Idiopathic Syringomyelia Research Articles

Prevalence of neurological diseases associated with cervical pain and/or signs of cervical myelopathy in French bulldogs: a retrospective analysis of 105 cases.

French bulldogs can be affected by several neurological diseases, with myelopathies representing the most frequent cause of neurological signs. Studies focusing on the epidemiology of cervical diseases in this breed are lacking. This study aims to describe the prevalence of neurological pathologies responsible for cervical pain and/or signs of cervical myelopathy, assuming that intervertebral disc herniation represents the most common disease; a second aim was to evaluate how often different pathologies coexist in this spinal region in French bulldogs. A retrospective analysis of medical records from the database of a single referral center (AniCura I Portoni Rossi Veterinary Hospital-Zola Predosa, Bologna, Italy) was performed, including French bulldogs presented for neck pain and/or neurological deficits consistent with cervical myelopathy. Clinical and imaging data were reviewed and used as inclusion criteria. Based on the number of MRI-diagnosed diseases, the eligible dogs were divided into three groups. One hundred five French bulldogs met the inclusion criteria. The most commonly diagnosed condition was an intervertebral disc herniation (66.9%), followed by C2 idiopathic hypertrophic ganglioneuritis (15.1%), cervical syringomyelia (11.5%), congenital osseous malformations (1.4%), spinal arachnoid diverticula (1.4%), neoplasms (1.4%), steroid-responsive meningitis-arteritis (0.7%), traumatic vertebral fractures (0.7%), and other (0.7%). For the vast majority of dogs (75/105) a single pathology was diagnosed, with intervertebral disc herniations accounting for 86.7% of cases, involving C3-C4 IVD most commonly. In the remaining 30 dogs, two or three different and concurrent diseases were detected. Among these 30 dogs, intervertebral disc herniations still remained the most diagnosed condition, in combination with C2 idiopathic hypertrophic ganglioneuritis and syringomyelia in 19 and seven cases, respectively. The results of this study highlight that different pathologies can affect the cervical spinal cord in French bulldogs, with intervertebral disc herniations representing the most frequent condition, as previously described in the veterinary literature. In almost a third of cases, different pathologies can coexist at the cervical level. However, for cases in which different pathologies are present at the same time, it is not always possible to clearly establish their clinical significance.

Single-stage correction of severe scoliosis with syringomyelia: performed with traction assistance without prophylactic neurosurgical decompression.

There is still controversy about whether it is necessary to perform prophylactic neurosurgical decompression for severe scoliosis (SS) with syringomyelia (SM) to reduce the risk of neurological complications during subsequent spinal correction. This study aimed to explore the safety and effectiveness of using traction-assisted single-stage spinal correction as a treatment for patients who had SS with SM (SS-SM). The patients who had SS-SM without previous neurosurgical intervention and who underwent traction-assisted single-stage posterior spinal correction at a single center were included, and the initial, posttraction, and postoperative clinical data were reviewed. Based on preoperative MRI, the included patients were divided into two categories: those with versus those without Chiari malformation type I (CM-I-related SM [CS] vs idiopathic SM [IS]), and those with a moderate syrinx (MS) versus those with a large syrinx (LS). Different groups' traction and operation contributions were calculated for comparisons (CS vs IS, MS vs LS). A total of 28 patients were included. The initial mean major scoliosis was 101.0° with a mean flexibility of 21.4%. After the operation, the mean total correction rate for scoliosis was 63.9%. The mean traction and operation contributions were 61.5% and 38.5%, respectively. Most of the patients (75%) underwent spinal corrections without 3-column osteotomies, and only 1 patient reported postoperative regional numbness without motor deficits. No differences were found in the mean total correction rates, traction, and operation contributions when comparing CS versus IS and MS versus LS with the comparable initial clinical data (p > 0.05). More than 50% of the total corrections were achieved by preoperative traction in all groups. Traction-assisted single-stage spinal correction can safely and effectively correct SS-SM without prophylactic neurosurgical decompression under strict patient selection. Additionally, traction can achieve more than half of the final spinal correction, even for patients with varying sizes of SMs.

Syringo-Subarachnoid Shunt with Tube Versus T-Tube via the Dorsal Root Entry Zone Approach for Eccentric Syringomyelia

OBJECTIVEThe objective of this study was to compare the clinical therapeutic efficacy of syringo-subarachnoid shunt with direct tube and T-tube via the dorsal root entry zone(DREZ) approach for the treatment of eccentric syringomyelia. METHODSA retrospective study was performed on the clinical data of 41 patients associated with idiopathic or secondary eccentric syringomyelia from November 2011 to December 2022. Syringo-subarachnoid shunt with a direct tube or T-tube via the DREZ approach were conducted. The modified Japanese Orthopedic Association (mJOA) low back pain scale was used to investigate the severity of clinical symptoms. MRI was used to investigate therapeutic efficacy（reduction of the cavity volume by over 10% was considered an improvement and 50% was considered a significant improvement）. RESULTSThe incision length of the spinal cortex in the direct tube group was shorter than in the T-tube group (3.10±0.28cm vs. 5.03±0.19cm), with a significant difference between this two groups(t=-52.56, P<0.001). JOA 3 months post-surgery was significantly better than pre-operation JOA both in direct tube group（t=40.954,P<0.001） and in T-tube group（t=24.769,P<0.001）. The statistical comparison revealed there were no difference in image improvement condition between direct tube group and T-tube group 3 months post-surgery( χ2=0.20，p=0.655) and 12 months post-surgery(χ2=0.21，p=0.647). CONCLUSIONSyringo-subarachnoid shunt with direct tube via the DREZ approach for the treatment of eccentric syringomyelia is safer than with T-tube via the DREZ approach due to more minimal incision length and lesser occupying effect with same therapeutic efficacy.

Neuro-cranio-vertebral syndrome related to coccygeal dislocation: A preliminary study

BackgroundNeuro-cranio-vertebral syndrome (NCVS) includes a set of idiopathic diseases: Arnold-Chiari syndrome type 0,1 and 1.5, idiopathic scoliosis, and idiopathic syringomyelia. It is caused by the pathological traction transmitted by the filum terminale on the neuraxis. Considering that the filum terminale is inserted at sacrococcygeal level, it is logical to think that an alteration of this segment’s anatomy, such as an anterior coccyx dislocation, can increase the tension exerted by the filum terminale on the neuraxis, which in turn triggers NCVS. MethodsWe collected data from 372 patients with NCVS and 15 patients with coccygeal dislocation and NCVS from our database. We analyzed the relationship between the sacrococcygeal and intercoccygeal angles with signs, symptoms and associated diagnoses. T-tests for independent samples and linear regression were used for analysis (p <0.05). We describe the MRI findings and clinical features of NCVS caused by coccygeal dislocation and compare its prevalence with that of a sample without coccygeal dislocation. Results65% of the signs, symptoms, and imaging features were present in similar proportions in both samples. There was a similar prevalence of cerebellar tonsillar descent and scoliosis in both groups, whereas the incidence of syringomyelia differed. A sacrococcygeal angle between 89-110° prevails in cases of syringomyelia while an intercoccygeal angle between 90-140° prevails in cases with cerebellar tonsillar descent. ConclusionsThe high clinical and radiological prevalence of coccygeal dislocation in patients with Neuro-cranio-vertebral syndrome suggests an association between them. The mechanism involved in coccygeal dislocation can directly influence the development of Neuro-cranio-vertebral syndrome.

Central or axial atlantoaxial dislocation and craniovertebral junction alterations: a review of 393 patients treated over 12 years.

The authors reviewed their scientific publications and updated their clinical material obtained over the last 12 years for cases of central or axial atlantoaxial dislocation (CAAD) identified in the presence of craniovertebral musculoskeletal and/or neural alteration(s). The management implications of diagnosing and treating CAAD are highlighted. During a 12-year period, CAAD was diagnosed in 393 patients with craniovertebral junction-related musculoskeletal and neural alterations who underwent atlantoaxial fixation. No bone decompression was done. All CAAD-related craniovertebral junction structural changes were identified to have a naturally protective role. Hence, in this paper the term "craniovertebral alterations" is used for "craniovertebral junction anomalies" and the term "Chiari formation" is used instead of the commonly used term "Chiari malformation." The major radiological diagnosis was determined either singly or in cohort with one or more of other so-called pathological entities that included Chiari formation (367 cases), syringomyelia with Chiari (306 cases), idiopathic syringomyelia (12 cases), type B basilar invagination (147 cases), bifid arch of the atlas (9 cases), assimilation of the atlas (119 cases), C2-3 fusion (65 cases), Klippel-Feil alteration (4 cases), and dorsal kyphoscoliosis (15 cases). The follow-up period ranged from 6 to 155 months. Clinical improvement was observed in all patients. Understanding and treating CAAD may have significant implications in the surgical treatment of a number of clinical entities. The gratifying clinical outcomes obtained in patients after atlantoaxial fixation, without any type of decompression involving bone or soft-tissue resection, consolidate the concept that atlantoaxial instability has a defining role in the pathogenesis.

The Management of Idiopathic and Refractory Syringomyelia.

Idiopathic syringomyelia (IS) and refractory syringomyelia (RS) are types of syringomyelia that often pose a management challenge and are associated with long-term clinical sequela. They are usually an epiphenomenon reflecting an underlying pathology where the treatment of the primary cause should be the aim for any surgical intervention. In the case of IS, the initial step is agreeing on the definition of the terms idiopathic and syringomyelia. After a rigorous exhaustive clinic-radiological workup, only IS patients with progressive neurology are treated, usually unblocking subarachnoid cerebrospinal fluid (CSF) pathway obstruction somewhere in the thoracic spinal canal and reserving shunting techniques to nonresponsive cases. Similar to IS, also RS is multifactorial, and its management varies based on the initial pathology, strongly supported by radiological and clinical features. We aim to address this topic focusing on the etiopathology, investigation paradigm, and surgical pathway, formulating algorithms of management with available evidence in literature. Surgical techniques are discussed in detail.

Pattern of Syringomyelia in Presumed Idiopathic and Congenital Scoliosis.

Study DesignRetrospective study.PurposeThis study was undertaken to compare the patterns of syringomyelia in patients with presumed idiopathic and congenital scoliosis.Overview of LiteratureThe incidence of neuraxial anomalies presenting as idiopathic scoliosis ranges from 2% to 14%; the common ones are idiopathic syringomyelia (IS) and Chiari malformation type 1 (CM1) with syringomyelia. Some authors have speculated that scoliosis is caused by the asymmetrical compression of anterior horn cells by a syrinx, which causes an imbalance of the trunk musculature. In congenital scoliosis, syringomyelia is the second commonest cord anomaly, and the deformity progression depends upon the underlying vertebral abnormality, the location of the abnormality, and the age of patient, and is independent of the intraspinal anomaly.MethodsWe analyzed the radiological records of 44 consecutive patients with scoliosis and syringomyelia. Of these 44 patients, 13 had IS, 12 had CM1, and 19 had congenital scoliosis. The radiographs were evaluated to determine the curve magnitude, sagittal alignment, side of convexity, and type of vertebral anomaly, if any. T1- and T2-weighted magnetic resonance imaging of the whole spine was analyzed to determine the presence of craniovertebral anomalies, syrinx length, syrinx diameter, and syrinx-cord ratio (SCR).ResultsThe frequency of left convex curves was 26.1%, with no significant differences across the three groups. The mean length of the syrinx was 7.2±4.9 vertebral levels, and the mean SCR was 0.39±0.2. The mean syrinx length was significantly higher in patients with CM1 and IS, compared to patients with congenital scoliosis. The mean SCR was highest in patients with CM1. In congenital scoliosis, syringomyelia was seen most frequently in patients with a failure of formation, and 63.2% had concomitant cord anomalies.ConclusionsSyrinxes were smaller in size and length in patients with congenital scoliosis, and are distinct from those seen in IS and CM1.

Clinical Image of a Spinal Ependymoma Discovered 8 Years after Initial Misdiagnosis as an Idiopathic Syringomyelia

With the increasing frequency of neuroimaging, incidental intramedullary cavities are diagnosed more frequently. We present a case of asymptomatic incidental intramedullary cervical cavity diagnosed as an idiopathic syringomyelia as initial magnetic resonance imaging (MRI) showed an isolated cystic image without contrasted component. The patient had no subsequent MRI follow-up, but eventually showed symptoms 8 years later. By this stage of the disease, the MRI appearance had changed, showing a solid and enhanced component. The patient underwent surgical resection and histopathology concluded a papillary ependymoma (grade 2). This case illustrates how asymptomatic intramedullary cavities may hide an underlying tumoral process and why these cavities should not be considered as idiopathic syringomyelia by default, except after prolonged MRI follow-up.

Surgical Scoliosis Correction in Chiari-I Malformation with Syringomyelia Versus Idiopathic Syringomyelia.

Both patients with Chiari-I malformation (CIM) with syringomyelia and those with idiopathic syringomyelia (ISm) have a syrinx and can have scoliosis as well. However, there is no literature regarding differences between CIM and ISm in terms of radiographic outcomes and surgical complications after posterior fusion, to our knowledge. The aim of the present study was to compare radiographic features, clinical outcomes, and surgical complications after posterior spinal fusion between patients with CIM-associated scoliosis and those with ISm-associated scoliosis. One hundred and twenty patients with syringomyelia-associated scoliosis were retrospectively analyzed. Twenty-one patients with scoliosis secondary to CIM were enrolled and matched by sex, age, and the Cobb angle of the scoliotic curve with 21 patients with scoliosis secondary to ISm. All patients underwent 1-stage posterior fusion surgery. Coronal and sagittal radiographic parameters were evaluated before surgery, immediately after surgery, and at the final follow-up (at least 2 years). We also collected data regarding syringeal features, neurological deficits, intraoperative neuromonitoring, and complications. Sex, age, preoperative coronal/sagittal scoliosis parameters, and neurological deficits were similar between the matched CIM and ISm groups. On average, the CIM group had a longer syrinx (12.3 ± 3.6 versus 8.9 ± 4.5 vertebral levels, p = 0.010) than the ISm group. The CIM and ISm groups showed similar correction rates for primary curves (70.9% ± 10.6% versus 69.5% ± 16.3%, p = 0.739). There were no significant differences in coronal/sagittal correction, intraoperative neuromonitoring abnormalities, surgical complications, or Scoliosis Research Society-22 questionnaire scores between the 2 groups. Despite matched demographic and scoliotic coronal parameters, patients with CIM had longer syrinxes compared with patients with ISm. One-stage posterior fusion achieved comparable clinical and radiographic outcomes for both CIM- and ISm-associated scoliosis without significant differences in neurological complications. Prognostic Level III. See Instructions for Authors for a complete description of levels of evidence.

The Filum disease and the Neuro-Cranio-vertebral syndrome: definition, clinical picture and imaging features

BackgroundWe propose two new concepts, the Filum Disease (FD) and the Neuro-cranio-vertebral syndrome (NCVS), that group together conditions thus far considered idiopathic, such as Arnold-Chiari Syndrome Type I (ACSI), Idiopathic Syringomyelia (ISM), Idiopathic Scoliosis (IS), Basilar Impression (BI), Platybasia (PTB) Retroflexed Odontoid (RO) and Brainstem Kinking (BSK).MethodWe describe the symptomatology, the clinical course and the neurological signs of the new nosological entities as well as the changes visible on imaging studies in a series of 373 patients.ResultsOur series included 72% women with a mean age of 33.66 years; 48% of the patients had an interval from onset to diagnosis longer than 10 years and 64% had a progressive clinical course. The commonest symptoms were: headache 84%, lumbosacral pain 72%, cervical pain 72%, balance alteration 72% and paresthesias 70%. The commonest neurological signs were: altered deep tendon reflexes in upper extremities 86%, altered deep tendon reflexes in lower extremities 82%, altered plantar reflexes 73%, decreased grip strength 70%, altered sensibility to temperature 69%, altered abdominal reflexes 68%, positive Mingazzini’s test 66%, altered sensibility to touch 65% and deviation of the uvula and/or tongue 64%. The imaging features most often seen were: altered position of cerebellar tonsils 93%, low-lying Conus medullaris below the T12L1 disc 88%, idiopathic scoliosis 76%, multiple disc disease 72% and syringomyelic cavities 52%.ConclusionsThis is a paradigm shift that opens up new paths for research and broadens the range of therapeutics available to these patients.

Spinal subarachnoid space tapering in patients with syringomyelia.

Cervical spine tapering affects cerebrospinal fluid dynamics. Cervical spine taper ratios derived from anteroposterior diameters reportedly differ between patients with syringomyelia and controls. We attempted to verify the differences in diameter and to show differences in cross-sectional area between syringomyelia and controls. Cervical spine magnetic resonance images in syringomyelia patients (idiopathic or Chiari I related) and control patients were examined. In each subject, the anteroposterior diameter of the spinal canal was measured at each cervical level, and C1-C4, C4-C7, and C1-C7 taper ratios were calculated. Differences in taper ratio between groups were tested for statistical significance with the t-test. Cross-sectional areas of the spinal canal were measured at each cervical spinal level, and tapering was calculated. Eighteen patients with idiopathic syringomyelia, 28 with Chiari I, and 29 controls were studied. Chiari and syringomyelia patients had significantly steeper diameter-based taper ratios than controls. The dural sac areas tapered proportionally with the diameter-based taper ratio in all groups. Cervical spine anteroposterior diameter tapering and dural sac cross-sectional areas tapering differ between syringomyelia patients and controls.

Clinical manifestations and radiological characteristics in patients with idiopathic syringomyelia and scoliosis.

To clarify the clinical manifestation and radiological characteristics of idiopathic syringomyelia (IS) and to investigate the relationship between syrinx and scoliotic curves in IS-related scoliosis patients. Fifty-five patients with IS and scoliosis were identified and reviewed retrospectively from June 2009 to December 2016. Radiographic features of syrinx, scoliosis and clinical manifestations of neurological deficits were collected. The syrinx/cord (S/C) ratio was defined as the anteroposterior diameter of syrinx divided by the diameter of spinal cord at the same level. Patients were classified into two groups, the thoracic group (T group, apex vertebra located from T2 to intervertebral disk of T11-T12) and the thoracolumbar/lumbar group (TL/L group, apex vertebra located from T12 to L5). There was no correlation between the radiological features of idiopathic syrinx and scoliotic curve parameters. The TL/L group had a lower level of most caudal extent (13.7 compared with 10.6, P = 0.029) and lower level of largest S/C ratio (12.0 compared with 8.7, P = 0.016) than that in T group. The deviated side of syrinx was not coincident with major curve convexity (27.2% concordance rate, P = 0.522) or dominant side of neurological deficit (16.3% concordance rate, P = 0.212). Patients with major curves located on the thoracolumbar or lumbar spine had a much lower caudal extent and lower level of greatest S/C ratio compared to patients with major curves located on the thoracic spine. No significant relationships were detected between syrinx features, scoliotic curve parameters and neurological deficits. These slides can be retrieved under Electronic Supplementary Material.

Pain treatment and recovery of functionality in a former athlete diagnosed with myofascial pain syndrome in the course of syringomyelia. Case report

BACKGROUND AND OBJECTIVES: There are few studies that address non-surgical treatment in cases of syringomyelia, which reduces the possibilities of treatment for the patient. The objective of this study was to analyze the efficacy of the physiotherapeutic treatment for the symptoms of the pathology. CASE REPORT: Idiopathic syringomyelia is a condition in which a cystic-shaped cavity appears within the spinal cord. After the diagnosis of syringomyelia in C3-C6, 3 years ago, the patient, a former volleyball athlete, remained stable with no anesthetic dissociation, muscle atrophy or limb paresthesia. However, she began to have constant back and neck pain to the extent of limiting her functioning in jogging, volleyball, and difficulties of movement in daily routine. The physiotherapeutic treatment in 6 sessions, worked in the muscle and fascial release with dry needling and manual myofascial release associated with specific vertebral adjustments with chiropractic techniques, and after the cessation of the pain, specific muscle strengthening exercises. CONCLUSION: Physiotherapy showed to be an effective treatment for patient with syringomyelia that presented symptoms to myofascial pain syndrome.

Syrinx to Subarachnoid Shunting for Syringomyelia.

Surgery for syringomyelia generally aims to treat the underlying cause, if it is known. Optimal management is unclear for idiopathic syringomyelia, or when treatment of the putative cause has failed or is high risk. Syrinx to subarachnoid shunting is an option for these cases; a series is reported to assess the outcomes of this approach. We retrospectively analyzed the clinical and radiologic features of a consecutive series of patients with syringomyelia treated with syrinx to subarachnoid shunting. Forty-one patients (19 male, 4-79 years old) were treated from 2000 to 2016, including 15 patients with idiopathic syringomyelia, 13 with spinal trauma, 5 with Chiari malformation, 4 with arachnoiditis, 3 with tethered cord, and 1 with arachnoid bands. The patients were treated with a syrinx to subarachnoid shunt, and a subsetalso underwent expansile duraplasty. At follow-up (3-108 months, mean 36 months) syrinx size was reduced in 37 patients, and there was improvement or stabilization of symptoms in all but 1 patient. Three patients had temporary lower limb sensory symptoms after surgery. Other complications were 2 transient cerebrospinal fluid leaks, a pseudomeningocoele, and 1 postoperative myocardial infarction. Two cases of shunt dislodgement required reoperation, and a third case required early reoperation for an enlarging syrinx. There were no cases of shunt blockage or infection. Syrinx to subarachnoid shunting is a safe and effective treatment for idiopathic syringomyelia and for patients who are not suitable for, or have not responded to, other treatment.

Cervical spinal canal narrowing in idiopathic syringomyelia.

The cervical spine in Chiari I patient with syringomyelia has significantly different anteroposterior diameters than it does in Chiari I patients without syringomyelia. We tested the hypothesis that patients with idiopathic syringomyelia (IS) also have abnormal cervical spinal canal diameters. The finding in both groups may relate to the pathogenesis of syringomyelia. Local institutional review boards approved this retrospective study. Patients with IS were compared to age-matched controls with normal sagittal spine MR. All subjects had T1-weighted spin-echo (500/20) and T2-weighted fast spin-echo (2000/90) sagittal cervical spine images at 1.5T. Readers blinded to demographic data and study hypothesis measured anteroposterior diameters at each cervical level. The spinal canal diameters were compared with a Mann-Whitney U test. The overall difference was assessed with a Friedman test. Seventeen subjects were read by two reviewers to assess inter-rater reliability. Fifty IS patients with 50 age-matched controls were studied. IS subjects had one or more syrinxes varying from 1 to 19 spinal segments. Spinal canal diameters narrowed from C1 to C3 and then enlarged from C5 to C7 in both groups. Diameters from C2 to C4 were narrower in the IS group (p < 0.005) than in controls. The ratio of the C3 to the C7 diameters was also smaller (p = 0.004) in IS than controls. Collectively, the spinal canal diameters in the IS were significantly different from controls (Friedman test p < 0.0001). Patients with IS have abnormally narrow upper and mid cervical spinal canal diameters and greater positive tapering between C3 and C7.

Progressive Neurological Deficit in Adult Idiopathic Syringomyelia (IS): Case Report and Literature Review

A syringomyelia is a clinical entity of any tubular fluid-filled cavity within the spinalcord that causes slowly but relentlessly progressive symptoms as well as expansion of thecavity, which is most commonly associated with Chiari malformation Type I, which due toadvancement of imaging techniques has resulted in more incidental idiopathic syringes that arenot associated with tumor, trauma, or postinfectious causes. Idiopathic syringomyelia (IS) is apathological entity in which no overt etiology is evident for a syrinx. In this study, we describe acase in a 45 year-old woman presented with progressive difficulties in walking and also hadmyelopathic signs evidenced by hyperreflexia in the lower extremities and underwent foramenmagnum decompression and C1 lamiectomy. Idiopathic syringomyelia is a pathological entityin which no overt etiology is evident for a syrinx. It can be managed succesfully byconservative treatment but if there is a progression of neurological deficit, surgicaldecompression is a mandatory.Keywords: idiopathic syringomyelia, neurological deficit

Ciddi Nörolojik Patolojilerin Nadir Görülen Bir Kutanöz Bulgusu: Torakal Dermal Sinüs

A 39 year old adult female patient was reported with a rare combination of thoracal dermal sinus tract, diastematomyelia, idiopathic syringomyelia and multipl vertebral anomalies in this patient. Spinal dermal sinus tracts were located most frequently in the lumbar and lumbosacral regions. Thoracal localization of congenital dermal sinus tracts is considerably infrequent. Because of this unusual localization, the lesion was confused with the chronic epidermal cyst which we mostly come across in clinical practice and further radiological examination was not thought. Our case has the property of being a rare case, showing the association of thoracal dermal sinus tract with diastematomyelia, idiopathic syringomyelia and multipl vertebral anomalies without any neurolojic deficit clinically. This case showed us that severe neurological pathologies could be found under a lesion considered as a simple cyst.

Dorsal Arachnoid Web and Scalpel Sign: A Diagnostic Imaging Entity.

A case of 60-year-old woman with idiopathic syringomyelia and progressive myelopathy by dorsal arachnoid web is discussed. One focal indentation along the dorsal surface of the spinal cord at the T3 vertebral body level revealed the scalpel sign, a radiologic entity diagnostic of a dorsal thoracic arachnoid web. T2-T3 laminectomy for the resection of dorsal arachnoid band was performed. Careful sectioning of the web resulted in visual apparent relief of the compression and good functional recovery. In patients with presumed idiopathic syringomyelia, imaging studies should be closely inspected for the presence of transverse arachnoid web. This extramedullary transverse band of arachnoid tissue extends to dorsal surface of the spinal cord, resulting in mass effect and dorsal indentation, known as scalpel sign because of its apparent resemblance to a scalpel on sagittal imaging. Early diagnosis with early intervention may benefit greatly patients. Surgical resection of transverse arachnoid web is a minimally invasive procedure with low morbidity, and can result in resolution of syringomyelia and improvement in neurological function.

Pulse wave myelopathy: An update of an hypothesis highlighting the similarities between syringomyelia and normal pressure hydrocephalus

Most hypotheses trying to explain the pathophysiology of idiopathic syringomyelia involve mechanisms whereby CSF is pumped against a pressure gradient, from the subarachnoid space into the cord parenchyma. On review, these theories have universally failed to explain the disease process. A few papers have suggested that the syrinx fluid may originate from the cord capillary bed itself. However, in these papers, the fluid is said to accumulate due to impaired fluid drainage out of the cord. Again, there is little evidence to substantiate this. This proffered hypothesis looks at the problem from the perspective that syringomyelia and normal pressure hydrocephalus are almost identical in their manifestations but only differ in their site of effect within the neuraxis. It is suggested that the primary trigger for syringomyelia is a reduction in the compliance of the veins draining the spinal cord. This reduces the efficiency of the pulse wave dampening, occurring within the cord parenchyma, increasing arteriolar and capillary pulse pressure. The increased capillary pulse pressure opens the blood–spinal cord barrier due to a direct effect upon the wall integrity and interstitial fluid accumulates due to an increased secretion rate. An increase in arteriolar pulse pressure increases the kinetic energy within the cord parenchyma and this disrupts the cytoarchitecture allowing the fluid to accumulate into small cystic regions in the cord. With time the cystic regions coalesce to form one large cavity which continues to increase in size due to the ongoing interstitial fluid secretion and the hyperdynamic cord vasculature.

Treatment of selected syringomyelias with syringo-pleural shunt: The experience with a consecutive 26 cases

Summary of background dataIt is well established that syringomyelia can cause neurological symptoms and deficit by accumulation of fluid within syrinx cavities that lead to internal compression within the spinal cord. When other intervention treating the underlying etiology failed to yield any improvement, the next option would be a procedure to divert the fluid from the syrinx cavity, such as syringo-subarachnoid, syringo-peritoneal or syringo-pleural shunting. The indications and long term efficacy of these direct shunting procedures are still questionable and controversial. ObjectiveTo investigate the clinical indication, outcome and complication of syringe-pleural shunt (SPS) as an alternative for treatment of syringomyelia. Study designWe reported a retrospective 26 cases of syringomyelia were found to have indication for a diversion procedure. SPS was offered. Patients’ symptoms, mJOA score, and MRI were collected to evaluate the change of the syringomyelia and prognosis of the patients. 2-tailed wilcoxon signed-rank test was used to perform the statistical analysis of the mJOA scores. MethodsAll 26 patients underwent SPS. The clinical information was collected, the mean follow-up time was 27.4 months, 2-tailed wilcoxon signed-rank test was used to perform the statistical analysis of the mJOA scores. The key surgical technique, outcome and complications of SPS were reported in detail. ResultsNo mortality and severe complications occurred. Postoperative MRIs revealed near-complete resolution of syrinx in 14 patients, significant shrinkage of syrinx in 10 patients, no obvious reduction or unchanged in remaining 2 patient. Postoperatively, the symptoms improved in 24 cases (92.3%). Statistical analysis of the mJOA scores showed a statistical significance (P<0.001) between the preoperative group and the 2-week postoperative group. No further significant improvement between 2 weeks to the final follow up at 27 months. ConclusionCollapse or remarkable shrinkage of the syrinx by SPS could ameliorate or at least stabilize the symptoms for the patient. We recommend small laminectomy and a less than 3mm myelotomy either at PML or DREZ. The SPS procedure can be an effective and relatively long-lived treatment for the idiopathic syringomyelia and those that failed other options.