-, vibratory-, thermal-detection thresholds, and the mechanical and thermal pain thresholds. Results showed evidence of deficits in temperature and pain sensibility in 17 cases, often associated with deficits in vibration and touch sensitivity (11 cases). Magnetic resonance scan, including axial images, demonstrated good correlation between paramedian extension of the syrinx and the laterality of thermal deficits. Somatosensory evoked potentials (11 patients) were abnormal in 9 cases at level, and showed good correlation with deficits in vibration. The magnitude of the thermal and tactile deficit was similar between areas of spontaneous pain and adjacent non painful areas. Surgery induced a significant decrease of tactile deficits, and to a lesser extent, of thermal deficits. Effects on neuropathic pain were positive in 3 patients (total disappearance of pain) and negligible or negative in 3 patients, despite collapse of the syrinx (in 2 cases). These results confirm that QST are useful in clinical practice to quantify the clinical results of surgery in patients with syringomyelia, and allow some hypotheses about the mechanisms of neuropathic pain in these patients.

Adult↗
PubMed1999

Diffusion-weighted MR imaging in a rat model of syringomyelia after excitotoxic spinal cord injury.

BACKGROUND AND PURPOSE: Recent experimental data have shown that an increase of excitatory amino acids and the initiation of inflammatory responses within the injured spinal cord may play a role in post-traumatic syringomyelia. The purpose of this study was to determine whether diffusion-weighted MR imaging with apparent diffusion coefficient (ADC) maps could provide earlier evidence of spinal cord cavitation in a rat model of syringomyelia than available with conventional MR imaging. METHODS: The spinal cord gray matter of four rats was injected with the alpha-amino-3 hydroxy-5 methyl-4 isoxazole propionic acid/metabotropic receptor agonist quisqualic acid. Animals were sacrificed at 1, 4, or 8 weeks after injection, and the spinal cords were fixed in formalin for 1 week and imaged with T1-, T2-, and diffusion-weighted sequences. One control specimen was also imaged. ADC maps were constructed from the diffusion-weighted data. Histopathologic analyses of sections stained with cresyl violet were compared with the MR images. RESULTS: By 1 week after injection, ADC maps at the level of injection showed areas within the gray matter of increased intensity and increased ADC values as compared with the control specimen. These bright areas corresponded to cysts or cavities within the cord parenchyma on the histopathologic sections. The ADC values within affected gray matter areas progressively increased at 4 and 8 weeks, also corresponding to cyst formation. Conventional T1- and T2-weighted images showed corresponding lesions with cystic characteristics at 4 and 8 weeks, but not at 1 week. CONCLUSION: In an animal model of syringomyelia, diffusion-weighted imaging with ADC maps detected cystic lesions within spinal cord gray matter before they were seen on conventional T1- and T2-weighted images.

Animals↗
PubMed2000

Spinal dural arteriovenous fistula associated with syringomyelia.

The previously undescribed association of a spinal dural arteriovenous fistula with syringomyelia was found in a 60-year-old male, who developed increasing paresis, numbness of both lower extremities and sphincteric dysfunction. Symptoms and signs were attributed to a syringomyelia at T5-L1 and an arteriovenous spinal dural fistula at L1. The fistula was successfully immobilised with N-butyl-cyano-acrylate. Six months after the procedure, all abnormalities had nearly disappeared. Whether the relation between the fistula and the syringomyelia was coincidental or causative could not be determined.

Angiography↗
PubMedDate not supplied

Sagittal profiles of the spine in scoliosis associated with an Arnold-Chiari malformation with or without syringomyelia.

The sagittal spine of children with Arnold-Chiari I malformation with or without syringomyelia and associated scoliosis (ACS) has been poorly studied. A retrospective review of scoliosis secondary to ACS from three centers was undertaken. Sagittal and coronal plane variables were measured from standing radiographs. There were 30 ACS children (19 girls, 11 boys) with an average curve of 50 +/- 20 degrees and age of 11.2 +/- 3.2 years. Syringomyelia was present in 26 (87%). The scoliosis was thoracic in 25, thoracolumbar in 3 and lumbar in 2; 18 curves were right and 12 were left. A positive correlation was noted between cervical lordosis (CL) and thoracic kyphosis (TK). The 30 children with ACS scoliosis were compared with 26 children with adolescent idiopathic scoliosis (AID). The ACS group had more left curves (40% vs. 0%, P = 0.0002), more boys (37% vs. 8%, P = 0.01), and was younger (11.3 +/- 3.7 years vs. 14.2 +/- 1.8 years, P = 0.004). TK and CL were increased in ACS (TK: 40 +/- 13 degrees vs. 30 +/- 13 degrees, P = 0.005; CL: 16 +/- 21 degrees vs. -5 +/- 12 degrees, P < 0.0001). The ACS and AID groups were subdivided by CL >0 degrees and <0 degrees. In the ACS group, 19% (5 of 21) had CL <0 degrees, in the AID group 77% (20 of 26) had CL <0 degrees (P = 0.0001). When cervical lordosis is >0 degrees or thoracic kyphosis is >40 degrees (Cobb method), the clinician should strongly suspect the presence of an Arnold-Chiari I malformation with or without syringomyelia.

Adolescent↗
PubMed2002

[Coexistence of Jarcho-Levin syndrome, syringomyelia and osteoporosis in 15 years old boy].

Autosomally inherited Jarcho-Levin syndrome is an illness leading to death usually in the foetal, neonatal or infantile period or in early childhood. The children rarely survive to the age of adolescence. Hardly 130 cases of the syndrome were described up to now. There are two types of the syndrome: milder spondylocostal dysplasia (SCD) and more severe spondylothoracic dysplasia (STD)--the proper Jarcho-Levin syndrome. The authors present a case of coexistence of Jarcho-Levin syndrome with syringomyelia and osteoporosis in a 15 years old boy. His disease manifested itself in developmental anomaly of structure and segmentation of vertebrae, occult cervicothoracic spina bifida and characteristic crablike deformation of thorax. These anomalies led to kyphoscoliosis and considerable shortening of trunk as well as to the growth which increased the proportions of limbs length to the trunk and body height. The syndrome was accompanied by the syringomyelia in C5-Th1 segment and osteoporotic skeleton with significantly decreased bone density (in the L2-4 segment Z = -5.17). According to the best of our knowledge the presented case is the first description of the coexistence of Jarcho-Levin syndrome, syringomyelia and osteoporosis in world literature. The anomaly diagnosed as STD has also the features of SCD. The verification of the classification principles of Jarcho-Levin syndrome may take place in future basing on bigger number of observation and on genetics development.

Abnormalities, Multiple↗
PubMed2003

[Post-traumatic syringomyelia].

INTRODUCTION: A longitudinal cavity in the medulla spinalis is known as a syringomyelia. If the cavity is a widening of the central canal, it is known as hydromyelia. Traumatic spinal cord injury may lead to the development of syringomyelia. MATERIAL AND METHODS: During the period 1959-1990 twenty patients with spinal cord injury in East Denmark (including Greenland) developed later symptomatic posttraumatic syringomyelia. RESULTS: The mean time from trauma to diagnosis was 12 years. Mean time from first symptom to time of diagnosis was three years. Thirteen patients were operated with syringoperitoneal drain, myelotomy or decompression of the spinal cord and dural reconstruction. The best effect of the operations was on pain; little or no effect was found on activities of daily living, motor function, spasticity, sensibility, and bladder and bowel function. DISCUSSION: The main objective of performing operation is to stop further development of disabling symptoms. We recommend that patients with spinal cord injury have an MRI performed three months after injury independent of recovery. If a cyst or syrinx is present or relevant clinical symptoms emerge, MRI should be performed every three months. If no cyst or syrinx or changes in neurological status develop, MRI should be performed every six months. If no changes are seen in two years, MRI should be performed every two or more years.

Activities of Daily Living↗