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PubMed1995

[Hydrodynamics of syringomyelia].

Abnormality of CSF hydrodynamics is an important factor of the pathogenesis of syringomyelia. Recent development of cine-MRI visualized real time CSF movement in the syrinx or subarachnoid space. In the present study, twenty cases of syringomyelia associated with Chiari I malformation, 10 cases of syringomyelia associated with spina bifida aperta, and 10 cases of syringomyelia associated with spina bifida occulta were studied with MRI and cine-MRI. The result demonstrated that hydrodynamics of these three groups were different to each other. Chiari I malformation showed CSF hydrodynamic abnormality at the foramen magnum and marked flow void in the syrinx. On the other hand, myelomeningocele showed hydrocephalus and less prominent flow void in the syrinx. Spinal lipoma which is the majority of spina bifida occulta showed no abnormality of CSF hydrodynamics. In conclusion, the selection of treatments should be individualized depending on the hydrodynamical abnormality of each patient.

Arnold-Chiari Malformation↗
PubMed1997

Posttraumatic syringomyelia: a review of 21 cases.

A retrospective study was conducted on 21 consecutive patients with combined clinical and radiologic evidence of posttraumatic syringomyelia. Medical records and radiologic studies were reviewed to determine the following: age at injury, mechanism of trauma, spinal column injury and resultant neurologic deficit, latency period between injury and clinical manifestations of posttraumatic syringomyelia, clinicoradiologic findings of posttraumatic syringomyelia, and results of treatment. The patients were found uniformly to have sustained significant trauma (gunshot wounds, falls, or vehicular accidents) with marked neurologic dysfunction at the time of injury. Latent periods ranged from 1 month to 23 years. The most commonly presenting symptoms were radicular pain, spasticity, sensory loss, hyperhidrosis, and weakness. The most common physical findings were spasticity, hypesthesia, and weakness. Long term followup was obtained in 17 (81%) of the cases and suggested a higher rate of satisfaction among patients treated with surgical decompression of the syrinx. Radicular pain and sensory disturbance responded most predictably to surgical intervention, whereas spasticity responded least favorably. It is concluded that posttraumatic syringomyelia is a potentially disabling but treatable late complication of spinal injury, warranting a high index of suspicion among physicians who observe patients with such trauma.

Adolescent↗
PubMed1997

[Clinical evaluation on etiology and surgical outcome in syringomyelia associated with Chiari type I malformation].

Etiology of syringomyelia associated with Chiari type I malformation has been unknown. Moreover, the surgical procedure of foramen magnum decompression for this type of syringomyelia has not been standardized yet. No one procedure has been always successful, leading to many alternative procedures. The purpose of the present study is to elucidate pathway of cerebrospinal fluid into the syrinx cavity and to find out the best procedure for this disease. Fourty two patients with syringomyelia associated with Chiari type I malformation, which were diagnosed with magnetic resonance imaging (MRI), underwent surgical treatment. In all patients, craniocervical junction anomalies, cervical disc herniation and other spinal diseases were ruled out. There were 26 men and 16 women, ranging from 6 to 72 (mean: 42.3) years in age. The size, length and position of upper end of each syrinx cavity and the degree of the tonsillar herniation were measured on preoperative T1-weighted image and were compared each other. There were no significant relationship between the degree of tonsillar herniation and the size, length and position of syrinx cavity. No case showed that the upper end of syrinx cavity communicated to the 4th ventricle. The results suggest that the pathway of cerebrospinal fluid into the the syrinx cavity was not central canal from the 4th ventricle but microcanals in the spinal cord. All patients were carried out with foramen magnum decompression, which was divided into 4 groups according to the degree of decompression: 1) tonsillectomy group: 12 patients underwent subocciptital craniectomy (SOC) with patcy-graft dural plasty using lyophilized dura mater and tonsillectomy, 2) lysis group: 7 underwent SOC, dural plasty and microsurgical lysis of arachnoidal trabecula and fibrinoid filament around herniated tonsil, 3) plasty group: 17 underwent SOC and dural plasty and 4) dural group: 6 underwent SOC and removal of the outer layer of the dura mater. The mean follow-up periods were 3.7 years in tonsillectomy group, 3.6 years in lysis group, 2.3 years in plasty group, 1.8 years in dural group, respectively. Evaluation of the result following four types of surgical treatments was performed on clinical symptoms and the volume of syrinx cavity on sagittal MRI. The ratio in the area of the syrinx and spinal cord on preoperative and postoperative sagittal MRI were measured. There was no significant difference among 4 groups on the degree of reduction of syrinx in the sagittal plane as evaluated on MRI, whereas with regards to improvement of the clinical symptoms, dural group was significantly worse than the other three groups. The surgical procedure of dural plasty was clearly less invasive than those of tonsillectomy and lysis of subarachnoidal trabecula. These results suggest that we should select dural plasty as a primary surgical procedure for syringomyelia associated with Chiari type I malformation.

Adolescent↗
PubMed1978

Anterior cervical arachnoid cyst simulating syringomyelia: a case with preceding posterior arachnoid cysts.

An arachnoid cyst lying anterior to the cervical cord at level C6-7 was found in a 28-year-old woman believed to have syringomyelia. This diagnosis was based both on previous findings at laminectomy and on computerized tomography. The diagnosis of arachnoid cyst was suspected because of clinical features atypical for classical syringomyelia and a history of arachnoid cysts found during childhood. Air myelography demonstrated an extramedullary intradural mass anteriorly that proved to be an arachnoid cyst. Drainage and subtotal resection resulted in marked clinical improvement. This case illustrates the need for reevaluation when a patient with "known" syringomyelia presents an atypical clinical picture. Anterior cervical arachnoid cyst, which may accompany or succeed posterior arachnoid cysts, should be considered.

Adolescent↗
PubMed1987

Syringomyelia and cervical spondylosis: a clinicoradiological investigation.

Eighty eight patients with proven syringomyelia have been studied retrospectively to explore the relationship between syringomyelia and spondylotic degeneration of the cervical spine. When compared with age and sex matched control subjects, they showed no significant increase in incidence or severity of spondylosis or of vertebral subluxation. Duration of symptoms, degree of disability, and the pattern of analgesia or lower motor neurone lesions in the arms did not appear to be related to the development of spondylosis. The onset of moderate spondylosis was, however, earlier than in the controls, and individual cases with a remarkably early onset or unusual site and/or severity of spondylosis and subluxation were encountered, particularly following laminectomy. It was also noted that in a small number of patients the concomitant development of spondylosis contributed to clinical deterioration. It is concluded that while syringomyelia does not have a consistent, major role in the development of cervical spondylosis or vertebral luxation, it may contribute to progression of spinal degenerative disease in some patients.

Adolescent↗
PubMed1977

Familial syringomyelia: a report of four cases.

The clinical and neurological features of four siblings (2 male, 2 female) affected by syringomyelia are described. A fifth sister was affected by an acoustic neurinoma. Since neither parent showed signs of syringomyelia, this is considered to be a datum substantiating the dysembryogenetic theory of the syringomyelia syndrome.

Adult↗
PubMed2000

Surgical management of syringomyelia-Chiari complex.

Great variety exists in the indications and techniques recommended for the surgical treatment of syringomyelia-Chiari complex. More recently, magnetic resonance (MR) imaging has increased the frequency of diagnosis of this pathology and offered a unique opportunity to visualize cavities inside the spinal cord as well as their relationship to the cranio-cervical junction. This report presents 18 consecutive adult symptomatic syringomyelia patients with Chiari malformation who underwent foramen magnum decompression and syringosubarachnoid shunting. The principal indication for the surgery was significant progressive neurological deterioration. All patients underwent preoperative and postoperative MRI scans and were studied clinically and radiologically to assess the changes in the syrinx and their neurological picture after surgical intervention. All patients have been followed up for at least 36 months. No operative mortality was encountered; 88.9% of the patients showed improvement of neurological deficits together with radiological improvement and 11.1% of them revealed collapse of the syrinx cavity but no change in neurological status. None of the patients showed further deterioration of neurological function. The experience obtained from this study demonstrates that foramen magnum decompression to free the cerebro-spinal fluid (CSF) pathways combined with a syringosubarachnoid shunt performed at the same operation succeeds in effectively decompressing the syrinx cavity, and follow-up MR images reveal that this collapse is maintained. In view of these facts, we strongly recommend this technique, which seems to be the most rational surgical procedure in the treatment of syringomyelia-Chiari complex.

Adult↗
PubMed2001

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.

Adult↗
PubMed2003

Chiari I malformation related syringomyelia: radionuclide cisternography as a predictor of outcome.

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.

Adolescent↗
PubMed1998

Transverse microincisions of the outer layer of the dura mater combined with foramen magnum decompression as treatment for syringomyelia with Chiari I malformation.

Numerous surgical procedures have been proposed for treatment of syringomyelia associated with Chiari I malformation, but the optimal treatment has not yet been uniformly standardised. The main aim of the surgical treatment of syringomyelia/Chiari I complex is directed toward restoration of physiological cerebrospinal fluid dynamic at the craniovertebral junction. We report the surgical results of eight patients, affected by syringomyelia and Chiari I malformation, age range from 18 to 62 years, treated by bony foramen magnum decompression combined with transverse microincisions of the outer layer of the dura mater. In an average postoperative follow-up period of two years neurological symptoms and signs improved in seven patients. Postoperative Magnetic Resonance showed a decrease in size of the syrinx in seven patients. These results suggest that foramen magnum decompression combined with transverse microincisions of the outer layer of the dura 1) is an effective and safe treatment option for syringomyelia and Chiari I malformation, 2) corrects the circulatory disturbances of cerebrospinal fluid dynamic, 3) leads to a decrease in size of the syrinx and to a significant improvement in neurological signs and symptoms, 4) avoids complications of intradural approaches and syringosubarachnoid shunting.

Adult↗
PubMed1998

The acquired Chiari malformation and syringomyelia following spinal CSF drainage: a study of incidence and management.

Firstly, 14 patients are described who developed either an acquired Chiari malformation (ACM) alone (7 cases) or ACM and syringomyelia (7 cases) after lumbar subarachnoid space (SAS) shunting or in one case, epidural anaesthesia with SAS penetration. Four groups are considered: 3 cases with craniofacial dysostosis and communicating hydrocephalus (CH), 4 cases with CH alone, 3 cases with pseudotumour cerebri (PTC) and a miscellaneous group (4 cases). Initial treatment was varied: resiting the shunt to ventricle or cisterna magna [6], adding an H-V valve [1], syrinx shunting [4] and posterior fossa decompression [3]. Further treatment was required in 6 cases. Secondly, incidence was examined in 87 patients with PTC initially treated either by lumbar SAS shunting [70] or cisterna magna shunting [17]. In the first sub-group, 11 cases (15.7 per cent) developed an ACM, 3 symptomatic (as above) and eight asymptomatic with 1 case also having syringomyelia whereas 1 case occurred in the second group with a questionanably symptomatic ACM. While accurate for symptomatic lesions, these figures are tentative with respect to asymptomatic lesions due to inadequate pre-treatment radiology and detailed MR follow-up. The main conclusions are, first, that the incidence of symptomatic ACM and/or syringomyelia is not high enough to warrant abandoning SAS shunting; second that asymptomatic lesions need not necessarily be treated and third, that when treatment is required, shunt resiting is the first choice.

Adolescent↗
PubMed1998

Syringomyelia associated with type I Chiari malformation. A 21-year retrospective study on 75 cases treated by foramen magnum decompression with a special emphasis on the value of tonsils resection.

The purpose of the present study is to evaluate retrospectively the effects of several intra-operative manipulations on the results of foramen magnum decompression (FMD) in patients having syringomyelia associated with type I Chiari malformation. Seventy-five patients having syringomyelia associated with Chiari I malformation were operated on between 1975 and 1996. This population was grouped into 4 subgroups according to the surgical protocol: group I = 42 patients with FMD alone; group II = 16 patients with FMD and third ventricle shunting; group III = 9 patients with FMD and syringosubarachnoid shunting (SSS); group IV = 8 patients with FMD and cerebellar tonsils resection. Pre- and postsurgical magnetic resonance imaging (MRI) studies were analyzed (and compared). Nine patients were lost to follow-up. The results were evaluated in the 66 remaining patients (mean follow-up: 52 months), using the Bidzinski's outcome scale (ref). Two patients (3%) died postoperatively, 31 (47%) had very good results (after additional surgery in 7), 16 (24.2%) had good results (after additional surgery in 7) and 17 (25.7%) had poor results despite further surgery in 9. A total of 27 reoperations were undertaken after primary FMD in 23 patients (35%). Thirty-nine patients (59%) had both pre- and postsurgical MRI evaluation. In 28 (72%) the syrinx had markedly decreased whereas it had remained stable in 11 (28%). Clinical results were not significantly different between the patients of groups I, II and III. Very good or good results were obtained in 24 patients (64.8%) of group I (after additional surgery in 10), in 8 (61.5%) of group II (after additional surgery in 1) and in 7 (87.5%) of group III (after additional surgery in 3). Results in group IV were as follows: 7 patients (87%) had very good results and one had a good result. With a mean follow-up of 28 months, no patient required additional surgery. Postsurgical MRI syrinx reduction was observed in all 8 patients either in the early postoperative course or on delayed followup. It is suggested that tonsils resection might enhance the results of FMD in individuals having Chiari I-related syringomyelia.

Adolescent↗
PubMed1999

Expansive suboccipital cranioplasty for the treatment of syringomyelia associated with Chiari malformation.

In order to treat syringomyelia associated with adult type Chiari malformation, the authors developed a method of expansive suboccipital cranioplasty (ESC) that involves enlarging the small posterior fossa to obtain a sufficient flow of cerebrospinal fluid (CSF). The relative effectiveness of ESC with the obex plugged and not plugged was also examined, as well as other factors influencing the operative results. Twenty patients without arachnoid adhesion at the major cistern underwent ESC without opening the arachnoid membrane at the major cistern. After surgery, all improved with no recurrence and CSF flow study using magnetic resonance (MR) imaging showed significant improvement of the flow at the major cistern. Another 20 patients without arachnoid adhesion also underwent ESC but with obex plugging. Sixteen improved and one displayed only temporary improvement with recurrent syringomyelia due to postoperative arachnoid adhesions. The remaining three showed no change in spite of shrinkage of the syrinx on postoperative MR imaging. These three patients had displayed pre-operative symptoms over an approximately 10-year period involving almost the entire axial plain of the spinal cord, and presented a large syrinx before surgery. In 4 patients with arachnoid adhesions, all required intra-arachnoid procedures in addition to ESC. Intra-arachnoid procedures are not necessary to facilitate restoration of CSF flow in patients without arachnoid adhesions, because ESC can release the CSF flow blockage in the major cistern even without plugging of the obex. An associated arachnoid adhesion at the major cistern or a long-standing syringomyelia with irreversible damage of the spinal cord results in a poor operative prognosis. When posterior fossa surgery fails, insufficient decompression or postoperative arachnoid adhesions at the major cistern as the cause of treatment's failure should be evaluated by CSF flow studies using phase contrast MR imaging.

Adult↗
PubMed2002

Regression of symptomatic syringomyelia after resection of posterior fossa tumour.

Syringomyelia associated with posterior fossa tumours is a very infrequent combination of pathological entities. The few cases which have been reported generally were asymptomatic in respect of the spinal cavitations. The authors report on a 36-year-old woman with a large extradural posterior fossa epidermoid tumour with a concomitant holocord symptomatic syringomyelia. Some of her symptoms were clearly attributed to the intraspinal cavitation. The lesions were both diagnosed by magnetic resonance imaging (MR). The patient did well after surgery of the brain lesion, with an objective improvement in her neurological status and a complete resolution of the syrinx documented by the MR 7 months after tumour removal. Syringomyelia in this case could be explained by blockage of the cerebrospinal fluid (CSF) circulation at the foramen magnum which in turn resulted in cranio-spinal pressure dissociation. This led to an accumulation of extracellular fluid (ECF) in the central canal, starting cavitation. Consequently, the syrinx was slowly expanded by the long-standing "slosh" effect of the systolic pressure waves. However, also via a distortion mechanism within the posterior fossa a pathologically plugged obex could have contributed to syrinx formation by means of preventing drainage of fluid from the ventricular CSF system.

Adult↗
PubMed2003

Syringomyelia following Listeria meningoencephalitis: report of a case.

A case of symptomatic syringomyelia which appeared six years after Listeria meningoencephalitis is described. Chronic spinal arachnoiditis, as shown by standard MRI and dynamic phase contrast (PC) cine-MRI, may occur after spinal infection and is likely the cause of syringomyelia. To our knowledge, there are no previous reports of delayed spinal complications following Listeria monocytogenes infection. The possibility of developing syringomyelia should be always considered in any patient with a history of central nervous system infection.

Alcoholism↗
PubMed2004

Circumferential decompression of the foramen magnum for the treatment of syringomyelia associated with basilar invagination.

Posterior fossa decompression utilizing suboccipital craniectomy and duraplasty remains the standard surgical treatment for Chiari-associated syringomyelia. In the presence of basilar invagination, anterior decompression, typically transoral odontoidectomy, or posterior decompression may be performed. We report two cases in which anterior and posterior (circumferential) decompression of the foramen magnum was used to treat cervical syringomyelia successfully. These cases demonstrate that circumferential decompression of the foramen magnum may be necessary in some cases of cervical syringomyelia associated with basilar invagination and Chiari malformation.

Adult↗
PubMed2004

Acute brainstem dissection of syringomyelia associated with cervical intramedullary neurinoma.

Intramedullary tumors and syringomyelia typically present with slowly progressing deficits. More rarely, they are characterized by acute presentation or worsening, at times mimicking other more common etiologies. The acute onset of syringomyelia is most likely attributable to an acute increase in cerebrospinal fluid and epidural venous pressure that results in impulsive fluid movement and, ultimately, in the rupture of the syrinx and dissection into the spinal cord or brainstem. Reported here is a case of acute presentation of a small cervical intramedullary neurinoma due to the upward dissection of its associated syrinx. Critical questions are: (1) how can a small tumor produce a large syrinx? and (2) in the absence of craniospinal interferences, which mechanism underlies the acute expansion of the cavity, resulting in a rapid onset? The authors examined the pathophysiology of syrinx formation and enlargement in intramedullary tumors and reviewed the literature, emphasizing the relationship between spinal cord movements and intramedullary pressure. On the basis of current pathogenetic concepts, the authors concluded that tumor-related syringomyelia might be caused by an association of mechanisms, both from within (obstruction of perivascular spaces; increase in extracellular fluid viscosity due to the tumor itself; intramedullary pressure gradients among different cord levels and between the cord and the subarachnoid space) and from without (the cerebrospinal fluid entering the tissue). All these factors may be amplified, as in the reported case, by a tumor located dorsally at the cervical level. Abnormal postures of the spine, such as a prolonged and excessive flexed neck position, may ultimately contribute to the acute dissection of the syrinx.

Acute Disease↗
PubMed1980

Experimental communicating syringomyelia in dogs after cisternal kaolin injection. Part 1. Morphology.

Intracisternal kaolin injection in dogs usually produces hydrocephalus and out of 16 such dogs 11 have shown enlargement of the central canal of the spinal cord which has proceeded to syringomyelia-like cavities in 7 instances. The morphology of the cavities is described, the absence of ischaemic changes in the cord noted and the occasional communication of the cavities with the spinal subarachnoid space at the level of the filum terminale confirmed. The similarities to human syringomyelia are noted together with important differences in the etiology. The dogs all had arachnoiditis, all had hydrocephalus and all had a radiologically demonstrable communication of the cavities with the 4th ventricle, findings which are unusual in human syringomyelia.

Animals↗