Clinical features and pathomechanisms of syringomyelia associated with spinal arachnoiditis

doi:10.1016/j.surneu.2004.05.038

Surgical Neurology

Volume 63, Issue 4, April 2005, Pages 350-355

https://doi.org/10.1016/j.surneu.2004.05.038 Get rights and content

Abstract

Background

Syringomyelia is a common intramedullary lesion associated with spinal arachnoiditis and obstruction of the foramen magnum such as in Chiari's malformation. Disturbance of cerebrospinal fluid flow around the spinal cord has an important role in the development of syringomyelia due to spinal arachnoiditis; however, the exact mechanisms have not been clarified. The purpose of this retrospective study is to understand the clinical features and pathomechanisms of syringomyelia secondary to spinal arachnoiditis and to provide the current choice of surgical treatment in this difficult clinical entity.

Methods

Clinical and radiological findings in 15 patients with syringomyelia associated with spinal arachnoiditis who underwent surgical treatment in our institutes between 1982 and 2000 were reviewed. All patients presented with paraparesis or tetraparesis on admission.

Results

Magnetic resonance imaging (MRI) or computed tomography-myelography revealed that the syrinx predominantly existed at the thoracic levels. Five patients showed complete block of the thoracic subarachnoid space by conventional myelography. T2-weighted MRI showed diffuse intramedullary hyperintensity at the level of arachnoiditis. As the first surgical treatment, 10 patients underwent syringo-peritoneal shunt placement. Three patients were treated with a syringo-subarachnoid shunt, and 2 patients were treated with a ventriculoperitoneal shunt. Eight patients required further shunting operations for syringomyelia 2 months to 12 years after the first surgery. Neurologic improvement was obtained in 9 patients (60%) with decreased size of the syrinx. One patient remained stable; 5 patients showed gradual deterioration.

Conclusions

The syrinx originated from the thoracic levels where severe adhesion of the subarachnoid space was present. The mechanisms of syrinx formation may be based on the increased interstitial fluid in the spinal cord. Shunting procedures were effective in some population of the patients. Decompression procedures of the spinal subarachnoid space may be an alternative primary surgical treatment except for patients with longitudinally extensive arachnoiditis.

Introduction

Adhesive spinal arachnoiditis is a chronic inflammatory process in the pia-arachnoid of the spinal cord. Several factors such as spinal surgery, myelography, or meningitis are known to cause adhesive arachnoiditis. In the literature, spinal arachnoiditis can be divided into 2 entities according to the etiology and clinical features. Lumbar or lumbosacral adhesive arachnoiditis is usually related to lumbar disc diseases [6], [9], [27], and radicular pain due to involvement of the cauda equina is a main symptom. By contrast, spinal arachnoiditis at the cervicothoracic level is characterized by slowly progressive myelopathy due to degenerative changes of the spinal cord [2], [16], [17]. Syringomyelia is a common intramedullary lesion in cervicothoracic spinal arachnoiditis [4], [21]. Disturbance of cerebrospinal fluid (CSF) flow around the spinal cord is considered to produce syringomyelia [5], [19]. However, the exact mechanisms of syrinx formation have not been clarified.

In the present study, we retrospectively analyzed the clinical course and radiological findings of syringomyelia in patients with spinal arachnoiditis who underwent surgical treatment in our institutes. The purpose of this study was to understand the clinical features and pathomechanisms of syringomyelia with spinal arachnoiditis and to provide the current choices of surgical treatment.

Section snippets

Materials

Fifteen consecutive patients were reviewed. They underwent surgical treatment of syringomyelia due to spinal arachnoiditis in our institutes between 1982 and 2000. There were 6 men and 9 women aged 32 to 65 years (mean, 46.9 years). The causes of spinal arachnoiditis were meningitis in 9 patients (tuberculous meningitis 6, unknown organism 3), spinal surgery (thoracic laminectomy for spinal tumor) in 2 patients, and unknown in 4 patients. Conventional myelography or computed

Clinical course

All patients showed tetraparesis (13 patients) or paraparesis (2 patients) on admission. Eleven patients were not ambulatory. Five patients showed complete motor and sensory paralysis of legs. Interval from the initial onset of paraparesis to admission to our hospital ranged from 1 to 33 years (mean, 16.3 years). The clinical courses can be divided into 2 patterns (Table 1). Five patients (cases 1-5: meningitis 4, spinal surgery 1) showed acute onset of paraparesis followed by gradual

Pathomechanisms of syringomyelia

Several authors [4], [16], [19], [21], [25], [28] proposed that the initial stage of syringomyelia in spinal arachnoiditis would be intramedullary cystic degeneration caused by ischemia due to circulatory disturbance in the pia-arachnoid. The blockage of CSF pathways around the spinal cord contribute to formation of the intramedullary cystic cavities [3]. Experimental studies indicated that the disturbed CSF flow around the spinal cord had an important role in the development of syringomyelia.

Conclusions

This study demonstrates clinical and radiological characteristics of syringomyelia associated with spinal arachnoiditis. The syrinx originated from the thoracic levels where severe adhesion of the subarachnoid space was present. The pathomechanisms of syringomyelia may be based on the increased interstitial fluid of the spinal cord at the level of arachnoiditis. Shunting procedures were effective in some population of the patients. Decompression procedures of the spinal subarachnoid space may

References (29)

M.J. Ball et al.
Pathogenesis of syringomyelia
Lancet
(1972)
R.A. Dolan
Spinal adhesive arachnoiditis
Surg. Neurol.
(1993)
K. Ohata et al.
Surgical management of syringomyelia associated with spinal adhesive arachnoiditis
J. Clin. Neurosci.
(2001)
M. Takayasu et al.
Rapid enlargement of a syringomyelia cavity following syringo-subarachnoid shunt: case report
Surg. Neurol.
(1996)
B. Bose et al.
Arachnoiditis presenting as a cervical cord neoplasm: two case reports
Neurosurgery
(1983)
T.B. Brammah et al.
Syringomyelia as a complication of spinal arachnoiditis
Spine
(1994)
L.R. Caplan et al.
Syringomyelia and arachnoiditis
J. Neurol. Neurosurg. Psychiatr.
(1990)
K.H. Cho et al.
Experimental model of posttraumatic syringomyelia: the role of adhesive arachnoiditis in syrinx formation
J. Neurosurg.
(1994)
D.G. Gomez et al.
The spinal cerebrospinal fluid absorption pathways
Neuroradiology
(1974)
D. Greitz et al.
Pathogenesis and mechanics of spinal cord cysts: a new hypothesis based on magnetic resonance studies of cerebrospinal fluid dynamics
Int. J. Neuroradiol.
(1999)

D.W. Guyer et al.

The long range prognosis of arachnoiditis

Spine

(1989)

Y. Iwasaki et al.

Syringo-subarachnoid shunt for syringomyelia using partial hemilaminectomy

Br. J. Neurosurg.

(1999)

A. Josephson et al.

A spinal thecal sac constriction model supports the theory that induced pressure gradients in the cord cause edema and cyst formation

Neurosurgery

(2001)

M.Y. Kaynar et al.

Syringomyelia—as a late complication of tuberculous meningitis

Acta Neurochir. (Wien)

(2000)

Cited by (59)

Immunotherapies in chronic adhesive arachnoiditis - A case series and literature review
2021, eNeurologicalSci
Citation Excerpt :
Additional surgical procedures include cyst fenestration, intradural exploration, syrinx drainage, shunt placement, duraplasty, myelotomy, intraventricular drain placement, discectomy, and anterior fusion. In the field of shunt placement, various types of shunts have been implanted, i.e., cystoperitoneal cystopleural, cystosubarachnoid, ventriculoperitoneal, etc. [24,58]. Surgical treatment of adhesive arachnoiditis seems to be effective in the short term, but the long-term outcome proved unsatisfactory.
Chronic spinal adhesive arachnoiditis (CSAA) is a rare condition with limited therapeutic options. Surgical treatment proves effective in approximately 60% of cases. Conservative treatment options have not been extensively investigated. Here, we report the course of the disease, analyze the effect of immune treatments in patients with CSAA who were treated in the University Hospital Essen between 2015 and 2020, and conduct a literature review. Three out of four patients showed no improvement after treatment with corticosteroids, methotrexate, or plasmapheresis. All non-responders suffered from CSAA for several years, while one patient who had a disease duration of less than one month fully recovered. It is necessary to verify whether treatment at an early stage of the disease is better than treatment after chronic adhesion manifestation, as it interrupts the development of adhesions and all subsequent complications.
Arachnoiditis – A challenge in diagnosis and success in outcome – Case report
2021, Interdisciplinary Neurosurgery: Advanced Techniques and Case Management
Spinal arachnoiditis is an insidious disease caused by an inflammatory process of the arachnoid membrane resulting from many possible causes. Several factors such as spinal surgery, myelography, or infectious meningitis are known to cause adhesive arachnoiditis. Multiple studies proposed that the initial stage of syringomyelia in spinal arachnoiditis would be intramedullary cystic degeneration caused by ischemia due to circulatory disturbance in the pia-arachnoid. Disturbance of cerebrospinal fluid flow around the spinal cord has an important role in the development of syringomyelia due to spinal arachnoiditis. The presentation of spinal arachnoiditis depends on the level of the disease. For instance, Lumbar or lumbosacral adhesive arachnoiditis is usually related to lumbar disc diseases, and radicular pain due to involvement of the cauda equina is the main symptom. Our case represents a rare cause of arachnoiditis for a patient was presented with lower limb weakness and found to have extensive syringomyelia.
29-year-old male patient, presented with 3 days history of progressive lower limbs weakness. He had 3 months history of mild lower back pain and subjective left lower limb weakness. MRI showed extensive intramedullary hyperintense lesion extending from T3 to L1 with scattered post-contrast nodular enhancement. Diagnosis of arachnoiditis with syringomyelia done. The patient underwent Thoracic 8 laminectomy, arachnoid biopsy, and syrinx to subarachnoid space shunt. Tissue culture-confirmed Staphylococcus cohnii.
Arachnoiditis is a common pathology with multiple causes. However, isolated bacterial arachnoiditis is rare and has not been mentioned in literature before to our knowledge. We present a case of arachnoiditis proven to be bacterial by tissue culture from the thickened arachnoid layer we took during the surgery after we exclude all other causes.
Management of syringomyelia associated with tuberculous meningitis: A case report and systematic review of the literature
2021, Journal of Clinical Neuroscience
Citation Excerpt :
While anti-mycobacterial drugs may sufficiently reduce arachnoiditis, preventing syrinx formation and reducing the size of existing syrinxes, this treatment is only effective during active or re-activated TB infection [3]. Thus, surgical interventions, involving shunt drainage (syringo-peritoneal, syringo-pleural, or syringo-subarachnoidal) or decompression of the central canal (arachnoid adhesiolysis, subpial suction, or duraplasty), may provide better neurological improvement [3,4]. Here, we describe the case of a 30-year-old man who presented with chronic paraparesis post-TB meningitis and, after surgical treatment, regained motor function and ambulation ability.
Determinants of tuberculosis (TB) syringomyelia, its management options and outcomes are still under investigation. The aim of this study is to present a case of TB syringomyelia with markedly improved symptoms status-post surgery and to understand the clinical characteristics and outcomes of 33 TB syringomyelia cases reported in the literature. Specifically, we examined the differences between patients who were managed medically and those who underwent surgical intervention. Inclusion criteria for the cases were (1) syringomyelia caused by TB infection rather than co-occurrence of these conditions, (2) management protocol described, and (3) post-treatment outcome described. The median age was 30 years (interquartile range (IQR): 23–40) with 55% males. The median time between TB onset to syringomyelia diagnosis was 2 years. Nineteen patients were surgically treated, 11 were medically treated, and 3 received no treatment. Twenty-one patients showed improvement in at least one prior symptom, but no patient experienced a full recovery. Those that underwent surgical intervention were more likely to have TB meningitis (95% vs. 64%, p < 0.05) upon initial TB presentation and have a greater interval between TB onset and syringomyelia presentation (median of 2.6 vs. 0.33 years, ns). A greater proportion of the surgically managed patients experienced improvement in any symptom (74% vs. 45%, ns). Future case-controlled studies with larger sample sizes are required to validate and further understand the outcomes of surgically-managed TB syringomyelia.
Syringomyelia and hydromyelia: Current understanding and neurosurgical management
2021, Revue Neurologique
Citation Excerpt :
Briefly, syringomyelia is thought to be the result of CSF flux disorders in the SAS between the cranial and the spinal compartments over the cardiac systolic/diastolic cycle. Nonetheless, the pathological mechanisms are closely related to the main etiologies of syringomyelia [10–17]. Therefore, the current classification is based on pathogenesis and distinguishes five entities, four of them corresponding to the term syringomyelia:
Syringomyelia is a rare disorder in which a fluid-filled cyst forms within the spinal cord, resulting in myelopathy. Meanwhile, the abnormal dilatation of the central canal is referred to as hydromyelia or slit-like syrinx. The most prevailing classification is based on anatomical features and pathogeny rather than pathophysiological mechanisms. It is usual to distinguish foraminal syringomyelia related mainly to abnormalities at the craniocervical junction, non-foraminal syringomyelia dealing with any cause of arachnoiditis (infection, inflammation, trauma…) and more rarely syringomyelia associated with intramedullary tumors. Although many pathophysiological theories have been argued over time, the prevailing one is that disturbances in cerebrospinal fluid (CSF) flow in the sub-arachnoid spaces disrupt flow velocity leading to the syrinx. Symptoms of paralysis, sensory loss and chronic pain commonly develop during the third/fourth decades of life. The natural history of syringomyelia is typically one of gradual, stepwise neurological deterioration extending over many years. Diagnosis is based on magnetic resonance imaging (MRI) including excellent morphological sequences (T1-, T2-, FLAIR-, T2*-, enhanced T1-) and dynamic MRI with careful study of CSF velocity (CISS, cine-MR sequences). Surgical management is at first dedicated to treat the cause of the syringomyelia, mainly to re-establish a physiological CSF pathway in the subarachnoid spaces. Mostly, the surgical goal is to enlarge the craniocervical junction with duraplasty. Other surgical strategies such as arachnoidolysis or shunt procedures are performed based on the pathogenic mechanisms or as second-line treatment. Medical treatments are also necessary as chronic pain is the main long-lasting symptom. As evolutive syringomyelia is a severe disease with a high impact on quality of life, it is recommended to treat without delay. There is no evidence for surgery for incidental asymptomatic syringomyelia or hydromyelia. Finally, syringomyelia associated with intramedullary tumors resolves spontaneously after tumor resection. Syringomyelia is a rare disease, which requires a dedicated multidisciplinary approach, emphasizing the need for a nationwide scientific organization so as to offer optimal care to the patient.
Recurrent arachnoid cysts secondary to spinal adhesive arachnoiditis successfully treated with a ventriculoperitoneal shunt
2020, Clinical Neurology and Neurosurgery
Citation Excerpt :
SAA from an infectious etiology may carry a higher risk of recurrent adhesion and associated cyst recurrence than SAA from other causes due to a high level of inflammation [12]. Mycobacterium tuberculosis was the most commonly found inciting organism among cases reported in the literature [2–5]. Perhaps the chronic nature of mycobacteria meningitis allows for a greater degree of inflammation and scarring than would be found with an acute infection such as Streptococcus meningitis [26].
Spinal adhesive arachnoiditis (SAA) with cyst formation secondary to infectious meningitis is a rare clinical entity. These cysts can compress the spinal cord and cause neurologic decline. We present a case of a patient who underwent resection for an intradural schwannoma which was complicated post-operatively by bacterial meningitis and development of several recurrent thoracic arachnoid cysts. After two separate thoracic decompressions with lysis of intradural adhesions, a permanent ventriculoperitoneal shunt (VPS) was eventually placed with complete recovery of his symptoms. Our review of the literature showed that CSF shunts have previously been successfully used to treat spinal fluid collections. Although there are many factors to consider when treating these patients, CSF diversion may be beneficial for similar SAA presentations in order to simultaneously treat and prevent recurrence of symptoms.
Safety and Efficacy of Syringoperitoneal Shunting with a Programmable Shunt Valve for Syringomyelia Associated with Extensive Spinal Adhesive Arachnoiditis: Technical Note
2019, World Neurosurgery
Although syringomyelia associated with extensive spinal adhesive arachnoiditis (SAA) can be a progressive disease that has potentially devastating clinical consequences, its surgical resolution has remained poorly defined. The aim of the present study was to verify the safety and efficacy of syringoperitoneal shunting for syringomyelia associated with extensive SAA.
The present retrospective study included 15 patients who had undergone syringoperitoneal shunting with a programmable shunt valve for the diagnosis of syringomyelia associated with extensive SAA from October 2012 to June 2018. The shunt pressure was appropriately adjusted according to the postoperative sequential clinical condition and change in syringomyelia evaluated using magnetic resonance imaging. The average postoperative follow-up duration was 32.7 months.
No surgery-related complications such as shunt dysfunction or infection occurred during the follow-up period, except for 2 patients with minor issues with the shunt tube. The average shunt pressure at the last follow-up examination was 4.5 cm H₂O. The findings from the clinical assessment suggested that the average grade on the sensory pain scale was 2.9 before surgery and had improved significantly to 2.5 at the most recent follow-up examination. Radiological analysis suggested that improvement of syringomyelia was noted in 14 of the 15 patients (93.3%), with no cases of radiological aggravation. No recurrence of syringomyelia developed during the follow-up period in the present study.
Syringoperitoneal shunting with a programmable shunt valve was safe and effective for clinical control of syringomyelia associated with extensive SAA. Long-term follow-up is mandatory to monitor for shunt dysfunction and mechanical trouble.

View all citing articles on Scopus

View full text

SpineClinical features and pathomechanisms of syringomyelia associated with spinal arachnoiditis

Abstract

Background

Methods

Results

Conclusions

Introduction

Section snippets

Materials

Clinical course

Pathomechanisms of syringomyelia

Conclusions

Lancet

Surg. Neurol.

J. Clin. Neurosci.

Surg. Neurol.

Arachnoiditis presenting as a cervical cord neoplasm: two case reports

Neurosurgery

Syringomyelia as a complication of spinal arachnoiditis

Spine

Syringomyelia and arachnoiditis

J. Neurol. Neurosurg. Psychiatr.

Experimental model of posttraumatic syringomyelia: the role of adhesive arachnoiditis in syrinx formation

J. Neurosurg.

The spinal cerebrospinal fluid absorption pathways

Neuroradiology

Pathogenesis and mechanics of spinal cord cysts: a new hypothesis based on magnetic resonance studies of cerebrospinal fluid dynamics

Int. J. Neuroradiol.

The long range prognosis of arachnoiditis

Spine

Syringo-subarachnoid shunt for syringomyelia using partial hemilaminectomy

Br. J. Neurosurg.

A spinal thecal sac constriction model supports the theory that induced pressure gradients in the cord cause edema and cyst formation

Neurosurgery

Syringomyelia—as a late complication of tuberculous meningitis

Acta Neurochir. (Wien)

Spine
Clinical features and pathomechanisms of syringomyelia associated with spinal arachnoiditis